The canadian biliary atresia registry

The Canadian Biliary Atresia
Registry
Abstractor's Manual
December 2014
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This manual describes the data elements that are collected as part of the Canadian Biliary Atresia
Registry (CBAR). It is intended to be a reference for participating site investigators, data abstractors, and
researchers who are involved in the collection and use of CBAR data.

The Canadian Pediatric Hepatology research group (CPHRG) and the Canadian Association of Pediatric
Surgeons Network (CBAR), the two well-established networks in Canada involved with the care of
children with biliary atresia, have assembled to develop the registry. Both CBAR and CPHRG have well
established track records for national data collection, data management, education, and research. The
establishment of a national BA registry will afford opportunity to prospectively evaluate the assessment
and outcome of BA in Canada. It will also allow for the development of uniform protocols, the
introduction of novel strategies and the capacity for knowledge translation to optimize the current
outcomes of Canadian children with BA.
The specific aims of CBAR are to:
Follow prospectively the current assessment, management and outcome of biliary atresia in Canadian children. To collect epidemiologic data of BA in Canada in a uniform fashion To standardize the surgical Kasai Procedure and the post Kasai management for BA in Canadian To provide a framework to assess the feasibility and cost effectiveness of novel diagnostic or therapeutic strategies in Canadian children with biliary atresia To promote education and research for Canadian children with BA through knowledge translation with local and national foundation and agencies Ethical approval must be obtained from each site's institutional review board (IRB) prior to collecting data. Biliary atresia is a rare congenital disease ( 25 cases/year across Canada). It is critical that CBAR captures 100% of Canadian patients with the disease. CBAR requires patient consent to be obtained prior to collecting data. The optimal time to obtain consent is during the first admission to hospital for the Kasai procedure. This hospital admission will normally be at least one week long. If data collection continues until the patient is mature enough to provide assent, assent should be obtained in addition to parent/guardian consent. Signed consent and assent forms should be stored separately from data in a locked filing cabinet in a locked room. March 2014 Version 1.0 Page 3 of 50 All data collection will be conducted by medical chart review. Abstraction should occur at the bedside or in the clinic both to maximize the amount of available information (i.e., while you have the option of asking attending clinicians to clarify confusing or incomplete chart entries) and to save the time involved in tracking down medical records once the patient has left the hospital. Data should be recorded from the chart either directly into REDCap or onto a data collection form, which is then entered into REDCap. The data collection forms may be filled out by the abstractor or by the site investigator. If the abstractor has difficulty finding information, they should request help from the site investigator. The Operative Data forms should be provided to the operating surgeon prior to surgery. Ideally, the form should be included in the patient's chart immediately following referral so that the operating surgeon can fill out the form in the OR. Forms should also be made available in the surgery department and operating room for emergency cases. CBAR abstractors are expected to record the information as it is reported in medical records, with no interpretation. In cases where the reported data is unclear and interpretation is needed, the abstractor should ask the CBAR site investigator(s) to help understand what data to record for a specific variable. During orientation and training, abstractors will be taught how to read through charts to extract CBAR variables as they are recorded. Abstractors are responsible for abstracting every patient that meets the following criteria: All infants diagnosed with Biliary Atresia and treated at one of the 15 study sites. Patients initially diagnosed and/or treated outside of Canada who later receive treatment at one of the study sites. The site investigator is responsible for notifying the abstractor when a patient has been referred to the site for biliary atresia. Once a patient has been deemed eligible for the study and the patient's parents/guardians have provided informed consent, abstractors are responsible for following the patient for the duration of the time they receive care at the institution, until death, liver transplant, or 10 years following initial diagnosis (whichever occurs earliest). If you are aware that one of the patients cared for at your site is being transferred to another Canadian site, please contact the coordinating centre. March 2014 Version 1.0 Page 4 of 50

Each site is expected to maintain a password protected tracking log linking the name and MRN of the patient to their unique study ID. The tracking log should be a password protected Excel spreadsheet on a password protected computer in a secure area at your site. Please take the utmost care to protect patient confidentiality. Sites must also maintain a list which assigns a number to each of their surgeons (starting at 1). This list must also be in a secure location so that data is not lost. The tracking sheet and surgeon code should be backed up every time new information is added so that data is not lost. The backup should be stored in an equally secure location. Once information is added to either of these sheets, it should be maintained until the study is complete. Do not delete a surgeon or a patient if they leave the site. The CBAR data collection can be conceptualized as three sections: Baseline and Operative Data:
 Demographics  Referral  Tests by CBAR consultant  Operative Data Immediate Post-Operative Data:
 Immediate Post-Operative Data Prior to Discharge Post-Operative Follow-up Data:
 Post-Operative Data: 1 month, 3 months, 6 months, 12 months, 2 years of age, 3 years of age, 4 years of age, and 5 years of age  Unscheduled Post-Operative Data Forms to be updated repeatedly:
 Transplant  Summary Abstractors should make every possible attempt to locate all required variables and should contact either the site's Principle Investigator or the National Coordinating Centre when a variable is routinely difficult to locate and/or report. In some cases, the abstractor may need to have the site investigator ask the consulting physician for values that they have not reported in the chart. When an abstractor cannot locate the data or when the data is not available leave the field blank or select "not specified" when that March 2014 Version 1.0 Page 5 of 50

option is available. Blank fields will be interpreted as missing values. Once a form has been labeled ‘complete', it will be understood that any blank fields could not be located. Most numeric entries need not be rounded. For those numeric entries that need to be rounded for entry, abstractors should adhere to these guidelines: 2.4 and smaller should be rounded to 2; 2.5 and larger should be rounded to 3. Generally, if values are listed as "<", as in "<2", score as one less than what is written, e.g. <2 would become 1 (or 1.9 in the case where an integer is not required). March 2014 Version 1.0 Page 6 of 50

Log in to the REDCap database (https://redcap.pediatrics.ubc.ca) and select "CBAR Canadian Biliary Atresia Registry" under "My Projects". A new record first needs to be created. Click "Add/Edit Records" under "Data Collection" on the left hand side of the screen.
Click "Add new record" and a new study ID will be generated automatically.
Note: The record will not be saved unless you enter some data and click "save record". See below.
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Once you have generated a study ID for the patient, record this ID along with the patient's name and MRN in your site tracking log. The tracking log should be a password protected Excel spreadsheet on a password protected computer in a secure area at your site. Please take the utmost care to protect patient confidentiality. Patients will typically be followed in the database for several years. Each time the patient visits the CBAR site, you will be required to fill out one or more "forms" in REDCap. To find a patient record, select "Record Status Dashboard". This will open a record status dashboard for all records at your site. The record status dashboard allows you to see which forms/events you have started, completed, and have yet to complete. Click the circle icon of the form you wish to enter data into.
After entering data into a form, click the "Save Record" button at the bottom of the screen.
**Warning: If you do not click the "Save Record" button, your work will be
erased.
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The field at the bottom of the screen labeled "Complete" allows you to mark the form as incomplete,
unverified, or complete. The answer you select here will result in a different colour icon being displayed
on the dashboard. This allows you to easily see the status of each form. Select "unverified" once the
form is complete, but still needs to be checked over. Select "complete" once you have completed the
form and checked it for completion and accuracy, and all blank fields are intended to be blank. Once
you have marked the form as complete, the National Coordinating Centre will "lock" the
form.
After a record is marked "complete", the National Coordinating Centre will "lock" the form, preventing accidental changes and allowing the Coordinating Centre to perform data checks. Once a form has been locked, only the National Coordinator will be able to unlock the form. Please contact the coordinator if you need to make changes to a locked form. The Calendar function can be found on the left-hand side of the REDCap screen under ‘Applications'. This tool should be used to create a schedule of expected follow-up visits for each patient. The schedule will ensure that the abstractor completes the forms for each follow-up visit in a timely manner. Please use this tool rather than any personal calendars you may have for confidentiality purposes. To enter an event on the calendar, navigate to the day of the event and click ‘+ New'. The following screen will appear: March 2014 Version 1.0 Page 9 of 50 Enter the form that is to be filled in that day in the ‘Notes' section and select the study ID of the patient from the drop-down. Click ‘Add Calendar Event'. The computer program will have several error checking systems in place. At a primary level, the program will perform error checks during data entry, to help ensure accurate data capture. For instance, there are checks for "reasonableness". For example, if a value of '66:66' is entered for time, the program will not allow the value to be entered, requiring the abstractor to enter the correct value before proceeding. There are also checks for consistency between forms. Abstractor's can use the "Complete?" tab at the bottom of each form as a self-check. Select "unverified" once the form is complete, but still needs to be checked over. Select "complete" once you have completed the form and checked it for completion and accuracy, and all blank fields are intended to be blank. The national coordinating centre will "lock" the form once it is marked as complete so that no further changes can be made. An additional error check will be performed by the National Coordinating Centre after the record has been locked. Hence, abstractors may be contacted by the CBAR National Coordinator to confirm unusual entries. In addition to computer checks and National Coordinating Centre checks, abstractors should take responsibility for everything that they enter into the program. Therefore it is crucial that data entered into the program makes sense to the abstractor. If the data does not make sense or is undecipherable, abstractors should take questions to the unit staff and/or Study Coordinator in order to protect the data's integrity. If you created a record in error, you can delete the record by clicking the ‘Delete Record' form at the bottom of each form. March 2014 Version 1.0 Page 10 of 50 Selecting the ‘Delete Record' button will delete the ENTIRE record, including all forms, so ensure that you do not click it by accident. If you have any questions or comments regarding a particular field for any of your patients, you can create a comment for the CBAR coordinator to respond to by clicking on the speech bubble next to every question. The bubble will turn yellow once a comment has been entered. Computer requirements for running REDCap include:  Internet connection  Supported internet browsers: Internet explorer 7, 8, or 9; Google Chrome, and Firefox. o REDCap works most efficiently with Google Chrome; however, it also works well with Firefox. If you are using Internet Explorer, use the newest version possible, otherwise REDCap may be slow. March 2014 Version 1.0 Page 11 of 50 Complete the baseline and operative data forms following the confirmed diagnosis of biliary atresia, which will occur during surgery (biliary atresia cannot be diagnosed without surgery). Since study subjects cannot be definitively identified until after surgery, ensure that surgical data sheets are available both in the charts of suspected biliary atresia patients and in the operating room for unexpected cases. Demographics

Demographics Information
Gender
Indicate whether the patient is male or female. Mother's Ethnicity Arab (Egyptian, Iraqi, Lebanese, Maghrebian, Moroccan, Palestinian, Syrian, and Other Arab) Black (African Black, Barbadian, Cuban, Ethiopian, Ghanaian, Haitian, Jamaican, Other Caribbean, Other West Indian, Puerto Rican, and Somali) Aboriginal Inuit Aboriginal North American Indian Latin American (Argentinean, Brazilian, Chilean, Columbian, Ecuadorian, Guatemalan, Hispanic, Mexican, Nicaraguan, Peruvian, Salvadoran, and Other Latin, Central and South American) Chinese (Chinese, Mongolian, Tibetan) Filipino Korean Japanese Pacific Islander (Fijian, Polynesia, and Other Pacific South Asian (Bengali, Gujarati, Punjabi, Tamil, East Indian, Bangladeshi, Pakistani, Singhalese and Sri-Lankan) South East Asian (Vietnamese, Burmese, Cambodian, Laotian, Thai, Malay, and Indonesian) West Asian (Afghan, Armenian, Iranian, Israeli, Kurdish, Turk, and West Asian) British Origin (English, Irish, Scottish, Welsh and Other Eastern European (Baltic Origins, Byelorussian, Czech, Slovak, Hungarian [Magyar], Polish, Romanian, Russian and Ukrainian) French Origin (Acadian, Franco-Manitoban, Franco- Ontarian, French, French Canadian and Quebecois) February 2014 Version 5.1.0.1 Page 12 of 50 Northern European (Finnish and Scandinavian Origins) Southern European (Balkan Origins, Cypriot, Greek, Italian, Maltese, Portuguese and Spanish) Western European (Austrian, Belgian, Dutch [Netherlands], Flemish, German, Luxembourg and Swiss) If other, specify in the box below. Father's ethnicity Arab (Egyptian, Iraqi, Lebanese, Maghrebian, Moroccan, Palestinian, Syrian, and Other Arab) Black (African Black, Barbadian, Cuban, Ethiopian, Ghanaian, Haitian, Jamaican, Other Caribbean, Other West Indian, Puerto Rican, and Somali) Aboriginal Inuit Aboriginal North American Indian Latin American (Argentinean, Brazilian, Chilean, Columbian, Ecuadorian, Guatemalan, Hispanic, Mexican, Nicaraguan, Peruvian, Salvadoran, and Other Latin, Central and South American) Chinese (Chinese, Mongolian, Tibetan) Filipino Korean Japanese Pacific Islander (Fijian, Polynesia, and Other Pacific South Asian (Bengali, Gujarati, Punjabi, Tamil, East Indian, Bangladeshi, Pakistani, Singhalese and Sri-Lankan) South East Asian (Vietnamese, Burmese, Cambodian, Laotian, Thai, Malay, and Indonesian) West Asian (Afghan, Armenian, Iranian, Israeli, Kurdish, Turk, and West Asian) British Origin (English, Irish, Scottish, Welsh and Other Eastern European (Baltic Origins, Byelorussian, Czech, Slovak, Hungarian [Magyar], Polish, Romanian, Russian and Ukrainian) French Origin (Acadian, Franco-Manitoban, Franco- Ontarian, French, French Canadian and Quebecois) Northern European (Finnish and Scandinavian Origins) Southern European (Balkan Origins, Cypriot, Greek, Italian, Maltese, Portuguese and Spanish) Western European (Austrian, Belgian, Dutch [Netherlands], Flemish, German, Luxembourg and Swiss) March 2014 Version 1.0 Page 13 of 50 If other, specify in the box below. Provide the first 3 digits of the mother's postal code Provide the mother's province of residence Alberta British Columbia Manitoba New Brunswick Newfoundland and Labrador Northwest Territories Nova Scotia Nunavut Ontario Prince Edward Island Quebec Saskatchewan Yukon Use the calendar function to enter the infant's date of birth. Gestational age (GA) Enter the completed weeks gestation of the infant at birth. (Ex. if the infant is gestational age 34 weeks + 6 days, enter 34) Enter the birth weight of the baby in grams. Gestational History Gravida: Indicate the number of times the mother has been
pregnant, including the pregnancy with the CBAR patient Para: Indicate the number of pregnancies the mother has carried
beyond 20 wks gestation, including the pregnancy with the CBAR
patient
Abortions: Indicate the number pregnancies that were lost for any
reason.
Maternal Factors
Maternal age at delivery of child
Enter in completed years Prenatal U/S done Indicate if a prenatal ultrasound was done. If yes, complete the questions that appear below. Gestational age Indicate the infant's GA at the time of the ultrasound. If more than (GA) at U/S one ultrasound, provide information about the ultrasound where an abnormality was detected. Give the date of the ultrasound where the abnormality was detected. If more than one ultrasound, provide information about the ultrasound where an abnormality was detected. Indicate whether the ultrasound was abnormal. If yes, specify the abnormality in the box below. Hepatobiliary Indicate whether a hepatobiliary abnormality was abnormality detected in the abnormal ultrasound. If yes, specify in the box below. Family history of Liver disease Indicate whether there is a history of family liver disease. March 2014 Version 1.0 Page 14 of 50 If yes, briefly describe in the box below. Indicate whether the infant had any illness between birth and referral. Please include ANY illness recorded (ie. diarrhea, flu, neonatal unit admission after birth, etc.) If yes, briefly describe in the box below. Gestational diabetes Indicate if the mother had gestational diabetes during the pregnancy. Maternal infectious illness Indicate if the mother had an infectious illness during the pregnancy. If yes, select all that apply: UTI: Urinary tract infection Gastro: Gastro-intestinal infection Other: Other infection If other, specify in the box below. Premature rupture of membranes Indicate if premature rupture of membranes occurred (ie. rupture of the membrane of the amniotic sac and chorion more than 1 hour before onset of labor). Indicate if the mother smoked at all during the pregnancy. Do not record for mothers who smoked prior to being pregnant, but do record for those mothers who smoked while unaware they were pregnant, although they may have stopped once they became aware of their pregnancy. Record smoking even if described as occasional only. Indicate if the mother drank alcohol during the pregnancy. Do not record for mothers who drank alcohol prior to being pregnant, but do record for those mothers who drank alcohol while unaware they were pregnant, although they may have stopped once they became aware of their pregnancy. Record use of alcohol even if described as occasional use only. Indicate if the mother used illicit substances during the pregnancy. Do not record for mothers who used prior to being pregnant, but do record for those mothers who used while unaware they were pregnant, although they may have stopped once they became aware of their pregnancy. Record use of illicit drugs even if described as occasional use only. Natural products or traditional Indicate if the mother took any natural products or traditional medications during the pregnancy. Include any non-medically prescribed products. March 2014 Version 1.0 Page 15 of 50 Referral
Referral Date
Enter date when letter was written or fax was received. If referral occurred before the infant's birth, enter the date of birth here. Age at referral (days) Enter the age in days of the patient on the day the referral letter was sent. Date of first CBAR Enter the date of the first appointment at the CBAR site. consultation Patient age at first CBAR Enter the patient's age in days at the first CBAR consultation. consultation (days) Weight at first CBAR Enter the weight of the patient at the first CBAR consultation in consultation (kgs) Height at first CBAR Enter the height of the patient at the first CBAR consultation in cms. consultation (cms) Breast fed Enter whether the patient was breast fed at the time of their first CBAR consultation. Enter whether the patient was formula fed at the time of their first CBAR consultation. Is the stool colour normal or abnormal? If abnormal, complete the questions that appear below. As of the first CBAR consultation date, indicate how many days had passed since the onset of pale stool colour (to the best recollection of the parent). This may be noted in the referral letter. Indicate the colour at the onset of abnormal stool colour: White Pale yellow Pale green Grey Other If other, briefly describe in the box below. Physical Exam at First Consultation
The questions below refer to the physical exam performed at the first CBAR consultation.
Jaundice
Indicate if the baby has jaundice (ie. a yellowish pigmentation of the skin, the whites of the eyes, and other mucous membranes caused by increased levels of bilirubin in the blood). Enter the date of jaundice onset using the calendar function. jaundice onset Onset of Enter how many days had passed between date of jaundice onset and the first consultation date. Indicate whether the liver is palpable. If yes, answer the questions that appear below. Indicate whether the consistency of the liver is firm. March 2014 Version 1.0 Page 16 of 50 Specify the location of the palpable section of liver as: Right Left Midline Indicate whether the spleen is palpable Specify whether the baby has ascites (ie. an accumulation of fluid in the peritoneal cavity.) Cardiac Abnormalities Does the infant have any cardiac abnormalities? If yes, briefly describe in the box below. Other dysmorphic features Does the infant have any other dysmorphic features not noted above? If yes, briefly describe in the box below. Laboratory studies pre-consultation
This section should include only results on tests ordered prior to consult with the physician at the CBAR
site.
Hgb
Hemoglobin (gm/L) White blood cell count (10 9/L) Platelet count (giga/L; also written as 10 9/L) International normalized ratio Dipstick urine normal Indicate whether dipstick urine was normal If not, specify in the box below. Aspartate Aminotransferase Alanine transaminase Alkaline phosphatase Gamma-glutamyl transpeptidase Total Bili (µmol/L) Conj Bili (µmol/L) Conjugated bilirubin Was conjugated bilirubin Indicate whether conjugated bilirubin levels were tested for in the first requested in the first 2-3 2-3 weeks of life. weeks of life? U/S Indicate whether an ultrasound was done prior to consult. If yes, answer the questions that appear below. Enter the date of the ultrasound using the calendar function. U/S Normal Indicate whether the ultrasound was normal or abnormal. If abnormal, complete the questions that appear below. Liver echotexture Indicate whether the liver echotexture was normal or abnormal in the U/S. If not specifically mentioned, leave blank. Indicate whether the gallbladder was visualized in Indicate if the patient fasted prior to the U/S. Select ‘don't know' if this was not recorded in the ultrasound report and the patient did not know whether they were fasted when asked during the March 2014 Version 1.0 Page 17 of 50 CBAR consultation. Indicate whether the extrahepatic ducts were ducts visualized visualized in the U/S. Indicate whether the intrahepatic ducts were ducts visualized visualized in the U/S. Indicate whether the spleen is large. This may be referred to in the U/S report as splenomegaly. Indicate whether polysplenia was identified in the U/S. If not noted in the report, select ‘no'. Polysplenia is a congenital diseases manifested by multiple small accessory spleens Indicate whether heterotaxia was identified in the U/S. If not noted in the report, select ‘no'. Heterotaxia = an abnormal arrangement of organs Indicate whether the SMA/SMV (Superior mesenteric artery/vein) orientation was normal, abnormal, or not specified. Indicate whether preduodenal portal vein was identified in the U/S. Preduodenal portal vein = a rare vascular anomaly in which the portal vein passes ventral to the duodenum and the head of the pancreas Indicate if triangular cord sign was identified during the ultrasound. Triangular cord sign is a triangular or tubular echogenic cord of fibrous tissue in the porta hepatis Indicate if any cystic lesions were identified during the ultrasound. A cystic lesion is a closed cavity or sac that is lined with epithelium. It may or not contain a liquid or semisolid material. Indicate the location where the U/S was Your hospital: the CBAR hospital or a geographically linked hospital.* Outside clinic: any non-hospital setting. Pediatric University Centre: Any university health centre with a pediatric unit. Adult University Centre: Any university health centre without a pediatric unit. March 2014 Version 1.0 Page 18 of 50 Community Hospital: Any hospital that does not fall into the above categories. *In some cities, hospitals may be geographically linked, but serve in different functions. For example, the site where surgical care occurs may not be the same site as where gastroenterological care occurs; however, the two sites are considered linked in some way. Indicate whether any other tests were performed prior to the CBAR consultation. If yes, indicate one or more of the following: Alpha-1-antitrypsin TORCH screen: a group of blood tests checking for several different infections Urine C&S: urine culture and sensitivity TSH: thyroid stimulating hormone Other If other, specify the test in the box below. March 2014 Version 1.0 Page 19 of 50 Tests by CBAR Consultant
The "Tests by CBAR Consultant" page should include only data on tests ordered by the consultant at the
CBAR site.

Laboratory tests performed
Were laboratory tests ordered by the physician at the CBAR site? If yes, enter results: Hgb: Hemoglobin; gm/L WBC: White blood cell count; 10 9/L Platelets: Platelet count; giga/L or 10 9/L INR: International normalized ratio AST: Aspartate aminotransferase; IU/L ALT: Alanine transaminase; IU/L ALP: Alkaline phosphatase; IU/L GGT: Gamma-glutamyl transpeptidase; IU/L Total Bili: Total Bilirubin level µmol/L Conj Bili: Conjugated Bilirubin level µmol/L Was an ultrasound performed by the physician at the CBAR site? Use the calendar function to enter the date the ultrasound was performed Indicate whether the ultrasound was normal or abnormal. If abnormal, complete the questions that appear below: Liver echotexture Record whether the liver echotexture was normal or abnormal. If not specifically mentioned, leave blank. Gallbladder visualized Indicate whether the gallbladder was visualized in the U/S. Was patient fasted Indicate whether the patient fasted prior to U/S. Select ‘don't know' if this was not recorded in the ultrasound report and the patient did not know whether they were fasted when asked during the CBAR consultation. Extrahepatic ducts Indicate whether the extrahepatic ducts were visualized in the U/S. Intrahepatic ducts Indicate whether the intrahepatic ducts were visualized in the U/S. Indicate whether the spleen is large. This may be referred to as splenomegaly in the U/S report. Indicate whether polysplenia was identified in the U/S. If not noted in the report, select ‘no'. Polysplenia is a congenital diseases manifested by multiple small accessory spleens March 2014 Version 1.0 Page 20 of 50 Indicate whether heterotaxia was identified in the U/S. If not noted in the report, select ‘no'. Heterotaxia = an abnormal arrangement of organs SMA/SMV orientation Indicate whether the SMA/SMV (Superior mesenteric artery/vein) orientation was normal, abnormal, or not specified. Preduodenal portal vein Indicate whether preduodenal portal vein was identified in the U/S. Preduodenal portal vein = a rare vascular anomaly in which the portal vein passes ventral to the duodenum and the head of the pancreas Triangular cord sign Indicate if triangular cord sign was identified during the ultrasound. Triangular cord sign is a triangular or tubular echogenic cord of fibrous tissue in the porta hepatis Indicate if a cystic lesion was identified during the ultrasound. A cystic lesion is a closed cavity or sac that is lined with epithelium. It may or not contain a liquid or semisolid material. Indicate whether a Hepatobiliary Iminodiacetic Acid (HIDA) scan (also known as a cholescintigraphy scan) was performed. If yes, complete the questions that appear below. Use the calendar function to indicate the date of the HIDA scan. Indicate whether the child was given phenobarbital prior to the HIDA Indicate whether HIDA uptake was normal or delayed. Indicate whether the results of the HIDA scan are consistent with biliary atresia. If the results were inconclusive, select uncertain. Pre-Kasai Liver biopsy Indicate whether a liver biopsy was performed prior to the Kasai procedure. If yes, complete the questions that appear below. Use the calendar function to enter the date the biopsy was performed. Bx date Bile duct Indicate if bile duct proliferation was noted in the liver biopsy. March 2014 Version 1.0 Page 21 of 50 Indicate if bile plugging was noted in the liver biopsy. Indicate if giant multinucleated cells were noted in the liver biopsy. multinucleated cells Findings Were the liver biopsy findings consistent with biliary atresia? If not, specify the other findings or diagnoses below. Pre-Kasai Percutaneous Was a percutaneous cholangiogram (imaging of the bile duct) performed prior to the Kasai procedure? If yes: Use the calendar function to indicate the date of the pre-Kasai percutaneous cholangiogram. cholangiogram date Pre-Kasai Indicate whether the percutaneous cholangiogram results were normal/abnormal or whether the cholangiogram was unsuccessful. cholangiogram result Were any other tests performed by the physician at the CBAR site? If yes, briefly describe in the box below. March 2014 Version 1.0 Page 22 of 50 Operative Data
Fill out this form with information from the patient's initial surgery. The initial surgery is performed with
the intention to confirm the diagnosis of biliary atresia and most often to perform the Kasai procedure.
In rare cases, the surgeon will not perform the Kasai Procedure during the surgery and instead will
immediately list the patient for transplant. Data entered here should be obtained from the surgical
worksheet, which should be filled out by the surgeon in the operating room. Please ensure the
operating surgeon has the worksheet prior to surgery, either by placing it in the chart of the patient with
suspected biliary atresia, or by making it available in the operating room. The surgical form should be
filled out during surgery even if consent has not yet been obtained; however, the abstractor must not
obtain the completed surgical form until after consent has been obtained.
Date of surgery
Enter the date of the surgery. Surgery duration Indicate how long the surgery was (in minutes). Surgery start time Indicate the surgery start time (24 hour clock), defined as the time of the first incision. If laparoscopy followed by an open Kasai, time starts at the start of laparoscopy. Surgery end time Indicate the surgery end time (24 hour clock), defined as the time of application of dressing. Operating Surgeon Indicate whether the surgeon(s) who performed the surgery was: Faculty Trainee Unknown Attending surgeon (coded id) Each site should maintain a coded list of the surgeons at their site. Enter the coded identifier for the attending surgeon here. Operative findings
Biliary atresia splenic
Indicate whether the infant has Biliary atresia splenic malformation malformation syndrome syndrome. If yes, answer the question that appears below. Indicate which of the following were found during surgery. Indicate more than one as appropriate: Polysplenia: a congenital disease manifested by multiple small accessory spleens Malrotation: a type of obstruction caused by abnormal development of the intestines. Situs Inversus: a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. Preduodenal portal vein: preduodenal position of the portal If other, describe below. Liver consistency Specify the consistency of the liver observed during surgery: Normal Firm March 2014 Version 1.0 Page 23 of 50 Indicate which of the following qualities were observed in the gall bladder during surgery. Select more than one option as appropriate. Absent: the patient has no gallbladder Fibrotic: the gallbladder is fibrotic Patent Lumen: the gallbladder has a patent lumen (open Clear/Mucous (ie. white bile): the gallbladder contains colourless fluid/mucous (also referred to as white bile). Biliary atresia type Indicate the type of biliary atresia identified during surgery with
reference to the image above:
1 – Patency of common hepatic duct with atresia of CBD 2 – Patency of left and right hepatic ducts with atresia of CHD and CBD 3a – Patency of GB, cystic and common bile duct with atresia of CHD 3b – Fibrotic cord with no patent ducts (most common) Other If other, describe below. Procedure
Initial laparoscopic exploration
Indicate whether laparoscopic exploration was performed prior to surgery. With laparoscopic portal Indicate whether the portal dissection was performed laparoscopically. If yes, answer the question below. Converted to open Indicate whether the surgery was converted to open. Intraoperative cholangiogram Indicate whether an intraoperative cholangiogram was performed: Not attempted: no cholangiogram was attempted Attempted, but no lumen or extravasation only: a cholangiogram was attempted, but was not successful because the common bile duct had no lumen or the dye was injected into extravascular space rather than the common bile duct. March 2014 Version 1.0 Page 24 of 50 Yes, injection: The cholangiogram was successful, dye was injected into the common bile duct Liver ligament mobilization Indicate the liver ligament mobilization as: None Partial Extracorporeal (triangular ligaments and falciform) Use of diathermy near portal Indicate whether diathermy was used near the portal plate: None Monopolar: current passes from one electrode near the tissue to be treated (the portal plate) to another fixed electrode elsewhere in the body. Bipolar: both electrodes are mounted on the same device, current passes only through the tissue to be treated (near the portal plate) Division of portal plate Indicate whether the division of the portal plate is: Flush with liver Through Glisson's capsule Extent of portal dissection of Indicate the extent of portal dissection with reference to the image above. Select all that apply: A-Standard dissection between right and left portal vein B-Small PV branches to caudate lobe divided and peritoneal reflection opened to "free" PV bifurcation from caudate and expose Glisson's capsule posterior to PV C-Right extended portal dissection D-Left extended to rex fossa (with or without division of Indicate whether the roux en y loop was: Antecolic March 2014 Version 1.0 Page 25 of 50 Retrocolic Length of Y limb Indicate the length of the Y limb: 25-30cm: greater than or equal to 25cm, and equal to or less than 30cm 30-35cm: greater than 30cm, but less than 35cm 35-40cm: greater than or equal to 35cm, and less than or Antireflux valve Indicate whether an antireflux valve was implanted. If yes, indicate the type below. Indicate whether the Kasai procedure was performed during portoenterostomy)? surgery (in rare cases, the surgeon will close without performing the Kasai procedure and directly list the patient for transplant). If yes, complete the questions that appear below: Use the calendar function to enter the date the Kasai was performed (this should be the same date as the date of surgery above). Enter the age of the infant in days when the Kasai procedure was performed. Indicate whether the hepatic portoenterostomy (also referred to as Portoenterostomy a Kasai portoenterostomy) was: Interrupted single layer Other If other, specify below. Absorbable Sutures Indicate whether the sutures used were absorbable. Indicate the size of the sutures used in the procedure: 3-0 PDS 4-0 PDS 5-0 PDS 6-0 PDS Other If other, describe in the box below. Indicate whether the sutures were monofilament or braided. Other sutures, describe If the fields above do not describe the sutures used, please describe them here. Frozen section of fibrous cone Indicate whether a frozen section of fibrous cone was sent for rapid pathology report. If yes, describe the findings below. Liver biopsy for permanent Select whether a liver biopsy was performed during the surgery. Select the type(s) of liver biopsy performed. Select more than one option if applicable: Wedge: A tissue sample is taken during surgery. Core: Also known as a core needle biopsy. Tissue is obtained using a needle. Indicate whether any tissue or serum samples were sent to be March 2014 Version 1.0 Page 26 of 50 stored for future research during the surgery. If yes, briefly describe the samples stored in the box below. Estimated blood loss (mLs) Enter the estimated volume of blood lost during the surgery in mLs. Intraoperative transfusion Indicate whether an intraoperative transfusion was performed. Indicate whether bile was seen during the surgery. Indicate whether a bile or abdominal drain was placed. Other operative findings or Describe any other operative findings or comments not listed March 2014 Version 1.0 Page 27 of 50 Immediate Post-Operative Data
The "Immediate post-operative data" form should include data from the patient's hospital stay
immediately following their Kasai procedure. The form should be filled out after the patient's discharge
date. Based on the discharge date, the expected dates of the follow-up appointments should be added
to the calendar function in REDCap as a reminder for the abstractor (se
Admission date
Use the calendar function to enter the date of admission to the CBAR site for the surgical stay. The date of admission may be prior to the date of surgery. Use the calendar function to enter the date of discharge for the surgical stay from the CBAR site. Days from operation to Calculate the number of days the infant was in hospital from the surgery date to the discharge date (=surgery date – discharge date). Weight at discharge (kgs) Enter the infant's weight at discharge in kilograms. Height at discharge (cms) Enter the infant's height at discharge in cms. Indicate whether the Kasai procedure was performed. If yes, complete the questions that appear below: Select the hospital the Kasai procedure was performed at. If other, enter the name of the hospital below. Use the calendar function to enter the date the Kasai procedure was performed. Indicate the infant's age in days on the day of the Kasai procedure. Indicate whether the infant received total parenteral nutrition (intravenous feeding, including proteins such as primene and/or fats/neutralipids) during the immediate post-surgery hospital stay. Record the first day on which the infant received Total Parenteral Nutrition (TPN). Record the last day on which the infant received Total Parenteral Nutrition (TPN). If the infant was discharged on TPN, enter the
discharge date as the last TPN date.
Enter the last day only if TPN is stopped for more than 72 hours (3
days). If TPN is stopped and started again within 72 hrs, do not record
this date, but record the next date in which TPN was stopped for
greater than 72 hrs. Conversely, if TPN is stopped for more than 72
hrs and then restarted again, record the last TPN date as the date TPN
was stopped the first time (for more than 72 hrs).
Enteral nutrition Indicate whether the infant received enteral nutrition during the
immediate post-surgery hospital stay. Enteral feeds include EITHER
oral OR gavage feeding using tubes to deliver feeds (e.g., nasogastric
(NG), oro-gastric (OG), naso-duodenal (ND), naso-jejunum (NJ) or via
gastrostomy (G-tube)).
March 2014 Version 1.0 Page 28 of 50 Record the date in which enteral feeds are established. Score the date regardless of the feeding regime (i.e. feeds may be ad lib, q1h, q2h, q3h, q4h or continuous).
Note that this item does not include "sham feeds," defined as feed
with NG still on suction without the intent to provide true enteral
nourishment. If still unclear, please ask your site investigator.
Note that this item does not require that the infant receives purely
enteral feeds.
Indicate whether the infant was fed breast milk during the hospital stay. Select ‘yes' regardless of whether the infant received breast milk orally or through a feeding tube. Indicate whether the infant was fed formula during the hospital stay. If yes, name formula type in the box below. Select ‘yes' regardless of whether the infant received breast milk orally or through a feeding tube. If yes, describe the type of formula below. Indicate whether steroids were administered to the infant during the hospital stay (ex. cortisol (solucortef), dexamethasone (decadron), hydrocortisone, methylprednisolone (solumedrol) and prednisone.) If yes, complete the questions that appear below: Use the calendar function to enter the date steroids were first Enter the starting dose of the steroid in mg/kg/day Provide a brief description of the steroid tapering protocol (ie. explain how dosing was withdrawn) tapering protocol Indicate whether antibiotics were administered/prescribed during the immediate post-surgery hospital stay. Use the calendar function to indicate the date antibiotics were first given during the hospital stay. started IV antibiotics Indicate whether antibiotics were administered by IV during the hospital stay. If yes, describe the antibiotics given in the box below. Provide the name of the antibiotic and the dose. Indicate whether the infant was discharged on any oral antibiotics. If yes, complete the question that appears below: Indicate which oral antibiotics the infant was prescribed at discharge: Septra Keflex March 2014 Version 1.0 Page 29 of 50 Indicate whether the infant was prescribed URSO (ursodiol) during the hospital stay. Enter the date that URSO was first administered. Enter the dose the infant was receiving at discharge (mg/kg/day). Vitamins at discharge Indicate if the infant was receiving any vitamins at discharge. Enter the form of vitamin D (ex. D2, D3) and the daily dose (in Units) the infant was receiving at discharge. Enter the daily dose (in Units) of vitamin E the infant was receiving at discharge. Enter the daily dose (in mg) of vitamin K the infant was receiving at discharge. Enter the daily dose (in Units) of vitamin A the infant was receiving at discharge. Indicate if other drugs were administered/prescribed during the hospital stay. If yes, name the drugs in the box below. Indicate whether the infant developed ascites (an accumulation of fluid in the peritoneal cavity) during the hospital stay. Diuretics at discharge Indicate if the infant was prescribed diuretics (ex. Lasix, Spironolactone) at discharge from the hospital. If yes, state the name of the diuretic(s) in the box below. Date of last bilirubin test prior Use the calendar function to enter the date of the last bilirubin test prior to discharge. Last total Bili prior to discharge Enter the total bilirubin level measured in the last bilirubin test prior to discharge (µmol/L). Last conjugated Bili prior to Enter the conjugated bilirubin level measured in the last bilirubin test prior to discharge (µmol/L). Other complications Indicate if there were any other complications noted during the hospital stay. If yes, briefly describe in the box below. March 2014 Version 1.0 Page 30 of 50 Post-Operative Data
Fill in the post-operative data form each time the child visits the CBAR site for a regularly scheduled
post-operative follow-up visit. Forms are provided on REDCap for visits at 1 month, 3 months, 6 months,
and 12 months post-surgery, and 2 years, 3 years, 4 years, and 5 years of age. There is another form for
a visit at an undefined time. At each regularly scheduled follow-up visit, select the form closest to the
post-surgery time (1, 3, 6, and 12 months) or age (2, 3, 4, and 5 years) the child is at the visit.
Visit date
Enter the date of the follow-up visit. This is an automatically calculated field showing the patient's age in months. If the value appears to be incorrect, check that the date of birth and visit date are correct. Enter the child's weight in kilograms at the follow-up visit Enter the child's height in cm at the follow-up visit Indicate whether the child has experienced growth failure.
Use the MELD/PELD chart below to determine whether the child has
experienced growth failure.
The chart on the left is for males, the chart on the right is for females.
The first two columns in each chart indicate the age range. The other
two columns indicate the height and weight at which a child is
considered to have experienced growth failure in each age range.
Find the age range the child falls under and if EITHER their height OR
weight is equal to or less than the corresponding numbers, indicate
that they have experienced growth failure. See image of chart below
as seen on REDCap.
March 2014 Version 1.0 Page 31 of 50 March 2014 Version 1.0 Page 32 of 50 Vaccination up to date Indicate whether the child's vaccinations are up to date. Select "don't know" if this is not noted in the chart or if it was noted as unknown by the parent/guardian. Indicate if the child had been taking any vitamins as of the appointment date. If yes, complete the questions that appear below: Indicate if the child had been taking vitamin A as of the appointment date. If yes, indicate their current dose in IU/day below. Indicate if the child had been taking vitamin D as of the appointment date. If yes, indicate their current dose in IU/day below. Indicate if the child had been taking vitamin E as of the appointment date. If yes, indicate their current dose in IU/day below. Indicate if the child had been taking vitamin K as of the appointment date. If yes, indicate their current dose in mg/day below. Indicate if the child had been taking steroids (ex. cortisol (solucortef), dexamethasone (decadron), hydrocortisone, methylprednisolone (solumedrol) and prednisone) as of the appointment date. If yes, describe the type of steroid and their current dose in the box below. Indicate if the child had been taking antibiotics as of the appointment date. If yes, describe the type of antibiotic and their current dose in the box below. Indicate whether the patient had been taking URSO (ursodiol) as of the appointment date. If yes, enter their current dose in mg/kg/day in the box below. Naturopathic/herbal agents Indicate if the patient had been taking any naturopathic or herbal agents as of the appointment date. This includes any non-medically prescribed products. Indicate if the patient was being fed orally at the time of the appointment. If yes, describe whether the patient is being breast-fed or formula-fed below. If formula fed, enter the type of formula. Indicate if the patient was being fed through a tube at the time of the appointment. This includes gavage feeding using tubes to deliver feeds (e.g., nasogastric (NG), oro-gastric (OG), naso-duodenal (ND), naso-jejunum (NJ) or via gastrostomy (G-tube)). If yes, describe whether the patient is being breast-fed or formula fed. If formula-fed, enter the type of formula. Indicate whether the patient was receiving total parenteral nutrition March 2014 Version 1.0 Page 33 of 50 at the time of the appointment (intravenous feeding, including proteins such as primene and/or fats/neutralipids). Indicate whether jaundice was noted at the appointment: New: Jaundice appeared since the previous follow-up form was filled out (or since referral if this is the first follow-up). Persistent: The patient currently has jaundice and jaundice was also noted in the previous follow-up form (or at referral if this is the first follow-up). None: The patient does not currently have jaundice. If new, complete the questions that appear below. Use the calendar function to enter the date of onset of jaundice. of jaundice Onset of Enter how many days the patient has had jaundice as of the appointment date. Indicate whether the patient's stool is: Normal: The patient's stool colour has been consistently normal since the previous follow-up form was filled out (or since referral if this is the first follow-up). Abnormal: The patient's stool colour has been consistently abnormal since the previous follow-up form. Fluctuating: The patient's stool colour has fluctuated between normal and abnormal since the previous follow-up form. Indicate if hepatomegaly (enlarged liver) was noted at the appointment. Indicate whether the consistency of the liver is firm. Indicate the location of the enlarged portion of the liver. Select more than one option if appropriate: Right Left Midline Indicate if splenomegaly (enlarged spleen) was noted at the appointment. Indicate if ascites (accumulation of fluid in the peritoneal cavity) was noted at the appointment. Features of portal hypertension Indicate if features of portal hypertension (high blood pressure in the portal vein system) were noted during the appointment (ex. large spleen, veins on abdomen wall, ascites). Other complications Indicate if other complications were noted during the appointment. If yes, briefly describe in the box below. March 2014 Version 1.0 Page 34 of 50 Lab Tests
Enter results for lab tests ordered at the follow-up appointment.
Date of last bilirubin test
Use the calendar function to indicate the date of the last bilirubin test prior to the follow up. Total bilirubin (µmol/L) Conjugated bilirubin (µmol/L) Conjugated bilirubin normal Indicate whether the patient's conjugated bilirubin is within the normal range Serum total bilirubin Indicate whether the patient's serum total bilirubin has normalized. If yes, indicate the date that the serum bilirubin was found to be normal below. Albumin level (gm/L) Hemoglobin (gm/L) White blood cell count (10 9/L) Platelet count (giga/L; equivalent to 10 9/L) Blood urea nitrogen (BUN; µmol/L) Creatinine (µmol/L) International normalized ratio Aspartate Aminotransferase (IU/L) Alanine transaminase (IU/L) Alkaline phosphatase (IU/L) Gamma-glutamyl transpeptidase (IU/L) Indicate whether the patient's 25 OH vitamin D level is: Low Normal High Not done/not available Indicate whether the patient's vitamin E level is: Low Normal High Not done/not available Indicate whether the patient's vitamin A level is: Low Normal High Not done/not available O2 saturated in room air Indicate whether the patient's blood oxygen saturation in room air is: <=90%: less than or equal to 90% 90-95%: greater than 90% and equal to or less than 95% >95%: greater than 95% Not done/not available Indicate if any other tests were done. If yes, describe in the box below. March 2014 Version 1.0 Page 35 of 50 Indicate if an ultrasound was ordered at the appointment. Enter the date of the ultrasound using the calendar function. Indicate if the liver echotexture was normal or abnormal. echotexture Intrahepatic Indicate whether the intrahepatic ducts were visualized in the U/S. ducts visualized Large spleen Indicate whether the spleen was noted to be large in the U/S. Polysplenia Indicate whether polysplenia was identified in the U/S. If not noted, assume that it was not identified. Polysplenia is a congenital diseases manifested by multiple small accessory spleens Indicate whether heterotaxia was identified in the U/S. If not noted, assume that it was not identified. Heterotaxia = an abnormal arrangement of organs Cystic lesion Indicate if a cystic lesion was identified in the liver during the ultrasound A cystic lesion is a closed cavity or sac lined with epithelium, containing a liquid or semisolid material. If yes, provide any details about the lesion in the box below. Signs of varices or portal Indicate whether the ultrasound showed signs of varices (abnormally dilated vessels) or portal hypertension. Portal hypertension can be indicated by ascites, large spleen, or reversal of flow. Sometimes signs of portal hypertension will be noted in the U/S report. This field is automatically calculated. The field requires Age and
Growth failure to be filled in. If the patient is less than 144 months of
age (12 years), the field also requires Total bilirubin, INR, and Albumin
to be filled in.
PELD Calculation:
If the child is less than 144 months (12 years) of age, the calculation is:
10 x (0.48log(total bilirubin) + 1.857log(INR) - 0.687log(albumin) +
0.436 (if the child is less than 12 months)) + 0.667 (if the child has
experienced growth failure)
If the child is 144 months or older and has experienced growth failure,
PELD = 0.667
If the child is 144 months or older and has not experienced growth
failure, PELD = 0
This field is automatically calculated. The field requires AST and
platelets to be filled in.
March 2014 Version 1.0 Page 36 of 50 APRI Calculation: ((AST/45)*100)/platelets (10 9) This field is automatically calculated. It will only be calculated for
children 144 months (12 years) of age or older. For children less than
12 years of age, the field will contain a 0. The field requires Age, Total
Bili, Creatinine, and INR to be filled in.
MELD Calculation:
3.78log(Total Bili) + 11.2log(INR) + (9.57log(creatinine)/88.4) + 6.43
Transplant referral? Indicate whether the patient had been referred for liver transplant by their treating physician as of the follow-up appointment. If yes, enter the date of the referral below. Listed for transplant? Indicate whether the patient is on the liver transplant waitlist as of the follow-up appointment. If yes, indicate the date the patient was added to the waitlist in the box below. Indicate whether the patient has received a liver transplant as of the
follow-up appointment.
If yes, enter the date of the transplant in the box below and ensure
the transplant form is filled out.
Hospital Admission since last Indicate whether the patient has been admitted to hospital (for any reason) since the last follow-up visit.
If yes, complete the questions that appear below and COMPLETE AN
UNSCHEDULED EVENT FORM FOR EACH INCIDENT.
Enter the number of unique hospital admissions since the last follow- since last visit Last discharge Describe the patient's discharge diagnosis at their most recent hospital diagnosis stay prior to the follow-up visit. This should also be included in the unscheduled event form. Enter the discharge date of the patient's most recent hospital stay prior to the follow-up visit. Any new event since last visit? If yes, COMPLETE AN UNSCHEDULED EVENT FORM.
Indicate whether surgery was required since the last visit.
If yes, describe type of surgery below and COMPLETE AN
UNSCHEDULED EVENT FORM.
Any other intervention Indicate whether any non-surgical interventions requiring general requiring general anesthesia? anesthesia were performed since the last visit.
If yes, describe below and COMPLETE AN UNSCHEDULED EVENT
March 2014 Version 1.0 Page 37 of 50 Any change in medication? Indicate whether there have been any changes in medications since
the last follow-up visit, including any changes made at this follow-up
appointment.
If yes, describe below.
Current main problems Indicate all problems that apply to the patient as of the follow-up appointment, including those mentioned previously above: Well, no issues: select if the patient is not currently having FTT: Failure to thrive Persistent jaundice: the patient has had jaundice continuously since the last follow-up form was filled out. Ascites: an accumulation of fluid in the peritoneal cavity Recent GI bleed: gastro-intestinal bleed since last follow-up appointment form completed. Interval episode of cholangitis: Episode of cholangitis (an infection of the common bile duct) since the last follow-up form was completed. Hypersplenism: Large spleen with low WBC/platelet count. Developmental delay: the patient has a delay in the any area of development. Referred for transplant: the patient has been referred for transplant. On transplant waiting list: the patient is on the transplant waiting list. If the patient is currently experiencing other problems, briefly describe below. March 2014 Version 1.0 Page 38 of 50 Post-Operative Unscheduled
Complete the Post-operative Unscheduled form every time the patient visits the CBAR site for an
unexpected event (ie. any visit to the CBAR site that is not for a regularly scheduled follow-up visit) or if
the patient has died.
Date of event onset
Use the calendar function to enter the date event first occurred (ex. date GI bleed first noted) Outpatient event Indicate whether the patient was treated as an outpatient during the entire event (ie. no hospital admission required). Hospital admission Indicate whether the patient was admitted to hospital at any time during the event. Enter the date the patient was first admitted to hospital for the event. admission Date of first Enter the date the patient was first discharged from hospital for the Length of first Enter the length of stay in days between first admission and first stay If there was more than one hospital stay for the same event (ex.
patient transferred between hospitals), enter the date of the second admission here. If there was more than one hospital stay for the same event (ex.
patient transferred between hospitals), enter the date of the second discharge here. If there was more than one hospital stay for the same event (ex.
patient transferred between hospitals), enter the length of stay in days of the second hospital stay here. Is the patient still alive? If the unscheduled event form was created to report a patient death, select "no" and answer the questions below. Date of death Enter the patient's date of death. Age at death This is an automatically calculated field showing the patient's age in months. If the value appears to be incorrect, check that the date of birth and date of death are correct. Event Diagnosis and Intervention
FTT
Indicate whether the patient was treated for failure to thrive (FTT). Indicate how the patient was treated for FTT. Select more than one option as appropriate: Change: select if any dietary change has been made (ex. calories, formulas, etc). Enteral feed: the patient was given N/G feeds as part of the FTT Parenteral feed: the patient was given parenteral feeding (intravenous feeding) as part of the FTT management. Briefly describe the treatment in the box below. March 2014 Version 1.0 Page 39 of 50 Indicate whether the patient had ascites during the event. Specify whether the ascites was: New onset: the patient was treated for a new onset of ascites (ie. the patient had not had ascites or had fully recovered from ascites prior to the date of event onset) Worsening: the patient was treated for a worsening case of ascites (ie. the patient had ascites prior to the date of event onset) Stable: the patient had ascites prior to the date of event onset, but it was stable at the time of the event. If the patient did not have ascites, leave blank. Indicate how the patient was treated for ascites during the event. Select more than one option as appropriate: Diuretics: the patient was prescribed medication that promotes the production of urine Paracentesis: the patient underwent a procedure to remove fluid accumulated in the abdominal cavity. Antibiotics: the patient was prescribed antibiotics Other If other, describe below. Indicate whether the patient was treated for spontaneous bacterial peritonitis (SBP; infection in the abdominal cavity), that was either: Proven: infection confirmed through lab tests Suspected: infection not confirmed through lab tests, but Indicate whether the patient was treated for a gastrointestinal bleed. If so, answer the questions that appear below. Indicate the type of bleed. Choose more than one option as appropriate: Hematemesis: the patient is vomiting blood Melena: the patient has black, tarry feces Blood per rectum: the patient has rectal bleeding Indicate whether the patient required transfusion. Indicate whether the patient underwent an upper gastrointestinal endoscopy (a procedure to view the interior lining of the esophagus, stomach, and duodenum) during the event. Indicate which of the following were identified during the endoscopy. Select more than one option as appropriate: Bleeding source identified Esophageal varices: extremely dilated sub- mucosal veins in the lower third of the esophagus Gastric varices: dilated submucosal veins in the March 2014 Version 1.0 Page 40 of 50 Ulcer: a discontinuity/break in the gastrointestinal membrane Portohypertensive gastropathy: Changes in the mucosa of the stomach in patients with portal hypertension. Also known as portal hypertensive gastropathy. If other, describe below. Indicate whether the patient was treated for esophageal varices with band ligation, a procedure where a band is used to stop blood supply to the hemorrhoidal mass. Sclerotherapy Indicate whether the patient was treated for esophageal varices with sclerotherapy, a procedure in which a medication is injected into blood vessels to cause shrinkage. Indicate whether the patient was treated for GI bleeds with a heater probe, an endoscopic procedure in which heat is used to ligate vascular or ulcer bleeds. Indicate whether the patient was treated for cholangitis (infection of the common bile duct), that was either: Proven: Infection confirmed through lab tests Suspected: Infection not confirmed through lab tests, but treated as cholangitis. No: the patient was not treated for cholangitis If proven or suspected, answer the questions that appear below. Briefly describe the cholangitis diagnosis. Indicate whether the patient had a fever during the event Increase LFTS Indicate whether the patient's liver function tests (LFTS) had increased. Note any increase, regardless of how small. Indicate whether the patient's AST had increased over previously recorded levels. Indicate whether the patient's AST had increased over previously recorded levels. Indicate whether the patient's AST had increased over previously recorded levels. Blood culture Indicate whether a blood culture was performed during the event. If yes, indicate whether the blood culture was positive below. Indicate whether a urine culture was performed during the event. If yes, indicate whether the urine culture was positive below. Indicate whether a liver biopsy was performed during the event. If yes, answer the questions that appear below. Indicate whether cholangitis was confirmed through histological analysis. March 2014 Version 1.0 Page 41 of 50 Liver tissue sent Indicate whether liver tissue was cultured. for culture If yes, indicate whether the liver tissue culture was positive below. Indicate whether the patient was taking antibiotics prior to the event (ie. prophylactic antibiotics). antibiotics Patient on Indicate whether the patient was prescribed urso during the event for the treatment of cholangitis. Indicate whether the patient received IV antibiotics during the event. If yes, answer the questions that appear below. Indicate whether the patient received oral antibiotics during the event. If yes, answer the questions that appear below. Jaundice new onset Indicate whether the patient had a new onset of jaundice during the event. If yes, enter total bilirubin level below (umol/L) Indicate whether the patient was treated for another condition not listed above. If yes, describe below. Indicate whether the patient underwent a colonoscopy during the event. If yes, answer the questions that appear below. Indicate why the colonoscopy was performed: Surveillance: the colonoscopy was performed to assess for GI Bleeding: the colonoscopy was performed to examine the source of gastrointestinal bleeding. If other, describe below. Indicate whether the colonoscopy was normal. Indicate whether the patient underwent surgery during the event. If yes, describe below. Other intervention Indicate whether the patient received any other interventions not described above during the event. If yes, describe below. March 2014 Version 1.0 Page 42 of 50 The Transplant and Summary forms should be updated on an ongoing basis, as described below. Transplant
The transplant form should be filled out at baseline and after discharge home from the patient's initial
transplant hospital stay. In most cases the only fields filled out at baseline will be "Was the Kasai
procedure done" (yes) and "Liver transplant" (no). All other questions in this section apply to the
patient's first hospitalization for a transplant. The end point of follow-up is the patient's discharge
home from their initial transplant hospitalization.
Was Kasai procedure done?
Indicate whether the patient had a Kasai procedure done. Liver transplant Indicate whether a liver transplant was performed. If yes, answer the questions that appear below. Indication for Select the patient's indication(s) for transplant. Select one or more of transplant Persistent jaundice post KP: the patient has had persistent jaundice since the Kasai procedure Bleeding: the patient has had recurrent gastrointestinal Recurrent cholangitis: the patient has had recurrent infection of the common bile duct. FTT: failure to thrive, insufficient weight gain Ascites: an accumulation of fluid in the peritoneal cavity HPS/PHT: the patient has had hepatopulmonary syndrome or portal hypertension If other, describe below. Indicate the type of liver transplant the received during their first Living related: the transplant was from a living relative of the Living unrelated: the transplant was from a living non-relative Cadaveric: the transplant was from a non-living donor. Enter the date of the liver transplant. transplant Age at liver This is an automatically calculated field showing the patient's age in months. If the value appears to be incorrect, check that the date of birth and date of liver transplant are correct. Enter the date of discharge from the transplant hospitalization. discharge Alive until Indicate whether the patient was alive until their discharge from hospital for the initial transplant hospitalization. March 2014 Version 1.0 Page 43 of 50 If answered How many
This is an automatically calculated field showing "yes" to months was the
how many months passed between the date of ‘Alive until patient post-
transplant and the date of hospital discharge. If discharge' transplant as of
the value appears to be incorrect, check that these dates are correct. If answered Date of death
Enter the date the patient died. "no" to ‘Alive
Some patients may reject their first transplant and immediately require another transplant during the same hospital stay. Indicate whether the
patient received a second liver transplant during their initial
transplant hospitalization stay.
Enter the date of the second liver transplant. liver transplant Age at second This is an automatically calculated field showing liver transplant the patient's age in months. If the value appears to be incorrect, check that the date of birth and date of second liver transplant are correct. Indicate whether the patient received a third liver transplant before transplant during their initial transplant hospitalization stay. This is an automatically calculated field showing the patient's age in months. If the value appears to be incorrect, check that the date of birth and date of third liver transplant are correct. Enter the date of the third liver Select the final total number of liver transplants the patient received by the time the patient was discharged from their initial transplant hospitalization: 1 2 3 March 2014 Version 1.0 Page 44 of 50 Summary

The summary form will be calculated automatically each time it is opened. In order to save the
calculations in the database, "Save Record" at the bottom of the page will have to be clicked. The
Summary page should be opened and saved each time data is entered in the database. Prior to
clicking "Save Record", fill the ‘Today's Date' field.

The summary page will generate responses to the following questions (except ‘Today's date') using the
indicated source information. Check through the responses to ensure they are correct before saving. If
any responses appear to be incorrect, correct the error in the source data entry form.
Today's date:
The date the form is viewed. Date of last F/U? The ‘discharge date' from the immediate post-operative data form or the ‘Visit date' from the most recently completed Post-op form, whichever has the latest date. Age at last F/U(mos): The ‘discharge date' or ‘visit date' (whichever is later)-‘date of birth' Number of F/U visits including The number of follow-up forms for which a date has been entered. last F/U: Number of hospitalizations: The sum of ‘number of admissions since last visit' for all completed post-operative follow-up forms. Patient had Kasai? ‘Kasai?' from the operative data form. Time since Kasai (mos): Date of last follow-up - ‘Kasai date' (operative data form) Age at Kasai (days) ‘Age at kasai' (operative data form) Patient had Transplant? ‘Liver transplant' (transplant form) Is the patient still alive? If ‘no' is selected for this question in any of the unscheduled event forms or the transplant form, ‘no' will be selected here. Transferred from the unscheduled event form or transplant form when ‘no' is selected for ‘Is the patient still alive'. If the answer to 'Is the patient still alive' is "no", this will be calculated from the ‘date of death' above and the ‘date of birth' in months Patient died on Tx list? If the response to ‘listed for transplant' in the last follow-up form is "yes", ‘Liver transplant' is "no", and the response to ‘Is the patient still alive' above is "no", then the response to this question will be "yes". Otherwise, "no" will be selected here. Patient had Kasai only? If "yes" is selected for ‘Kasai?', and "no" is selected for ‘Liver transplant', then "yes" will be selected here. Otherwise "no" will be selected here. Patient had Tx only? If "no" is selected for ‘Kasai?', and "yes" is selected for ‘Liver transplant', then "yes" will be selected here. Otherwise "no" will be selected here. Patient had Kasai and then If "yes" is selected for ‘Kasai?', and "yes" is selected for ‘Liver transplant', then "yes" will be selected here. Otherwise "no" will be selected here. Time from Kasai to Liver Tx ‘Date of Kasai' – ‘Liver transplant date' March 2014 Version 1.0 Page 45 of 50 *Only shows if ‘did patient have Kasai and then Liver Tx' indicates yes How long did/has patient If ‘liver transplant' is "yes", then calculation = ‘liver transplant date'- survived with native liver? ‘date of birth' If ‘liver transplant' is "no", and the answer to ‘Is the patient still alive' above is "no", calculation = ‘date of death' (above)-‘date of birth' If ‘liver transplant' is "no", and the answer to ‘Is the patient still alive' above is "yes", calculation = ‘date of last follow-up' (above)-‘date of birth' Did patient have Liver Tx "yes" will be selected if ‘date of liver transplant'-‘date of birth'<1 year before age 1 year? *Will only appear if ‘yes' is "no" will be selected if ‘date if liver transplant'- ‘date of birth' ≥1 year entered for ‘Liver transplant'. March 2014 Version 1.0 Page 46 of 50
Anastomosis: An opening created by surgical, traumatic or pathological means between two normally
separate spaces or organs.
Ascites: anoin th
Biopsy: A procedure obtainingfotoa
opsies can be accomplished with into in question) or by Common bile duct: The tube that carries bile from the liver to the gallbladder and intestines.
Cholangitis: An infection of the common bile duct, the tube that carries bile from the liver to the
gallbladder and intestines.
Diuretics: Medications that help to increase the volume of urine or fluid excreted from the body. Also
known as "water pills".
Heater probe: An endoscopic procedure in which heat is used to ligate vascular or ulcer bleeds.
Hepatopulmonary syndrome: A syndrome of shortness of breath and hypoxemia caused by vasodilation
in the lungs of patients with liver disease.
Hepatobiliary abnormality: Any abnormality in the liver (hepato) or draining (biliary) system.
Heterotaxia: An abnormal arrangement of organs.

Jaundice: A yellowish pigmentation of the skin, the conjunctival membranes over the sclerae (whites of
the eyes), and other mucous membranes caused by hyperbilirubinemia (increased levels of bilirubin in the blood).
Hypersplenism: An overactive spleen. The spleen helps filter old and damaged cells from your
bloodstream. If your spleen is overactive, it removes the blood cells too early and too quickly.
Polysplenia: a congenital diseases manifested by multiple small accessory spleens.

Portal Hypertension: High pressure in the portal system can be indicated by ascites, large spleen or
reversal of flow.
Portohypertensive gastropathy: Changes in the mucosa of the stomach in patients with portal
hypertension. Also known as portal hypertensive gastropathy. March 2014 Version 1.0 Page 47 of 50 Thrombophilia: of thin which there is ato the occurrence

Thrombosis/Embolism: An aggregation of blood factors (primarily platelets and fibrin) causing vascular
obstruction at the point of its formation. (Embolism may also include foreign material, other than a clot, causing blockage. March 2014 Version 1.0 Page 48 of 50 Alison Butler, CBAR Research Coordinator (604) 875-2345 ext. 4313
Memorial University (Janeway Children's Health and Rehabilitation Centre)
Dr. David Price, Surgeon
Dr. Pushpa Sathya, Gastroenterologist

Dalhousie University (IWK Health Centre)
Dr. Natalie Yanchar, Surgeon
Dr. Mohsin Rashid, Gastroenterologist

McGill University (Montreal Children's Hospital)
Dr. Jean-Martin Laberge, Surgeon
Dr. Sherif Emil, Surgeon
Dr. Najma Ahmed, Gastroenterologist

Université de Montreal (CHU Ste Justine)
Dr. Michel Lallier, Surgeon
Dr. Fernando Alvarez, Gastroenterologist

Laval University (CHUQ)
Dr. Julie Castilloux, Surgeon
Dr. Anna Wieckowska, Gastroenterologist

University of Ottawa (CHEO)
Dr. Ahmed Nasr, Surgeon
Dr. Carolina Jimenez

McMaster University (MCH),
Dr. Brian Cameron, Surgeon
Dr. Herbert Brill, Gastroenterologist

University of Toronto (Sick Kids)
Dr. Annie Fecteau, Surgeon
Dr. Simon Ling, Gastroenterologist
March 2014 Version 1.0 Page 49 of 50 University of Western Ontario (LHSC)
Dr. Andreana Butter, Surgeon
Dr. Ashok Dandhapani, Gastroenterologist

University of Manitoba (CHOW)
Dr. Melanie Morris, Surgeon
Dr. Mark Deneau, Gastroenterologist

University of Saskatchewan (SCH)
Dr. Grant Miller, Surgeon
Dr. Garth Bruce, Gastroenterologist

University of Alberta (Stollery Children's Hospital)
Dr. Gordon Lees, Surgeon
Dr. Jason Yap, Gastroenterologist

University of Calgary (CCH)
Dr. Mary Brindle, Surgeon
Dr. Steven Martin, Gastroenterologist
University of British Columbia (BC Children's Hospital)
Dr. James Murphy, Surgeon
Dr. Richard Schreiber, Gastroenterologist
March 2014 Version 1.0 Page 50 of 50

Source: http://cbar.ca/wp-content/uploads/2013/04/CBAR-Manual-Draft-V1.1-Dec-2014.pdf