Cystic fibrosis is an inherited condition in which cell secretions are thick and sticky, instead of thin and slippery. The secretions obstruct passageways and ducts, leading to severe internal damage. Patients with cystic fibrosis sometimes develop advanced liver disease (CFLD). Hepatic cirrhosis can occur with CFLD, as a result of the inflammation and scarring caused by clogged bile ducts.
The Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis Liver Disease (CFLD PUSH) study was a prospective longitudinal study that aimed to determine the utility of abdominal ultrasound to predict the development of cirrhosis in patients with cystic fibrosis as indicated by a nodular liver on ultrasound. Participants underwent abdominal ultrasound at enrollment and based on the outcome, were placed in one of four groups. Within a 10-year period, participants underwent other specimen collection procedures in addition to abdominal ultrasound. This study also monitored the effects of cystic fibrosis on associated pulmonary and nutritional issues.
The objective of the CFLD PUSH study was to determine the usefulness of abdominal ultrasound when attempting to predict the development of cirrhosis in participants with cystic fibrosis.
The primary outcome measure was development of cirrhosis as indicated by the development of a nodular ultrasound during a six-year period of monitoring. The secondary outcome measures were effects on associated pulmonary and nutritional issues.
Inclusion criteria:
· Age 3-12 years old
· Diagnosed with cystic fibrosis and pancreatic insufficiency
· Enrolled in the CFF Registry Study or Toronto CF Registry
Exclusion criteria:
· Had cirrhosis
· Presence of Burkholderia cepacian
· Short bowel syndrome
· Had serious disease that would prevent participation
Of the 764 participants enrolled at baseline, 722 were assigned a final ultrasound (US) grade: 592 normal US pattern (NL), 65 heterogeneous increased echogenicity of the liver (HTG), 41 nodular pattern (NOD), and 24 homogeneous increased echogenicity of the liver (HMG). There were 62 HTG that were matched to 125 NL and followed for up to 10 years. At 6 years, those with a HTG pattern at baseline had a 9.5 RR (95% CI: 3.4, 26.7) for the development of NOD compared to NL. HTG who developed NOD were younger, had higher GGT, and higher GGT to platelet ratio (GPR) compared to HTG who did not develop NOD. From the results, the study developed a nomogram to predict risk of outcome using US findings, GPR, and age.
Liver Disease, Pancreatic Disease, Multidisciplinary Research
Observational
11
2010-01
2023-06
Cystic Fibrosis, Pancreatic Insufficiency, Cirrhosis of Liver
Cystic Fibrosis, Advanced Liver Disease, Liver Disease, Pancreatic Insufficiency, Abdominal Ultrasound, Cirrhosis
Division of Digestive Diseases and Nutrition
Document Name | Description | Document Type | File Format |
---|---|---|---|
PUSH CFLD Variable Count | Number of responses for the variables of all datasets associated with the CFLD PUSH Study | Summary Statistics | |
PUSH CFLD Variable Summary Statistics | Summary statistics for the variables of all datasets associated with the CFLD PUSH Study | Summary Statistics |
Dataset Name | Description | # of Records | # of Variables | File Format(s) |
---|---|---|---|---|
Additonal Labs (Form 08D )Raw Dataset | Captures raw data from Form 08D - additional labs | 1038 | csv (169.33 KB); sas7bdat (512 KB) | |
Child Report Ages 8-12 (Form QL8C) Raw Dataset | Captures raw data from Child Report Ages 8-12 (Form QL8C) | 798 | csv (172.24 KB); sas7bdat (1.46 MB) | |
Eligibility (Form 01A) Raw Dataset | Contains raw data from Form 01A - Eligibility | 774 | csv (405.77 KB); sas7bdat (2.32 MB) | |
Consensus Grades (Form 25) Raw Dataset | Captures raw consensus grades data from Form 25 | 1015 | csv (251.88 KB); sas7bdat (2.3 MB) | |
Enrollment Raw Dataset | Captures raw enrollment data | 777 | csv (95.98 KB); sas7bdat (512 KB) | |
Final Status (Form 35) Raw Dataset | Captures raw final status data from Form 35 | 774 | csv (188.93 KB); sas7bdat (3.94 MB) | |
Teen Report Ages 13-18 (Form QL13C) Raw Dataset | Captures raw data from Teen Report Ages 13-18 (Form QL13C) | 776 | csv (167.42 KB); sas7bdat (1.2 MB) | |
UDG_Reread Raw Dataset | Captures raw UDG reread data | 429 | csv (70.42 KB); sas7bdat (1.01 MB) | |
Liver Transplant (Form 27) Raw Dataset | Captures raw liver transplant data from Form 27 | 2 | csv (1.88 KB); sas7bdat (512 KB) | |
Events Raw Dataset | Captures raw events data | 42 | csv (3.63 KB); sas7bdat (128 KB) | |
Ultrasound Raw Dataset | Contains raw ultrasound data | 3042 | csv (462.75 KB); sas7bdat (5.64 MB) | |
Prior Labs and Medications Link Raw Dataset | Captures raw prior labs and medications linking data | 245 | csv (55.28 KB); sas7bdat (256 KB) | |
BCT_DUPS Raw Dataset | Captures raw BCT DUPS data | 461 | csv (253.8 KB); sas7bdat (2.25 MB) | |
Parent Report for Young Child Ages 5-7 (Form QL5P) Raw Dataset | Captures raw data from Parent Report for Young Child Ages 5-7 (Form QL5P) | 317 | csv (68.99 KB); sas7bdat (672 KB) | |
Young Adult Report Ages 18-25 (Form QL18C) Raw Dataset | Captures raw data from Young Adult Report Ages 18-25 (Form QL18C) | 256 | csv (34.69 KB); sas7bdat (192 KB) | |
Subject Demographics (Form 02A) Raw Dataset | Captures raw subject demographics data from Form 02A | 774 | csv (141.88 KB); sas7bdat (1.42 MB) | |
Physical Exam (Form 07) Raw Dataset | Captures raw physical exam data from Form 07 | 1929 | csv (510.43 KB); sas7bdat (3.05 MB) | |
GI Endoscopy (Form 41) Raw Dataset | Captures raw GI endoscopy data from Form 41 | 21 | csv (3.67 KB); sas7bdat (360 KB) | |
Parent Report for Toddler (Form QL2P) Ages 2-4 Raw Dataset | Captures raw data from Parent Report for Toddler (Form QL2P) Ages 2-4 | 182 | csv (36.06 KB); sas7bdat (432 KB) | |
Schedule (Form 00S) Raw Dataset | Captures raw Schedule (Form 00S) data | 774 | csv (410.94 KB); sas7bdat (1.85 MB) | |
Parent Report for Child Ages 8-12 (Form QL8P) Raw Dataset | Captures raw data from Parent Report for Child Ages 8-12 (Form QL8P) | 799 | csv (172.54 KB); sas7bdat (1.31 MB) | |
Adverse Event Log Raw Dataset | Captures data on adverse events | 1 | csv (1.15 KB); sas7bdat (512 KB) | |
URSO Log (Form 08B) Raw Dataset | Captures raw URSO medication log data, including dosage (mg) and start/stop dates from Form 08B | 280 | csv (62.37 KB); sas7bdat (256 KB) | |
Physical Exam_DE Raw Dataset | Captures raw physical exam DE data | 1014 | csv (283.23 KB); sas7bdat (1.64 MB) | |
Medical History (Form 04) Raw Dataset | Captures raw medical history data from Form 04 | 248 | csv (74.59 KB); sas7bdat (1020 KB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Parent for Ages 6-13 Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Parent for Ages 6-13 | 1145 | csv (384.5 KB); sas7bdat (3.06 MB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Young Child Ages 6-11 Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Young Child Ages 6-11 | 788 | csv (223.24 KB); sas7bdat (1.68 MB) | |
US Grade (Form 25) Raw Dataset | Contains raw US Grade data from Form 25 | 1181 | csv (268.89 KB); sas7bdat (448 KB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Parents/Caregivers for Ages 3-6 Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Parents/Caregivers for Ages 3-6 | 280 | csv (94.93 KB); sas7bdat (980 KB) | |
Young Child Report Ages 5-7 (Form QL5C) Raw Dataset | Captures raw data from Young Child Report Ages 5-7 (Form QL5C) | 292 | csv (65.51 KB); sas7bdat (680 KB) | |
Paternal Family History (Form 06) Raw Dataset | Captures raw paternal family history data from Form 06 | 248 | csv (86.09 KB); sas7bdat (1.5 MB) | |
GI Bleed (Form 42) Raw Dataset | Captures raw GI bleed data from Form 42 | 4 | csv (988 B); sas7bdat (128 KB) | |
Matching (Form 01B) Raw Dataset | Captures raw matching data from Form 01B | 774 | csv (92.57 KB); sas7bdat (1.12 MB) | |
Biosample Raw Dataset | Captures raw data on study biosamples | 21292 | csv (2.41 MB); sas7bdat (15.75 MB) | |
Visits Raw Dataset | Captures raw visits data | 5668 | csv (412.34 KB); sas7bdat (896 KB) | |
Family Enrollment (Form 01C) Raw Dataset | Captures raw family enrollment data from Form 01C | 773 | csv (104.48 KB); sas7bdat (1.25 MB) | |
Doppler (Form 26) Raw Dataset | Contains raw doppler data from Form 26 | 1491 | csv (445.89 KB); sas7bdat (3.13 MB) | |
Parent Report for Teens Ages 13-18 (Form QL13P) Raw Dataset | Captures raw data from Parent Report for Teens Ages 13-18 (Form QL13P) | 771 | csv (166.22 KB); sas7bdat (1.2 MB) | |
Protocol Deviation (Form 40) Raw Dataset | Contains raw protocol deviation data from Form 40 | 984 | csv (489.35 KB); sas7bdat (18.05 MB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Preschool Ages 3-6 Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Preschool Ages 3-6 | 183 | csv (52.05 KB); sas7bdat (624 KB) | |
BCT Raw Dataset | Captures raw BCT data | 4591 | csv (2.48 MB); sas7bdat (19.25 MB) | |
EXP Raw Dataset | Captures raw EXP data | 6 | csv (2.06 KB); sas7bdat (336 KB) | |
Eligibility Clarification Request (Form 15A) Raw Dataset | Captures raw data from Form 15A - Eligibility Clarification Request | 5 | csv (1.86 KB); sas7bdat (336 KB) | |
Family Demographics (Form 02B) Raw Dataset | Captures raw family demographics data from Form 02B | 248 | csv (98.59 KB); sas7bdat (1.25 MB) | |
Quality of Life Raw (Form 21) Dataset | Captures raw Quality of Life (Form 21) data | 770 | csv (89.27 KB); sas7bdat (1.12 MB) | |
Matches Raw Dataset | Contains raw matches data | 63 | csv (4.15 KB); sas7bdat (128 KB) | |
ZPBB Raw Dataset | Captures raw ZPBB data | 243 | csv (30.03 KB); sas7bdat (256 KB) | |
GI Symptoms Raw Dataset | Captures raw GI symptoms data | 1733 | csv (283.64 KB); sas7bdat (2.43 MB) | |
Maternal Family History (Form 05) Raw Dataset | Captures raw maternal family history data from Form 05 | 248 | csv (86.54 KB); sas7bdat (1.5 MB) | |
MBR Raw Dataset | Contains raw MBR data | 762 | csv (259.19 KB); sas7bdat (924 KB) | |
Lab Raw Dataset | Contains raw laboratory results data | 1900 | csv (990.37 KB); sas7bdat (4.1 MB) | |
Prior Labs and Medications (Form 08A) Raw Dataset | Captures raw prior labs and medications data from Form 08A | 247 | csv (93.58 KB); sas7bdat (720 KB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Child Self Report Ages 12-13 Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Child Self Report Ages 12-13 | 382 | csv (108.49 KB); sas7bdat (936 KB) | |
MBR_S24 Raw Dataset | Contains raw MBR S24 data | 762 | csv (259.19 KB); sas7bdat (924 KB) | |
Parent Report for Young Adult Ages 18-25 (Form QL18P) Raw Dataset | Captures raw data from Parent Report for Young Adult Ages 18-25 (Form QL18P) | 252 | csv (35.8 KB); sas7bdat (192 KB) | |
Cirrhosis (Form 07A) Raw Dataset | Captures raw cirrhosis data from Form 07A | 223 | csv (42.29 KB); sas7bdat (1.75 MB) | |
Additonal Medications (Form 08C) Raw Dataset | Captures raw data from Form 08C - Additional Medications | 1403 | csv (225.7 KB); sas7bdat (2.75 MB) | |
PUSH Eligibility Raw Dataset | Captures raw PUSH eligibility data | 254 | csv (238.44 KB); sas7bdat (6.64 MB) | |
USCD Raw Dataset | Captures raw USCD data | 1208 | csv (111.49 KB); sas7bdat (2.86 MB) | |
Matching by Site Raw Dataset | Contains raw matching by site data | 762 | csv (58.09 KB); sas7bdat (192 KB) | |
Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Teen/Adult Ages 14 and Older Raw Dataset | Captures raw data from Cystic Fibrosis Questionnaire - Revised (CFQ-R) - Teen/Adult Ages 14 and Older | 848 | csv (292.61 KB); sas7bdat (1.88 MB) | |
Specimen | Count |
---|---|
Cells | 196 |
DNA | 979 |
EBV Transformed Cell Lines | 1567 |
Lymphocytes | 194 |
Plasma | 8004 |
Serum | 7874 |
Urine | 7246 |