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Citation
Magee, John (2024). Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis Liver Disease (CFLD PUSH) (Version 2) [Dataset] NIDDK Central Repository. https://doi.org/10.58020/vj16-wz20
Data Availability Statement
Data from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis Liver Disease (CFLD PUSH) [(Version 2) https://doi.org/10.58020/vj16-wz20] reported here are available for request at the NIDDK Central Repository (NIDDK-CR) website, Resources for Research (R4R), https://repository.niddk.nih.gov/.
Acknowledgement Statement
The CFLD PUSH study was conducted by the study investigators and supported by the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The resources from the CFLD PUSH (https://doi.org/10.58020/vj16-wz20) study reported here were supplied by NIDDK Central Repository (NIDDK-CR) and are available for request at https://repository.niddk.nih.gov. This manuscript was not prepared under the auspices of the CFLD PUSH study and does not necessarily reflect the opinions or views of the CFLD PUSH study, NIDDK-CR, or NIDDK.
Data Package Version
Version 2 (Updated on: Nov 27, 2024)
Resource Availability
  • Data Available for Request
  • Specimens Require Collaboration with Parent Study
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General Description

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.

Objectives

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.

Outcome Measure

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.

Eligibility Criteria

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

Outcome

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.

Research Area

Liver Disease, Pancreatic Disease, Multidisciplinary Research

Study Type

Observational

Study Sites

11

Study Start Date

2010-01

Study End Date

2023-06

Condition

Cystic Fibrosis, Pancreatic Insufficiency, Cirrhosis of Liver

Keywords

Cystic Fibrosis, Advanced Liver Disease, Liver Disease, Pancreatic Insufficiency, Abdominal Ultrasound, Cirrhosis

NIDDK Division

Division of Digestive Diseases and Nutrition

774
Participants

Target Population
Children

Sex

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Age

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The chart has 1 Y axis displaying values. Data ranges from 774 to 774.
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Race

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The chart has 1 Y axis displaying values. Data ranges from 4 to 729.
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Ethnicity

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The chart has 1 Y axis displaying values. Data ranges from 3 to 710.
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Public Documents Table
Document Name
Description
Document Type
File Format
Compliance
Download
CFLD PUSH Data Dictionary
Data Dictionary used as a collection of metadata - defines the variable contents of each datasetCodebook/Data DictionaryPDF
If you need accessible versions of documents, please email your request to NIDDK-CRsupport@niddk.nih.gov

Permitted Use(s) of the Resources
  • Use is allowed only for the specified disease(s), disorder(s), condition(s), or research area(s): Cystic Fibrosis Liver Disease
Non-Public Documents (2)
Non-Public Documents Table
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 StudySummary StatisticsPDF
PUSH CFLD Variable Summary Statistics
Summary statistics for the variables of all datasets associated with the CFLD PUSH StudySummary StatisticsPDF
Datasets (74)
Datasets Table
Dataset Name
Description
# of Records
# of Variables
File Format(s)
Additonal Labs (Form 08D )Raw Dataset
Captures raw data from Form 08D - additional labs1038csv (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)798csv (172.24 KB); sas7bdat (1.46 MB)
Eligibility (Form 01A) Raw Dataset
Contains raw data from Form 01A - Eligibility774csv (405.77 KB); sas7bdat (2.32 MB)
Consensus Grades (Form 25) Raw Dataset
Captures raw consensus grades data from Form 251015csv (251.88 KB); sas7bdat (2.3 MB)
Enrollment Raw Dataset
Captures raw enrollment data777csv (95.98 KB); sas7bdat (512 KB)
Final Status (Form 35) Raw Dataset
Captures raw final status data from Form 35774csv (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)776csv (167.42 KB); sas7bdat (1.2 MB)
UDG_Reread Raw Dataset
Captures raw UDG reread data429csv (70.42 KB); sas7bdat (1.01 MB)
Liver Transplant (Form 27) Raw Dataset
Captures raw liver transplant data from Form 272csv (1.88 KB); sas7bdat (512 KB)
Events Raw Dataset
Captures raw events data42csv (3.63 KB); sas7bdat (128 KB)
Ultrasound Raw Dataset
Contains raw ultrasound data3042csv (462.75 KB); sas7bdat (5.64 MB)
Prior Labs and Medications Link Raw Dataset
Captures raw prior labs and medications linking data245csv (55.28 KB); sas7bdat (256 KB)
BCT_DUPS Raw Dataset
Captures raw BCT DUPS data461csv (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)317csv (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)256csv (34.69 KB); sas7bdat (192 KB)
Subject Demographics (Form 02A) Raw Dataset
Captures raw subject demographics data from Form 02A774csv (141.88 KB); sas7bdat (1.42 MB)
Physical Exam (Form 07) Raw Dataset
Captures raw physical exam data from Form 071929csv (510.43 KB); sas7bdat (3.05 MB)
GI Endoscopy (Form 41) Raw Dataset
Captures raw GI endoscopy data from Form 4121csv (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 2-4182csv (36.06 KB); sas7bdat (432 KB)
Schedule (Form 00S) Raw Dataset
Captures raw Schedule (Form 00S) data774csv (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)799csv (172.54 KB); sas7bdat (1.31 MB)
Adverse Event Log Raw Dataset
Captures data on adverse events1csv (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 08B280csv (62.37 KB); sas7bdat (256 KB)
Physical Exam_DE Raw Dataset
Captures raw physical exam DE data1014csv (283.23 KB); sas7bdat (1.64 MB)
Medical History (Form 04) Raw Dataset
Captures raw medical history data from Form 04248csv (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 6-131145csv (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 6-11788csv (223.24 KB); sas7bdat (1.68 MB)
US Grade (Form 25) Raw Dataset
Contains raw US Grade data from Form 251181csv (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 280csv (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)292csv (65.51 KB); sas7bdat (680 KB)
Paternal Family History (Form 06) Raw Dataset
Captures raw paternal family history data from Form 06248csv (86.09 KB); sas7bdat (1.5 MB)
GI Bleed (Form 42) Raw Dataset
Captures raw GI bleed data from Form 424csv (988 B); sas7bdat (128 KB)
Matching (Form 01B) Raw Dataset
Captures raw matching data from Form 01B774csv (92.57 KB); sas7bdat (1.12 MB)
Biosample Raw Dataset
Captures raw data on study biosamples21292csv (2.41 MB); sas7bdat (15.75 MB)
Visits Raw Dataset
Captures raw visits data5668csv (412.34 KB); sas7bdat (896 KB)
Family Enrollment (Form 01C) Raw Dataset
Captures raw family enrollment data from Form 01C773csv (104.48 KB); sas7bdat (1.25 MB)
Doppler (Form 26) Raw Dataset
Contains raw doppler data from Form 261491csv (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)771csv (166.22 KB); sas7bdat (1.2 MB)
Protocol Deviation (Form 40) Raw Dataset
Contains raw protocol deviation data from Form 40984csv (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 3-6183csv (52.05 KB); sas7bdat (624 KB)
BCT Raw Dataset
Captures raw BCT data4591csv (2.48 MB); sas7bdat (19.25 MB)
EXP Raw Dataset
Captures raw EXP data6csv (2.06 KB); sas7bdat (336 KB)
Eligibility Clarification Request (Form 15A) Raw Dataset
Captures raw data from Form 15A - Eligibility Clarification Request5csv (1.86 KB); sas7bdat (336 KB)
Family Demographics (Form 02B) Raw Dataset
Captures raw family demographics data from Form 02B248csv (98.59 KB); sas7bdat (1.25 MB)
Quality of Life Raw (Form 21) Dataset
Captures raw Quality of Life (Form 21) data770csv (89.27 KB); sas7bdat (1.12 MB)
Matches Raw Dataset
Contains raw matches data63csv (4.15 KB); sas7bdat (128 KB)
ZPBB Raw Dataset
Captures raw ZPBB data243csv (30.03 KB); sas7bdat (256 KB)
GI Symptoms Raw Dataset
Captures raw GI symptoms data1733csv (283.64 KB); sas7bdat (2.43 MB)
Maternal Family History (Form 05) Raw Dataset
Captures raw maternal family history data from Form 05248csv (86.54 KB); sas7bdat (1.5 MB)
MBR Raw Dataset
Contains raw MBR data762csv (259.19 KB); sas7bdat (924 KB)
Lab Raw Dataset
Contains raw laboratory results data1900csv (990.37 KB); sas7bdat (4.1 MB)
Prior Labs and Medications (Form 08A) Raw Dataset
Captures raw prior labs and medications data from Form 08A247csv (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 12-13382csv (108.49 KB); sas7bdat (936 KB)
MBR_S24 Raw Dataset
Contains raw MBR S24 data762csv (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)252csv (35.8 KB); sas7bdat (192 KB)
Cirrhosis (Form 07A) Raw Dataset
Captures raw cirrhosis data from Form 07A223csv (42.29 KB); sas7bdat (1.75 MB)
Additonal Medications (Form 08C) Raw Dataset
Captures raw data from Form 08C - Additional Medications1403csv (225.7 KB); sas7bdat (2.75 MB)
PUSH Eligibility Raw Dataset
Captures raw PUSH eligibility data254csv (238.44 KB); sas7bdat (6.64 MB)
USCD Raw Dataset
Captures raw USCD data1208csv (111.49 KB); sas7bdat (2.86 MB)
Matching by Site Raw Dataset
Contains raw matching by site data762csv (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 Older848csv (292.61 KB); sas7bdat (1.88 MB)
Specimens (26,060)
Specimens Table
Specimen
Count
Cells196
DNA979
EBV Transformed Cell Lines1567
Lymphocytes194
Plasma8004
Serum7874
Urine7246