Methods
The current cross-sectional study included 50 CF cases from the
Children‘s Hospital’s Pediatric Pulmonology Clinic, Ain Shams
University, Cairo, Egypt, and was approved by the hospital ethics
committee. Patients’ guardians/parents provided informed consent prior
to starting the study.
Patients ≤16 years of age were diagnosed with CF through a positive
sweat chloride test, and/ or two diseases resulting in the mutation of
CF transmembrane conductance regulator (CFTR) were enrolled in this
study. Patients were excluded if they have been on hepatotoxic drugs as
antifungals and anti-tuberculous drugs; or having associated liver
disease (e.g., Chronic viral hepatitis).
The following were collected from each patient: medical history
including age, sex and age at diagnosis, past medical history, family
history and socioeconomic histories, clinical assessment of pulmonary,
hepatobiliary, and gastrointestinal symptoms, laboratory testing
including complete blood count (neutrophils, lymphocytes, white blood
cell count, hemoglobin, and platelets), liver enzymes as ALT, AST, and
albumin, coagulation parameters (PT, PTT, INR). In addition,
pelvic-abdominal ultrasound was done for evaluation of the size and
echogenicity of the liver, size of the spleen, presence or absence of
ascites, portal vein, common bile duct, and gall bladder sludge or
stones. Observation of a bright echo pattern of the liver on ultrasound
is commonly considered a sign of hepatic steatosis.
A full clinical examination was done including vital signs, weight,
height, as well as body mass index (BMI), chest and abdominal
examination. Shwachman-Kulczycki
score was utilized for assessing CF disease severity based on four
different parameters: radiological findings, nutrition status, physical
examination, and general activity. Each characteristic is assigned a
value between 5 (severely impaired) and 25 (normal), resulting in a
total score that is classified as excellent (86-100), good (71-85), mild
(56-70), moderate (41-55) or severe (<40).9