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