What clinical and laboratory features in this case support the diagnosis of cystic fibrosis?
This patient had several features: 1. Intestine: Within the intestinal tract, unusually thick meconium may cause obstruction of the bowel. (Meconium is the green semi-liquid stool present in utero and in the newborn infant.) This is referred to as meconium ileus and may develop in utero. This complication, which manifests itself in the neonatal period, occurs in 10-15% of patients. Perforation of the bowel in utero as a consequence of the obstruction may result in meconium peritonitis and/or ileal atresia. Thick secretions in the older child may cause recurrent abdominal pain (meconium ileus equivalent). 2. Pancreas: The destruction of pancreas results in protein and fat malabsorption. This patient had bulky, foul-smelling stools that floated, suggestive of fat malabsorption. Deficiency of Vitamin K, a fat soluble vitamin, impairs the production of clotting factors, and hence the patient had a prolonged prothrombin time. Protein malnutrition is reflected by low serum albumin, edema and
