Ramalingam Arumugam

Serum leptin and igf-i levels in cystic fibrosis

The role of leptin in states of negative energy balance such as cystic fibrosis (CF) has not been explored. We hypothesized that leptin levels in patients with CF would be low due to correlation with body weight. Despite the importance of IGF-I in normal growth and anabolism, there are few data on IGF-I in CF. We studied 27 CF patients (25+/-5 yrs, 57+/-9 kg, 10M/17F) and 12 control subjects (25+/-4 yrs, 57+/-9 kg, 6M/6F). Each subject underwent analysis of lean body mass (LBM) and percent body fat by dual-energy x-ray absorptiometry (DEXA). Serum leptin and IGF-I levels were measured by radioimmunoassay. Serum leptin levels were similar both in CF and in controls (CF=5.3+/-4.1 ng/ml, C=4.4+/-3.6ng/ml; p=0.3), and there was no difference in percent body fat between the two groups (CF=26+/-13%, C=21+/-7%; p=0.3). Leptin levels were significantly lower in CF males than females corresponding to lower fat levels in males in both CF and controls. Leptin levels were positively correlated with percent body fat both in CF and controls (CF: r=0.8; p=0.01, CONTROL: r=0.8; p =0.2). Serum IGF-I levels were significantly lower in CF patients than in controls (CF=1.13+/-0.41 ng/ml, C=6.72+/-3.62 ng/ml; p=<0.01). We conclude that the physiological regulation of leptin is maintained in relation to body fat even in chronic illness and that the negative energy balance in CF is not caused by high leptin levels.

Crohn's Disease Presenting as Chronic Constipation: A Case Report

A 12-year-old white male presented to a local hospital with a 1week history of fever, vomiting, diarrhea, abdominal distention, and diffuse abdominal pain. There was no hematemesis or bloody or mucous-containing stool. His history was significant for constipation and fecal soiling from early childhood, until recently. He was managed with intermittent stool softeners and enemas. A full-thickness rectal biopsy performed at age 5 years revealed normal ganglion cells. Laboratory data were as follows: white blood cell count of 30,000, 70% neutrophils, 23% band cells, blood urea nitrogen of 48 mg/dL (17.1 mmol/L), creatinine 2.3 mg/dL (203 pmol/L), and albumin 2.3 g/dL (23 gm/L); arterial blood gas pH 7.19, Co2 29,02 128, and Hco3-16. An abdominal x-ray film showed multiple air fluid levels and grossly dilatated large bowel loops, measuring up to 15 cm (Figure 1). Blood and stool cultures were negative. He was managed conservatively for presumed

Cystic Fibrosis Presenting as Massive Hepatomegaly

S ome unusual presenting features of cystic fibrosis reported in the literature include anemia, hypoprotinemia with edema, nasal polyposis, enlarged submaxillary salivary gland, focal hepatic biliary cirrhosis, rectal prolapse and male sterility. This report describes a child with cystic fibrosis presenting with massive hepatomegaly, initially thought to have a liver tumor. He had no prior history of respiratory symptoms. The correct diagnosis became apparent only after detailed analysis of the complete clinical picture. This case illustrates the importance of the consideration of cystic fibrosis in the differential diagnosis of children with liver disease. Patient Report

Osteopenia in Cystic Fibrosis |[diams]| 399

BACKGROUND: Cystic fibrosis (CF) patients have many potential risk factors for osteopenia/osteoporosis, such as poor nutrition, malabsorption of vitamin D, glucocorticoid use, and inactivity. Several previous reports document a high prevelance of osteoporosis in CF; however these studies have compared CF to national standards or to subjects not matched for lean body mass (LBM) or height. The goals of our study were to evaluate the presence of osteopenia or osteoporosis in CF adults from our center and to compare their results to controls who were matched for LBM and height.