Nutritional status between 5-10 years is associated with cystic fibrosis-related diabetes in adolescence.

BACKGROUND: Cystic fibrosis-related diabetes (CFRD) is a risk factor for adverse clinical outcomes including poor nutritional status, deterioration in lung functions, and increased mortality. The association between nutritional status between 5 and 10 years of age and later diagnosis of CFRD is not known. METHODS: A retrospective chart review was performed for our patients with CF between 10 and 18 years. Data was collected at age 5 and 10 years. Comparison made between patients with and without CFRD. RESULTS: Two groups were comparable for age and sex. At age 5, groups had no differences in weight, height, and body mass index. At age 10, the CFRD group had a lower body mass index (40.2 ± 24.7 vs. 61.5 ± 22.5 percentile, p = 0.02). Spirometry was similar between groups at 5 and 10 years. Patients with CFRD had lower growth velocity (5 ± 0.9 vs. 5.7 ± 0.9 cm/year, p = 0.03) and reduced weight gain rate (2.2 ± 0.9 vs. 3.2 ± 1.2 kg/year, p = 0.03) compared to patients without CFRD between 5 and 10 years. Patients with a weight gain less than 2.5 kg/year between 5 and 10 years were nine times more likely to develop CFRD in adolescence (Unadjusted Odds Ratio: 8.9; 95% CI:1.4, 47.2; p = 0.01). CONCLUSION: Patients who later developed CFRD had significantly lower weight gain rate and height growth between 5 and 10 years of age than those without diabetes. Close monitoring of nutritional status in before age 10 years may help identify CF patients at-risk of developing CFRD.

A Rare and Potentially Fatal Etiology of Hypercalcemia in an Infant.

Hypercalcemia is an uncommon finding in children. Hypercalcemia has various etiologies including parathyroid dependent and independent mechanisms. Increased activity of the 1-alpha-hydroxylase enzyme in granulomatous diseases is a well-defined but an extremely rare cause of hypercalcemia in pediatric patients, particularly in infants. We describe the case of an infant who presented with failure to thrive, hepatosplenomegaly, and hypercalcemia who was initially treated with steroids but was later diagnosed with disseminated histoplasmosis in the absence of an underlying immunodeficiency. Extra caution should be used before considering steroids for the treatment of hypercalcemia and, whenever possible, steroids should not be initiated until a definite etiology is identified.

Sleep Fainting: A Neurocardiogenic Entity.

Fainting is a common clinical presentation, with vagally mediated (neurocardiogenic) causes being the most common for syncope presentation to the emergency room, and for hospital admissions. Classic teaching is that upright posture is a prerequisite for vagally mediated syncope (VMS) and that syncope in the supine position has more sinister causes. We present five patients, three males and two females, with a mean age of 44.4 (range 29-67) years, who presented with VMS in the supine position (sleep fainting). Four patients also had a history of classic upright syncope. Based on their clinical features and thorough investigations, we excluded other causes of loss of consciousness and diagnosed these patients to be having VMS in the supine position (sleep fainting). We further describe the management and follow-up of these patients. Sleep fainting/syncope is a new entity and has to be recognized for appropriate management. A diagnosis can be established if there is clinical suspicion, preserved left ventricular function without evidence of coronary artery disease, no high-risk electrocardiographic evidence of pre-excitation, long or short QT syndrome, Brugada syndrome or arrhythmogenic right ventricular dysplasia, and normal neurological work-up.

Management of bleeding gastric varices: a single session of histoacryl injection may be sufficient.

OBJECTIVE: Gastric variceal bleeding is one of the most feared complications of portal hypertension and hence merits investigation for its optimal therapy. We aimed to evaluate the efficacy and long-term outcome(s) of patients treated with a single session of histoacryl sclerotherapy for active gastric variceal bleeding. METHODS: The medical records of patients who presented with active gastric variceal bleeding between 1998 and 2011 in a tertiary care setting were evaluated retrospectively and the eventual outcome(s) (initial hemostasis, rebleeding, and mortality rate) was assessed at least 1 year after the index bleed. RESULTS: Ninety-seven patients were included. The mean age of the patients was 51.0 ± 12.5 years; 62% were men. Hepatitis C was the most common etiology, found in 63 (65%) patients. The majority of the patients were classified as Child-Pugh grade B and C: 44 (46%) and 29 (31%) patients, respectively. A total of 40 (41%) patients were IGV1, 35 (36%) patients were GOV 2, 20 (21%) patients were GOV 1, and 2 (2%) patients were IGV2. A single session of histoacryl was successful in controlling bleeding in 83 (86%) patients. Seven (7%) patients died during the hospital stay. Rebleeding was observed in 24 (27%) patients during the 1-year follow-up, of whom 12 (50%) were managed successfully with repeated histoacryl injection. The overall mortality rate at 6 weeks, 6 months, and 1 year was 14 (14%), 19 (20%) and 26 (27%) patients, respectively. Child-Pugh classification was a significant prognostic factor of survival (P<0.001). CONCLUSION: A single session of histoacryl sclerotherapy is effective in the majority of patients with active gastric variceal bleeding. Rebleeding was observed in one-fourth of patients, half of whom were controlled successfully by repeated histoacryl sclerotherapy.