High variability in oral glucose tolerance among 1,128 patients with cystic fibrosis: a multicenter screening study.

BACKGROUND: In cystic fibrosis, highly variable glucose tolerance is suspected. However, no study provided within-patient coefficients of variation. The main objective of this short report was to evaluate within-patient variability of oral glucose tolerance. METHODS: In total, 4,643 standardized oral glucose tolerance tests of 1,128 cystic fibrosis patients (median age at first test: 15.5 [11.5; 21.5] years, 48.8% females) were studied. Patients included were clinically stable, non-pregnant, and had at least two oral glucose tolerance tests, with no prior lung transplantation or systemic steroid therapy. Transition frequency from any one test to the subsequent test was analyzed and within-patient coefficients of variation were calculated for fasting and two hour blood glucose values. All statistical analysis was implemented with SAS 9.4. RESULTS: A diabetic glucose tolerance was confirmed in 41.2% by the subsequent test. A regression to normal glucose tolerance at the subsequent test was observed in 21.7% and to impaired fasting glucose, impaired glucose tolerance or both in 15.2%, 12.0% or 9.9%. The average within-patient coefficient of variation for fasting blood glucose was 11.1% and for two hour blood glucose 25.3%. CONCLUSION: In the cystic fibrosis patients studied, a highly variable glucose tolerance was observed. Compared to the general population, variability of two hour blood glucose was 1.5 to 1.8-fold higher.

Diabetes in cystic fibrosis: multicenter screening results based on current guidelines.

BACKGROUND: Published estimates on age-dependent frequency of diabetes in cystic fibrosis (CF) vary widely, and are based mostly on older data. However, CF treatment and prevention of comorbidities changed over recent years. In many studies, definition of cystic fibrosis-related diabetes (CFRD) is not in line with current guideline recommendations. Therefore, we evaluated age-dependent occurrence of glucose abnormalities and associated risk factors in CF patients who participated in a multicenter screening program using oral glucose tolerance tests (OGTT). METHODS: Between 2001 and 2010, 43 specialized CF centers from Germany and Austria serially performed 5,179 standardized OGTTs in 1,658 clinically stable, non-pregnant CF patients with no prior steroid medication or lung transplantation. Age-dependent occurrence of impaired fasting glucose (IFG), impaired glucose tolerance (IGT), IFG+IGT, one (DGT) or two consecutive (CFRD) diabetic OGTTs was analyzed, using Kaplan Meier curves. Cox proportional-hazards models were created to elucidate the influence of sex or underweight. RESULTS: At baseline/last OGTT, median age was 15.9 years/18.2 years and 30.6%/31.8% of patients were underweight. 25% of patients showed IFG at age 14.3 years; IGT at age 16.3 years; IFG+IGT combined at age 17.7 years. DGT was observed in 25% of patients at age 22.6 years; CFRD at age 34.5 years. Females had a 3.54 [95% CI 1.23-10.18] times higher risk for CFRD; risk for DGT was 2.21 [1.22-3.98] times higher. Underweight was a risk factor for IGT (HR [95% CI]: 1.38 [1.11-1.71]) and IFG+IGT (1.43 [1.11-1.83]), and in males also for DGT (1.49 [1.09-2.04]). CONCLUSIONS/SIGNIFICANCE: If confirmation of diabetes by a second test is required, as recommended in current guidelines, age at CFRD diagnosis was higher compared to most previous studies. However, known risk factors for glucose abnormalities in CF were confirmed. Confirmation of diabetic OGT by a repeat test is important for a consistent diagnosis of CFRD.

Pneumonitis and pneumatoceles following accidental hydrocarbon aspiration in children.

Accidental ingestion and aspiration of hydrocarbons in children are common. Among the various clinical and pathological manifestations of hydrocarbon (HC) poisoning, pneumonitis is the most significant and occurs in up to 40% of children, whereas formation of pneumatoceles is believed to be a rare event. We report two children with HC pneumonitis and pneumatoceles as a reversible complication after ingestion and aspiration of lamp oil with very low viscosity. Patient 1, a 21-month-old boy, started to cough and developed tachypnea, sternal retractions and mild cyanosis immediately after aspiration. Patient 2, a 24-month-old girl, was asymptomatic during the first days after the accident; subsequently, she started to cough and developed fever, dyspnea and chest pain. Chest x-ray and computed tomography revealed multiple patchy infiltrates in both cases; after several days, these confluent infiltrates developed into pneumatoceles. Both children were treated with antibiotics and steroids. They recovered within three and four weeks, respectively, with complete remission of the radiologic abnormalities and had an uneventful follow-up after discharge.