Vitamin D deficiency in pediatric patients with cystic fibrosis: associated risk factors in the northern United States.

OBJECTIVES: Cystic fibrosis (CF) is associated with vitamin D deficiency, which can lead to adverse effects including recurrent pulmonary infections and osteoporosis. We longitudinally investigated calcifediol or 25-hydroxyvitamin D (25(OH)D) levels for our pediatric patients with CF based on the time of year as well as vitamin D supplementation dosing ranges for these patients at our CF center. METHODS: We retrospectively evaluated vitamin D deficiency in our pediatric CF center for 2 years (baseline and annually) while evaluating 25(OH)D serum changes based on vitamin D supplementation, seasonality, patient age, and other factors associated with CF. RESULTS: Vitamin D supplementation was noted to be higher than current Cystic Fibrosis Foundation dosing recommendations, and no patient experienced vitamin D toxicity. Seasonality was a strong indicator of 25(OH)D levels, especially during summer or fall. Significantly fewer patients with initially low 25(OH)D levels maintained low levels at the conclusion of the study, suggesting benefit. Older patient age and higher supplemental dosing correlated with significantly lower 25(OH)D levels. CONCLUSIONS: This study suggests that targeted intervention among pediatric patients with CF living in northern latitudes of the United States, especially older children, is needed to prevent vitamin D deficiency.

Endoscopy in the diagnosis of intestinal graft-versus-host disease: is lower endoscopy with biopsy as effective in diagnosis as upper endoscopy combined with lower endoscopy?

BACKGROUND: Graft-versus-host disease (GvHD) causes morbidity and mortality in recipients of hematopoietic stem cell transplantation (SCT). This study assessed the distribution of GvHD in gastrointestinal (GI) biopsies from the upper and lower GI tract in pediatric patients who had undergone SCT and evaluated if there was correlation between biopsy findings and possible extra-intestinal manifestations of GvHD. PROCEDURE: We performed a retrospective chart review for all patients diagnosed with GvHD, who underwent both upper and lower endoscopy. We also reviewed pathology and clinical reports to determine which biopsy sites were diagnostic of GvHD and to evaluate for the possible presence of extra-intestinal manifestations GvHD at the time of biopsy. RESULTS: Twenty patients were identified who had undergone both upper and lower endoscopy for evaluation of GvHD. Patients with GvHD diagnosed on upper endoscopy also had GvHD identified in the sigmoid colon region 100% of the time (positive predictive value [PPV] = 1). In patients that were found to have underlying liver disease, GvHD was diagnosed in the sigmoid colon region 90% of the time (PPV = 0.9). CONCLUSION: Use of sigmoid biopsy for GvHD diagnosis is effective, safe, and less expensive compared to other endoscopic interventions.

Clostridium difficile Infection and Proton Pump Inhibitor Use in Hospitalized Pediatric Cystic Fibrosis Patients.

Children with cystic fibrosis (CF) often take proton pump inhibitors (PPIs), which helps improve efficacy of fat absorption with pancreatic enzyme replacement therapy. However, PPI use is known to be associated with Clostridium difficile-(C. diff-) associated diarrhea (CDAD). We retrospectively evaluated the incidence of C. diff infection from all pediatric hospital admissions over a 5-year period at a single tertiary children's hospital. We found significantly more C. diff-positive stool tests in hospitalized patients with CF compared to patients with no diagnosis of CF. However, use of a PPI was not associated with an increased risk of CDAD in hospitalized CF patients. In summary, C. diff infection is more common in hospitalized pediatric CF patients although PPI use may not be a risk factor for CDAD development in this patient population.

Sew it up! A Western Trauma Association multi-institutional study of enteric injury management in the postinjury open abdomen.

BACKGROUND: Use of damage control surgery techniques has reduced mortality in critically injured patients but at the cost of the open abdomen. With the option of delayed definitive management of enteric injuries, the question of intestinal repair/anastomosis or definitive stoma creation has been posed with no clear consensus. The purpose of this study was to determine outcomes on the basis of management of enteric injuries in patients relegated to the postinjury open abdomen. METHODS: Patients requiring an open abdomen after trauma from January 1, 2002 to December 31, 2007 were reviewed. Type of bowel repair was categorized as immediate repair, immediate anastomosis, delayed anastomosis, stoma and a combination. Logistic regression was used to determine independent effect of risk factors on leak development. RESULTS: During the 6-year study period, 204 patients suffered enteric injuries and were managed with an open abdomen. The majority was men (77%) sustaining blunt trauma (66%) with a mean age of 37.1 years±1.2 years and median Injury Severity Score of 27 (interquartile range=20-41). Injury patterns included 81 (40%) small bowel, 37 (18%) colonic, and 86 (42%) combined injuries. Enteric injuries were managed with immediate repair (58), immediate anastomosis (15), delayed anastomosis (96), stoma (10), and a combination (22); three patients died before definitive repair. Sixty-one patients suffered intra-abdominal complications: 35 (17%) abscesses, 15 (7%) leaks, and 11 (5%) enterocutaneous fistulas. The majority of patients with leaks had a delayed anastomosis; one patient had a right colon repair. Leak rate increased as one progresses toward the left colon (small bowel anastomoses, 3% leak rate; right colon, 3%; transverse colon, 20%; left colon, 45%). There were no differences in emergency department physiology, injury severity, transfusions, crystalloids, or demographic characteristics between patients with and without leak. Leak cases had higher 12-hour heart rate (148 vs. 125, p=0.02) and higher 12-hour base deficit (13.7 vs. 9.7, p=0.04), suggesting persistent shock and consequent hypoperfusion were related to leak development. There was a significant trend toward higher incidence of leak with closure day (χ for trend, p=0.01), with closure after day 5 having a four times higher likelihood of developing leak (3% vs. 12%, p=0.02). CONCLUSIONS: Repair or anastomosis of intestinal injuries should be considered in all patients. However, leak rate increases with fascial closure beyond day 5 and with left-sided colonic anastomoses. Investigating the physiologic basis for intestinal vulnerability of the left colon and in the open abdomen is warranted.