Development of pediatric oncology supportive care indicators: Evaluation of febrile neutropenia care in the north of the Netherlands.

INTRODUCTION: Febrile neutropenia (FN) is a common complication of the intensive treatment strategies used in pediatric oncology. By close adherence to high-quality guidelines, which can be evaluated by indicators, the burden of FN can potentially be reduced. OBJECTIVES: The aims of this study were tripartite-(1) to develop structure, process, and outcome indicators, (2) to evaluate the implementation of the Dutch Childhood Oncology Group (DCOG) guideline on FN, and (3) to produce baseline measures on local quality of FN care (in the north of the Netherlands). METHODS: Seven indicators derived from the DCOG guideline were developed. Regarding structure indicators, we gathered information from all local centers providing care for children with cancer (n = 9). Regarding process and outcome indicators, we collected individual patient data from one academic and two shared-care hospitals. Children (<18 years) were included if they had been diagnosed with cancer in 2014 or 2015 and had suffered from FN. RESULTS: Six out of nine hospitals used the DCOG guideline on FN and three hospitals used an outdated supportive care handbook. Regarding individual patient data, we included 119 FN episodes in 59 patients. All FN episodes without focus were initially treated with guideline-based antibiotics. Of all FN episodes, 18.5% resulted in intensive care unit (ICU) admittance. Cumulative incidence of death during FN was 1.74%. CONCLUSION: Adherence to the DCOG guideline at the individual patient level was excellent. However, indicators concerning mortality and ICU admittances showed that FN still has devastating consequences. Subsequently, we will implement these indicators nationwide in order to improve FN care.

Renal function in glycogen storage disease type I, natural course, and renopreservative effects of ACE inhibition.

BACKGROUND AND OBJECTIVES: Renal failure is a major complication in glycogen storage disease type I (GSD I). We studied the natural course of renal function in GSD I patients. We studied differences between patients in optimal and nonoptimal metabolic control and possible renoprotective effects of angiotensin converting enzyme inhibition. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Thirty-nine GSD I patients that visited our clinic were studied. GFR and effective renal plasma flow (ERPF) were measured by means of I(125) iothalamate and I(131) hippuran clearance and corrected for body surface area. Microalbuminuria was defined as >2.5 mg albumin/mmol creatinine and proteinuria as >0.2 g protein per liter. Optimal metabolic control was present when blood glucoses were >3.5 mmol/L, urine lactate/creatinine ratios <0.06 mmol/mmol, triglycerides <6.0 mmol/L, and uric acid concentrations <450 micromol/L. RESULTS: Quadratic regression analysis showed a biphasic pattern in the course of GFR and ERPF related to age. Microalbuminuria was observed significantly less frequently in the patients with optimal metabolic control compared with the patients with nonoptimal metabolic control. A significant decrease in GFR was observed after starting ACE inhibition. CONCLUSIONS: This study describes a biphasic pattern of the natural course of GFR and ERPF in GSD I patients, followed by the development of microalbuminuria and proteinuria. Optimal metabolic control has a renoprotective effect on the development of microalbuminuria and proteinuria in GSD I patients. Treatment with ACE inhibitors significantly decreases the GFR, especially in GSD I patients with glomerular hyperfiltration.

Pregnancies in glycogen storage disease type Ia.

OBJECTIVE: Reports on pregnancies in women with glycogen storage disease type Ia (GSD-Ia) are scarce. Because of improved life expectancy, pregnancy is becoming an important issue. We describe 15 pregnancies by focusing on dietary treatment, biochemical parameters, and GSD-Ia complications. STUDY DESIGN: Carbohydrate requirements (milligrams per kilogram per minute), triglyceride and uric acid levels, liver ultrasonography, and creatinine clearance were investigated before, during, and after pregnancy. Data from the newborn infants were obtained from the records. RESULTS: In the first trimester, a significant increase in carbohydrate requirements was observed (P = .007). Most patients had acceptable triglyceride and uric acid levels during pregnancy. No increase in size or number of adenomas was seen. In 3 of 4 patients, a decrease in glomerular filtration rate was observed after pregnancy. In 3 pregnancies, lactic acidosis developed during delivery with severe multiorgan failure in 1. All but 1 of the children are healthy and show good psychomotor development. CONCLUSION: Successful pregnancies are possible in patients with GSD-Ia, although specific GSD-Ia-related risks are present.