SHOX gene variants: growth hormone/insulin-like growth factor-1 status and response to growth hormone treatment.

CONTEXT: Short stature homeobox-containing gene (SHOX) variants of unknown clinical significance occur frequently among children with short stature, yet their growth hormone (GH)/insulin-like growth factor-1 (IGF-1) status and response to GH have not been studied. OBJECTIVE: To define GH and IGF-1 status in children with SHOX variants and assess their response to GH. PATIENTS AND METHODS: This is a retrospective review of children with short stature. Children with SHOX variants were compared to those with no variants. Height standard deviation scores (SDS) and IGF-1 SDS at baseline and during GH treatment at 6, 12, and 24 months were analyzed. Growth velocity (GV), maximum GH dose, IGF-BP3, and changes in height SDS, IGF-1 SDS, and GV were compared. RESULTS: Among 355 children, 83 (23%) had SHOX variants. Nineteen different SHOX variants were detected. There was no difference in age, height SDS, IGF-1 SDS, or IGF-BP3 between children with SHOX variants and those with normal SHOX. Height SDS, IGF-1 SDS, IGF-BP3, GV, and GH dose were not different between patients with SHOX variants and those without. CONCLUSIONS: The GH and IGF-1 characteristics of children with short stature were not different between children with SHOX+ variants and children with no variants. Although these findings suggest that SHOX variants are polymorphisms, studies prospectively comparing individual SHOX variants are needed.

National study of continuity clinic satisfaction in pediatric fellowship training.

BACKGROUND: A national online survey was conducted to evaluate pediatric subspecialty fellow satisfaction regarding continuity clinic experience. METHODS: An anonymous online survey (SurveyMonkey™) was developed to evaluate demographics of the program, clinic organization, and patient and preceptor characteristics, and to compare fellow satisfaction when fellows were the primary providers with faculty supervision versus attending-run clinics assisted by fellows or a combination of the two models. Pediatric subspecialty fellows in a 3-year Accreditation Council for Graduate Medical Education accredited program in the United States (excluding emergency medicine, neonatology, and critical care) were invited to participate. RESULTS: There were 644 respondents and nearly half (54%) of these had fellow-run clinics. Eighty-six percent of fellows responded that they would prefer to have their own continuity clinics. Higher satisfaction ratings on maintaining continuity of care, being perceived as the primary provider, and feeling that they had greater autonomy in patient management were associated with being part of a fellow-run clinic experience (all P < 0.001). Additionally, fellow-run clinics were associated with a feeling of increased involvement in designing a treatment plan based on their differential diagnosis (P < 0.001). There were no significant associations with patient or preceptor characteristics. CONCLUSION: Fellow-run continuity clinics provide fellows with a greater sense of satisfaction and independence in management plans.

Foster care and type 1 diabetes in the Bronx: a case series.

OBJECTIVE: The objective of this retrospective study was to describe the health status of children with type 1 diabetes mellitus (T1DM) in foster care. RESEARCH DESIGN AND METHODS: A retrospective chart review of children with T1DM in foster care at the Children's Hospital at Montefiore (CHAM) in Bronx, NY, USA, was performed. RESULTS: All patients were either African American or Hispanic and raised by single mothers. The majority of referrals were for medical neglect. The time spent in foster care ranged from 1 to 7 years, with 1-12 placements. Only two children were reunified with their biological mothers. Extensive financial burdens on the health-care system for children with diabetes including prolonged hospitalizations awaiting placement, frequent hospital admissions, and support services were noted. CONCLUSIONS: To our knowledge, this is the first report on children with T1DM in foster care. Poor glycemic control and suboptimal social outcomes were noted in the children we report in our case series. Programs geared to improve and reform foster care for children with diabetes are needed.

Hyperglycemia and outcome in the pediatric intensive care unit.

OBJECTIVE: To identify the frequency of hyperglycemia in children who are nondiabetic and critically ill and assess the independent effect of hyperglycemia on outcome. STUDY DESIGN: Consecutive admissions to the pediatric intensive care unit (PICU) were reviewed. The Pediatric Risk of Mortality III score (PRISM) measured patient acuity. Because maximum glucose level in the first day of PICU admission (GLFD) >200mg/dL contributes to PRISM, 200 mg/dL was used to differentiate high glucose (HG) from normal glucose. RESULTS: Of 1550 patients, 221 (14.3%) had HG. GLFD correlated with PRISM (r = 0.39, P < .001). Without controlling for PRISM, the HG group had more mechanical ventilation days (MVD; P < .001), longer PICU length of stay (PLOS; P < .001) and lower percent survival (P < .001) than the normal glucose group. Controlling for PRISM in survivors, GLFD was not associated with PLOS (P = .75) or with MVD (P = .06). GLFD was not significantly associated with survival (P = .76). In nonsurvivors, GLFD was not associated with PLOS (P = .19) or MVD (P = .31). CONCLUSION: When controlling for disease severity, hyperglycemia within 24 hours of PICU admission was not independently associated with increased mechanical ventilation time, length of stay, or mortality. Prospective evaluation of glycemic control in critically ill children is needed to elucidate its effects on outcome.

Hyperglycemia in the pediatric intensive care unit.

PURPOSE OF REVIEW: Studies on critically ill adults demonstrate the benefits of glycemic control. There is a paucity of data, however, in pediatric intensive care settings. This review summarizes sentinel papers in the adult literature, outlines mechanisms by which hyperglycemia mediates its effects in the critically ill, highlighting those described in pediatrics, and discusses studies that associate hyperglycemia with negative outcome in critically ill children. RECENT FINDINGS: Retrospective studies and prospective cohort studies have linked hyperglycemia to worse outcome in critically ill children. Investigations in small, homogenous groups, such as trauma, sepsis, burn and neonatal patients, have shown negative associations between hyperglycemia and injury-specific outcomes and have elucidated previously proposed mechanisms of tissue injury in children. In addition, certain properties of hyperglycemia, such as duration, peak, and excursion, may be more relevant than absolute levels of glucose. Larger studies generalize findings to heterogeneous pediatric intensive care populations, across ages and diagnoses. Further, in studies accounting for insulin administration, no obvious increases in hypoglycemia-related morbidity have been noted. SUMMARY: Glucose control in pediatric intensive care has been receiving increasing attention. Large, prospective studies are needed to address certain issues in pediatrics, such as differences in diseases, target values, complications of disease, risks and sequelae of hypoglycemia and logistical challenges.