Pediatrics Subject Collection: Endocrinology

Index of Suspicion [ARTICLES]

Vo, M. — 2009-11-01

Case 1 Presentation Case 1 Presentation Case 2 Presentation Case 3 Presentation Case 1 Discussion Case 2 Discussion Case 3 Discussion A previously healthy 9-year-old girl presents with a 1-week history of intermittent confusion, dysphagia, visual changes, and involuntary movements of the face and left hand. During this period, she has had two episodes of disorientation and memory loss that were not associated with alteration in consciousness or behavioral changes. The involuntary movements of the left arm and leg have become progressively worse throughout the week. The facial movements are described as lip smacking and excessive blinking. Four days ago, she complained of one episode of diplopia. She denies nausea, vomiting, fever, symptoms of uppe ...

Disaster Preparedness in Pediatric Type 1 Diabetes Mellitus [ARTICLES]

Renukuntla, V. S. — 2009-11-01

OBJECTIVE: The goal was to assess emergency preparedness among families caring for children with type 1 diabetes mellitus. METHODS: A total of 115 English-speaking families caring for children with type 1 diabetes mellitus who were attending the diabetes clinic at Texas Children's Hospital agreed to a questionnaire study designed to ascertain their level of preparedness for a disaster or emergency. The study was conducted from June through September 2008 and ended just before Hurricane Ike made landfall. RESULTS: Families were better prepared for self-management of diabetes, compared with general disaster preparedness. Sixty-two percent of the families were generally unprepared for a major disaster. For self-management of diabetes specifically, however, 75% of families had adequate supplies to maintain care for 3 days. Families in higher and moderate socioeconomic status strata were better equipped for an emergency (P < .002). Preparedness was found to be independent of age, gender, ethnicity, and previous experience of a disaster. CONCLUSION: Disaster preparedness still lags in families of lower socioeconomic status.

A Pilot Trial of Pramlintide Home Usage in Adolescents With Type 1 Diabetes [ARTICLES]

Kishiyama, C. M. — 2009-11-01

OBJECTIVE: The objective of this study was to evaluate the safety and efficacy of home pramlintide use in adolescents with type 1 diabetes. PATIENTS AND METHODS: This was a randomized, 28-day pilot trial of pramlintide (maximum dose: 30 {micro}g per meal) in 10 adolescents aged 13 to 17 years. End points included changes in hemoglobin A1c (HbA1c) values, body weight, and postprandial peak blood glucose levels and area under the curve on continuous glucose monitoring. RESULTS: Changes in HbA1c values, body weight, and total insulin dose declined in the treatment group compared with the control group (bootstrapped, P [≤] .02 for each). The treatment group also demonstrated lower average dinner area under the curve (P = .02) and lower maximum breakfast (P = .03) and dinner (P = .02) postprandial blood glucose values. CONCLUSIONS: Pramlintide can help some adolescents to decrease postprandial hyperglycemia, HbA1c values, body weight, and insulin dosages. Additional large-scale trials should now be considered.

Pediatrics in the Community: Global Help For Children Who Have Diabetes: AYUDA and Campo Amigo Ecuador [ARTICLES]

2009-10-01

Fourteen years ago, when he was a freshman in high school, Nicolas Cuttriss, currently a resident at Harbor-UCLA, met Jose Gabriel, a boy who had diabetes and was sent by his parents from Quito, Ecuador, to the United States for help. A subsequent trip by Dr Cuttriss to Quito provided an opportunity to live with Jose's family. After listening to stories of the difficulties Ecuadorian families faced in coping with diabetes, Nick resolved to do something about the situation (Fig 1.). Along with a friend, he founded American Youth Understanding Diabetes Abroad (AYUDA), a nonprofit organization built on the traditional diabetes camp model. AYUDA's work began with Campo Amigo Ecuador (CAE), a weeklong experience that brings youth who have diabetes together in a safe recreational environment ...

Impact of Growth Hormone Therapy on Adult Height of Children Born Small for Gestational Age [REVIEW ARTICLES]

Maiorana, A. — 2009-09-01

CONTEXT: Use of growth hormone (GH) therapy to promote growth in short children born small for gestational age (SGA) was recently approved in the United States and Europe, but there is still disagreement about the magnitude of effectiveness of GH. OBJECTIVE: To determine the impact of GH therapy on adult height in short SGA children by a meta-analysis of randomized, controlled trials (RCTs). METHODS: We performed a systematic review of controlled studies using as data sources the Cochrane Central Register of Controlled Trials, Medline, and the bibliographic references from all retrieved articles describing RCTs up to November 2008. A meta-analysis of all RCT studies conducted up to the achievement of adult height was performed. Inclusion criteria were birth weight and/or length below -2 SD score (SDS), initial height less than -2 SDS, and GH dose range of 33 to 67 {micro}g/kg per day. Adult height SDS and overall height gain SDS were the primary outcome measures. RESULTS: Four RCTs (391 children) met the inclusion criteria. The adult height of the GH-treated group significantly exceeded controls by 0.9 SDS. Mean height gain was 1.5 SDS in treated versus 0.25 SDS in untreated SGA subjects. No significant difference in adult height was observed between the 2 GH dose regimens. CONCLUSIONS: GH therapy seems to be an effective approach to partially reduce the adult height deficit in short SGA children. However, the response to therapy is highly variable, and additional studies are needed to identify the responders.

Hospitalization Subsequent to Diagnosis in Young Patients With Diabetes in Chicago, Illinois [ARTICLES]

Estrada, C. L. — 2009-09-01

OBJECTIVES: Rehospitalization after a diabetes diagnosis in youth signals the failure of outpatient management. We examined risk factors for rehospitalization among young patients with diabetes. PATIENTS AND METHODS: We queried 535 participants diagnosed before 18 years of age from the Chicago Childhood Diabetes Registry. Demographic, social, and clinical data were used in logistic models of diabetes-related rehospitalization, as well as, among those rehospitalized, frequent ([≥] once per 2 years' duration) versus infrequent rehospitalization rates. RESULTS: Mean (range) duration was 5.1 years (0.1-19.2 years). The sample was 55% non-Hispanic black, 11% non-Hispanic white, 26% Hispanic, and 7% other/mixed race; 86% had presumed type 1 diabetes; and 47% were underinsured. Overall, 46% reported rehospitalization for diabetes. In multivariable logistic regression, ever being rehospitalized was significantly associated with diabetes duration (per year, odds ratio [OR]: 1.26; P < .01), female gender (OR: 1.67; P = .01), underinsurance (versus private insurance; OR: 1.79; P < .01), presumed phenotype (non-type 1 diabetes versus type 1; OR: 0.32; P < .01), and diagnosis at a community hospital (versus tertiary care facility; OR: 1.96; P < .01) and tended to be higher for those of nonwhite race (OR: 1.94; P = .07). Among those rehospitalized, multivariable associations with frequent rehospitalization were presumed phenotype (non-type 1 diabetes versus type 1; OR: 2.74; P = .04), head of household not working (versus employed; OR: 1.88; P = .02), and younger age at questionnaire (per year; OR: 0.94; P = .01). CONCLUSIONS: Rehospitalization is common in young patients with diabetes, especially for those with limited resources, indicating the need for improved outpatient services. Comprehensive initial education and support available to young patients with diabetes diagnosed at tertiary care facilities and their families may have lasting protective effects.

Phase 1 Trial of 4 Thyroid Hormone Regimens for Transient Hypothyroxinemia in Neonates of <28 Weeks' Gestation [ARTICLES]

La Gamma, E. F. — 2009-08-01

BACKGROUND: Transiently low levels of thyroid hormones occur in [~]50% of neonates born 24-28 weeks' gestation and are associated with higher rates of cerebral palsy and cognitive impairment. Raising hormone levels shows promise for improving neurodevelopmental outcome. OBJECTIVE: To identify whether any of 4 thyroid hormone supplementation regimens could raise T4 and FT4 without suppressing TSH (biochemical euthyroidism). METHODS: Eligible subjects had gestational ages between 24[Formula] and 27[Formula] weeks and were randomized <24 hours of birth to one of six study arms (n = 20-27 per arm): placebo (vehicle: 5% dextrose), potassium iodide (30 {micro}g/kg/d) and continuous or bolus daily infusions of either 4 or 8 {micro}g/kg/d of T4 for 42 days. T4 was accompanied by 1 {micro}g/kg/d T3 during the first 14 postnatal days and infused with 1 mg/mL albumin to prevent adherence to plastic tubing. RESULTS: FT4 was elevated in the first 7 days in all hormone-treated subjects; however, only the continuous 8 {micro}g/kg/d treatment arm showed a significant elevation in all treatment epochs (P < .002 versus all other groups). TT4 remained elevated in the first 7 days in all hormone-treated subjects (P < .05 versus placebo or iodine arms). After 14 days, both 8 {micro}g/kg/d arms as well as the continuous 4 {micro}g/kg/d arm produced a sustained elevation of the mean and median TT4, >7 {micro}g/dL (90 nM/L; P < .002 versus placebo). The least suppression of THS was achieved in the 4 {micro}g/kg/d T4 continuous infusion arm. Although not pre-hypothesized, the duration of mechanical ventilation was significantly lower in the continuous 4 {micro}g/kg/d T4 arm and in the 8 {micro}g/kg/d T4 bolus arm (P < .05 versus remaining arms). ROP was significantly lower in the combined 4 thyroid hormone treatment arms than in the combined placebo and iodine arms (P < .04). NEC was higher in the combined 8 {micro}g/kg/d arms (P < .05 versus other arms). CONCLUSIONS: Elevation of TT4 with only modest suppression of TSH was associated with trends suggesting clinical benefits using a continuous supplement of low-dose thyroid hormone (4 {micro}g/kg/d) for 42 days. Future trials will be needed to assess the long-term neurodevelopmental effects of such supplementation.

Index of Suspicion in the Nursery [ARTICLES]

Podraza, J. — 2009-08-01

Case Presentation Case Presentation Case Discussion Suggested Reading A female infant presents with extreme tachycardia and tachypnea on the sixth day after birth. She was born at 35-1/7 weeks' gestation dated by the mother's last menstrual period and first trimester ultrasonography. The 26-year-old G1P0 mother has a history of anemia and fibroids. Prenatal laboratory results were: AB+ blood type, syphilis screen-nonreactive, hepatitis B surface antigen-negative, human immunodeficiency virus-negative, rubella-immune, and gonorrhea- and chlamydia-negative. Group B streptococcal culture was pending at delivery. The mother presented in preterm labor several hours after rupture of membranes at home with clear amniotic fluid. The infant was delivered by cesarean section due to ...

Insulin Resistance and Oxidative Stress in Children Born Small and Large for Gestational Age [ARTICLES]

Chiavaroli, V. — 2009-08-01

OBJECTIVE: Our aim was to evaluate the effect of BW and obesity on oxidative stress and IR in prepubertal SGA and LGA children compared with appropriate-for-gestational-age (AGA) children. METHODS: We performed a cross-sectional study comparing oxidative stress and IR in 103 children categorized into 6 groups according to BW (26 SGA, 15 AGA, and 16 LGA normal-weight children) and obesity (15 SGA, 15 AGA, and 16 LGA obese children). Indexes of IR (HOMA-IR, G/I) and the marker of oxidative stress (urinary isoprostanes) were evaluated. RESULTS: Homeostasis Model Assessment was higher in both normal-weight SGA and LGA children than in normal-weight AGA children (all P [≤] .02). Furthermore, a difference was detected between obese SGA and obese LGA subjects compared with normal-weight SGA (all P [≤] .0007) and LGA (all P [≤] .01) children, respectively. The G/I ratio was lower in the 3 obese groups than normal-weight AGA (all P [≤] .009) and normal-weight SGA children (all P [≤] .02). Furthermore, a difference was detected between obese SGA and obese LGA children compared with normal-weight LGA children (all P [≤] .0002). Isoprostane levels were higher in both normal-weight SGA and LGA children than in normal-weight AGA children (all P [≤] .002). Moreover, both obese SGA and LGA children showed higher levels than obese AGA children (all P [≤] .01) and in comparison to the 3 normal-weight groups (all P [≤] .04). CONCLUSION: Increased IR and oxidative stress are already present in prepubertal normal-weight SGA and LGA children with a continuous alteration in relation to obesity, suggesting that BW and adiposity represent 2 independent risk factors for degenerative diseases.

Congenital Adrenal Hyperplasia: Diagnosis, Evaluation, and Management [INTERNET-ONLY ARTICLE]

Antal, Z. — 2009-07-01

Objectives Objectives Introduction Case Presentation Adrenal Steroidogenesis Sex Differentiation Genetics Clinical Features Biochemical and Hormonal Studies Treatment Prenatal Diagnosis and Treatment Newborn Screening Differential Diagnosis of... Long-term Outcome Suggested Reading After completing this article, readers should be able to: Describe the pathophysiology of congenital adrenal hyperplasia (CAH). Characterize the signs and symptoms of CAH. Describe the appropriate laboratory evaluation of CAH. Know that CAH can be diagnosed prenatally. Recogn ...