Neuromuscular Electrical Stimulation to Immobilized Lower Extremities Directly Following Orthopaedic Surgery in Three Children with Cerebral Palsy: A Case Series.

Cerebral palsy (CP) is a non-progressive, neurological disorder often resulting in secondary musculoskeletal impairments affecting alignment and function which can result in orthopaedic surgery. Neuromuscular electrical stimulation (NMES) is a modality that can be used for rehabilitation; however, NMES immediately following orthopaedic surgery in children with CP using surface electrodes has not been previously reported. The purpose of this case series is to describe the novel use of NMES in the acute rehabilitation phase directly after orthopaedic surgery. This case series included three children with spastic diplegia CP, Gross Motor Function Classification System level II who underwent Single Event Multi-Level orthopaedic Surgery. Each long leg cast contained window cast cut-outs to allow for surface electrode placement for daily NMES intervention to the quadriceps muscles while immobilized. Children were assessed pre- and post-operatively using the Functional Mobility Scale (FMS), Gross Motor Function Measure (GMFM-66), and 6-Minute Walk Test (6MWT). All children demonstrated no adverse effects using NMES intervention and had improvements in the 6MWT. Most children demonstrated gains in the FMS and GMFM-66. Use of NMES through window cast-cuts in a long leg cast is a novel practice approach for delivery of early rehabilitation following lower extremity orthopaedic surgery.

Hypomagnesemia due to two novel TRPM6 mutations.

BACKGROUND: Although most hypocalcemia with hypomagenesemia in the neonatal period is due to transient neonatal hypoparathyroidism, magnesium channel defects should also be considered. CASE: We report a case of persistent hypomagnesemia in an 8-day-old Hispanic male who presented with generalized seizures. He was initially found to have hypomagnesemia, hypocalcemia, hyperphosphatemia and normal parathyroid hormone. Serum calcium normalized with administration of calcitriol and calcium carbonate. Serum magnesium improved with oral magnesium sulfate. However, 1 week after magnesium was discontinued, serum magnesium declined to 0.5 mg/dL. Magnesium supplementation was immediately restarted, and periodic seizure activity resolved after serum magnesium concentration was maintained above 0.9 mg/dL. The child was eventually weaned off oral calcium and calcitriol with persistent normocalemia. However, supraphysiologic oral magnesium doses were necessary to prevent seizures and maintain serum magnesium at the low limit of normal. METHODS AND RESULTS: As his clinical presentation suggested primary renal magnesium wastage, TRPM6 gene mutations were suspected; subsequent genetic testing revealed the child to be compound heterozygous for TRPM6 mutations. CONCLUSION: Two novel TRPM6 mutations are described with a new geographic and ethnic origin. This case highlights the importance of recognizing disorders of magnesium imbalance and describing new genetic mutations.

DIABETES AND PREDIABETES ARE SIGNIFICANTLY HIGHER IN MORBIDLY OBESE CHILDREN COMPARED WITH OBESE CHILDREN.

OBJECTIVE: The objective of this study was to examine the prevalence and characteristics of comorbidities in obese and morbidly obese children with a comparison between the 2 sets of children. METHODS: This was a retrospective electronic chart review of obese and morbidly obese children and adolescents as defined by body mass index. We evaluated medical history of comorbid conditions, medication use, and cardiovascular risk markers, including blood pressure, lipid profile, and glycosylated hemoglobin. RESULTS: There were 1,111 subjects (African American = 635; non-Hispanic white = 364; Hispanic = 36; others = 86), of which 274 were obese and 837 were morbidly obese children with a mean age of 12.7 ± 3.37 years. Morbidly obese children had a higher prevalence of prediabetes (19.5% of obese versus 27.3% of morbidly obese; P<.0001) and type 2 diabetes (39.8% of obese versus 52.4% of morbidly obese; P<.0001). Use of medications for treatment of asthma was significantly higher in the morbidly obese group compared with the obese group (21% versus 14%; P = .01). CONCLUSION: Morbidly obese children have a higher prevalence of diabetes, prediabetes, and use of asthma medications compared with obese children.

Diabetes Status and Race are Associated with Cardiovascular Risk Markers in Obese Adolescents.

OBJECTIVE: The objective of this study was to evaluate differences in cardiovascular disease (CVD) risk markers in obese adolescents based on diabetes status and race in order to improve risk-reduction intervention strategies. METHODS: This was a retrospective, cross-sectional study of obese adolescents, age 10 to 21 years, who were evaluated at Children's of Alabama between 2000 and 2012. Subjects were classified by glycated hemoglobin (HbA1c) as having normoglycemia, prediabetes, or type 2 diabetes mellitus (T2DM). RESULTS: There were a total of 491 African American (AA) or Caucasian American (CA) subjects. Body mass index was not different between HbA1c and racial groups. Compared to subjects with normoglycemia or prediabetes, subjects with T2DM had higher levels of total cholesterol (TC) (178.6 ± 43.8 mg/dL vs. 161.5 ± 32.5 mg/dL vs. 162.4 ± 30.6 mg/dL; P<.0001) and low-density-lipoprotein cholesterol (107.4 ± 39.2 mg/dL vs. 97.0 ± 31.0 mg/dL vs. 97.5 ± 26.9 mg/dL; P = .0073). Compared with AA subjects, CA subjects had lower high-density-lipoprotein cholesterol (HDL-C) levels (40.4 ± 10.4 mg/dL vs. 44.3 ± 11.9 mg/dL; P = .0005) and higher non-HDL-C levels (129.6 ± 36.2 mg/dL vs. 122.5 ± 37.5 mg/dL; P = .0490). Of the characteristics studied, HbA1c had the most significant positive association with dyslipidemia and was strongly correlated with both TC (ß, 4.21; P<.0001) and non-HDL-C (ß, 4.3; P<.0001). CONCLUSION: Obese adolescents with T2DM have more abnormal lipoprotein profiles than those with normoglycemia or prediabetes. Obese CA adolescents have more abnormal lipids than obese AA adolescents. HbA1c was the characteristic most highly associated with abnormal lipoprotein profiles in our subjects. Our results show that CVD risk markers in obese adolescents vary by race and HbA1c concentration.

Anatomy of female puberty: The clinical relevance of developmental changes in the reproductive system.

Puberty is the period of biologic transition from childhood to adulthood. The changes that occur at this time are related to the increasing concentrations of sex steroid hormones. In females, most pubertal changes are caused by estrogen stimulation that results from the onset of central puberty. Significant development occurs in the organs of the female reproductive system and results in anatomic changes that characterize reproductive maturity. Adrenal and ovarian androgens also increase during puberty, affecting change that includes the promotion of certain secondary sex characteristics. The ability to recognize normal pubertal anatomy and distinguish between estrogen and androgen effects is important in the ability to diagnose and treat disorders of sex development, precocious puberty, pubertal delay, and menstrual irregularities in children and adolescents. An understanding of this developmental process can also help clinicians identify and treat reproductive pathology in adults and across all female life stages.