Volume 23, Issue 6

ABSTRACT Genotropin (somatropin) is licensed for the treatment of children with growth hormone deficiency, Prader-Willi syndrome, Turner syndrome, chronic renal insufficiency and in children born small for gestational age. This systematic review (SR) evaluated the clinical efficacy and effectiveness of Genotropin in these conditions to inform a NICE Technology Appraisal of growth hormone for the treatment of growth failure in children. Search terms were used to search seven databases, including Medline and Embase, for English language studies. Randomised controlled trials (RCTs) or observational studies investigating Genotropin in children were included. Out of 30 RCTs identified, one reported final height data. Eleven observational studies reported final height and seven were based on the Pfizer International Growth Survey (KIGS). This SR highlights the lack of long-term RCTs reporting final height data and other important qualitative outcomes, such as quality of life. Observational data, such as those from KIGS, remain vital for informing therapy.

ABSTRACT Background : The Thriposha programme in Sri Lanka provides a combination of energy, protein and micronutrients as a ‘ready-to-eat’ cereal-based food. Objective : To assess the effectiveness of calcium and vitamin D 3 in the Thriposha on bone mineralization among preschool children aged 3-5 years. Design : Subjects (n=30) were fed with conventional Thriposha while the control group (n=30) children were fed without mineral and vitamin premix (Corn-Soya-Blend - CSB) for a period of nine months. Dual-energy X-ray absorptiometry (DXA) of total spine was measured at the baseline and after the intervention. Results : The mean baseline total spine BMD was 0.464(0.050) g/cm 2 in the interventional group and 0.453(0.035) g/cm 2 in the control group (p=0.09). At the end of the study, the BMD levels were 0.487(0.047) and 0.454(0.031) g/cm 2 (p<0.001) respectively. Conclusion : Daily supplementation of cereal based food supplement over a period of nine months improved the total spine BMD.

ABSTRACT Psychosexual development is influenced by biological and psychosocial factors. Human beings show a great variability in psychosexual development both between and within gender-groups. However, there are relatively stable gender-related behaviors and self-perceptions, in which males and females differ distinctly. There is strong evidence that high concentrations of androgens lead to more male-typical behavior and that this also influences gender identity. Disorders of sex development (DSD) provide the opportunity to analyze the role of different factors on psychosexual development. We examined 166 children age 4 to 12 with DSD using instruments concerning gender role behavior, gender identity, and friendship. Results underline the hypothesis, that androgens play a decisive role in the masculinization of gender role behavior in children. There are also some relations between the experience of gender change and psychosexual outcomes which have to be discussed. Nevertheless, results indicated a high congruence between the children's gender identity and gender of rearing.

ABSTRACT In this study we examined whether salivary hormones, physical activity and adiposity were correlated with secretory immunoglobulin A (sIgA) and frequency of upper respiratory tract infections (URTI) in 43 early-pubertal and 59 late-pubertal girls. Physical activity was measured using accelerometers and relative body fat was assessed using bioelectrical impendence. Resting saliva samples were obtained between 1500 and 1800hr and assayed for sIgA, cortisol and testosterone. Participants completed a one-month health log to record URTI frequency. Early-pubertal girls were more physically active, had less adiposity, but lower concentrations of sIgA than late-pubertal adolescents (122.7±91.6 vs 201.9±102.9 pg/ml, respectively). The frequency of URTI was similar in the two groups. Neither sIgA nor URTI were correlated with salivary hormones, physical activity or adiposity within the early-pubertal girls. In the late-pubertal group, sIgA was negatively associated (r=-0.44; p ≤0.05) with cortisol, and positively associated (r=0.41; p ≤0.05) with the testosterone to cortisol ratio. These results suggest that mucosal immunity increases with pubertal maturation, while higher cortisol is associated with lower mucosal immunity in adolescents.

ABSTRACT Childhood obesity has increased to epidemic levels and is considered a public health problem due to its association with a number of metabolic abnormalities, which are being detected at earlier stages of life. The objective was to evaluate the association between the presence of subclinical metabolic abnormalities (SMA) and obesity in a sample of pre–pubertal Mexican schoolchildren. Children of both sexes and 6 to 13 years old were questioned for signs of puberty, underwent anthropometric measurement and had their Body Mass Index (BMI) calculated. Two groups were formed: those with obesity (case group) and those with normal weight paired by age and chosen randomly (control group). Fasting insulin, glucose and cholesterol were measured. 92 children were included, 46 in each group, mean age 9.9 and 9.5 years old, respectively (p=0.97). A higher frequency of hyperinsulinism was found in the case group: Fasting insulin >15 mU/ml, 75% vs. 21% (case group vs. control group, respectively); fasting glucose to insulin ratio <6, 72% vs. 24%; HOMA IR >2.7, 83% vs. 14%; and decrease in QUICKI (<0.3), 80% vs. 19% (p=0.000). Hypercholesterolemia was 25% vs. 15% (p=0.22), impaired fasting glucose 28% vs. 8% (p=0.01), and family history of diabetes mellitus (DM) 35% vs. 9% (OR=5.6; 95%CI=1.5–22.2; p=0.002). In this sample of Mexican schoolchildren, obesity was associated to a higher frequency of SMA, such as hyperinsulinism and impaired fasting glucose, and to a family history of DM.

ABSTRACT Rickets is an important problem in children. The majority of rickets in children is due to deficiency of calcium, phosphorus or vitamin D. However, rickets may also be a feature of renal diseases, e.g. renal tubular acidosis, hypophosphatemic rickets or rickets associated with renal insufficiency. The treatment varies with etiology and hence complete workup is essential before initiating therapy.

ABSTRACT Cushing's syndrome is an endocrine disorder caused by hypercortisolism. Despite its varied clinical spectrum of presentation, Cushing's disease has not been reported to present as arthritis. Moreover, keratilysis exfolitiva has not been described in Cushing disease. We report a 12 year old male child with pituitary micro adenoma initially presenting as arthritis and developing keratilysis exfolitiva

ABSTRACT Cushing's syndrome (CS) is rare in childhood and adolescence and its diagnosis and work up are often challenging. We report the case of a 15-year-old girl with a recurrent corticotrophin (ACTH)-secreting adenoma, located in the posterior lobe of the pituitary gland. At the age of 11, she presented with classic CS symptoms; biochemical investigation was compatible with ACTH-dependent Cushing disease, although pituitary gland imaging did not show any tumor. Following transsphenoidal surgery (TSS), histopathological analysis identified an ACTH-secreting pituitary microadenoma arising from the posterior gland. The patient went into remission but 4 years later she presented with recurrent CS; this time, pituitary gland imaging showed a microadenoma located in the posterior lobe, which was resected after TSS. Posterior lobe pituitary adenomas are very rare and often hard to diagnose and treat; this is the first case of such a tumor causing recurrent Cushing's disease in a child.

ABSTRACT Cushing's syndrome (CS) is uncommon in childhood. CS may be either dependent or independent of adrenocorticotrophic hormone (ACTH). ACTH independent micronodular adrenocortical (MAD) disease may present in the second to third decade of life or between ages 2-3years. It may occur in isolation, or as a part of the Carney complex and it represents an elusive entity to diagnose. We present a 3 year 7 month old boy with isolated MAD (iMAD). Abdominal CT revealed prominent mildly lobulated anteromedial margin of adrenals with nodular appearance. Cardiac echo, thyroid and testicular ultrasounds performed as a work up for Carney complex were normal. Bilateral adrenalectomy confirmed MAD as the cause of CS. We present the history and identification of a unique case of iMAD.

ABSTRACT Adrenocortical tumors are the most common cause of endogenous Cushing syndrome in infancy and early childhood. Cushing syndrome resulting from ectopic adrenocorticotrophic hormone (ACTH) producing tumor has been infrequently reported in adults. Cortisol secreting pheochromocytoma is rarely reported in literature. We report an eleven month old child presenting to us as Cushing syndrome with hypertension due to left adrenal tumor. He was treated with antihypertensives and left adrenalectomy was done under perioperative glucocorticoid coverage. Diagnosis of pheochromocytoma was made only after histopathology. Despite the rare association of Cushing syndrome and pheochromocytoma, preoperative diagnosis of pheochromocytoma is required for appropriate perioperative medical and anaesthetic management to prevent life threatening complications.

ABSTRACT Background : even after successful surgical cure, Cushing's Syndrome (CS) may induce cardiovascular (CV) diseases including hypertension, or elicit onset of CV risk factors, such as obesity. Objectives : to evaluate some early markers of CV dysfunction in adolescent girls before and after successful surgical cure of CS; to compare after surgery data with those of a control group (C) of healthy young girls Methods : 23 girls affected by CS were enrolled (range: 11-17 years; mean: 14.3 ± 1.7 years). Epicardial fat thickness (EFT), intima-media thickness (IMT), N-terminal pro B-type natriuretic peptide (NT-proBNP) were assessed; transthoracic echocardiogram coupled with Tissue Doppler imaging (TDI) was performed. Results (CS before surgery vs. CS after surgery): EFT (p<0.05). (CS after surgery vs. C): EFT (p=0.0001); IMT (p=0.0001); NT-proBNP (p=0.0001). TDI: isovolumetric relaxation time (p=0.001); isovolumetric contraction time (p=0.001); myocardial performance index (p=0.001). Significant correlations: NT-proBNP with IRT ( r = 0.45, p = 0.026 ), ICT (r = 0.47, p = 0.028), and myocardial performance index (r = 0.51, p = 0.0032). Conclusions : important structural and functional modifications of heart and vessels are present in young female CS despite successful surgical cure, even in paediatric age. It underlines their significantly higher cardiovascular risk. Our findings confirm that EFT, IMT, NT-proBNP and TDI diastolic parameters, not previously tested in paediatric CS, are early markers of cardiovascular dysfunction. Because of its relationship with TDI, the assessment of NT-proBNP proves to be the best marker in detecting a cardiovascular dysfunction in this specific population.