Insulin Resistance in children on Sodium Valproate - A hospital based cross-sectional study in Indian children.

Our objective was to compare the point prevalence of insulin resistance (IR) in children taking sodium valproate (VPA) and phenytoin sodium (PS) monotherapy for >1 year. 150 children, aged 6-18 years, were categorized (50 each) into - group A (VPA), group B (PS) and group C (healthy controls age-sex matched with group A). Groups were compared for metabolic complications and risk factors assessed. The point prevalence of IR and non-alcoholic fatty liver disease was significantly higher in children on VPA (12% and 34% respectively) than on PS and healthy controls, regardless of age, sex, pubertal and nutritional status. The presence of central obesity, acanthosis, hypertension, dyslipidaemia was significantly associated with IR but none showed an independent association on multivariate analysis. Therapy with VPA makes children susceptible to metabolic complications. Close monitoring will facilitate early detection and timely intervention.

Anisotropy in the thermal hysteresis of resistivity and charge density wave nature of single crystal SrFeO3-δ: X-ray absorption and photoemission studies.

The local electronic and atomic structures of the high-quality single crystal of SrFeO3-δ (δ~0.19) were studied using temperature-dependent x-ray absorption and valence-band photoemission spectroscopy (VB-PES) to investigate the origin of anisotropic resistivity in the ab-plane and along the c-axis close to the region of thermal hysteresis (near temperature for susceptibility maximum, Tm~78 K). All experiments herein were conducted during warming and cooling processes. The Fe L 3,2-edge X-ray linear dichroism results show that during cooling from room temperature to below the transition temperature, the unoccupied Fe 3d e g states remain in persistently out-of-plane 3d 3z2-r2 orbitals. In contrast, in the warming process below the transition temperature, they change from 3d 3z2-r2 to in-plane 3d x2-y2 orbitals. The nearest-neighbor (NN) Fe-O bond lengths also exhibit anisotropic behavior in the ab-plane and along the c-axis below Tm. The anisotropic NN Fe-O bond lengths and Debye-Waller factors stabilize the in-plane Fe 3d x2-y2 and out-of-plane 3d 3z2-r2 orbitals during warming and cooling, respectively. Additionally, a VB-PES study further confirms that a relative band gap opens at low temperature in both the ab-plane and along the c-axis, providing the clear evidence of the charge-density-wave nature of SrFeO3-δ (δ~0.19) single crystal.

Filarial dance--sonographic sign of filarial infection.

A 17-year-old boy presented with a 3-month history of swelling and vague pain in the scrotum. US revealed multiple anechoic cyst-like lesions in the body of left epididymis. These cysts showed tubular echogenic internal structures with peculiar twirling motion. This was recognised as the sonographic filarial dance-sign of live adult filarial worms. The boy subsequently underwent needle aspiration of the lesion, which microscopically demonstrated microfilaria of Wuchereria bancrofti. Our report includes an online video clip that will help familiarise readers with the filarial dance.

Bilateral multicystic dysplastic kidneys -- an unusual case diagnosed on antenatal ultrasound.

While it is true that unilateral multicystic dysplastic kidney is commonly associated with abnormalities in the contralateral kidney, it is not widely known that the condition may be bilateral. The recognition of the bilaterality of the multicystic dysplastic kidney by ultrasound has grave prognostic implications as this condition is uniformly fatal. High resolution ultrasound examination of the foetus enables early detection of congenital malformations of the urinary tract. This information is of value in determining foetalprognosis, in deciding the method of delivery, and in alerting the paediatricians to supportive and corrective measures which may need to be taken in postnatal period.

Avascular necrosis: a rare complication of steroid therapy for pemphigus.

A patient of pemphigus vulgaris presented with avascular necrosis of the femur after long-term high-dose corticosteroid therapy. Corticosteroids used on a long-term basis can cause avascular necrosis of bone and this has been seen in various diseases. This is attributable to both the disease process itself and the therapy i.e. corticosteroid usage. In dermatological practice avascular necrosis of bone has been seen more commonly with SLE and also with psoriasis using long-term steroids. Avascular necrosis in a case of pemphigus on steroid therapy is a rare finding. We report such a case of pemphigus vulgaris developing avascular necrosis of bone following corticosteroid therapy.

Colocalized nevus depigmentosus and lentigines with underlying breast hypoplasia: a case of reverse mutation?

Nevus depigmentosus (ND) is classically defined as a congenital nonprogressive hypopigmented macule, stable in size and distribution. There have been many reports of colocalization of ND and lentigines. We describe development of multiple lentigines over ND in a 9-year-old girl along with hypoplasia of the underlying breast. The case is being reported to highlight the phenotypic manifestation of reverse mutation and the coincidental breast hypoplasia that has not been reported before.

Pelvic ultrasonography in pubertal girls.

OBJECTIVE: To derive norms for the size of uterus, uterine shape (fundal-cervical ratio) and ovarian volume in girls in various Tanners stages of puberty. METHODS: Pelvic ultrasound was performed in ninety-two healthy girls in the age group of 8-15 years. These included twenty girls each in Tanner stages 1-4 and twelve in stage 5. All the subjects enrolled in the study had a weight and height within 5th-95th percentile of NCHS standards and their bone ages corresponded to the chronological age. Uterine height, fundal-cervical ratio (FCR) and ovarian volume were measured in all the subjects. The data was stratified according to various pubertal stages as well as for different ages. Statistical analysis was carried out to derive the percentiles for the three parameters in different pubertal stages and to study the correlation between these parameters and age, weight and height of the subjects. RESULTS: A statistically significant increase in uterine height, FCR and ovarian volume was observed with progressive pubertal stages. Maximum increase in uterine height was observed during the transition from stage 2 to stage 3. All girls beyond the age of 10 years or beyond Tanner stage 2 had a FCR>1. The ovarian volume, after showing an initial increase, tended to plateau and there was no significant increase from stage 4-stage 5. A significant correlation was found between the three parameters and the subject's age, weight and height, the maximum correlation was with age (correlation coefficients being 0.748, 0.648, 0.568 for uterine height, FCR and ovarian volume respectively). Centiles for these parameters were obtained for different pubertal stages. CONCLUSION: This work has provided some guidelines for normative data for various pubertal stages as well as for ages between 8-15 years. These may be used as a reference in evaluation of patients with suspected disorders of puberty.

Pseudoexstrophy in a female child.

A six-year-old female patient presenting with a swelling in the infraumbilical part of the abdomen, bulging out on straining, was diagnosed to have pseudoexstrophy bladder. The urinary tract was normal. The patient had bifid clitoris. There was no other associated malformation. Surgical repair of abdominal wall defect was done successfully. A new classification of exstrophy variants is proposed.

Ellis-van Creveld Syndrome: a report of two cases.

Ellis-van Creveld syndrome (EVC) or chondroectodermal dysplasia, a rare autosomal recessive disorder, is a tetrad of chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital heart disease, of which chondrodystrophy of the tubular bones is the most common feature, while central nervous system (CNS) and urinary tract anomalies are some of its rarer associations. This report describes EVC syndrome in two sisters of Indian origin, ages 8 and 6 years, the products of nonrelated, unaffected parents. The patients had chondrodysplasia of tubular bones resulting in disproportionate dwarfism, polydactyly, severely dystrophic nails, partially absent teeth, and short and bound-down upper lips with multiple frenulae. Other features noted in the girls were syndactyly and mild mitral regurgitation. All four of the classic features of EVC syndrome were present in patient 1 and three in patient 2. Additional findings were ichthyosis and plantar keratoderma in the former and absent clavicles in the latter, which have not been reported previously. The importance of prenatal diagnosis of EVC is stressed and a multidisciplinary approach for the management of these patients is highlighted.

Caudal duplication--a case report.

Caudal duplication is a rare entity due to incomplete separation of mono-ovular twins. It is usually associated with various congenital malformations involving mainly the genitourinary and gastrointestinal tracts. We report such a case operated upon successfully in which there was no other associated anomaly.

Cystic lymphangioma with special reference to rare sites.

A 10 year retrospective study of 45 cases of cystic lymphangioma (CL) in children is presented. There were 25 females and 20 males. Age ranged from 6 months to 8 years. Common sites were involved in 38 and rare sites in 7 patients. Rare sites were--gluteal region (1), pelvis (1), retroperitoneum (1), mesentery (2), inguinal region (1) and inguinoscrotal region (1). The clinical presentation included sudden increase in size (25), lump abdomen (3), gluteal abscess (1), abdominal distension (1) and inguinal swelling (2). Diagnosis was established preoperatively in 38 cases, and after surgery and histopathology in 7 cases. Near total or subtotal excision was carried out in all cases. Facial nerve palsy (1) and recurrence (2) were the complications of surgery. The study is presented to highlight the occurrence of the cystic lymphangioma at rare sites to avoid diagnostic errors and unnecessary mutilating surgery.