Wernicke’s encephalopathy (WE) is caused by thiamine (vitamin B) deficiency and most commonly found in individuals with chronic alcoholism and malnutrition. Clinically, its characteristic features are mental status disorders and oculomotor abnormalities as well as stance and gait ataxia. The diagnosis of WE is frequently missed and delay in treatment can lead to death or Korsakoff’s amnestic syndrome. Thus, in suspected cases of WE, recommendation is not to await confirmation of diagnosis, but to immediately administer high-dose intravenous thiamine and simultaneously treat magnesium deficiency, especially in alcoholics. It is mainly a clinical diagnosis, and can be supported by laboratory tests and imaging studies.

Plexiform neurofibromas (PNF) are benign nervous system tumors falling within the scope of neurofibromatosis type I, also known as von Recklinghausen’s disease. PNF are currently treated surgically and surgery is required in many cases of symptomatic PNF. Surgery may result in added morbidity in some cases. Modelante surgery is proposed for giant and complex tumors, in which full excision is often impossible or mutilating. We report our management experience of a 20-year-old woman who presented with a large symptomatic PNF on her left thigh. It was causing difficulty in schooling and her social integration. The inextirpable nature of the lesion necessitated successive modelante resections which significantly improved her quality of life.

Clear cell nodular hidradenoma is an exceedingly rare cutaneous adnexal neoplasm arising from the eccrine sweat glands and is typically benign in nature. Although it has special predilection for the head, trunk and the extremities, it can involve any part of the body. We are reporting a case of a 47-year man who presented with progressively enlarging painless pedunculated growth on the outer aspect of left thigh. Clinical impression was consistent with a primary cutaneous malignancy. MRI of the thigh showed a multiloculated cystic lesion arising from the dermal layer of skin with no deeper infiltration. Fine needle aspiration cytology was inconclusive as multiple attempts on sampling the tumour revealed nothing except frank blood because of the hypervascular nature of the tumour. Wide-excision of the growth was followed by an uneventful recovery of the patient. Histopathological evaluation of the resected specimen supplemented by immunohistochemistry was consistent with the diagnosis of clear cell nodular hidradenoma with no evidence of atypia or malignancy.

BACKGROUND: Vitamin D deficiency is a major contributor to secondary hyperparathyroidism in patients with chronic kidney disease (CKD). The objective of this study was to determine the vitamin D status in children with CKD.

 METHODS: This cross-sectional study was conducted in the Department of Pediatric Nephrology, National Institute of Child Health (NICH), Karachi from January 2013 to December 2013. Ninety patients aged 3 months to 17 years with CKD were included. CKD was categorized into 5 stages based on glomerular filtration rate (GFR) and stage 4 and 5 were labeled as advanced CKD. Patients on 25-hyroxy vitamin D (25OHD) therapy were excluded. Radioimmunoassay was used to measure 25OHD level and it was defined as sufficient (>30-80 ng/ml), insufficient (16-30), mild to moderate deficiency (5-15 ng/ml) and severe deficiency (<5ng/ml). Data including age, gender, blood urea (U), serum creatinine (Cr), GFR, CKD-stage, etiology,   serum calcium (Ca), serum phosphorus (P) , serum alkaline phosphatase (ALP), serum parathyroid hormone (PTH) and serum 25OHD level  were collected and analyzed  by SPSS using descriptive statistics.

RESULTS: Of the 90 patients, 55 (61.1%) were males. Mean age was 7.4+ 4.6 years. Mean U, Cr and GFR were 113.5+ 82.0 mg/dL, 3.9+3.4 mg/dL and 28.8+28.3 mL/min/1.73 m² respectively. Majority (66.7%) had advanced CKD and 44.4 % were undergoing dialysis. Etiologies of CKD were renal hypoplasia–dysplasia (36.6%), stone-disease (18.9%), posterior-urethral valves (16.7%) and   juvenile- nephronophthisis (12. 2%). Mean ± standard deviation of serum Ca was 8.3 + 1.6 mg/dL, P 5.2+1.7 mg/dL, ALP 418.3+298 U/L and PTH 370.6+320.7 pg/ml. Mean 25OHD level was 18.9+16.8 ng/ml and its levels in males (17.73+10.63) and females   (16.91 + 9.74)  were similar (p=0.79).   Mean 25OHD level was suboptimal in 79 patients (87.8%); 37 (41.1%) had insufficiency, 33 (36.7%) had mild to moderate deficiency whereas 9 (10%) had severe 25OHD deficiency.

 CONCLUSION: Vitamin D status was suboptimal in 87.8% of cases and 25OHD deficiency was severe   in advanced CKD.