Vitamin D Profile in Juvenile Idiopathic Arthritis Patients in a Tertiary Care Hospital in Bangladesh.

There are multi-factorial causes of decrease in bone mass in Juvenile Idiopathic Arthritis (JIA) patients who correlate with the duration of active disease. By measuring the vitamin D level we can assess the deficiency or insufficiency earlier and can predict the risk of osteoporotic bone fracture & can give appropriate supplementation of vitamin D & calcium. This study was done to determine the status of serum 25(OH)D in patients with JIA and to see the relationship among various subtypes and disease duration. In this cross sectional study 30 (Thirty) newly diagnosed cases of JIA attending the pediatric rheumatology clinic of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2014 to December 2015 were included. Thirty age and sex matched control were selected and serum 25(OH)D was measured in cases and controls. Among JIA patients, 60% and among controls 33% had hypo-vitaminosis D. In JIA group the mean level of serum 25(OH)D was lower than control group and the result is statistically significant in cases of poly-articular JIA and systemic JIA (SJIA). There is significant difference of the mean values of vitamin D levels in JIA and control groups for the cases of hypo-vitaminosis D. Level of serum 25(OH)D significantly decreased as disease duration continue increased. More than half of JIA patients had hypo-vitaminosis D. It is more significant in cases of poly-articular JIA and systemic JIA (SJIA). There was negative relationship between serum 25(OH)D level and disease duration.

Intraspinal Intramedullary Primitive Neuroectodermal Tumor in a Young Girl: A Case Report from BSMMU, Bangladesh.

Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Here we describe a girl of 4½ years age admitted with the complaints of, low back pain for 2 months, gradually worsening weakness in both lower limbs for the same duration along with urinary incontinence and constipation for 1 month. On examination she was afebrile, mildly pale, no lymphadenopathy, having normal vital signs and anthropometrically well thriving. Back and spine examination showed angulation of spine involving T11 - L4, without paravertebral swelling. Neurological examination of lower limbs revealed decreased muscle tone, diminished muscle power (3/5), both knee and ankle jerks were also diminished and bilaterally equivocal planter response, sensory function of all modalities were impaired. MRI findings were suggestive of ependymoma. Consultation was done with department of neurosurgery and they did the relevant surgery. Tissue histopoathology and immune histochemistry confirmed the diagnosis of primitive neuroectodermal tumor. Finally the child was referred to Paediatric Haemato-oncolgy department for further management.

Macrophage Activation Syndrome in Paediatric Rheumatic Diseases.

Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic disorders, which commonly occurs in systemic juvenile idiopathic arthritis (sJIA).This study was carried out with the aims of describing the clinical features, laboratory findings and outcomes of MAS associated with paediatric rheumatic diseases in the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU) and compare these results with previous studies on MAS. This retrospective study was conducted in the paediatric rheumatology wing of the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Clinical and laboratory profile of all the diagnosed cases of MAS were analyzed from the medical records from January 2010 to July 2015. Among 10 MAS patients, 6 were female and 4 were male. Seven patients of systemic JIA, two patients of SLE and one patient with Kawasaki Disease developed MAS in their course of primary disease. Mean duration of primary disease prior to development of MAS was 2.9 years and mean age of onset was 9.1 years. High continued fever and new onset hepatosplenomegaly were the hallmark of the clinical presentation. White blood cell count and platelet count came down from the mean of 16.2 to 10.2×109/L and 254 to 90×109/L. Mean erythrocyte sedimentation rate was dropped from 56 to 29 mm/hr. Six patients had abnormal liver enzyme level (ALT) and 5 had evidence of coagulopathy (prolonged prothrombin time and APTT) at the onset of disease. Hyperferritinnemia were found in all the patients. Bone marrow study was done in 5 patients but features of hamophagocytosis were found only in 2 patients. All patients received intravenous steroid and 3 patients who did not respond to steroid received additional cyclosporine. Mortality rate was 30% in this series. Macrophage activation syndrome is a fatal complication of paediatric rheumatic diseases among which s-JIA was predominant. Early diagnosis and aggressive therapy is essential to reduce the morbidity and mortality of this illness.

Poncet's disease (tubercular rheumatism) - a case report.

Poncet's disease is a rare condition in childhood. It occurs due to immunological reaction to tubercular protein resulting in reactive arthritis and manifest with polyarthritis associated with features of active tuberculosis. We are reporting a case of Poncet's disease that was initially treated as a case of Juvenile Idiopathic Arthritis (JIA) without any improvement. The diagnosis was made clinically from history and physical findings with supportive radiological findings and confirmed by granulomatous changes on FNAC. Our patient improved dramatically after treatment with anti-tubercular drugs. Though very rare, Poncet's disease should be strongly considered in the differential diagnosis of fever and polyarthritis of obscure cause, especially in tubercular endemic countries like ours.

Adolescent nutrition in a rural community in Bangladesh.

The objective of this study was to assess the nutritional status of adolescent boys and girls in a rural community in Bangladesh. Between December 1996 and January 1997, a cross-sectional survey was carried out in 803 households, each containing at least one adolescent, sampled consecutively from four purposely-selected villages in Rupganj Thana, Narayanganj district. Initially, the guardians of 1483 healthy and unmarried 10-17 year old adolescents (51% boys and 49% girls) were interviewed about family structure and socio-economic status. Out of these children, 906 (47% boys and 53% girls) from 597 households were weighed, had their height and MUAC measured and were clinically examined. Blood was then collected from 861 adolescents for haemoglobin estimation. The median monthly income per person in these 597 families was approximately Taka 554 (US $12). Twenty seven per cent of the household heads were labourers, 21% were solvent farmers, 14% ran small scale businesses and 6% were unemployed. Sixty seven per cent of adolescents were thin (defined as BMI < 5th centile of WHO recommended reference) with 75% boys and 59% girls being affected. The percentage of thin adolescents fell from 95% at age 10 years to 12% at age 17 years. The prevalence of stunting (height for age < 3rd centile NCHS/WHO) was 48% for both boys and girls and rose from 34% at age 10 to 65% at age 17. On clinical examination angular stomatitis was present in 46%, 27% had glossitis, 38% had pallor, 11% had dental caries, 3.2% had an conspicuously enlarged thyroid and 2.1% had eye changes of vitamin A deficiency. According to INACG (International Nutritional Anaemia Consultative Group, 1985) cut-off values, 94% of the boys and 98% of the girls were anaemic. We conclude that rural Bangladesh adolescents suffer from high rates of malnutrition and almost universal anaemia. Nutritional interventions to target this population are urgently required.