Search

INTRODUCTION: Nephropathic cystinosis is a rarely occurring inherited metabolic disorder, leading to Fanconi syndrome, progressive renal failure and a range of extra-renal manifestations including endocrinopathies. There is no definitive cure for Cystinosis. Never the less, early introduction of cysteamine treatment and other supportive measures can adequately retard the occurrence of complications and improve prognosis. CASE DESCRIPTION: An 18-year-old boy who presented with complaints of abdominal pain, vomiting, polyuria and episodes of periodic paralysis for the last 01 year to the Endocrine Unit, Medical Teaching Institution Hayatabad Medical Complex, and Peshawar, Pakistan. He was lean and wasted with a BMI of 12.9kg/m2 (weight=32.59kg, height=159cm) having frontal bossing and splaying of bones at wrist joint with normal to low blood pressure. Investigations revealed hypokalemia, alkaline urine and metabolic acidosis on arterial blood gas analysis. Consequently, his symptoms were attributed to underlying renal tubular acidosis. Later, slit lamp examination revealed Cysteine crystals in the cornea confirming the diagnosis of Cystinosis as the underlying cause. He was started on supportive therapy including potassium chloride and citrate supplements, intravenous fluids, calcium and vitamin D supplements and Indomethacin. Senior nephrologist and Nutritionist were also taken on board regarding his management. Follow up at 02 months was suggestive of marked symptomatic and biochemical improvement. CONCLUSION: Data regarding the definitive cure for Cystinosis is scarce. Never the less, early introduction of cysteamine therapy and other supportive measures can adequately retard the occurrence of complications and improvise prognosis.

Non-governmental organizations are claimed to have impacts on the sustainable development in rural areas of the developing countries and the Bangladesh Rural Advancement Committee (BRAC) is identified as an example. This study has considered BRAC as the case and has been aimed to critically outline the roles of this selected non-governmental organization (BRAC) in sustainable development process. With this particular aim, this study has also explored the trends of current poverty situations in Bangladesh. In addition, impacts of the BRAC sustainable development initiatives have also been analyzed, to some extent. This study has selected two villages of the Chandpur district of Bangladesh, where BRAC has been operating intense programmes. This study has used both the qualitative and quantitative methods of research. Results suggest that programmes undertaken by NGOs are capable of having positive contributions in the sustainable development process to a certain level. The fact is that the programme implementation, where efficiency of the NGO employees is an indicator of success factor. The research is empirical and is expectedly fill the gap of literature.