This study aimed to present clinical and laboratory features of 30 children with Wilson's disease. Material and Methods:This cross-sectional study was carried out at the Department of Z.H. Sikder Women's Medical College Hospital, Dhaka on 30 children more than three years old fulfilling the diagnostic criteria of Wilson's disease.Result: Mean age of WD patients was 8.9±2.78 years, with a male female ratio of 1.3:1 Regarding presentation of WD 79.3% patients presented only with hepatic manifestation, 3% only with neurological features and 15% manifested with others. Among hepatic presentation 16(53.3%) patients presented with chronic liver disease, 3(10%) with Acute hepatitis and 3(10%) with Acute liver failure.Two patients(6%) were diagnosed on family screening & one patient(3%) was HBsAg positive. Other (15%) patients presented with WD with Acute glomerulonephritis (1), WD with betta Thalassemia (1), WD with Acute pancreatitis (1). There was significant low level of serum ceruloplasmin (35 of 40 patients) / 93.33% of cases (p<.001). After penicillamine challenge, 24-hour urinary copper excretion was found significantly higher in patients with WD (median 3626.5±1698 μg/24h, range 1262-195000) (p<.001). K-F ring was found in 15 (50%) patients, that was statistically significant . Conclusion:Wilson's disease in children is frequently missed due to its diversity of clinical presentation. So, for early detection and treatment any children with liver injury of unknown etiology should be screened for Wilson's Disease.
Nevus of Ota is a benign congenital melanosis bulbi. It is a rare entity, commonly seen in female. It occurs among Asians and Africans. It mainly involves the eye, skin and ear. It is predominantly unilateral but could be bilateral. Typical clinical manifestation was present in our case, however, biopsy is the investigation of choice. There is no specific treatment but ophthalmological and dermatological intervention can be opted. Outcome is remarkably good. Here we reported a case of a child with Nevus of Ota who was primarily admitted at paediatric department with enteric fever. We managed her conservatively and followed up in specific department. Bangladesh J Child Health 2020; VOL 44 (1) :60-63
Hypokalemic periodic paralysis (HKPP) is a rare genetic disorder with autosomal dominant inheritance and characterized by recurrent attacks of skeletal muscle weakness with associated hypokalemia which is precipitated by stress, cold, carbohydrate load, infection, glucose infusion, hypothermia, metabolic alkalosis, anesthesia and steroids. Hypokalemic Periodic Paralysis is one form of Periodic Paralysis, a rare group of disorders that can cause of sudden onset weakness. A case of a 29 year old male is presented here. The patient presented with sudden onset paralysis of his extremities. Laboratory evaluation revealed a markedly low potassium level. The patient’s paralysis resolved upon repletion of his low potassium and he was discharged with no neurologic deficits. Although rare, Periodic Paralysis must differentiated from other causes of weakness and paralysis so that the proper treatment can be initiated quickly.
Background: Surgical management of lower cervical spine injury is a very important issue among these patients. Objective: The purpose of the present study was todetermine the early neurological outcome of delayed anterior decompression and stabilization of lower cervical spine injury. Methodology: This cross sectional study was carried out in the Department of Neurosurgery at Dhaka Medical College and Hospital, Dhaka, Bangladesh within the period of January 2010 to July 2011 for a period of one year and 6 months. Neurological outcome following anterior decompression and stabilization of lower cervical spine injury was observe. Patients presented with lower cervical spine injury were included in this prospective study. Quantification of neurologic deficit in lower cervical spine injury patients were carried out by following The American Spinal Injury Association (ASIA) impairment scale. Early neurological outcome was also assessed after operation by using ASIA impairment scale in the follow up period at one, three and six months interval. Result: A total of 31 patients were included in this study. Based on the ASIA impairment scale, preoperatively, 6.4 5% of the injuries were grade A, 16.13 % of the injuries were Grade B, 16.13% of the injuries were Grade C and 35.48 % of the injuries were Grade D. early post-operative complications include dysphagia in 3 5.48% cases, donor site infection in 9.67% cases, CSF leak in 3.22%; catheter related urinary tract infection 9.67% and bed sore occurred in 6.45% cases. Conclusion: In conclusion significant neurological recovery can be expected following delayed anterior decompression and stabilization of lower cervical spine in lower cervical spine injury patient. Journal of National Institute of Neurosciences Bangladesh, 2018;4(2): 82-86
Salmonella hepatitis is one of the atypical presentations of typhoid fever and can be defined as reversible involvement of liver during the course of typhoid fever. There have been more than 150 cases of salmonella hepatitis reported both in developed and developing countries. The documented incidence varies widely from 1 to 26% of patients with Typhoid fever. It presents with jaundice and tender hepatosplenomegaly. Investigation shows slightly raised transaminase levels with or without 5 adenosine neucleosidase and or decreased prothombin time index. It complicates into hepatic encephalopathy and bleeding diathesis. A positive culture for Salmonella from blood or stool is essential to differentiate Salmonella hepatitis from other causes of acute hepatitis. Hepatic pathology is characterized by the presence of typhoid nodules with marked hyperplasia of reticuloendothelial system.The ALT/LDH ratio < 9 is suggestive of Salmonella hepatitis which is > 9 in viral hepatitis. The prognosis is usually good as Salmonella hepatitis responses with specific antibiotic therapy and jaundice resolves with clinical improvement. The clinical course can be severe with high mortality (20%) sometime. In our country where enteric fever is endemic, the recognition of Salmonella hepatitis is of clinical importance .
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