Niemann-Pick Disease is an autosomal recessive disorder of infancy characterized by failure to thrive, hepatosplenomegaly and neurodegenerative changes that leads to sphingomyelin and cholesterol storage within the lysosome.Although the disease is common among Jewish individual but is rare in South Asian particularly in Bangladesh. So, we present the case as a rare incidence in our country.

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It has been suggested that a birthweight limit of 2.5 kg should not be regarded as valid for all populations as the cut-off point of low-weight births because of demographic, genetic and environmental differences. Countries often choose alternative cut-off values for low birthweight for clinical purposes. Bangladesh also needs to choose a convenient cut-off value for low birthweight. A total of 770 live singleton full-term normal newborns were included in this study by stratified sampling; birthweight was measured using the Detecto-type baby weight machine. Newborns were followed up to the end of their first week of life. For data collection a pretested structured questionnaire and an Apgar Score estimating checklist were used. Chi-square test was applied to assess the association of different birthweight strata and neonatal health outcomes. Multiple logistic regression analyses were carried out to identify the independent effects of different levels of birthweight on early neonatal health. The neonates having birthweight < or = 2 kg had a significantly higher risk of early neonatal mortality and morbidity than the higher level birthweight group. Birth asphyxia was the commonest cause of early neonatal mortality and morbidity. Borderline birthweight (>2 to <2.5 kg) neonates experienced the same mortality and morbidity rates as the normal birthweight neonates during their early neonatal life. Birthweight < or = 2 kg may be one of the criteria for admission to a neonatal intensive care unit whereas more than 2 kg may not require admission unless otherwise necessary.

It was concluded that the ORT corner was vary effective simple and safe in the management of diarrhoea in children and reduced the number of diarrhoeal admissions in the hospital.

Various clinical presentations from chronic abdominal pain to acute midgut volvulus with ischaemic bowel injury may result from failure of normal intestinal rotation and fixation. A patient having malrotation and internal herniation of gut clinically presented to us with intermittent abdominal pain, bilious vomiting and abdominal mass mimicking mesenteric cyst. This is a very rare and exceptional form of presentation of malrotation of gut. Rarity of the clinical presentation leads us to report this case.

A total of 7667 patients were admitted In Paediatrics and Paediatric surgery Department of Rajshahi Medical College Hospital from January 1999 to December 2000. Among these patients congenital malformation was found in 222 cases. The result showed that the occurrence of major congenital malformation was 2.26% and imperforated anus topped the list followed by congenital heart diseases. Minor malformation was 0.62% of which hypospadiasis and syndactyly were highest and lowest in number respectively. TAJ 2002; 15(1): 22-24

Background: Thalassemia is a common hematological disorder in our country having wide spectrum of clinical presentation. The frequency and severity of the several types of thalassemia depend on the racial background of the population. Hb-E Beta thalassemia is prevalent in our country. Objective: To see the clinical features of different types of Thalassemia in northern area of Bangladesh. Methods: Hundred cases were selected from Thalassemia patient admitted in department of pediatrics, on May 2012 to October 2012. A prescribed questionnaire was used to record the information. The methods were explained to the patients and consent was taken. Necessary physical examination was performed and investigations were done. The data was analyzed by standard procedure. Results: Out of hundred (100) cases, most (61%) were Hb-E beta Thalassemia, less common (1%) was Hb-E disease, and 1 % case was Hb-E trait. Majority (64%) manifested clinically under one year of age. 54% were male and 46% were female. The major presenting symptom was progressive pallor in 70% cases. Others presenting complaints were low grade fever (40%). Hemoglobin concentration at the time of diagnosis was below 5 gm/dl in 53.33% patients. In hemoglobin electrophoresis it was Hb-E ranged from 54.64 ± 13.02%, Hb-F 34.84±13.73%, Hb-A 23.32± 18.15% and Hb-A2 3.5± 70%. Radiological findings revealed gross bony changes occur in long standing cases. Enlarged cardiac shadow was found in those cases having severe anemia with heart failure. Conclusion: In countries with a high incidence of thalassemia, it is vitally important to offer prospective genetic counseling and to warn carriers about the risks of intramarriage. Nutritional and folic acid supplementation with regular blood transfusion along with iron chelation therapy is essential to improve the prognosis. TAJ 2018; 31(2): 6-11

Teaching is a highly complex process. The medical teaching is more complex and difficult. There are certain basic skills of teaching e.g. the way the teacher presents lecture, use various teaching media, use of basic questionnaire and motivation etc. Besides these basic skills there are certain microskills of teaching. These are explaining, introductory procedure, closures and advanced questioning. Out of these microskills, explaining is the most important and powerful skill. Explaining has some components; e.g. clarity, fluency of language, use of illustration, variation of voice manners, structuring statements and finally obtaining feedback. Every medical teacher should use these microskills in the lecture room for effective teaching.