Moyamoya disease in a child

A 6 year old boy presented with sign symptoms of cerebral stroke. After clinical and radiological evaluation, a diagnosis of bilateral moyamoya disease was made. An indirect bypass surgery encephaloduroateriosynangiosis was done on the right side at first then on the left side.  Patient was partially improved. At one year follow-up, there was the evidence of occlusion of collateral circulation on the right side and the patient became symptomatic again. It is noted that, although the early indirect bypass surgery is indicated in the pediatric patients for preventing the stroke but the outcome is difficult to judge

Silver-Russell syndrome

Silver-Russell syndrome is clinically and genetically a heterogeneous disorder. In most of the cases, etiology is unknown, only in 10% cases defect in chromosome 7 is identified. It bas distinctive facial features and asymmetric limbs. Most predominant symptom is growth failure. A case of Silver-Russell syndrome reported here who presented with growth failure, hemihypertrophy ofleft side oftbe body, dysmorphic facial profile and difficulty in speech. Counseling was done with the parents regarding the etiology, progression and outcome of the disease

Types and Clinical Profile of Rickets in a Tertiary Care Hospital

Background: There are different types of rickets. Rickets presents with various clinical signs and symptoms. Familial X linked hypophosphatamic rickets (XLHR) is reported to be the commonest one. Objectives: To find out the types of rickets and the presenting features of rickets. Setting: Department Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU). Design: Descriptive type of study. Methods: A total number of 20 children with rickets were included in this study during the period of January 2004 to July 2008. A questionnaire was used for compiling the information. Data were compiled manually and expressed as frequency distribution table. Result: Male: Female ratio was 13:7. Mean age of the patients was 49.36 months. Nutritional and familial hypophosphatamic rickets constituted the majority, each of the type being 40%. Common clinical presentations included limb weakness (90%), growth failure (90%) and repeated respiratory tract infection (80%). Widening of the wrist were present in 90% of patients. Rachitic rosary and protruded abdomen were present in 80% of cases. Radiological findings of rickets were present in 100% of patients. Conclusion: Nutritional and XLHR rickets were the common type. Common clinical features were weakness of limbs, growth failure and widening of wrists. Key words: Rickets; nutritional; XLHR; RTA.DOI: 10.3329/bsmmuj.v2i1.3703 BSMMU J 2009; 2(1): 3-

Moyamoya disease in a child

A 6 year old boy presented with sign symptoms of cerebral stroke. After clinical and radiological evaluation, a diagnosis of bilateral moyamoya disease was made. An indirect bypass surgery encephaloduroateriosynangiosis was done on the right side at first then on the left side.  Patient was partially improved. At one year follow-up, there was the evidence of occlusion of collateral circulation on the right side and the patient became symptomatic again. It is noted that, although the early indirect bypass surgery is indicated in the pediatric patients for preventing the stroke but the outcome is difficult to judge

12 ORIGINAL ARTICLES Types and Clinical Profile of Rickets in a Tertiary Care Hospital

Background: There are different types of rickets. Rickets presents with various clinical signs and symptoms. Familial X linked hypophosphatamic rickets (XLHR) is reported to be the commonest one. Objectives: To find out the types of rickets and the presenting features of rickets. Setting: Department Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU). Design: Descriptive type of study. Methods: A total number of 20 children with rickets were included in this study during the period of January 2004 to July 2008. A questionnaire was used for compiling the information. Data were compiled manually and expressed as frequency distribution table. Result: Male: Female ratio was 13:7. Mean age of the patients was 49.36 months. Nutritional and familial hypophosphatamic rickets constituted the majority, each of the type being 40%. Common clinical presentations included limb weakness (90%), growth failure (90%) and repeated respiratory tract infection (80%). Widening of the wrist were present in 90 % of patients. Rachitic rosary and protruded abdomen were present in 80 % of cases. Radiological findings of rickets were present in 100 % of patients. Conclusion: Nutritional and XLHR rickets were the common type. Common clinical features were weakness of limbs, growth failure and widening of wrists