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

Multiple Epiphyseal Dysplasia (MED): A Rare Type of Skeletal Dysplasia

Multiple epiphyseal dysplasia (MED) is a congenital disorder of skeletal development that primarily affects the ends of long bones, causing progressive joint and bone inflammation and short stature. Mutations in several genes are responsible for pathogenesis of this disease. We are reporting a case of MED who presented with the complaints of multiple swelling of the joints which was associated with pain during movement for last seven years. The patient had flexion deformity of all the affected joints along with restriction of movement. These were associated with kyphosis, pectus carnitum, knock-knee and short stature. Radiological findings were suggestive of MED. Counseling was done with the parents regarding the etiology, progression and outcome of the disease.DOI: http://dx.doi.org/10.3329/bsmmuj.v5i1.11025 BSMMU J 2012; 5(1):57-60

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-

Pediatric gastrointestinal tuberculosis with hematochezia and polyarthritis: An unusual presentation

Tuberculosis may present with variable symptoms and signs, sometimes presentations are unusual. An eight year old boy was admitted with the complaints of low grade fever, weight loss, abdominal pain, hematochezia and polyarthritis. Colonic biopsy was suggestive of tuberculosis and Gene-Xpart of sputum showed of multidrug resistant tuberculosis. We are reporting a case presented with hematochezia and poloyarthritis, an unusual presentation who was subsequently diagnosed as a case of multi drug resistant intestinal tuberculosis

Pediatric gastrointestinal tuberculosis with hematochezia and polyarthritis: An unusual presentation

Tuberculosis may present with variable symptoms and signs, sometimes presentations are unusual. An eight year old boy was admitted with the complaints of low grade fever, weight loss, abdominal pain, hematochezia and polyarthritis. Colonic biopsy was suggestive of tuberculosis and Gene-Xpart of sputum showed of multidrug resistant tuberculosis. We are reporting a case presented with hematochezia and poloyarthritis, an unusual presentation who was subsequently diagnosed as a case of multi drug resistant intestinal tuberculosis

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

The Efficacy of Subcutaneous Versus Oral Methotrexate in Patients with Juvenile Idiopathic Arthritis

Objective: To investigate the efficacy of subcutaneous versus oral methotrexate in patients with Juvenile Idiopathic Arthritis (JIA) according to ACR 30 improvement criteria. Design: Prospective control trial (Parallel design) study. Setting: This study was carried out in the department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, during the period from September 2006 to April 2008. Patients: A total of 40 patients participated in the study. They were divided in 2 equal groups (Group A and Group B) alternatively matching with age, sex, and subtypes of the disease. Methotrexate was given orally in group A patients and subcutaneously in group B patients. Results: Amomg the core set variables active arthritis had the highest percentage of improvement in both the groups and laboratory criteria (ESR) showed lowest improvement. According to ACR-30 criteria, improvement rate was higher in the subcutaneous group than oral group (85 % versus 65%). Side effects of MTX were also less in subcutaneous group than oral group. Conclusion: From this study it may be concluded that the efficacy of subcutaneous MTX is more than oral MTX in JIA patients. Key words: Subcutaneous, oral, methotrexate, juvenile idiopathic arthritis

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