Introduction : Haemophilias are the most common inherited coagulation disorders transmitted by X- linked recessive fashion affecting the males and females are the carriers of the disease. Haemophilias are distributed worldwide and have heterogeneous presentation depending upon its severity starting from neonatal period. Knowledge of spectrum of presentation of haemophilia helps in early diagnosis and planning of management. Objectives : To observe the clinical presentation of haemophilia in children Methodology : This observational study was carried out in the Department of Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University for a period of one year from 1st July 2007 to 30th June 2008. Clinical profile of 50 diagnosed cases of haemophilia <15 years of age was analyzed. Result: All the 50 cases of haemophilia were male. Mean age of the patients was 6.62±3.87 years with an age range of 6 months to 14 years. Forty (80%) cases were haemophilia A and 10 (20%) cases were Haemophilia B. Only 40% cases had family history of bleeding. Among the Haemophilia A, 52.5% cases had mild, 47.5% cases had moderate disease and among the Haemophilia B, 40% cases had mild, 50% cases had moderate and 10% cases had severe disease. Heamarthrosis of knee joint was the major presentation followed by oral cavity bleeding, bleeding following tooth extraction and circumcision. Sixty two percent cases had initial bleeding episode before 1 year of age and by 5 years of age 94% of cases had produced symptoms. No patient had history of bleeding during neonatal period. Bruises and hematoma were the most common initial manifestation followed by joint bleeding, cut injury of lips and chin, scalp and facial hematoma. Conclusion: Bruises, hematoma and joint bleeding either spontaneous or after trauma were the main feature at initial presentation of haemophilia in children, so presence of these features in an otherwise normal child should be considered for evaluation of haemophilia. More vigilance is to be needed for detection of haemophilia in newborn. DOI: http://dx.doi.org/10.3329/bjch.v37i2.17266 BANGLADESH J CHILD HEALTH 2013; VOL 37 (2) : 90-96

Methods: In this randomized double blind trial 60 children (4-11 years) with acute lymphoblastic leukemia (ALL) who received high dose methotrexate (HDMTX-2.5gm/ m 2) . Each patients received either ondansetron 4 mg or granisetron 1mg (n=30) orally half an hour before HDMTX. Nausea and vomiting were assessed based on ''modified Morrow Assessment of Nausea and Emesis'' (MANE) scale for application to the children. Results: Complete response of granisetron significantly differed from ondansetron from day 2-4 (delayed emesis) (p=0.028).Complete response to acute CINV

Linear psoriasis is a rare form of disease which is very difficult to differentiate from inflammatory linear verrucous epidermal nevus. Sometimes clinical history, physical examination and histopathology analysis may not be sufficient to confirm the diagnosis. We report a case of25 year-old male presented with a linear plaque covered with silvery scales on left upper extremity extending from tip of the index to mid forearm for last 2 years. A skin biopsy was consistent with psoriasis, and the unilateral distribution in a linear pattern led to a diagnosis of linear psoriasis, which is a rare variant of psoriasis. Although histopathologically it can be difficult to distinguish from inflammatory linear verrucous epidermal nevus (ILVEN), linear psoriasis presents in adulthood and responds to conventional topical antipsoriatic therapies.

Normal functioning thyroid is much more important for healthy skin. The association thyroid disorders with cutaneous manifestations is complex. Various studies have revealed multitude of cutaneous changes (in skin, hair and nails) that occur in thyroid hormonal dysfunction, in both hyper and hypothyroid states. The present study was designed to ascertain the varied cutaneous manifestations of hypothyroidism. This is a case control hospital based clinical study conducted in the Department of Dermatology and Venereology, BSMMU, over a period of one year, from May 2010 to May 2011. Fifty diagnosed cases of hypothyroidism constituted the subject material for the study and additional 50 Euthyroid persons were taken as control who were evaluated for the presence of any cutaneous manifestation. There are 50 patients of hypothyroidism among which 6 male & 44 female. The most common cutaneous feature in patients with hypothyroidism was coarse, rough, dry skin followed by pallor of skin, with carotenemia in 52.75% of the patients, nail changes in 38.9% of the patients, diffuse hair loss in 33.3% of the patients, palmoplantar keratoderma in 33.3% of the patients, and loss of lateral third of the eyebrows in 22.2% of the patients. Myxedematous facies were seen in 12% of the hypothyroid patients in our study. The relation between hypothyroidism and skin have profound clinical importance in dermatological practice. CBMJ 2017 January: Vol. 06 No. 01 P: 24-27

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