A case of hypofibrinogenemia presenting with submental hamatoma

Hereditary hypofibrinogenemia is a rare disease and the usual presentation is difficult to stop bleeding or hamatoma in the muscle or intracranial space after injury. There may also be adverse pregnancy outcome, and increased tendency to throm­bosis. The usual measure is replacement of fibrinogen. Here a case of this rare disease with unusual presentation is reported, and the special aspects of management are discussed

Compound heterozygosity for hemoglobin D and hemoglobin E

We present two cases of compound heterozygous state for hemoglobin (Hb) D and HbE who reported to the hospital for fever and incidentally found moderate microcytic hypochromic anemia. Later on they were investigated by capillary hemoglobin electrophoresis. Capillary Hb electrophoresis revealed compound heterozygous state for hemoglobin D and hemoglobin E. On family screening, father of one patient turned out to be HbD trait and mother as HbE trait. Due to unavailability of parents and siblings of other patient, family screening was not possible. Compound or double heterozygous state for HbD and HbE is rare. There are only six published reported cases of such double heterozygous state for HbD and HbE in Southeast Asia. Marriage between third degree relatives, which are more common among Muslims as well as inter caste marriages, common in some states of India have resulted into this compound heterozygous condition. Such double heterozygous case is clinically silent as compared to HbE/beta thalassemia or HbD/beta thalassemia

Arthritis as a presentation of acute leukemia

A 14 year old boy presented with 3 months history of low back pain as well as pain and swelling of both knee joints. The pain was inflammatory in nature. He was thoroughly evaluated but no underlying cause was detected. However, the boy was treated with NSAIDs and sulphasalazine. A few weeks later he developed anemia and took the blood transfusion. Then he developed fever and the joint pain re-appeared. On examination, the patient was found moderately anemic, positive bony tenderness and splenomegaly. Musculoskeletal examination revealed right knee joint effusion. The investigation revealed pancytopenia. Bone marrow morphology and immunophenotyping revealed acute lymphoblastic leukemia. We treated the patient according to modified UKALL-2003 protocol and patient condition improved and arthritis disappeared

Arthritis as a presentation of acute leukemia

A 14 year old boy presented with 3 months history of low back pain as well as pain and swelling of both knee joints. The pain was inflammatory in nature. He was thoroughly evaluated but no underlying cause was detected. However, the boy was treated with NSAIDs and sulphasalazine. A few weeks later he developed anemia and took the blood transfusion. Then he developed fever and the joint pain re-appeared. On examination, the patient was found moderately anemic, positive bony tenderness and splenomegaly. Musculoskeletal examination revealed right knee joint effusion. The investigation revealed pancytopenia. Bone marrow morphology and immunophenotyping revealed acute lymphoblastic leukemia. We treated the patient according to modified UKALL-2003 protocol and patient condition improved and arthritis disappeared

Atypical presentation of sickle cell disease

A 20 year old female presented with a history of frequent syncopal attack since her childhood. Each episode persisted 5-10 min without having any aggravating factor or prodrome. She had persistent generalized bodyache aggravating during the winter. She had jaundice and episodic abdominal pain. She received 1 unit of blood transfusion 4 months back and improved sympto-matically. Patient was mildly anemic, moderately icteric and had mild splenomegaly. Over these long periods of her illness she was thoroughly evaluated several times. Her biochemical and neurological evaluation revealed no abnormalities. But she was treated with anticonvulsant for long time empirically without significant improvement. Her CBC showed microcytic hypochromic anemia. She was negative for Wilsons disease. Reticulocyte count was high. Coombs test was negative. Osmotic fragility test was positive. Hemoglobin electrophoresis revealed Hb-S 60%. Sickling test was found positive. Finally it was diagnosed as a case of HbS/?+

Atypical presentation of sickle cell disease

A 20 year old female presented with a history of frequent syncopal attack since her childhood. Each episode persisted 5-10 min without having any aggravating factor or prodrome. She had persistent generalized bodyache aggravating during the winter. She had jaundice and episodic abdominal pain. She received 1 unit of blood transfusion 4 months back and improved sympto-matically. Patient was mildly anemic, moderately icteric and had mild splenomegaly. Over these long periods of her illness she was thoroughly evaluated several times. Her biochemical and neurological evaluation revealed no abnormalities. But she was treated with anticonvulsant for long time empirically without significant improvement. Her CBC showed microcytic hypochromic anemia. She was negative for Wilsons disease. Reticulocyte count was high. Coombs test was negative. Osmotic fragility test was positive. Hemoglobin electrophoresis revealed Hb-S 60%. Sickling test was found positive. Finally it was diagnosed as a case of HbS/?+

Effects of nitrification inhibitor with organic manure and urea on protein and mineral contents in grain of Oryza sativa cv. MR219 cultivated in acid sulphate soil

Several approaches to improve grain nutritive values involve in increasing seed accumulation of protein and micronutrients in rice. Therefore, a study was conducted to select a suitable combination of dicyandiamide (DCD) with organic manure (OM) and urea to improve protein and mineral content in rice grain of MR219. The protein (9.07-12.50%) and Ca, Mg, Zn, Cu, Fe and Mn concentrations increased from 1.92 to 21.05, 3.56 to 18.25, 2.25 to 20.22, 9.14 to 25.66, 3.34 to 27.20 and 5.17 to 23.86%, respectively due to the application of DCD with urea and OM. Moreover, the highest content of protein in grain was obtained for the application of DCD with urea and oil palm compost (OPC). Iron and Mn contents were also highest for DCD with urea and OPC, but Ca, Mg, Zn and Cu contents were highest for DCD with urea and poultry dung (PD) and kept similarity with DCD with urea and OPC

Haemoglobin E/Beta Thalassaemia- A Study in BSMMU

Background: Thalassaemias and haemoglobinopathies have been found sporadically in every ethnic group and geographic region, they occur with particularly high frequency from the shores of the Mediterranean and Africa through the Middle East, the Indian subcontinent, Burma and Southeast Asia. Objective: The study was designed to find out the incidence of HbE/beta thalassaemia in BSMMU. Method: A total of 700 patients suspected to have been suffering from haemolytic anaemia were included in the study. Patients having evidence of haemolysis in peripheral blood film were selected for reticulocyte count and haemoglobin electrophoresis in cellulose acetate membrane at pH 8.6. Result: The study group of 700 patients underwent Hb-electrophoresis of which only 52 (7.4%) cases were diagnosed as HbE/beta thalassaemia. Out of 52 cases, 34 (65.4%) patients were found symptomatic and the remaining 18 (34.6%) patients were asymptomatic. Out of 34 symptomatic cases of HbE/beta thalassaemia, only 14 cases needed blood transfusion. Among the 14 patients, only 8 patients needed more than 10 units of transfusion and 6 patients needed frequent transfusion that is two units of blood in every month. Conclusion: It is clearly evident from the present and other studied so far carried out in this Indian subcontinent and South-East Asia that hereditary haemolytic anaemia due to globin chain defects are quite common in this region, especially in Bangladesh and are responsible for considerable morbidity and mortality. Key words: Thalassaemia; Haemoglobinopathies; HbE/beta thalassemia. DOI: 10.3329/bsmmuj.v2i2.4762 BSMMU J 2009; 2(2): 78-8

Impact of nitrification inhibitor with organic manure and urea on nitrogen use efficiency and yield performance of MR219 rice in acid sulphate soil

A glasshouse experiment was designed to study the effects of nitrification inhibitor (Dicyandiamide; DCD) with organic manure and urea on nitrogen use efficiency (NUE), growth performance and yield of MR219 rice. DCD along with N source increased NUE and apparent N recovery over that of sole application of urea. Application of DCD along with urea and oil palm compost (OPC) resulted in the highest NUE (25.94 kg/ha) and fertilizer N recovery by rice (61.75%) together with the highest increase of panicles/hill (17.43%) and filled grains/panicle (9.42%) over urea alone. The highest grain (21.95%) and straw (13.86%) yield increase over control was achieved from DCD with urea and OPC reflecting as the most potential combination to improve NUE and rice yield in acid sulphate soil

Impact of nitrification inhibitor with organic manure and urea on nitrogen dynamics and N2O emission in acid sulphate soil

The accurate prediction of N transformation is an important requisite for optimizing N use efficiency in cropping systems. An incubation study was conducted to verify the impacts of nitrification inhibitor (NI) with organic manure (OM) and urea on N dynamics and N2O emission in acid sulphate soil. The conducted experiment was two-level factorial with 4 N sources (N1 = 100% of N from urea, N2 = 75% of N from urea + 25% N from rice straw, N3 = 75% of N from urea + 25% of N from cow dung and N4 = 75% of N from urea + 25% of N from poultry dung) and two levels of NI (with and without DCD). The NI (Dicyandiamide - DCD) with OM + urea enhanced mineral N contents and it was the highest (255.07 µg∙g-1) for urea with DCD applications. The highest net N-mineralization (213.07 µg∙g-1) was recorded for the application of urea with DCD and net nitrification (16.26 µg∙g-1) was recorded for the application of urea alone, but the highest cumulative N2O emission (5.46 µg∙g-1) was in urea + poultry dung (PD). In addition, DCD most effectively inhibited net nitrification (28.78%) and N2O emission (32.40%) from cow dung (CD) and urea in the tested soils. The combination of DCD with CD and urea was more effective in reducing N2O emissions (43.69%). These results suggest that the DCD with CD and urea may be the most potential combination to reduce nitrification and N2O emission as well as N loss from acid sulphate soil