Objectives: The objectives of this study were to the demographic, clinicolaboratory profile and outcome scrub typhus in affected children. Material Methods: Retrospective descriptive children aged 1 month–16 years who diagnosed with between January 2014 November 2021, admitted paediatric ward intensive care unit our hospital. Results: A total 15 patients included study, which 60% male 40% femal...

A 35-year-old man from Bangladesh, who had been in Malaysia for approximately a year, was extensively investigated for more than two months in a state hospital for pyrexia with hepatosplenomegaly. However, no obvious cause of his illness was found. He was treated with multiple antibiotics with no resolution of pyrexia and hepatosplenomegaly. He was later referred to the Haematology Unit, Univer...

Methods and result 14-year-old patient diagnosed with TGA at birth, at the age of 14 she presented a sudden hepatosplenomegaly. The systematic examination confirmed WG. According to our knowledge this is the first case of association of these two infrequent diseases. Furthermore hepatosplenomegaly as initial symptom of WG is especially rare feature. The second patient suffering from asthma for ...

We report a 23-year-old Bangladeshi man who presented with fever and hepatosplenomegaly. The initial laboratory findings were bicytopenia with elevated serum globulins. The diagnosis of visceral leishmaniasis (Kala Azar) was suspected. The parasite Leishmania donovani was found on bone marrow aspiration. He was treated with liposomal amphotericin B and had a good response to treatment. The case...

A 21-year-old man with a history of sudden rectal hemorrhage was referred to our hospital. Examination disclosed thrombocytopenia and hepatosplenomegaly. A liver biopsy specimen demonstrated Gaucher cells in Glisson's capsule. Additional investigations revealed a low level of leukocyte β-glucosidase activity and common mutations of the glucocerebrosidase gene, L444P/D409H. We diagnosed the pati...

Congenital leukemia is a very rare malignancy of childhood with a poor prognosis. The incidence is nearly 1 in 5 million live births. The majority of cases are acute myeloblastic leukemia with trisomy. Clinical manifestations are usually leukocytosis, petechia, ecchymosis, cutaneous nodules, hepatosplenomegaly and central nervous system symptoms. 23-days old girl was presented with complaints o...

Sandhoff disease is a neurodegenerative disease caused due to deficiency of hexosaminidase (HEX) A and B. A 1-year-old male child presented with regression of milestones, exaggerated startle response, decreased vision, and seizures from 6 months of age. The child had coarse facies without hepatosplenomegaly. Serum levels of β hexosaminidase total (A + B) were low. Genetic testing for Sandhoff d...

A 27-year-old womanwas admitted for further examination of thrombocytopenia. Symptomswere absent, but physical examination demonstrated hepatosplenomegaly without neurological abnormalities. Bone marrow examination revealed many Gaucher cells, and glucocerebrosidase activity from cultured skin fibroblasts was markedly reduced. A 1448C (L444P) mutation was detected on one allele of the glucocere...

Brucellosis is a systemic zoonotic infectious disease that may cause fever, fatigue, sweating, arthritis, hepatosplenomegaly, cytopenia, and lymphadenopathy. It continues to be an important health problem worldwide. Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, hepatosplenomegaly, cytopenias, high serum levels of ferritin and triglycerides, low serum fibrinogen levels, and...

Gaucher Disease (GD) is the most prevalent inherited lysosomal storage disorder characterized by a glucocerebroside enzyme deficiency. This hydrolase that plays role in breakdown of glycosphingolipid complex. In general population, this disease rare, with an incidence about 0.39 to 5.80 per 100.000 birth and prevalence 0.70 1.75 birth. We reported two-year-old female patient who presented gradu...