We studied 53 newborn babies with congenital syphilis. The common clinical features seen were low birth weight, hepatosplenomegaly, anaemia, jaundice, and symmetrical superficial desquamation of the skin affecting palms and soles. The presence of these clinical signs is highly suggestive of early congenital syphilis. Hydrops fetalis without rhesus or ABO isoimmunisation should always arouse the...

She was the youngest in a family of seven. Her siblings were well. There was no family history of bleeding disorders. On examination, she had multiple ecchymotic lesions of varying size and shape on her left forearm, gluteal regions, left leg and dorsum of both wrists. The rest of the physical examination was normal: in particular, she had no lymphadenopathy, hepatosplenomegaly or retinal haemo...

Introduction The Hiper-IgD Syndrome (HIDS) is an autoinflammatory disease characterized by recurrent febrile episodes each 4– 8 weeks accompanied by an intense inflammatory reaction, lymphadenopathy, abdominal pain, diarrhea, arthralgias, hepatosplenomegaly and cutaneous signs [1]. Mutations in the gene that codifies the enzyme mevalonate kinase (MVK), located in the chromosome 12q24, have been...

Examination General examination showed emaciated, oriented, febrile, pallor, icteric, tenderness and swelling present in right thigh. Impetigo scars seen over the both lower limbs. Clubbing and bilateral pitting pedal edema was present. Oral examination shows fissures in tongue. Systemic examination reveals cardio vascular, central nervous system was normal. Respiratory system shows occasional ...

Key words: antineutrophil cytoplasmic antibody; normal in March 1996. She received no medication. chronic lymphocytic leukaemia; crescentic glomerulo-Upon admission her general condition was good: no nephritis; T-cell defect fever, normal blood pressure, no lymphadenopathy or hepatosplenomegaly. Laboratory findings were: serum creatinine 314 mmol/l, BUN 15 mmol/l, total proteins 65 g/l,

Babesia is a malaria-like protozoan parasite spread by Ixodes ticks primarily from the white-footed deer mouse to humans. Typically it causes subclinical disease, but occasionally causes acute febrile disease with hepatosplenomegaly. We report a case of spontaneous splenic rupture of a 56-yr-old man with acute Babesia microti infection.

Niemann-Pick group of diseases are rare lysosomal storage disorders. The clinical phenotype is variable. We report a child who first time presented with tremors of tongue and tremors of one side of the body. On examination child had hemiparesis and hepatosplenomegaly. Bone marrow examination shows storage cells suggestive of Niemann-Pick cells and enzyme assay confirmed the diagnosis.

After an otolaryngologic examination, the patients were investigated to determine the extent of the disease. These included full blood count, a search for hepatosplenomegaly, bone marrow examination, plain radiography and computerised tomography (CT) of the paranasal sinuses. Examination of the nose including the nasopharynx and biopsy of the nasal tumours under general anaesthesia were perform...

A neonate presented with poor feeding, jaundice and hepatosplenomegaly. He was in liver failure with disseminated coagulopathy. Post-mortem liver biopsy revealed discrete granulomas with central necrosis and acid-fast bacilli on Ziehl-Nielsen's stain. The mother had active tuberculosis at delivery. The principal characteristics of congenital tuberculosis are reemphasized in this observation.

A case of systemic lupus erythematosus is described in which for 10 years the only significant findings were erythema multiforme and vasculitis. Gross hepatosplenomegaly with persistent pancytopenia developed, and bone marrow examination revealed the presence of sideroblasts. The patient's condition deteriorated, and subsequently she developed a severe bleeding tendency, terminating in erythrol...