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Approach To A Child With Hepatosplenomegaly Dr. Pushpa Raj Sharma Professor of Child Health Institute of Medicine.

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Presentation on theme: "Approach To A Child With Hepatosplenomegaly Dr. Pushpa Raj Sharma Professor of Child Health Institute of Medicine."— Presentation transcript:

1 Approach To A Child With Hepatosplenomegaly Dr. Pushpa Raj Sharma Professor of Child Health Institute of Medicine

2 Case History Eight months, male, from Rupandehi. Fever with cough and difficulty breathing for one week, more severe for one day. Progressive abdominal distension for 4 months. Repeated pneumonia since 3 months of life

3 History contd. Uneventful perinatal period. Exclusive breast feeding for 3 months. Repeated treatment with antibiotics since four months of age for cough and fever. Gaining weight. Single child of parent. Other histories including developmental are normal

4 Examination Wt: 5 Kgs; Length: 64 cms; OFC: 39.5 cm Apyrexial; R/R: 66/min; P/R: 150/min. No pallor; no lymphadenopathy; no rash; no cataract; no specific body odour. Chest indrawing present; bilateral creps. Normal heart sounds. Liver and spleen 5 and 8 cms below costal margin respectively; non tender; smooth; firm. no ascitis; Other system normal

5 Investigations Hb: 12.2.g/dL; TLC/DLC: normal; ESR: 20mm/hr. peripheral film/platelets: normal. Blood sugar: 79mg/dLSerum cholesterol:90mg/dL; Liver function: normal Aldehyde test: negative. Stool/Urine: normal HIV:-ve; TORCH: -ve; USG abdomen: no ascitis, hepatosplenomegaly, no structural abnormality

6 Hepatosplenomegaly: Causes Infective: Viral: Hepatotrophic (A,B,C,D,E) and other viruses (herpes, cytomegalo, Ebstein-Barr, varicella,HIV, rubella, adeno, entero, arbo.) Protozoal: malaria, kalazar, amoebic, toxoplasma Bacterial: sepsis; tuberculosis, brucellosis, syphilis, Helminths: hydatid, visceral larva migrans Fungal: histoplasmosis

7 Hepatosplenomegaly: Causes Haemopoetic: Haemolytic: haemolytic disease of newborn thalassaemia; Anaemia Metabolic: Neiman-Pick, gangliosidosis, Gaucher, fucosidosis, Wolman, glycogen storage, sialiodosis, galactosialiodosis, a-mannosidosis.

8 Hepatosplenomegaly: Causes Malignancies: Leukemia, histiocytic syndromes, myeloproliferative syndromes, lymphomas, Immunological: Chronic granulomatous, heriditory neutrophilia, Ommen syndrome. Developmental: Congenital hepatic fibrosis Congestive: Hepatic vein obstruction, constrictive pericarditis

9 This patient Infective cause that can have lung, liver and spleen involvement: Sepsis/other bacterial infection unlikely: normal blood count, normal immunological reports for HIV, TORCH, syphilis. Viral infections unlikely: normal liver function test, no clinical evidence of congenital infections as: Neonatal jaundice, retinitis, microcephaly, hydrocephaly, intracranial infections, osteochondritis, rash, normal weight and height for age.

10 This patient Protozoal unlikely: Negative blood report (anaemia, jaundice, pancytopenia), big hepatosplenomegaly. Haematological unlikely: Absence of anaemia, jaundice and in the presence of huge spleenomegaly. Malignancies unlikely: Normal blood report, absence of lymphadenopathy

11 This patient Immunological unlikely: Normal liver function, absence of hypereosinophilia and diarrhoea Developmental unlikely: Absence of features of portal hypertension Congestive unlikely: Absence of cardiac insufficiency signs, absence of ascitis.

12 This patient: Metabolic Diseases Causing Hepatosplenomegaly Infantile GM1 gangliodidosis (type 1) Hepatosplenomegaly at birth, oedema, skin erruptions, retardation development, seizure Gauchers: Features of bone marrow involvement, skeletal complications Fucosidosis: Macroglossia, neurodegenerative features Wolman disease: Failure to thrive, steatorrhoea, relentless vomiting

13 Metabolic Diseases Causing Hepatosplenomegaly Glycogen storage Type IV: Failure to thrive, cardiomyopathy, myopathy Mucopolysaccharidoses: Corneal clouding, coarse hair, short stature, joint stiffness. Sialidosis and galactodialidosis: Neonatal sepsis, dysostosis multiplex, seizures, cherry red spots, mental retardation. a-mannodidosis: Psychomotor retardation, dystosis multiplex,

14 This patient Neiman-Pick disease : This could be the likely cause. Neiman-Pick disease: Two types A and B Type A: hepatosplenomegaly, moderate lymphadenopathy, psychomotor retardation. Type B: splenomegaly first manifestation, recurrent pneumonias, normal IQ.

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