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Approach To A Child With Hepatosplenomegaly
Approach To A Child With Hepatosplenomegaly
Approach To A Child With Hepatosplenomegaly
With
Hepatosplenomegaly
Dr. Pushpa Raj Sharma
Professor of Child Health
Institute of Medicine
Case History
Malignancies:
Leukemia, histiocytic syndromes, myeloproliferative
syndromes, lymphomas,
Immunological:
Chronic granulomatous, heriditory neutrophilia, Omm
syndrome.
Developmental:
Congenital hepatic fibrosis
Congestive:
Hepatic vein obstruction, constrictive pericarditis
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.
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
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.
This patient:
Metabolic Diseases Causing Hepatosplenomegaly
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.