Primary immunodeficiency disorders in the developing world: data from a hospital-based registry in India.

Abstract:

:Primary immunodeficiency disorders (PID) are under-reported from the developing world. We present data regarding diagnosis and outcome from a hospital-based registry in India. Forty-seven patients fulfilled diagnostic criteria. Majority were males. Subgroups were disorders of immune dysregulation-29%, B&T-cell abnormalities-28%, predominant antibody deficiencies-23%, other well-defined immunodeficiencies-15%, and phagocyte disorders-4%. Molecular diagnosis was attempted in 12 and was positive in seven. Overall 24 children died. Only three out of 28 children needing stem cell transplant (SCT) underwent the same. Registry data highlights that molecular diagnosis and SCT are a rarity for children with PIDs in the developing world and mortality is high.

journal_name

Pediatr Hematol Oncol

authors

Chinnabhandar V,Yadav SP,Kaul D,Verma IC,Sachdeva A

doi

10.3109/08880018.2013.805346

subject

Has Abstract

pub_date

2014-04-01 00:00:00

pages

207-11

issue

3

eissn

0888-0018

issn

1521-0669

journal_volume

31

pub_type

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