OBJECTIVES:
To describe the clinical details and follow up of
children with
idiopathic pulmonary hemosiderosis.
DESIGN:
Retrospective case series.
SETTING:
Pediatric
chest clinic of a
tertiary care hospital.
SUBJECTS:
Children diagnosed as suffering from
idiopathic pulmonary hemosiderosis (IPH).
METHODS:
Charts of
patients diagnosed as IPH were reviewed for clinical features and
treatment regimen.
Diagnosis was based on presence of
iron deficiency anemia,
chest radiography and demonstration of
hemosiderin laden
macrophages in
bronchoalveolar lavage (BAL), gastric aspirate, or
sputum.
Treatment consisted of oral
prednisolone,
hydroxychloroquine (HCQ) and inhaled
corticosteroids (ICS).
RESULTS:
The common clinical features in 26
children with IPH (mean age 75 months) included
cough,
breathlessness,
fever,
hemoptysis and
wheezing in 26 (100%), 22 (85%), 19 (73%),15 (58%) and 14 (54%)
children, respectively. Clubbing,
hepatomegaly and
splenomegaly was seen in 16 (62%), 15 (58%) and 10 (38%)
children, respectively.
Hemosiderin laden
macrophages were documented in BAL and gastric aspirate in 92% and 30%
patients, respectively. Symptoms did not recur in 17
patients who received
prednisolone and HCQ initially. 5
patients had
recurrence of symptoms and required short
courses of oral
prednisolone, 4
patients required frequent
courses of
prednisolone and were started on
azathioprine. Older age, longer duration of illness,
history of
hemoptysis and
jaundice were associated with poor response.
CONCLUSION:
Treatment with
prednisolone and
hydroxychloroquine followed by inhaled
corticosteroids may improve
survival in
children with IPH.