Pulmonary Hemosiderosis
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hemosiderosis" OR "pulmonary haemosiderosis".)
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Eur Respir J. 2004 Jul;24(1):162-70.
Idiopathic pulmonary haemosiderosis revisited.
Ioachimescu OC, Sieber S, Kotch A. Dept of Pulmonary Allergy
and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, OH, USA. oioac@yahoo.com
Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar
haemorrhage of unknown aetiology. It occurs most frequently in children, has a
variable natural history with repetitive episodes of diffuse alveolar
haemorrhage, and has been reported to have a high mortality. Many patients
develop iron deficiency anaemia secondary to deposition of haemosiderin iron
in the alveoli. Examination of sputum and bronchoalveolar lavage fluid can
disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung
biopsy shows numerous siderophages in the alveoli, without any evidence of
pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of
immunoglobulins. Contrary to earlier reports, corticosteroids alone or in
combination with other immunosuppressive agents may be effective for either
exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.
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Tijdschr Kindergeneeskd 1988 Jun;56(3):115-9
[Idiopathic pulmonary
hemosiderosis].
[Article in Dutch]
van Dijken PJ, van der Laag J,
Marx JJ.
Universiteitskliniek voor kinderen
en jeugdigen, Het Wilhelmina
Kinderziekenhuis, Utrecht.
Idiopathic pulmonary
haemosiderosis is a rare disease of unknown etiology. It is
most frequently seen in childhood. Three case histories are presented,
backgrounds and therapeutic possibilities are discussed. Centralized
registration is necessary for better understanding of the disease.
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Heel oud artikel:
Maandschr Kindergeneeskd 1973 Apr;41(1):53-65
[Idiopathic pulmonary
hemosiderosis. Description of 3 patients and a review of
the literature].
[Article in Dutch]
Le Maire PA.
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Acta Paediatr Taiwan 2001 Mar-Apr;42(2):101-4
Idiopathic pulmonary hemosiderosis
in a child: report of one case.
Chiu JH, Wong KS, Hsia SH, Lai SH.
Department of Pediatrics, Li-Shin
Hospital, Taoyuan, Taiwan.
Idiopathic pulmonary hemosiderosis
(IPH) is uncommon in children. We report a
3-year-old girl who was presented with acute pale-looking appearance,
hemoptysis, hematemesis and shortness of breath. This patient was confirmed to
have pulmonary hemorrhage by the presence of hemosiderin-laden macrophages in
the bronchoalveolar lavage fluid using a flexible bronchoscope. Other causes
of
PH including glomerular, cardiac and immunological disorder were excluded by
normal laboratory studies. She was primarily treated by oral prednisolone, but
due to recurrent hemoptysis, immunosuppressive agent was added for maintenance
therapy. Pediatricians should consider PH in a patient who has recurrent
dyspnea, hemoptysis and iron deficiency anemia.
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Medicine (Baltimore) 2000 Sep;79(5):318-26
Long-term outcome of idiopathic
pulmonary hemosiderosis in children.
Le Clainche L, Le Bourgeois M,
Fauroux B, Forenza N, Dommergues JP, Desbois JC,
Bellon G, Derelle J, Dutau G, Marguet C, Pin I, Tillie-Leblond I, Scheinmann
P,
De Blic J.
Hopital Necker-Enfants Malades,
Paris, France.
We retrospectively analyzed the
long-term outcome of idiopathic pulmonary
hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and
the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients
developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid
polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome
showed that 3 patients had severe symptoms: 2 patients developed severe
pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1
patient had severe asthma. Twelve patients (80%) had mild or no respiratory
problems and were able to lead a normal life. According to chest X-ray and
pulmonary function test data, 4 patients had normal chest X-ray and no
evidence
of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray
and evidence of restrictive pattern, 1 patient had an interstitial pattern but
normal lung function, and 1 patient had a normal chest X-ray but evidence of
mixed obstructive and restrictive pattern. Our results show that long-term
survival is possible in patients with IPH. Factors of poor prognosis seem to
be
the presence of antineutrophil cytoplasm antibodies (ANCA) or other
autoantibodies. No other clinical or biological predictive factors for
prolonged
survival were found.
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Indian J Pediatr 2000 Sep;67(9):693-4
Idiopathic pulmonary hemosiderosis.
Dua T, Chandra J, Jain M, Passah
SM, Dutta AK.
Department of Pediatrics, Lady
Hardinge Medical College, New Delhi.
Two cases of idiopathic pulmonary
hemosiderosis in children are reported. Both
cases presented with a combination of acute/recurrent respiratory symptoms
along
with iron deficiency anemia. In one case diagnosis was delayed for 18 months
after onset of symptoms. After initial stabilisation with corticosteroid
therapy, both cases were put on chloroquine therapy and showed improvement.
Various other forms of therapy and outcome are discussed. It is suggested that
in cases with recurrent respiratory symptoms, idiopathic pulmonary
hemosiderosis
should also be considered after excluding infectious etiology.
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N Engl J Med 2000 Sep
14;343(11):781
Images in clinical medicine.
Childhood idiopathic pulmonary hemosiderosis.
Keller C, Langston C.
Baylor College of Medicine,
Houston, TX 77030, USA.
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Cardiology 2000;93(3):201-4
Segmental wall motion abnormalities in an individual with idiopathic
pulmonary
hemosiderosis.
Bavry AA, Knoper S, Alpert JS.
Idiopathic pulmonary hemosiderosis (IPH) is a rare condition characterized
by
diffuse pulmonary hemorrhage of unknown etiology. Cardiac involvement in the
form of myocarditis and right ventricular hypertrophy have been reported to
occur in association with IPH, although findings on echocardiography have
not
been described. Herein is presented a case of an adult with IPH and
echocardiographic abnormalities. Copyright 2000 S. Karger AG, Basel
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Turk J Pediatr 1997 Jan-Mar;39(1):111-5
Chloroquine in idiopathic pulmonary hemosiderosis. A case report.
Meral A, Gunay U, Kucukerdogan A, Canitez Y, Ozuysal S.
Department of Pediatric Hematology, Uludag University Faculty of Medicine,
Bursa.
This report describes an 11-year-old boy with idiopathic pulmonary
hemosiderosis. His only presenting symptom was severe anemia due to iron
deficiency. Idiopathic pulmonary hemosiderosis was diagnosed nine years
after
the onset of symptoms. During this period many invasive and
non-contributory
investigations were performed. This report describes the patient's
diagnostic
problems, clinical features and dramatic improvement with chloroquine (250
mg/day) after failing to respond to megadose methylprednisolone (30
mg/kg). One
year later, chloroquine was discontinued. The patient has remained in
remission
since March 1994. Chloroquine should be used for this life-threatening
condition
since it is less toxic than other immunosuppressive drugs.
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Chest 1999 Sep;116(3):721-5
Prognosis in pediatric idiopathic pulmonary hemosiderosis.
Saeed MM, Woo MS, MacLaughlin EF, Margetis MF, Keens TG.
Division of Pediatric Pulmonology, Childrens Hospital Los Angeles, and
the
University of Southern California School of Medicine, 90027, USA.
STUDY OBJECTIVES: Previously, IPH patients have been reported to have an
average
survival of 2.5 years. However, at our institution, many IPH patients
have
survived longer than that. Therefore, we conducted this study to
determine the
clinical course and current mortality of pediatric IPH patients treated
with
immunosuppressants. DESIGN: Retrospective chart review. SETTING:
Children's
hospital. PARTICIPANTS: Seventeen patients in whom IPH was diagnosed
between
1972 and 1998. MEASUREMENTS AND RESULTS: Mean age at diagnosis was 4.5
+/- 3.5
years, and 12 patients were female. At diagnosis, all patients had
anemia and
pulmonary infiltrates; 85% had hypoxemia, 65% had hemoptysis, and 70%
had fever.
The diagnosis was made by open lung biopsy in 13 patients (76%),
hemosiderin-laden macrophages in BAL fluid in 1 patient (6%),
hemosiderin-laden
macrophages in gastric aspirate in 2 patients (12%), or by clinical
presentation
alone in 1 patient (6%). The mean duration of follow-up for all patients
was 3.6
+/- 3.4 years (range, 0.7 to 10.2). Initial treatment consisted of
prednisone
only in 14 patients (82%), and prednisone and hydroxychloroquine in two
patients
(12%). Thirteen patients (76%) required long-term corticosteroids
because of
recurrent hemoptysis. Eight patients (47%) required other
immunosuppressants
(hydroxychloroquine or azathioprine) in addition to prednisone to
control their
hemoptysis. One patient who was not treated with prednisone remained
asymptomatic for 1.8 years. Three patients (17%) died of acute massive
pulmonary
hemorrhage (4.1 +/- 5.0 years postdiagnosis). CONCLUSION: Five-year
survival for
IPH patients in our study was 86% (by Kaplan-Meier method). We conclude
that
these IPH patients who received long-term treatment had a better outcome
than
those previously reported who were not treated with extended courses of
immunosuppressive therapy. We speculate that long-term immunosuppression
therapy
may improve the prognosis in IPH.
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Ann Trop Paediatr 2000 Jun;20(2):153-9
Pulmonary haemosiderosis in Saudi children: report of two cases and
review of
the literature.
Lutfi SA, Abdullah MA, Abumelha A, al Bakry L, Coode P.
Department of Pediatrics, Security Forces Hospital, Riyadh, Saudi Arabia.
Two children with idiopathic pulmonary haemosiderosis are reported.
These are
the first cases of this disease from Saudi Arabia and highlight the
diagnostic
problem caused by their presentation as cases of iron deficiency anaemia.
The
aetiopathogenesis, clinical presentation, diagnosis and management are
discussed. The attention of paediatricians is drawn to this relatively
rare
cause of iron deficiency anaemia.
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Pediatr Pulmonol 1999 Mar;27(3):180-4
Long-term clinical course of patients with idiopathic pulmonary
hemosiderosis
(1979-1994): prolonged survival with low-dose corticosteroid therapy.
Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol D.
Department of Pediatrics, Hacettepe University, Faculty of Medicine,
Ankara,
Turkey.
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown
etiology
characterized by recurrent episodes of pulmonary symptoms such as cough,
hemoptysis, and dyspnea. Our study consisted of 23 patients: 12 males
and 11
females with IPH. The diagnosis was based on history, presence of anemia,
and
characteristic chest X-ray, and was confirmed by showing macrophages
laden with
hemosiderin in gastric washings or bronchoalveolar lavage and/or open
lung
biopsy. All but one patient were diagnosed in our department between
1979-1994.
There was a history of multiple blood transfusions for anemia in 10
patients.
Consanguinity between parents was noted in 11 patients. Severe pallor,
cough,
hemoptysis, and hepatomegaly were the most common findings on physical
examination. All but 2 patients had hypochromic microcytic anemia of
varying
severity. In 12 children, moderate reticulocytosis was noted.
Corticosteroids
were administered with doses ranging from 5 mg every other day to 2
mg/kg/day
depending on the severity of the episodes (duration of disease from 2-14
years).
It is our impression that patients with IPH, benefit from long-term
steroid
treatment which in turn results in a milder course. Long-term low-dose
steroid
treatment appeared to prevent crises and assured a prolonged survival.
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Am J Med Sci 1999 Jan;317(1):67-74
Idiopathic pulmonary hemosiderosis.
Cohen S.
This article discusses an investigation of two children with idiopathic
pulmonary hemosiderosis (IPH) over a long period of time that included
several
cycles of the disease in each patient, each cycle consisting of clinical
remission, a preacute or linking phase, and acute lung bleeding. The
acute phase
was divided into two parts; the preacute phase takes place 5 to 10 days
before
the onset of the acute alveolar hemorrhage. At the beginning of the
preacute
phase, there was neutropenia followed by increased blood eosinophilia.
The lung
function test showed gas trapping. Bronchoalveolar lavage during
remission
showed increased numbers of neutrophils; the histologic study suggested
that the
neutrophils are obligatory participants in the occurrence of lung
bleeding. The
finding of the preacute phase and the role of the neutrophils in the
acute
bleeding permitted development of a new hypothesis related to the
physiopathology of IPH.
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Pneumologie 1998 Jul;52(7):412-6
[Pulmonary hemosiderosis and gastroesophageal reflux in an infant].
[Article in German]
Weimer B, Ahrens P, Miething R, Jansen S.
Abteilung fur allgemeine Padiatrie II der Universitatsklinik Frankfurt
am Main.
Pulmonary hemosiderosis (PH) has been described in association with a
variety of
immunological and non-immunological diseases. It is characterised by
iron-deficiency anaemia, hemoptysis and diffuse pulmonary infiltrates
based on
recurrent intraalveolar hemorrhages. We present the case of a child with
pulmonary hemosiderosis and a pathological gastroesophageal reflux
activity. The
child suffered from recurrent anaemic episodes the age of three months
(hemoglobin level up to 5.4 g/dl). The symptoms decreased after removal
of the
gastroesophageal reflux and accompanying steroid therapy. There has been
one
relapse of pulmonary hemorrhage seven months later (hemoglobin level 6.1
g/dl).
Since then the patient has been in good general condition and the
steroid was
slowly reduced. No more anaemic episodes occurred. We discuss a possible
association of pathological gastroesophageal reflux activity in
pulmonary
hemosiderosis.
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