Twenty-One Cases of Misdiagnosed Idiopathic Pulmonary Hemosiderosis
- VernacularTitle:特发性肺含铁血黄素沉着症误诊21例
- Author:
zhi-gang, LIU
;
min, HE
;
xiao-xia, YANG
;
ling-ling, GENG
- Publication Type:Journal Article
- Keywords:
idiopathic pulmonary hemosiderosis;
clinical manifestation;
chest radiograph;
diagnosis;
treatment
- From:Journal of Applied Clinical Pediatrics
1994;0(04):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical characteristics,diagnosis,treatment and misdiagnosis matter of children with idiopathic pulmonary hemosiderosis(IPH).Methods The data of clinical characteristics,laboratory examination,treatment and follow-up of 21 children admitted from Jun.1993 to May 2007 were retrospectively analyzed,included 9 males,12 females,aged 1-14 years old,course of di-sease were 1-6 months.Twenty-one patients were diagnosed as IPH by chest X-ray radiography,CT scan,bone marrow biopsy,hemosiderin-laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid.Then,therapy and prognosis of IPH were analyzed.Results All patients had varied degrees of anemia,11(52.38%) children had cough,9 (42.86%) children had fever,6(28.50%) cases had shortage of orexia,4( 19.05%) children had hemoptysis.Chest X-ray radiography and CT scan demonstrated diffuse patchy,nodular,reticulate pattern.Eighteen children received bone marrow biopsy and presented hyperplastic erythropoiesis,4(22.2%) cases were accompanied iron deficiency anemia.Nineteen (90.80%) cases shown the presence of hemosiderin-laden macrophages in either sputum or gastric juice or bronchoalveolar lavage fluid.Twenty-one misdiagnosed patients consisted of bronchopneumonia combined anemia(8 cases),lung tuberculosis combined anemia(5 cases),nulli-iron anemia(4 cases),hemolytic anemia(3 cases),myelodysplastic syndrome(1 case)and received corticosteroid therapy.Four cases of all patients were associatated with large-dose human-?-globulin and 3 cases were associatated with vincristine therapy.The therapeutic effect was significant.Eighteen patients were followed-up,3 patients were of which cured and had stopped treatment for over 2 years,11 patients presented clinically persistent remission,4 patients were recurred and aggravated.Conclusions Early diagnosis and long-term therapy of corticosteroid are very important for controlling acute onset,lessening the frequency of IPH recurrence and improving prognosis of the disease.
