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PubMed1995

Treatment of idiopathic pulmonary hemosiderosis with inhaled flunisolide.

Idiopathic pulmonary hemosiderosis (IPH), a rare clinical entity, is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. It is usually treated with systemic corticosteroids during acute bleeding episodes and with prolonged courses of oral corticosteroids and/or other immunosuppressant drugs for maintenance therapy. Because prolonged treatment with these agents is frequently associated with significant side effects, favorable response to inhaled corticosteroids would be ideal in this condition. We report the case of a patient with IPH successfully treated with inhaled flunisolide.

Administration, Inhalation↗
PubMed2002

Recurrence of idiopathic pulmonary hemosiderosis in a young adult patient after bilateral single-lung transplantation.

BACKGROUND: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by hemoptysis, diffuse pulmonary infiltration, and anemia. Diagnosis requires a detailed clinical history and transbronchial lung biopsy (TLB). METHODS AND RESULTS: A 19-year-old man developed progressive dyspnea, hemoptysis, and anemia. The chest x-rays showed bilateral opacities. IPH was diagnosed on the basis of clinical findings and TLB. The patient was treated with corticosteroidal therapy. His respiratory function worsened, and he underwent lung transplantation in 1997. The pathological examination on native lungs confirmed the previous histologic diagnosis. In 2000, the patient again developed hemoptysis, fever, and hypoxemia. A recurrence of the disease was established by TLB. CONCLUSIONS: This is the first report of recurring IPH. The possibility of recurrent IPH raises the question whether these patients should be disqualified from lung transplantation. This question is unanswerable because incidence of recurrence, time course, and impact on the graft function are presently unknown and unpredictable.

Adult↗
PubMed2004

Legionella pneumonia: infection during immunosuppressive therapy for idiopathic pulmonary hemosiderosis.

We report a case of Legionella pneumonia in a 10-year-old girl with idiopathic pulmonary hemosiderosis who was chronically immunosuppressed and had exposure to a hot tub. Prompt diagnosis with bronchoalveolar lavage and subsequent antimicrobial therapy resulted in full recovery. Legionellosis should be included in the differential diagnosis of the immunosuppressed child with respiratory illness. High risk patients should avoid exposure to hot tubs.

Child↗
PubMed1994

Antineutrophil cytoplasmic antibodies (ANCA) in idiopathic pulmonary hemosiderosis.

Four children were diagnosed with idiopathic pulmonary hemosiderosis (IPH), over a period of 4 years. Retrospectively, antineurtrophil cytoplasmic antibodies (ANCA) were studied by indirect immunofluorescence (IIF) and ELISA in 18 sera from these patients, stored at -20 degrees C. ANCA-positive sera, from 1/20 to 1/1, 200 dilution, were found in 3/4 of the patients, by IIF. The patient with the highest titre of ANCA died 3 months later during an acute crisis, the other two patients need a minimal dose of steroids. In one case only, a patient who is still without treatment, had no ANCA. The antibodies anti-myeloperoxidase and anti-proteinase-3 were negative or at border line levels. Rheumatoid factor, antinuclear (Hep-2), anti-endomysial, anti-reticulin and antibasement membrane antibodies were negative in all sera. The surviving patients were followed-up for more than 10 years with no systemic or renal disease appearances. The presence of serum ANCA may help to classify children with pulmonary haemorrhage and may have a prognostic value.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1982

Heart block and idiopathic pulmonary hemosiderosis.

A young woman with idiopathic pulmonary hemosiderosis (IPH) and advanced heart block below the atrioventricular node requiring pacemaker therapy is discussed. Thirteen years after the onset of IPH she is free of pulmonary symptoms despite having never received steroid or antimetabolite therapy.

Adolescent↗
PubMed2005

Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia.

Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.

Adult↗
PubMed1998

Study of toxin production by isolates of Stachybotrys chartarum and Memnoniella echinata isolated during a study of pulmonary hemosiderosis in infants.

A cluster of cases of pulmonary hemosiderosis among infants was reported in Cleveland, Ohio, during 1993 and 1994. These unusual cases appeared only in infants ranging in age from 1 to 8 months and were characterized by pulmonary hemorrhage, which caused the babies to cough up blood. A case-control study identified major home water damage (from plumbing leaks, roof leaks, or flooding) as a risk factor for development of pulmonary hemorrhage in these infants. Because of an interest in the possibility that trichothecene mycotoxins might be involved in this illness, a number of isolates of Stachybotrys chartarum were grown in the laboratory on rice, and extracts were prepared and analyzed both for cytotoxicity and for specific toxins. Two isolates of Memnoniella echinata, a fungus closely related to S. chartarum, were also included in these studies. S. chartarum isolates collected from the homes were shown to produce a number of highly toxic compounds, and the profiles of toxic compounds from M. echinata were similar; the most notable difference was the fact that the principal metabolites produced by M. echinata were griseofulvins.

Animals↗
PubMed2000

Quantification of siderophore and hemolysin from Stachybotrys chartarum strains, including a strain isolated from the lung of a child with pulmonary hemorrhage and hemosiderosis.

A strain of Stachybotrys chartarum was recently isolated from the lung of a pulmonary hemorrhage and hemosiderosis (PH) patient in Texas (designated the Houston strain). This is the first time that S. chartarum has been isolated from the lung of a PH patient. In this study, the Houston strain and 10 strains of S. chartarum isolated from case (n = 5) or control (n = 5) homes in Cleveland were analyzed for hemolytic activity, siderophore production, and relatedness as measured by random amplified polymorphic DNA analysis.

Animals↗
PubMed1984

Magnetic resonance imaging of transfusional hemosiderosis complicating thalassemia major.

Tissue deposits of hemosiderin, a paramagnetic iron-protein complex, resulted in marked abnormalities of magnetic resonance (MR) spin-echo signal intensity within the viscera of three children with transfusional hemosiderosis and thalassemia major. In all patients the liver and bone marrow demonstrated abnormally low spin-echo intensities and the kidneys and muscles had abnormally high intensities. These observations correlate with in vitro MR observations of ferric (Fe+3) solutions, in which concentrations of ferric salts greater than 20 mmol yielded a low MR intensity signal and ferric concentrations less than 15 mmol yielded higher intensities than did water alone. MR imaging is sensitive to the tissue deposition of hemosiderin, and MR intensity appears to provide a rough measure of the amount of iron deposited.

Adolescent↗
PubMed2004

VEGF causes pulmonary hemorrhage, hemosiderosis, and air space enlargement in neonatal mice.

To determine whether increased levels of VEGF disrupt postnatal lung formation or function, conditional transgenic mice in which VEGF 164 expression was enhanced in respiratory epithelial cells were produced. VEGF expression was induced in the lungs of VEGF transgenic pups with doxycycline from postnatal day 1 through 2 and 6 wk of age. VEGF levels were higher in bronchoalveolar lavage fluid (BALF) and lung homogenates of VEGF transgenic mice compared with endogenous VEGF levels in controls. Neonatal mortality was increased by 50% in VEGF transgenic mice. Total protein content in BALF was elevated in VEGF transgenic mice. Surfactant protein B protein expression was unaltered in VEGF transgenic mice. Although postnatal alveolar and vascular development were not disrupted by VEGF expression, VEGF transgenic mice developed pulmonary hemorrhage, alveolar remodeling, and macrophage accumulation as early as 2 wk of age. Electron microscopy demonstrated abnormal alveolar capillary endothelium in the VEGF transgenic mice. In many locations, the endothelium was discontinuous with segments of attenuated endothelial cells. Large numbers of hemosiderin-laden macrophages and varying degrees of emphysema were observed in adult VEGF transgenic mice. Overexpression of VEGF in the neonatal lung increased infant mortality and caused pulmonary hemorrhage, hemosiderosis, alveolar remodeling, and inflammation.

Aging↗
PubMed2000

Segmental wall motion abnormalities in an individual with idiopathic pulmonary hemosiderosis.

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.

Adult↗
PubMedDate not supplied

Pediatric idiopathic pulmonary hemosiderosis diagnosed by sputum analysis: plain radiography and computed tomography findings.

OBJECTIVE: Idiopathic pulmonary hemosiderosis (IPH) is an uncommon disorder, which is characterized by recurrent hemoptysis, iron deficiency anemia and diffuse parenchymal infiltration on chest radiographs in pediatric patients. We wish to present clinical and radiological (plain radiography and CT) findings of this rare pathology. CLINICAL PRESENTATION AND INTERVENTION: A 14-year-old girl was admitted to the pediatric emergency department with complaints of cough, dyspnea, fatigue and bloody sputum for 6 months. She had been hospitalized 3 times during this period and received antibiotics and blood transfusion. Chest X-rays revealed prominent perihilar and bibasilar consolidation. CT showed a ground glass pattern and consolidated areas with increased density. Sputum analysis yielded hemosiderin-laden macrophages. With presumptive diagnosis of IPH, prednisolone was administered. Her symptoms improved on the 5th day of treatment and 1 month later, plain chest radiography demonstrated marked improvement. CONCLUSION: Although IPH is a rare condition, the diagnosis of IPH should be considered, among others, in a patient with hemoptysis and bilateral infiltration in the chest X-ray. This may prevent antibiotic misuse and risk of death due to severe hemorrhage.

Adolescent↗
PubMed1985

Detoxification in hemosiderosis.

Transfusional iron overload in patients with chronic renal failure is a growing concern and its therapeutic approach develops both on the lines of prevention and of cure. It is now proved that the control mechanisms which relate iron absorption to body iron stores are intact in patients on dialysis. Oral iron therapy is therefore recommended in almost all patients. In some dialysis patients with transfusion-dependent anemia, a measure currently acknowledged to reduce transfusion iron overload consists of the use of young instead of mature erythrocytes for transfusions. The tissue depletion of iron by desferrioxamine is an alternative therapy which can prevent hemosiderosis or cure organ dysfunction due to iron overload. In some patients, the simultaneous occurrence of iron and aluminum overload suggests there may be competition between chelation of iron and aluminum. In fact, clinical studies show that both iron and aluminum can be removed by administration of desferrioxamine.

Administration, Oral↗
PubMed1992

Peritoneal hemosiderosis in pediatric patients with nephrogenic ascites.

Nephrogenic ascites associated with maintenance hemodialysis is a complex problem with poorly understood pathophysiology. We report 4 pediatric patients investigated between 1986 and 1990. All the patients treated with maintenance hemodialysis required multiple blood transfusions. Each patient was carefully evaluated for factors potentially relevant to ascites, and serum ferritin levels were found to be extremely high. Peritoneoscopy which was utilized in all patients led to a specific diagnosis of hemosiderosis in the peritoneum and liver biopsies. In 1 patient, lymph node biopsy also showed iron deposition. We believe that iron deposition played a role in changing the permeability of the peritoneum and is presumed to be a pathogenetic factor in nephrogenic ascites.

Ascites↗
PubMed1993

Management of hemosiderosis complicated by coexistent anemia with recombinant human erythropoietin and phlebotomy.

Patients with hemosiderosis who also suffer from coexistent anemia may be unable to tolerate frequent phlebotomies needed for depletion of body iron stores. Chelation therapy, an alternative approach, may be unsuitable for some patients due to allergic reactions, poor response or intolerance of long-duration subcutaneous administration. The use of recombinant human erythropoietin in such patients could increase the hematocrit and improve exercise tolerance allowing for more frequent phlebotomies. We report the successful use of this combined approach in two such patients.

Aged↗
PubMed2003

Treatment of pediatric idiopathic pulmonary hemosiderosis with low-dose cyclophosphamide.

OBJECTIVE: To report the safety and efficacy of long-term, low-dose cyclophosphamide therapy in a child with idiopathic pulmonary hemosiderosis (IPH). CASE SUMMARY: A 7-year-old boy diagnosed with IPH 4 years previously was initially prescribed prednisolone. Because he only had a transient response to prednisolone, oral cyclophosphamide 2 mg/kg/d was later added. A dramatic improvement was noted during the subsequent follow-up. One year after cyclophosphamide therapy, the patient suddenly developed thrombocytopenia (platelet count 75 x 10(3)/mm(3)), with the platelet count decreasing to 10 x 10(3)/mm(3) over the following 10 months. Cyclophosphamide was tapered to an alternating daily dosage of 1 mg/kg. The tapering resulted in a subsequent increase in the platelet count, which was maintained between 20 and 50 x 10(3)/mm(3) without occurrence of petechiae or spontaneous bleeding. Under this reduced dosing regimen, the disease has remained in remission for >1 year. DISCUSSION: Due to the low prevalence of IPH, only limited data document the safety and efficacy of immunosuppressive therapy in treating this disease. Although our patient showed a good response to low-dose cyclophosphamide, he developed thrombocytopenia with its use. The mechanism is unclear, but it may be similar to that of high-dose cyclophosphamide-induced myelosuppression. Due to the development of thrombocytopenia, the use of cyclophosphamide was maintained under a reduced dosing regimen. The benefit of long-term immunosuppressive therapy is controversial, and more clinical evidence is required to support its continued usage. CONCLUSIONS: Long-term, low-dose cyclophosphamide is effective in treating childhood IPH, but caution should be exercised due to the possible development of thrombocytopenia. Periodic monitoring of the platelet count in long-term treatment is recommended.

Child↗