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PubMed1978

Idiopathic pulmonary hemosiderosis: two cases in a family.

Two patients, a mother and her son, had idiopathic pulmonary hemosiderosis. In the son, the onset was acute and he died of massive hemoptysis. The mother's disorder developed less acutely and the condition improved after temporary respiratory support and massive corticosteroid therapy. This is the first reported instance of idiopathic pulmonary hemosiderosis occurring in more than one member of the same family. The diagnosis of idiopathic pulmonary hemosiderosis depends on compatible clinical and pathologic data and on exclusion of secondary causes of intrapulmonary hemorrhage.

Adult↗

PubMedDate not supplied

[Clinical and pathogenetic observation in a case of pulmonary hemosiderosis].

Pulmonary hemosiderosis is a rare disease of unknown aetiology, whose clinical, pathogenetic and prognostic aspects are still open to discussion. The authors report a case of pulmonary hemosiderosis in a 2 years and 3 months old girl. Peculiar features of this case include antibody deficiency (which may contribute to the pathogenesis of the disease) and severe and protracted melena. Fatal outcome, despite several therapeutic trials, confirms the poor prognosis of pulmonary hemosiderosis.

Albuterol↗

PubMed1997

Hepatic hemosiderosis in common marmosets, Callithrix jacchus: effect of diet on incidence and severity.

We examined the effect of dietary iron concentration on the incidence of hepatic hemosiderosis in common marmosets (Callithrix jacchus) and assessed the impact of hemosiderosis on animal health. Thirteen young adult common marmosets were fed nutritionally balanced natural-ingredient diets formulated to contain either 100 or 500 ppm of iron. Six were fed the low-iron and seven received the high-iron diet. Baseline blood values and liver iron content were determined for each animal. Animals were weighted monthly, blood work (hematologic analysis, serum iron concentration, total iron-binding capacity, percent of transferrin saturation) was performed semi-annually, and liver biopsies for iron analysis were obtained after marmosets had consumed the test diets for 13 months or at necropsy. Midway in study, the high-iron diet was reformulated to contain 350 ppm of iron because of the death of a male which had consumed that diet for 7 months. Four of seven marmosets fed the high-iron diet died during the first year of the study, compared with one death in the low-iron cohort. The mean increase in liver iron content of the marmosets fed the high-iron diet was 6,371 micrograms/g, dry weight analysis. In contrast the low-iron cohort had a mean decrease of 621.5 micrograms/g. These results indicate that liver iron content can be affected by dietary iron intake. The increased mortality in the marmosets fed the high-iron diet also suggests that hepatic hemosiderosis can be detrimental to marmoset health.

Animals↗

PubMed1998

[Pulmonary hemosiderosis and gastroesophageal reflux in an infant].

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.

Bronchoalveolar Lavage Fluid↗

PubMed1996

Severe hemosiderosis post allogenic bone marrow transplantation.

Abnormal liver function persisting late after allogeneic BMT is usually attributed to chronic GvHD, viral hepatitis or drug toxicity. We describe a patient who had negative hepatitis serology, was on no hepatotoxic medication, had no evidence of GvHD but had abnormal liver function 15 months post MBT. She was diagnosed as having grade IV hemosiderosis of the liver. Her total red cell support had only been 52 units. We therefore postulate that in a proportion of patients receiving allogeneic BMT impaired intestinal iron absorption may be an important cause of hemosiderosis.

Bone Marrow Transplantation↗

PubMed2004

Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease.

We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung function improved markedly by immunosuppression with daily glucocorticosteroid and azathioprine treatment.

Aconitine↗

PubMed1996

Hereditary ceruloplasmin deficiency with hemosiderosis.

Hereditary ceruloplasmin deficiency with hemosiderosis (aceruloplasminemia) is a new disease characterized by systemic hemosiderosis, diabetes mellitus, neurological abnormalities and pigment degeneration of the retina. Loss of the ferroxidase activity of ceruloplasmin results in systemic iron deposition and tissue damage. Neuroimaging studies reveal iron deposition in basal ganglia and in the red and dentate nuclei. Cerebellar ataxia, extrapyramidal signs and dementia develop after middle age. We report a patient with undetectable serum ceruloplasmin levels and the above clinical manifestations. Sequence analysis of the cDNA of ceruloplasmin from this patient revealed an insertion of adenine in exon 3; this produced a premature stop codon.

Amino Acid Sequence↗

PubMed2000

Idiopathic pulmonary hemosiderosis.

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.

Adrenal Cortex Hormones↗

PubMed1975

Alveolar capillary basement membrane lesions in Goodpasture's syndrome and idiopathic pulmonary hemosiderosis.

An ultrastructural study of lung distinguised between lesions of the alveolar capillary basement membrane in a case of Good pasture's syndrome and in three cases of idiopathic pulmonary hemosiderosis. In Goodpasture's syndrome, diffuse vascular injury with wide endothelial gaps, diffusely fragmented basement membranes and an electron dense layer on the basement membrane was found. In idiopathic pulmonary hemosiderosis, focal ruptures of the basement membrane were associated with hydropic changes in pneumocytes and, although fibroblasts were not seen, collagen deposition occurred within the basement membrane. immunofluorescent studies failed to show deposition of immunoglobulins G (IgG), A (IgA), M (IgM) or C3 in the lung in either disease. The ultrastructural lesions appear to separate these clinically similar entities.

Adolescent↗

PubMed1978

Asymptomatic IgA nephropathy associated with pulmonary hemosiderosis.

A glomerular lesion identical to that of IgA nephropathy was demonstrated unexpectedly in a 17 year old boy who presented with clinical manifestations of pulmonary hemosiderosis and with no evidence of renal disease. This subclinical glomerular lesion would have remained undetected in this patient unless kidney tissue was obtained and examined by immunofluorescence or electron microscopy. It is unknown if the glomerular lesion in this case is causally related to pulmonary hemosiderosis.

Adolescent↗

PubMed1995

Hemosiderin deposition in portal endothelial cells: a novel hepatic hemosiderosis frequent in chronic viral hepatitis B and C.

We have recently noted a hitherto undescribed hepatic hemosiderosis confined to endothelial cells of the portal tract in chronic viral hepatitis. In this study, this lesion was surveyed in 156 liver biopsy specimens from patients with chronic hepatitis C and in 21 liver biopsy specimens from patients with chronic hepatitis B. As controls, we examined 110 liver biopsy specimens from patients with primary biliary cirrhosis (PBC), 36 from patients with alcoholic liver injury, nine from patients with autoimmune hepatitis (AIH), and five from patients with primary hemochromatosis. Hemosiderin deposition was found in the endothelial cells of venous vessels in portal tracts regardless of the presence or degree of hemosiderin deposition in hepatic parenchyma. This phenomenon was observed in 65 of 156 cases (42%) of chronic hepatitis C and in eight of 21 (38%) cases of chronic hepatitis B. In controls, this lesion was frequent in AIH (78%), but infrequent in PBC (8.1%) and alcoholic liver injury (11%). The incidence of this lesion showed significant differences between chronic hepatitis C, B, and AIH, and between PBC and alcoholic liver injury. There was a positive correlation between the progression of disease and the incidence of this feature in chronic viral hepatitis; the incidence was 18.3% and 11.1% in milder chronic hepatitis C and B, respectively, and 61.2% and 58.3%, respectively, in more severe cases. However, this correlation was not evident in either PBC or alcoholic liver injury. This hemosiderin deposition was positively correlated with the degree of piecemeal necrosis in chronic hepatitis C, and to a lesser degree, the positive correlation was shown in chronic hepatitis B. These findings suggest that the progression of chronic hepatitis and the piecemeal necrosis in chronic hepatitis C and B, followed by the release of hepatocellular iron to portal and periportal areas, are directly or indirectly responsible for endothelial hemosiderosis. Further studies focusing on this peculiar phenomenon in relation to choice of therapy and evaluation of chronicity of viral hepatitis are encouraged.

Adult↗

PubMed1996

Dark ring sign: finding in patients with fungal liver lesions and transfusional hemosiderosis undergoing treatment with antifungal antibiotics.

OBJECTIVE: To describe the MR appearance of necrotizing fungal granulomas occurring in the liver of leukemic patients with hepatosplenic fungal disease and transfusional hemosiderosis on antifungal antibiotics. SUBJECTS AND METHODS: Four patients with acute myelogenous leukemia (n = 2) or acute lymphocytic leukemia (n = 2) who developed hepatosplenic fungal disease, and were treated with antifungal medication, underwent MRI examination on a 1.5 T MR imager. MR images were prospectively evaluated and correlated with liver biopsy (three patients), and clinical picture (one patient). RESULTS: Multiple liver lesions measuring approximately 1 cm in diameter were identified in all patients. Lesions possessed a distinctive MR appearance: central mild hyperintensity with a peripheral ring of very low signal intensity on precontrast T1- and T2-weighted images. The central region of the lesions enhanced following gadolinium administration with the peripheral ring remaining low in signal intensity. CONCLUSION: Necrotizing fungal granulomas in the liver of patients with transfusional hemosiderosis on treatment with antifungal antibiotics have a distinctive appearance of moderate high signal intensity center on T1- and T2-weighted and postgadolinium MR images with a peripheral rim of low signal intensity. This appearance reflects the presence of iron-laden macrophages in the periphery of granulomas and may be expected in processes that initiate an immune response involving aggregation of macrophages in the liver of patients with transfusional iron overload.

Acute Disease↗

PubMed1975

Hyperreactivity to cow milk in young children with pulmonary hemosiderosis and cor pulmonale secondary to nasopharyngeal obstruction.

Six black infants and young children with high titers of milk precipitins were identified by screening the sera of 160 children with idiopathic chronic lung disease. None of the six had immunoglobulin deficiency, elevation of sweat chlorides, SS hemoglobin, or recurrent aspiration. All six children had typical manifestations of milk-induced pulmonary hemosiderosis: recurrent pulmonary infiltrates (6/6), hemosiderin-laden pulmonary macrophages (5/6), intermittent wheezing (5/6), eosinophilia (4/6), anemia (4/6), iron deficiency (4/4), failure to thrive (4/6), and elevated levels of serum IgE (4/4). Three children also had chronic rhinitis and eventually developed large adenoids, hypercapnia and acidosis during sleep, and right heart failure. Elimination of cow milk from the diet, symptomatic therapy, and adenoidectomy when indicated resulted in improvement of all six patients. Pulmonary hemosiderosis and some cases of upper airway obstruction with pulmonary hypertension appear to be two stages, early and delayed, of the same immunophysiologic process. Early dietary intervention may prevent the cardiovascular complications of this process.

Black or African American↗

PubMed1983

Epidemiologic observations in idiopathic pulmonary hemosiderosis.

We did an epidemiologic survey of 30 children in whom idiopathic pulmonary hemosiderosis was diagnosed. Eighteen patients had died; 12 patients were still living. The majority of the cases were diagnosed in between 1962 and 1971, mainly in the spring and fall. Eighty percent of the patient lived in villages, whereas only 30% of the total population resided in the same type of rural area. Only one (5%) of the deceased children lived in the city, whereas four (33%) of the living children were city dwellers. The socioeconomic conditions were poor, and in 50% of the cases continuous exposure to highly toxic insecticides was elicited by history and investigation of housing conditions. The incidence of newly diagnosed cases decreased with the improvement of living conditions and the prohibition of the use of certain insecticides. We believe that environmental factors, perhaps insecticides, may cause idiopathic pulmonary hemosiderosis in genetically predisposed persons. An epidemiologic history and genetic investigation should be included in the evaluation of patients with any disease when the cause is obscure.

Child↗

PubMed1999

Microvesicular steatosis, hemosiderosis and rapid development of liver cirrhosis in a patient with Pearson's syndrome.

BACKGROUND/AIMS: Pearson's marrow-pancreas syndrome consists of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreas dysfunction. Patients with this disease usually have large deletions of the mitochondrial genome. We report a patient with Pearson's syndrome who had predominantly hepatic manifestations such as microvesicular steatosis, hemosiderosis and rapidly developing cirrhosis. METHODS: Analysis of the mitochondrial and nuclear genomes, determination of enzyme activities and of the hepatic iron content were performed using standard techniques of molecular biology and biochemistry. RESULTS: The patient had typical ringed sideroblasts in a bone marrow smear and a 7436-bp deletion of the mitochondrial genome in all tissues investigated, compatible with Pearson's syndrome. He died within 3 months after birth due to liver failure. Histopathological analysis of the liver revealed complete cirrhosis with signs of chronic cholestasis, microvesicular steatosis and massive hemosiderosis. In addition, the patient was heterozygous for the C282Y and H63D mutations of the hemochromatosis gene. CONCLUSIONS: Pearson's syndrome should be added to the list of neonatal diseases which can cause microvesicular steatosis, hepatic accumulation of iron and liver cirrhosis.

Disease Progression↗

PubMed2002

Prolonged liver dysfunction caused by hemosiderosis in a renal transplant recipient.

BACKGROUND: Liver dysfunction is a frequent complication that arises in the period following kidney transplantations, often resulting in death. We reported a case proving hemosiderosis as a cause of prolonged liver dysfunction after cadaveric kidney transplantation. METHODS: A 47-year-old man, who had been undergoing hemodialysis, was referred to our hospital on 2 November 1999. On the same day, cadaveric kidney transplantation was performed, and serum creatinine level reached a normal level within 2 weeks after surgery. However, serum transaminase gradually increased in the postoperative period. Serum ALT rose up to 116 IU/L on day 20 after the operation and 215 IU/L on day 30. Microscopic examination by needle biopsy revealed hemosiderosis of the liver. Recombinant human erythropoietin was administered and phlebotomy was performed. Liver function improved as a result. CONCLUSION: Early histological diagnosis can be a useful marker in predicting the course of chronic liver disease.

Cadaver↗

PubMedDate not supplied

[Idiopathic lung hemosiderosis in a 3-year-old child following ductus ligation in the neonatal period].

A premature girl (birth weight 1 405 g, gestational age 32 weeks) was mechanically ventilated for 36 days because of respiratory distress, pulmonary hemorrhage and ductus arteriosus. The hemodynamically active ductus was ligated on the 22nd day of life. At the age of three years a mycoplasma pneumonia and a pulmonary hemosiderosis developed. The possible etiologic role of the neonatal lung problems and the mycoplasma infection for the pulmonary hemosiderosis is discussed. Because a similar case has thus for not been reported the coincidence may be by chance.

Bronchopneumonia↗

PubMed1982

Hemosiderosis: an unrecognized complication in renal allograft recipients.

During the past decade, 258 patients received 293 renal transplants at Hennepin County Medical Center Minneapolis, Minnesota. Twenty-three of these patients had a pretransplant liver biopsy. Thirty-six patients had liver biopsies done during the post-transplant period. Liver tissue at autopsy was available from 24 patients who died after receiving a kidney transplant. Systematic analysis of the biopsy and autopsy specimens with appropriate tissue stains showed significant iron deposition (3+ to 4+) within the hepatic parenchymal cells in 1 of the 23 (4%) pretransplant biopsies and 17 of the 60 (28%) post-transplant specimens (P = 0.02). Eight of the 17 (47%) patients with post-transplant hemosiderosis also exhibited histological features of liver cirrhosis. As hemosiderosis is reversible with intermittent phlebotomies, early recognition and adequate management are highly desirable. We suggest that the serum ferritin level, which is a reliable index of body iron stores, should be monitored in all patients at periodic intervals following renal transplantation.

Adolescent↗