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Pulmonary hemosiderosis represents challenges during anesthesia and intensive care management. This is a report of a case of idiopathic pulmonary hemosiderosis in a child which was successfully managed during anesthesia and in the intensive care unit with total remission of the disease. The management of this and similar cases are discussed.
The presence of widespread hemorrhage from the microvasculature of the lung into the alveolar spaces defines what is called the "alveolar hemorrhage syndrome", which can occur in association with a wide variety of clinical disorders. The cardinal manifestations of this syndrome include: hemoptysis, unexplained anemia and diffuse alveolar infiltrates on chest roentgenograms. Since the pulmonary features are similar, the diagnosis usually depends on the clinical, laboratory and pathologic evaluations. Early diagnosis and treatment is crucial since the occurrence of pulmonary hemorrhage in this syndrome may represent a catastrophic event with fatal consequences. Idiopathic pulmonary hemosiderosis (IPH) has been identified as a cause of alveolar hemorrhage in a small number of cases, mainly by exclusion criteria. We report a case of a 70-year old man who presented with a 40-year history of intermittent hemoptysis and bilateral upper lobes alveolar infiltrates proved to be secondary to idiopathic pulmonary hemosiderosis. Although the lung apices are frequently spared in IPH, they were the site of the infiltrates in our case. To our knowledge, our patient had the longest survival time ever reported in the literature in adult IPH. A brief review of some of the disorders commonly associated with alveolar hemorrhage is also presented.
OBJECTIVE: To examine the prevalence of coronary artery disease (CAD) in autopsies of patients with iron-overload syndromes. DESIGN: Retrospective autopsy study of CAD in cases of hemochromatosis and multiorgan hemosiderosis. SETTING: Registry of nearly 48,000 autopsies performed at The Johns Hopkins Hospital between 1889 and 1992. SUBJECTS: One hundred twenty-three subjects were studied. In a 2:1 control-case ratio, 82 controls matched by age, race, and sex were compared with 41 cases with iron overload. MAIN OUTCOME MEASURE: Severity of CAD. RESULTS: Pathological description of the coronary arteries were recorded as advanced or severe in 12% of iron-overload cases (n = 41) (mean age, 57.6 +/- 13.2 years) compared with 38% of controls (n = 82) (mean age, 57.0 +/- 13.8 years) (P = .01). The prevalence of three-vessel disease assessed by postmortem coronary arteriography was 11.1% in iron-overload cases (n = 18) (mean age, 61.7 +/- 12.2 years) compared with 33.3% in controls (n = 36) (mean age, 61.1 +/- 12.5 years) (P = .04). The odds ratio of CAD with iron overload was 0.18 (95% confidence interval, 0.04 to 0.73). CONCLUSIONS: Iron overload resulting from hemochromatosis or multiorgan hemosiderosis is not associated with an increased prevalence of CAD.
Transfusion-induced hemosiderosis is a serious and potentially life-threatening complication for some patients with sickle cell anemia. The use of high-dose intravenous deferoxamine (DFO) has become widespread in spite of a paucity of published data on safety and efficacy. We report a randomized double-blind study of the dose-response relationship of intravenous DFO in six subjects with sickle cell anemia and severe transfusion-induced hemosiderosis (serum ferritin 4100 to 14,176 ng/ml). Each subject received three different doses of intravenous DFO for 3 days each while consuming a constant diet. Total iron excretion (urine and fecal) was 91% greater at 180 mg/kg/day DFO than at 60 mg/kg/day DFO, and fecal iron excretion became a relatively larger proportion of total excretion at higher doses. Subsequent treatment for 3 months with 150 mg/kg/day DFO caused a 33% to 60% reduction in serum ferritin and demonstrable improvement in hepatic function in all patients. No toxicity was encountered, but DFO at 180 mg/kg/day was associated with a significant increase in fecal zinc excretion when compared with that observed at lower doses.
The authors report a case of renal hemosiderosis in a 33 year old patient with mitral valve replacement with a Saint Jude Medical prosthesis. Chronic, well-tolerated hemolysis developed after surgery and a peri-prosthetic leak was demonstrated. Alteration of renal function and abnormalities on urinalysis led to renal biopsy which showed massive localised hemosiderosis, mainly in the interstitial tissues. Repeat mitral valve replacement led to a regression of the hemolysis. Significant hemolysis in patients with mechanical cardiac valves prostheses should lead to investigation of prosthetic valve function and, if dysfunction is demonstrated, the patient should be considered for reoperation because of the potential severity of renal complications.
We report on a 44-year-old man with paroxysmal nocturnal hemoglobinuria, who developed renal hemosiderosis, interstitial nephritis, and chronic renal failure. Classically, it is assumed that the renal function is only uncommonly affected by this pathology. In most instances the renal hemosiderosis is an incidental finding at postmortem examination. In our case the presence of iron in the kidney, as confirmed by CT scan, magnetic resonance imaging and renal biopsy, occurred concomitantly with the development of renal insufficiency. A direct nephrotoxic effect of iron by the induction of highly reactive hydroxyl radicals is suggested.
Description of roentgenographic changes in idiopathic pulmonary hemosiderosis and Goodpasture's syndrome. Diagnostic criteria for differentiation from alveolar edema are shown. Separation of disseminated alveolar infections without clinical information is not possible. The close relation to idiopathic pulmonary hemosiderosis and Goodpasture's syndrome is emphasized.
An intradural cavernoma of the spinal cord located at T12 was identified in a 39-year-old man presenting with recurrent episodes of lower back pain on the basis of MRI findings. The patient was hospitalized for severe subarachnoid hemorrhage complicated with hydrocephalia requiring ventriculo-peritoneal draining. Marginal asymptomatic hemosiderosis of the spinal cord above the malformation was observed. The cavernoma which did not involve nervous tissue was removed surgically to avoid the risk of recurrent bleeding and further development of marginal hemosiderosis.
In a three and a half old girl, we have noticed the apparition: first, of on idiopathic pulmonary hemosiderosis; then, five months later, of a rheumatoid arthritis with LE cells and antinuclear antibodies of very difficult detection. The meaning of this articular involvement is discussed: rheumatoid arthritis with LE cells, or rheumatoid arthritis marking the beginning of a systemic lupud erythematosus. The literature concerning the association of an hemosiderosis and a connectivite is reviewed.
The authors present the case of a child diagnosed as having idiopathic pulmonary hemosiderosis at five years of age who had a good clinical outcome at the age of ten years. Initially the patient was treated with prednisone and chloroquine with poor results. When cyclophosphamide was added to prednisone, the patient demonstrated clinical and radiological remission. To date, the patient has been followed for one year without any medication, and has had only one limited episode of hemosiderosis. The authors also suggest that the therapeutic regimen with cyclophosphamide and prednisone may be useful for some selected cases.
Idiopathic pulmonary hemosiderosis is a rare condition manifested by recurrent pulmonary hemorrhage of unknown cause, diffuse radiologic abnormalities, cough, hemoptysis and moderate to severe hypochromic anemia. Diagnosis can be confirmed by iron stains of the sputum or lung aspiration or by biopsy. Prolonged spontaneous remission may occur without the use of corticosteroid therapy. Studies here reported indicated that the anemia is hypochromic and microcytic anemia of blood loss and iron deficiency, in spite of the presence of large amounts of iron in the pulmonary tissue. Correction of the anemia by intensive iron therapy and transfusion is considered an important part of therapy.
Three cases of Goodpasture's syndrome (pulmonary hemosiderosis and glomerulonephritis) are described. Each presented with unexplained hemoptysis and subsequently developed glomerulonephritis which caused uremia. These cases include the youngest and the oldest individuals yet reported with the condition. Steroid therapy was administered to one patient but apparently did not influence the course of the disease. The variations in the clinical course and the pathology of the disease are discussed.
Two boys are presented who fulfilled criteria for a diagnosis of idiopathic pulmonary hemosiderosis. A lung biopsy specimen from the first patient showed alveolar-capillary basement membrane abnormalities, together with abnormalities of capillary endothelial cells and hemosiderin-laden macrophages. A lung biopsy specimen from the second patient showed mainly capillary endothelial abnnormalities and interestitial fibrosis. Both patients had a noticeable improvement in symptoms and relative stabilization of their roentgenographic and pulmonary function abnormalities following azathioprine therapy.
Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.
The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases.
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.
This paper aims to examine the relative contributions made by alcohol and iron overload and hypovitaminosis C to the osteoporosis associated with African hemosiderosis. To characterize this bone disorder, we examined double-tetracycline-labeled iliac crest bone biopsies and serum biochemistry in 53 black male drinkers, 38 with (Fe+) and 15 without (Fe-) iron overload, and in controls. We reasoned that abnormalities found in both patient groups were likely to be caused by alcohol abuse and those found only in the Fe+ group to be caused by iron overload and hypovitaminosis C (iron/C-). The patient groups differed only with respect to greater erosion depth (p < 0.05) and abnormal markers of iron overload in the Fe+ group. Ascorbic acid levels were lower in the Fe+ group than in controls (p < 0.001). Bone volume and trabecular thickness were significantly lower in both patient groups compared with controls and therefore likely caused by alcohol. There were no positive correlations between formation and erosion variables in either patient group, which suggests uncoupling of formation from erosion, possibly as a result of alcohol abuse. Prolonged mineralization lag time associated with thin osteoid seams was found in 32% of patients, affecting both groups. This rules out osteomalacia and suggests osteoblast dysfunction, probably caused by alcohol. The number of iron granules in the marrow correlated with erosion depth (r = 0.373, p < 0.01), trabecular number (r = -0.295, p < 0.05), and trabecular separation (r = 0.347, p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
Superficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron-chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.