Generalized siderosis with fibrosis of liver and pancreas in Cooley's (Mediterranean) anemia; with observations on the pathogenesis of the siderosis and fibrosis.
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Anemia↗
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Haemochromatosis African nutritional siderosis and experimental siderosis in animals.
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Anemia, Sideroblastic↗
[Illustration of 2 cases of endogenous pulmonary siderosis and of one case of exogenous siderosis previously interpreted as silicosis].
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Hemosiderosis↗
[Studies on the primary lesion of the experimental retinal siderosis. 3. The primary lesion of the experimental siderosis from the viewpoint of histochemical studies on the lactic dehydrogenase].
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Animals↗
[Studies on the primary lesion of experimental retinal siderosis. IV. The primary lesion of experimental retinal siderosis from the viewpoint of histochemical studies on glucose-6-phosphate dehydrogenase].
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Animals↗
Hepatic siderosis in alcoholics.
In a population of 157 (120 males, 37 females) predominantly British alcoholics with liver disease, the incidence of some degree of hepatic siderosis, as estimated by stainable parenchymal iron, was 57.3%. The incidence of significant siderosis (grades III and IV) was 7%, and was similar for both sexes. In the female alcoholics there was a significant correlation between age and the degree of siderosis (P less than 0.05)--four of the five females with significant siderosis being premenopausal. In the male alcoholics there was a significant inverse relationship between the grams of ethanol consumed per day and the degree of siderosis (P less than 0.05) and a significant correlation between the percentage saturation of iron-binding protein and the degree of siderosis (P less than 0.05). The mean daily iron intake from alcoholic beverages was 1.5 mg; there was no relationship between the amount of iron ingested in the alcohol and the degree of siderosis. In this population of alcoholics the incidence of significant siderosis in both sexes was low.
Adult↗
Superficial siderosis associated with multiple cavernous malformations: report of three cases.
OBJECTIVE AND IMPORTANCE: Superficial siderosis is a rare but potentially devastating syndrome caused by recurrent subarachnoid hemorrhage. We present three cases of superficial siderosis associated with multiple cavernous malformations, and we review previous reports of superficial siderosis attributable to vascular malformations. CLINICAL PRESENTATION: Patients most commonly present with progressive sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. Magnetic resonance imaging diagnosis may precede symptom development, however. In two of our cases, superficial siderosis was identified on magnetic resonance imaging scans in the absence of clinical symptoms. INTERVENTION: Magnetic resonance imaging studies revealed hemosiderin deposition, characteristic of superficial siderosis, and multiple cavernous malformations in all three cases. Surgical intervention was not pursued. CONCLUSION: We conclude that patients with multiple cavernous malformations and those with perisubarachnoid lesions are at risk for the development of superficial siderosis. Clinicians should recognize the radiographic appearance of superficial siderosis and its clinical presentation in patients with vascular malformations.
Adult↗
Liver histology after current intensified therapy for childhood acute lymphoblastic leukemia: microvesicular fatty change and siderosis are the main findings.
BACKGROUND: During modern intensified therapy for childhood acute lymphoblastic leukemia (ALL) serum liver enzymes reach fairly high levels. Since no recent data on liver histopathology after therapy are available, we conducted a study of the subject. PROCEDURE: Liver biopsy specimens were evaluated and serum liver function tests and lipid profiles measured from 27 consecutive children, aged 3.5-17.6 years, treated according to the regimens for standard (SR) and intermediate risk (IR) ALL. RESULTS: None of the patients had entirely normal liver histology. Fatty infiltration was detected in 25 out of 27 (93%) and siderosis in 19 out of 27 patients (70%). Fourteen (52%) had both. Three (11%) also had mild portal and/or periportal fibrosis in addition to fatty change and siderosis. Fatty change was mainly microvesicular. Siderosis was in most cases grade II/IV to III/IV (in 16/19 or 84%). No hepatitis or cirrhosis was found. Serum total and LDL-cholesterol levels were higher in the patients with fibrosis than in the patients with fatty change (P = 0.036, P = 0.042) or with siderosis +/- fatty change (P = 0.036, P = 0.042). In serial ALT measurements a value of 300 U/L or more was oftener reached in the fibrosis than in the fatty change or siderosis groups (in 33 vs. in 12 or in 4% of the measurements, respectively, P = 0.014, in Kruskall-Wallis test). CONCLUSIONS: Microvesicular fatty change and siderosis are the main liver findings after current therapy for childhood ALL. Fibrosis occurs rarely. High values in serial serum ALT measurements repeatedly or a disturbed serum lipid profile may facilitate decisions about the need for a liver biopsy.
Adolescent↗
[Histologic quantification of hemochromatotic and non-hemochromatotic liver siderosis. A stidu of 254 liver biopsies].
Non hemochromatotic liver siderosis often present an heterogeneous iron distribution, but histological scoring of iron overload are validated only in case of genetic hemochromatosis, where iron is homogeneously distributed. The aim of this work was to study the improvement of histological scoring by introducing a coefficient of heterogeneity in cases of heterogeneous liver siderosis. Thus, on 254 liver biopsies with siderosis were determined: i) the histologic total iron score (TIS) as previously described; ii) the coefficient of heterogeneity, leading to corrected TIS (corTIS); iii) the liver iron content (LIC). Liver siderosis was homogeneously distributed in 160 biopsies and heterogeneously distributed in 94. Correlation between histological scoring and LIC, in the group with heterogeneous liver siderosis, was improved by the use of the coefficient of heterogeneity. This coefficient leads to accurately quantify all liver siderosis.
Adult↗
Superficial siderosis of the central nervous system.
There have been 87 cases of superficial siderosis of the CNS reported in the world literature and 63 cases had developed the clinical syndrome with sufficient details to be reviewed. It is a distinct clinical syndrome characterized by sensorineural deafness (95%), cerebellar ataxia (88%) and pyramidal signs (76%). Other features include dementia (24%), bladder disturbance (24%), anosmia (at least 17%), aniscoria (at least 10%) and sensory signs (13%). Less frequent features are extra-ocular motor palsies, neck or backache, bilateral sciatica and lower motor neuron signs (5-10% each). Males are more often affected than females (3:1). The age of onset ranged from 14 to 77 years, age at death from 29 to 78 years and duration until death from 1 to 38 years excluding premature death due to the underlying cause or as a result of surgery. Up to 27% become bed bound at 1-37 years from the first symptom due to either cerebellar ataxia, a myelopathic syndrome or both. Symptomatic subarachnoid haemorrhage occurred in 37% and the CSF was haemorrhagic and/or xanthochromic in 75%. It is now accepted that superficial siderosis is due to chronic subarachnoid haemorrhage and a source of bleeding has been reported in 54% of cases; it was either due to dural pathology (47%) including a CSF cavity lesion or cervical root lesion, a vascular tumour (35%) or a vascular abnormality (18%). Arguments are presented that the remaining cases were also due to chronic haemorrhage and that there is no evidence for a non-haemorrhagic form of superficial siderosis. There have been 14 incidental cases diagnosed by MRI or at post-mortem with no symptoms attributable to superficial siderosis during life, supporting the notion of a pre-symptomatic phase to the illness. In 22 patients who had developed the syndrome, the duration of this pre-symptomatic phase could be calculated and ranged from 4 months to 30 years with an average of 15 years. At present the most promising treatment for superficial siderosis is surgical ablation of the bleeding sources.
Adolescent↗
Renal siderosis in patients with heart-valve prostheses: clinical implications.
We have studied the clinical and morphological implications of renal siderosis, reviewing the autopsy protocols of 33 patients with valve prostheses in the heart. Seventeen patients had variable amounts of iron in the proximal tubules of the kidney. Renal siderosis was more frequent in women, in patients with longest time of evolution from the surgical procedure, and in patients with two valve prostheses. Histologically three degrees of renal siderosis may be defined: mild and moderate degrees of iron overload do not alter the kidney architecture, but kidneys, with severe siderosis show tubular atrophy and interstitial fibrosis. Episodes of acute renal failure (ARF) were more frequent in patients with more pronounced iron deposits, especially in the premortem stages. We conclude that renal siderosis may damage the proximal tubular epithelium of patients with valve prostheses in the heart; patients with renal overload of iron are more susceptible to episodes of ARF.
Acute Kidney Injury↗
Incidence of lung cancer among shipyard welders investigated for siderosis.
The incidence of lung cancer among 428 shipyard welders exposed for more than ten years to welding fumes was investigated. The welders were examined for siderosis by the Directorate of Labor Inspection in 1975. The present study was a follow-up based on historical information from the Norwegian registry of dust-exposed workers. Twenty-three welders with siderosis, and 156 welders working at the same shipyards as the siderosis cases were studied as sub-cohorts. There was no loss on follow-up. The observation period was 1976 through 1992. There were 32 cases of cancer from all causes vs 41.3 expected. A nonsignificant excess of lung cancer was observed; 10 cases vs 6.5 expected. The incidence of lung cancer was highest for the welders with more than 30 years since first exposure (7 cases vs 4.1 expected). The sub-cohort of welders with siderosis had no case of lung cancer vs 0.5 expected. These welders were assumed to have experienced high exposure levels for welding fumes. The morbidity of cancer from all causes was low for this small group of blue-collar workers, but the incidence of lung cancer was slightly increased. The increase was not attributable to welders with siderosis. Smoking and asbestos exposure are potential confounders.
Adolescent↗
Superficial siderosis of the central nervous system: a late complication of cerebellar tumors.
OBJECTIVE: To describe the clinical and radiologic features of superficial siderosis of the CNS after treatment of a cerebellar tumor. METHODS: Clinical assessment and MRI in four patients with superficial siderosis were performed. RESULTS: Four patients with superficial siderosis had been treated for a primary cerebellar tumor (astrocytoma in three patients, medulloblastoma in one patient) during childhood. All patients were treated with surgery and three received radiotherapy. Slowly progressive bilateral sensorineural hearing loss, gait ataxia, and limb ataxia appeared 8 to 22 years after diagnosis of the cerebellar tumor. Other clinical features were mild cognitive impairment, dysarthria, nystagmus, optic neuropathy, anosmia, and upper motor neuron signs. The CSF contained erythrocytes and increased protein. MRI with fast spin-echo T2-weighted and gradient-echo T2* sequences showed a hypointense rim of iron coating the surface of the cerebellum and brainstem. Twenty-one other patients who had survived more than 5 years after treatment of a primary cerebellar tumor did not have symptoms or signs suggestive of superficial siderosis. CONCLUSIONS: Superficial siderosis is an uncommon late complication of the treatment of a childhood cerebellar tumor, but it is probably underrecognized. The diagnosis should be suspected in patients who present with slowly progressive sensorineural hearing loss and ataxia many years after eradication of a childhood cerebellar tumor.
Adolescent↗
Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report.
In many patients with superficial siderosis of the central nervous system (CNS) no source of bleeding can be established, despite extensive examinations. The authors report a patient with superficial siderosis and a spinal arteriovenous malformation (AVM) that was not visible on magnetic resonance (MR) imaging or myelography but was identified on angiographic studies. This 71-year-old man presented with a 2-year history of progressive gait difficulties and hearing loss. Examination showed ataxia, hearing loss, and quadriparesis. On MR imaging superficial siderosis of the brain and spinal cord as seen; however, MR imaging of the CNS, as well as cerebral angiography and myelography studies, did not reveal the source of hemorrhage. Spinal angiography revealed a small slow-flow pial AVM at the C-5 level originating from the anterior spinal artery. A C-5 corpectomy was performed and the AVM was obliterated. The patient did well and reported no further progression of his symptoms during 3 months of follow up. Spinal angiography is indicated to complete the evaluation of patients with superficial siderosis, even if results of spinal MR imaging and myelography studies are normal. Obliteration of spinal AVMs may successfully prevent the progression of superficial siderosis.
Aged↗
[Renal siderosis in cardiac hemolysis].
Renal siderosis caused by intravasal haemolysis has been studied in 202 autopsy cases, which based on the origin of the intravasal haemolysis have been divided into three groups: 1. patients deceased months or years after artificial valve implantation; 2. patients with congenital or acquired defective heart valves without surgical treatment; 3. patients deceased several days after open heart surgery. Renal siderosis has been detected by prussian-blue staining method. In 1/3 of the cases quantitative determination of the iron content has also been carried out. In cases of chronic defective heart valves without an operation renal siderosis has not developed. Among patients, who underwent open-heart operation it has been observed only in one case. After valvular replacement-except one case-renal siderosis developed only in patients with valvular failure. These findings seem to indicate that unless rare exceptions renal siderosis develops only in cases of long lasting intravasal haemolysis.
Heart Valve Prosthesis↗
Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches.
OBJECTIVE: Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches. RESULTS: The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years. CONCLUSIONS: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.
Adult↗
A long term follow up of ocular siderosis: quantitative assessment of the electroretinogram.
Siderosis oculi is a severe sequel of retained, iron made, intraocular foreign body. Iron atoms or ions, dissolved from the foreign body, may diffuse to the retina and produce irreversible cellular damage. Therefore, early extraction of an iron foreign body is recommended. When the risks of surgical intervention outweigh the danger of siderosis, the patient is periodically examined in order to detect the initial signs of siderosis. The most commonly used test for quantitative and objective assessment of retinal function is the electroretinogram (ERG). We report here a long term ERG follow-up (about 8 years) of a patient suffering from a unilateral iron intraocular foreign body. The development of siderosis was detected by any of the ERG responses; cone-dominated, rod-dominated or mixed cone-rod responses. However, the degree of the assessed damage varied and strongly depended upon the flash intensity used to elicit the ERG response and upon the ERG wave chosen to assess retinal function. The relationship between the ERG b- and a-waves showed a profound deterioration reflecting a reduction in signal transmission from the photoreceptors to the inner nuclear layer. These findings suggested that iron toxicity produced more damage to the inner retina than to the outer retina.
Electroretinography↗
Elevated CSF lactoferrin in superficial siderosis of the central nervous system.
This study sought to identify abnormalities in the levels of iron transport proteins in patients with superficial siderosis of the central nervous system. We compared patients with superficial siderosis (n = 7) with patients suffering from various other neurological disorders (n = 176, total). CSF and serum levels of lactoferrin, and CSF levels of transferrin were measured by enzyme-linked immunosorbent assay. Serum transferrin was measured by nephelometry. Lactoferrin, but not transferrin, levels in the CSF were significantly elevated in superficial siderosis. Unexpectedly, CSF transferrin was decreased in multiple sclerosis patients. Enhanced CSF lactoferrin may reflect an increased iron transport requirement in the central nervous system in superficial siderosis and might be a useful measure for monitoring response to therapy.
Central Nervous System Diseases↗