Sixteen working welders with more than seven years' exposure and a chest radiograph suggestive of siderosis and 13 healthy unexposed men were studied. Each subject had a comprehensive medical and work history, physical examination, standard chest film, and pulmonary function investigation (lung volumes, ventilatory capacities, pulmonary compliance in static and dynamic conditions, specific compliance, and elastic work of breathing).
Seven of the welders had some exertional dyspnoea and three complained of cough. Although spirographic values were generally within the normal range, the arc welders had a statistically significant reduction in static and functional compliance. Seven had a functional compliance under the lower limit of the control group. Differences in elastic work of breathing and specific compliance were not significant.
The possible causes of pulmonary function impairment in welders are discussed.
Haemophilus pittmaniae was described in 2005 as a new species distantly related to Haemophilus parainfluenzae. This member of the human saliva microbiota has also been further isolated from various body fluids without formal description of the patients.
We report the case of H. pittmaniae isolate made from a sputum specimen collected from a 58-year-old Caucasian man with a massive fibrotic form of siderosis who was awaiting lung transplantation. Identification of the isolate was ascertained by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry and 16S rRNA gene sequencing. H. pittmaniae was considered to be responsible for the worsening of the patient’s chronic respiratory failure and was successfully treated with oral amoxicillin.
H. pittmaniae should be regarded as a new pathogen responsible for respiratory tract infection in patients with chronic lung diseases.
A patient with hypertrophic cardiomyopathy (HCM) and transfusion-dependent sideroblastic anemia, who presented with decompensated heart failure, is described. The present case demonstrates the usefulness of cardiac magnetic resonance imaging as a noninvasive imaging modality to assess the etiology of new systolic dysfunction in the setting of HCM. Cardiac magnetic resonance imaging is able to differentiate between the dilated ‘burned-out’ phase of HCM and a concomitant dilated cardiomyopathy secondary to myocarditis or hemosiderosis.
Cardiac MRI; Hemochromatosis; Hypertrophic cardiomyopathy
The histological features of 40 spleens surgically removed from maintenance haemodialysis patients are reported. Twenty-four of the 40 (60%) showed massive iron loading and a significant direct correlation was found between iron loading and the amount of intravenous iron dextran administered. Since parenteral iron dextran appears to be a major factor in causing iron overload in haemodialysis patients its use as a method of iron replacement in these patients would appear inappropriate.
In the present study, cirrhosis of the liver was found in one of six cases of transfusional siderosis. The relationship between iron overload and cirrhosis is discussed and the suggestion is made that factors other than iron deposition may have been responsible for the hepatic fibrosis. Three patients with a greater degree of iron overload, and for a longer period, did not develop fibrosis or cirrhosis. The possibility remains that iron absorbed from the gut and stored in excess without having passed through the haemoglobin molecule or the reticuloendothelial system may be toxic, as cases have been reported in which cirrhosis of the liver was attributed to prolonged iron medication.
The cirrhosis in idiopathic haemochromatosis may be due to lack of an unknown hepatic cell enzyme, as in galactosaemia, and the increased iron absorption may be a concomitant finding. Transfusional or secondary haemochromatosis is not a definite entity, and the liver cirrhosis is probably due to causes other than the excess deposition of haemosiderin.
The authors studied the penetration of iron administered extrabulbarly into the ocular tissues of rabbits. It was found that iron passes from the orbit into the eyeball and accumulates in considerable quantities in the sclera, choroid, retina, ciliary body, and even in the vitreous and corneal epithelium. However, light microscopy failed to show any damage to the ocular tissues. The mechanism by which iron penetrates into the eyeball is discussed, and comparison is made between changes in the tissues, which characterise siderosis produced by an intrabulbar iron foreign body, and those in which an extrabulbar foreign body is involved.
Two unusual events concerning intraocular foreign bodies are presented. The first patient had an occult or unsuspected intraocular foreign body. He showed iridoplegia with mydriasis, siderosis iridis, and an intraocular piece of iron lying posteriorly near the retina. The foreign body was removed and the patient regained normal iris color and pupillary activity. His vision remains 20/15 six years postoperatively dispite ensuing retinal detachment one year after removal of the foreign body. The second patient was a young boy injured by a blasting cap explosion. He lost one eye from the injury and had a piece of intraocular brass in his left eye. In spite of the development of chalcosis and a mature cataract the lens gradually shrank in the pupillary space permitting a clear aphakic area and 20/25 vision. The brass fragment migrated forward and inferiorly and was finally extruded under the conjunctiva five years later, where it was removed and chemically analyzed by x-ray diffraction.
Superficial siderosis (SS) is a rare disorder due to chronic bleeding into the subarachnoid or intraventricular space. The most common clinical presentation is progressive ataxia and hearing loss. The authors report two patients who presented with dementia as the primary manifestation of SS. The cognitive impairment marked by cortical frontotemporoparietal dysfunction was consistent with the pattern of signal abnormalities seen on brain magnetic resonance imaging (MRI). Diagnosis of SS must be considered when T2*-weighted MRI shows typical signal hypointensity outlining the brain and spinal cord surfaces. Performing such MRI sequences appears to be of particular interest in the context of dementia etiological diagnosis.
Dementia; Diagnosis; Superficial siderosis
Superficial siderosis is a potentially manageable neurodegenerative disorder, caused by chronic subarachnoid haemorrhage and iron deposition along the central nervous system surfaces. Association with oral anticoagulant therapy is well known, but its definite role as a causative agent is yet to be clarified. Two Caucasian women, both under long-term oral anticoagulation: a 74 year old woman with slowly progressive hearing loss and mild cerebellar ataxia; a 72 year old woman suffering from behavioural changes, rapidly progressive cognitive decline and latter developing paraparesis. Magnetic resonance imaging showed striking hypointensities along the surfaces of cerebellum, brainstem, frontotemporal cortices, spinal cord, and lumbar arachnoid therefore suggesting superficial siderosis. No specific bleeding source was found in any of the patients. Anticoagulation could not be stopped in the first patient due to a mechanic valve and slowly progressive worsening occurred. In contrast, for the second patient anticoagulation withdrawal was feasible and marked motor and cognitive improvement ensued. Superficial siderosis is associated with unvarying progression, mostly when no direct source of bleeding is identified. Nonetheless, we verified striking motor and cognitive improvement after anticoagulants withdrawal in one of the patients. This may reinforce the need to consider such modifiable factor in future patient management.