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PubMed1996

[Mobilization of intraocular foreign bodies by magnetic resonance tomography].

A 49-year-old patient suffered from a binocular perforating trauma with metal foreign bodies in 1974. During an MRI examination in 1992 for a lumbar spine herniation a metal foreign body was mobilised from the deeper vitreal and retinal area, now causing optical disturbances freely floating in the anterior vitreous. Refusing an operation, the patient, an electrical engineer, tried himself to remove the foreign body out of the optical axis by exposing his head to the electro-magnetic field of an induction coil (pulsed magnetic induction B at t0 of 0.26 Tesla). The foreign body was split into multiple small parts no longer disturbing the patient. To early detect a siderosis regular ophthalmological controls including ERG are necessary. This example stresses that even small intraocular metal foreign bodies are a contraindication for the usually applied field strength of MRI examinations.

Electromagnetic Fields↗

PubMedDate not supplied

[Beta-thalassemia in Germany. Results of cooperative beta-thalassemia study].

At present, about 300 patients with thalassemia major are living in Germany. Starting in 1991, a multicenter study in Germany has concentrated on identifying all patients suffering from thalassemia as well as on establishing a uniform therapy protocol including follow-up diagnostic procedures. After six years of study, the data of 198 patients suffering from thalassaemia major were analysed. The majority of these patients originate from endemic regions around the Mediterranean Sea. The patient's median age is 13.8 years (range 1-37.5 yrs.). At present, about 20% of patients are older than 21 years. Regarding transfusion therapy, a shortening of the average transfusion interval to 3 weeks in most cases occurred. Throughout the entire period, median baseline haemoglobin concentrations of 10.0 g/dl could be observed. The evaluation of serum ferritin levels revealed considerable differences depending on patients age. 60% of patients in the first decade of life showed good therapeutic results with serum ferritin levels below 1800 ng/ml. In contrast, 52% of patients older than ten years presented with ferritin levels above 2500 ng/ml. During the observation, a decreasing number of patients with ferritin levels above 2500 ng/ml was observed in patients aged 15 to 21 years of age. The situation of patients aged 9 to 15 years proved to be more problematic. More than half of all treated patients presented with siderotic complications as cardiac disease in 13%, liver disease in 21%, impaired glucose metabolism in 14%, hypothyroidism in 24% and hypogonadism in 59% of all patients. These values did not change considerably during the observation apart from an increase of cardiac disorders to 20%. Since the situation concerning siderosis and the lack of compliance proved to be particularly difficult in adolescent patients, further efforts has to concentrate on this age group.

Adolescent↗

PubMed2001

Efficacy of eye patching for traumatic corneal abrasions: a controlled clinical trial.

STUDY OBJECTIVE: We sought to evaluate the efficacy of eye patching in the treatment of traumatic corneal abrasions in terms of time to healing and reduction in pain and discomfort. METHODS: One hundred sixty-three patients presenting at the emergency department of a large university-affiliated hospital with traumatic corneal abrasions were included in this single-blind prospective controlled trial. Eligible patients were assigned to 1 of 2 treatment regimens: topical antibiotic ointment and occlusive patch over the affected eye (n=82) or topical antibiotic ointment 4 times a day without an occlusive patch (n=81). Patients were reexamined every 24 hours until corneal healing occurred. Healing evaluation was performed by the emergency physician, using a slit lamp with fluorescein staining, without knowledge of the patient's assignment to a treatment group. The degree of discomfort was assessed at each visit by using a visual analog scale. RESULTS: Both treatment groups were similar regarding size of the corneal lesions, delay from trauma to first ED visit, presence of foreign body or siderosis, initial degree of discomfort, and presence of specific symptoms (irritation, foreign body sensation, photophobia, redness, and pain). In the patched group, cumulative incidences of healing were 51%, 78%, and 92% after 1, 2, and 3 days, respectively, compared with 60%, 83%, and 88% in the nonpatched group. In the patched group, symptoms of initial discomfort decreased by 4.8, 4.1, and 5.5 cm after 1, 2, and 3 days, respectively, compared with 3.3, 5.1, and 6.5 cm in the nonpatched group. CONCLUSION: Eye patching does not appear to be beneficial in the treatment of traumatic corneal abrasions compared with topical antibiotic ointment.

Administration, Topical↗

PubMed2001

A mouse model of familial porphyria cutanea tarda.

Approximately one-third of patients with porphyria cutanea tarda (PCT), the most common porphyria in humans, inherit a single mutant allele of the uroporphyrinogen decarboxylase (URO-D) gene. PCT associated with URO-D mutations is designated familial PCT. The phenotype is characterized by a photosensitive dermatosis with hepatic accumulation and urinary excretion of uroporphyrin and hepta-carboxylic porphyrins. Most heterozygotes for URO-D mutations do not express a porphyric phenotype unless hepatic siderosis is present. Hemochromatosis gene (HFE) mutations are frequently found when the phenotype is expressed. We used homologous recombination to disrupt one allele of murine URO-D. URO-D(+/-) mice had half-wild type (wt) URO-D protein and enzymatic activity in all tissues but did not accumulate hepatic porphyrins, indicating that half-normal URO-D activity is not rate limiting. When URO-D(+/-) mice were injected with iron-dextran and given drinking water containing delta-aminolevulinic acid for 21 days, hepatic porphyrins accumulated, and hepatic URO-D activity was reduced to 20% of wt. We bred mice homozygous for an HFE gene disruption (HFE(-/-)) to URO-D(+/-) mice, generating mice with the URO-D(+/-)/HFE(-/-) genotype. These animals developed a porphyric phenotype by 14 weeks of age without ALA supplementation, and URO-D activity was reduced to 14% of wt. These data indicate that iron overload alone is sufficient to reduce URO-D activity to rate-limiting levels in URO-D(+/-) mice. The URO-D(+/-) mouse serves as an excellent model of familial PCT and affords the opportunity to define the mechanism by which iron influences URO-D activity.

Aminolevulinic Acid↗

PubMed2003

Tumour necrosis factor alpha and its promoter polymorphisms' role in the phenotypic expression of hemochromatosis.

BACKGROUND: The majority of hemochromatosis patients are homozygous for the HFE-C282Y mutation. However, less than half of C282Y homozygous subjects identified by population screening studies actually develop the disease. The cytokine TNF-alpha is implicated in the regulation of iron metabolism at different levels. Our aim was to study the role of TNF-alpha and its promoter polymorphisms in the phenotypic expression of hemochromatosis in individuals with and without the C282Y mutation. METHODS: We studied 4 groups of 10 subjects each: (1) C282Y homozygotes without clinical hemochromatosis; (2) C282Y homozygotes with hemochromatosis; (3) secondary hemochromatosis (without C282Y mutation); and (4) controls. Groups were age-matched and sex-matched. Peripheral blood mononuclear cells (PBMC) were stimulated with lipopolysaccharide (LPS) and the release of TNF-alpha was measured. Additionally, the G/A polymorphisms at position -238 and -308 of the TNF-alpha, gene were determined by PCR and RFLP analysis in 178 hemochromatosis patients and 41 controls. RESULTS: TNF-alpha production from PBMC at 8 and 24 h after increasing concentrations of LPS stimulation were similar in the four groups. The prevalence of TNF-alpha polymorphisms was similar in patients and controls. The prevalences of cirrhosis, siderosis, median s-ferritin and median ALT values were similar in patients with and without the TNF-alpha polymorphisms. CONCLUSIONS: Neither TNF-alpha, released from PBMC nor the presence of TNF-alpha polymorphisms seem to be associated with disease manifestation in hemochromatosis.

Adult↗

PubMed2006

Low serum ferritin levels are misleading for detecting cardiac iron overload and increase the risk of cardiomyopathy in thalassemia patients. The importance of cardiac iron overload monitoring using magnetic resonance imaging T2 and T2*.

The incidence of cardiomyopathy was monitored in a 6-year follow-up study involving 56 transfused thalassemia patients treated with deferoxamine (DFO), deferiprone (L1) or their combination. During this period, five female patients on regular subcutaneous or intravenous DFO presented with cardiac complications. Three patients suffered congestive heart failure and the other two arrhythmias. Four of the five patients maintained serum ferritin levels of about 1 mg/L or below and the fifth about 1.5 mg/L for several years prior to the cardiomyopathy. Cardiac magnetic resonance imaging (MRI) T2* and T2 was performed in four patients after the cardiomyopathy, identifying the presence of moderate-to-heavy siderosis. The treatment of the five patients has since changed, involving mainly the use of L1. Low serum ferritin levels appear to be misleading for detecting cardiac iron overload and this may increase the risk of cardiomyopathy. The MRI T2 and T2* relaxation time measurements are a more accurate method of detecting cardiac iron overload. Chelation therapy using L1 or appropriate L1/DFO combinations can reduce cardiac iron overload and the mortality rate in thalassemia patients.

Administration, Oral↗

PubMed2006

Effective combination therapy of deferiprone and deferoxamine for the rapid clearance of excess cardiac IRON and the prevention of heart disease in thalassemia. The Protocol of the International Committee on Oral Chelators.

The International Committee on Oral Chelators (ICOC) combination therapy protocol involving the administration of deferiprone (L1) during the day (80-110 mg/kg/day) and deferoxamine (DFO) (40-60 mg/kg at least 3 days/week) during the night for 8-12 hours using a pump, or the whole 24 hours using an elastomeric pump infuser, has been tested in 11 thalassemia patients (seven males, four females) over a period of 9-28 months. The patients had variable serum ferritin levels (0.54-4.6 mg/L) and cardiac iron load ranging from normal to severe siderosis levels (MRI T2*: 4.7-45 ms). There was a substantial overall reduction in serum ferritin levels (0.17-2.16 mg/L) and normalization of cardiac iron (MRI T2* >20 ms) in all patients. In two patients with severe and moderate cardiac iron load range levels, cardiac iron normalization was achieved within 9-10 months. Two patients on L1 monotherapy (80-120 mg/kg/day) maintained normal range MRI T2* cardiac iron levels over the same period. The ICOC combination therapy protocol appears to be the most effective and least cumbersome form of chelation treatment for the rapid clearance of excess iron from the heart.

Administration, Oral↗

PubMedDate not supplied

Pathology teach and tell: neonatal hemochromatosis with massive hepatic necrosis.

Neonatal hemochromatosis (NH) is an uncommon disorder clinicopathologically defined by severe liver disease of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements. NH phenotypically is a similar disorder to hereditary hemochromatosis. However, its extremely early onset of liver failure makes it notably unique. Massive liver necrosis in the newborn is a rare occurrence, but whenever present hemochromatosis should be considered in the differential diagnosis. Herein, we report a case of neonatal hemochromatosis that had massive hepatic necrosis with sparing only little parenchyma. The outcome was fatal within the first month of life.

Diagnosis, Differential↗

PubMed2004

Pulmonary responses to welding fumes: role of metal constituents.

It is estimated that more than 1 million workers worldwide perform some type of welding as part of their work duties. Epidemiology studies have shown that a large number of welders experience some type of respiratory illness. Respiratory effects seen in full-time welders have included bronchitis, siderosis, asthma, and a possible increase in the incidence of lung cancer. Pulmonary infections are increased in terms of severity, duration, and frequency among welders. Inhalation exposure to welding fumes may vary due to differences in the materials used and methods employed. The chemical properties of welding fumes can be quite complex. Most welding materials are alloy mixtures of metals characterized by different steels that may contain iron, manganese, chromium, and nickel. Animal studies have indicated that the presence and combination of different metal constituents is an important determinant in the potential pneumotoxic responses associated with welding fumes. Animal models have demonstrated that stainless steel (SS) welding fumes, which contain significant levels of nickel and chromium, induce more lung injury and inflammation, and are retained in the lungs longer than mild steel (MS) welding fumes, which contain mostly iron. In addition, SS fumes generated from welding processes using fluxes to protect the resulting weld contain elevated levels of soluble metals, which may affect respiratory health. Recent animal studies have indicated that the lung injury and inflammation induced by SS welding fumes that contain water-soluble metals are dependent on both the soluble and insoluble fractions of the fume. This article reviews the role that metals play in the pulmonary effects associated with welding fume exposure in workers and laboratory animals.

Air Pollutants, Occupational↗

PubMedDate not supplied

Nesidioblastosis in sickle cell disease.

Although the endocrine pancreas appears to play an important role in the pathophysiology of sickle cell disease, very little is known about the morphologic changes in this tissue. Our study was initiated to delineate the microscopic features of the endocrine pancreas in a large autopsy series of sickle cell hemoglobinopathies. From more than 650 cases archived at the Centralized Pathology Unit for Sickle Cell Disease (Mobile, AL), 224 autopsy cases were identified for review of clinical and gross autopsy findings and/or for microscopic studies, including histochemical stains (trichrome, reticulin, iron), and immunohistochemical stains (insulin, glucagon, somatostatin, and pancreatic polypeptide). The gross examinations were recorded as unremarkable in 65% of the autopsies. In childhood and adolescence (< or = 18 years), pancreas weights (50.76 +/- 5.16SE gm) were significantly greater (p < 0.0001) than age-matched controls (30.42 +/- 3.59SE gm). In adulthood, pancreas weights (108.34 +/- 5.29SE gm) were not significantly different from controls (110 gm). Microscopic findings included vascular congestion (48%), edema (65%), siderosis (31%), and nesidioblastosis (76%), which included islet cell dispersion (53%), hyperplasia (23%), and hypertrophy (25%). Analysis by age groups suggested that islet cell dispersion/hyperplasia persists unchanged, whereas diameters of compact islets tend to increase with age. These findings may be related to local tissue hypoxia and/or increased metabolic energy needs in sickle cell disease.

Adolescent↗

PubMed1985

Compartmentalization of iron in sickle cell anemia--an autopsy study.

In autopsy studies of two sickle cell anemia (SCA) patients, iron was totally absent from all organs in one case in which the serum ferritin had been low, while the other patient demonstrated hepatic, splenic, and renal siderosis but no iron stores in the bone marrow. Serum ferritin had been very high in this case. These findings suggest that in SCA bone marrow biopsy may be an unreliable means to determine iron stores, but rather the ferritin level should be used.

Adult↗

PubMed1998

High frequency of iron bone deposits in a Mexican population with renal osteodystrophy.

Renal osteodystrophy (ROD) is a multifactorial disease. Aluminium deposits have been implicated in its physiopathology but iron deposits have seldom been described. The purpose of this study was to investigate the presence of iron on the mineralization front, in 70 patients with ROD. Their mean age was 48+/-16 years, 36 were female, 34 male, 55 were admitted on peritoneal dialysis (78.5%) and 15 to haemodialysis (21.5%), for a period of 28+/-22 months. A bone biopsy was obtained from each patient after double tetracycline labelling. Blood samples were also obtained at the time of bone biopsy. The histomophometric analysis was performed following the criteria of Sherrard et al., with slight modifications; beside the usual stains, aluminium, iron and amyloid stainings were done on all bone specimens. Biochemical findings were: Ca 8.8+/-0.9 mg/dl, P 6.1+/-1.5 mg/dl; total alkaline phosphatase 197+/-258; PTHm 4.9+/-4.05ng/ml (normal 0.4-0.7 ng/ml), calcitonin 11+/-6 pg/ml (normal 1-26 pg/ml). Osteitis fibrosa was found in 31 patients (44.28%), mixed bone disease in two patients (2.28%); mild bone disease in 20 subjects (28.57%), adynamic bone lesion in 15 cases (21.42%) and osteomalacia in two patients (2.28%). Iron deposits were found on the mineralization front in 43 patients (61.4%); in 17, the percentage was <25 and, in 26, >25%. The iron deposits in the osteitis fibrosa group were highly significant (25/31). The aluminium deposit at the mineralization front was observed in eight patients (11.4%); in all but one, the percentage of this metal was <10%. Amyloid deposits were negative in all cases. The results show: (i) a Mexican population with ROD, present a highly significant incidence of siderosis on the bone mineralization front; (ii) in contrast, the aluminium deposits in this group of patients is lower than that reported in other series, and (iii) the spectrum of RO in this Mexican population is similar to that reported in other studies.

Adult↗

PubMed2000

Spontaneous and GnRH-provoked gonadotropin secretion and testosterone response to human chorionic gonadotropin in adolescent boys with thalassaemia major and delayed puberty.

To elucidate whether the cause of sexual maturation arrest in thalassaemia is of gonadal or pituitary etiology, 10 males with thalassaemia and delayed puberty and 10 with constitutional delay of growth and pubertal maturation (CSS) were extensively studied. Their spontaneous nocturnal gonadotropin secretion and gonadotropin response to intravenous 100 micrograms gonadotropin-releasing hormone (GnRH) were evaluated. Circulating testosterone concentration and clinical response were evaluated after 3 days, 4 weeks and 6 months of intramuscular administration of human chorionic gonadotropin (HCG) (2500 U/m2/dose). Thalassaemic boys had significantly lower circulating concentrations of testosterone compared to those with constitutional delay of growth and sexual maturation (CSS) at the same pubertal stage. Short- and long-term testosterone response to administrations of HCG was markedly decreased in thalassaemic boys. After 6 months of HCG administration 50 per cent (5/10) of the boys did not show significant testicular enlargement or genital changes. Despite the low circulating concentrations of testosterone, none of the patients had high basal or exaggerated gonadotropin response to gonadotropin releasing hormone (GnRH) stimulation. Luteinizing hormone (LH) peak responses to GnRH were significantly lower as compared to controls. Follicle-stimulating hormone (FSH) peak responses to GnRH did not differ among the two study groups. The mean nocturnal LH and FSH secretion was significantly decreased in all thalassaemic boys as compared to boys with CSS at the same pubertal stage (testicular volume). These data proved that hypogonadotropic hypogonadism is the main cause of delayed/failed puberty in adolescents with thalassaemia major. MRI studies revealed complete empty sella (n = 5), marked diminution of the pituitary size (n = 5), thinning of the pituitary stalk (n = 3) with its posterior displacement (n = 2), and evidence of iron deposition in the pituitary gland and midbrain (n = 8) in thalassaemic patients, denoting a high incidence of structural abnormalities (atrophy) of the pituitary gland. Moreover, in many of the thalassaemic boys, the defective testosterone response to long-term (6 months) HCG therapy denoted significant testicular atrophy and/or failure secondary to siderosis. It appears that testosterone replacement might be superior to HCG therapy in these patients. This therapy should be introduced at the proper time in these hypogonadal patients to induce their sexual development and to support their linear growth spurt and bone mineral accretion.

Adolescent↗

PubMed1980

Unusual histiocytic reaction to Monsel's solution.

Monsel's solution for hemostasis was applied to a wound produced at the site of a punch excision biopsy from the wrist of a 51-year-old white man. The biopsy specimen showed an incompletely excised basal cell carcinoma. Thirty days later, the residual carcinoma was excised. Adjacent to a basal cell carcinoma in the second biopsy specimen was an intradermal nodule composed of large polygonal cells and multinucleated histiocytic giant cells containing granules and clumps of dark brown and black pigment. This pigment was strongly positive for iron when stained by Perl's method. This unusual histiocytic reaction to topically applied ferric subsulfate must be differentiated from malignant melanomas and from histiocytic neoplasms with siderosis.

Biopsy↗

PubMed1984

Problems in the necropsy diagnosis of alcoholic liver disease.

Problem areas in the necropsy diagnosis of alcoholic liver disease are reviewed, potential sources of confusion delineated, and diagnostic guidelines proposed. The entire spectrum of alcoholic liver disease, including alcoholic hepatitis, may be perfectly mimicked by severe obesity, diabetes, and perhexiline maleate toxicity. Focal fatty change in the liver introduces sampling errors in the assessment of steatosis. Nodular regenerative hyperplasia of the liver mimics a micronodular cirrhosis both clinically and macroscopically. Measurement of the liver iron concentration reliably differentiates between alcoholic liver disease with siderosis and idiopathic hemochromatosis. The evaluation of preexisting fibrosis or cirrhosis in cases of massive hepatic necrosis is aided by stains for elastic fibers. Alcohol abusers taking acetaminophen (paracetamol) in excessive, but not suicidal doses are at risk of developing fatal "late" acetaminophen hepatotoxicity. Fatal viral hepatitis may be overlooked in an alcoholic with preexisting liver disease.

Acetaminophen↗

PubMedDate not supplied

Effects of phlebotomy on urinary porphyrin pattern and liver histology in patients with porphyria cutanea tarda.

Urinary porphyrin profiles and liver histology have been investigated in a group of adult alcoholics with porphyria cutanea tarda (PCT) before and after one year phlebotomy. Both parameters were evaluated during the same period in a group of patients who did not undergo specific therapy for PCT. All patients were advised to abstain from alcohol. At the end of the one year observation period there was a significant fall of urinary total porphyrins and in the uro/coproporphyrin ratio in treated patients compared to basal values whereas no changes were found in controls. Liver biopsy findings revealed a significant reduction of hepatic fatty degeneration and siderosis with no changes in inflammatory infiltrates and fibrosis in treated patients, so the progression of liver disease was similar to controls. These results show that clinical and biochemical remission of PCT can occur independently of the evolution of the concomitant liver disease.

Alcoholism↗

PubMed1997

Pure red cell aplasia associated with hepatitis C infection.

We report the case of a 34-year-old woman with recurrent pure red cell aplasia and evidence of hepatitis B and C infection. Review of the English literature identified 19 prior cases in which pure red cell aplasia was associated with hepatitis. This case is the first in which serologic evidence of hepatitis C infection was documented. This patient also had porphyria cutanea tarda and marked hepatic siderosis but no active hepatitis or cirrhosis. Treatment with cyclophosphamide and prednisone produced complete remission of the pure red cell aplasia. Erythroid colony formation (colony-forming unit-erythroid and erythroid burst-forming unit) was reduced in cultures of bone marrow obtained during relapse but was normal in remission marrow. However, addition of the patient serum, whether collected during relapse or remission, inhibited erythroid colony formation by her bone marrow. These observations, and the known extrahepatic immunologic manifestations of hepatitis C infection, suggest that the pure red cell aplasia occurred because of autoimmune mechanism provoked by the infection.

Adult↗

PubMed1992

Noninvasive liver-iron quantification by computed tomography in iron-overloaded rats.

RATIONALE AND OBJECTIVES: The benefit of computed tomography (CT) for the noninvasive determination of liver-iron concentration in human iron-overload diseases is a controversy in the literature. To study the sensitivity of CT for liver-iron quantification under experimental conditions, the authors measured single- and dual-energy CT numbers in vivo in the livers of iron-overloaded rats. METHODS: Thirty-five rats were subjected to an iron-rich diet for various periods, from 1 to 20 weeks, then scanned by single- and dual-energy CT. CT absorption was correlated to liver-iron content, which was determined by wet ashing and spectrophotometry. RESULTS: Whereas a good correlation (r = 0.99 at 96 kV; r = 0.95 at 125 kV) between CT numbers and liver-iron concentration was found, CT was insensitive to low concentrations of iron. Dual-energy CT scanning results showed greater scattering in liver-iron quantification compared with single-energy CT. CONCLUSIONS: In rats, the sensitivity of single- and dual-energy CT is too low to quantify liver iron in the diagnostically most relevant region of mild liver siderosis (1-3 mg iron/g wet weight [w.wt]).

Animals↗