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At least 955 records · Page 53Linked to original sources
PubMed2000

Cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions.

OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: Cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, Israel. PATIENTS: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, Arnold-Chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.

Adult↗
PubMed1978

Yersinia enterocolitica osteomyelitis in a child.

Yersinia enterocolitica systemic infections are uncommon and osteomyelitis due to this organism is very rare. We report a 9-year-old girl with thalassemia major, liver cirrhosis, and hemosiderosis who developed osteomyelitis of the rib caused by Y enterocolitica type 0:3. Serologic response to the infection was confirmed. No definite source of infection was identified.

Child↗
PubMed2006

Pulmonary hypertension and beta-thalassemia major: report of a case, its treatment, and a review of the literature.

Pulmonary hypertension is a common complication of beta-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with beta-thalassemia major and review the literature on pulmonary hypertension and beta-thalassemia major. A 28-year-old man with beta-thalassemia major, splenectomy, hepatitis C, and hemosiderosis who presented with increasing dyspnea on exertion was diagnosed with pulmonary hypertension. After receiving continuous epoprostenol infusion and desferoxamine, his functional capacity and hemodynamic status improved. To our knowledge, this is the first case of pulmonary hypertension associated with beta-thalassemia treated with continuous epoprostenol infusion and desferoxamine. Epoprostenol, beneficial in the treatment of other types of pulmonary hypertension, may ameliorate the morbidity and mortality of pulmonary hypertension associated with thalassemia.

Adult↗
PubMedDate not supplied

Up-and-down procedure (UDP) determinations of acute oral toxicity of nitroso degradation products of hexahydro-1,3,5-trinitro-1,3,5-triazine (RDX).

Hexahydro-1,3,5-trinitro-1,3,5-triazine (RDX), a widely used military explosive and soil and ground water contaminant of munitions manufacturing and artillery training sites, undergoes microbial nitroreductase metabolism to hexahydro-1-nitroso-3,5-dinitro-1,3,5-triazine (MNX), hexahydro-1,3-dinitroso-5-nitro-1,3,5-triazine (DNX), and hexahydro-1,3,5-trinitroso-1,3,5-triazine (TNX). Human occupational and accidental exposures to RDX, as well as acute oral exposures in rats, result in seizures, but little is known about the toxicity of the RDX degradation products. The main objective of the present study was to determine the oral LD50 of the most potent RDX N-nitroso product in female Sprague-Dawley rats using the recently validated up-and-down procedure (UDP). With only 26 rats, MNX was identified as the most potent metabolite and a maximum likelihood estimate of 187 mg kg(-1) (95% confidence interval 118-491 mg kg(-1)) for its LD50 was established and found equivalent to that of RDX determined with the same protocol. CNS toxicity, manifested as forelimb clonic seizures progressing to generalized clonic-tonic seizures, was the critical adverse effect. Further, confirmation of the UDP LD50 for MNX with a fixed-dose design enabled identification of 94 mg kg(-1) as the highest nonlethal dose. An ED50 of 57 mg kg(-1) was determined for neurotoxicity, while splenic hemosiderosis and decreased blood hematocrit and hemoglobin concentration occurred with a threshold at 94 mg kg(-1) in 14-day survivors. These studies, while providing new toxicity data necessary for the management of RDX-contaminated sites, illustrate the efficiency of the UDP for comparative acute toxicity determinations and its value in guiding further characterization of dose dependency of identified adverse effects.

Animals↗
PubMed2002

Theory of nonexponential NMR signal decay in liver with iron overload or superparamagnetic iron oxide particles.

A quantitative theory is proposed for the nonexponential NMR proton signal decay observed in liver with iron overload or superparamagnetic iron oxide particles. This effect occurs for Carr-Purcell-Meiboom-Gill (CPMG) sequences and is argued to be a direct consequence of the strong magnetic field inhomogeneities generated by the iron, rather than being due to tissue compartments. An approximate mathematical form is given for the signal decay, which is fit to experimental data for samples of rat liver with iron oxide particles, for samples of marmoset liver with hemosiderosis, and for in vivo human liver with hereditary hemochromatosis. The fitting parameters obtained are consistent with the pattern of iron deposition determined from histology. For the case of hereditary hemochromatosis, a good correlation is found between a parameter characterizing the nonexponential decay and the iron concentration. Implications for practical MR quantification of hepatic iron are discussed.

Animals↗
PubMed1976

[Data on the pathogenesis of solitary juvenile bone cysts (author's transl)].

On the basis of the histologic analysis of 45 solitary (juvenile) bone-cysts the author reveals new aspects of the pathogenesis. I. Data on sex-age-localisation are referred to. II. Due to the hemosiderosis regularly manifesting itself in the loose connective tissue layer lining the cyst and owing to the similarity to the liquified hematomas to be found elsewhere in the organism, as well as on the basis of the specific blood-supply conditions of the spongiosa of the increasing metaphysis the author presumes the intramedullary hemorrhage--possibly occurring also without fracture--to be the primary change. III. In the lining layer of the cyst, as well as in the adjacent bony tissue the author frequently observed capillary stase and to a considerable degree lymphedemas (resorption insufficiency). On the basis of these data the author presumes that the following mechanism might be hold responsible for the persisting of the cyst: The congestive (anoxic) wall of the capillary gets permeable for the proteins. Hence the fluid of high protein content, filling the cyst is constantly replaced. It is emphasized that solely the lymphatic vessels are apt to convey proteins. On the basis, however, of the histologic phenomena (lymphedema) observed, the pericystic lymphatic system is in the stage of relative insufficiency.

Adolescent↗
PubMed1980

Endocrine studies in Blackfan-Diamond anemia: evidence for hypothalamic-pituitary dysfunction under frequent transfusion therapy.

A 13 year old boy with Blackfan-Diamond anemia treated with frequent transfusions was investigated for endocrine abnormalities. Prepubertal plasms LH and FSH values, lack of sleep-related hormone rhythms of the gonadotropins, as well as prepubertal responses of LH and FSH to acute stimulation with LHRH strongly suggests that a hypothalamic-pituitary abnormality is the cause of the hypogonadotropic hypogonadism observed in this patient. As a result of impaired stimulation of the gonads plasm testosterone was prepubertal. A three-to fourfold increase of basal plasma PRL values was found without any signs of a typical sleep-dependent increase. Values obtained ranged between 21 ng/ml and 24 ng/ml (normal range 5-8 ng/ml). A normal response to TRH stimulation was found. These results suggest that hemosiderosis may be responsible for the hyperprolactinemia as a result of hypothalamic-pituitary dysfunction. Furthermore, dysfunction is demonstrated by prepubertal responses of LH and FSH to LHRH stimulation.

Adolescent↗
PubMed1996

Pharmaceutical analysis of the oral iron chelator deferiprone (DMHP,L1).

The oral iron chelator deferiprone (1,2-dimethyl-3-hydroxy-pyrid-4-one, DMHP, LT or CP20) can be a useful drug in patients with transfusional hemosiderosis. From 1987 about 1000 patients in 16 countries have taken this drug on the base of clinical trials or compassionate use. Since this compound is only available as a raw substance, it is important to ascertain its purity before bringing the drug into a pharmaceutical formulation. Because deferiprone is administered chronically and in high doses, intake of potential toxic impurities can be substantial. In this article a proposal for the quality control of deferiprone is presented in the form of a pharmaceutical monograph. This includes the analytical methods required for identification, purity checking and assay. Furthermore the way we synthesized the drug to get hold of it in a pure form is described. This synthesis is also used in manufacturing the drug commercially. The monograph can be used as a guideline for standardization of the quality of deferiprone to be used for further study and treatment.

Administration, Oral↗
PubMed1975

Bone marrow transplantation for aplastic anaemia from a HL-A and MLC-identical unrelated donor.

Bone-marrow transplantation (BMT) from an unrelated, HL-A-phenotype-identical, MLC-negative donor was performed in a 31 year old woman with severe longlasting aplastic anemia. In vitro assays failed to demonstrate humoral or cellular sensitization of the recipient against donor-type antigens. Following conditioning with cyclophosphamide, prompt but only transient engraftment of the transplant occurred accompanied by signs of mild graft-versus-host-disease (GVHD) of the liver. The results of a second bone marrow transplantation from the same donor cannot be evaluated due to early death of the recipient. It is concluded that bone marrow from unrelated, HL-A and MLC-identical donors may engraft without severe GVHD. Rejection of the graft in our patient may have been related to greater antigenic differences that can be expected to exist between HL-A and MLC-identical unrelated individuals than between HL-A and MLC-identical siblings. However, insufficient preparative immunosuppression with cyclophosphamide due to severe hepatic hemosiderosis appears equally likely as the cause of graft rejection. The possibly increased risk of graft rejection or severe GVHD should not preclude the use of unrelated HL-A and MLC-identical marrow donors, when histocompatible sibling donors are not available; but more potent immunosuppressive regimens than the cyclophosphamide protocol may be necessary to ensure permanent engraftment.

Adult↗
PubMed1986

Magnetic resonance appearance of the kidneys in paroxysmal nocturnal hemoglobinuria.

A diagnosis of paroxysmal nocturnal hemoglobinuria may be suggested with magnetic resonance imaging, based on the massive renal cortical hemosiderosis that occurs in this disease. A diffuse bilateral depression of renal cortical signal intensity will be observed that is caused mainly by a shortening of the T2 relaxation time of renal cortical protons by the paramagnetic effect of the locally increased ferric ion concentration in the proximal convoluted tubule.

Adult↗
PubMed2001

[Malignant melanoma of the ciliary body. A diagnostic problem].

BACKGROUND: Ciliary body melanoma is often diagnosed too late and therefore has a poor prognosis. The circular infiltration of the iridocorneal angle by malignant cells can lead to secondary glaucoma, often misdiagnosed as pigmentary glaucoma. MATERIAL AND METHODS: In a 40-year-old patient the diagnosis "atypical pigmentary glaucoma" was made due to elevated intraocular pressure and pigmentation of the iridocorneal angle; no changes were present in the iris. In a second case pronounced pigmentation of the chamber angle and secondary glaucoma led to the clinical diagnosis of a ring melanoma and resulted in enucleation of the globe. RESULTS: In the first case, increasing pigmentation of the chamber angle over 4 weeks led to the presumed diagnosis of a ring melanoma. Histopathological evaluation confirmed the diagnosis of a ring melanoma of the ciliary body. In the second case histology revealed retinal detachment with intraocular bleeding without intraocular melanoma. The pigmentation in the lower part of the chamber angle was due to hemosiderosis. CONCLUSION: Diagnosis of ciliary body melanomas may be difficult in some cases due to its similarity to other eye diseases. The differential diagnosis of malignant melanoma of the ciliary body should be considered in cases of unilateral pigmentary glaucoma.

Adult↗
PubMed1977

Treatment of iron overload in adults with continuous parenteral desferrioxamine.

Adult patients with transfusional hemosiderosis were given ascorbic acid and treated with the iron chelator, desferrioxamine B. The drug was administered by continuous subcutaneous or intravenous infusions using a light weight portable constant infusion device. On this regimen, four of the five patients studied were able to excrete significant amounts of iron (greater than 35 mg/da) when receiving a daily desferrioxamine dose of 1.5 to 2.2 g. Continuous subcutaneous infusion was well tolerated and about 80 per cent as effective as intravenous therapy in chelating iron. The number of prior transfusions, the hepatic iron content and the serum ferritin levels appear to be useful in predicting which patients will respond to iron chelation therapy, especially if there is little bone marrow erythropoietic activity. One patient with ineffective erythropoiesis did not have significantly increased hepatic iron stores but responded to the administration of desferrioxamine. Continuous subcutaneously administered desferrioxamine may prove to be adaptable for long-term outpatient therapy, allowing patients with ongoing transfusion requirements to go into negative iron balance. Long-term studies will be needed to demonstrate reversal of endocrine, hepatic and cardiac dysfunction secondary to iron deposition in these patients.

Adult↗
PubMed1987

Deferoxamine-enhanced fecal losses of aluminum and iron in a patient undergoing continuous ambulatory peritoneal dialysis.

Aluminum-associated osteomalacia and transfusion-induced hemosiderosis developed in an anephric patient receiving long-term maintenance treatment with continuous ambulatory peritoneal dialysis. Intravenous administration of 1.0 g of deferoxamine led to marked increases in the fecal elimination of aluminum and iron. Dialysate removal of these same metals also increased but to a lesser extent. This indicates that the biliary/fecal route of excretion may contribute significantly to deferoxamine-induced losses of aluminum and iron in patients undergoing continuous peritoneal dialysis.

Adult↗
PubMed1975

Perivascular hemosiderin deposition in muscle in Waldenström's macroglobulinemia.

A 63-year old woman with Waldenström's macroglobulinemina of 5 years' duration developed disabling pain in her extremities. The patient had required frequent plasmapheresis and transfusion for treatment of hypervisosity. Muscle biopsy from 2 separate sites showed striking deposition of hemosiderin in macrophages surrounding small vessels and features of slight denervation. Immunofluorescence demonstrated IgM deposition in vessel walls. Perivascular hemosiderin deposition in muscle has not previously been desicribed and may reflect traansfusion-hemosiderosis in a patient with a dysglobulinemia.

Basement Membrane↗
PubMed1986

Subchronic inhalation toxicity of p-nitroaniline and p-nitrochlorobenzene in rats.

For evaluation of subchronic toxicity of the two single-ring nitroaromatics, p-nitroaniline (PNA) and p-nitrochlorobenzene (PNCB), groups of 10 male and 10 female Sprague-Dawley rats were exposed to an aerosol/vapor of PNA in isopropanol at target concentrations of 0, 10, 30, or 90 mg/m3 or to PNCB vaporized from a solution in ethylene glycol monoethyl ether at target concentrations of 0, 5, 15, or 45 mg/m3 for 6 hr/day, 5 days/week for 4 weeks. Clinical signs of toxicity, body weights, results of ophthalmoscopic exam, hematology and clinical chemistry tests, organ weights, gross and histopathological changes were recorded. Exposure to PNA or PNCB resulted in a dose-related increase in blood methemoglobin levels. Mean red blood cell counts, hematocrit, and hemoglobin were significantly decreased in mid and high level animals exposed to PNCB. Mean spleen weights (absolute and relative to body weight) were significantly increased at the high dose levels in the two studies. A slight increase in spleen weights was also observed at the low concentration level in the PNA study. Absolute and relative liver weights also were increased among animals exposed to 45 mg/m3 PNCB. Microscopic changes were observed mainly in the spleen and included an increase in intensity of extramedullary hematopoiesis and hemosiderosis with both compounds. Spleens of animals exposed to PNCB also exhibited congestion. Neither PNA nor PNCB exhibited significant toxicological effects other than those of methemoglobinemia, anemia, and splenic changes classically associated with nitroaromatics at levels significantly above presently accepted occupational standard. Our data suggest that the current TLV for PNA which is 3 mg/m3 will provide adequate protection to the workers. OSHA's PEL of 1 mg/m3 for PNCB is to be preferred over the current TLV of 3 mg/m3 to provide a comparable margin of safety.

Aerosols↗
PubMed1995

Cutaneous oxalate deposition in a hemodialysis patient.

We describe calcium oxalate and amyloid arthropathy with cutaneous calcinosis without vitamin C supplement. A 34-year-old woman developed glomerulonephritis requiring chronic hemodialysis. Seven years after beginning hemodialysis, multiple crystal deposits appeared in her skin; she also presented with arthralgia and gait disturbance. A skin biopsy was performed, which disclosed calcium oxalate deposition. In addition, a right femoral neck prosthetic replacement was performed. Pathologic examination of the hip synovia revealed diffuse calcium oxalate, amyloid, and iron deposition. Calcium oxalate and amyloid arthropathy with synovial hemosiderosis was diagnosed, and therapy with desferal and high-flux membrane dialysis was started. Clinical improvement occurred after 6 months.

Adult↗
PubMed2006

Iron chelation treatment with combined therapy with deferiprone and deferioxamine: a 12-month trial.

The simultaneous use of deferioxamine (DFO) and deferiprone (DFP) has an additive effect in iron excretion in transfusion-dependent thalassemic patients. In a prospective study, we evaluated the safety and effectiveness of combined therapy with these two chelators. Fifty patients with beta-thalassemia were uniformly treated with DFP for 4 days per week and combined therapy with DFP and DFO for 3 days of the week. Efficacy was evaluated by ferritin and cardiac shortening fraction (SF). Hepatic hemosiderosis was also assessed by estimation of the T2 relaxation time by magnetic resonance in a subgroup of patients. Forty-three patients completed 1 year of therapy. Mean ferritin decreased from 3363.7 +/- 2144.5 microg/L to 2323.2 +/- 1740.8 microg/L (P < 0.0001). The reduction was significant even in the group of patients with ferritin <2500 microg/L. Significant improvement in T2 relaxation and SF was observed. The most common adverse events were gastrointestinal symptoms (20%) and transaminasemia (18%). The rate of agranulocytosis was 4.2 cases per 100 patient-years. Prolonged use of combined therapy with DFP and DFO is effective in decreasing iron load and improving cardiac function. Its possible association with higher incidence of agranulocytosis emphasizes the need for close monitoring.

Adolescent↗