1/61. Apparent cure of Graves-Basedow disease after sibling allogeneic bone marrow transplantation.Evidence that allogeneic bone marrow transplantation (BMT) can cure or alter the course of intractable autoimmune diseases comes from both extensive experimental work in animal models and anecdotal case reports in humans. We describe a female patient diagnosed as having severe aplastic anaemia (SAA), hyperthyroidism and ophthalmopathy of Graves-Basedow disease who received a BMT from her histocompatible sister. Fifty-three months after BMT, complete remission of hyperthyroidism and ocular signs persists. The SAA is cured and she is free of any chronic graft-versus-host disease (GVHD). In the early post-BMT period, PCR analysis of bone marrow and peripheral blood cells confirmed a complete chimerism of donor origin. Thus, it is plausible to attribute the resolution of the patient's thyroid hyperfunction and opththalmopathy to the replacement of the host immune system.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/61. Congenital hyperthyroidism: autopsy report.We report the autopsy of a stillborn fetus with congenital hyperthyroidism born to a mother with untreated Graves' disease, whose cause of death was congestive heart failure. The major findings concerned the skull, thyroid, heart, and placenta. The cranial sutures were closed, with overlapping skull bones. The thyroid was increased in volume and had intense blood congestion. Histological examination showed hyperactive follicles. The heart was enlarged and softened, with dilated cavities and hemorrhagic suffusions in the epicardium. The placenta had infarctions that involved at least 20% of its surface, and the vessels of the umbilical cord were fully exposed due to a decrease in Wharton's jelly. hyperthyroidism was confirmed by the maternal clinical data, the fetal findings of exophthalmia, craniosynostosis, and goiter with signs of follicular hyperactivity. Craniosynostosis is caused by the anabolic action of thyroid hormones in bone formation during the initial stages of development. The delayed initiation of treatment in the present case contributed to the severity of fetal hyperthyroidism and consequent fetal death.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
3/61. Reversal of dysthyroid optic neuropathy following orbital fat decompression.AIMS: To document the successful treatment of five patients with dysthyroid optic neuropathy by orbital fat decompression instead of orbital bone decompression after failed medical therapy. methods: Eight orbits of five patients with dysthyroid optic neuropathy were selected for orbital fat decompression as an alternative to bone removal decompression. Treatment with systemic corticosteroids and/or orbital radiotherapy was either unsuccessful or contraindicated in each case. All patients satisfied clinical indications for orbital bone decompression to reverse the optic neuropathy. High resolution computerised tomographic (CT) scans were performed in all cases and in each case showed signs of enlargement of the orbital fat compartment. As an alternative to bone decompression, orbital fat decompression was performed on all eight orbits. RESULTS: Orbital fat decompression was performed on five patients (eight orbits) with optic neuropathy. Optic neuropathy was reversed in all cases. There were no cases of postoperative diplopia, enophthalmos, globe ptosis, or anaesthesia. All patients were followed for a minimum of 1 year. CONCLUSIONS: In a subset of patients with an enlarged orbital fat compartment and in whom extraocular muscle enlargement is not the solitary cause of optic neuropathy, fat decompression is a surgical alternative to bony decompression.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
4/61. Increased levothyroxine requirements presenting as "inappropriate" TSH secretion syndrome in a patient with nephrotic syndrome.patients with primary thyroid failure on levothyroxine (LT4) replacement who develop nephrotic syndrome (NS) may rarely present with an increase in LT4 requirements. In this report, we describe a patient with thyroid failure following radioactive iodine ablation for Graves' disease who required an escalation of LT4 doses following the onset of NS. The case presented with disproportionately elevated TSH levels in the presence of normal (or slightly subnormal) thyroid hormone levels, thus, masquerading as a state of "inappropriate" TSH secretion. This pattern of extreme dysregulation in thyroid function indices due to urinary loss of thyroid hormones has not been previously described in NS, and, therefore, extends the spectrum of endocrine manifestations of NS.- - - - - - - - - - ranking = 10.364800637681keywords = endocrine (Clic here for more details about this article) |
5/61. Hyperfunction of neutrophils in a patient with BCR/ABL negative chronic myeloid leukemia: a case report with in vitro studies.BACKGROUND: Among patients diagnosed with chronic myeloid leukemia (CML), a small percentage lack a BCR/ABL fusion gene, a landmark of CML. Their clinical features are distinct from patients with BCR/ABL positive CML, although to the authors' knowledge the pathogenesis to date has been unknown. methods: A 50-year-old female patient with BCR/ABL negative CML and multiple complications of graves disease, sweet syndrome, and a fatal pulmonary alveolar proteinosis (PAP) is described in the current study. To show a clonal origin of her myeloid cells, hypoxanthine phosphoribosyltransferase (HPRT) assay was applied. Because the patient developed a progressive and fatal neutrophilia, a screening of cell functions in neutrophilic lineage, including in vitro colony assay of her bone marrow cells and production of superoxide and interleukin-8 (IL-8) by blood neutrophils was performed. RESULTS: Southern blot analysis based on the polymorphism of the HPRT gene was compatible with monoclonality of her neutrophils. The patient had an increased amount of bone marrow granulocyte-macrophage progenitor cells, which formed colonies in response to a very low dose (0.1 ng/mL) of granulocyte-colony stimulating factor. in vitro production of superoxide and IL-8, which is an inducer of positive chemotaxis of neutrophils, by her peripheral neutrophils was markedly augmented. Her bronchoalveolar lavage fluid also contained a significant amount of IL-8 as well as an unusual infiltration of neutrophils. CONCLUSIONS: In the patient in the current study, hyperfunction of the neutrophils might have contributed to the onset of PAP as well as sweet syndrome and to the pathogenesis of BCR/ABL negative CML.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
6/61. Transfer of autoimmune hypothyroidism following bone marrow transplantation from a donor with Graves' disease.We describe the transfer of a different autoimmune thyroid disorder by bone marrow transplantation. The donor had euthyroid Graves' disease after treatment with antithyroid drugs and stable oxopthalmos, with persistent thyroid autoantibodies. One year after bone marrow transplant, the recipient developed atrophic autoimmune hypothyroidism. Six months later the donor developed transient subclinical hypothyroidism followed by clinical hyperthyroidism. In both recipient and donor the presence of thyrotropin (TSH)-binding inhibitory immunoglobulins was documented. Although TSH receptor antibodies usually act as a TSH agonist causing thyrotoxicosis, they can also can act as a TSH antagonist causing primary hypothyroidism. This case may be an example of how the same antibody can act as an agonist in the donor and an antagonist in the bone marrow recipient, causing two different autoimmune disorders: primary hypothyroidism in the recipient and Graves' disease in the donor.- - - - - - - - - - ranking = 1.1666666666667keywords = bone (Clic here for more details about this article) |
7/61. Two cases of refractory endocrine ophthalmopathy successfully treated with extracorporeal immunoadsorption.Endocrine ophthalmopathy (EO) is a severe disease entity that is characterized by retrobulbar swelling due to accumulation of glycosaminoglycans on an autoimmune basis. This disorder can lead to the loss of vision and often is resistant to conventional therapy. There is a relation to Graves' hyperthyroidism, but probably no close association. Two patients with severe EO that was resistant to usual therapeutic approaches including steroids and radiological and surgical measures underwent a 20 session course of intensive immunoadsorption therapy (Plasmaselect/Therasorb Anti-IgG) with a mean 2- to 3-fold plasma volume treated. After the first sessions, both patients voiced an impressive relief of their major symptoms, which was confirmed by ophthalmological investigation. Throughout the time of therapy until present, these patients have remained at their respective levels of improvement. We consider immunoadsorption an effective therapeutic opportunity in severe EO resistant to conventional treatment.- - - - - - - - - - ranking = 41.459202550723keywords = endocrine (Clic here for more details about this article) |
8/61. Polyserositis as a rare component of polyglandular autoimmune syndrome type II.Polyglandular autoimmune (PGA) syndromes (types I and II) may affect various endocrine and non-endocrine organs in the body. In the commoner PGA type II, primary adrenal insufficiency, autoimmune thyroid disease and type I diabetes mellitus are the most frequent manifestations. serositis with pericardial or pleural involvement is not a well known component of the disease. Here, we report a 21-year-old man who first presented with a pleuropericardial effusion and Graves' disease, and who then developed type I diabetes mellitus.- - - - - - - - - - ranking = 20.729601275361keywords = endocrine (Clic here for more details about this article) |
9/61. Massive plasmocytosis due to methimazole-induced bone marrow toxicity.pancytopenia is a rare complication of the thionamide therapy reported secondary to aplastic anemia, the bone marrow being invariably hypocellular. We present a case of a 16-year-old female with Graves' disease who presented with massive bone marrow plasmocytosis mimicking multiple myeloma. The patient had already been on methimazole for a month when she was admitted to the Pediatric Unit with the diagnosis of sepsis. CBC revealed pancytopenia. Bone marrow aspirations showed hypocellular-normocellular bone marrow, 98% of plasma cells. At that time, MMI was discontinued and the patient was started on broad-spectrum antibiotics, dexamethasone, and G-CSF. Bone marrow aspiration day 4 still showed hypo-normocellular marrow, with remaining 6% plasma cells. Myeloma screen was negative; ANC >1,000 at day 7, platelets >50,000 at day 24. Twenty-four months after patient's discharge, her clinical condition, CBC, and bone marrow remained normal. To our knowledge this is the first report of pancytopenia due to MMI, where the usual hypoplasia found is replaced by massive plasmocytosis.- - - - - - - - - - ranking = 1.3333333333333keywords = bone (Clic here for more details about this article) |
10/61. Changes in autoimmune thyroid disease following allogeneic bone marrow transplantation.autoimmune diseases can be transmitted and eliminated by bone marrow transplantation (BMT). There have been several cases of autoimmune thyroid disease (AITD) occurring after BMT, but AITD remission has been rarely reported. We present four cases in which the remission or transfer of AITD occurred after an allogeneic BMT. Two patients with severe aplastic anemia (SAA) showed evidence of remission of Hashimoto's thyroiditis which they had before allogeneic BMT. One patient with SAA, which developed during treatment with propylthiouracil for Graves' disease, underwent allogeneic BMT and showed evidence of Graves' disease remission following BMT. In one patient, new AITD occurred after an allogeneic BMT from an HLA-matched sibling who already had AITD. These cases support the evidence that the immune system is newly reconstituted after BMT, and severe autoimmune disease can be an indication for BMT. To fully understand the real changes in autoimmune status after BMT, long-term prospective studies are necessary.- - - - - - - - - - ranking = 0.83333333333333keywords = bone (Clic here for more details about this article) |
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