1/67. Severe hyperparathyroidism with hypercalcemia associated with chronic renal failure at pre-dialysis stage.We report a case of a 23-year-old Japanese woman who had severe hyperparathyroidism associated with chronic renal failure before the start of dialysis treatment. Her chief complaints were swelling and pain in both shoulders. Laboratory examination revealed renal failure (BUN 134 mg/dl, serum Cr 7.3 mg/dl), severe normocytic normochromic anemia (hemoglobin 4.3 g/dl), hypercalcemia (11.8 mg/dl), and hyperphosphatemia (9.7 mg/dl). serum PTH levels were extremely increased (intact PTH >1,000 pg/ml: normal range 10-50 pg/ml). X-ray examination of the skull and shoulders showed a salt and pepper appearance, and cauliflower-like deformity of the distal end of both clavicles, respectively. Accelerated ectopic calcification was observed in the costal cartilages, internal carotid arteries, and splenic arteries. Ultrasonographic examination revealed enlargement of the four parathyroid glands. thallium-technetium subtraction scintigraphy of the parathyroid glands showed increased uptake into the upper two. Renal needle biopsy revealed severe impairment of the interstitium and tubules with much milder changes in glomeruli. The etiology of the renal failure could not be identified. Hemodialysis, total parathyroidectomy and auto-transplantation into the forearm were immediately performed. The pathological diagnosis was chief cell hyperplasia of the parathyroid glands. Based on the presence of chronic renal failure, remarkable hyperphosphatemia with mild hypercalcemia, an unusually high level of serum PTH, and accelerated ectopic calcification, the patient was diagnosed to have severe secondary hyperparathyroidism caused by chronic renal failure with major impairment of the renal interstitium and tubules.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/67. PHEX expression in parathyroid gland and parathyroid hormone dysregulation in X-linked hypophosphatemia.X-linked hypophosphatemia (XLH), a renal phosphate (Pi) wasting disorder with defective bone mineralization, is caused by mutations in the PHEX gene (a Pi-regulating gene with homology to endopeptidases on the x chromosome). parathyroid hormone (PTH) status in XLH has been controversial, with the prevailing belief that hyperparathyroidism develops in response to Pi therapy. We report a 5-year-old girl with XLH (patient 1) who had significant hyperparathyroidism at presentation, prior to initiation of therapy. We examined her response to a single oral Pi dose, in combination with calcitriol, and demonstrated a rise in serum concentration of intact PTH, which peaked at 4 h and paralleled the rise in serum Pi concentration. We also present two other patients whose parathyroid glands were analyzed for PHEX mRNA expression following parathyroidectomy. Patient 2 had autonomous hyperparathyroidism associated with chronic renal insufficiency, and patient 3, with XLH, developed autonomous hyperparathyroidism after 8 years of therapy with Pi and calcitriol. Following parathyroidectomy, patient 3 exhibited an increase in both serum Pi concentration and renal Pi reabsorption. The abundance of PHEX mRNA, relative to beta-actin mRNA, in parathyroid glands from patients 2 and 3 was several-fold greater than that in human fetal calvaria, as estimated by ribonuclease protection assay. In summary, we have shown that hyperparathyroidism can be a primary manifestation of XLH and that PHEX is abundantly expressed in the parathyroid gland. Given that PHEX has homology to endopeptidases, we propose that PHEX may have a role in the normal regulation of PTH.- - - - - - - - - - ranking = 2.3333333333333keywords = gland (Clic here for more details about this article) |
3/67. dna cytofluorometric analysis using HP/DAPI double staining of parathyroid carcinoma arising in a patient with chronic renal failure and secondary hyperparathyroidism.A 55-year old female patient on long-term hemodialysis began to suffer from pain in her knees and ankles. An ultrasonographic study showed enlargement of all four parathyroid glands. serum parathyroid hormone and calcium levels were increased. parathyroidectomy was performed. The right superior gland was enlarged and adherent to surrounding tissues. The other three glands were slightly enlarged. Histologically, the largest gland was a parathyroid carcinoma because capsular and vascular invasion were observed. To our knowledge, there have been only 13 cases of parathyroid carcinomas arising in patients with chronic renal failure reported in the English literature. To evaluate the characteristics of this tumor, we measured nuclear dna and protein content using stains for HP (hematoporphyrin)/DAPI (4,6-diamidino-2-phenylindole dihydroporphyrin chloride). The nuclear dna pattern was typically diploid or tetraploid. The cellular protein content was similar to that seen in the controls. The discrepancy between the histology, indicative of malignancy and the cytofluorometrical findings show that it is difficult to determine the prognosis for patients with secondary hyperparathyroidism and parathyroid carcinomas solely from the results of tumor dna cytometry.- - - - - - - - - - ranking = 1.3333333333333keywords = gland (Clic here for more details about this article) |
4/67. What is tertiary hyperparathyroidism?Five patients who had gross abnormalities of calcium and phosphorus metabolism due to long standing renal failure are described to illustrate the difficulties with the term "tertiary hyperparathyroidism". One patient who had unequivocal biochemical tertiary hyperparathyroidism was found histologically to have nodular hyperplasia of all four glands even though one gland weighed twice as much (12g) as the combined weight of the other three. Another patient was not hypercalcaemic but had all the other features of the condition including rapid onset of osteitis fibrosa, vascular calcification and a probable parathyroid adenoma, with hyperplasia of the three glands. The other three had hypercalcaemia only after a reduction in the plasma inorganic phosphorus due either to renal transplantation or aluminum hydroxide therapy. The bone histology of the five patients varied from severe osteomalacia to severe osteitis fibrosa. A consideration of the factors involved in causing hypercalcaemia in these patients and a review of the literature leads to the conclusion that the term tertiary hyperparathyroidism is often misleading and best avoided.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/67. Kimura's disease: a case report.Kimura's disease is a rare, idiopathic condition that usually affects young men of Asian descent. The decrease is characterized by swelling and lesions in the head and neck region, with involvement of the subcutaneous soft tissue, major salivary glands, and lymph nodes. patients almost always have eosinophilia and elevated serum immunoglobulin e levels. The diagnosis is established by biopsy. Kimura's disease is usually self-limiting. Its etiology is unknown but is thought to be a manifestation of an aberrant allergic response. In this paper, we describe the case of a 30-year-old patient who was diagnosed with Kimura's disease at our institution.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
6/67. radio-guided surgery in recurrent renal hyperparathyroidism: report of a case.BACKGROUND: It has been demonstrated that radio-guided surgery offers several advantages in treating primary hyperparathyroidism. Even if it is considered less helpful in renal hyperparathyroidism, it could be of tremendous advantage in the treatment of persistent or recurrent secondary hyperparathyroidism. methods: We report a case of recurrent secondary hyperparathyroidism treated by the use of radio-guided surgery. The preoperative assessment consisting of ultrasonography, magnetic resonance imaging, and 99mTc-sestamibi scintigraphy identified a parathyroid in the upper mediastinum. The patient underwent a radio-guided neck re-exploration that allowed a rapid localization and excision of the ectopic gland, which was located in the anterosuperior mediastinum, in front of the trachea, between the innominant and the left common carotid artery. RESULTS: The operative time was 45 minutes. The patient was discharged on the first postoperative day. A decrease in serum calcium and parathyroid hormone was observed subsequently. A follow-up of 6 months did not show any recurrence. CONCLUSIONS: The case reported indicates that radio-guided surgery can help surgeons detect parathyroid tissue in selected cases of renal hyperparathyroidism.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
7/67. A case of living-related kidney transplantation in Bartter's syndrome.Bartter's syndrome is a renal tubular disorder characterized by hypokalemia, metabolic alkalosis, increased urinary excretion of potassium and prostaglandins, a relative vascular resistance to the pressor effects of exogenous angiotensin ii, and hyperplasia of the juxtaglomerular apparatus. In most patients, the glomerular filtration rate is normal and chronic renal failure does not develop. We report here on a case of living-related kidney transplantation in Bartter's syndrome, in which a non-steroidal anti-inflammatory drug is suspected to be the cause of the end-stage renal disease.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
8/67. Secondary hyperparathyroidism and brown tumor in dialyzed patients.Secondary hyperparathyroidism is one of the most common complications of chronic renal failure (CRF). Its pathogenesis is multifactorial and still not completely understood. Pathological mechanism of hypocalcemia, hyperphosphatemia and calcitriol deficiency are basic characteristics of CRF and main reason for morphological changes in parathyroid glands and hyperparathyroidism (HP). We present a case of a female patient born in 1975. At the age of 10, a urinary infection was diagnosed for the first time and treated. Six years later, as nausea and vomiting started, CRF based on bilateral reflux was diagnosed and the patient was included in the hemodialysis treatment. The patient was again examined in 1997, when biochemical parameters, including the level of parathyroid hormone, ultrasonography of the neck, scintigraphy of the skeleton and densitometry revealed secondary HP. Parathyreoidectomy was perfomed in 1998. During the follow up period, a tumefaction on a ramus mandibulae dex. was noticed, which was cytologically diagnosed as osteitis fibrosa, "brown tumor", a rare complication of the secondary HP. Surgery was performed and PHD was granuloma gigantocelulare. Prevention and therapy of secondary HP is a problem that demands early actions to avoid possible complications.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
9/67. Calcification of all four parathyroid glands in a hemodialysis patient with secondary hyperparathyroidism revealed by computerized tomography.This report describes the parathyroid scan, computerized tomography and histologic findings in a young female hemodialysis patient with severe secondary hyperparathyroidism. These findings included hyperplasia and calcification of all four parathyroid glands.- - - - - - - - - - ranking = 1.6666666666667keywords = gland (Clic here for more details about this article) |
10/67. Amyloid goiter in familial mediterranean fever (FMF): a clinicopathologic study of 10 cases.FMF amyloidosis is an important etiological factor of end stage renal disease (ESRD) in Mediterranean Countries. Apart from major target organs as cardiovascular, respiratory and gastrointestinal system, endocrine organs can also be involved. We planned to investigate the thyroid involvement in our amyloidosis group. The aim of this study was to determine clinical characteristics of amyloid goiter in FMF patients and the abnormalities of thyroid function, as well as to identify pathologic characteristics. Twenty-two hemodialysis patients (mean age 34.1 /- 14 years, range 17-68) whose ESRD secondary to FMF amyloidosis were evaluated with physical examination, serum levels of thyroid hormones, ultrasound examination of thyroid glands, thyroid syntigraphic studies. goiter was found in 10 patients (4 male, 6 female) having enlarged neck mass (mean age 35 /- 14 years, range 23-64). The serum levels of thyroid hormones and TSH were normal in 4 patients. Other four cases had euthyroid sick syndrome. Only one patient developed tender enlarged neck mass with subacute thyroiditis symptoms and one had primary hyperthyroidism. Ultrasound examination showed; hypoecoic nodules in 6 patients diffuse multinodular enlargement in 4 patients. Thyroid syntigraphic studies revealed hypoactive nodules in 7 patients and hyperactive nodules in 3 patients. After the laboratory tests were completed, in 10 patients diagnosis were made with fine needle aspiration biopsy. Of 10 patients 5 underwent subtotal thyroidectomy. Immunohistochemical evaluation demonstrated the presence of amyloid AA immunoreactivity in all cases. In conclusion fine needle aspiration from the thyroid when enlarged is useful in the diagnosis of suspected amyloidosis, especially since it is a safe, easily performed procedure. With the help of amyloid goiter diagnosis the patient's prognosis on hemodialysis and with renal transplantation can be predicted. Amyloid goiter must be searched in hemodialysis patients especially in Mediterranean Countries.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
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