11/124. Primary neonatal hyperparathyroidism. Report of a case and review of the literature.The seventh case in the world literature of primary hyperparathyroidism in a neonate is reported. This is the fifth case in which an autopsy was performed. The clinical and anatomic findings in all seven cases are reviewed and compared. Neonates with primary hyperparathyroidism show diffuse hyperplasia of the parathyroid glands. The bones show disturbed osteogenesis, bone resorption, and widespread fibrosis of the marrow cavities. Bony cysts are not appreciated. Pathologic fractures are common. Marked hypercalciuria or hyperphosphaturia is usually not observed, perhaps because the immature renal tubules fail to respond to the influence of excess parathormone. Aminoaciduria and anemia are commonly observed. The prognosis is grave, and the etiology of this syndrome remains unexplained.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
12/124. Documented hyperparathyroidism of thirty-six years' duration.The fascinating history of the first Memorial Hospital patient who was diagnosed as having hyperparathyroidism is reviewed. The illness presented as a cystic mass in a femur in 1929, which was treated with radiation. When the patient was first seen at Memorial Hospital in 1931, the diagnosis of osteitis fibrosa cystica was made; serum calcium was 14 mg/100 ml. In 1932, 6 years after Mandl performed the first parathyroidectomy ever for osteitis fibrosa cystica, this patient's neck was explored, and a right hemithyroidectomy was done, with removal of two normal parathyroid glands. The parathyroid tumor was finally located and partially removed in 1937 after a second failure at neck exploration in 1936. Correspondence between Dr. Edward D. Churchill at the massachusetts General Hospital and Dr. Bradley Coley at Memorial Hospital indicated the concern at that time about uncontrollable tetany, which had been fatal in some contemporary cases and which had led to the practice of only partially removing the tumor. Following this, the patient was observed with documented hypercalcemia and chemical evidence of hyperparathyrodism until age 79. The physical and chemical abnormalities over the years up to and including her last exam are presented. The case is important not only from the historical viewpoint, but because it lends a perspective to long-term parathyroid disease, which is becoming less appreciated in this day of the routine serum calcium by SMA-12 screening. The question of partial parathyroidectomy for adenoma or hyperplasia is reviewed, and the question of observation of patients with mild hypercalcemia who probably have parathyroid tumors is discussed.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
13/124. Synchronous water-clear cell double parathyroid adenomas a hitherto uncharacterized entity?water-clear cell hyperplasia is a rare but well-documented cause of primary hyperparathyroidism. Parathyroid adenomas of the water-clear cell type are exceptionally rare, and only 2 cases have been reported. We describe a patient with synchronous water-clear cell double parathyroid adenomas, an entity that has not previously been reported. In our case, the enlarged superior parathyroid glands were completely replaced by water-clear cells, with only a minute rim of extracapsular, histologically unremarkable parathyroid tissue. The inferior parathyroid glands were grossly unremarkable, and incisional biopsy specimens were histologically normal (no foci of water-clear cells were identified). The findings in this case are most consistent with the diagnosis of double adenomas of the water-clear cell type. We acknowledge that despite molecular proof of monoclonality of the 2 lesions, it is not possible to entirely exclude the possibility that this unusual case could be due to asymmetric hyperplasia.- - - - - - - - - - ranking = 2keywords = hyperplasia (Clic here for more details about this article) |
14/124. hyperparathyroidism-an unusual case.A case of primary hyperparathyroidism is described that presented unusual features. The patient had been treated previously for squamous cell carcinoma of the lung and therefore it was necessary to exclude ectopic hyperparathyroidism. The results of the tests generally used to distinguish primary from ectopic hyperparathyroidism-the serum chloride level, the serum calcium response to cortisone suppression and the absence of residual or recurrent tumour-suggested that the patient did have the primary form of the disorder. Exploration of the neck revealed no parathyroid tumour or hyperplasia, and the mediastinal parathyroid adenoma was localized only by determination of parathyroid hormone in the venous drainage from the neck and mediastinum.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
15/124. multiple endocrine neoplasia (men)--an overview and case report--patient with sporadic bilateral pheochromocytoma, hyperparathyroidism and marfanoid habitus.The multiple endocrine neoplasia syndromes are divided into two categories: men type I and men type II. The men type II syndrome is further divided into men IIa and men IIb. The syndromes are characterized by benign and malignant changes in two or more endocrine organs, as well as incidental changes in nervous, muscular and connective tissue. Two main forms can be distinguished: the men-I syndrome with hyperplasia of the parathyroid gland, accompanied by islet cell tumor and pituitary adenoma; the men-II syndrome with medullary thyroid carcinoma in combination with bilateral pheochromocytoma and hyperplasia of the parathyroid gland (men IIa), while type IIb is characterized by the additional appearance of neurocutaneous manifestations without primary hyperparathyroidism. Characteristics shared by these syndromes include the involved cell type, most of the tumors are composed of one or more specific polypeptide- and biogenic amine-producing cell types (APUD--amine precursor uptake and decarboxylation). The second characteristic is the increased incidence in certain families. The hereditary component is autosomal dominant with variable expression but high penetrance. Mechanisms of tumorigenesis differ in these syndromes. While men I is caused by an inherited mutation of a tumor suppressor gene, menin, located on the long arm of chromosome 11, men II is caused by activation of the RET proto-oncogene. We have reported the case of a young man exhibiting bilateral pheochromocytoma. In addition, the patient showed mild primary hyperparathyroidism and marfanoid habitus, all these stigmata usually being part of the men-II syndrome. Although this described patient showed a phenotypic mixture of the men-IIa and men-IIb syndrome, the genetic analysis for men II and von-Hippel-Lindau gene did not reveal any pathologic mutations, the endocrine disorders described here are not related to multiple endocrine neoplasia syndromes.- - - - - - - - - - ranking = 2keywords = hyperplasia (Clic here for more details about this article) |
16/124. Unusual presentation of primary hyperparathyroidism with osteoporosis, hypercalcemia, and normal parathyroid hormone level.We report the case of a woman with osteoporosis, chronic hypercalcemia, and normal levels of parathyroid hormone (PTH). Surgical exploration revealed hyperplasia of the parathyroid glands. hypercalcemia was corrected immediately by surgery, and this was followed by a dramatic improvement in bone mineral density. This case represents a rarely reported presentation of primary hyperparathyroidism with an atypical laboratory finding.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
17/124. Parathyromatosis: a cause for recurrent hyperparathyroidism.OBJECTIVE: To report a case of parathyromatosis as a cause for recurrent hyperparathyroidism. methods: We present the case history, laboratory results, operative interventions, and pathologic findings in a 36-year-old woman. Relevant reports from the literature are reviewed. RESULTS: Our patient, who had been undergoing long-term hemodialysis because of renal failure, presented with secondary hyperparathyroidism and progressive bone pain. After an uneventful subtotal parathyroidectomy (removal of 3-1/2 glands), her symptoms resolved in conjunction with normalization of parathyroid hormone levels. Subsequently, however, recurrent hyperparathyroidism and severe bone pain necessitated second and third neck explorations, during which parathyromatosis was discovered. A total thyroidectomy was performed because of the bilateral nature of the disease. Postoperatively, the patient's bone pain resolved substantially, although her parathyroid hormone levels remained high. CONCLUSION: Parathyromatosis is a rare cause of recurrent hyperparathyroidism after parathyroidectomy. It consists of hyperfunctioning parathyroid tissues scattered throughout the neck, due either to intraoperative tissue spillage and subsequent implantation or to hyperplasia of parathyroid rests from embryologic development. This is one of the few case reports of parathyromatosis and the first case report of a mixed form of the disease, consisting of features of both subcapsular parathyroid rests and extracapsular implantation.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
18/124. Primary hyperparathyroidism in an infant with three parathyroid glands and pulmonary calcinosis.A 2 month-old male infant presented with severe hypercalcemia due to parathyroid hyperplasia. A total parathyroidectomy and partial heterotopic autotransplantation were carried out. hypercalcemia recurred two months later. Normocalcemia was re-established after removing one half of the implanted tissue. Despite two separate surgical explorations and several imaging studies, including 99mTc-sestamibi scintigraphy, ultrasonography, and MRI, only three parathyroid glands were found. Severe pulmonary calcinosis has not previously been reported in children with PHPT. In conclusion, developmental variations of the parathyroid glands may be difficult to identify with present imaging techniques. This may pose difficulties in management of PHPT. The present report describes pulmonary calcinosis as a sequela which can cause additional morbidity in these infants.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
19/124. Parathyroid hyperplasia: an unusual cause of neonatal hypercalcemia.Primary hyperparathyroidism (PHPT) in infants is caused by parathyroid chief cell hyperplasia. patients present with symptoms of chronic hypercalcemia, such as failure to thrive, irritability, abdominal pain, and anorexia. Medical therapy is inadequate, often resulting in chronic hypercalcemia or death. Partial or total surgical removal of the parathyroid gland is the preferred treatment. We describe a case of a 7-month-old infant with PHPT secondary to hyperplasia successfully treated with a subtotal parathyroidectomy.- - - - - - - - - - ranking = 6keywords = hyperplasia (Clic here for more details about this article) |
20/124. Inactivating mutations of calcium-sensing receptor results in parathyroid lipohyperplasia.Familial hypocalciuric hypercalcemia (FHH) is an autosomal dominant disease characterized by mild hypercalcemia, an inappropriately high parathyroid hormone level, and absence of hypercalciuria. Heterozygous inactivating mutations of calcium-sensing receptor (CaSR) are found in about two thirds of patients with FHH. Histologic examination of parathyroid glands in FHH is reported to show normal histology or chief cell hyperplasia. Thus, histologic features of the parathyroid glands in FHH vary, and there is no clear histologic criterion that indicates FHH. The authors have encountered three hypercalcemic patients with characteristic histologic features of enlarged parathyroid glands. Clusters of parenchymal cells were mixed with fat cells, and the area of fat cells was 33% to 49% of the total area. These features are similar to those described as parathyroid lipohyperplasia. Postoperative evaluation showed that fractional excretion of calcium was low in these patients. Direct sequencing of the polymerase chain reaction product showed that the first patient was heterozygous for an already reported inactivating mutation of CaSR (P55L). The second patient was also heterozygous for a novel inactivating mutation (R220W). The third was homozygous for an inactivating mutation (Q27R). These results indicate that histologic features of parathyroid lipohyperplasia suggest the presence of inactivating mutations of CaSR.- - - - - - - - - - ranking = 7keywords = hyperplasia (Clic here for more details about this article) |
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