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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary hyperparathyroidism (PHP) and myelofibrosis are rare entities in childhood. Myelofibrosis secondary to PHP is also extremely rare. We report a 15-year-old boy presented with generalized weakness, vomiting, and pallor. A parathyroid adenoma was detected on the left distal parathyroid gland. PHP was diagnosed together with hepatosplenomegaly and pancytopenia. Bone marrow biopsy revealed grade 3-4 reticulin fibrosis. As early as 2 months after the left distal parathyroidectomy, hematologic parameters improved without any other intervention. His liver and spleen also gradually decreased in size. We concluded that the pancytopenia was as a result of myelofibrosis from PHP.
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PMID:Primary hyperparathyroidism as an extremely rare cause of secondary myelofibrosis in childhood. 2375 88

The natural history of untreated asymptomatic primary hyperparathyroidism (PHPT) remains incompletely understood. Increased level of parathyroid hormone produces the characteristic biochemical phenotype of hypercalcemia, hypophosphatemia and the various clinical sequelae of chronic hypercalcemia. Periodic paralysis (PP) is a group of disorders of different etiologies with episodic, short-lived and hyporeflexic skeletal muscle weakness, with or without myotonia, but without sensory deficit and without loss of consciousness. However, PHPT has rare association with episodic quadriparesis mimicking as PP.
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PMID:An unusual case of episodic quadriparesis. 2425 Nov 70

A young woman with severe vitamin D deficiency presented with proximal muscle weakness, fragility fracture and pseudoarthrosis. On evaluation, she was found to have hypercalcaemia, a single parathyroid adenoma and an undetectable 25-hydroxyvitamin D level. She received parenteral cholecalciferol and subsequently underwent curative parathyroidectomy. Postoperatively, she had hungry bone syndrome, which she gradually recovered from with calcium and calcitriol replacement. Notably, her calcium levels were in the lower limit of normal range and associated with elevated alkaline phosphatase levels at postoperative Day 14. Follow-up for the next four years showed that the patient had remarkable symptomatic and radiological improvements. In this report, we discuss the pathophysiological interactions between vitamin D deficiency and associated primary hyperparathyroidism.
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PMID:Pseudoarthrosis and fracture: interaction between severe vitamin D deficiency and primary hyperparathyroidism. 2427 9

Primary hyperparathyroidism (PHPT) is extremely uncommon among children and is more likely to be associated with genetic syndromes, multiglandular involvement, and more severe symptoms. Rickets can very rarely be the presenting feature of PHPT in children. Rickets was diagnosed in a 12-year-old girl presenting with short stature, genu valgum, eversion deformity at the ankle joints, and flat feet. Radiograms showed generalized osteopenia, widening of the distal ends of the long bones along with splaying, cupping and fraying. Biochemical evaluation revealed low serum calcium (7.8 mg/dL), low phosphorus (1.4 mg/dL), vitamin-D deficiency [25-hydroxy-vitamin-D (25(OH)D): 8.7 ng/mL], and elevated intact parathyroid hormone (PTH, 811 pg/mL). Re-evaluation due to lack of clinical improvement following vitamin-D and calcium supplementation revealed hypercalcemia 11.9 mg/dL, normal 25(OH)D 41 ng/mL, persistence of elevated PTH 632 pg/mL. A 99mTc-sestamibi scan showed increased uptake at the lower pole of the right lobe of the thyroid. A right inferior parathyroidectomy was performed. Histopathology revealed chief cell type parathyroid adenoma. Last evaluated 4 months after surgery, the bone pains and proximal weakness had resolved, with significant improvement in the patient's quality of life. Rickets in the setting of PHPT often masks the classical phenotype of PHPT. In a child with rickets, lack of improvement following vitamin-D supplementation, hypercalcemia at presentation or following vitamin-D supplementation are warning signs which necessitate further evaluation to rule out PHPT.
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PMID:Primary hyperparathyroidism masquerading as rickets: diagnostic challenge and treatment outcomes. 2437 38

The clinical picture of primary hyperparathyroidism (PHPT) has changed over the last three decades and many asymptomatic patients are now diagnosed through the unexpected finding of high serum calcium levels. However, though not yet considered as typical features of the disease and therefore not included in the guidelines for surgery, many data are available on neuropsycological manifestations and their impact on quality of life in asymptomatic patients. PHPT patients indeed show early experience nonspecific symptoms, such as weakness, depression, sleep disturbance, memory loss and anxiety. Although the underlining mechanisms have not been still identified, the prevalence of psychiatric and cognitive deficits has been investigated in many studies, as well as the possible association with quality of life and well-being improvement after surgery. This article aims to review the current knowledge on quality of life in PHPT patients before and after surgery and the possible clinical implications of these findings.
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PMID:Quality of life in patients with primary hyperparathyroidism. 2439 7

Primary hyperparathyroidism (PHPT) may lead to skeletal deformities, fractures and renal failure in symptomatic patients if untreated. We present a case of a 30-year-old woman presented with muscle weakness, weight loss, hypercalcaemia and a pathological fracture, eventually with rapidly progressive musculoskeletal disease. Subsequent biochemical, radiographic and scintigraphy findings were consistent with PHPT from an ectopic mediastinal adenoma, and concomitant vitamin D deficiency. The severe hypercalcaemia was adequately temporised with hydration, forced diuresis and intravenous bisphosphonates. Removal of the adenoma by video-assisted thoracoscopic surgery was contemplated; however, consent was withdrawn precluding histological confirmation. A review of literature shows the changing profiles of patients with PHPT, the uncommon occurrence of parathyroid adenomas in ectopic locations and possible association between severity of PHPT and vitamin D status.
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PMID:Primary hyperparathyroidism from a probable ectopic parathyroid adenoma with severe skeletal disease and vitamin D deficiency. 2463 9

Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT.
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PMID:Bone disease in primary hyperparathyroidism. 2516 47

Acute primary hyperparathyroidism and parathyroid crisis are characterized by life-threatening hypercalcemia, a rare disorder. A 69-year-old female patient presented at our hospital's neurology clinic with weakness, nausea, vomiting, depression, and hypercalcemia. Treatment of hypercalcemia resulted in no improvement in neurological symptoms, indicating resistance to treatment. Thyroid ultrasonography and parathyroid scintigraphy revealed hypoechoic nodules in the right lobe, pieces of nodules in the left lobe, and high serum calcium and parathyroid hormone levels. After provision of intensive medical treatment including hydration, diuresis, and bisphosphonate infusion resulted in only minimal decrease in the calcium level, urgent surgical treatment was performed. Frozen biopsy of the right intrathyroidal giant parathyroid adenoma in the right lobe confirmed initial diagnosis of primary hyperparathyroidism. Based on the biopsy findings, right parathyroidectomy and right total and left subtotal thyroidectomy were performed. Histopathologic examination revealed a parathyroid adenoma localized inside large thyroid nodules. Review of the findings resulted in diagnosis of intrathyroidal parathyroid adenoma. Symptoms of hypercalcemia improved rapidly during the postoperative period.
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PMID:Life-threatening intrathyroidal parathyroid adenoma. 2578 64

Primary hyperparathyroidism is rare in children. We report a 12-year-old girl who presented with recurrent renal calculi, muscular weakness and inability to walk; was diagnosed to have parathyroid adenoma and underwent parathyroidectomy.
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PMID:Primary hyperparathyroidism in a child. 2606 Mar 68

Primary hyperparathyroidism (PHPT) is currently the most common endocrine disorder in Czech Republic after diabetes and thyroid diseases particularly in postmenopausal women. Over the past 40 years PHPT has changed from a rare severe disease of the bones and kidneys to common disease with hypertension, peptic ulcer, pancreatitis, easy fatigue and proximal muscle weakness. During 43 years we have examined one of the greatest groups of patients with PHPT. In the early 1970 the estimated incidence of PHPT in former Czechoslovakia was approximately 8 cases per 100 000 persons per year. Our data showed that the incidence of PHPT increased sharply to 24 cases per 100 000 persons per year in same community with the introduction of automated serum calcium and iPTH measurement. The disease is four times more frequent in women as in man. The ratio women to men did not changed since 1981. However the incidence of PHPT changed in Czech Republic from previous years, it develops around the fifth decade of life and is increasingly discovered with advancing age. The incidence of hypertension, diabetes mellitus, cholelithiasis, pancreatitis and peptic ulcer among patients with PHPT is higher as compared with the incidence of these diseases in the general population. However there are still patients suffering from bone and renal complication of PHPT. Removing the adenoma by an experienced surgeon is the first choice of treatment of patients with PHPT. The study offers valuable data on the actual state of hyperparathyroid patients in the Czech Republic.
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PMID:Changes in the Pattern of Primary Hyperparathyroidism in Czech Republic. 2609 66


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