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

We report a case of bone pain associated with primary hyperparathyroidism in a patient with sickle cell disease. A 17-year-old girl with sickle cell disease (SS phenotype) was seen for bilateral knee and back pain. She had had recurrent severe vaso-occlusive crises and acute chest syndrome in the course of her disease. In the last 2 years, she had frequent visits to the emergency department for severe bone pain. She complained of long-standing fatigue and lethargy. Her physical examination was normal. Hydroxyurea treatment, as well as and long- and short-acting narcotics were given, with little improvement in symptoms. Poor compliance with medication, family dysfunction, and potential narcotic addiction were felt to be significant contributors to the patient's symptoms. She was incidentally found to have an extremely elevated total calcium level of 3.19 mmol/L (range: 2.25-2.76) with an ionized calcium level of 1.9 mmol/L (range: 1.15-1.35). Phosphorus level was 0.82 mmol/L (range: 0.90-1.50), alkaline phosphatase level was elevated at 519 U/L (range: 10-170), and parathyroid hormone level was extremely high at 1645 pg/mL (range: 10-60). Her renal function was normal. Ultrasonography of the neck and a Sestamibi scan revealed a single left inferior parathyroid adenoma adjacent to the thyroid lobe. There was no evidence of an underlying multiple endocrine neoplasia. The patient was diagnosed with primary hyperparathyroidism. Fluid hydration, hydrocortisone, calcitonin, and bisphosphonates were initiated for acute hypercalcemia management before surgical excision of the left parathyroid adenoma. On review of previous blood work, a borderline calcium level of 2.72 was present 18 months before this admission. Two years postsurgery, she has normal renal function, calcium, and parathyroid hormone levels. The weekly visits to the emergency department for pain episodes decreased to 1 every 2 months within the first few months after her surgery. The decrease in pain episodes, even if it coincided with the treatment of primary hyperparathyroidism, may still reflect the natural evolution of sickle cell disease in this patient. However, the high morbidity associated with primary hyperparathyroidism was successfully prevented in this patient. Primary hyperparathyroidism is rare in childhood. In a recent study, it occurred more commonly in female adolescents and was because of a single adenoma, as in our patient. Significant morbidity, mainly secondary to renal dysfunction, was because of the delay in diagnosis after the onset of symptoms (2.0-4.2 years), emphasizing the need for a rapid diagnosis. Sickle cell disease affects approximately 1 of every 600 blacks in North America. Acute episodes of severe vaso-occlusive crisis account for > 90% of sickle cell-related hospitalizations and are a significant cause of morbidity in patients. There is no known association between sickle cell disease and primary hyperparathyroidism, and this case is most probably a random occurrence. However, as emphasized by this case report, pain may also be a harbinger of other disease processes in sickle cell disease. Because management may vary, we suggest that care providers consider the diagnosis of vaso-occlusive crisis as the diagnosis of exclusion and that other etiologies for pain be envisaged in this patient population, especially in the presence of prolonged pain or unusual clinical, radiologic, or biological findings.
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PMID:Primary hyperparathyroidism mimicking vaso-occlusive crises in sickle cell disease. 1688 90

We report a case of a brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism. A 19-year-old woman presented with a 3-month history of pain in the left pubic region. The laboratory data showed elevated serum calcium and intact parathyroid hormone, confirming the diagnosis of primary hyperparathyroidism. Plain radiography and computed tomography (CT) showed an expansile lytic lesion of the superior ramus of the left pubis. The cortex was thinned. On magnetic resonance (MR) images, the lesion was solid and cystic. The solid area of the lesion showed heterogeneous low to intermediate signal intensity on T1-weighted images and heterogeneous low to high signal intensity on T2-weighted images. The cystic area showed several fluid-fluid levels on T2-weighted images. Dynamic contrast-enhanced MR images after administration of Gd-DTPA showed marked, early enhancement of the solid area of the lesion. A delayed image showed prolonged enhancement of the solid area and enhancement of the septa and walls of the cystic area. Histopathology of a biopsy specimen showed fibroblastic proliferation, abundant giant cells, and focal hemosiderin deposition, which supported the diagnosis of a brown tumor. After removing the parathyroid adenoma, the brown tumor regressed and became sclerotic on radiographs.
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PMID:Brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism: radiological findings. 1711 Dec 72

Although the clinical symptoms of patients with benign parathyroid adenoma are usually nonspecific and benign, a malignant presentation of the benign disease may sometimes occur. Here, we report a case of a 58-year-old woman who presented with aggravated sacrum pain, general malaise, and polydipsia. Initial laboratory findings revealed hypercalcemia, normocytic anemia, and impaired renal function. Acute hypercalcemic crisis manifested and primary hyperparathyroidism was diagnosed together with myelofibrosis on account of the result of bone marrow biopsy. Excision of a parathyroid adenoma was performed, and the anemia and bone marker regressed later. These findings suggested that benign parathyroid adenoma may mimic the clinical presentation of parathyroid carcinoma, releasing excess parathyroid hormone and resulting in hyperparathyroid crisis. In addition, primary hyperparathyroidism can be associated with anemia and myelofibrosis.
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PMID:Benign parathyroid adenoma presenting with unusual parathyroid crisis, anemia and myelofibrosis. 1749 90

A case of giant cell lesion as an initial feature of primary hyperparathyroidism is presented. A 55-year-old woman reported with a complaint of swelling and occasional pain in the upper-left posterior region with 2 months' duration. The diagnosis of a giant cell lesion with primary hyperparathyroidism was confirmed on the basis of radiological, biochemical, and histopathological investigative procedures.
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PMID:Primary hyperparathyroidism presenting as multiple giant cell lesions. 1762 23

We present the clinical, laboratory, radiological and pathological findings in the case and review the literature. Our patient, a 37-year-old woman of short stature, was referred because of musculoskeletal pain. After primary evaluation, she underwent treatment with calcium and vitamin D supplement with the diagnosis of osteomalacia in Turner's syndrome. The rise of serum calcium during medical therapy, which was an unusual finding, attracted the clinician's attention to another underlying disorder. Further evaluation revealed primary hyperparathyroidism due to an adenoma of the parathyroid gland. Even though this is a rare diagnosis, its presence should be considered in any patient with Turner's syndrome presenting with severe osteoporosis and a rise in serum calcium during treatment.
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PMID:A rare association of hyperparathyroidism and Turner's syndrome--a case report. 1861 98

The pancreas is a retroperitoneal organ that releases water, bicarbonate and digestive enzymes by the main pancreatic duct (MPD) into the duodenum. Chronic pancreatitis (CP) is typically caused, in adults, by chronic alcohol abuse and, less frequently hypertriglyceridemia, primary hyperparathyroidism or cystic fibrosis. Exocrine dysfunction results in malabsorption of fat and subsequent steatorrhea. Damage to pancreatic endocrine function is a late finding in CP and results in hyperglycaemia or overt diabetes mellitus. Care of patients with CP principally involves management of pain. A significant change in the pain pattern or the sudden onset of persistent symptoms suggests the need to rule out other potential etiologies, including peptic ulcer disease, biliary obstruction, pseudocysts, pancreatic carcinoma, and pancreatic duct stricture or stones, then is important to establish a secure diagnosis. Management of pain should then proceed in a judicious stepwise approach avoiding opioids dependence. Patients should be advised to stop alcohol intake. Fat malabsorption and other complications may also arise. Management of steatorrhea should begin with small meals and restriction in fat intake. Pancreatic enzyme supplements can relieve symptoms and reduce malabsorption in patients who do not respond to dietary restriction. Enzymes at high doses should be used with meals. Treatment with acid suppression to reduce inactivation of the enzymes from gastric acid are recommended. Supplementation with medium chain triglycerides and fat soluble vitamin replacement may be required. Management of other complications (such as pseudocysts, bile duct or duodenal obstruction, pancreatic ascites, splenic vein thrombosis and pseudoaneurysms) often requires aggressive approach with the patient kept on total parenteral nutrition to minimize pancreatic stimulation.
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PMID:[Nutritional repercussions and management of chronic pancreatitis]. 1871 12

In recent years the advances in preoperative localization studies, the availability of intraoperative parathyroid hormone (PTH) assay and the introduction of cervicoscopy revolutionized the surgical treatment of primary hyperparathyroidism (PHPT). Several endoscopic and video-assisted techniques for parathyroidectomy have been described. In spite of the enthusiasm manifested by some authors, the role of these techniques with respect to the time-honored conventional surgery have been largely debated. Among them, video-assisted parathyroidectomy (VAP) has emerged as one of the leading and more diffuse techniques. To date many large and comparative studies have shown that VAP is an efficacious and feasible procedure with the same complication rate as conventional surgery. Moreover, VAP seems to have significant advantages in terms of cosmetic results, postoperative pain, recovery, and patient satisfaction. When compared with other minimally invasive techniques, it offers the significant advantages of being more similar to conventional surgery and reproducible in different surgical settings. Moreover, it permits bilateral neck exploration, associated thyroid resections and can be performed under locoregional anesthesia. All these characteristics and the excellent results obtained render VAP a valid and well-validated, and even preferable, alternative to conventional surgery for the surgical treatment of sporadic PHPT, especially in case of suspected single adenoma.
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PMID:Advantages of a video-assisted approach to parathyroidectomy. 1897 96

We report a 79-year-old Japanese woman who had primary hyperparathyroidism (HPT) with end-stage renal disease and severe bone changes. In 2004, she began to experience pain in her shoulders and knees, as well as muscle weakness and anorexia. She already had renal failure with a serum Cr of 4.7 mg/dl, while serum calcium was 9.6 mg/dl, PTH was 2,710 pg/ml, and serum alkaline phosphatase was 923 mU/ml. Multiple fractures of the pelvic bones and lumbar spine, osteoporosis, and subperiosteal bone resorption were detected. Although hemodialysis (HD) was started in February 2005, her symptoms became more severe. Total parathyroidectomy (PTX) and right iliac crest bone biopsy were performed. Histomorphometric analysis of the cancellous bone indicated a diagnosis of osteitis fibrosa, but a reduction of cortical bone and near absence of cancellous bone were also apparent. This showed that bone resorption by osteoclasts was predominant over bone formation by osteoblasts. Soon after PTX, her pain subsided completely. We conclude that primary HPT should be detected and treated early enough to avoid renal damage, since renal dysfunction markedly accelerates bone changes in patients with primary HPT.
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PMID:A case of primary hyperparathyroidism with severe bone and renal changes. 1925 17

We describe a case of delayed union in a tibial fracture secondary to primary hyperparathyroidism. A closed intra-articular proximal tibia fracture was stabilized with a hybrid external fixator. At 5 months clinical and radiological evaluation failed to demonstrate evidence of fracture healing. Fixation was stable and inflammatory markers ruled out infection. Further questioning revealed symptoms of anorexia, nausea and constipation. Plasma biochemistry showed an elevated corrected calcium and parathyroid hormone concentration. Further investigation included a sestamibi scan which confirmed a diagnosis of hyperparathyroidism secondary to a parathyroid adenoma. Six weeks following partial parathyroidectomy the fracture site was pain free, non-tender and the fracture had united radiologically. In cases of delayed-union, once an infective cause has been excluded with a mechanically stable fracture, other causes of delayed union like primary hyperparathyroidism should be ruled out.
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PMID:Primary hyperparathyroidism presenting as delayed fracture union. 1925 42

Primary hyperparathyroidism (PHPT) is characterized by the autonomous production of parathyroid hormone (PTH), in which there is hypercalcemia or normal-high serum calcium levels, in the presence of elevated or inappropriately normal serum PTH concentrations. Exceptionally, in symptomatic patients, a diagnosis can be established on the basis of clinical data. PHPT must always be evaluated in patients with clinical histories of nephrolithiasis, nephrocalcinosis, osseous pain, subperiosteal resorption, and pathologic fractures, as well as in those with osteoporosis-osteopenia on dual-energy X-ray absorptiometry (DEXA), a personal history of neck irradiation, or a family history of multiple endocrine neoplasia syndrome (types 1 or 2). Diagnosis of PHPT is biochemical. Asymptomatic hypercalcemia (total serum calcium corrected by albumin), without guiding signs or symptoms, is the most frequent manifestation of the disease. For the differential diagnosis, PTH(1-84) must be measured, as well as phosphate, chloride, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D and calcium-to-creatinine clearance. Suppressed or inappropriately low PTH1-84 guides the diagnose toward tumoral hypercalcemia and less frequently to granulomatous disease (sarcoidosis, tuberculosis, etc.), inadequate intake of 1alpha-hydroxyvitamin D or calcitriol, vitamin D or A intoxication, lithium intake, endocrinopathies (hyperthyroidism, Addison's disease, etc.) or treatment with thiazides, among other possibilities. Diagnosis of PHPT is confirmed by demonstrating persistent hypercalcemia (or normal-high serum calcium levels) in the presence of inappropriately normal or elevated serum PTH(1-84) concentrations, unless the urinary calcium-to-creatinine clearance ratio is lower than 0.01. In these cases, in the absence of thiazide intake or severe vitamin D deficiency, diagnosis should focus on benign familial hypercalcemic hypocalciuria. Parathyroid gland imaging is useful for localization of PHPT, but not for diagnosis of this entity.
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PMID:[Diagnostic evaluation and differential diagnosis of primary hyperparathyroidism]. 1962 56


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