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Query: UMLS:C0033774 (pruritus)
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Calciphylaxis is a rare, severe complication of secondary hyperparathyroidism. Patients present with painful, violaceous, mottled skin lesions of the upper and lower extremities, which become necrotic and produce nonhealing ulcers. Gangrene of fingers and toes frequently requires amputation, produces nonhealing wounds, and can lead to sepsis and death. We reviewed the clinical course of five patients with calciphylaxis treated in our institution. The three men and two women (aged 47 to 72 years) had secondary hyperparathyroidism from chronic renal failure. All patients had severe pruritus, painful ulcers, and severe hyperphosphatemia with elevated serum calcium-phosphate product (greater than 12 mmol2/L2), but the serum parathyroid hormone levels were only moderately elevated. Most patients had medical calcification of medium and small blood vessels, and some had soft-tissue calcification visible on roentgenography. Treatment consisted of local wound care, antibiotics, phosphate-binding agents, and parathyroidectomy. Two patients died of uncontrollable sepsis. The three survivors had dramatic improvement of pain and ulcers after parathyroidectomy. Calciphylaxis is a limb- and life-threatening complication of secondary hyperparathyroidism. Diagnosis can be made by recognizing the characteristic painful skin lesions, ulcers, and gangrene of the digits, and patients should be treated with subtotal parathyroidectomy.
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PMID:Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy. 192 21

In this randomised study subtotal parathyroidectomy (PTX) is being compared with total parathyroidectomy including autotransplantation (PTX + AT) in 40 patients with secondary hyperparathyroidism. Both groups were followed up twice, first 19 +/- 6 months (PTX + AT) respectively 19 +/- 7 months (subtotal PTX) and secondly 43 +/- 9 respectively 40 +/- 7 months after surgery. In each group there were 17 patients alive at the time of the second postoperative follow-up. In the meantime 2 patients with subtotal PTX required reoperation because of recurrences originating from the remaining parathyroid tissue, another 2 patients of this group were hypercalcaemic. After PTX + AT serum-calcium normalized significantly more often (p less than 0.03), a corresponding result was seen in alkaline phosphatase. Reoperation was not required in this group. Also radiological alterations reformed significantly more often after PTX + AT, so did clinical signs like pruritus (p less than 0.005) and muscular weakness (p less than 0.04). Considering these results and the fact that in case of recidive a reoperation of the autograft in the forearm is much easier to perform than a reoperation in the neck, PTX + AT is recommended as method of choice in surgical treatment of sHPT.
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PMID:[Subtotal parathyroidectomy versus total parathyroidectomy with autotransplantation in secondary hyperparathyroidism. A randomized study]. 203 94

The appropriate use of phosphate binders, calcium supplements and especially calcitriol therapy has significantly decreased the incidence of overt secondary hyperparathyroidism in dialysis patients. Nevertheless some patients may still need parathyroidectomy, especially in the event of severe clinical signs and symptoms such as persistent hypercalcemia, pruritus, calcifilaxis, or extensive extra-skeletal calcification. Since aluminum-induced bone disease may resemble hyperparathyroidism in dialysis patients, whenever parathyroidectomy is contemplated the diagnosis of secondary hyperparathyroidism must be firmly established. Thus, a bone biopsy is mandatory prior to parathyroidectomy. It is our experience that once the patient goes to surgery the most important factor in the surgical approach is the presence of a skilled surgeon who has extensive experience in parathyroid gland surgery. The data comparing subtotal parathyroidectomy with total parathyroidectomy and autotransplantation are similar. The most important shortcoming is the lack of long-term follow-up. Recently, new data by several investigators has been advanced reintroducing total parathyroidectomy. Long-term observations in patients who despite total parathyroidectomy still have normal PTH levels are of special interest. In addition, long-term follow-up of these patients has shown that normal plasma calcium and phosphorus levels may be maintained without the use of Vitamin D; this occurred in the presence of active mineralization. However, our major objection to this procedure is the risk of aluminum-induced bone disease. At the present time we feel that the relative high incidence of recurrent hyperparathyroidism following subtotal parathyroidectomy is a reasonable trade off for the risk of aluminum bone disease which may develop in absence of PTH.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Parathyroidectomy in chronic renal failure: indications, surgical approach and the use of calcitriol. 221 49

Recently, the role and timing of surgery for treating secondary and tertiary hyperparathyroidism (HPT) have been questioned. In order to delineate the indications for surgery in these patients, a retrospective analysis of 53 consecutive patients treated with parathyroidectomy was conducted. Subtotal thyroidectomy was done in 37 of 45 patients undergoing their initial operations for HPT. Eight additional patients were referred after failed operations. Of 33 patients with preoperative bone pain, 70% improved. Joint pain improved in 87% of 30 patients, pruritus improved in 81% of 27 patients, and preoperative malaise improved in 73% of 33 patients after parathyroidectomy. Abdominal pain and irritated eyes were unlikely to improve. The best predictors of a successful outcome were a markedly elevated preoperative immunoreactive parathyroid hormone (mid-region) level and an elevated alkaline phosphatase level. There were no perioperative deaths. One patient (1.6%) had a recurrent laryngeal nerve injury, and one patient required reoperation for a neck hematoma. No patient had permanent hypoparathyroidism, but transient hypocalcemia (less than 7 mg/dL) occurred in 22%. Postoperative hypocalcemia correlated with elevated preoperative alkaline phosphatase levels (r2 = 0.247).
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PMID:Results of surgical treatment for hyperparathyroidism associated with renal disease. 222 Dec 30

Somatic symptoms are common in patients on dialysis. Their causes are largely unknown and their therapy is unsatisfactory. To examine the relationship of psychological and clinical factors to these symptoms, 191 interviews were done in patients on hemo- and peritoneal dialysis. The severity of 8 somatic symptoms (tiredness, sleep disturbance, cramps, pruritus, headache, nausea, dyspnea, joint pain) of importance in dialysis patients was measured using previously validated scales. Indices of affect and quality of life were obtained, as was demographic, clinical and laboratory information. The severity of each symptom was significantly related to the indices of affect and quality of life. Using multiple logistic regression, poor affect score was the strongest correlate of each of the following somatic symptoms, tiredness, pruritus, sleep disturbance and cramps. It was ahead of any clinical or demographic variable and was also significantly correlated with the severity of the other symptoms. Indices of hyperparathyroidism were significantly associated with headache, joint pain, dyspnea and nausea. We conclude that the strongest correlate of common somatic symptoms in dialysis patients is affect disturbance, and that therapy aimed at improving the affect may improve the symptoms.
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PMID:Clinical and psychological correlates of somatic symptoms in patients on dialysis. 235 74

A retrospective analysis was undertaken to better define the clinical presentation and therapy of patients with secondary hyperparathyroidism and the timing of surgical removal of the parathyroid glands in secondary hyperparathyroidism. Sixty-eight patients with end-stage renal disease (ESRD) underwent 74 parathyroid procedures over a 10-year period. There were 38 per cent women and 62 per cent men in this population--the same proportion of men and women with ESRD. These proportions are reversed when compared with patients with primary hyperparathyroidism. The mean time between initiation of dialytic therapy and parathyroidectomy was 5.4 years. Preoperative mean serum calcium and phosphorus levels were 10.1 +/- 0.2 and 6.1 +/- 0.2 mg/dl, respectively. All patients were symptomatic and 60 per cent of the patients had at least two symptoms before surgery. Renal osteodystrophy was the most common symptom (74%), whereas pruritus was noted in 65 per cent of this population. Patients underwent either subtotal (88%) or total (12%) parathyroidectomy with autotransplantation. Six patients required reoperation: five for recurrent disease (2 to 5 years after the initial surgery) and one for persistent disease. All patients were symptomatically improved after the surgery. Complications included the following: transient hoarseness (7%), hypocalcemia requiring calcium, and/or vitamin D therapy (32%) for 6 months after surgery. This analysis demonstrates that although the initial therapy of secondary hyperparathyroidism is medical, surgical therapy should be instituted before multiple symptoms develop in the ESRD population.
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PMID:Secondary hyperparathyroidism. The role of surgery. 272 73

During the period from December 1984 to December 1986, ten patients with end-stage renal disease were evaluated with hyperparathyroidism refractory to medical management. Symptoms were bone pain in eight patients and pruritus and constipation in two. Two patients with bone pain also had impaired mentation. Biochemical preoperative assessment revealed calcium levels between 8.7 and 11.2 milligrams per deciliter, with a median of 10.5 milligrams per deciliter. Phosphate levels were normal in all but three patients, and the phosphate-calcium product was greater than 80 in two. Parathyroid hormone levels assessed with the radioimmunoassay method were elevated in all patients, and results of ultrasound of the neck, done in seven patients, revealed hyperplastic glands in six patients and normal glands in one patient. All patients underwent surgical exploration of the neck with removal of four parathyroid glands and immediate autotransplantation. No complications related to the operative procedure occurred. Postoperative calcium levels ranged between 6.5 and 9.5 milligrams per deciliter on the first postoperative day and normalized by the sixth day. Four patients experienced symptomatic hypocalcemia requiring intravenous calcium supplementation for one to six days postoperatively. The mean hospital stay was four days (three to seven days). All patients had histologically confirmed four gland parathyroid hyperplasia. Marked improvement of symptoms was accomplished in all patients after a period of three to 30 days. One patient required revision of the forearm parathyroid transplant after four months. One patient required calcium supplementation taken orally, two patients required 1,25-Dihydroxyvitamin D3 and five required treatment with both. Two patients did not require any further medical treatment, and no patient had recurrent symptoms after a mean follow-up period of eight months. We recommend total parathyroidectomy with autotransplantation in patients with end-stage renal disease as a safe and effective procedure for symptomatic refractory hyperparathyroidism. Symptom relief can be accomplished in the vast majority of patients.
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PMID:Surgical treatment of the parathyroid gland in patients with end-stage renal disease. 338 Nov 85

Between 1978 and 1984, 19 patients at Royal Perth Hospital (RPH) underwent parathyroidectomy for secondary (renal) hyperparathyroidism. This represented 6.0% of the overall dialysis population treated at RPH during this period of time. The mean duration of pre-operative dialysis for these 19 patients was 48 months, compared with a mean duration of 30 months for the overall dialysis population. The principal indications for parathyroidectomy were symptomatic hyperparathyroid bone disease (10), hypercalcaemia (six), progressive lower limb ischaemia (two) and painful peri-articular calcification (one). The complications of chronic renal failure that were most consistently improved by parathyroidectomy were the clinical, radiological and biochemical manifestations of hyperparathyroid bone disease and hypercalcaemia. Features such as pruritus, soft tissue calcification, vessel wall calcification and peripheral ischaemia responded less predictably. Hyperparathyroid bone disease and hypercalcaemia remain the principal indications for parathyroidectomy in chronic renal failure. Profound postoperative hypocalcaemia was the major early postoperative management problem (seven patients) and was closely linked with the severity of pre-operative hyperparathyroid bone disease. It was also seen more frequently in those patients undergoing total parathyroidectomy with immediate autotransplantation of parathyroid tissue (TP-A), than in those in whom residual parathyroid tissue was left in situ (subtotal parathyroidectomy or STP). Recurrent hyperparathyroidism (four patients) was the major late postoperative complication, but was more frequently the result of a supernumerary or previously overlooked fourth parathyroid gland (three), than due to hyperplasia of residual parathyroid tissue (one). STP and TP-A were equally effective in controlling or reversing renal hyperparathyroidism, but the former was associated with a lower incidence of postoperative management problems and should be the preferred operation in this group of patients.
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PMID:Parathyroidectomy in chronic renal failure. 345 32

A syndrome of intrahepatic cholestasis leading to death in early childhood was studied in 16 Greenland Eskimo children. The pedigrees are compatible with autosomal recessive inheritance. Jaundice, bleeding, pruritus, malnutrition, steatorrhoea, osteodystrophy and dwarfism were typical clinical features. Eight had died between the ages of six weeks and three years due to bleeding or infections. Hyperbilirubinaemia, profound hypoprothrombinaemia, thrombocytosis and elevated alkaline phosphatase levels were evident. Serum calcium, phosphate and parathyroid hormone levels indicated a secondary hyperparathyroidism. Hepatic fibrosis developed with increasing age. Follow-up of the surviving patients was 4 to 30 months. The aetiology of the disease is unknown. The syndrome has some features in common with previously described patients with familial intrahepatic cholestasis. No specific treatment is available. Genetic counselling is essential.
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PMID:Fatal familial cholestatic syndrome in Greenland Eskimo children. 356 58

This study was undertaken to determine the success of surgical treatment of advanced secondary (renal) hyperparathyroidism. From 1978 to 1985, total parathyroidectomy and autotransplantation (TPA) were performed for secondary hyperparathyroidism in 23 patients who had had dialysis for a mean of 6.5 years preoperatively. Indications for surgery included hypercalcemia, bone pain and pathologic fractures, metastatic calcification, and pruritus. Four glands were found and removed in all patients; 100-150 mg of diced tissue were autotransplanted to one forearm. Two patients died of myocardial infarction in the first postoperative week. Bone pain, present in 19 of 23 patients, was relieved almost immediately postoperatively and relief was sustained to death (of unrelated causes) or most recent follow-up in 13 patients. All fractures healed. All patients had markedly elevated serum parathormone (PTH) preoperatively and 14 of 23 were hypercalcemic. The group mean values of serum calcium, alkaline phosphatase, and PTH all fell to and remained in a normal range by 1 year postoperatively in that subset of patients who did not suffer recurrence. Six patients were reoperated on after 12 to 37 months with partial graft excision for recurrent bone pain and hypercalcemia. Bone pain in two of these patients was due to aluminum-associated bone disease and the diagnosis of recurrent secondary hyperparathyroidism was erroneous. The actual recurrence rate was thus 19 per cent. Consistent technical success, with no late hypocalcemia, was achieved and most patients were restored to medical manageability.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:A community hospital experience with total parathyroidectomy and autotransplantation for renal hyperparathyroidism. 368 58


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