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The case studies of four patients with post-transplantation calcinosis are presented. Three of the four patients died of inanition and sepsis secondary to infection of extensive soft tissue ulcers and diffuse cutaneous vascular calcification with gangrene. The fourth patient survived following removal of all four parathyroid glands and autografting of approximately one-half of one gland. Common to the patients was secondary hyperparathyroidism, elevated mean serum calcium levels after transplantation, and radiographic evidence of small and medium vessel calcification. No other differences could be found between these patients and other patients with post-transplantation hyperparathyroidism without calcinosis. In the face of apparently minor complaints of lower extremity discomfort, elevated parathyroid hormone levels (PTH) and positive xerography may indicate subtotal parathyroidectomy regardless of the serum calcium level.
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PMID:Lethal post-transplantation calcinosis. 37 90

We determined total plasma calcium levels in 44 critically ill patients without evidence of renal or hepatic failure, 25 of them with active systemic infection. Plasma Ca was 7.49 +/- 0.1 mg/dl in the septic group compared to 8.46 +/- 0.2 in non septic patients (p < 0.01). Corresponding levels of serum albumin were 2.26 +/- 0.1 and 3.05 +/- 0.2 milligrams, respectively (p < 0.01). Corresponding corrected plasma Ca levels were 9.19 +/- 0.1 and 9.39 +/- 0.1 mg/dl (NS). No correlation of lactate and plasma Ca levels was found. Mortality was 56% for septic and 16% for non septic patients. Patients with hypocalcemia had 53% mortality rate compared to only 16% in normocalcemic patients. PTH levels were normal in all but one patients, thus ruling out a rapid installation secondary hyperparathyroidism. Hypoperfusion does not appear related to ionic Ca levels given the lack of correlation of Ca and lactate levels. Mortality is related to sepsis and hypoalbuminemia but not to corrected Ca plasma levels.
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PMID:[Plasma calcium levels in critical patients with and without sepsis]. 184 18

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

Early diagnosis, adequate follow-up, and determination of the ideal moment for delivery are fundamental aspects of management of intrauterine growth retardation. This study evaluates the usefulness of cardiotocography in follow-up such cases and in the identification of the best moment for delivery. 57 cases of intrauterine growth retardation were studied at the Department of Perinatal Medicine of a Mexican Institute of Social Security hospital in Mexico City between July 1986-June 1987. The 57 cases were divided into 3 groups based on the results of the most recent nonstress test made 72 hours or less before delivery. Group 1 consisted of 15 cases with reactive tests, group 2 of 17 cases with nonreactive tests, and group 3 of 25 cases of nonreactive tests with decelerations. In group 1 there were no histories of previous complicated pregnancies. In group 2 there were 3 cases of low birth weight and 7 of preterm birth in previous pregnancies. In group 3 there were 3 cases of low birth weight, 2 of preterm birth in previous pregnancies. In group 3 there were 3 cases of low birth weight, 1 of preterm birth, 5 of stillbirth and 1 neonatal death. 77.1% of the 57 mothers were hypertensive, 5.2% had cardiopathies, and 1.7% each had lupus erythematosus, prolonged pregnancy, and hyperparathyroidism. There was no associated pathology in 12.2% of cases. There were 13 caesareans and 2 vaginal deliveries in group 1, with a fetal indication for cesarean in 3 cases. In group 2 there were 13 caesareans and 4 vaginal deliveries, with 9 maternal and 4 fetal indications for cesarean. 24 of 25 deliveries in group 3 were cesarean, with fetal indications in 22 cases and both fetal and maternal indications in 2 cases. The nonstress test was the basic criterion for the indication in group 3. The average birth weight and gestational age respectively were 1798 g and 37.2 weeks for group 1, 1681 g and 36.5 weeks for group 2, and 1551 g and 37 weeks for group 3. The average Apgar score at the minute of birth was 6.8 for group 1, 6.6 for group 2l, and 5.1 for group 3. There were 3 fetal deaths, 2 in group 2 including 1 case of anencephaly and 1 which occurred 48 hours after a recommendation for cesarean following a deterioration in the nonstress test. 1 death in group 3 followed a misinterpreted nonstress test. 4 neonatal deaths included 1 in group 1 from septicemia probably acquired in the nursery and 3 in group 3 attributed to cerebral hemorrhage. The results indicate that the presence of spontaneous decelerations in the fetal heart beat strongly suggest fetal compromise, and are an alarm signal in case of intrauterine growth retardation. The nonstress test is a useful tool in such cases.
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PMID:[Cardiotocography in intrauterine growth retardation]. 248 58

All patients with chronic renal failure have secondary hyperparathyroidism shown by elevated serum parathormone. Medical and surgical treatment is involves the use of phosphate binders, one alpha and increased frequency of dialysis. Surgery is indicated when medical treatment fails to control the Ca2+ PO4(2-) levels that activate renal osteodystrophy. High alkaline phosphatase and Ca2+ above 2.7 mmol/l are indications for surgery. Careful preoperative preparation and postoperative control minimise complications of haemorrhage, sepsis, tetany and cardiac arrhythmias. Long-term complications are hypoparathyroidism and recurrent hyperparathyroidism. Shortened dialysis periods may lead to increased parathyroid complications.
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PMID:Surgical treatment of secondary and tertiary hyperparathyroidism. 267 38

Critical surgical illness, commonly accompanied by shock, sepsis, multiple transfusions, and renal failure, is usually associated with low total calcium and/or low or normal ionized calcium. A seminal case of hypercalcemia in a surgical intensive care unit (SICU) patient prompted the review of 100 patients with longer than average SICU days (greater than 12) to determine the incidence, associated factors, and possible etiologies of this condition. Ten patients had elevated measured, and five others had elevated calculated, ionized calcium (5.9 +/- 0.25 mg%), an incidence of 15%. Compared to the 85 patients who did not develop hypercalcemia, this population had a significantly higher frequency of the following: renal failure, dialysis, total parenteral nutrition (TPN) usage greater than 21 days, bacteremic days greater than 1, transfusions greater than 24 units, shock greater than 1 day, SICU days greater than 36, and antibiotics used greater than 7. In addition, this group had significantly more days of hypocalcemia early in their hospital course. There was no difference in sex, age, mortality, or incidence of respiratory failure. Two patients studied in depth had renal failure requiring dialysis and no malignancy, milk-alkali syndrome, hyperthyroidism, or hypoadrenalism. Parathormone (PTH) concentrations were high normal or elevated (N terminal 20 and 21 pg/ml; C terminal 130 microliters Eq/ml and 1009 pg/ml) at the time of elevated calcium (total 9.2 to 14.6 mg%; ionized 4.9 to 8.2 mg%). Immobilization does not increase PTH. In renal failure, PTH elevation is a consequence of hypocalcemia rather than hypercalcemia. Moreover, five patients did not have renal failure. Shock, sepsis, and multiple transfusions containing citrate may lower total and/or ionized calcium and thus stimulate PTH secretion. Whatever the mechanism, approximately 15% of critically ill surgical patients develop hypercalcemia, which may represent a new form of hyperparathyroidism.
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PMID:Hypercalcemia in critically ill surgical patients. 393 94

Parathyroid carcinoma is a rare cause of hyperparathyroidism. Cure results from successful en bloc resection. However, because of its rarity, the malignant nature may not be appreciated at the initial operative procedure and as a result, definitive resection may not be accomplished. However, even with extensive en bloc resections, local recurrences do occur and patients die of metabolic derangements associated with hypercalcemia. Thus in addition to operative intervention, palliative chemotherapy may be required to control the hypercalcemia. Radiotherapy has been unsuccessful. A single case of nonfunctioning parathyroid carcinoma responding to treatment with methotrexate, Adriamycin, cyclophosphamide, and CCNU has been reported. We report a case of recurrent functioning parathyroid carcinoma treated with dacarbazine (DTIC) in which biochemical and pathologic evidence of at least a partial response was seen. The patient, a 33-year-old woman, had undergone five previous neck explorations during a 26-month period for aggressive locally recurrent disease. Before DTIC therapy the intact parathyroid hormone (PTH) level was 1032 pg Eq/ml (normal 163 to 347 pg Eq/ml) and the serum calcium level was 16.8 mg/dl (normal 8.8 to 10.0 mg/dl). After a course of DTIC there was a marked improvement in her clinical status and biochemical parameters (intact PTH 545 pg Eq/ml; serum calcium 11.8 mg/dl). For 2 months her condition stabilized, with PTH levels between 700 and 760 pg Eq/ml and serum calcium levels between 10.2 and 16.0 mg/dl. With a slowly progressive rise in biochemical parameters a second course of DTIC was initiated and a marked drop in serum calcium levels (5.7 mg/dl) occurred, but PTH levels remained unchanged. A progressive course of septicemia, malnutrition, and disseminated intravascular clotting ultimately lead to her death 4 weeks later. At autopsy examination the tumor was confined to the neck. Grossly and microscopically there was extensive central as well as peripheral necrosis of the tumor, which was thought to be the result of the cytotoxic effect of DTIC. From this experience and because of the grim prognosis in patients with recurring parathyroid carcinoma, it may be that aggressive use of chemotherapy with DTIC early in the course of treatment should be considered.
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PMID:Parathyroid carcinoma: biochemical and pathologic response to DTIC. 650 66

Between August 1975 and January 1981, 106 patients thought to have persistent or recurrent hyperparathyroidism underwent a total of 108 parathyroid re-explorations at the National Institutes of Health. These 106 patients had a total of 175 previous operations for hyperparathyroidism (156 cervical and 19 mediastinal). Nephrolithiasis (54% of patients) and bone disease (24% o patients) were the predominant symptoms. Arteriographic examination and selective venous sampling provided highly accurate localizing results in 33% of the patients, and were of some help in 64%. The final diagnoses after reoperation and re-evaluation were: single-gland disease in 58 patients, primary nonfamilial hyperplasia in 19 patients, familial hyperplasia in three patients, multiple endocrine neoplasia (MEN) Type I in ten patients, MEN Type II in two, parathyroid carcinoma in four patients, secondary hyperplasia in three patients, and familial hypocalciuric hypercalcemia (FHH) in two patients. The diagnosis was in doubt in five patients. In the 95 patients with unequivocal hyperparathyroidism, not due to parathyroid carcinoma, surgery eliminated hypercalcemia in 91 (96%). Two patients died after operation, one of disseminated candidiasis, and one patient, with an immunodeficiency, of sepsis. Five patients developed temporary, and one permanent, recurrent nerve damage; 41% of the patients were hypocalcemic, at the time of discharge from the hospital.
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PMID:Results of reoperation for persistent and recurrent hyperparathyroidism. 730 78

Systemic calciphylaxis is a rare, poorly understood syndrome of progressive ischemic necrosis, usually associated with hyperparathyroidism. The combination of hyperparathyroidism, usually secondary or tertiary, and chronic renal failure seems to produce a particular biochemical environment conducive to the development of an unusual progressive form of ischemia. Early recognition and diagnosis, appropriate therapy including wound care, and expeditious parathyroidectomy often will halt the progression of the disease, minimize limb loss, and prevent subsequent sepsis and death.
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PMID:Systemic calciphylaxis. 811 2

A 46-year-old male patient underwent long-term hemodialysis treatment had suffered from calciphylaxis (defined by Selye), such symptoms as advanced systemic vascular calcification, rapid progression of gangrene on both fingers and toes, disturbance of consciousness, and sclerosis and obstruction of the superficial vein after venipuncture during 11.5 years of dialysis. Furthermore, he had a long history (30 years) of heavy smoking. He died as a result of sepsis due to pneumonia after 12.5 years of dialysis. He had received dialysis treatment using a small amount of dialysate (50 liters on a recirculating system) for 8.5 years and had been dialysed 2 and 2 or 3 times a week for 10 years. As a result of this insufficient dialysis treatment, his characteristic laboratory data showed hypocalcemia, hyperphosphatemia, elevated calcium-phosphorus product, advanced metabolic acidosis, hyperalkaliphosphatemia and elevated serum parathyroid hormone. Autopsy revealed the following: 1) enlargement parathyroid gland enlarged in two (4.0 g and 2.0 g, respectively) showing adenomatous hyperplasia presenting cord-like arrangement of chief cells and water-clear cells, 2) systemic medial calcification in radial, ulnar, renal, mesenteric and brain arteries, and 3) Berline-blue positive iron deposit in calcified arteries in mesenteric and parathyroid tissue. From these results, we concluded that factors (challengers) related to the appearance of calciphylaxis might be as follows: 1) advanced secondary hyperparathyroidism, 2) long-term uremic state, 3) administration of VD2 and VD3, 4) iron salt injection, and 5) a long history of heavy smoking. We speculated that these challengers might act synergistically to cause calciphylaxis.
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PMID:[A long-term hemodialysis patient complicated with systemic calciphylaxis]. 823 Aug 23


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