Synovial cysts of the hips in seronegative arthritis

Calciphylaxis is a rare condition of widespread calcification of tissues and blood vessels with accompanying vascular thrombosis and ischemic necrosis. Most cases develop in association with hyperparathyroidism in patients with chronic renal failure. Pseudoxanthoma elasticum (PXE) is a hereditary condition of abnormal elastic tissue structure that leads to widespread abnormalities of the skin, retina, and visceral organs. Histologic changes of PXE have been observed as coincidental findings in several conditions such as following trauma to the skin manifest as isolated plaques often in scars. We observed histologic findings of PXE in a patient with chronic renal failure who developed fatal calciphylaxis. Complete evaluation failed to reveal evidence of systemic findings of PXE. Histologic changes of PXE may be seen in patients with calciphylaxis as a coincidental finding. Rapidly developing soft tissue calcification may lead to the expression of the characteristic histopathologic findings of PXE without evidence of classic clinical manifestations of PXE. Calciphylaxis should be added to the list of disorders that may lead to microscopic PXE-like changes.     

Calcific cerebral embolism in systemic calciphylaxis

Calciphylaxis represents a rare complication of end-stage renal disease with hyperparathyroidism. We report the case of a 26-year-old woman with systemic calciphylaxis secondary to chronic renal failure who developed mitral annular calcification and a right middle cerebral artery stroke. The high-density lesion seen on CT scan of the brain probably represents a calcified cerebral embolus originating from the mitral valve.     

Calciphylaxis in man. A syndrome of tissue necrosis and vascular calcification in 11 patients with chronic renal failure

Eleven patients with chronic renal failure and presumed secondary hyperparathyroidism developed a syndrome of medial calcinosis of the arteries and painful ischemic ulcers of the fingers, legs, or thighs, or any combination of the three. Five patients required maintenance hemodialysis; six had functioning renal homografts. Severe hyperphosphatemia had existed in each; seven showed roentgenographic evidence of subperiosteal resorption. Similarities are evident between the lesions and experimentally produced calciphylaxix. The lesions demonstrated a relentless, progressive course, with serious morbidity and mortality. Hyperplastic or adenomatours parathyroid tissue was removed from ten of 11 patients unergoing surgical procedures; healing followed in seven patients. Treatment with phosphate-binding antacids to lower serum phosphorus levels may prevent this syndrome. Total or subtotal parathyroidectomy should be considered when ischemic skin lesions appear in uremic patients or in renal transplant recipients.     

Calciphylaxis mimicking dermatomyositis: ischemic myopathy complicating renal failure

BACKGROUND: Among the complications of chronic renal failure is a syndrome of medial calcification of small- to medium-sized arteries associated with ischemic necrosis of the skin and other organ systems, leading to gangrene and a poor prognosis. The syndrome has been reviewed in the renal, dermatologic, and surgical literature under the term calciphylaxis, which describes a postulated pathogenetic mechanism whereby sensitization to an endogenous or exogenous substance (such as parathyroid hormone) predisposes to calcium deposition after exposure to a challenging agent. Myopathy has rarely been reported as the presenting feature, and the syndrome has not been discussed in the neurologic literature. METHODS: We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myopathic complaints and signs suggesting dermatomyositis. We also discuss possible disease mechanisms and treatment. CONCLUSIONS: Because early treatment (including aggressively lowering the calcium and phosphate levels and parathyroidectomy) may improve the outcome, early recognition of the syndrome of calciphylaxis is essential.     

Preoperative localization of parathyroid lesions in hyperparathyroidism: relationship between technetium-99m-MIBI uptake and oxyphil cell content

The aim of this study was to assess the relationship between parathyroid oxyphil cell content and early or late phases of uptake of 99mTc-MIBI, a radioisotope preferentially retained in mitochondria-rich cells. METHODS: This study is a retrospective, single-blind analysis of all double-phase 99mTc-MIBI parathyroid scintigraphy studies performed before surgery in our institution between 1990 and 1995. A total of 18 parathyroid lesions in 14 patients were reviewed. This sample included 11 cases of primary hyperparathyroidism (8 adenomas, 1 adenocarcinoma and 2 hyperplasias) and 3 cases of tertiary hyperparathyroidism secondary to chronic renal failure. RESULTS: Uptake of 99mTc-MIBI in the early phase of scintigraphy was associated with larger parathyroid lesions (1.61 +/- 1.61 ml versus 0.33 +/- 0.27 ml; p < 0.02) and higher serum calcium levels (3.00 +/- 0.41 mM versus 2.67 +/- 0.14 mM; p < 0.02). More importantly, we found that a parathyroid oxyphil cell content greater than 25% was more often associated with a positive uptake of 99mTc-MIBI in the late phase of the test (positive late uptake in 78% of lesions with a high oxyphil cell content versus 33% in lesions with an oxyphil cell content between 1% and 25% and 0% in lesions with no oxyphil cells; p < 0.04). CONCLUSION: These findings suggest that the late retention of 99mTc-MIBI in double-phase scintigraphy is related to parathyroid oxyphil cell content.     

D-penicillamine-induced myasthenia gravis in a case of eosinophilic fasciitis

During the period 1983-1995, 200 chronic renal failure patients (115 males and 85 females) were parathyroidectomized for hyperparathyroidism in our Department. In all of them, the presenting clinical symptoms, physical signs, biochemical and radiological tests were typically those of hyperparathyroidism. One hundred ninety patients were operated for the first time whereas 10 were re-operated due to relapse of the disease; 3 of these cases were primary hyperparathyroidism, 182 secondary and 5 tertiary. All three primary hyperparathyroidism cases underwent removal of the adenoma; in the group of secondary hyperparathyroidism, 50 underwent removal of all the parathyroid glands found, 25 underwent total parathyroidectomy with forearm or deltoid autograft and 60 subtotal parathyroidectomy whereas in 39 and 8 patients only 3 and 2 parathyroid glands were found respectively. In the group of tertiary hyperparathyroidism, we removed only the hyperplastic gland detected as the operative detection of the rest was not possible. Ten cases were re-operated for removal of the remaining glands. No complications were noted postoperatively, apart from severe hypocalcemia in 20 cases, treated successfully by Calcium and Vitamin D administration. The highest relapse rate was noted among the 8 patients with only the 2 parathyroid glands removed. It seems that total or subtotal parathyroidectomy represents the most successful methods for surgical treatment of hyperparathyroidism complicating chronic renal failure.     

Do children with repaired low anorectal malformations have normal bowel function?

To determine the association of Epstein-Barr virus (EBV) with primary cutaneous amyloidosis (PCA), a retrospective study was conducted on skin tissue from 27 Chinese patients with lichen amyloidosus and macular amyloidosis. In situ hybridization with oligonucleotide probes was used to detect the expression of EBV-encoded RNAs (EBERs). Eleven of 27 cases (40.7%) were found to contain the EBV genome. No EBV genome was detected in the skin of the control groups, including three cases of secondary cutaneous amyloidosis, two cases of primary systemic amyloidosis, and four cases of lichen simplex chronicus. Our study showed no correlation between the presence of EBV in PCA patients and the patients' age, sex, clinical type or severity of the skin lesions. Although our results suggest that EBV may be associated with some cases of PCA, the true aetiological role of EBV in PCA remains unknown.     

Are we mismanaging calcium and phosphate metabolism in renal failure?

Secondary hyperparathyroidism and renal osteodystrophy are the consequences of abnormal calcium, phosphate, and calcitriol metabolism ensuing from renal failure. Evidence suggests that calcium balance tends to become negative as we grow older than 35 years of age; however, the current dialysis modalities provide patients regardless of age with excessive calcium during dialysis. Administration of calcitriol in the management of hyperparathyroidism further increases the calcium and phosphate absorption. Furthermore, the current thrice-weekly renal replacement therapies fail to remove the daily absorbed phosphate, and we have to use calcium carbonate as a primary phosphate-binding agent to reduce intestinal phosphate absorption. The large calcium mass transfer and phosphate retention could lead to soft tissue calcification, especially in older end-stage renal disease (ESRD) patients. Consequently, only by maintaining a negative calcium balance during renal replacement therapy can we safely use calcitriol and calcium carbonate for the management of secondary hyperparathyroidism. Recent studies have indicated that phosphate restriction alone independent of plasma calcitriol or calcium can lower plasma parathyroid hormone (PTH) in renal failure and prevent hyperplasia of parathyroid glands. Therefore, phosphate control perhaps is the most important means to prevent secondary hyperparathyroidism. Previous studies have shown that ferric compounds are potent phosphate-binding agents; hence, these compounds warrant further trial in the management of phosphate metabolism in renal failure.     

Symmetrical peripheral gangrene

Two cases of symmetrical peripheral gangrene associated with intracardiac lesions are described. One had a secondary deposit from a uterine leiomyosarcoma in the wall of the right ventricle, the other had a free ball thrombus in the right atrium; there were no valvular lesions in either case. The published reports of this type of gangrene indicate that it may result from (1) va sospastic conditions, (2) small vessel obstruction, or (3) conditions producing a very low cardiac output. The two patients reported are unusual examples of the last.     

Systemic calciphylaxis revisited

A syndrome characterized by rapidly progressive ischemic necrosis involving large areas of the skin and muscle, and by peripheral gangrene associated with extensive vascular calcifications was observed in a patient with end-stage renal failure on chronic hemodialysis. In an effort to control the disease, parathyroidectomy was performed which resulted in rapid improvement of tissue perfusion. However, the patient eventually died from sepsis within 2 months after admission. This case presents the typical features of the syndrome of systemic calciphylaxis. The literature is reviewed searching for similar cases of this poorly recognized, but life-threatening, clinical syndrome. The pathogenesis, clinical manifestations, and therapy of this unusual and rapidly progressive, but potentially reversible, condition are reviewed with emphasis on its prompt recognition and appropriate management.     

Retinoic acid and its receptors

We report on a patient presenting with cutaneous leucocytoclastic vasculitis in association with sarcoidosis. Detailed investigations did not show evidence of infectious or neoplastic diseases. Vasculitis was therefore considered to be secondary to sarcoidosis. A review of 4 other previously reported cases reveals a common presentation with the association of vasculitic skin lesions with deep cutaneous nodules, mediastinal lymph nodes and arthritis. The role of antibody response in leucocytoclastic vasculitis is discussed.     

Parathyroidectomy after renal transplantation: a retrospective analysis of long-term outcome

BACKGROUND: Advanced hyperparathyroidism refractory to active vitamin D continues to be a problem and frequently forces the nephrologist to resort to parathyroidectomy. One particular aspect is persisting advanced hyperparathyroidism after renal transplantation. Published information on this point is fragmentary. DESIGN: Retrospective analysis. PATIENTS: Between 1983 and 1995 a total of 456 patients with renal secondary hyperparathyroidism were subjected to parathyroidectomy (PTX) of whom 103 were transplanted or had at least a history of renal transplantation. The present analysis concerns 37 patients who had a functional renal graft at the time of PTX and were followed for up to 13 years. PTX was performed after an average of 36.7 months after renal transplantation. OUTCOME: Thirteen patients experienced rejection and became dialysis-dependent. Twenty-four patients had stable function of the renal graft. Seven patients died during follow-up. Hypoparathyroidism post-PTX developed in 4/37 patients, but could be overcome by replantation of cryoconserved parathyroid tissue. FREQUENCY ESTIMATE: A total of 2632 renal transplants were performed in the catchment area. As a minimum estimate 3.91% of patients with a functional graft required PTX. RECOMMENDATION: Parathyroidectomy should be considered early in cases with advanced secondary renal hyperparathyroidism, since renal transplantation does not necessarily guarantee reversibility of parathyroid overactivity.     

Medical education on the World Wide Web

(Report of four cases and review of the literature) Calcification of the myocardium is a rare condition. The cause may be dystrophic or metastatic. An autosomal recessive inherited idiopathic arterial calcification of infancy is more rare abnormality. A dystrophic calcification is the more common of the three and may occur in areas of necrosis, hemorrhage, or fibrosis of the myocardium. Metastatic calcification is associated with hyperparathyroidism, D hypervitaminosis or renal failure, usually accompanied by the deposit of calcium in other organs, particularly the lungs, stomach, kidneys, spleen and liver. Authors report four cases of myocardial calcification diagnosed in intrauterine life. They give a review of literature of fetal and neonatal myocardial calcification.     

Pityriasis rotunda

Pityriasis rotunda is an uncommon cutaneous disorder consisting of asymptomatic, strikingly circular, scaly lesions on the trunk and extremities, with the histologic appearance of ichthyosis vulgaris. Previous cases have been described in Japanese, South African, and West Indian persons, usually in association with an underlying serious systemic disease. A case of pityriasis rotunda in a 32-year-old black man in whom no diagnosable disorder could be demonstrated is presented.     

Long-term follow-up after subtotal parathyroidectomy in patients with renal failure

OBJECTIVES/HYPOTHESIS: The most appropriate type of surgery for hyperparathyroidism secondary to renal failure remains controversial. We report a 5-year experience of patients with hyperparathyroidism secondary to end-stage renal disease who underwent subtotal parathyroidectomy. We believe that this is the procedure of choice, offering several advantages over total parathyroidectomy with and without reimplantation. STUDY DESIGN: Retrospective review. METHODS: Review of 14 consecutive renal failure patients who underwent subtotal parathyroidectomy by one surgeon (A.K.) was performed. Follow-up ranged from 4 to 54 months. All patients were receiving chronic maintenance dialysis. All patients came to surgery with clinical symptoms of parathyroid bone disease, elevated serum calcium levels (10.1-12.4 mg/dL), and intact parathyroid hormone levels (619-4160 pg/mL), despite maximal medical therapy. At exploration four glands were identified in all patients and three and a half were removed. RESULTS: All patients experienced symptomatic relief postoperatively with normalization or near-normalization of serum calcium concentration and intact parathyroid hormone concentrations. One patient developed recurrent disease 4 months after surgery, and on re-exploration a supernumerary substernal gland was identified. A second patient developed recurrent symptoms 4 years after surgery and at the time of this writing was awaiting re-exploration. CONCLUSIONS: All patients had either resolution of or marked improvement in their subjective complaints. There have been no cases of permanent hypoparathyroidism. We believe that subtotal parathyroidectomy is the best procedure for patients with refractory symptoms of secondary hyperparathyroidism.     

Indications for parathyroidectomy and extent of treatment for patients with secondary hyperparathyroidism

Some manifestations of secondary hyperparathyroidism affect most if not all patients with chronic renal failure and can affect many different organ systems. Proper medical treatment is essential and should be attempted before considering surgical intervention. The symptoms that most often resolve after parathyroidectomy include bone pain and intractable pruritus. Other useful indications for operation include a marked elevation of the parathyroid hormone level and the elevation of the calcium x phosphate product over 70. Both subtotal parathyroidectomy and total parathyroidectomy with autotransplantation have been advocated as the best operative approach. Each of these procedures has its own advantages and disadvantages which should be considered for each individual case. Localizing procedures should be reserved for patients with persistent or recurrent hyperparathyroidism, as diffuse parathyroid hyperplasia is the most common operative finding in secondary hyperparathyroidism.     

Skeletal lesions of the feet in diabetics and their relationship to cutaneous erythema with or without necrosis on the feet

Seventy patients with cutaneous erythema of the feet with or without necrosis were the subjects of this investigation. Sixty-five of them had open diabetes. The glucose tolerance of the remaining five patients was altered in a diabetic direction. Twenty-seven of the 70 patients had roentgenologically demonstrable destruction in the bones of the feet. These 70 patients were compared with 61 diabetic control patients of corresponding age and duration of diabetes but without these skin lesions of the feet. Only four of the 61 control patients had destruction in the bones of the feet and all these destructions were small. Precipitating factors were identified in general for the skin lesions, the most common being cardiac decompensation. A higher frequency of precipitating factors was seen in patients with skeletal destructions than in those without. The skeletal destructions and cutaneous necrosis are supposed to be equivalent lesions, localized to different tissues in the feet. When patients presenting skin lesions of the feet in the form of distal gangrene were compared with those who had cutaneous erythema and necrosis of the feet, but no distal gangrene, no differences were found with respect to age, duration of diabetes, occurrence of precipitating factors and the occurrence of skeletal destruction. Cutaneous erythema without necrosis is understood to be incipient diabetic gangrene.     

Case report: acute renal vein thrombosis in patients with spinal cord injury and secondary amyloidosis

Chronic spinal cord injury, when complicated by chronic suppurative infections, has replaced chronic tuberculosis as a leading cause of secondary amyloidosis. Renal involvement with secondary amyloidosis is characterized by the presence of nephrotic range proteinuria and an increased incidence of renal vein thrombosis. Two cases of acute renal vein thrombosis associated with secondary amyloidosis in patients with spinal cord injury are presented. In both cases, a past history of extensive decubitus ulcerations and urinary tract infections preceded the development of nephrotic range proteinuria. In case 1, nonoliguric acute renal failure occurred after the development of acute bilateral renal vein thrombosis. The patient declined dialytic therapy and expired with uremia. In case 2, worsening renal function and increased proteinuria resulted after the development of acute unilateral renal vein thrombosis. These cases include the clinical and anatomic findings of acute renal vein thrombosis that occur as a complication of secondary amyloidosis. Acute renal vein thrombosis should be considered whenever an acute change in renal function or increase in proteinuria is noted in this setting.     

Periosteal new bone formation developing during haemodialysis for chronic renal failure

Periosteal new bone formation in the pelvis is reported in five patients on maintenance haemodialysis for chronic renal failure. In two of the cases the shafts of long bones were also involved. The associated radiological and biochemical findings suggest that this unusual radiological feature may be a manifestation of secondary hyperparathyroidism. In one patient in whom the plasma phosphate and calcium X phosphate [Ca X P1] product were reduced there was partial incorporation of the periosteal new bone into the underlying cortex.