A case of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber's disease) with dystrophic calcinosis cutis and retinal lesions

A 65-year-old Japanese female developed a nodule on the distal interphalangealjoint of her right thumb. She also had multiple telangiectasias on her face, oral mucosa, tongue, nasal mucosa and upper extremities. A lip biopsy showed irregularly dilated capillaries and venules lined by flat endothelial cells. The nodule in the right thumb was resected. Histopathology revealed calcium deposits in the dermis. This case was diagnosed as dystrophic calcinosis cutis within hereditary hemorrhagic telangiectasia. Ophthalmologic examination showed bilateral retinal vascular lesions with dilated and tortuous retinal venules.     

Tumoral calcinosis with hyperphosphatemia

Tumoral calcinosis is a rare disorder of mineral metabolism among adolescents and young adults characterized by deposition of calcific masses around large joints. It is less commonly reported in pediatric population and commonly mistaken for bone tumors. Typical lab parameters include hyperphosphatemia with normal levels of serum calcium, parathyroid hormone (PTH) and alkaline phosphatase. A ten-year-old boy with typical features of tumoral calcinosis is presented.     

Primary Bypass Surgery from the Descending Aorta to the Iliac Arteries for a Severely Calcified Aorta: Report of Two Cases

We herein report two patients with end-stage renal failure who complained of disabling claudication due to abdominal aortic atherosclerosis. Both were on chronic hemodialysis for more than 15 years, and their abdominal aorta was densely calcified. We elected to perform a descending aorta to iliac artery bypass in order to avoid hazardous clamping of the calcified aorta. Hemodialyisis was able to be resumed on the first postoperative day. They tolerated the operation well and their symptoms disappeared. This operative procedure is therefore considered to be a useful option for patients with a porcelain aorta who are on chronic hemodialysis.     

Disproportionately severe calcinosis cutis in an 88-year-old patient with CREST syndrome

An 88-year-old woman with CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) presented with hyperglycemia, intravascular depletion, and atrial fibrillation. The patient was found to have unusually severe calcinosis cutis in both legs extending from the knees to the ankles bilaterally, as well as Raynaud’s phenomenon, sclerodactyly, and telangiectasias. The patient was normocalcemic and normophosphatemic. Although subcutaneous calcification is often seen with CREST syndrome, this case is unusual in that the area of involvement was much larger than previously described. Furthermore, the amount of calcinosis was disproportionately severe and was the major cause of symptoms and disability compared with the other components of the syndrome. Received: 1 February 2001 Revision requested: 21 March 2001 Revision received: 3 April 2001 Accepted: 4 April 2001     

稳定型与不稳定型心绞痛患者电子束CT 冠状动脉钙化检测的比较

【目的】比较稳定型心绞痛（SAP）与不稳定型心绞痛（UAP）患者冠状动脉(冠脉)钙化（CAC）的不同模式。【方法】67例SAP与94例UAP患者,行选择性冠脉造影和电子束CT（EBCT）检查,分析两组CAC积分（CS）的差异及其与冠脉狭窄程度之间的关系。【结果】①SAP组CAC检测阳性率（100%）显著高于UAP组（87.23%）（P<0.05）。②SAP组CAC积分显著高于UAP组,其自然对数转换值［ln（CS+1）］的均数按心脏整体计算为（5.24±1.72）对（3.13±1.96）（P<0.001）,按冠脉分支计算为（3.28±2.08）对（1.68±1.62）（P<0.001）。③在每一相同的年龄段内比较,SAP组的CAC程度均明显高于UAP组。④ln(CS+1)与冠脉狭窄程度在两组均呈正相关,但SAP组相关程度相对较好;SAP组重度狭窄的冠脉多发生重度或中度钙化,而UAP组重度狭窄的冠脉则多数无钙化或仅有轻度钙化。【结论】稳定型与不稳定型心绞痛患者冠脉钙化存在不同的模式,前者冠脉钙化大多较为严重,钙化积分与冠脉狭窄程度的相关性较好,而后者钙化程度多数较轻,钙化积分与冠脉狭窄程度的相关性很差。     

Haemodynamic and tubular renal dysfunction in rats with sustained arterial calcinosis

1. In humans, two of the principal characteristics of vascular ageing are arterial wall calcification and decreased arterial distensibility, which induce organ damage. To amplify arterial calcium accumulation in laboratory animals, it is necessary to use an overdose of vitamin D(3). 2. The aim of the present study was to assess the impact of arterial calcium overload on renal function. 3. Adult male Wistar rats were randomly divided into two groups: control and treated rats. Treated rats were injected 10 days before the experiment with a single dose of vitamin D(3) (300 000 IU/kg, i.m.). 4. Treated rats showed a decrease in renal blood flow and glomerular filtration rate. Tubular parameters were not modified under basal conditions. In contrast, a statistically significant increase in the fractional excretion of Na, K, Ca and H(2)O were observed in treated rats after the acute increment of sodium distal delivery, suggesting that the reabsorptive capacity of the thick ascending limb may be altered in treated rats. 5. Thus, Na(+)/K(+)-ATPase activity was evaluated in homogenates from renal cortex and medulla. Rats with arterial calcinosis presented a diminished activity of Na(+)/K(+)-ATPase in medulla homogenates. 6. An increment in the abundance of the Na-K-2Cl cotransporter (NKCC2) was observed in renal medulla homogenates from treated rats. It is suggested that this may compensate for the inefficiency of Na(+)/K(+)-ATPase under basal conditions but, in the presence of acute distal sodium overload, the increment in NKCC2 abundance may not be sufficient to compensate for the decrease in Na(+)/K(+)-ATPase activity. 7. In summary, in our experimental model of arterial calcinosis, renal function is impaired, presenting a vascular compromise and altered function of the medullar thick ascending limb that becomes evident in the presence of acute high distal sodium delivery.     

Bisacylphosphonates Inhibit Hydroxyapatite Formation and Dissolution in Vitro and Dystrophic Calcification in Vivo

Some geminal bisphosphonates are used clinically for a number of important bone/calcium related diseases; however, side effects and lack of selectivity impede their wide use. This work reports the synthesis and evaluation of bisacylphosphonates (e.g., adipoyl- and suberoylbisphosphonate). These compounds were found to inhibit significantly hydroxyapatite formation and dissolution in vitro and the calcification of bioprosthetic tissue implanted subdermally in rats. These are the first instances of nongeminal bisphosphonates [P–(C)n– P, n 2] that have been reported to be active in calcium-related disorders. The reported bisacylphosphonates possess apparent lower toxicity, and their calcium complexes/salts have improved solubility properties. Therefore, they are of potential importance for clinical applications.     

Tumoral calcinosis: radiologic-pathologic correlation

Objective Tumoral calcinosis is a frequently misdiagnosed disorder. This study details the radiologic and pathologic characteristics of tumoral calcinosis that distinguish it from most other entities.Design Radiologic and pathologic findings, and medical records of 12 patients with tumoral calcinosis were reviewed and compared with equivalent information about 5 patients with other calcified lesions.Patients The 12 patients ranged in age from 15 months to 62 years. Six had idiopathic tumoral calcinosis and 6 had secondary tumoral calcinosis.Results and conclusions A consistent radiologic finding for tumoral calcinosis was a dense calcified mass that was homogeneous except for a chicken wire pattern of lucencies, which correlated histologically with thin fibrous septae. Other characteristics of tumoral calcinosis included fluid-calcium levels, demonstrated in four patients, and smooth osseous erosions adjacent to the mass, demonstrated in three patients. Five cases of tumoral calcinosis were originally confused with other calcified lesions; however, the radiologic findings were characteristic of tumoral calcinosis in retrospect.     

Pulmonary Alveolar Septal Calcinosis Causing Progressive Respiratory Failure in Acute Lymphoblastic Leukemia in Childhood

A syndrome of pulmonary alveolar septal calcinosis, pneumothorax, and pneumomediastinum, leading to rapidly progressive acute respiratory insufficiency and death was observed in 2 children with acute lymphoblastic leukemia (ALL). Primary clinical and radiological considerations in these patients were pulmonary edema and infection, and the diagnosis of pulmonary alveolar septal calcification was established only at autopsy. One patient, a 15-year-old girl, was found also to have parathyroid hyperplasia typical of familial hyperparathyroidism. The other, a 16-month-old girl, showed osteitis fibrosa of the bones and parathyroid hyperplasia of secondary type, suggesting that the pulmonary calcinosis resulted from hypercalcemia caused by a parathormone or prostaglandin-secreting tumor. The cause of pneumothorax and pneumomediastinum may have been rupture of calcified alveolar septa induced by high PEEP during ventilation of these patients. Other possible mechanisms contributing to hypercalcemia and pulmonary calcinosis in children with acute leukemia include bone resorption due to marrow infiltration, immobilization syndrome, renal failure, and administration of calcium, phosphate, or bicarbonate. This complication of acute leukemia in childhood is rare (2 patients in 430 autopsied over the period 1961-1982 at Childrens Hospital of Los Angeles). How often the process can be reversed if diagnosed before severe respiratory insufficiency is present is not known.