Fatal Childhood Calciphylaxis in a 10‐Year‐Old and Literature Review

Feng, Jing; Gohara, Mona; Lazova, Rossitza; Antaya, Richard J.

doi:10.1111/j.1525-1470.2006.00232.x

Cited by 26 publications

(9 citation statements)

References 25 publications

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“…44,45 Calciphylaxis is more commonly seen in women compared to men with a 2:1 female predominance. 12,15,46 Calciphylaxis in our experience and as reported in the literature is also more common in whites compared to non-whites.…”

Section: Risk Factorsmentioning

confidence: 99%

Calciphylaxis: Risk Factors, Diagnosis, and Treatment

Nigwekar

Kroshinsky

Nazarian

et al. 2015

American Journal of Kidney Diseases

356

505

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Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in chronic kidney failure patients treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal renal function. In this In Practice feature, we review the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and non-uremic calciphylaxis. High quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence, poorly understood pathogenesis, and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by the Massachusetts General Hospital's Multi-disciplinary Calciphylaxis Team for calciphylaxis patients.

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Section: Risk Factorsmentioning

confidence: 99%

Calciphylaxis: Risk Factors, Diagnosis, and Treatment

Nigwekar

Kroshinsky

Nazarian

et al. 2015

American Journal of Kidney Diseases

356

505

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“…Vascular and soft tissue calcification is common in children occurring in up to 60% in those with ESRD; however, the incidence of CUA is unknown with only 10 case reports identified to date (census date 25 January 2010) 54,67–70. Previous reviewers have noted the following pertinent findings regarding the pediatric population: Increased risk in males (90% of the cases reported to date) with ESRD and secondary hyperparathyroidism, frequent distal extremity and visceral organ involvement, worse prognosis with acral-distal involvement, and increased resistance to medical treatment compared to the affected adult population 69. However, clinicians should keep in mind that with the continuing increase in childhood obesity there may be a changing trend in the future involving more proximal adipose tissue related skin ulceration.…”

Section: Cua/calciphylaxis In the Pediatric Populationmentioning

confidence: 99%

Calcific Uremic Arteriolopathy: Pathophysiology, Reactive Oxygen Species and Therapeutic Approaches

Sowers

Hayden

2010

Oxidative Medicine and Cellular Longevity

122

140

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Calcific uremic arteriolopathy (CUA)/calciphylaxis is an important cause of morbidity and mortality in patients with chronic kidney disease requiring renal replacement. Once thought to be rare, it is being increasingly recognized and reported on a global scale. The uremic milieu predisposes to multiple metabolic toxicities including increased levels of reactive oxygen species and inflammation. Increased oxidative stress and inflammation promote this arteriolopathy by adversely affecting endothelial function resulting in a prothrombotic milieu and significant remodeling effects on vascular smooth muscle cells. These arteriolar pathological effects include intimal hyperplasia, inflammation, endovascular fibrosis and vascular smooth muscle cell apoptosis and differentiation into bone forming osteoblast-like cells resulting in medial calcification. Systemic factors promoting this vascular condition include elevated calcium, parathyroid hormone and hyperphosphatemia with consequent increases in the calcium × phosphate product. The uremic milieu contributes to a marked increased in upstream reactive oxygen species—oxidative stress and subsequent downstream increased inflammation, in part, via activation of the nuclear transcription factor NFκB and associated downstream cytokine pathways. Consitutive anti-calcification proteins such as Fetuin-A and matrix GLA proteins and their signaling pathways may be decreased, which further contributes to medial vascular calcification. The resulting clinical entity is painful, debilitating and contributes to the excess morbidity and mortality associated with chronic kidney disease and end stage renal disease. These same histopathologic conditions also occur in patients without uremia and therefore, the term calcific obliterative arteriolopathy could be utilized in these conditions.

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“…Its description was first reported in skin, and the calciphylaxis of other organs has rarely been reported 3. Unlike metastatic calcification, calciphylaxis is accompanied by damage to ischemic and necrotic tissue 4,5. Metastatic calcification and calciphylaxis are metabolic disorders,3 for which the precise pathogenesis has not yet been determined.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Calciphylaxis Associated with Acute Respiratory and Renal Failure Due to Cryptogenic Hypercalcemia: An Autopsy Case Report

et al. 2012

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Metastatic calcification is rare; it is found during autopsy in patients who underwent hemodialysis. Diffuse calcium precipitation of small and medium-sized cutaneous vessels, known as calciphylaxis, can result in progressive tissue necrosis secondary to vascular calcification. This condition most commonly involves the skin; however, a rare occurrence of visceral calciphylaxis has been reported. Here we report on an autopsy case. Despite a thorough evaluation, and even performing an autopsy, the underlying cause of acute-onset hypercalcemia, resulting in the production of pulmonary calciphylaxis and metastatic renal calcification associated with acute respiratory and renal failure, could not be determined. Metastatic calcification often lacks specific symptoms, and the degree of calcification is a marker of the severity and chronicity of the disease. This unusual autopsy case emphasizes the importance of rapidly progressing visceral calciphylaxis, as well as its early detection.

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Fatal Childhood Calciphylaxis in a 10‐Year‐Old and Literature Review

Cited by 26 publications

References 25 publications

Calciphylaxis: Risk Factors, Diagnosis, and Treatment

Calciphylaxis: Risk Factors, Diagnosis, and Treatment

Calcific Uremic Arteriolopathy: Pathophysiology, Reactive Oxygen Species and Therapeutic Approaches

Pulmonary Calciphylaxis Associated with Acute Respiratory and Renal Failure Due to Cryptogenic Hypercalcemia: An Autopsy Case Report

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