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Calciphylaxis in pediatric end-stage renal disease

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Abstract

Calciphylaxis is a rare, but life-threatening complication of end-stage renal disease (ESRD) that has been reported mostly in adult patients. The exact etiology is unknown, but the disease is commonly associated with a high calcium-phosphorus product and elevated levels of parathyroid hormone (PTH). We herein review the published reports on calciphylaxis in ESRD patients less than 18 years old and report the case of a patient with severe calciphylaxis who presented with lower extremity pain, muscle tenderness and difficulty in walking. The serum PTH was low, and the calcium-phosphorus product was normal. The diagnosis of calciphylaxis was confirmed by a muscle biopsy. Treatment with low calcium peritoneal dialysate and substitution of calcium-based phosphorus binders with sevelamer (Renagel) was unsuccessful. The patient’s clinical condition progressed to extensive soft tissue calcification and ulcerating skin lesions. Nine months after the onset of symptoms, the patient died of cardiopulmonary arrest.

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References

  1. Adrogue HJ, Frazier MR, Zeluff B, Suki WN (1981) Systemic calciphylaxis revisited. Am J Nephrol 1:177–183

    PubMed  Google Scholar 

  2. Rubinger D, Friedlaender MM, Silver J, Kopolovic Y, Czaczkes WJ, Popovtzer MM (1986) Progressive vascular calcification with necrosis of extremities in hemodialysis patients: a possible role of iron overload. Am J Kidney Dis 7:125–129

    PubMed  Google Scholar 

  3. van Hamersvelt HW, van Dooren-Greebe RJ, Koene RA (1994) Calciphylaxis in renal failure. Nephrol Dial Transplant 9:1201–1202

    PubMed  Google Scholar 

  4. Tamura M, Hiroshige K, Osajima A, Soejima M, Takasugi M, Kuroiwa A (1995) A dialysis patient with systemic calciphylaxis exhibiting rapidly progressive visceral ischemia and acral gangrene. Intern Med 34:908–912

    PubMed  Google Scholar 

  5. Budisavljevic MN, Cheek D, Ploth DW (1996) Calciphylaxis in chronic renal failure. J Am Soc Nephrol 7:978–982

    PubMed  Google Scholar 

  6. Hafner J, Keusch G, Wahl C, Sauter B, Hurlimann A, von Weizsacker F, Krayenbuhl M, Biedermann K, Brunner U, Helfenstein U (1995) Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): a complication of chronic renal failure and benefit from parathyroidectomy. J Am Acad Dermatol 33:954–962

    Article  PubMed  Google Scholar 

  7. Fine A, Zacharias J (2002) Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int 61:2210–2217

    Article  PubMed  Google Scholar 

  8. Moe SM, Chen NX (2003) Calciphylaxis and vascular calcification: a continuum of extra-skeletal osteogenesis. Pediatr Nephrol 18:969–975

    Article  PubMed  Google Scholar 

  9. Reslerova M, Moe SM (2003) Vascular calcification in dialysis patients: pathogenesis and consequences. Am J Kidney Dis 41:S96–99

    PubMed  Google Scholar 

  10. McCullough PA, Sandberg KR, Dumler F, Yanez JE (2004) Determinants of coronary vascular calcification in patients with chronic kidney disease and end-stage renal disease: a systematic review. J Nephrol 17:205–215

    PubMed  Google Scholar 

  11. Zouboulis CC, Blume-Peytavi U, Lennert T, Stavropoulos PG, Schwarz A, Runkel N, Trautmann C, Orfanos CE (1996) Fulminant metastatic calcinosis with cutaneous necrosis in a child with end-stage renal disease and tertiary hyperparathyroidism. Br J Dermatol 135:617–622

    Article  PubMed  Google Scholar 

  12. Slovis TL, Chand N, Shanavos TO, Fleischmann LE, Brough AJ (1977) Pulmonary calcifications in a child with renal failure. Pediatr Radiol 6:112–115

    Article  PubMed  Google Scholar 

  13. Verma RP, Smergel EM, Chandrasekaran K (1993) Myocardial calcification in an extremely low birth weight infant with chronic renal failure and secondary hyperparathyroidism. J Perinatol 13:111–114

    PubMed  Google Scholar 

  14. Fulladosa X, Gonzalez MT, Cruzado JM, Andres E, Castelao AM, Grino JM, Alsina J (1995) Metastatic pulmonary calcifications in severe secondary hyperparathyroidism: evolution after renal transplantation. Transplant Proc 27:2272–2276

    PubMed  Google Scholar 

  15. Koch Nogueira PC, Giuliani C, Rey N, Said MH, Cochat P (1997) Calcifying panniculitis in a child after renal transplantation. Nephrol Dial Transplant 12:216–218

    Article  PubMed  Google Scholar 

  16. Saatci U, Tezic T, Kocak H, Besbas N (1982) Soft tissue calcification in chronic renal failure in childhood. Turk J Pediatr 24:263–265

    PubMed  Google Scholar 

  17. McLachlan MS, Wallace M, Seneviratne C (1968) Pulmonary calcification in renal failure. Report of three cases. Br J Radiol 41:99–106

    PubMed  Google Scholar 

  18. de Moraes CR (1986) Calcification of the heart: a rare manifestation of chronic renal failure. Pediatr Radiol 16:422–424

    PubMed  Google Scholar 

  19. Mawad HW, Sawaya BP, Sarin R, Malluche HH (1999) Calcific uremic arteriolopathy in association with low turnover uremic bone disease. Clin Nephrol 52:160–166

    PubMed  Google Scholar 

  20. Wilmer WA, Magro CM (2002) Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial 15:172–186

    Article  PubMed  Google Scholar 

  21. Korkmaz C, Dundar E, Zubaroglu I (2002) Calciphylaxis in a patient with rheumatoid arthritis without renal failure and hyperparathyroidism: the possible role of long-term steroid use and protein S deficiency. Clin Rheumatol 21:66–69

    Article  PubMed  Google Scholar 

  22. Denton MD, Mauiyyedi S, Bazari H (2001) Heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein S deficiency. Am J Nephrol 21:289–293

    Article  PubMed  Google Scholar 

  23. Riegert-Johnson DL, Kaur JS, Pfeifer EA (2001) Calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K. Mayo Clin Proc 76:749–752

    PubMed  Google Scholar 

  24. Goyal S, Huhn KM, Provost TT (2000) Calciphylaxis in a patient without renal failure or elevated parathyroid hormone: possible aetiological role of chemotherapy. Br J Dermatol 143:1087–1090

    Article  PubMed  Google Scholar 

  25. Perez-Mijares R, Guzman-Zamudio JL, Payan-Lopez J, Rodriguez-Fernandez A, Gomez-Fernandez P, Almaraz-Jimenez M (1996) Calciphylaxis in a haemodialysis patient: functional protein S deficiency? Nephrol Dial Transplant 11:1856–1859

    PubMed  Google Scholar 

  26. Rudwaleit M, Schwarz A, Trautmann C, Offermann G, Distler A (1996) Severe calciphylaxis in a renal patient on long-term oral anticoagulant therapy. Am J Nephrol 16:344–348

    PubMed  Google Scholar 

  27. Rostaing L, el Feki S, Delisle MB, Durand-Malgouyres C, Ton-That H, Bonafe JL, Bories P, Durand D, Suc JM (1995) Calciphylaxis in a chronic hemodialysis patient with protein S deficiency. Am J Nephrol 15:524–527

    PubMed  Google Scholar 

  28. Mehta RL, Scott G, Sloand JA, Francis CW (1990) Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis. Am J Med 88:252–257

    Article  PubMed  Google Scholar 

  29. Nelson RG, Gohdes DM, Everhart JE, Hartner JA, Zwemer FL, Pettitt DJ, Knowler WC (1988) Lower-extremity amputations in NIDDM: 12-year follow-up study in Pima Indians. Diabetes Care 11:8–16

    PubMed  Google Scholar 

  30. Navab M, Berliner JA, Subbanagounder G, Hama S, Lusis AJ, Castellani LW, Reddy S, Shih D, Shi W, Watson AD, Van Lenten BJ, Vora D, Fogelman AM (2001) HDL and the inflammatory response induced by LDL-derived oxidized phospholipids. Arterioscler Thromb Vasc Biol 21:481–488

    PubMed  Google Scholar 

  31. Fukui N, Zhu Y, Maloney WJ, Clohisy J, Sandell LJ (2003) Stimulation of BMP-2 expression by pro-inflammatory cytokines IL-1 and TNF-alpha in normal and osteoarthritic chondrocytes. J Bone Joint Surg Am 85-A [Suppl 3]:59–66

  32. Chen NX, O’Neill KD, Duan D, Moe SM (2002) Phosphorus and uremic serum up-regulate osteopontin expression in vascular smooth muscle cells. Kidney Int 62:1724–1731

    Article  PubMed  Google Scholar 

  33. Vattikuti R, Towler DA (2004) Osteogenic regulation of vascular calcification: an early perspective. Am J Physiol Endocrinol Metab 286:E686–696

    Google Scholar 

  34. Hale EK (2003) Metastatic calcification. Dermatol Online J 9:2

    Google Scholar 

  35. Beus KS, Stack BC Jr (2004) Calciphylaxis. Otolaryngol Clin North Am 37:941–948

    Article  PubMed  Google Scholar 

  36. Arch-Ferrer JE, Beenken SW, Rue LW, Bland KI, Diethelm AG (2003) Therapy for calciphylaxis: an outcome analysis. Surgery 134:941–944; discussion 944–945

    Article  PubMed  Google Scholar 

  37. Girotto JA, Harmon JW, Ratner LE, Nicol TL, Wong L, Chen H (2001) Parathyroidectomy promotes wound healing and prolongs survival in patients with calciphylaxis from secondary hyperparathyroidism. Surgery 130:645–650

    Article  PubMed  Google Scholar 

  38. Levin NW, Hoenich NA (2001) Consequences of hyperphosphatemia and elevated levels of the calcium-phosphorus product in dialysis patients. Curr Opin Nephrol Hypertens 10:563–568

    Article  PubMed  Google Scholar 

  39. Don BR, Chin AI (2003) A strategy for the treatment of calcific uremic arteriolopathy (calciphylaxis) employing a combination of therapies. Clin Nephrol 59:463–470

    PubMed  Google Scholar 

  40. Fine A, Fleming S, Leslie W (1995) Calciphylaxis presenting with calf pain and plaques in four continuous ambulatory peritoneal dialysis patients and in one predialysis patient. Am J Kidney Dis 25:498–502

    PubMed  Google Scholar 

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Acknowledgements

We thank Dr. William Kupsky for his help with pathology.

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Correspondence to Abubakr A. Imam.

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Imam, A.A., Mattoo, T.K., Kapur, G. et al. Calciphylaxis in pediatric end-stage renal disease. Pediatr Nephrol 20, 1776–1780 (2005). https://doi.org/10.1007/s00467-005-2026-2

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  • DOI: https://doi.org/10.1007/s00467-005-2026-2

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