Severe calciphylaxis in a patient on dialysis, with a liver transplant and long evolution hypocalcaemia
Calcifilaxis severa en paciente en diálisis, trasplantado hepático e hipocalcemia de larga evolución
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J. Bravo López, R. Blanco García, M.ª Borrajo Prol, A. Iglesias" "autores" => array:5 [ 0 => array:4 [ "nombre" => "María Jesús" "apellidos" => "Camba Caride" "email" => array:1 [ 0 => "marisu75@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 1 => array:3 [ "Iniciales" => "J. J." "apellidos" => "Bravo López" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 2 => array:3 [ "Iniciales" => "R." "apellidos" => "Blanco García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 3 => array:3 [ "nombre" => "M.ª" "apellidos" => "Borrajo Prol" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 4 => array:3 [ "Iniciales" => "A." "apellidos" => "Iglesias" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] ] "afiliaciones" => array:1 [ 0 => array:3 [ "entidad" => "Servicio de Nefrología, Complexo Hospitalario de Ourense, Ourense, España, " "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Calcifilaxis severa en paciente en diálisis, trasplantado hepático e hipocalcemia de larga evolución" ] ] "textoCompleto" => "<p class="elsevierStylePara">To the editor:</p><p class="elsevierStylePara">We report a 65 year-old male, who received a liver transplant in 1996 and is on treatment with prednisone, mycofenolate and cyclosporin. He presented early graft rejection that was treated with pulses of methylprednisolone, and developed acute renal failure due to cyclosporin-induced nephrotoxicity requiring hemodialysis on July 2002. He has also a history of type 2 diabetes mellitus, dilated cardiomyopathy and atrial fibrillation (on anticoagulation with Acenocoumarol). He developed severe hypocalcaemia and received calcium carbonate (up to 12 g/day) and oral calcitriol (1 μg/day). The iPTH levels were maintained between 100 and 250 pg/mL and the calcium × phosphorus product was lower than 50.</p><p class="elsevierStylePara">In December of 2002, he presented with bilateral skin lesions on the legs, which were painful and small in diameter. Some of them had an ecchymotic appearance and others were eroded and covered by a necrotic ulcer, with violaceous borders. Peripheral pulses were present. He required opiates to control the pain and topical antibiotics. In January 2003 he was admitted to the hospital because of hemodynamic instability during hemodialysis session, fever, anemia and progression of the lesions with increasing necrosis and infection.</p><p class="elsevierStylePara">The laboratory parameters were: 1,900 leucocytes/mm3, hemoglobin 6.7 g/dL, calcium 7 mg/dL, phosphate 3.5 mg/dl, iPTH 72.5 pg/mL, albumin 2.6 g/dL, CRP 13.9 mg/L. The viral serology and immunological study (ANA, ANCA) were negative. Hemocultures and antigenemia were positive for Cryptococcus neoformans. Abdominal plain X-ray film: multiple vascular calcifications. Skin biopsy: Calcification within the middle layer of dermal arterioles and arteries, thrombosis within the vessels, necrosis of the adipose tissue, suggestive of calciphylaxis.</p><p class="elsevierStylePara">Despite the treatment with wide spectrum antibiotics and intravenous fluconazole, the progressive diminution of mycofenolate, and the surgical debridement of the lesions, the evolution was torpid and the patient died in septic shock.</p><p class="elsevierStylePara">CUA is a syndrome of unknown origin, characterized by areas of ischemic necrosis and calcifications of the middle layers of dermoepidermal arterioles. It is associated to chronic renal insufficiency, dialysis, and kidney transplant<span class="elsevierStyleSup">1</span>. Other risk factors were identified: hyperparathyroidism, elevated calcium-phosphorus product, hyperphosphatemia,<span class="elsevierStyleSup">2, 4-8</span> adynamic bone disease,<span class="elsevierStyleSup">9</span> prolonged treatment with vitamin D supplements, calcium-based phosphorus chelating agents, oral anticoagulants, steroids, intravenous iron load, diabetes mellitus, hypoalbuminemia, deficit of proteins C or S, hyperlipidemia, local traumas and HIV infection.<span class="elsevierStyleSup">4,8,10-12</span> It is more frequent among obese and females patients.<span class="elsevierStyleSup">6</span></p><p class="elsevierStylePara">Diagnosis lies on clinical findings: presence of typical lesions with peripheral pulses and hyperesthesia, often resistant to analgesia.<span class="elsevierStyleSup">2</span> Histological confirmation is definitive. However it is associated to a high risk for superinfection and local dissemination of the ulcer, and some authors affirm that it should be reserved for those cases, in which the diagnosis is not clear.<span class="elsevierStyleSup">2,8</span></p><p class="elsevierStylePara">The approach to these patients must be multidisciplinary: treatment of underlying conditions,<span class="elsevierStyleSup">1, 6</span> control of the calciumphosphorus product and of secondary hyperparathyroidism, to limit the use of calcium-based phosphorus chelating agents and of vitamin D<span class="elsevierStyleSup">6,10,11</span>, and hemodialysis with low calcium content in the dialysis fluid.<span class="elsevierStyleSup">6</span> Parathyroidectomy is indicated in cases of severe hyperparathyroidism.<span class="elsevierStyleSup">8</span> Necrotic tissue should be surgically removed and wide spectrum antibiotics should be administered. In recent studies the use of steroids,<span class="elsevierStyleSup">8</span> hyperbaric oxygen, diphosphonates, pentoxifylline or sterile larvae<span class="elsevierStyleSup">9</span> have shown promising results. In spite of an aggressive therapy the mortality is very high (60-80%), mainly due to sepsis.<span class="elsevierStyleSup">4</span></p>" "pdfFichero" => "P-E-S-A513-EN.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Marrón B, Coronel F, López-Bran E, Barrientos A. Calcifilaxia: una patogenia incierta y un tratamiento controvertido. Nefrología 2001; 6: 596-600." "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 1 => array:3 [ "identificador" => "bib2" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Wilmer WA, Magro CM. Calciphylaxis: Emerging concepts in prevention, diagnosis, and treatment. Seminars in Dialysis 2002; 15: 172-186. <a href="http://www.ncbi.nlm.nih.gov/pubmed/12100455" target="_blank">[Pubmed]</a>" "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 2 => array:3 [ "identificador" => "bib3" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "García-Nieto, AV, Moreno Giménez JC. Calcifilaxis. Piel 2000; 15: 118-125." "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 3 => array:3 [ "identificador" => "bib4" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Mazhar AR, Johnson RJ, Gillen D, Stivelman JC, Ryan MJ, Davis CL, Stehman-Breen CO. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int 2001; 60: 324-332. <a href="http://www.ncbi.nlm.nih.gov/pubmed/11422768" target="_blank">[Pubmed]</a>" "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 4 => array:3 [ "identificador" => "bib5" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Mawad HW, Sawaya R, Sarin R, Malluche HH. Calcific uremic arteriolopathy in association with low turnover uremic bone disease. Clin Nephrol 1999; 52: 160-166. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10499311" target="_blank">[Pubmed]</a>" "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 5 => array:3 [ "identificador" => "bib6" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Llach F. The evolving pattern of calciphylaxis: therapeutic considerations. 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Kidney Int 2002; 61: 2210-2217. <a href="http://www.ncbi.nlm.nih.gov/pubmed/12028462" target="_blank">[Pubmed]</a>" "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 8 => array:3 [ "identificador" => "bib9" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Caramelo C, Goicochea M, Albalete M, Nieto L, Melero R, Berlanga JR, López MD, González Pacheco F, Marrón B, Álvarez Arroyo M.V, Castilla MA, Yagüe S, Deudero J, Ortiz A. Cambios estructurales y calcificaciones vasculares en la uremia. Nefrología 2001; 6: 538-547." "contribucion" => array:1 [ 0 => null ] "host" => array:1 [ 0 => null ] ] ] ] 9 => array:3 [ "identificador" => "bib10" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Zacharias JM, Fontaine B, Fine A. Calcium use increase risk of calciphylaxis: a case-control study. 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| 2017 March | 22 | 14 | 36 |
| 2017 February | 19 | 3 | 22 |
| 2017 January | 15 | 7 | 22 |
| 2016 December | 46 | 10 | 56 |
| 2016 November | 47 | 10 | 57 |
| 2016 October | 49 | 12 | 61 |
| 2016 September | 79 | 4 | 83 |
| 2016 August | 135 | 3 | 138 |
| 2016 July | 148 | 4 | 152 |
| 2016 June | 120 | 0 | 120 |
| 2016 May | 112 | 0 | 112 |
| 2016 April | 60 | 0 | 60 |
| 2016 March | 67 | 0 | 67 |
| 2016 February | 75 | 0 | 75 |
| 2016 January | 83 | 0 | 83 |
| 2015 December | 66 | 0 | 66 |
| 2015 November | 66 | 0 | 66 |
| 2015 October | 64 | 0 | 64 |
| 2015 September | 57 | 0 | 57 |
| 2015 August | 58 | 0 | 58 |
| 2015 July | 64 | 0 | 64 |
| 2015 June | 37 | 0 | 37 |
| 2015 May | 44 | 0 | 44 |
| 2015 April | 5 | 0 | 5 |
Show all
