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[Calciphylaxis, risk factors, treatment and outcomes. Retrospective study of 39 patients]. / Calcifilaxis, factores de riesgo, tratamiento y evolución. Estudio retrospectivo de 39 pacientes.
Torre, Ana C; Bastard, Dolores P; Diehl, Maria; Rosa-Diez, Guillermo; Volonteri, Victoria; Mazzuoccolo, Luis D; Belatti, Anahi L.
Affiliation
  • Torre AC; Servicio de Dermatología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. E-mail: ana.torre@hospitalitaliano.org.ar.
  • Bastard DP; Servicio de Dermatología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
  • Diehl M; Servicio de Endocrinología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
  • Rosa-Diez G; Servicio de Nefrología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
  • Volonteri V; Servicio de Anatomía Patológica, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
  • Mazzuoccolo LD; Servicio de Dermatología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
  • Belatti AL; Servicio de Dermatología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
Medicina (B Aires) ; 84(2): 196-205, 2024.
Article in Es | MEDLINE | ID: mdl-38683504
ABSTRACT

INTRODUCTION:

Calciphylaxis is a serious vascular disorder characterized by calcification of tunica media, intimal hyperplasia, thrombosis, and skin necrosis. It was described in patients with renal failure (UC), although it can occur in its absence (NUC). Its risk factors are under study and its diagnosis can be complex. Over a decade ago, its mortality was estimated at 60-80%. Recent studies indicate that it has decreased (40%).

METHODS:

A retrospective study was carried out in the period between January 1, 2011 and December 31, 2019. The past medical record, clinical characteristics, laboratory and histopathological findings, and evolution of all patients with calciphylaxis evaluated at the Hospital Italiano de Buenos Aires were reviewed.

RESULTS:

Thirty-nine patients were included. Sixtyone percent were men and 39% were NUC cases. Eightytwo percent had arterial hypertension, 66% obesity and 46% diabetes. Of those, 49% received coumarin anticoagulants. All patients with NUC and 75% with UC presented ulcers with necrosis, located more frequently on the legs. In 72% of the cases the histological diagnosis was made with one biopsy. In all the treatment was multimodal and mortality at one year was 42%.

CONCLUSION:

We observed a high proportion of patients with NUC, in relation to what is reported in the literature, and that half received vitamin K antagonists. The histological diagnosis was made with one biopsy in most of the cases, as the surgical technique for taking the sample, the Von Kossa staining and the evaluation by an expert pathologist were the key of it.
RESUMEN

Introducción:

La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica media arteriolar, trombosis y necrosis cutánea. Se describió en pacientes con insuficiencia renal (CU), aunque puede producirse en su ausencia (CNU). Sus factores de riesgo están en estudio y su diagnóstico puede ser complejo. Su mortalidad se estimaba en 60-80%, aunque trabajos recientes indican que ha disminuido (40%).

Métodos:

Estudio retrospectivo entre el 1/1/2011 y el 31/12/2019. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, y la evolución de todos los pacientes con diagnóstico de calcifilaxis evaluados en el Hospital Italiano de Buenos Aires.

Resultados:

Se incluyeron 39 pacientes. El 61.5% (24) eran hombres y 38.5% (15) fueron casos de CNU. De éstos, 82% presentaba hipertensión arterial, 66% obesidad y 46% diabetes. El 49% recibía anticoagulantes dicumarínicos. Todos los pacientes con CNU y 75% con CU presentaron úlceras de fondo necrótico, localizadas con mayor frecuencia en las piernas. En 72% de los casos el diagnóstico histológico se efectuó con una toma de biopsia. En todos, el tratamiento fue multimodal y la mortalidad al año fue de 42%.

Conclusión:

Observamos una elevada proporción de pacientes con CNU, en relación con lo comunicado en la literatura, y la mitad recibía anticoagulantes dicumarínicos. El diagnóstico histológico se efectuó por biopsia en la mayor parte de los casos, para lo cual la toma quirúrgica de la muestra, la tinción con Von Kossa y la evaluación por un patólogo experto fueron claves.
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Collection: 01-internacional Database: MEDLINE Main subject: Calciphylaxis Limits: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Country/Region as subject: America do sul / Argentina Language: Es Journal: Medicina (B Aires) Year: 2024 Document type: Article
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Collection: 01-internacional Database: MEDLINE Main subject: Calciphylaxis Limits: Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Country/Region as subject: America do sul / Argentina Language: Es Journal: Medicina (B Aires) Year: 2024 Document type: Article