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1.
Ann R Coll Surg Engl ; 102(2): e36-e38, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31530167

RESUMO

Penile calciphylaxis or calcific uremic arteriolopathy is a rare urological condition often associated with patients undergoing renal dialysis for end-stage renal disease. The majority of cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as an individual entity has brought little attention. The rates of comorbidity and mortality of these patients are often particularly high. Early diagnosis and a multidisciplinary approach are therefore essential. We report a case of penile calciphylaxis in a 59-year-old man with end-stage renal failure on haemodialysis who was successfully managed conservatively.


Assuntos
Angioplastia/métodos , Calciofilaxia/terapia , Tratamento Conservador/métodos , Doenças do Pênis/terapia , Inibidores da Agregação de Plaquetas/uso terapêutico , Bandagens , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Artéria Femoral/cirurgia , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Doenças do Pênis/diagnóstico , Doenças do Pênis/etiologia , Pênis/irrigação sanguínea , Pênis/patologia , Diálise Renal/efeitos adversos , Resultado do Tratamento
2.
Adv Skin Wound Care ; 32(5): 205-215, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31008757

RESUMO

GENERAL PURPOSE: To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant should be better able to:1. Recognize the pathogenesis and clinical features of and risk factors for calciphylaxis.2. Explain the diagnosis and management of a patient with calciphylaxis. ABSTRACT: Calciphylaxis is a cutaneous ischemic infarct caused by total occlusion of blood vessels initiated by vascular calcification. Until recently, treatments have been limited to controlling its risk factors and optimizing wound care. However, recent advances in clinical understanding of the mechanism of calciphylaxis have identified promising potential therapeutic targets. This article is a narrative review summarizing the clinical features, diagnosis, pathogenesis, and treatment of calciphylaxis.


Assuntos
Calciofilaxia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/patologia , Calciofilaxia/terapia , Quelantes/uso terapêutico , Humanos , Falência Renal Crônica/complicações , Transplante de Rim/efeitos adversos , Manejo da Dor , Diálise Renal , Fatores de Risco , Tiossulfatos/uso terapêutico , Trombose , Calcificação Vascular/prevenção & controle , Calcificação Vascular/terapia , Vitamina K/uso terapêutico , Deficiência de Vitamina K/complicações , Cicatrização
3.
Dermatol Online J ; 25(2)2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30865406

RESUMO

Non-uremic calciphylaxis is a rare, life-threatening condition characterized clinically by cutaneous necrosis and histologically by calcium deposition in small vessel walls. The etiology of non-uremic calciphylaxis remains the subject of ongoing speculation and debate. Herein we present a patient with calciphylaxis who had normal kidney function and numerous rheumatologic diseases, namely systemic lupus erythematosus (SLE), Sjogren syndrome (SS), and myasthenia gravis (MG). We review the pathophysiology, possible mechanisms, and management for non-uremic calciphylaxis.


Assuntos
Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Úlcera da Perna/etiologia , Calciofilaxia/tratamento farmacológico , Quelantes/uso terapêutico , Feminino , Humanos , Rim/fisiologia , Lúpus Eritematoso Sistêmico/complicações , Pessoa de Meia-Idade , Miastenia Gravis/complicações , Síndrome de Sjogren/complicações , Tiossulfatos/uso terapêutico
4.
Dermatol Online J ; 25(2)2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30865408

RESUMO

Calcific uremic arteriolopathy (CUA) or calciphylaxis is a syndrome characterized by calcification of vessels located in the dermis and adipose tissue. It commonly occurs in patients with diabetes mellitus, hypertension, and end-stage renal disease. Clinical presentation generally begins with severe pain, followed by the presence of liveloid or purpuric plaques. Later the formation necrotic ulcers occur. This condition is associated with a poor prognosis, with a high rate of mortality within months of the diagnosis. Penile involvement is an uncommon but severe manifestation. We present an 81-year-old man with a history of diabetes mellitus, hypertension, and end-stage renal disease with a one-month evolution of painful necrotic ulcers on his glans penis. He was diagnosed with CUA. Owing to infection complicated by sepsis; penectomy was performed. Unfortunately, the patient died of myocardial infarction during his hospitalization.


Assuntos
Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Doenças do Pênis/etiologia , Dermatopatias Vasculares/complicações , Úlcera Cutânea/etiologia , Pele/patologia , Idoso de 80 Anos ou mais , Humanos , Falência Renal Crônica/complicações , Masculino , Necrose/etiologia , Doenças do Pênis/patologia , Dermatopatias Vasculares/diagnóstico , Uremia/complicações
7.
Int Wound J ; 16(1): 250-255, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30393969

RESUMO

Calciphylaxis is a rare and potentially fatal disease that affects the subcutaneous layer of the skin. It is a calcific vasculopathy induced by a systemic process that causes occlusion of small blood vessels. The mortality rate for individuals diagnosed with calciphylaxis is estimated between 52% and 81% with sepsis being the leading cause of death. Uraemic calciphylaxis and its known effective treatments are well documented in the literature. Unfortunately, there is no known effective treatment for non-uraemic calciphylaxis. Most of the current treatments for non-uraemic calciphylaxis are derived from uraemic calciphylaxis treatment protocols. We report a case of a 75-year-old female with calciphylaxis on the right lower extremity who was successfully treated with four pamidronate infusions in addition to local wound care. This case represents a non-uraemic calciphylaxis wound successfully treated with pamidronate infusions and standard wound care, and suggests that IV pamidronate can be an effective treatment option.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Calciofilaxia/diagnóstico , Calciofilaxia/tratamento farmacológico , Pamidronato/administração & dosagem , Pamidronato/uso terapêutico , Administração Intravenosa , Idoso , Feminino , Humanos , Resultado do Tratamento
9.
QJM ; 112(1): 29-34, 2019 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-30304522

RESUMO

Calciphylaxis is a disease of significant morbidity and mortality, predominantly affecting dialysis patients. The term 'calciphylaxis' was coined by Seyle et al. in 1961 to describe calcium deposition in the skin and subcutaneous soft tissue of uremic rats in response to 'triggers' (e.g. trauma, metallic salts) after exposure to 'sensitizing agents' (e.g. vitamin D and parathyroid hormone). In humans, calciphylaxis, however, is not a disorder of induced hypersensitivity. Instead, it is a disorder of cutaneous microvascular occlusion caused by thrombosis and calcification. Progressive, excruciatingly painful, non-healing wounds develop in these patients, pre-disposing them to high risk of sepsis and death. Calciphylaxis has no approved therapies. Increased awareness and research in this field have facilitated identification of risk factors and causation pathways. Development of therapeutic options and wound care management, however, are still at a nascent stage. Certain therapies have shown a promise that needs evaluation in prospective clinical trials. It is hoped that ongoing research will help us better understand the pathogenesis of this complex disease and develop efficacious treatment options. In this review, we outline the components involved in calciphylaxis diagnosis and treatment.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Falência Renal Crônica/complicações , Calcificação Vascular/diagnóstico , Calcificação Vascular/terapia , Calciofilaxia/etiologia , Humanos , Falência Renal Crônica/terapia , Diálise Renal/efeitos adversos , Fatores de Risco , Calcificação Vascular/etiologia
10.
J Cutan Pathol ; 46(1): 16-25, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30280393

RESUMO

BACKGROUND: Calciphylaxis is a rare, painful, and debilitating disorder of vascular calcification and skin necrosis that typically affects patients with advanced kidney disease. During our routine pathology practice, we noted several missed diagnoses on calciphylaxis consultation cases originating from outside institutions and sought to explore factors associated with false-negative pathologic diagnosis of calciphylaxis. METHODS: The pathology database of a large tertiary academic medical center was retrospectively searched for "calciphylaxis" in inside reports on outside surgical consultation cases between 2007 and 2017. Inside and outside pathology reports were compared and medical records were searched for calciphylaxis clinical diagnosis and risk factors. RESULTS: Twenty-four calciphylaxis patients were identified, with median age of 63.5 years. Seven of 24 (29%) of specimens were inadequate (e.g., lack of subcutaneous adipose tissue for evaluation). Eight of 17 (47%) of adequate specimens had a first false-negative pathologic diagnosis of calciphylaxis. Histochemical staining for calcium significantly correlated with true-positive diagnosis (93% vs 55%, P = 0.004). Dermatopathology fellowship training significantly correlated with true-positive diagnosis (82% vs 38%, P = 0.047). CONCLUSIONS: Adequate sampling, dermatopathology training, and use of histochemical stains to identify calcium associate with decreased false-negative rate for calciphylaxis diagnosis. These findings need further evaluation in larger prospective studies.


Assuntos
Calciofilaxia , Cálcio/metabolismo , Dermatopatias , Adulto , Idoso , Idoso de 80 Anos ou mais , Calciofilaxia/diagnóstico , Calciofilaxia/metabolismo , Calciofilaxia/patologia , Bases de Dados Factuais , Reações Falso-Negativas , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Dermatopatias/diagnóstico , Dermatopatias/metabolismo , Dermatopatias/patologia
12.
Ann Dermatol Venereol ; 145 Suppl 7: VIIS1-VIIS10, 2018 Dec.
Artigo em Francês | MEDLINE | ID: mdl-30583751

RESUMO

In this article, we performed a review of all new data of clinical relevance for the dermatologists that were published in the medical literature between September, 2017 and September, 2018. Besides advances that will be useful in day-to-day dermatological practice, we intended to give a more general perspective to this review by taking into account certain international health issues that might in final concern each of us in a globalized world. Focus were more particularly done on the following topics: infectious diseases (including sexually transmitted infections), neglected tropical diseases, adverse effects of drugs, bullous diseases, allergology, wound healing, dermatology on black skin, and public health dermatology.


Assuntos
Dermatopatias/diagnóstico , Dermatopatias/etiologia , Medicamentos Biossimilares/efeitos adversos , Calciofilaxia/diagnóstico , Dermatologia/tendências , Hipersensibilidade a Drogas/complicações , Hipersensibilidade a Drogas/diagnóstico , Receptores ErbB/antagonistas & inibidores , Hipersensibilidade Alimentar/diagnóstico , Humanos , Carne/efeitos adversos , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Dermatopatias/epidemiologia , Preparações Clareadoras de Pele/efeitos adversos , Inibidores de Simportadores de Cloreto de Sódio/efeitos adversos
13.
Nefrología (Madrid) ; 38(6): 579-586, nov.-dic. 2018. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-178387

RESUMO

La calcifilaxis, también denominada arteriolopatía urémica calcificante, es un síndrome raro que causa típicamente necrosis cutánea y que afecta principalmente a los pacientes en diálisis. La patogénesis es multifactorial y depende de la suma de todos los factores que producen calcificaciones ectópicas en el paciente con enfermedad renal crónica, como las alteraciones del metabolismo calcio-fósforo, el hiper o el hipoparatiroidismo, la diabetes, la obesidad, la inflamación sistémica y el uso de inhibidores de vitamina K, entre otros. Desde un punto de vista clínico, la calcifilaxis puede evolucionar desde una púrpura dolorosa hasta extensas áreas de necrosis cutánea que pueden sobreinfectarse y llegar a causar el fallecimiento del paciente por sepsis. El tratamiento se basa fundamentalmente en el manejo de las heridas, la eliminación de todos los elementos que puedan precipitar la calcificación ectópica y el uso de agentes inhibidores del proceso de calcificación


Calciphylaxis, also known as calcific uraemic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. Its pathogenesis is multifactorial and is the consequence of many factors causing ectopic calcifications in patients with chronic kidney disease, such as calcium-phosphate metabolism disorders, hyper- or hypo-parathyroidism, diabetes, obesity, systemic inflammation and the use of vitamin K antagonists, among others. From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. Treatment is primarily based on managing the wounds, eliminating all the possible precipitating factors of ectopic calcification and administering agents which are capable of inhibiting the process of calcification


Assuntos
Humanos , Insuficiência Renal Crônica/complicações , Calciofilaxia/etiologia , Insuficiência Renal Crônica/terapia , Diagnóstico Diferencial , Calciofilaxia/terapia , Fatores de Risco , Calciofilaxia/diagnóstico , Diálise/efeitos adversos
14.
Nephrol Ther ; 14(6): 439-445, 2018 Nov.
Artigo em Francês | MEDLINE | ID: mdl-30401462

RESUMO

Calcific uremic arteriolopathy, also called calciphylaxis, is a rare and severe disorder that presents with skin ischemia and necrosis, sometimes it presents with systemic necrosis, the process is secondary to the obliteration of the arterioles first by sub-intimal calcium deposits and then by thrombosis. These lesions can often lead to death due to infectious complications and comorbidities such as diabetes, obesity, arteritis, diffuse vascular calcifications, heart disease and undernutrition. The diagnosis is suggested by the characteristic ischemic skin lesions and their distribution, often bilateral and painful, associeted with calcific uremic arteriolopathy risk factors (phosphocalcic abnormalities, anti-vitamin K). The presence of radiological vascular calcifications is highly suggesting the diagnosis, but remains not very specific. The indication of skin biopsy is rare and reserved for difficult diagnoses. The goals of treatment are: reduce the extension of calcification and treatment of mineral and bone metabolism disorders of end-stage renal disease, dialysis adequacy, local treatment of skin lesions, tissue oxygenation, pain management, discontinuation and contraindication of medications that may contribute to the disorder. We propose to discuss it from a review of the literature and illustrate it with five clinical cases.


Assuntos
Calciofilaxia/diagnóstico , Diálise Renal/efeitos adversos , Calcificação Vascular/etiologia , Idoso , Arteríolas/patologia , Calciofilaxia/terapia , Diagnóstico Diferencial , Humanos , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Pele/patologia
15.
Nefrologia ; 38(6): 579-586, 2018.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30415999

RESUMO

Calciphylaxis, also known as calcific uraemic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. Its pathogenesis is multifactorial and is the consequence of many factors causing ectopic calcifications in patients with chronic kidney disease, such as calcium-phosphate metabolism disorders, hyper- or hypo-parathyroidism, diabetes, obesity, systemic inflammation and the use of vitamin K antagonists, among others. From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. Treatment is primarily based on managing the wounds, eliminating all the possible precipitating factors of ectopic calcification and administering agents which are capable of inhibiting the process of calcification.


Assuntos
Calciofilaxia/etiologia , Insuficiência Renal Crônica/complicações , Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Humanos , Diálise Renal , Insuficiência Renal Crônica/terapia , Fatores de Risco
17.
J Wound Ostomy Continence Nurs ; 45(6): 532-535, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30216332

RESUMO

BACKGROUND: Calciphylaxis is a potentially life-threatening condition involving painful necrotic skin ulcerations, especially of the lower extremities. It is generally associated with chronic kidney failure but may be seen in nonuremic cases. CASE REPORT: We report a case of calciphylaxis in a 60-year-old man with diabetes on dialysis for end-stage renal disease and known to have other typical combination of risk factors associated with calciphylaxis syndrome. On examination, he presented with multiple intensely painful calciphylaxis wounds on his legs and ankles. Despite regular wound management of his condition in our ambulatory care setting, his ulcers deteriorated. The patient's pain also increased and he was referred for hospital admission. CONCLUSION: This case presentation emphasizes the importance of an interdisciplinary team in the treatment of the complex, life-threatening cutaneous manifestations of calciphylaxis. When indicated, prompt referral to a hospital setting is necessary for appropriate care.


Assuntos
Calciofilaxia/diagnóstico , Falência Renal Crônica/complicações , Calciofilaxia/diagnóstico por imagem , Calciofilaxia/etiologia , Complicações do Diabetes , Diálise/métodos , Humanos , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Fatores de Risco
18.
Rom J Morphol Embryol ; 59(2): 591-594, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30173268

RESUMO

Calciphylaxis is a condition with unclear etiopathogeny with vascular calcifications and thrombotic occlusion that lead to necrotic lesions. It is usually described in patients with end-stage renal disease (ESRD), but also in other conditions. The mortality rate is high, due to sepsis and internal organ failure. We present the case of a patient with multiple comorbidities (ESRD, diabetes, hypertension, Mönckeberg's sclerosis) with problems of differential diagnostic due to the necrotic lesions that mislead initially to systemic lupus erythematosus (SLE) with necrotizing vasculitis.


Assuntos
Calciofilaxia/diagnóstico , Hiperparatireoidismo/complicações , Falência Renal Crônica/complicações , Calciofilaxia/patologia , Humanos , Hiperparatireoidismo/patologia , Falência Renal Crônica/patologia , Masculino , Pessoa de Meia-Idade
20.
Medicine (Baltimore) ; 97(27): e11300, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29979398

RESUMO

RATIONALE: Though to be rare, calcific uremic arteriolophathy (CUA) is an ectopic calcification entity causing pain and disabilities in patients with chronic renal insufficiency, thus increasing the morbidity and mortality. PATIENT CONCERN: We report a case of four years old boy admitted with acute respiratory failure. Physical examination revealed: irritability, purple subcutaneous hard nodules, tachypnea, dry spasmodic cough, respiratory rate 45/min, heart rate 110/min, blood pressure 100/60 mmHg, with normal heart sounds, no murmurs, hepatomegaly with hepato-jugular reflux. He was diagnosed at 2 years old with stage 5 chronic kidney disease due to untreated posterior urethral valve, and subsequently started peritoneal dialysis. He developed severe renal osteodystrophy, refractory to standard phosphate binders. DIAGNOSES: Pathology examination revealed the presence of diffuse calcifications involving the skin, brain, heart, lung, kidney, stomach and pancreas, consistent with the underlying diagnosis of CUA. INTERVENTION: Apart from standard treatment for end stage renal disease and associated co-morbidities, intensive care procedures have been initiated: oxygen therapy, continuous positive airway pressure, inotropic medication (Dopamine, Dobutamine), anticonvulsants (Diazepam), and antiedematous therapy (Dexamethasone). OUTCOME: His pulmonary function rapidly deteriorated up to the severe hypoxemia, seizures and cardio-respiratory arrest, despite the initiation of intensive care measures. LESSONS: A careful follow up of small children might detect in time an abnormal urinary pattern. The diagnosis of growth failure should also trigger urgent further investigation.


Assuntos
Calciofilaxia/diagnóstico , Falência Renal Crônica/complicações , Anticonvulsivantes/uso terapêutico , Calciofilaxia/complicações , Calciofilaxia/terapia , Cardiotônicos/uso terapêutico , Pré-Escolar , Pressão Positiva Contínua nas Vias Aéreas/métodos , Cuidados Críticos/métodos , Evolução Fatal , Glucocorticoides/uso terapêutico , Humanos , Falência Renal Crônica/terapia , Masculino , Oxigenoterapia/métodos , Diálise Renal , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia
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