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1.
BMJ Case Rep ; 14(5)2021 May 11.
Artigo em Inglês | MEDLINE | ID: mdl-33975849

RESUMO

Calcinosis cutis is an unusual disorder characterised by calcium-phosphate deposition into cutaneous and subcutaneous tissues. There are five subtypes: dystrophic, metastatic, idiopathic, iatrogenic and calciphylaxis. Our objective was to report a patient with a history of haemodialysis-dependent end-stage renal disease (ESRD) who developed metastatic calcinosis cutis, which is usually associated with abnormalities of calcium-phosphate metabolism in renal failure. In our patient, the serum calcium and phosphate levels were normal. Additionally, we describe the diagnostic approach and the difficulties involved in the management of calcinosis cutis in patients with haemodialysis-dependent ESRD.


Assuntos
Calcinose , Calciofilaxia , Transplante de Rim , Dermatopatias , Calcinose/etiologia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Humanos , Pele , Dermatopatias/etiologia
2.
BMJ Case Rep ; 14(4)2021 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-33827879

RESUMO

Calciphylaxis is commonly associated with end-stage renal disease (ESRD) and renal transplant. We present a rare case of early onset calciphylaxis in a patient presenting with acute kidney injury (AKI) secondary to anti-glomerular basement membrane (anti-GBM) antibody disease. A 65-year-old obese Caucasian woman with type 2 diabetes mellitus and hypertension presented with a 1-month history of painless gross haematuria and worsening lower extremity oedema. Laboratory results indicated AKI and nephrotic-range proteinuria. Anti-glomerular antibodies were elevated. Renal biopsy revealed focal crescentic glomerulonephritis with linear capillary immunoglobulin G staining consistent with anti-GBM antibody disease. She was treated with haemodialysis, plasmapheresis, steroids, bumetanide and cyclophosphamide. Two months later, she developed necrotic lesions on bilateral thighs. Wound biopsy was consistent with calciphylaxis. This case highlights that calciphylaxis, usually seen in patients with chronic kidney disease or ESRD, can manifest in patients with AKI as well.


Assuntos
Injúria Renal Aguda , Doença Antimembrana Basal Glomerular , Calciofilaxia , Diabetes Mellitus Tipo 2 , Glomerulonefrite , Injúria Renal Aguda/etiologia , Idoso , Doença Antimembrana Basal Glomerular/complicações , Autoanticorpos , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Feminino , Hemorragia , Humanos , Pneumopatias
3.
BMJ Case Rep ; 14(2)2021 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-33637497

RESUMO

A woman in her 60s with a history of lower extremity vascular disease presented with extreme pain and wounds in her legs which had kept her from walking for several weeks. The patient's pain became intolerable throughout her hospital stay despite multiple surgical revascularisations. Biopsy of the patient's calf wounds revealed evidence of calciphylaxis, a diagnosis which corresponds with this patient's extreme pain. Our patient had no history of end-stage renal disease.


Assuntos
Calciofilaxia , Falência Renal Crônica , Biópsia , Calciofilaxia/etiologia , Feminino , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Dor
5.
Rev Med Suisse ; 17(727): 394-398, 2021 Feb 24.
Artigo em Francês | MEDLINE | ID: mdl-33625805

RESUMO

Calciphylaxis is a rare but devastating condition characterized by a calcifying thrombosing microangiopathy resulting in painful necrotic skin lesions. Risk factors are multiple, the most important being obesity, disorders of phosphocalcic metabolism and acenocoumarol. Largely unknown by the medical community, its pathogenesis is still incompletely elucidated; its diagnosis by skin biopsy remains difficult and increasingly debated because potentially associated with an aggravation of lesions. Its treatment must be as premature as possible andmultimodal. However, the results are up to now unsatisfactory, as specific treatment of calciphylaxis does not yet exist.


Assuntos
Calciofilaxia , Doenças Vasculares , Biópsia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Feminino , Humanos , Rim , Necrose , Gravidez
6.
Dermatol Ther ; 34(2): e14769, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33421241

RESUMO

Calciphylaxis is a rare condition characterized by skin ulceration and necrosis as a result of vascular calcification of the small and medium blood vessels of skin and subcutaneous tissues. It mainly occurs in patients with advanced chronic kidney disease and sometimes leads to complications with a fatal outcome. In this report, we describe the case of a 67-year-old male patient with end stage renal disease presenting painful skin ulcers on his lower limbs. The lesions had progressively grown and were associated to severe pain and decreased quality of life. The ulcers did not respond to conventional treatments and the patient underwent skin biopsy of these lesions obtaining anatomopathological findings compatible with calciphylaxis. In this report, we present an innovative treatment for skin ulcers secondary to calciphylaxis using cryopreserved amniotic membrane (AM) as a dressing in order to promote epithelialization of the wounds. After four applications, healing of the main ulcer and reduction in pain was achieved. In summary, applying cryopreserved AM probed to be a promising strategy to reduce pain and to enhance epithelialization and healing of chronic non-responsive ulcers in calciphylaxis.


Assuntos
Calciofilaxia , Falência Renal Crônica , Úlcera Cutânea , Idoso , Âmnio , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Masculino , Qualidade de Vida , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/etiologia , Úlcera Cutânea/terapia
10.
Cutis ; 105(1): E11-E14, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32074157

RESUMO

Calciphylaxis is a potentially fatal disease caused by metastatic calcification of the small- and medium-sized blood vessels of the dermis and subcutis. It most commonly is seen in patients with renal disease requiring dialysis, but it also may be triggered by nonuremic causes in patients with known risk factors for calciphylaxis. We report a case of nonuremic calciphylaxis (NUC) occurring in the setting of multiple risk factors, including chronic corticosteroid use, obesity, rapid weight loss, and hypotension. A review of the literature also is provided with an in-depth discussion of the known risk factors and triggers of NUC.


Assuntos
Calciofilaxia/diagnóstico , Hipotensão/complicações , Perda de Peso/fisiologia , Idoso , Calciofilaxia/etiologia , Feminino , Glucocorticoides/administração & dosagem , Humanos , Obesidade/complicações , Fatores de Risco
11.
G Ital Nefrol ; 37(1)2020 Feb 12.
Artigo em Italiano | MEDLINE | ID: mdl-32068359

RESUMO

Primary hyperoxaluria (PH) is a rare genetic disorder with autosomal recessive transmission, characterized by high endogenous production and markedly excessive urinary excretion of oxalate (Ox). It causes the accumulation of calcium oxide crystals in organs and tissues including bones, heart, arteries, skin and kidneys, where it may cause oxalo-calcic nephrolithiasis, nephrocalcinosis and chronic renal failure. Some forms are secondary to enteric diseases, drugs or dietetic substances, while three primitive forms, caused by various enzymatic defects, are currently known: PH1, PH2 and PH3. An early diagnosis, with the aid of biochemical and genetic investigations, helps prevent complications and establish a therapeutic strategy that often includes liver and liver-kidney transplantation, improving the prognosis of these patients. In this work we describe the clinical case of a patient with PH1 undergoing extracorporeal hemodialysis treatment and we report the latest research results that could change the life of patients with PH.


Assuntos
Calciofilaxia/terapia , Hiperoxalúria Primária/genética , Hiperoxalúria Primária/terapia , Diálise Renal/métodos , Dermatopatias Metabólicas/terapia , Transaminases/genética , Calciofilaxia/etiologia , Calciofilaxia/patologia , Compostos de Cálcio/metabolismo , Feminino , Glioxilatos/metabolismo , Hemodiafiltração/métodos , Humanos , Hiperoxalúria Primária/diagnóstico , Falência Renal Crônica/etiologia , Transplante de Rim , Pessoa de Meia-Idade , Nefrocalcinose/etiologia , Nefrocalcinose/terapia , Uso Off-Label , Oxalatos/metabolismo , Óxidos/metabolismo , Dermatopatias Metabólicas/etiologia , Dermatopatias Metabólicas/patologia , Tiossulfatos/uso terapêutico
12.
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 Plaquetária/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
13.
J Dermatolog Treat ; 31(5): 545-548, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31075991

RESUMO

Background: Calciphylaxis is associated with end-stage renal failure or kidney transplant, although, cases of non-uremic calciphylaxis have been reported. It is a rare disorder, with high mortality, characterized by vascular calcification within cutaneous vessels; vascular calcification can occur in other organs, besides skin, like heart, lung, or pancreas, which is named visceral calciphylaxis.Objective: The purpose was to review current knowledge regarding diagnosis and therapeutic approach to cutaneous calciphylaxis.Methods: A literature review has been conducted associated to word "calciphylaxis".Results: The diagnosis is based mainly on clinical features and histologic findings. Therapeutic options are still controversial.Conclusions: Cutaneous calciphylaxis is a puzzling disease, with a challenging diagnosis, and a complex treatment, which requires a multidisciplinary team and expertise.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Cálcio/metabolismo , Guias como Assunto , Humanos , Oxigenação Hiperbárica , Falência Renal Crônica/complicações , Falência Renal Crônica/patologia , Transplante de Rim/efeitos adversos , Pele/patologia
14.
Wounds ; 32(11): 294-298, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33465045

RESUMO

INTRODUCTION: Calciphylaxis is a rare, highly morbid pathological syndrome of vascular calcification and tissue necrosis. It is predominantly seen in patients with end-stage renal disease (ESRD) on chronic dialysis. There is no definitive standard of care for calciphylaxis, and the overall prognosis for patients, particularly those with ulcerated lesions, is bleak. One important role of wound care clinicians during the COVID-19 pandemic is to ensure that the continuity of care of an at-risk population is maintained while limiting the patient's potential exposure to the virus. Innovative therapies paired with alternative treatment sites of service are one such method. CASE REPORT: A 56-year-old female with ESRD on at-home peritoneal dialysis (PD) presented to the outpatient wound clinic with a punch biopsy-proven calciphylaxis lesion. Within days, state-wide "shelter-at-home" orders due to the COVID-19 pandemic went into effect. To prevent disruption in care and to minimize risk to the patient, the lesion was treated with bi-weekly self-application of a continuous topical oxygen therapy (cTOT) device paired with weekly telemedicine visits. The wound completely resolved after 9 weeks of topical oxygen therapy with no complications or device malfunctions. CONCLUSIONS: This case, to the authors' knowledge, is the first to document healing in a calciphylaxis wound with the use of cTOT. Topical oxygen therapy may be a beneficial adjunctive therapy in the treatment of wounds caused by calciphylaxis. Finding creative ways to navigate this current health care crisis is essential to help mitigate risk for vulnerable patients with advanced comorbidities.


Assuntos
COVID-19/complicações , Calciofilaxia/terapia , Oxigênio/uso terapêutico , Diálise Peritoneal/efeitos adversos , Ferimentos e Lesões/terapia , Administração Tópica , Calciofilaxia/complicações , Calciofilaxia/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Oxigênio/administração & dosagem , Quarentena , Cicatrização , Ferimentos e Lesões/etiologia
16.
G Ital Nefrol ; 36(5)2019 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-31580546

RESUMO

Calcific uremic arteriolopathy (CUA) is a highly morbid condition usually found in ESRD patients that has rarely been reported after renal transplantation and renal function restoration. Furthermore, little is known about the optimal management of CUA in this setting. Herein, we report on the clinical case of AB, a 70-year-old woman who developed CUA after renal transplantation and renal function restoration. However, other risk factors for CUA such as diabetes and warfarin treatment, due to mechanical aortic valve implantation, were present. Thirty-eight months after renal transplantation she developed erythema and livedo reticularis in both legs and a gradually enlarging skin ulcer in the right leg. A skin biopsy of the ulcer showed features compatible with the CUA, such as sub-intimal calcification and luminal obstruction of the small dermal arterioles, tissue ischemia and signs of adipocytes degeneration. A multidisciplinary approach was adopted, including medical and non-medical treatments such as surgical debridement and vacuum-assisted closure therapy. Medical treatments included a five weeks course of once a week intravenous infusion of pamidronate and intravenous sodium thiosulfate (STS) at increasing doses. Four months after beginning the therapy with STS, a complete healing of the ulcer on the right leg and the disappearance of the livedo reticularis on the left leg was noted. In conclusion, although rare CUA may develop also in renal transplanted patients, a timely and combined therapeutic approach is essential for its resolutive treatment. Sodium thiosulfate therapy has proven to be effective and tolerated.


Assuntos
Calciofilaxia/terapia , Transplante de Rim/efeitos adversos , Úlcera da Perna/terapia , Doenças Raras/terapia , Idoso , Anticoagulantes/uso terapêutico , Conservadores da Densidade Óssea/administração & dosagem , Calciofilaxia/etiologia , Quelantes/administração & dosagem , Terapia Combinada/métodos , Diabetes Mellitus , Feminino , Humanos , Úlcera da Perna/etiologia , Livedo Reticular/etiologia , Livedo Reticular/terapia , Pamidronato/administração & dosagem , Doenças Raras/etiologia , Fatores de Risco , Tiossulfatos/administração & dosagem , Varfarina/uso terapêutico
17.
BMJ Case Rep ; 12(8)2019 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-31471365

RESUMO

A 70-year-old man with history of metastatic well-differentiated neuroendocrine carcinoma was presented to the hospital with a painful left lower extremity ulcer which started around 3 months prior to presentation. He was treated with antibiotics for cellulitis on multiple occasions with no improvement in his symptoms. On initial laboratory evaluation, he was found to have acute kidney injury and a normal calcium level. The patient underwent a skin biopsy and was found to have cellulitis and calciphylaxis of small-sized and medium-sized vessels. Since the patient did not have any underlying risk factors of calciphylaxis, the most likely cause of his calciphylaxis was thought to be his underlying malignancy. Physicians should keep this differential in mind while treating non-healing ulcers in such patients since they are at higher risk of superimposed infections and usually require aggressive wound care.


Assuntos
Injúria Renal Aguda/etiologia , Calciofilaxia/etiologia , Carcinoma Neuroendócrino/complicações , Úlcera da Perna/etiologia , Idoso , Humanos , Masculino
18.
Am J Case Rep ; 20: 1170-1174, 2019 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-31395848

RESUMO

BACKGROUND Calciphylaxis is a rare and fatal systemic disease which most commonly occurs in patients with end-stage renal disease. It is a poorly understood vascular calcification with unclear pathology that leads to vascular compromise due to vascular occlusion with endoluminal calcification. CASE REPORT We report a case of a 61-year-old male with chronic kidney disease stage 5 who developed calciphylaxis. The patient was diagnosed with dry gangrene of the second and third digits of the right hand and second, third, and fourth phalanges of the left hand. Despite medical therapy and local wound care, the lesions progressively worsened with time. The patient was found to have secondary hyperparathyroidism (parathyroid hormone was 1028 pg/mL) and underwent subtotal parathyroidectomy. In our patient, the skin lesions due to calciphylaxis completely resolved over the course of 12 months. CONCLUSIONS Parathyroidectomy has been associated with clinical benefit in patients with calciphylaxis. Clinicians should consider parathyroidectomy in the setting of high parathyroid hormone and calciphylaxis. Although parathyroidectomy is an effective treatment option for calciphylaxis it is not a definitive treatment and calciphylaxis can occur, though rarely, even after parathyroidectomy. There is a need to do further studies in order to confirm the efficacy of parathyroidectomy.


Assuntos
Calciofilaxia/etiologia , Dedos/patologia , Gangrena/etiologia , Hiperparatireoidismo Secundário/etiologia , Hiperparatireoidismo Secundário/cirurgia , Paratireoidectomia , Insuficiência Renal Crônica/complicações , Calciofilaxia/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade
20.
Curr Opin Nephrol Hypertens ; 28(5): 448-454, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31169527

RESUMO

PURPOSE OF REVIEW: Calciphylaxis is a disorder of cutaneous microvascular calcification and thrombosis leading to chronic, excruciatingly painful, progressive wounds with a high risk of sepsis and death. The diagnosis and treatment of calciphylaxis presents significant challenges. A poorly understood disease, the management of calciphylaxis has mostly been restricted to wound management and a few novel therapies. Data from patient registries and new studies on causal pathways is stimulating the development of pathogenesis-based medical therapies. RECENT FINDINGS: Much needed clinical trials are now underway to examine the safety and efficacy of sodium thiosulfate and other therapeutics for the indication of calciphylaxis. There is emerging data suggesting a potential role of therapeutic anticoagulation in these patients. There has also been a renewed emphasis on patient-oriented outcomes, such as improvement of pain scores and quality-of-life indices. SUMMARY: This review highlights ongoing clinical trials studying therapeutic options in calciphylaxis and emphasizes the causal pathways that led to the development of such therapies.


Assuntos
Calciofilaxia/terapia , Anticoagulantes/uso terapêutico , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Cinacalcete/uso terapêutico , Ensaios Clínicos como Assunto , Humanos , Manejo da Dor , Paratireoidectomia , Tiossulfatos/uso terapêutico
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