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1.
Ann Pathol ; 2024 Oct 04.
Artigo em Francês | MEDLINE | ID: mdl-39368935

RESUMO

As in other organs, the diagnosis of endogenous cutaneous overload diseases is based on histopathological analysis of the lesions using special stainings, even if the clinical appearance is sometimes very suggestive. The lesions are sometimes very subtle and can be included in the group of "invisible" dermatoses, such as primary macular cutaneous amyloidosis or calciphylaxis. Superficial dermal melanosis or pigmentary incontinence generally reflects the post-inflammatory stage of a chronic or recurrent interface dermatitis. Section levels should be systematically performed to look for active lesions of diagnostic interest: Alcian blue staining to identify dermal mucinosis (connectivitis) and pan-T markers (fixed pigmented erythema, lichenoid mycosis fungoides, and vitiligo). Some pathologies have a prognostic impact, either because they reflect an underlying disease, monoclonal gammopathies, in particular myeloma, being one of the most common conditions in this context (AL amyloidosis, xanthoma and xanthogranuloma, scleromyxedema), or because they can be associated with visceral damage (AL amyloidosis, scleromyxedema). The clinical-pathological comparison is mandatory to rule out differential diagnoses, especially for life-threatening diseases: nodular amyloidosis and primary cutaneous amyloidosis versus systemic AL amyloidosis, papular mucinosis versus scleromyxedema and calcific panniculitis versus calciphylaxis.

2.
Indian J Nephrol ; 34(5): 522-523, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39372626

RESUMO

Calciphylaxis is a rare life-threatening condition characterised by systemic medial calcification of arterioles causing tissue ischemia with subsequent necrosis. Calciphylaxis occur in areas of increased adiposity like abdomen, proximal extremities, buttocks and breast. We report an elderly lady with end-stage kidney disease who presented with severe mastalgia with breast nodules and overlying skin changes. Calciphylaxis was diagnosed based on clinical, mammography and histo-pathological findings. Poor response to standard conservative management prompted us to use glucocorticoids showing dramatic improvement. She had a relapse after 3 months necessitating glucocorticoids again. We report the use of glucocorticoids as an option in recalcitrant, recurrent breast calciphylaxis.

3.
EClinicalMedicine ; 75: 102784, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39252867

RESUMO

Background: In the CALCIPHYX trial, we investigated hexasodium fytate, an inhibitor of vascular calcification, for the treatment of calcific uraemic arteriolopathy (calciphylaxis), a rare condition characterised by painful, non-healing skin lesions. Methods: In this international, phase 3, randomised, double-blind, placebo-controlled trial, adults with an ulcerated calciphylaxis lesion and pain visual analogue scale (VAS) score ≥50/100 were randomised 1:1 to hexasodium fytate 7 mg/kg or placebo intravenously during maintenance haemodialysis. Primary efficacy outcomes were an 8-item modification of the Bates-Jensen Wound Assessment Tool (BWAT-CUA) and Pain VAS in the intention-to-treat population. ClinicalTrials.gov number: NCT04195906. Findings: Overall, 34/37 patients randomised to hexasodium fytate and 26/34 patients randomised to placebo completed the 12-week randomised treatment period. At Week 12, both groups (hexasodium fytate versus placebo) showed similar improvements in BWAT-CUA (mean [standard deviation (SD)], -5.3 [5.2] versus -6.0 [6.2]; least squares mean difference, 0.3 [96% confidence interval (CI): -2.5, 3.0]; p = 0.88) and Pain VAS (mean [SD], -19.5 [26.9] versus -32.2 [38.5]; least squares mean difference, 11.5 [96% CI: -4.8, 27.8]; p = 0.15). One patient randomised to placebo briefly received hexasodium fytate in error. Serious adverse events through Week 12 included: calciphylaxis-related events leading to hospitalisation (2/38 [5%] versus 11/33 [33%]) and death (1/38 [3%] versus 5/33 [15%]). During the subsequent 12 weeks of open-label hexasodium fytate and 4 weeks of follow-up, there were no additional calciphylaxis-related events leading to hospitalisation. Over the course of the entire trial, deaths were 2/38 [5%] for the hexasodium fytate group and 7/33 [21%] for the placebo group. Interpretation: In patients with calciphylaxis, BWAT-CUA and Pain VAS improved similarly in hexasodium fytate- and placebo-treated patients; over the course of the entire trial, there were fewer deaths and calciphylaxis-related events leading to hospitalisation in the hexasodium fytate group. Funding: Funded by Sanifit, a CSL Vifor company.

4.
Cureus ; 16(9): e68866, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39246637

RESUMO

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rapidly progressive, rare, and severe condition characterized by vascular calcification and skin necrosis. The pathophysiology involves cutaneous arteriolar calcification followed by subsequent tissue ischemia and infarction, which eventually causes extremely painful skin lesions. The condition is associated with substantial morbidity due to severe pain, non-healing wounds, increased susceptibility to infections, and frequent hospitalizations. Calciphylaxis is a highly fatal condition with one-year mortality rates greater than 50%, most frequently due to sepsis. This report presents a case of a 63-year-old male with end-stage kidney disease (ESKD) who presented with altered mental status and was found to have notable necrotic skin ulcers on the bilateral anterior thighs, a stage IV sacral decubitus ulcer, and necrotic lesions on the scrotum and penis. This case underscores the importance of maintaining a high clinical suspicion for rare conditions like calciphylaxis in patients with multiple risk factors. Diagnosing the disease earlier in its course may improve outcomes and overall prognosis. Unfortunately, in this case, the patient presented too late into the disease course, and ultimately discussions/placement with palliative care were undertaken.

5.
J Nephrol ; 2024 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-39349919
6.
Cureus ; 16(9): e69677, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39301455

RESUMO

Calciphylaxis, a rare and life-threatening condition, involves the calcification and occlusion of microvasculature, leading to tissue ischemia and necrosis. The pathophysiology of calciphylaxis remains complex, but it is often associated with derangements in calcium and phosphate metabolism, ultimately resulting in the deposition of calcium within small blood vessels. This process leads to compromised blood flow, tissue hypoxia, and subsequent skin necrosis and ulceration, often with catastrophic consequences. While calciphylaxis typically occurs in individuals with end-stage renal disease (uremic calciphylaxis), it can also afflict those without renal impairment (non-uremic calciphylaxis). Several risk factors predispose individuals to this condition, including diabetes mellitus, hyperparathyroidism, malignancies, warfarin-based anticoagulation, alcoholic liver disease, and autoimmune disorders. Understanding the etiology, risk factors, and clinical manifestations of calciphylaxis is critical for timely diagnosis and management to mitigate its devastating effects. Management includes sepsis control, wound debridement, and analgesic support.  We report a case of penile calciphylaxis in a 58-year old male with a past medical history significant for end stage renal disease on hemodialysis, diabetes mellitus, and hypertension. The patient presented with a painful lesion on the glans penis which rapidly progressed to necrosis and gangrene with wet features. The patient refused partial penectomy and wanted conservative management with local wound debridement and antibiotics.

7.
Cureus ; 16(8): e67156, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39295658

RESUMO

Penile calciphylaxis is a rare and severe complication primarily observed in patients with end-stage renal disease (ESRD) undergoing dialysis. A 50-year-old man presented with severe penile pain and phimosis. He had a history of hypertension and diabetes mellitus for 10 years, complicated by ESRD and was awaiting a cadaveric kidney transplant. He was on cinacalcet therapy for tertiary hyperparathyroidism. The patient underwent circumcision at which discolouration and necrotic patches involving the glans penis were noted. The histological findings were consistent with calciphylaxis and suppurative inflammation. However, due to persistent severe pain and progressive gangrene, a partial penectomy was performed. This report demonstrates the importance of consideration of calciphylaxis in patients with ESRD when presenting with penile pain, even phimosis.

8.
Cureus ; 16(9): e69527, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39286468

RESUMO

A diagnosis of calciphylaxis is rare amongst the vulnerable population of patients with end-stage renal disease (ESRD); however, it has poor outcomes when it does present. With ineffective clearance due to reduced kidney function, calcium and phosphorus accumulate and deposit in the intimal layer of blood vessels and other soft tissues throughout the body. It can be proven using biopsy of skin lesions characteristic of the disease or with less invasive methods including X-ray and bone scintigraphy. Calciphylaxis is typically seen in middle-aged patients who have undergone prolonged dialysis treatment and has a devastating prognosis unless the patient can obtain a renal transplant. In this report, we present a case of a 30-year-old female patient with calciphylaxis and highlight the value of bone scintigraphy for diagnosis, while noting the importance of organ transplant for proper treatment.

9.
J Dtsch Dermatol Ges ; 2024 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-39118582

RESUMO

Calcinosis cutis is defined as the deposition of calcium salts in the skin. The dystrophic form is the most common and usually occurs in chronic inflammatory processes associated with collagenoses. Therapeutic options include surgical excision as well as a few pharmacological treatments. Overall, the evidence for the known therapeutic interventions is very limited and there is a lack of valid recommendations. Intravenous sodium thiosulfate has been used successfully in the treatment of calciphylaxis. In our case series, five patients with dystrophic calcinosis cutis received intravenous sodium thiosulfate for at least six cycles on five consecutive days per month, with single doses of 12.5 g and 25.0 g, respectively. A reduction in the calcified lesions could not be proven with certainty, but stable disease conditions were achieved. Intravenous sodium thiosulfate may counteract the progression of calcinosis cutis. The successful use of epicutaneously applied sodium thiosulfate, as described in the literature, suggests that a higher cutaneous bioavailability can be achieved to exert a lytic effect on calcinosis cutis. This is further supported by the reported efficacy of high-dose sodium thiosulfate in the treatment of calciphylaxis.

10.
Interact J Med Res ; 13: e53821, 2024 08 20.
Artigo em Inglês | MEDLINE | ID: mdl-39078624

RESUMO

BACKGROUND: Hyperbaric oxygen (HBO2) treatment is used across a range of medical specialties for a variety of applications, particularly where hypoxia and inflammation are important contributors. Because of its hypoxia-relieving and anti-inflammatory effects HBO2 may be useful for new indications not currently approved by the Undersea and Hyperbaric Medical Society. Identifying these new applications for HBO2 is difficult because individual centers may only treat a few cases and not track the outcomes consistently. The web-based International Multicenter Registry for Hyperbaric Oxygen Therapy captures prospective outcome data for patients treated with HBO2 therapy. These data can then be used to identify new potential applications for HBO2, which has relevance for a range of medical specialties. OBJECTIVE: Although hyperbaric medicine has established indications, new ones continue to emerge. One objective of this registry study was to identify cases where HBO2 has been used for conditions falling outside of current Undersea and Hyperbaric Medical Society-approved indications and present outcome data for them. METHODS: This descriptive study used data from a web-based, multicenter, international registry of patients treated with HBO2. Participating centers agree to collect data on all patients treated using standard outcome measures, and individual centers send deidentified data to the central registry. HBO2 treatment programs in the United States, the United Kingdom, and Australia participate. Demographic, outcome, complication, and treatment data, including pre- and posttreatment quality of life questionnaires (EQ-5D-5L) were collected for individuals referred for HBO2 treatment. RESULTS: Out of 9726 patient entries, 378 (3.89%) individuals were treated for 45 emerging indications. Post-COVID-19 condition (PCC; also known as postacute sequelae of COVID-19; 149/378, 39.4%), ulcerative colitis (47/378, 12.4%), and Crohn disease (40/378, 10.6%) accounted for 62.4% (n=236) of the total cases. Calciphylaxis (20/378, 5.3%), frostbite (18/378, 4.8%), and peripheral vascular disease-related wounds (12/378, 3.2%) accounted for a further 13.2% (n=50). Patients with PCC reported significant improvement on the Neurobehavioral Symptom Inventory (NSI score: pretreatment=30.6; posttreatment=14.4; P<.001). Patients with Crohn disease reported significantly improved quality of life (EQ-5D score: pretreatment=53.8; posttreatment=68.8), and 5 (13%) reported closing a fistula. Patients with ulcerative colitis and complete pre- and post-HBO2 data reported improved quality of life and lower scores on a bowel questionnaire examining frequency, blood, pain, and urgency. A subset of patients with calciphylaxis and arterial ulcers also reported improvement. CONCLUSIONS: HBO2 is being used for a wide range of possible applications across various medical specialties for its hypoxia-relieving and anti-inflammatory effects. Results show statistically significant improvements in patient-reported outcomes for inflammatory bowel disease and PCC. HBO2 is also being used for frostbite, pyoderma gangrenosum, pterygium, hypospadias repair, and facial filler procedures. Other indications show evidence for improvement, and the case series for all indications is growing in the registry. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.2196/18857.

11.
Cureus ; 16(6): e62304, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-39006691

RESUMO

Calcinosis cutis is a common ailment of the skin, caused by the accumulation of calcium salts in the subcutaneous regions, though there are rare case reports from rural Maharashtra region. This condition is usually asymptomatic and might manifest as a single growth or several different-sized growths. Its clinical presentation is observed as nodules or plaques without causing injury to underlying tissues. The condition is reported to be a secondary presentation to trauma, malignancies, and connective tissue diseases and has multifactorial underlying etiologies. Recommendation for treatments including both medical and surgical procedures is contingent upon the manifestation and severity of the condition. The final decision can be based on the results obtained from diagnostic modalities like fine needle aspiration and radiological imaging. This is the case of a 35-year-old male with a swelling on his left foot for the past two years without any associated medical history. The patient was managed by surgical incision and had a good recovery.

12.
J Am Acad Dermatol ; 91(5): 834-842, 2024 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38972479

RESUMO

BACKGROUND: Calciphylaxis patients historically have experienced diagnostic challenges and high morbidity; however limited data is available examining these characteristics over time. OBJECTIVE: The primary goals were to a) investigate factors associated with diagnostic delay of calciphylaxis and b) assess morbidity outcomes. The secondary goal was to provide updated mortality rates. METHODS: A retrospective review of 302 adult patients diagnosed with calciphylaxis between January 1, 2006 and December 31, 2022 was conducted. Univariate and multivariate statistical analyses were performed. RESULTS: Nonnephrogenic calciphylaxis (P = .0004) and involvement of the fingers (P = .0001) were significantly associated with an increased diagnostic delay, whereas involvement of the arms (P = .01) and genitalia (P = .022) resulted in fewer days to diagnosis. Almost all patients with genitalia, finger, or toe involvement had nephrogenic disease. The number of complications per patient decreased with time, especially for wound infections (P = .028), increase in lesion number (P = .012), and recurrent hospitalizations (P = .020). Updated 1-year mortality rates were 36.70% and 30.77% for nephrogenic and nonnephrogenic calciphylaxis, respectively. LIMITATIONS: Limitations include the retrospective nature and data from a single institution. CONCLUSION: Diagnostic delay, particularly in nonnephrogenic calciphylaxis, and complications per patient decreased with time, highlighting the importance of continued awareness to expedite diagnosis. Mortality rates have continued to improve in recent years.


Assuntos
Calciofilaxia , Diagnóstico Tardio , Humanos , Calciofilaxia/mortalidade , Calciofilaxia/diagnóstico , Calciofilaxia/complicações , Estudos Retrospectivos , Masculino , Feminino , Diagnóstico Tardio/estatística & dados numéricos , Pessoa de Meia-Idade , Idoso , Adulto
13.
J Surg Case Rep ; 2024(6): rjae404, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38835946

RESUMO

Calciphylaxis is a disorder causing ischemic skin necrosis, typically associated with end-stage renal disease or those receiving dialysis. Occurrence is rare in those without end-stage renal disease, and treatment options are limited. This case report describes a patient with calciphylaxis without end-stage renal disease or history of dialysis. Treatment with sodium thiosulfate, a first line option, had to be stopped due to metabolic derangements, limiting the healing process. Diagnosis and treatment of this rare disorder are important to prevent further complications that may result.

14.
Medicina (B.Aires) ; Medicina (B.Aires);84(2): 106-205, jun. 2024. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1564773

RESUMO

Resumen Introducción : La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica me dia 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 ca racterísticas 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 di cumarínicos. 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.


Abstract Introduction : Calciphylaxis is a serious vascular dis order characterized by calcification of tunica media, in timal 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 stud ies 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, labo ratory 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. Sixty-one percent were men and 39% were NUC cases. Eighty-two 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 diagno sis 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 pa tients 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.

15.
Cureus ; 16(4): e58492, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38765385

RESUMO

Calciphylaxis is a unique medical condition characterized by calcification of the medial layer of arterioles and soft tissues in a patient's skin at the level of the dermis and subcutaneous adipose tissue. The rate of progression of calciphylaxis is rapid, starting with a reduction of blood flow that leads to ischemic changes in the skin that can manifest as painful cutaneous erythematous nodules or plaques and later as skin ulceration. The majority of patients affected by calciphylaxis have predisposing comorbidities such as end-stage renal disease with a long history of hemodialysis and electrolyte abnormalities in calcium, phosphate, and parathyroid hormone levels. This report presents the case of a 72-year-old female patient on hemodialysis who developed calciphylaxis. The methods for early prognosis (the methods of early diagnosis), including clinical presentation, risk factors, imaging techniques, and laboratory investigations, are discussed. The presented case is particularly noteworthy given the onset of calciphylaxis within a mere three months of initiating hemodialysis, a timeline significantly shorter than the typically observed period in most patients. (The case detailed in this report outlines the rapid onset of calciphylaxis in a patient who was receiving hemodialysis for only three months.) This patient with early-onset calciphylaxis highlights the unpredictable nature of calciphylaxis and the need for increased clinical vigilance even in the initial stages of hemodialysis.

16.
Kidney Int Rep ; 9(4): 951-959, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38765561

RESUMO

Introduction: Calciphylaxis is a rare disorder associated with significant morbidity and mortality. Data registries are an invaluable source of information for rare diseases. We reviewed cases of calciphylaxis recorded in the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) and evaluated associations and outcomes of this condition. Methods: Data was obtained on all cases of calciphylaxis reported between 2019 and 2022 in Australian and New Zealand patients on kidney replacement therapy (KRT). This cohort was compared to all patients in the registry who received KRT from 2019 to 2022 without an episode of calciphylaxis. Cox proportional hazards regression including a time-varying covariate for calciphylaxis episode was conducted for mortality with models restricted to patients on dialysis only. Results: From 2019 to 2022, 333 patients had calciphylaxis episodes reported. Overall incidence rate for patients on dialysis was 4.5 (4.1-5.1) episodes per 1000 patient-years on dialysis. Median age was 63 (interquartile range [IQR]: 55-73) years, 54% were female, 66% had diabetes, 59% were obese (body mass index [BMI] ≥ 30 kg/m2) and 77% were receiving hemodialysis (HD) treatment. Compared to patients without calciphylaxis (n = 46,526), patients with calciphylaxis were more likely to be older, female, and have diabetes, greater BMI, coronary artery, and peripheral vascular disease. The median time to calciphylaxis was 3.2 (IQR: 0.9-6.7) years after KRT commencement. Half of the patients with calciphylaxis died by 12 months from diagnosis. Adjusted hazard ratio (HR) of mortality for patients on dialysis with calciphylaxis <1 year and 1 to 4 years after an episode was 5.8 (4.9-6.9) and 1.5 (1.0-2.1), respectively compared to patients on dialysis without calciphylaxis. Conclusion: Calciphylaxis is a rare but life-threatening condition in people on KRT with the greatest mortality burden within 12 months of diagnosis.

18.
Medicina (B Aires) ; 84(2): 196-205, 2024.
Artigo em Espanhol | MEDLINE | ID: mdl-38683504

RESUMO

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.


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.


Assuntos
Calciofilaxia , Humanos , Estudos Retrospectivos , Masculino , Feminino , Fatores de Risco , Pessoa de Meia-Idade , Calciofilaxia/terapia , Calciofilaxia/patologia , Calciofilaxia/mortalidade , Calciofilaxia/diagnóstico , Idoso , Adulto , Idoso de 80 Anos ou mais , Anticoagulantes/uso terapêutico , Resultado do Tratamento , Argentina/epidemiologia
19.
J Palliat Med ; 27(9): 1279-1283, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38629826

RESUMO

Guidelines are lacking for patients with calciphylaxis on renal replacement therapy, often leading to difficulty optimally treating these patients. A 60-year-old male veteran receiving hemodialysis presented with calciphylaxis of the left lower extremity and intractable pain. His condition was complicated by chronic back pain, long-term opioid therapy, and psychological trauma history. He was ultimately transferred to a calciphylaxis treatment center but was unable to tolerate further treatments due to sepsis and hemodynamic instability. He was transitioned to comfort measures and died in the hospital. Addressing complicated pain physiologies and complex trauma is challenging even in well-resourced tertiary medical centers. Despite the availability of calciphylaxis therapies and trauma-informed care, there remains a high rate of suffering and mortality in this patient population. There is much work to be done in this cohort, particularly when considering the implications of past traumatic experiences on health care engagement and pain management.


Assuntos
Calciofilaxia , Dor Intratável , Veteranos , Humanos , Masculino , Calciofilaxia/etiologia , Calciofilaxia/terapia , Pessoa de Meia-Idade , Veteranos/psicologia , Dor Intratável/etiologia , Dor Intratável/psicologia , Diálise Renal , Trauma Psicológico/complicações , Evolução Fatal , Manejo da Dor/métodos
20.
Cureus ; 16(2): e55161, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38558589

RESUMO

Calciphylaxis is a rare and severe medical condition characterized by the calcification of small blood vessels and soft tissues, leading to tissue damage, skin ulcers, and intense pain. It most commonly affects individuals with underlying health conditions such as kidney disease, particularly end-stage renal disease (ESRD), and is associated with high mortality rates. Understanding the diagnosis and management of calciphylaxis is crucial for improving patient outcomes. Diagnosing calciphylaxis can be challenging due to its rarity and overlapping symptoms with other skin conditions. Healthcare professionals typically use a combination of clinical evaluation and diagnostic tests to reach a conclusive diagnosis. The management of calciphylaxis is multifaceted and typically involves a collaborative effort between various healthcare specialists, including nephrologists, dermatologists, and wound care experts. The primary goals of treatment are to alleviate pain, promote wound healing, address underlying causes, and prevent further complications.

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