Calciphylaxis: Pathogenesis and Therapy

1998 ◽  
Vol 2 (4) ◽  
pp. 245-248 ◽  
Author(s):  
Richard L. Worth
Keyword(s):  
Clinical Manifestations ◽  
Disease Process ◽  
Skin Lesions ◽  
Corticosteroid Administration ◽  
Inappropriate Treatment ◽  
Important Diagnostic Tool ◽  
Subcutaneous Tissues ◽  
Recent Debate ◽  
Stage Renal Disease ◽  
End Stage

Background: Calciphylaxis is a rare, painful and debilitating disease in which calcification of the skin and subcutaneous tissues or of internal organs can lead to skin necrosis, discolouration, and other skin lesions. The typical patient has end-stage renal disease (ESRD) and hyperparathyroidism. Selye originally characterized this syndrome in rats and distinguished it from other syndromes of abnormal calcification by the following sequence: sensitizers, latent period, and challengers. There has been recent debate regarding misdiagnosis and failure to differentiate this category of patients even in clinical studies. Objective: In this article the clinical manifestations of calciphylaxis are described; the importance of distinguishing this condition from other calcification syndromes is explained; risk factors, sensitizers, and challenges are reviewed; treatments of choice are discussed; and the merits of parathyroidectomy are evaluated. Conclusion: It is important to consider calciphylaxis in a differential diagnosis of calcification syndromes and in treating patients with ESRD and hyperparathyroidism, because early diagnosis and treatment can interrrupt the progression of the disease process; the disease is painful and debilitating; and inappropriate treatment such as corticosteroid administration may aggravate the condition. Skin biopsy is an important diagnostic tool when the condition is suspected. Parathyroidectomy may be justified because the untreated disease itself has significant rates of morbidity and mortality and because this treatment occasionally leads to dramatic clinical improvement.

scholarly journals Removing the problem: parathyroidectomy for calciphylaxis

2018 ◽  
Vol 11 (1) ◽  
pp. e226696 ◽  
Author(s):  
Mohamed Hassanein ◽  
Heather Laird-Fick ◽  
Richa Tikaria ◽  
Saleh Aldasouqi
Keyword(s):  
Treatment Options ◽  
Sodium Thiosulfate ◽  
Skin Lesions ◽  
Phosphate Binders ◽  
Treatment Resistant ◽  
Hungry Bone Syndrome ◽  
Calcific Uremic Arteriolopathy ◽  
Stage Renal Disease ◽  
End Stage ◽  
Hungry Bone

Calcific uremic arteriolopathy (CUA), widely known as calciphylaxis, is a rare and lethal disease that usually affects patients with end-stage renal disease. It is characterised by widespread vascular calcification leading to tissue ischaemia and necrosis and formation of characteristic skin lesions with black eschar. Treatment options include sodium thiosulfate, cinacalcet, phosphate binders and in resistant cases, parathyroidectomy. We report a case of recurrent, treatment-resistant CUA successfully treated with parathyroidectomy. Her postoperative course was complicated by hungry bone syndrome and worsening of her wounds before they completely healed. We then discuss the morbidity of CUA, including the controversy around the use of parathyroidectomy and risk of aggressive management of hungry bone syndrome.

Skin Examination: An Important Diagnostic Tool in Renal Failure Patients

2018 ◽  
Vol 45 (1-3) ◽  
pp. 187-193 ◽  
Author(s):  
Karen M. Van de Velde-Kossmann
Keyword(s):  
Renal Failure ◽  
Renal Disease ◽  
Genetic Disorders ◽  
The United States ◽  
Underlying Disease ◽  
Careful Examination ◽  
Common Skin ◽  
Important Diagnostic Tool ◽  
Stage Renal Disease ◽  
End Stage

Renal failure is common in the United States with an estimated prevalence of 660,000 treated end-stage renal disease patients in 2015 [<xref ref-type="bibr" rid="ref1">1</xref>]. Causes of renal failure are many, and complications from renal failure, underlying disease, and treatment are not infrequent. Examples of common skin manifestations include xerosis, pigmentary change, and nail dystrophies. Frequent disease-specific skin changes may be helpful in the diagnosis of primary disorders leading to renal disease or severity of disease including bullosis diabeticorum, sclerodactyly, or leukoctoclastic vasculitis. Some cutaneous changes, such as the multiple angiokeratomas of Fabry disease or the plexiform neurofibromas of neurofibromatosis, are pathognomonic of genetic disorders, which often lead to renal failure. Careful examination of the skin can provide crucial clues to diagnosis of renal failure causation and aid in monitoring complications.

Staphylococcus-induced glomerulonephritis: potential role for corticosteroids

2021 ◽  
Vol 14 (1) ◽  
pp. e237011
Author(s):  
Rui Filipe Nogueira ◽  
Nuno Oliveira ◽  
Vítor Sousa ◽  
Rui Alves
Keyword(s):  
Kidney Function ◽  
Potential Role ◽  
Graft Infection ◽  
Clinical Manifestations ◽  
Rapidly Progressive Glomerulonephritis ◽  
Vascular Graft Infection ◽  
Systemic Corticosteroids ◽  
Life Threatening ◽  
Stage Renal Disease ◽  
End Stage

Staphylococcus aureus is a troublesome pathogen, responsible for a broad range of clinical manifestations, ranging from benign skin infections to life-threatening conditions such as endocarditis and osteomyelitis. The kidney can be affected through a rapidly progressive glomerulonephritis mediated by an inflammatory reaction against a superantigen deposited in the glomerulus during the infection’s course. This glomerulopathy has a poor prognosis, often leading to chronically impaired kidney function, eventually progressing to end-stage renal disease. Treatment rests on antibiotherapy. Despite the inflammatory role in this disease’s pathophysiology, most authors discourage a simultaneous immunosuppressive approach given the concomitant infection. However, there are some reports of success after administration of systemic corticosteroids in these patients. We present a 66-year-old man with a staphylococcus-induced glomerulonephritis brought on by a vascular graft infection, with rapidly deteriorating kidney function despite extraction of the infected graft and 3 weeks of antibiotherapy with achievement of infection control. Kidney function improved after the introduction of corticosteroids. This case highlights the potential role of corticosteroids in selected cases of staphylococcus-induced glomerulonephritis, particularly those in which the infection is under control.

La diagnosi precoce di malattia

2019 ◽  
Vol 31 (1) ◽  
pp. 58-60
Author(s):  
Federica Rossi ◽  
Federico Pieruzzi
Keyword(s):  
Wide Spectrum ◽  
Delayed Diagnosis ◽  
Clinical Manifestations ◽  
The Body ◽  
Lysosomal Storage ◽  
Cerebrovascular Complications ◽  
Risk Patients ◽  
Alpha Galactosidase ◽  
Stage Renal Disease ◽  
End Stage

Anderson-Fabry disease is an X-linked, lysosomal, storage disorder characterized by the decreased activity of alpha-Galactosidase A, which results in accumulation of globotriaosylceramide (Gb-3) in cells and tissues throughout the body, leading to a wide spectrum of clinical manifestations. Patients are often misdiagnosed or diagnosed late in their life. This is due to the phenotypic heterogeneity, the poor awareness of this rare disease, and many pitfalls when making a differential diagnosis, in adulthood, as well as in the early stages. Delayed diagnosis has significant clinical implications, because the progression of the disease over time can lead to irreversible end-stage renal disease and life-threatening cardiovascular or cerebrovascular complications. Early diagnosis remains essential in order to start an early treatment and reduce the progression of the disease, thus maximizing the chance to improve patient outcomes. Newborn screening, high-risk patients’ identification, and increasing pediatricians’ and clinicians’ knowledge on this condition, are good strategies to avoid late referrals of Anderson-Fabry patients to reference centers.

P0078INVESTIGATION THE VARIANTS AT THE BINDING SITE OF INFLAMMATORY TRANSCRIPTION FACTOR IN PATIENTS WITH ESRD

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
SUI-LUNG SU
Keyword(s):  
Transcription Factor ◽  
Binding Site ◽  
Disease Process ◽  
Binding Activity ◽  
Luciferase Reporter ◽  
Healthy Controls ◽  
Chip Assay ◽  
Potential Binding ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims Inflammation is an important factor for enhancing the disease process from chronic kidney disease (CKD) to end-stage renal disease (ESRD). Nuclear factor-kappa B (NF-κB) is a transcription factor that regulates the expression of genes involved in inflammation. We investigated the potential association with the gene polymorphism of transcription factor binding site of NF-κB in ESRD patients. Method We used the Taiwan Biobank database, University of California, Santa Cruz, reference genome, chromatin immunoprecipitation sequencing database to find the SNPs at potential binding sites of NF-κB. In addition, we performed a case–control study and genotyped 847 patients with ESRD and 846 healthy controls at Tri-Service-General-Hospital from 2015 to 2016. Further we used ChIP-assay and Luciferase reporter assay to identify the binding activity at different genotype. Results Results of biometrics screening in the databases revealed 15 SNPs with the potential binding site of NF-κB. Genotype distributions of rs9395890 were significantly different in ESRD cases and healthy controls (P = 0.032). In the Dominant model, rs9395890 with T allele had a higher risk of ESRD (P = 0.032; odds ratio [OR] = 1.32, 95% confidence interval [CI] = 0.99–1.76). The ChIP assay reveals that around 1.49 times enrichment of NF-κB of the variant type TT when compared to that of the wild type GG in the rs9395890 (P&lt;0.027; TT=3.20±0.16, GT=2.81±0.20, GG=1.71±0.18,) and the luciferase activity curve showed T allele was higher than G allele. Conclusion In conclusion, we demonstrate that rs9395890 may be associated with ESRD in the Taiwanese population.

scholarly journals Clinical Manifestations of Cardiac Disease in Patients with End Stage Renal Disease Under Maintenance Hemodialysis in a Tertiary Level Hospital of Nepal

2016 ◽  
Vol 15 (1) ◽  
pp. 61-65
Author(s):  
Kumar Roka ◽  
Pratibha Bista Roka
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Low Voltage ◽  
Clinical Manifestations ◽  
Left Ventricular Diastolic Dysfunction ◽  
Left Ventricular ◽  
Maintenance Hemodialysis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Introduction: End stage renal disease presents with multiple clinical and systemic manifestations. The aim of the present study was to identify the early cardiac and other morbidities in end stage renal disease (ESRD) patients who were under maintenance hemodialysis.Methods: This was an observational, prospective study conducted in fifty established ESRD patients of 20 to 74 years under maintenance hemodialysis in Nephrology unit of Shree Birendra Hospital. Clinical examination, laboratory parameters, electrocardiogram and echocardiography findings were used to identify the morbidities. Results: Among all patients enrolled in the study 88.7% had anemia, 64.2 % systolic murmurs, 62.26 % pedal edema, 73.6 % fatiguability, 71.7 % angina, 24.4 % palpitations and 13.2 % had breathlessness on exertion.  62.26% of the patients had hypertension and 13.20 % had diabetes. In the electrocardiogram, prolonged QTc was observed in 10.4%, followed by T wave inversion in 9.4 % and finally low voltage complex comprised 7.6 %. The echocardiogram showed left ventricular diastolic dysfunction in 58.5 %, left ventricular hypertrophy (overall type) 49 % and valvular lesion like mitral regurgitation and tricuspid regurgitation 83 % and 58.5 % respectively. Conclusion: Cardiac co-morbidities are common in patients diagnosed with ESRD on maintenance hemodialysis.

scholarly journals Calciphylaxis in a Patient on Home Hemodialysis

2020 ◽  
Vol 8 ◽  
pp. 232470962092271 ◽  
Author(s):  
Sreedhar Adapa ◽  
Srikanth Naramala ◽  
Vijay Gayam ◽  
Narayana Murty Koduri ◽  
Subba Rao Daggubati ◽  
...  
Keyword(s):  
Wound Care ◽  
Sodium Thiosulfate ◽  
Skin Lesions ◽  
Current Treatment ◽  
Home Hemodialysis ◽  
Calcium Phosphorus ◽  
Calcific Uremic Arteriolopathy ◽  
Stage Renal Disease ◽  
End Stage ◽  
Subcutaneous Adipose

Calciphylaxis is a rare and severe complication characterized by calcification of arterioles and capillaries in the dermis and subcutaneous adipose tissue that leads to ischemia, necrosis, and painful skin lesions in patients with end-stage renal disease (ESRD). It is also known as calcific uremic arteriolopathy. Calciphylaxis occurs most commonly with the ESRD with skin ulceration as a predominant presenting feature. Calcium-phosphorus dysregulation in dialysis patients are traditionally considered as a risk factor for the development of calciphylaxis. The involvement of an integrated interdisciplinary and multifaceted approach is key to the success of the calciphylaxis treatment. We present a case of a 51-year-old female with ESRD on home hemodialysis who developed calciphylaxis, which was successfully managed with increasing dialysis prescription, diligent wound care, and sodium thiosulfate infusion. Management of calciphylaxis in a patient receiving home hemodialysis has never been reported as per the review of the literature. Calciphylaxis is a sporadic disease, frequently encountered in the patients undergoing hemodialysis and carries a very grave prognosis. Current treatment is rarely effective, so preventive strategies play an important role by modifying the risk factors that promote the development of calciphylaxis.

scholarly journals Calciphylaxis of the Postmenopausal Female Breast: An Uncommonly Encountered Mimic of Carcinoma

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Aaron G. Novotny ◽  
Ashley B. Simpson ◽  
Melinda A. Kral ◽  
Benjamin C. Calhoun ◽  
Amy E. Cocco ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Breast Lesion ◽  
Multidisciplinary Approach ◽  
Medical Condition ◽  
Skin Lesions ◽  
Female Breast ◽  
Stage Renal Disease ◽  
End Stage ◽  
Postmenopausal Female

Calciphylaxis is a serious medical condition that is typically associated with end-stage renal disease and presents as the sequelae of calcifications in arterioles with subsequent ischemia of affected tissues. Classically, calciphylaxis produces ulcerated and necrotic skin lesions. These may be biopsied to aid in confirmation of the diagnosis. Herein we report a case of a large necrotic breast lesion in the clinical setting of calciphylaxis, and we emphasize that a multidisciplinary approach to diagnosis and management is important to avoid unnecessary oncological resection.

scholarly journals Clinical Manifestations and Diagnostic Challenges in Acute Porphyrias

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Henry Trier ◽  
Vikram P. Krishnasamy ◽  
Pashtoon Murtaza Kasi
Keyword(s):  
End Stage Renal Disease ◽  
Laboratory Testing ◽  
Biosynthetic Pathway ◽  
Clinical Manifestations ◽  
Definitive Diagnosis ◽  
Enzyme Deficiency ◽  
Different Types ◽  
Acute Porphyrias ◽  
Stage Renal Disease ◽  
End Stage

The porphyrias are a group of disorders characterized by an enzyme deficiency in the heme biosynthetic pathway. These can be classified into either erythropoietic or hepatic forms depending on the site of the major enzyme deficiency. The diagnosis of acute porphyrias, however, can be very challenging due to overlapping features amongst the various types. Initial suspicion is based on a myriad of clinical manifestations, which then are confirmed by laboratory testing where available. Genetic testing is now also available for the different types of porphyrias, aiding in the definitive diagnosis. Here, we present a challenging case of porphyria in a patient with end-stage renal disease and present the diagnostic challenges associated with the case and the ways forward.

scholarly journals Soluble Urokinase Receptor and the Kidney Response in Diabetes Mellitus

2017 ◽  
Vol 2017 ◽  
pp. 1-9 ◽  
Author(s):  
Ranadheer R. Dande ◽  
Vasil Peev ◽  
Mehmet M. Altintas ◽  
Jochen Reiser
Keyword(s):  
Molecular Model ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Urokinase Receptor ◽  
Cellular Targets ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Novel Therapeutic Strategies ◽  
Stage Renal Disease ◽  
End Stage

Diabetic nephropathy (DN) is the leading cause of end-stage renal disease (ESRD) worldwide. DN typically manifests by glomerular hyperfiltration and microalbuminuria; then, the disease progresses to impaired glomerular filtration rate, which leads to ESRD. Treatment options for DN include the strict control of blood glucose levels and pressure (e.g., intraglomerular hypertension). However, the search for novel therapeutic strategies is ongoing. These include seeking specific molecules that contribute to the development and progression of DN to potentially interfere with these “molecular targets” as well as with the cellular targets within the kidney such as podocytes, which play a major role in the pathogenesis of DN. Recently, podocyte membrane protein urokinase receptor (uPAR) and its circulating form (suPAR) are found to be significantly induced in glomeruli and sera of DN patients, respectively, and elevated suPAR levels predicted diabetic kidney disease years before the occurrence of microalbuminuria. The intent of this review is to summarize the emerging evidence of uPAR and suPAR in the clinical manifestations of DN. The identification of specific pathways that govern DN will help us build a more comprehensive molecular model for the pathogenesis of the disease that can inform new opportunities for treatment.

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