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Calciphylaxis

CUA


Presentation

  • We present a case of calciphylaxis leading to lower gastrointestinal (GI) bleeding and rectal ulceration of the GI mucosa.[ncbi.nlm.nih.gov]
Hypoxemia
  • His pulmonary function rapidly deteriorated up to the severe hypoxemia, seizures and cardio-respiratory arrest, despite the initiation of intensive care measures.[ncbi.nlm.nih.gov]
Cough
  • 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[ncbi.nlm.nih.gov]
Tachypnea
  • 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[ncbi.nlm.nih.gov]
Hepatomegaly
  • 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[ncbi.nlm.nih.gov]
Severe Osteoporosis
  • We present a 51-year-old woman with alcoholic cirrhosis who developed multiorganic calciphylaxis shortly after starting teriparatide treatment associated with calcium and 25-hydroxyvitamin D supplements for severe osteoporosis.[ncbi.nlm.nih.gov]
Diplopia
  • A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness.[ncbi.nlm.nih.gov]
Blepharoptosis
  • A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness.[ncbi.nlm.nih.gov]
Lacrimation
  • Fundus exam and brain magnetic resonance imaging (MRI) showed normality of optic nerves, chiasma, retrochiasmatic area, ocular muscles, eyeballs, lacrimal glands, periorbital fat, cavernous sinuses, and occipital cortex.[ncbi.nlm.nih.gov]
Eczema
  • […] acanthoma),Pilar tumor;Malignant proliferating trichilemmal cyst/tumor,Proliferating tricholemmal cystic squamous cell carcinoma 増殖性外毛根鞘(性)嚢腫(増殖性外毛根鞘嚢腫性棘細胞腫);悪性増殖性外毛根鞘(性)嚢腫,増殖性外毛根鞘嚢腫性有棘細胞癌 144.P rurigo chronica multiforme 多形慢性痒疹 145.P rurigo nodularis,Eczema[mall-md.seikyou.ne.jp]
  • […] acanthoma),Pilar tumor;Malignant proliferating trichilemmal cyst/tumor,Proliferating tricholemmal cystic squamous cell carcinoma 増殖性外毛根鞘(性)嚢腫(増殖性外毛根鞘嚢腫性棘細胞腫);悪性増殖性外毛根鞘(性)嚢腫,増殖性外毛根鞘嚢腫性有棘細胞癌 144.Prurigo chronica multiforme 多形慢性痒疹 145.Prurigo nodularis,Eczema[nankodo.co.jp]
Bitemporal Headache
  • Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis.[ncbi.nlm.nih.gov]
Kidney Failure
  • It is predominantly seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function.[ncbi.nlm.nih.gov]
  • Urgent Kidney Transplantation as Successful Treatment of Calciphylaxis in Three Patients with End-Stage Kidney Failure. Am J Transplant. 2016;16 (suppl 3).[atcmeetingabstracts.com]
  • People who have calciphylaxis usually have kidney failure and are on dialysis or need a kidney transplant. The condition can also occur in people without kidney disease.[delawareonline.com]
  • Diabetes and hypertension are major causes of kidney failure, although Huber notes that many kidney failure patients are not obese.[news-medical.net]
Incontinence
  • Over the course of 2 months, his wound worsened in the setting of continued urinary incontinence, and before planned diversion with a suprapubic catheter, he progressed to gangrene and sepsis.[ncbi.nlm.nih.gov]
Urinary Incontinence
  • Over the course of 2 months, his wound worsened in the setting of continued urinary incontinence, and before planned diversion with a suprapubic catheter, he progressed to gangrene and sepsis.[ncbi.nlm.nih.gov]

Workup

Rectal Ulceration
  • We present a case of calciphylaxis leading to lower gastrointestinal (GI) bleeding and rectal ulceration of the GI mucosa.[ncbi.nlm.nih.gov]

Treatment

  • Most of the current treatments for non-uraemic calciphylaxis are derived from uraemic calciphylaxis treatment protocols.[ncbi.nlm.nih.gov]

Prognosis

  • Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis.[jasn.asnjournals.org]
  • Calciphylaxis is a rare syndrome of vascular calcification with thrombosis that occurs most often in patients with end-stage renal disease, and it frequently portends a guarded prognosis.[ncbi.nlm.nih.gov]

Etiology

  • It is most often seen in patients with renal failure among many other occasionally reported etiologies.[ncbi.nlm.nih.gov]
  • Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis[jasn.asnjournals.org]

Epidemiology

  • Department, Guy's and St Thomas' Hospitals, London, UK. 5 Division of Nephrology, Ambroise Paré University Hospital, APHP, University of Paris Ouest-Versailles-St-Quentin-en-Yvelines (UVSQ), Boulogne-Billancourt, Paris INSERM U1018, Research Centre in Epidemiology[ncbi.nlm.nih.gov]
  • As high as 85% mortality from secondary infections leading to sepsis Synonym(s): calcific uremic arteriolopathy; uremic gangrene syndrome; calcifying panniculitis Epidemiology Incidence 3.5 new cases/1,000 patient-years among patient with ESRD on chronic[unboundmedicine.com]
  • These theories remain speculative. [8, 11] Epidemiology Frequency Calciphylaxis is an uncommon condition that affects 1-4% of the population with ESRD. [18, 19] A concern exists that the incidence has increased during the last decade because of a number[emedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory.[jasn.asnjournals.org]
  • The etiology and pathophysiology of calciphylaxis aren't well understood. There are many risk factors, and the reported median survival time is 2.6 months after diagnosis.[ncbi.nlm.nih.gov]

Prevention

  • As the pathogenesis of calciphylaxis is better understood, targeted therapies aimed at disease prevention and reversal will follow.[ncbi.nlm.nih.gov]
  • Calciphylaxis Prevention This is an incurable and fatal condition, which means preventing the occurrence of the disorder is very important for individuals at risk of developing these lesions.[hxbenefit.com]

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