scholarly journals Chronic renal disease is more prevalent in patients with hemolytic uremic syndrome who had a positive history of diarrhea

2010 ◽  
Vol 78 (6) ◽  
pp. 598-604 ◽  
Author(s):  
Ajay P. Sharma ◽  
Guido Filler ◽  
Prabo Dwight ◽  
William F. Clark
Keyword(s):  
Renal Disease ◽  
Hemolytic Uremic Syndrome ◽  
Chronic Renal Disease ◽  
Positive History ◽  
History Of ◽  
Uremic Syndrome

scholarly journals SP188PREDICTIVE FEATURES OF CHRONIC RENAL DISEASE IN ATYPICAL HEMOLYTIC UREMIC SYNDROME

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii166-iii166
Author(s):  
Matthieu Jamme ◽  
Quentin Raimbourg ◽  
Dominique Chauveau ◽  
Amélie Seguin ◽  
Claire Presne ◽  
...  
Keyword(s):  
Renal Disease ◽  
Hemolytic Uremic Syndrome ◽  
Chronic Renal Disease ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Uremic Syndrome

scholarly journals Eculizumab discontinuation in atypical hemolytic uremic syndrome: TMA recurrence risk and renal outcomes

2021 ◽  
Author(s):  
Gema Ariceta ◽  
Fadi Fakhouri ◽  
Lisa Sartz ◽  
Benjamin Miller ◽  
Vasilis Nikolaou ◽  
...  
Keyword(s):  
Family History ◽  
Hemolytic Uremic Syndrome ◽  
Univariate Analysis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Response ◽  
Pathogenic Variants ◽  
Increased Risk ◽  
History Of ◽  
Uremic Syndrome

ABSTRACT Background Eculizumab modifies the course of disease in patients with atypical hemolytic uremic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited. Methods Patients enrolled in the Global aHUS Registry who received ≥1 month of eculizumab before discontinuing, demonstrated hematologic or renal response prior to discontinuation and had ≥6 months of follow-up were analyzed. The primary endpoint was the proportion of patients suffering thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: eGFR changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function. Results We analyzed 151 patients with clinically diagnosed aHUS who had evidence of hematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants, or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; 7 (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation. Conclusions Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.

scholarly journals Familial Atypical Hemolytic Uremic Syndrome with Positive pS1191L (c.3572C> T) Mutation in CFH A Single-Center Experience

2019 ◽  
Author(s):  
Fadime ERSOY DURSUN ◽  
Gözde YESIL ◽  
Hasan DURSUN ◽  
Gülşah SASAK
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Family History ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Gene Mutations ◽  
Factor H ◽  
The Other ◽  
History Of ◽  
Uremic Syndrome

Abstract Background: Atypical hemolytic uremic syndrome is a condition characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury, which can exhibit a poor prognosis. Gene mutations play a key role in this disease, which may be sporadic or familial. Methods: We studied, 13 people from the same family were investigated retrospectively for gene mutations of familial atypical hemolytic uremic syndrome after a patient presented to our emergency clinic with atypical hemolytic uremic syndrome and reported a family history of chronic renal failure. Results: The pS1191L mutation in the complement factor H gene was heterozygous in 6 people from the family of the patient with atypical hemolytic uremic syndrome. One of these people was our patient with acute renal failure and the other two are followed up by the Nephrology Clinic due to chronic renal failure. The other 3 persons showed no evidence of renal failure. The index case had a history of 6 sibling deaths; two of them died of chronic renal failure. Plasmapheresis and fresh frozen plasma treatment was given to our patient. When patient showed no response to this treatment, eculizumab therapy was started. Conclusions: The study demonstrated that a thorough family history should be taken in patients with atypical hemolytic uremic syndrome. These patients may have familial type of the disease and they should be screened genetically. Eculizumab should be the first choice in the treatment with plasmapheresis. It should be kept in mind that the use of eculizumab as prophylaxis in post-transplant therapy is extremely important for prevention of rejection.

Ischemic Colitis and Stricture After Hemolytic-Uremic Syndrome

PEDIATRICS ◽  
1978 ◽  
Vol 61 (2) ◽  
pp. 315-317
Author(s):  
Hadi Sawaf ◽  
Marcia J. Sharp ◽  
Kum J. Youn ◽  
Patrick A. Jewell ◽  
Ali Rabbani
Keyword(s):  
Renal Failure ◽  
Hemolytic Uremic Syndrome ◽  
Sudden Onset ◽  
Late Complications ◽  
Surgical Removal ◽  
Review Of The Literature ◽  
Follow Up Studies ◽  
History Of ◽  
Uremic Syndrome

The hemolytic-uremic syndrome (HUS) was first described by Von Gasser et al.1 in 1955 as a syndrome of acute renal failure, hemolytic anemia, and thrombocytopenia in children. Follow-up studies on HUS have emphasized hypertension and uremia as late complications.2,3 A review of the literature has revealed no previously reported cases of persistent colitis and bowel stenosis after HUS. We present a child who continued to have intermittent intestinal obstruction and diarrhea until surgical removal of a segment of colon almost seven months after the onset of HUS. CASE REPORT A 26-month-old white boy who had no history of gastrointestinal disturbance had sudden onset of diarrhea with blood and mucus in the stool.

Renal Emergencies in Children

2020 ◽  
pp. 302-310
Author(s):  
Liza Kearl ◽  
Maureen McCollough
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Severity Of Illness ◽  
Streptococcal Pharyngitis ◽  
Acute Glomerulonephritis ◽  
Wide Range ◽  
History Of ◽  
Uremic Syndrome ◽  
Tract Infections ◽  
Bloody Stools

Renal emergencies in pediatric patients range from more common conditions such as urinary tract infections to rarer conditions such as hemolytic uremic syndrome. This chapter reviews emergency conditions that are less commonly seen, with potentially significant sequelae, and with possible nonspecific or more subtle presentations. Acute kidney injury can be due to a wide range of causes, including benign gastroenteritis and post-streptococcal or toxin-related causes. Less common but more serious conditions, such as nephrotic syndrome or Henoch–Schönlein purpura, present with nonspecific signs such as edema or rash. Acute glomerulonephritis needs to be considered in a child with a history of streptococcal pharyngitis or skin infection. Hemolytic uremic syndrome is typically Shiga toxin-related and should be considered in any ill-appearing child, especially those presenting with bloody stools. Disposition of a child with a renal emergency will depend on the severity of illness, laboratory results, and the ability to follow-up with their primary care provider or specialist.

scholarly journals Renal Pelviceal Keratinizing Squamous Metaplasia with Sparing of Pyramidal Zones

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Richard H. Siderits ◽  
Jared Fingerman ◽  
Anup Hazra ◽  
Cheryl Rimmer ◽  
Marc Colaco ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Urinary Tract ◽  
Renal Disease ◽  
Renal Pelvis ◽  
Chronic Renal Disease ◽  
Squamous Metaplasia ◽  
Upper Urinary Tract ◽  
Muscularis Mucosa ◽  
Chronic Infections ◽  
History Of

Metaplastic changes in the urothelium of the upper urinary tract are relatively infrequent. Metaplasia may present as either squamous or less often glandular differentiation. The process may be associated with chronic inflammation or associated chronic infections. There may be malignant transformation to either squamous cell carcinoma or adenocarcinoma. The demarcation of the metaplastic process in the minor calyces has not been well documented to date. We report the case of a 74-year-old female patient who presented with a history of chronic renal disease and acute pyohydronephrosis. The patient underwent a nephroureterectomy which revealed keratinizing desquamative squamous metaplasia throughout the renal pelvis and upper urinary tract with abrupt termination of metaplasia at the junction of the renal pelvis and the minor calyx (pyramidal zone). Immunohistochemical evaluation documents metaplastic urothelium stained positive for CK5, before converting sharply to simple cuboidal epithelium in the minor calyx (pyramidal zones) which stained positive CK7. At the junction of the metaplastic components and low cuboidal lined minor calyceal surfaces, the underlying stroma showed loss of ureteral muscularis mucosa with transition to renal parenchymal type stroma. We believe that this observation is unique and potentially relevant to the etiology and pathophysiology of pelviceal metaplasia.

scholarly journals Atypical Hemolytic Uremic Syndrome Presenting as Acute Heart Failure—A Rare Presentation: Diagnosis Supported by Skin Biopsy

2019 ◽  
Vol 7 ◽  
pp. 232470961984290
Author(s):  
Asim Kichloo ◽  
Savneek Singh Chugh ◽  
Sanjeev Gupta ◽  
Jay Pandav ◽  
Praveen Chander
Keyword(s):  
Heart Failure ◽  
Skin Biopsy ◽  
Hemolytic Uremic Syndrome ◽  
Thrombotic Microangiopathy ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Systolic Heart Failure ◽  
Kidney Injury ◽  
Rare Presentation ◽  
History Of ◽  
Uremic Syndrome

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder of uncontrolled complement activation that manifests classically as anemia, thrombocytopenia, and renal failure, although extrarenal manifestations are observed in 20% of the patient most of which involving central nervous system, with relatively rare involvement of the heart. In this article, we report the case of a 24-year-old male with no history of heart disease presenting with acute systolic heart failure along with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given his presentation of thrombotic microangiopathy (TMA), along with laboratory results significant for low haptoglobin, platelets, hemoglobin, C3, C4, CH50, and normal ADAMTS13 levels, with no diarrhea and negative STEC polymerase chain reaction in stool, aHUS diagnosis was established with strong clinical suspicion, and immediate initiation of treatment was advised. Kidney biopsy to confirm diagnosis of aHUS was inadvisable because of thrombocytopenia, so the skin biopsy of a rash on his arm was done, which came to be consistent with thrombotic microangiopathy. Our case highlights a relatively rare association between aHUS and cardiac involvement, and the use of skin biopsy to support diagnosis of aHUS in patients who cannot undergo renal biopsy because of thrombocytopenia.

scholarly journals Baseline Demographics and Characteristics of 466 Patients with Atypical Hemolytic Uremic Syndrome in the Global aHUS Registry

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4204-4204
Author(s):  
Christoph Licht ◽  
Gianluigi Ardissino ◽  
Gema Ariceta ◽  
David Cohen ◽  
Christoph Gasteyger ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Board Of Directors ◽  
Research Funding ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Organ Damage ◽  
Advisory Committees ◽  
Life Threatening ◽  
History Of ◽  
Uremic Syndrome ◽  
The Uk

Abstract Introduction:Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, life-threatening disease of chronic, uncontrolled complement activation leading to thrombotic microangiopathy, renal, and other end-organ damage. Started in April 2012, the aHUS Registry is an observational, non-interventional, multicenter, global registry designed to collect information on patients (pts) with aHUS. By making follow-up data on the aHUS indication for eculizumab (ECU) available, the Registry fulfills postmarketing regulatory requirements while also highlighting the need for and benefit of a sponsor/academia partnership. An independent Scientific Advisory Board ensures data are made accessible for publication. Herein, we report baseline demographics and clinical characteristics of pts enrolled in the aHUS Registry. Methods:Pts of all ages with a clinical diagnosis of aHUS (irrespective of treatment) are eligible for enrollment into the Registry. An identified complement abnormality is not required. Demographic, medical and disease history, treatment, and efficacy and safety outcomes data are collected at Registry enrollment and prospectively thereafter. Results:By July 1, 2014, 466 pts (from Australia, Austria, Belgium, Canada, France, Germany, Israel, Italy, Russia, Spain, Sweden, Switzerland, the UK, and USA) were enrolled in the aHUS Registry. Of these, 261 (56.0%) were treated with ECU and 286 (61.4%) were ≥18 years of age. Family history of aHUS, prior kidney grafts, dialysis, plasma exchange/plasma infusion (PE/PI), and renal impairment were assessed at baseline in ECU- and non–ECU-treated pts (Table). Mean time from aHUS diagnosis to ECU initiation was 2.5 years. Sixty-five pts (24.9%) discontinued ECU; of these, 8 (12.3%) restarted. Conclusions:Since the public disclosure of data from the last aHUS Registry update, pt enrollment has continued to increase. Ongoing and future analyses will further clinicians’ understanding of the history and progression of aHUS. Additional clinical sites are encouraged to enroll pts into the aHUS Registry to facilitate knowledge acquisition and optimization of pt care and quality of life. Disclosures Licht: Achillon Pharmaceuticals: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Alexion Pharmaceuticals: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Ardissino:Alexion Pharmaceuticals: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Ariceta:Alexion Pharmaceuticals: Consultancy, Membership on an entity's Board of Directors or advisory committees. Cohen:Alexion Pharmaceuticals: Consultancy. Gasteyger:Alexion Pharma International Sàrl: Employment, Equity Ownership. Greenbaum:Alexion Pharmaceuticals: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Ogawa:Alexion Pharmaceuticals: Employment. Schaefer:Alexion Pharmaceuticals: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding. Vande Walle:Alexion Pharmaceuticals: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Fremeaux-Bacchi:Alexion Pharmaceuticals: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; CLS Behring: Honoraria.

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