scholarly journals A rare presentation of Kikuchi Disease with Hemolytic Uremic Syndrome

2019 ◽  
Vol 35 (2) ◽  
Author(s):  
Salwa Tauseeq Khan ◽  
Rubina Naqvi ◽  
Rahma Rashid ◽  
Sana Abbas Naqvi
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Case Reports ◽  
Kidney Injury ◽  
Rare Condition ◽  
Pathological Findings ◽  
Rare Presentation ◽  
Benign Condition ◽  
Creative Commons ◽  
Uremic Syndrome ◽  
Open Access Article

Kikuchi disease (KD) or also known as Kikuchi Fujimoto disease is named after scientists Kikuchi and Fujimoto who describe the disease in Japan in 1972. KD originally reported from Asia but later case reports from different regions of world have been published. It is a benign condition of necrotizing histiocytic lymphadenitis which mimic like Lymphoma, diagnosis of KD is based on histo-pathological findings from lymphnodes. It is a rare condition and mostly case reports have been published, it can have an association with other pathologies. We aim to report a case where KD has been found in a young woman in association with hemolytic uremic syndrome and acute kidney injury. How to cite this:Khan ST, Naqvi R, Rashid R, Naqvi SA. A rare presentation of Kikuchi Disease with Hemolytic Uremic Syndrome. Pak J Med Sci. 2019;35(2):586-588. doi: https://doi.org/10.12669/pjms.35.2.735 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Eculizumab Modifies Outcomes in Adults with Atypical Hemolytic Uremic Syndrome with Acute Kidney Injury

2018 ◽  
Vol 48 (3) ◽  
pp. 225-233 ◽  
Author(s):  
Mercedes Cao ◽  
Bruna N. Leite ◽  
Tamara Ferreiro ◽  
María Calvo ◽  
Constantino Fernández ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Kidney Function ◽  
Case Reports ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Complete Recovery ◽  
Genetic Abnormalities ◽  
Uremic Syndrome ◽  
Meta Analyses

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with congenital or acquired genetic abnormalities that result in uncontrolled complement activation, leading to thrombotic microangiopathy and kidney failure. Until recently, the only treatment was plasma exchange or plasma infusion (PE/PI), but 60% of patients died or had permanent kidney damage despite treatment. Eculizumab, a complement inhibitor, has shown promising results in aHUS. However, data are mainly extracted from case reports or studies of heterogeneous cohorts, and no direct comparison with PE/PI is available. Methods: An observational retrospective study of adult, dialysis-dependent aHUS patients with acute kidney injury (AKI) who were treated with either PE/PI alone or with second-line eculizumab in our center. We compared the effect of PE/PI and eculizumab on kidney function, hypertension, proteinuria, hematologic values, relapse, and death. Results: Thirty-one patients were included (females, 18; sporadic aHUS, 29; mean age, 46 ± 20 years). Twenty-six patients were treated with PE/PI alone, and 5 were deemed to be plasma-resistant and received eculizumab after stopping PE/PI. Among patients receiving eculizumab, 80% attained complete recovery of kidney function, 100% stopped dialysis, 20% had decreased proteinuria, and no patient relapsed (vs. 38.5, 50, 15.4, and 11.5%, respectively, of patients receiving only PE/PI). At 1-year of follow-up, no deaths had occurred in either group. Conclusion: Eculizumab shows greater efficacy than PE/PI alone for the treatment of adult aHUS patients with AKI. Prospective studies and meta-analyses are warranted to confirm our findings and set guidelines for treatment, monitoring, and maintenance.

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.

Tissue-resident macrophages mediate neutrophil recruitment and kidney injury in shiga toxin-induced hemolytic uremic syndrome.

2021 ◽  
Author(s):  
Julia K. Lill ◽  
Stephanie Thiebes ◽  
Judith-Mira Pohl ◽  
Jenny Bottek ◽  
Nirojah Subramaniam ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Kidney Injury ◽  
Neutrophil Recruitment ◽  
Uremic Syndrome

scholarly journals Successful Remission of Hemolytic-Uremic Syndrome during the Third-line Weekly Gemcitabine for Metastatic Breast Cancer

2014 ◽  
Vol 8 ◽  
pp. BCBCR.S14920
Author(s):  
Victor C. Kok ◽  
Sheng-Chung Wu ◽  
Chien-Kuang Lee
Keyword(s):  
Breast Cancer ◽  
Metastatic Breast Cancer ◽  
Hemolytic Uremic Syndrome ◽  
Cancer Progression ◽  
Palliative Chemotherapy ◽  
Metastatic Breast ◽  
Kidney Injury ◽  
Peripheral Blood Smear ◽  
Uremic Syndrome ◽  
Third Line

Sequential palliative chemotherapy for metastatic breast cancer incorporating weekly gemcitabine administered as three-weeks-on, one-week-off schedule is widely adopted throughout the East Asia region. Hemolytic-uremic syndrome (HUS) associated with weekly gemcitabine for a breast cancer patient is extremely rare. We report here a case of 43-year-old woman with metastatic breast cancer who received weekly gemcitabine as a third-line palliative chemotherapy for her disease. She developed HUS after a cumulative dose of 11,000 mg/m2 gemcitabine, evidenced by microangiopathic hemolytic anemia (MAHA) with schistocytes seen in peripheral blood smear, decreased haptoglobin level (<0.29 mmol/L), thrombocytopenia, negative direct Coombs test, and acute kidney injury. Owing to the ease of administration of weekly gemcitabine, gemcitabine-induced thrombocytopenia, multifactorial anemia in metastatic breast cancer, and possibility of cancer progression, HUS could have gone unnoticed. Breast cancer oncologist should be cognizant of this rare HUS even during weekly gemcitabine treatment.

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.

Novel presentation of Plasmodium vivax malaria with acute kidney injury and hemolytic uremic syndrome

2015 ◽  
Vol 4 (3-4) ◽  
pp. 34-37
Author(s):  
Mohan P. Patel ◽  
Prakash P. Ugale ◽  
Abhijeet B. Jagtap ◽  
Sandip T. Chaudhari ◽  
Pitambar N. Dighore
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Plasmodium Vivax ◽  
Vivax Malaria ◽  
Kidney Injury ◽  
Plasmodium Vivax Malaria ◽  
Uremic Syndrome

scholarly journals Collapsing glomerulopathy and hemolytic uremic syndrome associated with falciparum malaria: completely reversible acute kidney injury

2012 ◽  
Vol 37 (2) ◽  
pp. 286-290 ◽  
Author(s):  
Vivek Balkrishna Kute ◽  
Hargovind L. Trivedi ◽  
Aruna V. Vanikar ◽  
Pankaj R. Shah ◽  
Manoj R. Gumber ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Falciparum Malaria ◽  
Kidney Injury ◽  
Collapsing Glomerulopathy ◽  
Uremic Syndrome

scholarly journals Acute Kidney Injury with Hemolytic Uremic Syndrome

2021 ◽  
Vol 4 (1) ◽  
pp. 193-198
Author(s):  
Rubina Naqvi ◽  
Khawar Abbas ◽  
Syed Fazal Akhtar
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Case Series ◽  
Kidney Injury ◽  
Febrile Illness ◽  
Renal Recovery ◽  
P Value ◽  
Co Morbidity ◽  
Uremic Syndrome

We report here, case series of patients with acute kidney injury (AKI) developing in associationwith hemolytic uremic syndrome (HUS). Different causes of HUS and outcome of patients in this group of patients is aimed to be reported. Patients and Methods: subjects for the study reported here comprised of a cohort of 105 patients admitted with the diagnosis of AKI due to HUS. AKI was defined according to RIFLE criteria and HUS on basis of hematological, biochemical and/or histological features. All patients had normal size kidneys on ultrasonography and no previous co- morbidity. Results: One hundred and five patients with AKI due to HUS were brought to this institute from January 2000 - July 2019; among these 76 were females, mean age of these patients was 27.83±10.50 years. Causes of HUS were febrile illness, with or without diarrhea, diarrhea alone, pregnancy related complications and one each from snake bite, HCV infection / IFN therapy and use of combination of drugs. In pregnancy related HUS one patient had HUS during pregnancy while rest were during postpartum. Renal replacement was required in 95.23 % patients. Complete renal recovery was observed in 22 patients, while 15 died during acute phase of illness. CKD-V developed in 24 patients, 41 patients lost long term follow up, but were dialysis free till last follow up. Treatment with plasmapheresis revealed significantly better renal recovery (p value 0.026) in this group of patients. Conclusion: HUS can be severe life threatening disease; AKI with background of HUS may remain irreversible in many of these patients. Plasmapheresis should be offered to patients with established diagnosis of HUS.

scholarly journals P0334HEMOLYTIC UREMIC SYNDROME: CLINICAL PRESENTATION, LABORATORY FINDINGS, MANAGEMENT AND OUTCOMES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Issa Al Salmi ◽  
AbdelMasiah Metry ◽  
SUAD Hannawi
Keyword(s):  
Escherichia Coli ◽  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Shiga Toxin ◽  
Post Partum ◽  
Kidney Injury ◽  
Partial Recovery ◽  
Laboratory Findings ◽  
Study Results ◽  
Uremic Syndrome

Abstract Background and Aims Hemolytic-uremic syndrome (HUS) is a disease that has been described as a triad of hemolytic anemia, thrombocytopenia and renal impairment. There are two types of HUS, typical and atypical. HUS remains a leading cause of acute renal injury in children and is increasingly recognized as a cause of renal failure in adults. In our study we evaluated the demographic features, clinical characteristics, management and outcome of HUS. Method This is a descriptive study evaluating all cases of Hemolytic Uremic Syndrome (HUS) that have been admitted at Royal Hospital (RH) Oman in the period between 2008 and 2017. Results Thirty-six patients identified. The mean age (SD) of 10.68 (14.07) years. Eighteen (50%) presented with abdominal pain, nausea, vomiting and diarrhea, 9 (25 %) with hypertension, 23 (64 %) with acute kidney injury (AKI), 6 (16.67%) with seizure, 2 (5.56%) with confusion and 1 (2.78%) with cerebrovascular accident (CVA). Twenty-one (58.33 %) diagnosed as typical HUS of which 19 with Shiga toxin producing Escherichia Coli (STEC) HUS and 2 had post Streptococal HUS. Six (16.67%) diagnosed with aHUS, 2 (5.56%) with HELLP, 2 (5.56%) with G6PD, 3 (8.33%) with Autoimmune hemolytic anemia (AIHA ), one (2.78%) with congenital TTP and 1 (2.78%) with post partum HUS. Twenty three (63.89%) needed renal replacement therapy (RRT), while remaining 13 (36.11%) did not require RRT. Eleven (30.56%) received plasma exchange, 5 (13.89%) received Eculizumab while 2 (5.56%) received plasma infusion and 1 (2.78%) patient received Rituximab. The majority 22 (61.11%) had partial recovery after treatment, 5 (13.89 %) had compelte recovery and 3 (8.33% ) ended with end stage kidney disease (ESKD) and 1 (2.78%) died from hypertensive crises. Conclusion The study results showed that HUS population were mostly due to Shiga toxin producing Escherichia Coli (STEC). It showed that HUS population were young, mostly male and only 25% have known medical comorbidities at time of presentation. Also, the majority presented with AKI requiring dialysis, of which PD was main stay of therapy. The duration of RRT and recovery time was almost a month period.

scholarly journals A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Prianka Puri ◽  
Anida Hanxhiu ◽  
Daniel V. O’Hara ◽  
Danny Hsu ◽  
Mirna Vucak-Dzumhur
Keyword(s):  
Kidney Injury ◽  
Rare Condition ◽  
Foetal Death ◽  
Case Presentation ◽  
Haemolytic Uremic Syndrome ◽  
Genetic Sequencing ◽  
Planned Pregnancy ◽  
Life Threatening ◽  
Uremic Syndrome ◽  
Antenatal Visits

Abstract Background Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy. Case presentation A 27-year-old female of Lebanese descent presented at 36 weeks’ gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2 weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2 months she became dialysis independent. Conclusion This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her risk of relapse remains high with subsequent pregnancies. Currently we are awaiting her genetic sequencing to complete her assessment for underlying mutations and are determining the safest approach to a future planned pregnancy.

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