Hemolytic-uremic syndrome in children, associated with diarrhea (literature review)

2021 ◽  
Vol 25 (3) ◽  
pp. 43-51
Author(s):  
E. V. Gunkova ◽  
A. A. Vyalkova ◽  
I. V. Zorin

Typical hemolytic-uremic syndrome (tGUS) is an acute disease in which non-immune microangiopathic hemolytic anemia, thrombocytopenia and acute renal damage develop against the background of infection-related diarrhea in the prodromal period. Hemolytic-uremic syndrome is the main cause of acute kidney injury in children under 5 years of age. Hemolytic-uremic syndrome is one of the causes of the progression of renal dysfunction in children with the formation of chronic kidney disease. The clinical picture of hemolytic-uremic syndrome is characterized by multi-organ manifestations with symptoms of acute renal damage, damage to the gastrointestinal tract, nervous, cardiovascular, respiratory systems and hemostasis. The article presents the data of modern literature on the epidemiology, etiology, pathogenesis and clinical picture of HUS in children, own scientific results on the clinical and paraclinical characteristics of a typical hemolytic-uremic syndrome in children of the Orenburg region.

Author(s):  
T. P. Makarova ◽  
R. R. Nigmatullina ◽  
L. A. Davlieva ◽  
Yu. S. Melnikova

Hemolytic-uremic syndrome is a serious problem in pediatrics and pediatric nephrology. Hemolytic-uremic syndrome is one of the leading causes of acute kidney injury with potential transformation into terminal chronic kidney disease. Currently, the endothelial dysfunction is strongly associated with changes in the serotonergic system in the pathogenesis of hemolytic-uremic syndrome. There are few studies that have revealed an increase in the blood plasma serotonin concentration in children with hemolytic-uremic syndrome, but its role in the pathogenesis of chronic kidney disease has been insufficiently studied. The progressive course of hemolytic-uremic syndrome, up to the terminal stage of renal failure, requires the search for markers of renal tissue damage as prognostically significant factors for the development of nephrosclerosis, which is of particular importance for optimizing the management of such children.


2020 ◽  
Vol 2020 ◽  
pp. 1-4 ◽  
Author(s):  
Eigo Ikushima ◽  
Manabu Hisahara ◽  
Takuya Nishijima ◽  
Hikaru Uchiyama ◽  
Tatsushi Onzuka ◽  
...  

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)-related disease that manifests as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused by uncontrolled activation of the complement system. We report the case of a 61-year-old woman with acute type A aortic dissection that subsequently developed into aHUS. The hematologic disorders underlying aHUS improved after treatment with the complement inhibitor eculizumab. It is important to consider aHUS when a patient clinically develops a triad of microangiopathic hemolytic anemia, thrombocytopenia, and an increasing creatinine level following cardiovascular surgery.


2017 ◽  
Vol 9 (2) ◽  
Author(s):  
Kuixing Zhang ◽  
Yuxin Lu ◽  
Kevin T. Harley ◽  
Minh-Ha Tran

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (<em>CFH, CFI, CD46</em> or <em>THBD</em>) or gain-of-function in an effector gene (<em>CFB</em> or <em>C3</em>). Treatment with the late complement inhibitor, eculizumab – a monoclonal antibody directed against C5 – is effective.


Author(s):  
Julia K. Lill ◽  
Stephanie Thiebes ◽  
Judith-Mira Pohl ◽  
Jenny Bottek ◽  
Nirojah Subramaniam ◽  
...  

2014 ◽  
Vol 8 ◽  
pp. BCBCR.S14920
Author(s):  
Victor C. Kok ◽  
Sheng-Chung Wu ◽  
Chien-Kuang Lee

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.


2021 ◽  
Vol 75 (2) ◽  
pp. 138-142
Author(s):  
Vladimír Teplan

Accurate measurement of renal function in serious liver disease is very important not only for the estimation of renal damage (chronic kidney disease – CKD), safe drug management, prediction of illness follow-up, intensive methods including hemodialysis, hemodiafiltration or hemoperfusion, but also for the indication of liver transplantation. All methods of renal function measurement using serum creatinine for the estimation of glomerular filtration rate (GFR) are not accurate: they overestimate the value of GFR; the worse the liver damage is, the higher the level of overestimation; predominantly due to decreased endogenous creatinine production (creatinine generation rate – CGR). Using of cystatin C for GFR in liver disease is mainly promising in acute kidney injury (AKI), but obtained results have not been defi nitive yet and need more relevant data from diff erent methods of GFR estimation.


2017 ◽  
Vol 4 (3) ◽  
pp. 136-138
Author(s):  
M.O. Gonchar ◽  
T.B. Ishenko ◽  
N.V. Orlova ◽  
G.R. Muratov ◽  
T.F. Kolibaeva ◽  
...  

Gonchar M.O., Ishchenko T.B., Orlova N.V., Muratov G., Kolibaeva T., Khmara N., Podvalnaya N.Currently, hemolytic-uremic syndrome is one of the frequent causes of acute kidney failure in children, so the timeliness of diagnosis and treatment determines the outcome of the disease. In the given clinical case, a set of certain factors that lead to an unfavorable outcome of the disease and the progression of chronic renal failure are presented. Clinical case of a 14-year-old child K., who was admitted to the nephrology department of the Regional Children's Clinical Hospital with the diagnosis: 3rd stage CKD, subcompensated stage of chronic renal failure and condition after hemolytic-uremic syndrome.KeyWords: hemolytic-uremic syndrome in children, chronic kidney disease. СТАН ПЫСЛЯ ПЕРЕНЕСЕНОГО ГЕМОЛІТИКО-УРЕМІЧНОГО СИНДРОМУ У ДИТИНИ З III СТАДІЄЮ ХРОНІЧНОГО ЗАХВОРЮВАННЯ НИРОК (КЛІНІЧНЕ СПОСТЕРЕЖЕННЯ)Гончарь М.О., Іщенко Т.Б., Орлова Н.В., Муратов Г.Р., Колібаєва Т.Ф., Хмара Н.В., Підвальна Н.А. В даний час гемолітико-уремічний синдром є однією з найчастіших причин гострої ниркової недостатності у дітей, тому своєчасність постановки діагнозу і лікування визначає результат захворювання. На наведеному клінічному випадку, представлено сукупність певних факторів, які привели до несприятливого результату захворювання і прогресування хронічної ниркової недостатності. Клінічний випадок дитини К. 14 років, який знаходився в нефрологічному відділенні Обласної дитячої клінічної лікарні з діагноз: ХХН III ст. Хронічна ниркова недостатність субкомпенсированная стадія. Стан після перенесеного гемолітико-уремічного синдрому.Ключові слова: гемолітико-уремічний синдром, діти, клінічний випадок, хронічне захворювання нирок. СОСТОЯНИЕ ПОСЛЕ ПЕРЕНЕСЕННОГО ГЕМОЛИТИКО-УРЕМИЧЕСКОГО СИНДРОМА У РЕБЕНКА С III СТАДИЕЙ ХРОНИЧЕСКОГО ЗАБОЛЕВАНИЯ ПОЧЕК (КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ)Гончарь М.А., Ищенко Т.Б., Орлова Н.В., Муратов Г.Р., Колибаева Т.Ф., Хмара Н.В., Подвальная Н.А. В настоящее время гемолитико-уремический синдром является одной из частых причин острой почечной недостаточности у детей, поэтому своевременность постановки диагноза и лечения определяет исход заболевания. На приведенном клиническом случае, представлено совокупность определенных факторов, которые привели к неблагоприятному исходу заболевания и прогрессированию хронической почечной недостаточности. Клинический случай ребенка К. 14 лет, который находился в нефрологическом отделении Областной детской клинической больнице с диагноз: ХБП III ст. Хроническая почечная недостаточность субкомпенсированная стадия. Состояние после перенесенного гемолитико-уремического синдрома.Ключевые слова: гемолитико-уремический синдром, дети, клинический случай, хроническое заболевание почек.


2020 ◽  
pp. 302-310
Author(s):  
Liza Kearl ◽  
Maureen McCollough

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.


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