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Hemolytic uremic syndrome(HUS)

MedGen UID:
42403
Concept ID:
C0019061
Disease or Syndrome
Synonyms: Acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia associated with distorted erythrocytes ('burr cells'); HUS
SNOMED CT: HUS - Hemolytic uremic syndrome (111407006); Hemolytic uremic syndrome (111407006); Gasser's syndrome (111407006)
 
Related genes: CFHR3, CFHR4, THBD, CD46, CFI, CFHR1, CFH, C3, CFB
OMIM® Phenotypic series: PS235400
HPO: HP:0005575

Conditions with this feature

Upshaw-Schulman syndrome
MedGen UID:
224783
Concept ID:
C1268935
Disease or Syndrome
The classic pentad of TTP includes hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and nonfocal neurologic findings, decreased renal function, and fever. Congenital TTP, also known as Schulman-Upshaw syndrome, is characterized by neonatal onset, response to fresh plasma infusion, and frequent relapses (Savasan et al., 2003; Kokame et al., 2002). Acquired TTP, which is usually sporadic, usually occurs in adults and is caused by an IgG inhibitor against the von Willebrand factor-cleaving protease.
Methylmalonic acidemia with homocystinuria
MedGen UID:
341256
Concept ID:
C1848561
Disease or Syndrome
The clinical manifestations of disorders of intracellular cobalamin metabolism can be highly variable even within a single complementation group. The prototype and best understood is cblC; it is also the most common of these disorders. The age of initial presentation of cblC spans a wide range, including: Newborns, who can have intrauterine growth retardation (IUGR) and microcephaly; Infants, who can have poor feeding, failure to thrive, pallor, and neurologic signs, and occasionally hemolytic uremic syndrome (HUS) and/or seizures including infantile spasms; Toddlers, who can have failure to thrive, poor head growth, cytopenias (including megaloblastic anemia), global developmental delay, encephalopathy, and neurologic signs such as hypotonia and seizures; and Adolescents and adults, who can have neuropsychiatric symptoms, progressive cognitive decline, and/or subacute combined degeneration of the spinal cord.
Atypical hemolytic-uremic syndrome 1
MedGen UID:
412743
Concept ID:
C2749604
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).
Atypical hemolytic-uremic syndrome 6
MedGen UID:
414541
Concept ID:
C2752036
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).
Atypical hemolytic-uremic syndrome 5
MedGen UID:
442875
Concept ID:
C2752037
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).
Atypical hemolytic-uremic syndrome 4
MedGen UID:
416691
Concept ID:
C2752038
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).
Atypical hemolytic-uremic syndrome 3
MedGen UID:
414542
Concept ID:
C2752039
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).
Atypical hemolytic-uremic syndrome 2
MedGen UID:
414167
Concept ID:
C2752040
Finding
Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).

Recent clinical studies

Etiology

Freist M, Garrouste C, Szlavik N, Coppo P, Lautrette A, Heng AE
Medicine (Baltimore) 2017 Dec;96(51):e9358. doi: 10.1097/MD.0000000000009358. PMID: 29390523Free PMC Article
Zhao SA, Ning BT, Mao JH
World J Pediatr 2017 Apr;13(2):183-185. Epub 2017 Mar 28 doi: 10.1007/s12519-017-0021-x. PMID: 28353273
Kreuzer M, Sollmann L, Ruben S, Leifheit-Nestler M, Fischer DC, Pape L, Haffner D
Pediatr Nephrol 2017 Jun;32(6):1005-1011. Epub 2017 Feb 8 doi: 10.1007/s00467-016-3574-3. PMID: 28180952
Keenswijk W, Vanmassenhove J, Raes A, Dhont E, Vande Walle J
Eur J Pediatr 2017 Mar;176(3):355-360. Epub 2017 Jan 11 doi: 10.1007/s00431-016-2846-z. PMID: 28078430
Shimizu M, Inoue N, Kuroda M, Mizuta M, Sugimoto N, Kaneda H, Ohta K, Yachie A
Clin Exp Nephrol 2017 Feb;21(1):76-82. Epub 2016 Mar 5 doi: 10.1007/s10157-016-1254-z. PMID: 26945868

Diagnosis

Zhao SA, Ning BT, Mao JH
World J Pediatr 2017 Apr;13(2):183-185. Epub 2017 Mar 28 doi: 10.1007/s12519-017-0021-x. PMID: 28353273
Kreuzer M, Sollmann L, Ruben S, Leifheit-Nestler M, Fischer DC, Pape L, Haffner D
Pediatr Nephrol 2017 Jun;32(6):1005-1011. Epub 2017 Feb 8 doi: 10.1007/s00467-016-3574-3. PMID: 28180952
Keenswijk W, Vanmassenhove J, Raes A, Dhont E, Vande Walle J
Eur J Pediatr 2017 Mar;176(3):355-360. Epub 2017 Jan 11 doi: 10.1007/s00431-016-2846-z. PMID: 28078430
Shimizu M, Inoue N, Kuroda M, Mizuta M, Sugimoto N, Kaneda H, Ohta K, Yachie A
Clin Exp Nephrol 2017 Feb;21(1):76-82. Epub 2016 Mar 5 doi: 10.1007/s10157-016-1254-z. PMID: 26945868
Rigamonti D, Simonetti GD
Eur J Pediatr 2016 Dec;175(12):1927-1931. Epub 2016 Sep 23 doi: 10.1007/s00431-016-2790-y. PMID: 27659663

Therapy

Freist M, Garrouste C, Szlavik N, Coppo P, Lautrette A, Heng AE
Medicine (Baltimore) 2017 Dec;96(51):e9358. doi: 10.1097/MD.0000000000009358. PMID: 29390523Free PMC Article
López Rubio ME, Rodado Martínez R, Illescas ML, Mateo Bosch E, Martinez Díaz M, de la Vara Inesta L, Cabezuelo B, Morales Albuja ME, Lucas Guillén E, Jimeno García L
Clin Nephrol 2017 Feb;87 (2017)(2):100-106. doi: 10.5414/CN108838. PMID: 27879189
Jenssen GR, Vold L, Hovland E, Bangstad HJ, Nygård K, Bjerre A
BMC Infect Dis 2016 Jun 13;16:285. doi: 10.1186/s12879-016-1627-7. PMID: 27297224Free PMC Article
Freedman SB, Xie J, Neufeld MS, Hamilton WL, Hartling L, Tarr PI; Alberta Provincial Pediatric Enteric Infection Team (APPETITE)., Nettel-Aguirre A, Chuck A, Lee B, Johnson D, Currie G, Talbot J, Jiang J, Dickinson J, Kellner J, MacDonald J, Svenson L, Chui L, Louie M, Lavoie M, Eltorki M, Vanderkooi O, Tellier R, Ali S, Drews S, Graham T, Pang XL
Clin Infect Dis 2016 May 15;62(10):1251-1258. Epub 2016 Feb 24 doi: 10.1093/cid/ciw099. PMID: 26917812Free PMC Article
Caillaud C, Zaloszyc A, Licht C, Pichault V, Frémeaux-Bacchi V, Fischbach M
Pediatr Nephrol 2016 Jan;31(1):157-61. Epub 2015 Sep 23 doi: 10.1007/s00467-015-3207-2. PMID: 26399238

Prognosis

Zhao SA, Ning BT, Mao JH
World J Pediatr 2017 Apr;13(2):183-185. Epub 2017 Mar 28 doi: 10.1007/s12519-017-0021-x. PMID: 28353273
Kreuzer M, Sollmann L, Ruben S, Leifheit-Nestler M, Fischer DC, Pape L, Haffner D
Pediatr Nephrol 2017 Jun;32(6):1005-1011. Epub 2017 Feb 8 doi: 10.1007/s00467-016-3574-3. PMID: 28180952
Keenswijk W, Vanmassenhove J, Raes A, Dhont E, Vande Walle J
Eur J Pediatr 2017 Mar;176(3):355-360. Epub 2017 Jan 11 doi: 10.1007/s00431-016-2846-z. PMID: 28078430
Shimizu M, Inoue N, Kuroda M, Mizuta M, Sugimoto N, Kaneda H, Ohta K, Yachie A
Clin Exp Nephrol 2017 Feb;21(1):76-82. Epub 2016 Mar 5 doi: 10.1007/s10157-016-1254-z. PMID: 26945868
Rigamonti D, Simonetti GD
Eur J Pediatr 2016 Dec;175(12):1927-1931. Epub 2016 Sep 23 doi: 10.1007/s00431-016-2790-y. PMID: 27659663

Clinical prediction guides

Freist M, Garrouste C, Szlavik N, Coppo P, Lautrette A, Heng AE
Medicine (Baltimore) 2017 Dec;96(51):e9358. doi: 10.1097/MD.0000000000009358. PMID: 29390523Free PMC Article
Zhao SA, Ning BT, Mao JH
World J Pediatr 2017 Apr;13(2):183-185. Epub 2017 Mar 28 doi: 10.1007/s12519-017-0021-x. PMID: 28353273
Keenswijk W, Vanmassenhove J, Raes A, Dhont E, Vande Walle J
Eur J Pediatr 2017 Mar;176(3):355-360. Epub 2017 Jan 11 doi: 10.1007/s00431-016-2846-z. PMID: 28078430
Shimizu M, Inoue N, Kuroda M, Mizuta M, Sugimoto N, Kaneda H, Ohta K, Yachie A
Clin Exp Nephrol 2017 Feb;21(1):76-82. Epub 2016 Mar 5 doi: 10.1007/s10157-016-1254-z. PMID: 26945868
Suyama K, Kawasaki Y, Miyazaki K, Kanno S, Ono A, Ohara S, Sato M, Hosoya M
Nephrol Dial Transplant 2015 Jun;30(6):969-77. Epub 2015 Feb 17 doi: 10.1093/ndt/gfv004. PMID: 25694534

Recent systematic reviews

Freedman SB, Xie J, Neufeld MS, Hamilton WL, Hartling L, Tarr PI; Alberta Provincial Pediatric Enteric Infection Team (APPETITE)., Nettel-Aguirre A, Chuck A, Lee B, Johnson D, Currie G, Talbot J, Jiang J, Dickinson J, Kellner J, MacDonald J, Svenson L, Chui L, Louie M, Lavoie M, Eltorki M, Vanderkooi O, Tellier R, Ali S, Drews S, Graham T, Pang XL
Clin Infect Dis 2016 May 15;62(10):1251-1258. Epub 2016 Feb 24 doi: 10.1093/cid/ciw099. PMID: 26917812Free PMC Article
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V; HUS International.
Pediatr Nephrol 2016 Jan;31(1):15-39. Epub 2015 Apr 11 doi: 10.1007/s00467-015-3076-8. PMID: 25859752
Igarashi T, Ito S, Sako M, Saitoh A, Hataya H, Mizuguchi M, Morishima T, Ohnishi K, Kawamura N, Kitayama H, Ashida A, Kaname S, Taneichi H, Tang J, Ohnishi M; Study group for establishing guidelines for the diagnosis and therapy of hemolytic uremic syndrome.
Clin Exp Nephrol 2014 Aug;18(4):525-57. doi: 10.1007/s10157-014-0995-9. PMID: 25099085
Thomas DE, Elliott EJ
BMC Public Health 2013 Sep 3;13:799. doi: 10.1186/1471-2458-13-799. PMID: 24007265Free PMC Article
Palermo MS, Exeni RA, Fernández GC
Expert Rev Anti Infect Ther 2009 Aug;7(6):697-707. doi: 10.1586/eri.09.49. PMID: 19681698

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