Neonatal Hemolytic-Uremic Syndrome

Figueras, Josep; Roldan, Andrea; Botet, Francesc; Palacı́n, Antonio; Jiménez, Rafael

doi:10.1177/000992289503400409

Cited by 6 publications

(3 citation statements)

References 5 publications

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“…There are few well documented cases of HUS in the neonatal age group [40,[47][48][49][50][51]. Previous case reports had suggested that HUS in this age group is usually not preceded by any prodrome and often there is no clinical evidence of either renal or cerebral disease [51].…”

Section: Discussionmentioning

confidence: 99%

Hemolytic uremic syndrome in Kuwaiti Arab children

Al‐Eisa¹,

Al-Hajeri

2001

Pediatric Nephrology

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Twenty-five children with hemolytic uremic syndrome (HUS) were diagnosed between January 1985 and January 2000 in the Pediatric Nephrology Unit at Mubarak Al-Kabeer Hospital. Fourteen patients (56%) had typical (D+) HUS whereas 11 (44%) had atypical (D-) HUS. No bacterial or viral pathogens could be isolated in the majority of cases. The atypical HUS group had more severe anemia (P=0.03), which was significantly more prolonged than in the typical HUS group (P=0.0028). There was no significant difference between the two groups in the mean maximum serum creatinine (P=0.1), blood urea nitrogen values (P=0.8) and severity of leukocytosis (P=0.4). Anuria and the need for dialysis were not significantly different between the two groups (P=0.1 and 0.05, respectively). Mortality was significantly higher in the D- HUS patients (P<0.0001). Recurrence of HUS was documented in 63.3% of the D- HUS group compared to 14.2% of the D+ HUS group (P=0.0053). Family history of HUS was reported in 72.7% of the atypical HUS group and 14.2% of the typical HUS group. There were no significant differences in chronic renal sequelae between the two groups. In conclusion, the pathogenesis of HUS in Kuwaiti children appears to be influenced by genetic factors rather than certain environmental pathogens. Atypical HUS has a higher mortality rate, a definite familial tendency and a high relapse rate.

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Section: Discussionmentioning

confidence: 99%

Hemolytic uremic syndrome in Kuwaiti Arab children

Al‐Eisa¹,

Al-Hajeri

2001

Pediatric Nephrology

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“…13 15 16 19 20 Familial occurrence accounts for approximately 10% of cases of D−HUS and the prognosis is often poor 810 15 Neonatal D−HUS represents a rare subgroup8 17 28-30 and its familial occurrence is associated with a worse outcome 828 Rare hereditary, autosomal recessive disorders, excluded in our patients, have been identified in a few families: hypocomplementaemia,20 23 31 32 methylmalonic aciduria, and homocystinuria due to intracellular vitamin B12 deficiency (CBLC phenotype),24 33 34 and collagen type III glomerulopathy 35.…”

Section: Discussionmentioning

confidence: 99%

“…D-HUS or atypical HUS is less common and is itself heterogeneous14: some are familial,15 some are recurring,16 some have a neonatal onset,17 and some are related to neuraminidase producing microbes 18. D−HUS is generally associated with a greater morbidity and mortality than D+HUS 3-10…”

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confidence: 99%

Heterogeneity of atypical haemolytic uraemic syndromes

Neuhaus

Calonder

Leumann

1997

Archives of Disease in Childhood

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Hemolytic uremic syndrome and thymic dysplasia in an infant

Andronikou

Kollios

Dimou

et al. 1998

Pediatric Nephrology

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A 33-day-old male infant was admitted to the neonatal intensive care nursery because of respiratory distress, grunting, cyanosis, and radiological findings of bilateral bronchopneumonia. He responded well to intensive therapy, but 11 days later developed hemolytic uremic syndrome, which was treated conservatively with prednisone and plasma transfusions with good response. The hemolytic uremic syndrome resolved, but he subsequently developed severe recurrent infections of unknown etiology and died at the age of 78 days. Necropsy findings revealed necrotizing enterocolitis as well as dysplasia of the thymus and other lymphoid tissues, compatible with the diagnosis of immunodeficiency disorder.

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Neonatal Hemolytic-Uremic Syndrome

Cited by 6 publications

References 5 publications

Hemolytic uremic syndrome in Kuwaiti Arab children

Hemolytic uremic syndrome in Kuwaiti Arab children

Heterogeneity of atypical haemolytic uraemic syndromes

Hemolytic uremic syndrome and thymic dysplasia in an infant

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