Endotheliotropic (hemolytic) nephroangiopathy and its various manifestation forms (thrombotic microangiopathy, primary malignant nephrosclerosis, hemolytic-uremic syndrome)

Thoenes, W.; Hyttel, John

doi:10.1007/bf01476776

Cited by 38 publications

(15 citation statements)

References 32 publications

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“…Patients with extensive cortical necrosis predictably had a poor outcome. So too did those with arterial rather than purely glomerular TMA, an observation confirmed by others [13].…”

Section: Discussionmentioning

confidence: 52%

Clinico-pathological findings in diarrhoea-negative haemolytic uraemic syndrome

Taylor

Chua

Howie

et al. 2004

Pediatric Nephrology

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This is a retrospective, national clinico-pathological study of past and current patients with haemolytic uraemic syndrome not associated with diarrhoea (D- HUS). Thirty-four patients were analysed and notified by members of the British Association for Paediatric Nephrology in 1998-1999. There was a 2:1 excess of males. Ten presented in infancy. The aetiology included 5 patients with complement abnormalities, 2 patients with complications of pneumococcal infection, and 2 with malignancies. Parental consanguinity was noted in 6 patients. Five children died, 9 developed chronic renal failure, and 10 end-stage renal failure. Only 7 made full recoveries. With a single exception, the pathological findings were unlike the previously reported glomerular thrombosis that is characteristic of diarrhoea-associated HUS, or HUS complicating verocytotoxin-producing Escherichia coli infection. Early and late glomerulopathy could be distinguished. Arteriolar and arterial disease was observed in 8 and 7 patients, respectively. Arterial disease correlated with a poor outcome. The pathology of D- HUS is of prognostic value, but this study was not powered to identify specific aetiological/pathological correlations.

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“…Patients with extensive cortical necrosis predictably had a poor outcome. So too did those with arterial rather than purely glomerular TMA, an observation confirmed by others [13].…”

Section: Discussionmentioning

confidence: 52%

Clinico-pathological findings in diarrhoea-negative haemolytic uraemic syndrome

Taylor

Chua

Howie

et al. 2004

Pediatric Nephrology

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“…In our patient, renal histomorphology showed the typical lesions of glomerular thrombotic microangiopathy. Whereas arteriolar changes are more often found in "atypical" HUS, the observed glomerular capillary lesions are more common in toxin associated HUS [8].…”

Section: Discussionmentioning

confidence: 90%

“…The arrow points to the characteristic 150-kDa band of factor H. An additional band in the patient's serum is indicated by the second arrow. The two arrows below indicate the two FHR-1 bands (42 kDa and 38 kDa) necrotic toxin playing an unknown role in whooping cough, which causes skin necrosis if injected subcutaneously in mice and (2) the tracheal cytotoxin, which exerts cytotoxic effects on ciliar epithelium [8,9]. It can be speculated that in the case of severe local and systemic inflammation one of these cytotoxins might enter the bloodstream and act cytopathically on endothelial cells causing complement activation.…”

Section: Discussionmentioning

confidence: 99%

Hemolytic uremic syndrome due to an altered factor H triggered by neonatal pertussis

Berner¹,

Krause²,

Gordjani³

et al. 2002

Pediatr Nephrol

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We report on a previously healthy newborn suffering from severe Bordetella pertussis infection who developed hemolytic uremic syndrome (HUS) a few weeks after the onset of whooping cough, with a fatal outcome. A factor H protein with abnormal mobility was found in the serum of the patient as analyzed by Western blotting, indicating that B. pertussis infection might have triggered HUS in a genetically predisposed patient.

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“…9,10,[12][13][14] The renal histologic changes can be separated into two categories, or patterns, that are correlated with long-term renal function. 15,16 The case under discussion falls into the category of primary glomerular involvement with little or no arterial change. This morphologic pattern is usually, but not exclusively, seen in children, and is typically associated with gastroenteritis and bloody diarrhea.…”

Section: Pathological Discussionmentioning

confidence: 99%

“…The other pattern, which is characterized by fibrin exudation, fibrinoid necrosis, thrombosis, or intimal thickening -or a combination of these findings -is often encountered in cases of scleroderma or malignant hypertension or after treatment with mitomycin. The first pattern is associated with a much more favorable prognosis than the second, 9,[15][16][17] especially if fewer than half the glomeruli are involved. 18 Features associated with poor renal recovery include arteriolar changes, patchy cortical necrosis, prolonged anuria, and an elevated peripheral-blood neutrophil count in children with gastroenteritis, as well as advanced age.…”

Section: Pathological Discussionmentioning

confidence: 99%