Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2006-5-11
pubmed:abstractText
Hemophagocytic syndrome (HPS) is defined by bone marrow and organ infiltration by activated, nonmalignant macrophages, which phagocytose blood cells. The clinical spectrum of HPS is broad, but renal involvement has rarely been investigated. We report a previously unknown renal manifestation of HPS: nephrotic syndrome. This multicentric retrospective study included patients fulfilling the following criteria: (i) no history of nephropathy; (ii) HPS diagnosis with histologic evidence of hemophagocytosis; (iii) occurrence of nephrotic syndrome during HPS; and (iv) available renal histology. Using the same criteria, we also searched the literature for additional cases. We identified nine patients retrospectively and found two additional cases in the literature (five males and six females, whose mean age was 34 +/- 27 years). Black African patients predominated (63.6%). HPS was due to lymphoma (six cases), infectious disease (three cases), and autoimmune disease (one case), and was primary in one patient. Acute renal failure was associated with nephrotic syndrome in 10/11 cases. Renal histology showed acute tubular necrosis associated with collapsing glomerulopathy in five patients (all Africans with negative human immunodeficiency virus serology), minimal change glomerulopathy in four, and thrombotic-microangiopathy with abnormal podocytes in two. Death occurred in seven cases. Nephrotic syndrome should be included among the renal complications of HPS with acute renal failure. We postulate that abnormal T-cell activation and/or high pro-inflammatory cytokine levels during HPS might cause podocyte injuries, especially among African patients with a susceptible genetic background.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0085-2538
pubmed:author
pubmed:issnType
Print
pubmed:volume
69
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1892-8
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:16557222-Adolescent, pubmed-meshheading:16557222-Adult, pubmed-meshheading:16557222-African Continental Ancestry Group, pubmed-meshheading:16557222-Aged, pubmed-meshheading:16557222-Antineoplastic Agents, Alkylating, pubmed-meshheading:16557222-Antineoplastic Agents, Phytogenic, pubmed-meshheading:16557222-Asian Continental Ancestry Group, pubmed-meshheading:16557222-Child, pubmed-meshheading:16557222-Child, Preschool, pubmed-meshheading:16557222-Cyclophosphamide, pubmed-meshheading:16557222-Cytokines, pubmed-meshheading:16557222-Etoposide, pubmed-meshheading:16557222-European Continental Ancestry Group, pubmed-meshheading:16557222-Female, pubmed-meshheading:16557222-Humans, pubmed-meshheading:16557222-Lymphohistiocytosis, Hemophagocytic, pubmed-meshheading:16557222-Male, pubmed-meshheading:16557222-Middle Aged, pubmed-meshheading:16557222-Nephrotic Syndrome, pubmed-meshheading:16557222-Retrospective Studies
pubmed:year
2006
pubmed:articleTitle
Nephrotic syndrome associated with hemophagocytic syndrome.
pubmed:affiliation
Department of Nephrology, Hôpital Necker-Enfants Malades, Paris, France. olivier.thaunapastu@free.fr
pubmed:publicationType
Journal Article, Comparative Study