Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis.
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis.


METHODS
We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous complications (many of them reported to accompany NS as single events). Clinical and laboratory signs of HLH evolved with time and led to deterioration of her condition and death, within 5 months of her original presentation.


CONCLUSIONS
To our knowledge, this is the first report of NS antedating the presentation of HLH.
Department of Pediatrics, Soroka University Medical Center, Ben Gurion University, Beer Sheva, Israel, ldaniel@bgu.ac.il.
Daniel
D
Landau
Evgenia
E
Gurevich
Joseph
J
Kapelushnik
Hannah
H
Tamary
Ilan
I
Shelef
Isaac
I
Lazar
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology*; D009102:Multiple Organ Failure / Q000209:etiology; D009404:Nephrotic Syndrome / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy; D012008:Recurrence; D013997:Time Factors; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones