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2014 ; 89
(12
): 1142-6
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Hereditary xerocytosis revisited
#MMPMID25044010
Archer NM
; Shmukler BE
; Andolfo I
; Vandorpe DH
; Gnanasambandam R
; Higgins JM
; Rivera A
; Fleming MD
; Sachs F
; Gottlieb PA
; Iolascon A
; Brugnara C
; Alper SL
; Nathan DG
Am J Hematol
2014[Dec]; 89
(12
): 1142-6
PMID25044010
show ga
A 21 year old male student presented in 1980 as an Olympic athlete with a 12 year
history of jaundice, pallor, and darkened urine induced by the atraumatic
exercise of swimming (1). Physical examination at that time was remarkable only
for moderate scleral icterus without hepatosplenomegaly. Hematological
examination revealed moderate macrocytosis (MCV 102 fL) without anemia (Hct 50%,
Hb 17 g/dL, 9% reticulocytes). The peripheral blood smear showed occasional
target cells. Red cell osmotic fragility was decreased. Red cell Na content was
increased and K content was decreased, with reduced total monovalent ion content.
Passive red cell permeability of both Na and K were increased. A supervised 2.5
hr swimming workout increased free plasma Hb from <5 to 45 mg/dL and decreased
serum haptoglobin from 25 to 6 mg/dL. The post-exercise urine sediment was
remarkable for hemosiderin-laden tubular epithelial cells, without frank
hemoglobinuria. The circulating 15 day erythrocyte half-life measured after 6
days without exercise was further shortened to 12 days after resumption of
twice-per-day swimming workouts for 1 week. The patient?s red cells were
hypersensitive to in vitro shear stress applied by cone-plate viscometer.
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