PMID- 10590041
OWN - NLM
STAT- MEDLINE
DCOM- 20000110
LR  - 20210216
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 94
IP  - 12
DP  - 1999 Dec 15
TI  - Correction of leukocyte adhesion deficiency type II with oral fucose.
PG  - 3976-85
AB  - We describe a simple, noninvasive, and effective therapy for leukocyte adhesion 
      deficiency type II (LAD II), a rare inherited disorder of fucose metabolism. This 
      disorder leads to an immunodeficiency caused by the absence of carbohydrate-based 
      selectin ligands on the surface of neutrophils as well as to severe psychomotor 
      and mental retardation. The fucosylation defect in LAD II fibroblasts can be 
      corrected by addition of L-fucose to the culture medium. This prompted us to 
      initiate dietary fucose therapy on a patient with LAD II. Oral supplementation of 
      fucose in this patient induced the expression of fucosylated selectin ligands on 
      neutrophils and core fucosylation of serum glycoproteins. During 9 months of 
      treatment, infections and fever disappeared, elevated neutrophil counts returned 
      to normal, and psychomotor capabilities improved.
FAU - Marquardt, T
AU  - Marquardt T
AD  - Klinik und Poliklinik fur Kinderheilkunde and the Institut fur Zellbiologie, 
      ZMBE, Universitat Munster, Munster, Germany.
FAU - Luhn, K
AU  - Luhn K
FAU - Srikrishna, G
AU  - Srikrishna G
FAU - Freeze, H H
AU  - Freeze HH
FAU - Harms, E
AU  - Harms E
FAU - Vestweber, D
AU  - Vestweber D
LA  - eng
GR  - R01 GM55695/GM/NIGMS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Ligands)
RN  - 0 (Selectins)
RN  - 28RYY2IV3F (Fucose)
SB  - IM
CIN - Blood. 2000 Jun 1;95(11):3641-3. PMID: 10877554
MH  - Administration, Oral
MH  - Fucose/*administration & dosage/therapeutic use
MH  - Humans
MH  - Leukocyte-Adhesion Deficiency Syndrome/*drug therapy/metabolism
MH  - Ligands
MH  - Selectins/metabolism
EDAT- 1999/12/10 00:00
MHDA- 1999/12/10 00:01
CRDT- 1999/12/10 00:00
PHST- 1999/12/10 00:00 [pubmed]
PHST- 1999/12/10 00:01 [medline]
PHST- 1999/12/10 00:00 [entrez]
AID - S0006-4971(20)36631-3 [pii]
PST - ppublish
SO  - Blood. 1999 Dec 15;94(12):3976-85.