A CASE OF LEUCOCYTE ADHESION DEFICIENCY

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BEHVAD A.; LESANI M.

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Journal: Research in Medicine Year:2001 | Volume:24 | Issue:4 Page(s): 345-352

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Title

A CASE OF LEUCOCYTE ADHESION DEFICIENCY

Author(s)

BEHVAD A. | LESANI M. | Issue Writer Certificate

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Abstract

LAD is a rare authosomal recessive disorder characterized by a genetic defect of a group Leucocyte membrane glycoprotein that confer adhesivenous on lymphocytes, and neutrophile surface called MAC-1, LFA-1 and P 150,45 for type I and sialyl Lewis x a carbohydrate ligament on neutrophile surface for E-selection which in turn is present on the surfaces of activated endothelial cells for type II disorder. Type I LAD has been described in over 50 patients and type II LAD first described in 1992 in 2 unrelated boys of consanguineous parents. Besides the occurance of repeated bacterial infections that are the hallmark of all the phagocyte disorders, LAD is characterized by an unusual persistent granulocytosis, severe gangrenous periodontitis frequent Superficial bacterial infections that may invade locally or systematically and show repair wound healing that is often first manifested as delayed umbilical cord separation, staphylococcus aureus and gram negative bacteria are the most common pathogens. We have presented in this report 4 months old girl admitted for bacterial cellulitis of groins along with severe omphalitis and delayed separation of umbilical cord. Umbilical cord inflammation with oozing was apparent shortly after birth, with episodes of exacerbation which finally led to hospitalization for definite diagnosis and therapy. Skin infection and cellulitis were not completely eradicated by oral antibacterial, laboratory examination revealed severe leucocytosis, elevated ESR, normal serum gammaglubulin, slightly elevated CH50 and definite deficiency of αIFA, C3b-1 and P150, 95. Clinical diagnosis was therefore confirmed by laboratory finding and patient was placed on ceftazidime and Amikacin and was advised to have regular weekly visit to dispensary for further evaluation.

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APA: Copy

BEHVAD, A., & LESANI, M.. (2001). A CASE OF LEUCOCYTE ADHESION DEFICIENCY. PAJOUHESH DAR PEZESHKI, 24(4), 345-352. SID. https://sid.ir/paper/41861/en

Vancouver: Copy

BEHVAD A., LESANI M.. A CASE OF LEUCOCYTE ADHESION DEFICIENCY. PAJOUHESH DAR PEZESHKI[Internet]. 2001;24(4):345-352. Available from: https://sid.ir/paper/41861/en

IEEE: Copy

A. BEHVAD, and M. LESANI, “A CASE OF LEUCOCYTE ADHESION DEFICIENCY,” PAJOUHESH DAR PEZESHKI, vol. 24, no. 4, pp. 345–352, 2001, [Online]. Available: https://sid.ir/paper/41861/en

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