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AMNIOTIC FLUID SPECIFIC BIOCHEMICAL PARAMETERS FOR PRENATAL DIAGNOSIS OF MUCOPOLYSACCHARIDOSIS IN NON-IMMUNE HYDROPS FETALIS

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Last updated: 04 Jan 2025

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Abstract

52 amniotic fluid (AF) of women at (18-22 weeks) of pregnancy {10-15ml each were sampled because of ab­normal ultrasound findings mainly non-immune hydrops fetalis (N.I.H.F). They were analysed by a procedure involving AF supernatant analysis for total glycosaminoglycans (Total GAG) by formation of complex­es with Quinacrine reagent and the pregnancies at increased risk of mu-copolysaccharidosis (MPs) i.e with in­creased total GAG (over 100 mg/l) (12 cases) were enzymatically as­sayed for a group of enzymes : N-acetyl-B-glucose aminidase (NAGA), p-glucuronidase (B-GLA), p-galactosidase (B-GALS) and Aryl sul-fatase (ASA) by measuring the fluo­rescence of the corresponding 4-
methylumbelliferyl- glycoside to each one. Eight cases of different types af MPs were diagnosed. The reported procedures allowed detection of 4 cases of type VII MPs, 2 cases of type VI MPs, 1 case of type III B MPs and 1 case of type IVB MPs. 4 cases of unclassified MPs were found. Be­cause of the poor prognosis of MPs fetuses so the need for a reliable chemical method for estimation of to­tal GAG in AF is the goal of our study. We aimed to know if that Quinacrine method is a potentially valuable ana­lytical tool in the prenatal diagnosis of MPs as a primary step, which must be followed by enzymatic activities assay of expected defective enzymes in AF to detect the type of MPs. This study showed that Quinacrine method is the best chemical method for total GAG.

DOI

10.21608/mjmu.2000.126967

Authors

First Name

Iman

Last Name

El-Shabrawy

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Affiliation

Medical Biochemistry and Qbestatrics & Gynacology* Departments Faculty of Medicin, Mansoura University

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Orcid

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First Name

Fagr

Last Name

El-Shahat

MiddleName

-

Affiliation

Medical Biochemistry and Qbestatrics & Gynacology* Departments Faculty of Medicin, Mansoura University

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City

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Orcid

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First Name

Amina

Last Name

Bauomy

MiddleName

-

Affiliation

Medical Biochemistry and Qbestatrics & Gynacology* Departments Faculty of Medicin, Mansoura University

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City

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Orcid

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First Name

Hamed

Last Name

Yossif

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-

Affiliation

Medical Biochemistry and Qbestatrics & Gynacology* Departments Faculty of Medicin, Mansoura University

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Volume

29

Article Issue

2

Related Issue

18942

Issue Date

2000-12-01

Receive Date

2020-12-02

Publish Date

2000-12-01

Page Start

357

Page End

371

Print ISSN

1110-211X

Online ISSN

2735-3990

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https://mjmu.journals.ekb.eg/article_126967.html

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https://mjmu.journals.ekb.eg/service?article_code=126967

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Journal

Publication Title

Mansoura Medical Journal

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https://mjmu.journals.ekb.eg/

MainTitle

AMNIOTIC FLUID SPECIFIC BIOCHEMICAL PARAMETERS FOR PRENATAL DIAGNOSIS OF MUCOPOLYSACCHARIDOSIS IN NON-IMMUNE HYDROPS FETALIS

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Article

Created At

23 Jan 2023