EXPERIENCE OF A SINGLE CENTER IN NTBC USE IN MANAGEMENT OF HEREDITARY TYROSINEMIA TYPE I IN LIBYA

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ALOBAIDY HANNA; BARKAOUI EMNA

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Journal: Iranian Journal of Pediatrics Year:2015 | Volume:25 | Issue:5 Page(s): 0-0

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Title

EXPERIENCE OF A SINGLE CENTER IN NTBC USE IN MANAGEMENT OF HEREDITARY TYROSINEMIA TYPE I IN LIBYA

Author(s)

ALOBAIDY HANNA | BARKAOUI EMNA | Issue Writer Certificate

Keywords

ALPHA FETOPROTEIN

NTBC

HEREDITARY TYROSINEMIA TYPE I

LIBYA

Abstract

Objectives: This study reports beside its clinical and biochemical presentation, the outcome of NTBC [2- (2-nitro-4-trifloro-methylbenzoyl)-1, 3-cyclohexanedion] treatment of the disease and evaluates its biochemical markers in 16 pediatric LIBYAn patients.Patients and Methods: The diagnosis was based on presence of high tyrosine levels in blood and succinylacetone in urine.Results: The consanguinity rate was 81.2%, the median age at onset, at diagnosis and at starting treatment were 4.5, 8, and 9.5 months respectively. At presentation hepatomegaly, jaundice, rickets and high gamma glutamyl transferase (GGT) were observed in 87.5% of patients. All patients had extremely high ALPHA FETOPROTEIN (AFP) and high alkaline phosphatase (ALP) levels. Fifteen patients were treated with NTBC, normalization of PT (Prothrombine time) was achieved in average in 14 days. The other biochemical parameters of liver function (transaminases, GGT, ALP, bilirubin and albumin) took longer to improve and several months to be normalized. Survival rate with NTBC was 86.6%. Patients who started treatment in a median of 3 months post onset observed a fast drop of AFP in 90.6% of patients (P=0.003). Abnormal liver function and rickets were the common presentations, GGT was an early cholestatic sensitive test. ALP was constantly high even in asymptomatic patients.Conclusions: In HT1 a faster dropping of AFP is a marker of good prognosis.

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APA: Copy

ALOBAIDY, HANNA, & BARKAOUI, EMNA. (2015). EXPERIENCE OF A SINGLE CENTER IN NTBC USE IN MANAGEMENT OF HEREDITARY TYROSINEMIA TYPE I IN LIBYA. IRANIAN JOURNAL OF PEDIATRICS, 25(5), 0-0. SID. https://sid.ir/paper/665184/en

Vancouver: Copy

ALOBAIDY HANNA, BARKAOUI EMNA. EXPERIENCE OF A SINGLE CENTER IN NTBC USE IN MANAGEMENT OF HEREDITARY TYROSINEMIA TYPE I IN LIBYA. IRANIAN JOURNAL OF PEDIATRICS[Internet]. 2015;25(5):0-0. Available from: https://sid.ir/paper/665184/en

IEEE: Copy

HANNA ALOBAIDY, and EMNA BARKAOUI, “EXPERIENCE OF A SINGLE CENTER IN NTBC USE IN MANAGEMENT OF HEREDITARY TYROSINEMIA TYPE I IN LIBYA,” IRANIAN JOURNAL OF PEDIATRICS, vol. 25, no. 5, pp. 0–0, 2015, [Online]. Available: https://sid.ir/paper/665184/en

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