Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran

BACKGROUND AND OBJECTIVE: Phenylketonuria (PKU) is one of the most frequent metabolic diseases which is transmitted as autosomal recessive pattern due to phenylalanine hydroxylase deficiency. Appropriate diet with restricted phenylalanine is the base of treatment. According to some difficulties in o...

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Autor principal:	Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh S
Formato:	article
Lenguaje:	EN FA
Publicado:	Babol University of Medical Sciences 2011
Materias:	nutrition phenylketonuria iron deficiency anemia Medicine R Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/a39a93c357f641b1860005c1e0daf81b
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spelling	oai:doaj.org-article:a39a93c357f641b1860005c1e0daf81b2021-11-10T08:56:48ZEvaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran1561-41072251-7170https://doaj.org/article/a39a93c357f641b1860005c1e0daf81b2011-09-01T00:00:00Zhttp://jbums.org/article-1-3874-en.htmlhttps://doaj.org/toc/1561-4107https://doaj.org/toc/2251-7170BACKGROUND AND OBJECTIVE: Phenylketonuria (PKU) is one of the most frequent metabolic diseases which is transmitted as autosomal recessive pattern due to phenylalanine hydroxylase deficiency. Appropriate diet with restricted phenylalanine is the base of treatment. According to some difficulties in our country for these patients and limited native studies exist about the results of such treatment means protein limitation and replacing an expensive and unavailable foods, this study was designed to evaluate nutritional status with calculating the calorie, protein, carbohydrate and fat of patients’ diet and iron storage and iron deficiency anemia in PKU patients.METHODS: This is a cross sectional study which evaluated all of the PKU patients in Mazandaran province during 2009-2010 in metabolic clinics in Babol and Sari, Iran. Nutritional status was evaluated according to 72 hours diet recall sheet which the patient or his/her parent recorded all of the nutrients that was eaten within 3 days then nutritional contents: carbohydrate, protein, fat and calories were evaluated by nutritionist. Demographic information was studied according to questionnaire and iron deficiency and iron deficiency anemia was studied according to blood sample and measurement of hemoglobin, hematocrit MCV, MCH, MCHC, Ferritin and response to treatment with ferrous sulfate.FINDINGS: Twenty one PKU patients were studied which 7 ones (33.3%) were female and 14 ones (66.7%) were male with mean age of 7.26±6.64 years. Iron deficiency and iron deficiency anemia was present in respectively in 10 (47.1%) and 6 patients (28.6%). Deficiency of energy, protein, carbohydrate and fat was seen in 9 patients (42.86%), 14 patients (66.67%), 9 patients (42.86%) and 6 patients (28.57%), respectively. 23.8% of patients were underweight, 23.8% of patients were microcephal and 4 patients (19%) were short stature.CONCLUSION: The results of this study showed that the nutritional status and growth of patients was not good, so attention to nutritional demands in PKU patients to reduce malnutrition and iron deficiency is essential.Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh SBabol University of Medical Sciencesarticlenutritionphenylketonuriairon deficiency anemiaMedicineRMedicine (General)R5-920ENFAMajallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Bābul, Vol 13, Iss 5, Pp 58-62 (2011)
institution	DOAJ
collection	DOAJ
language	EN FA
topic	nutrition phenylketonuria iron deficiency anemia Medicine R Medicine (General) R5-920
spellingShingle	nutrition phenylketonuria iron deficiency anemia Medicine R Medicine (General) R5-920 Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh S Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
description	BACKGROUND AND OBJECTIVE: Phenylketonuria (PKU) is one of the most frequent metabolic diseases which is transmitted as autosomal recessive pattern due to phenylalanine hydroxylase deficiency. Appropriate diet with restricted phenylalanine is the base of treatment. According to some difficulties in our country for these patients and limited native studies exist about the results of such treatment means protein limitation and replacing an expensive and unavailable foods, this study was designed to evaluate nutritional status with calculating the calorie, protein, carbohydrate and fat of patients’ diet and iron storage and iron deficiency anemia in PKU patients.METHODS: This is a cross sectional study which evaluated all of the PKU patients in Mazandaran province during 2009-2010 in metabolic clinics in Babol and Sari, Iran. Nutritional status was evaluated according to 72 hours diet recall sheet which the patient or his/her parent recorded all of the nutrients that was eaten within 3 days then nutritional contents: carbohydrate, protein, fat and calories were evaluated by nutritionist. Demographic information was studied according to questionnaire and iron deficiency and iron deficiency anemia was studied according to blood sample and measurement of hemoglobin, hematocrit MCV, MCH, MCHC, Ferritin and response to treatment with ferrous sulfate.FINDINGS: Twenty one PKU patients were studied which 7 ones (33.3%) were female and 14 ones (66.7%) were male with mean age of 7.26±6.64 years. Iron deficiency and iron deficiency anemia was present in respectively in 10 (47.1%) and 6 patients (28.6%). Deficiency of energy, protein, carbohydrate and fat was seen in 9 patients (42.86%), 14 patients (66.67%), 9 patients (42.86%) and 6 patients (28.57%), respectively. 23.8% of patients were underweight, 23.8% of patients were microcephal and 4 patients (19%) were short stature.CONCLUSION: The results of this study showed that the nutritional status and growth of patients was not good, so attention to nutritional demands in PKU patients to reduce malnutrition and iron deficiency is essential.
format	article
author	Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh S
author_facet	Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh S
author_sort	Eshraghi P, Abbaskhanian A, Bijani A, Hosseinzadeh S
title	Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
title_short	Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
title_full	Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
title_fullStr	Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
title_full_unstemmed	Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
title_sort	evaluation of nutritional status and growth in phenylketonuria patients in mazandaran province iran
publisher	Babol University of Medical Sciences
publishDate	2011
url	https://doaj.org/article/a39a93c357f641b1860005c1e0daf81b
work_keys_str_mv	AT eshraghipabbaskhanianabijaniahosseinzadehs evaluationofnutritionalstatusandgrowthinphenylketonuriapatientsinmazandaranprovinceiran
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Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran

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