Stress degree demonstrated in mothers with phenylketonuria or hyperphenylalaninemia infant when requested for total or partial breastfeeding replacement

IF 0.1 Q4 OBSTETRICS & GYNECOLOGY

Case Reports in Perinatal Medicine Pub Date : 2021-01-01 DOI:10.1515/crpm-2019-0079

K. Schulpis, K. Iakovou

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Abstract

Abstract Obejctives Phenylketonuria (PKU) and hyperphenylalaninemia (HPHE) are metabolic disorders of phenylalanine (phe) metabolism due to the liver enzyme phe hydroxylase deficiency resulting in mental retardation. The only treatment of these disorders is the total or the partial breastfeeding replacement with special formula, respectively. Case presentation We aimed to evaluate the maternal stress degree in the two groups of mothers and compare the stress degree between mothers who were obliged to replace breastfeeding completely with those who replaced it partially. Mothers (n=50) were divided into two groups: n=25 with PKU and n=25 with HPHE infants. Mothers with PKU newborns were obliged to replace breastfeeding completely and feed their newborns with a common formula plus phe free medical food, whereas mothers with HPHE infants partially replaced breastfeeding with phe free medical food. Stress degrees were calculated using special questionnaire checklist by Bourne E. Psychological support was provided for both groups of mothers with stress. Conclusions Most mothers with PKU infants experienced high or severe stress, whereas mothers with HPHE newborns suffered from low and moderate symptoms and signs of stress. After psychological support most mothers with PKU newborns were ameliorated from stress symptoms; mothers with HPHE neonates experienced almost no stress symptoms. Mothers with PKU infants obliged to complete breastfeeding replacement experienced high and/or severe stress degrees as compared to mothers with HPHE newborns with partial breastfeeding replacement. Psychological support resulted in amelioration of both tested groups.

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有苯丙酮尿症或高苯丙氨酸血症婴儿的母亲在要求全部或部分母乳喂养时表现出的压力程度

【摘要】目的苯丙酮尿症(PKU)和高苯丙氨酸血症(HPHE)是由于肝酶苯丙氨酸羟化酶缺乏而导致的苯丙氨酸代谢代谢紊乱。治疗这些疾病的唯一方法是分别用特殊配方母乳喂养全部或部分母乳喂养。我们旨在评估两组母亲的压力程度，并比较被迫完全替代母乳喂养和部分替代母乳喂养的母亲的压力程度。母亲(n=50)分为两组:PKU组25名，HPHE组25名。患有PKU新生儿的母亲必须完全替代母乳喂养，用普通配方奶粉加免费医疗食品喂养新生儿，而患有HPHE婴儿的母亲则必须部分替代母乳喂养，使用免费医疗食品。采用Bourne e编制的特殊问卷表计算压力程度，并对两组有压力的母亲提供心理支持。结论:大多数患有PKU婴儿的母亲都有高度或严重的压力，而患有HPHE新生儿的母亲则有轻度和中度的压力症状和体征。经心理支持后，多数PKU新生儿母亲的应激症状得到改善;有HPHE新生儿的母亲几乎没有出现压力症状。与有部分母乳喂养替代的HPHE新生儿的母亲相比，被迫完成母乳喂养替代的PKU婴儿的母亲经历了高和/或严重的压力程度。心理支持对两组患者均有改善作用。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Case Reports in Perinatal Medicine OBSTETRICS & GYNECOLOGY-

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期刊介绍： Case Reports in Perinatal Medicine is a double-blind peer-reviewed journal. The objective of the new journal is very similar to that of JPM. In addition to evidence-based studies, practitioners in clinical practice esteem especially exemplary reports of cases that reveal specific manifestations of diseases, its progress or its treatment. We consider case reports and series to be brief reports describing an isolated clinical case or a small number of cases. They may describe new or uncommon diagnoses, unusual outcomes or prognosis, new or infrequently used therapies and side effects of therapy not usually discovered in clinical trials. They represent the basic concept of experiences for studies on representative groups for further evidence-based research. The potential roles of case reports and case series are: Recognition and description of new diseases Detection of drug side effects (adverse or beneficial) Study of mechanisms of disease Medical education and audit Recognition of rare manifestations of disease.