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 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Children with special health care needs are at increased risk for oral diseases. The purpose of this article was to discuss: nutritional and oral health factors routinely observed in most chronic childhood disorders; dietary modifications associated with select systemic disorders and how they may impact oral health in children; and the following factors common to chronic disorders associated with diet modifications-decreased appetite and increased nutritional risk; frequency of food intake; parental overindulgence; long-term use of cariogenic medications; and xerostomia. Characteristics of childhood disorders that require dietary modifications (congenital heart disease, cystic fibrosis, cancer, AIDS/HIV, diabetes mellitus, and phenylketonuria) are summarized. In addition, healthy dietary modifications and oral health recommendations are suggested. Implementation of these recommendations can assist the dentist and dental team as they join physicians and nutritionists in delivering the best possible care to children with special health care needs.
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PMID:Nutrition and oral health considerations in children with special health care needs: implications for oral health care providers. 2083 54

Screening of the newborn begins in the case room with a search for gross congenital anomalies. Early detection of such anomalies may help to prevent serious consequences. Problems include spina bifida, cleft palate, orthopedic abnormalities, congenital heart disease, imperforate anus, and esophageal atresia. In the hospital nursery, tests for phenylketonuria, hypothyroidism, hearing impairment, strabismus, and congenital hip dislocation are performed. Studies indicate that five to six well-baby visits in the first two years of life provide adequate screening of healthy infants from intact homes. During well-baby visits the physician should monitor rate of growth and risk of child abuse, and take steps to prevent childhood injuries. Appropriate screening for these conditions in every Canadian child will improve the health of the population.
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PMID:Preventive Health Examinations Part II. 2125 75

Untreated maternal phenylketonuria (MPKU) is a major cause of microcephaly, congenital heart disease, intrauterine growth retardation and mental retardation in the offspring of mothers who have the disease. There is evidence, however, that dietary restriction of phenylalanine in the mother before conception and throughout the pregnancy will reduce the risk of these congenital anomalies in the fetus. It is important to be alert to this preventable cause of developmental retardation and congenital abnormalities in all pregnancies until the stage is reached where every woman of child-bearing age has been through the neonatal PKU-screening program. Family physicians are advised to consider prenatal or premarital screening for PKU of all female patients of child-bearing age for the next generation.
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PMID:Maternal Phenylketonuria (MPKU). 2126 27

Maternal Phenylketonuria Syndrome (MPKU) can occur in infants born to mothers with PKU with poor metabolic control during pregnancy. Elevated phenylalanine (phe) acts as a teratogen to the developing fetus with consequences including intellectual disability, microcephaly, facial dysmorphism, growth retardation, and congenital heart disease. MPKU can be prevented if metabolic control is achieved by 8-10 weeks gestation. If control is not achieved, there is a significant risk for MPKU. Therefore, in women with poor metabolic control at time of pregnancy, establishing metabolic control quickly is important.Clinically, establishing metabolic control in women with PKU can present challenges. Social issues, psychological issues, and insufficient education about PKU play an important role in a patient's inability to reinstitute this challenging diet. Maintaining phe levels within a range to allow for infant growth, while preventing toxicity, is challenging, particularly for those women who no longer follow the PKU diet. Gastrostomy tube placement is an option to deliver medical formula to women who are unable to restart diet due to severe nausea or palatability issues.Here we discuss two pregnancies in which a gastrostomy tube was placed to achieve metabolic control after other measures failed to reduce phe concentrations into the recommended range. For these two pregnancies, placement of the gastrostomy tube led to improvement in phe levels with normal infant outcomes including normal growth, head circumference, and heart structure.
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PMID:Use of Gastrostomy Tube to Prevent Maternal PKU Syndrome. 2343 Sep 33

Treatment of maternal phenylketonuria (PKU) consists of a low phenylalanine diet, begun before conception, which is believed to prevent mental retardation, microcephaly, and congenital heart disease in offspring of women with PKU. Experiences in treating these women indicate that their cooperation with medical recommendations is generally poor. We present a psychosocial model on adjustment and coping in maternal PKU. The proposed model defines four stages in the reproductive years of women with PKU, each of which has specific behavioral goals. The four stages are: (1) prevention of unplanned pregnancies; (2) reproductive decision-making; (3) diet initiation; and (4) diet continuation through pregnancy. Review of the literature on the different behaviors expected at the different stages enabled indentification of psychosocial factors that may explain success or failure in achieving the goals at each stage. Based on this theoretical framework, practical implications for treating women with PKU have been suggested.
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PMID:A psychosocial model of a medical problem: Maternal phenylketonuria. 2426 62

Strict control of hyperphenylalaninemia is necessary in pregnant women with phenylketonuria (PKU) in order to prevent phenylalanine embryopathy in the fetus, characterized by intrauterine growth restriction, dysmorphic facies, congenital heart disease, microcephaly and intellectual disability, collectively known as maternal PKU syndrome. Sapropterin dihydrochloride (SD), an alternative or adjunct to dietary therapy in patients with tetrahydrobiopterin (BH4)-responsive PKU, has recently been used in several cases to treat PKU during pregnancy with satisfactory results. Here, we report two pregnancies treated with SD and unrestricted diet in a patient with BH4-responsive mild PKU. The first pregnancy resulted in a partial hydatidiform mole and was terminated, whereas a healthy infant was born from the second pregnancy. Phenylalanine control was optimal in both pregnancies. To the best of our knowledge, this is the first report on the development of partial hydatidiform mole associated with SD treatment and the second report on molar pregnancy in PKU. While the relation between SD and molar pregnancy is unknown, further studies may be needed to investigate the possible effects of SD on fertilization.
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PMID:Partial hydatidiform mole in a phenylketonuria patient treated with sapropterin dihydrochloride. 2789 72


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