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2.
J Pediatr ; 137(4): 504-9, 2000 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-11035829

RESUMEN

BACKGROUND: Children with phenylalanine-hydroxylase deficiency (type-I hyperphenylalaninemia, HPA) follow a low-phenylalanine diet, severely restricted in animal foods and long-chain polyunsaturated fatty acids (LCPUFA). Consequently, they have a poor LCPUFA status, particularly for docosahexaenoic acid (DHA). DHA is relevant to visual and neural development. OBJECTIVE: To investigate the effects of a 12-month supplementation with LCPUFA in a double-blind, placebo-controlled trial in treated children with HPA. STUDY DESIGN: Twenty children with well-controlled HPA were randomly allocated to receive either a fat supplement (supplying 26% as fatty acids including DHA, 8%) or a placebo. The fatty acid composition of erythrocyte lipids and the visual evoked potentials were measured at baseline and after 12 months of supplementation. Reference data were obtained from healthy children of comparable age. RESULTS: At baseline children with HPA had a poorer DHA status and prolonged P100 wave latencies than the reference group. At the end of the trial the LCPUFA group showed a significant increase in DHA levels of erythrocyte lipids. In the LCPUFA group P100 wave latency decreased and was negatively associated with the DHA changes. CONCLUSIONS: A balanced dietary supplementation with LCPUFA in children with HPA is associated with an increase of the DHA pool and improved visual function.


Asunto(s)
Suplementos Dietéticos , Ácidos Grasos Insaturados/farmacología , Ácidos Grasos/metabolismo , Fenilalanina Hidroxilasa/deficiencia , Fenilalanina/sangre , Visión Ocular/efectos de los fármacos , Niño , Método Doble Ciego , Femenino , Humanos , Masculino , Visión Ocular/fisiología
4.
Infection ; 26(2): 93-9, 1998.
Artículo en Inglés | MEDLINE | ID: mdl-9561378

RESUMEN

Twenty-two Italian HIV-infected patients developed leishmaniasis, clinically manifested as visceral (13 cases), cutaneous (2 cases) and disseminated disease (7 cases). Twenty were males and two females (mean age: 32.8 years) with a mean CD4+ cell count of 46.8/microliter at diagnosis; risk factors were intravenous drug use (17 patients) and sexual behaviour (two bisexual, two homosexual, one heterosexual). All but one patient lived or travelled in hypoendemic Italian regions and other Mediterranean countries. Apart from the two patients with cutaneous leishmaniasis, the clinico-pathological and biological spectrum of the infection was often atypical, especially in patients with disseminated disease. The diagnosis was routinely made by direct recovery of parasites in biological specimens, mainly in bone marrow aspirate, whereas serology was negative or borderline in most of the patients. Among 17 in vitro isolates, Leishmania infantum was the only species involved with previously undescribed isoenzyme patterns in two cases. Treatment with antimonials and other drugs showed only temporary clinical improvement in some patients. Relapses were the rule. Leishmaniasis confirms itself as an opportunistic infection in HIV-positive persons. Secondary chemoprophylaxis should be considered in cases of relapsing disease.


PIP: The majority of the 850 HIV-associated Leishmania infections reported worldwide involve men and women from Mediterranean countries, particularly Spain, Italy, and France. This article describes a retrospectively identified series of 22 patients (20 men and 2 women) from northern Italy's Lombardy region with HIV/Leishmania coinfection in the period 1989-97. At leishmania diagnosis, the mean CD4+ lymphocyte count was 46.8/mcl and 21 patients had been previously diagnosed with an AIDS-defining illness. Intravenous drug use was the HIV risk factor in 17 patients; an additional 4 were bisexual or homosexual. The diagnosis of leishmaniasis was made by direct recovery of parasites in biologic specimens, mainly in bone marrow aspirate. Serology was generally negative or borderline due to the frequent occurrence of humoral immunity imbalances. Anemia, leukopenia, thrombocytopenia, and hypergammaglobulinemia were present in all but 1 patient. Leishmaniasis was clinically manifested as visceral in 13 cases, cutaneous in 2 cases, and disseminated disease in 7 cases. The clinicopathologic and biologic spectrum of infection tended to be atypical, especially in patients with disseminated disease. Leishmania infantum was the only species involved in 17 in vitro isolates; 2 cases exhibited previously undescribed isoenzyme patterns. Treatment with antimonials and other drugs produced, at best, only temporary clinical improvement. Relapses were the rule during follow-up in all but the 2 patients with cutaneous leishmaniasis. Inclusion of Leishmania spp. among the infectious agents of AIDS-defining diseases is recommended.


Asunto(s)
Infecciones Oportunistas Relacionadas con el SIDA/parasitología , Infecciones Oportunistas Relacionadas con el SIDA/diagnóstico , Infecciones Oportunistas Relacionadas con el SIDA/epidemiología , Adulto , Animales , Biopsia , Brasil/epidemiología , Côte d'Ivoire/epidemiología , Femenino , Humanos , Leishmania infantum/aislamiento & purificación , Leishmaniasis Visceral/diagnóstico , Leishmaniasis Visceral/tratamiento farmacológico , Leishmaniasis Visceral/epidemiología , Leishmaniasis Visceral/inmunología , Leishmaniasis Visceral/parasitología , Masculino , Región Mediterránea/epidemiología , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento
5.
J Pediatr ; 119(4): 562-7, 1991 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-1919887

RESUMEN

The fatty acid composition of plasma and erythrocyte lipids was analyzed in 15 children with phenylketonuria (aged 3 to 12 years) during dietary treatment aimed to maintain plasma phenylalanine levels at less than 8 mg/dl (485 mumol/L), and compared with those of 12 matched control subjects. The diet of children with phenylketonuria provided less protein, with a very low proportion of animal proteins, less fat, but a higher proportion of linoleic acid as a percentage of calories, and a higher carbohydrate content versus that in the diet of control subjects. The children with phenylketonuria had higher plasma levels of oleic acid but lower levels of arachidonic (n-6) and n-3 fatty acids. Linoleic and eicosatrienoic (n-9) acid levels were the same in both groups. These changes in patients with phenylketonuria resemble those observed in vegetarians and may be due to the absence of preformed arachidonic acid and long-chain n-3 fatty acids in the phenylketonuric diet.


Asunto(s)
Ácidos Grasos Insaturados/sangre , Fenilcetonurias/sangre , Niño , Preescolar , Colesterol/sangre , Dieta , Ingestión de Energía , Ácidos Grasos Insaturados/administración & dosificación , Femenino , Humanos , Masculino , Fenilcetonurias/dietoterapia
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