PubMed Health⌕ Search

Biomedical subjects

W Bonacci

Publications and source records attributed to W Bonacci.

At least 19 recordsLinked to original sources

Maternal dietary PUFAs intake and human milk content relationships during the first month of lactation.

Maternal dietary fatty acids (FFAs) intake and corresponding human milk composition relationships have been assessed throughout the first month of lactation in 34 lactating women consecutively enrolled. All mothers were on their habitual diet. Food records (95 items) were administered to the mothers, six-times during the first month of lactation (1 day after delivery, 4, 7, 14, 21, and 28 days after colostrum appearance) and referred to maternal dietary intake of the day before. Milk collected on day 1 was considered as colostrum, day 4 and 7 samples as transitional milk, and day 14, 21 and 28 samples as mature milk. Five gas chromatographic analyses were performed on each sample. Statistics were made using Friedman's and Pearson's test. Maternal dietary saturated fatty acids (SFAs) and monounsaturated fatty acids (MUFAs) were significantly related to the corresponding milk pattern in the phase of transitional milk (P<0.01), while total polyunsaturated (PUFAs) content was significantly related only to the mature milk (P<0.01); in this phase about 42% of the variations occurring in PUFAs milk content can be related to variation of maternal PUFAs dietary intake. The results in the present study provide evidence of the relationships between maternal diet and milk composition. The degree of correlation between maternal diet and PUFAs milk content increases throughout milk maturational process and reaches significance only in mature milk. This would imply that advancing lactation, milk PUFAs provision sources gradually shift from adipose tissue catabolism to maternal diet.

Adipose Tissue↗

A randomised control study comparing the Infant Flow Driver with nasal continuous positive airway pressure in preterm infants.

OBJECTIVE: To compare the effectiveness of the Infant Flow Driver (IFD) with single prong nasal continuous positive airway pressure (nCPAP) in preterm neonates affected by respiratory distress syndrome. DESIGN: Randomised controlled study. PATIENTS: Between September 1997 and March 1999, 36 preterm infants who were eligible for CPAP treatment were randomly selected for either nCPAP or IFD and studied prospectively for changes in oxygen requirement and/or respiratory rate. The requirement for mechanical ventilation, complications of treatment, and effects on mid-term outcome were also evaluated. RESULTS: Use of the IFD had a significantly beneficial effect on both oxygen requirement and respiratory rate (p < 0.0001) when compared with nCPAP. Moreover, O(2) requirement and respiratory rate were significantly decreased by four hours (p < 0.001 and p < 0.03 respectively). The probability of remaining supplementary oxygen free over the first 48 hours of treatment was significantly higher in patients treated with the IFD than with nCPAP (p < 0.02). IFD treated patients had a higher success (weaning) rate (94% v 72 %) and shorter duration of treatment (49.3 (31) v 56 (29.7) hours respectively; mean (SD)), although the difference was not significant. CONCLUSIONS: IFD appears to be a feasible device for managing respiratory distress syndrome in preterm infants, and benefits may be had with regard to oxygen requirement and respiratory rate when compared with nCPAP. The trend towards reduced requirement for mechanical ventilation, shorter clinical recovery time, and shorter duration of treatment requires further evaluation in a multicentre randomised clinical trial.

Female↗

[Neonatal hemochromatosis: significance of early identification].

Neonatal hemochromatosis (NH) is a rare clinical-pathologic entity defined by severe neonatal liver failure (NLF) of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements. Whether this entity is the result of a distinct disease process or is the pathologic end-result of many other forms of NLF remains to be assessed. Death from multisystem organ failure usually occurs in the first few days or weeks of life. We report two sisters with neonatal hemochromatosis with different outcome. The first died at 21 days of life for multiorgan failure and sepsis. The second was diagnosed and treated very early by aggressive support with fresh-frozen plasma, packed red cells, platelets, coagulation factors, diuretics, and pressors, and she is well at 2-year-follow-up. The few therapeutic options are discussed. In general, few patients survived NH. Among patients treated only by medical approachs and that did not undergo orthotopic liver transplantation (OLTX), favorable outcome is only very rarely reported. For management of NH infants early recognition of the disease is critical. Our experience seems to suggest that as early is the start of treatment as high is the possibility of survival. At this moment very early aggressive supportive treatment should appear to be the most useful approach to stabilize the patient hoping in spontaneous liver recovery, or, in turn, to permit OLTX before septic complications bring to clinical decline.

Female↗

Elevated S100 blood level as an early indicator of intraventricular hemorrhage in preterm infants. Correlation with cerebral Doppler velocimetry.

The aim of this study was to assess the use of S100 protein in blood as a means of identifying preterm infants at risk of intraventricular hemorrhage. In 25 preterm newborns, S100 blood concentrations were measured by an immunoradiometric assay during the first 48 h. Cerebral Doppler velocimetry waveform patterns were also tested at the time the blood sample was taken, when clinical and cerebral ultrasound scanning were still normal. Of the 25 newborns studied, 14 were controls and 11 developed intraventricular hemorrhage as revealed by ultrasound scanning more than 72 h after birth, and clinically confirmed by neurological examination on the seventh day of follow-up. S100 blood concentrations were significantly higher (P<0.002) in infants with intraventricular hemorrhage than in control infants and also correlated significantly (r=0.81, P<0.003) with the grade of hemorrhage. A significant correlation (r=0.70, P<0.05) between the S100 blood concentration and the middle cerebral artery pulsatility index was also observed. The present data show that S100 blood concentrations offer a measurable parameter of brain lesion in preterm infants before a radiological assessment of hemorrhage can be performed, when clinical symptoms may be silent and preventive/therapeutic action could be especially useful.

Blood Flow Velocity↗

Diagnosis of neonatal hemochromatosis with MR imaging and duplex Doppler sonography.

Neonatal hemochromatosis is a rare congenital disorder which affects both fetuses and newborns. It is characterized by hepatocellular failure, often appearing on the first day of life in the form of coagulopathy, hypoalbuminemia, hypoglycemia, and jaundice. Most of the affected infants die early in life, and definitive diagnosis has often been made only by post-mortem evaluation. With the help of MRI, plus increasing awareness of the disorder, diagnosis is now often made early, even in utero. Duplex Doppler sonography does not provide information on siderosis but shows abnormalities in the liver or blood-flow patterns associated with liver disease.

Biopsy↗

Severe complex I deficiency in a case of neonatal-onset lactic acidosis and fatal liver failure.

We report a newborn admitted to our service on the 2nd day of life because of hypotonia and metabolic acidosis. A progressive hepatocellular dysfunction dominated the clinical picture and the patient died at 13 months of age because of severe hepatic failure. Persistent lactic acidosis, high ketone bodies levels and high-normal lactate/pyruvate and 3-hydroxybutyrate/acetoacetate molar ratios in plasma were found. Investigation of a liver biopsy revealed low activities of all the mitochondrial respiratory chain enzymes but in particular a marked decrease of complex I (NADH cytochrome c reductase) activity. All respiratory chain enzyme activities were normal in cultured skin fibroblasts. Mitochondrial DNA analysis failed to detect any major rearrangements. Although only a few cases have been reported so far, it is becoming clear that liver should be considered as one of the organs involved in oxidative phosphorylation disorders. The finding of unexplained progressive liver failure with poor neurological conditions, lactic acidaemia and ketonuria strongly warrants investigation for a respiratory chain disorder. Moreover, the finding of normal respiratory enzyme activities in a tissue other than liver does not rule out the existence of an oxidative phosphorylation disorder in patients with hepatocellular disease of unexplained origin.

Acidosis, Lactic↗

Fatty acid composition of human milk in Italy.

The fatty acid composition of breast milk from 20 Italian women, delivering at term and on ad libitum diets, was analyzed with high-resolution gas chromatography. Milk samples were collected twice a day, on the 1st, 4th, 7th, 14th, 21st and 28th day after colostrum appearance. No significant differences were detected between the two daily samples. During the maturation process a significant reduction in long-chain polyunsaturated fatty acids of the n-6 series (p = 0.002) and n-3 series (p = 0.005) was recorded, particularly in arachidonic acid (p = 0.035), docosatetraenoic acid (p = 0.035) and docosahexaenoic acid (p = 0.032). The linoleic acid/n-6 and alpha-linolenic acid/n-3 ratios increased (p = 0.024 and p = 0.037), while the docosatetraenoic/docosahexaenoic acid ratio decreased (p = 0.032). The fatty acid composition of mature milk was the following: saturated 45.50%; unsaturated 54.51%; monounsaturated 42.69%; polyunsaturated 11.82%; long-chain polyunsaturated 1.27%; linoleic acid 9.79%, and alpha-linolenic acid 0.36%. The fatty acid composition of milk collected from Italian women appears similar to that of women in other southern European countries and, therefore, could reflect dietary habits.

Adult↗

Biochemical diagnosis and outcome of 2 years treatment in a patient with combined methylmalonic aciduria and homocystinuria.

We describe a patient with methylmalonic aciduria and homocystinuria due to a defect in cobalamin metabolism of the Cbl-C type mutant (McKusick 277400). Our case was diagnosed within the first 2 months of life by amino acid analysis (ion-exchange chromatography) and by biochemical studies in cultured fibroblasts ([14C]propionate incorporation, methionine and serine formation). We discuss the clinical course and the biochemical evolution after 2 years of hydroxycobalamin treatment that led to an improvement in general clinical condition and neurological performance.

Amino Acids↗

Therapeutic treatment of systemic candidiasis in newborns.

This report details the management of a group of newborns who had a diagnosis of systemic Candida albicans septicemia. All the infants were extremely preterm (29 weeks' gestation or less) and had predisposing factors to disseminated fungal infections. Diagnosis was made by cultures from blood, urine, and from the tip of indwelling catheters. Therapy was based in all the infants on the combination of amphotericin B (Amph. B) and 5-fluorocytosine (5FC). Amph. B was intravenously administrated at a test dose of 0.1 mg/kg which if tolerated, was increased with scalar doses until a maximum of 0.35-0.50 mg/kg/die. 5FC was intravenously or orally administered at a 100-200 mg/kg/die dose. Time of treatment with Amph B was prolonged from 13 to 30 days and that with 5FC from 11 to 40 days. In two infants there was an increase of transaminases and Gamma GT and no infant had signs of nephrotoxicity. The high therapeutic success (75% of cases) confirms the validity of the utilized therapeutic schemes.

Amphotericin B↗

[Cerebral arteriovenous malformations (CAVM)].

Two infants with endocranic A-V malformations and irreversible picture of congestive cardiac failure are presented: the first is a newborn with a very large angioma, the second is a newborn with a large aneurysm of the vein of Galen. A review of the literature is presented: the salient age-related features required to make a diagnosis are discussed.

Cerebral Arteries↗

Cerebral ultrasound images in prenatal cytomegalovirus infection.

A male newborn with prenatal cytomegalovirus infection was referred for cranial ultrasound. The cranial ultrasound demonstrated areas of increased echogenicity in the thalamic and gray nuclei resembling "a branched candlestick". Doppler technique located the "branched candlestick" along the thalamostriate arteries. This image is particularly interesting because to our knowledge it has never before been described in congenital cytomegalovirus infection, but only in congenital rubella.

Brain↗