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Biomedical subjects

T B Ferrara

Publications and source records attributed to T B Ferrara.

21 records · Page 2Linked to original sources

Maternal phenylketonuria-chronology of the detrimental effects on embryogenesis and fetal development: pathological report, survey, clinical application.

Maternal phenylketonuria (PKU) is likely to have detrimental effects on embryogenesis and fetal development. Manifestations in the offspring include spontaneous abortion, various congenital malformations, intrauterine growth retardation, and microcephaly. The time at which the metabolic abnormalities induce pathologic embryogenesis can be documented by knowing the time of the development of specifically damaged organ systems. This review reveals that, while the most recognized congenital malformations occur in the heart, the most common abnormality is growth inhibition occurring throughout pregnancy. The organ system most commonly affected by this growth inhibition is the brain, resulting in a high incidence of micrencephaly. It appears that maternal phenylketonuria interferes with appropriate fetal growth and that this effect occurs during the entire course of pregnancy and has no tissue specificity. This information can be both informative to pathologists and useful to clinicians.

Embryonic and Fetal Development↗

Nissen fundoplication for gastroesophageal reflux in infants.

Some degree of gastroesophageal reflux is very common in infants and tends to reverse with time. Therefore, the indications for an antireflux operation are not well defined. Furthermore, the complication rate and the ability of the fundoplication to grow remain to be determined. To answer these questions, we reviewed the records of patients 6 months of age or younger who underwent a Nissen fundoplication with gastrostomy tube placement between 1979 and 1985. There were 45 patients (25 boys and 20 girls) with birth weights of 0.65 to 4.3 kg. The consequences of gastroesophageal reflux were more varied than in older children. Severe respiratory problems were common, including recurrent aspiration or bronchopulmonary dysplasia in 60% and frequent apneic and bradycardiac spells in 17%. Failure to gain weight was present in 20% and intractable vomiting in 2.0%. As expected, 78% of these patients had congenital anomalies or acquired problems which, in many cases, were important to the prognosis. The diagnosis was confirmed by barium swallow in all but one patient in whom gross reflux during feedings was present. Initially, medical management was tried for 3 to 4 weeks. In one patient, however, the severity of the respiratory problems precluded trial beyond 12 days. The recommendation for operation was based only on the severity of symptoms attributed to gastroesophageal reflux. All patients underwent Nissen fundoplication with gastrostomy tube placement at 2 weeks to 6 months of age and weighing 1.02 to 6.95 kg. The only surgical complication was one gastrostomy leak. Prematurity or preexisting anomalies led to a 20% incidence of late unrelated deaths between 2 weeks and 23 months postoperatively. Improvement in symptoms occurred in our survivors with follow-up of 5 to 72 months. We conclude: Significant gastroesophageal reflux in infancy most frequently produces respiratory problems that can be life threatening. Nissen fundoplication can be a safe and effective procedure in infants 6 months of age or younger. Fundoplication appears to have good growth potential, and no late complications or feeding problems have occurred. Consequently, surgical correction can be recommended for infants not responding to conservative medical therapy.

Barium Sulfate↗

Localization and quantitation of anionic charge sites in fetal and neonatal alveolar basement membranes.

A method utilizing biopsy sized samples of lung for anionic charge site localization in alveolar and capillary basement membranes in human tissue is discussed. Tissue fixed in either paraformaldehyde-lysine-periodate or 1% paraformaldehyde with 0.05% glutaraldehyde, cut into 30 mu sections, and incubated with the cationic probe, polyethyleneimine, was processed for electron microscopic analysis using standard techniques. Anionic charge sites were identified and regularly distributed in increments of approximately 40-50 nm in the lamina rara externa of the alveolar basement membrane, with lesser amounts found in the lamina rara interna and lamina densa. Anionic charge sites were also demonstrated in the interstitial portion of the capillary basement membrane and on cell surfaces. These methods can be used to more broadly define the localization of anionic charge sites in human lung tissue in both normal and pathologic states.

Anions↗