Cardiac outputs of control individuals and cancer patients and evidence of deficiencies of coenzyme Q10 and vitamin B6.
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Biomedical subjects
Publications and source records attributed to L Baker.
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Eighteen appropriate-for-gestational-age premature infants with birth weights ranging from 0.77 to 1.89 kg received 1 gm/kg of body weight of fat emulsion, intravenously, over a four-hour period. Infants less than 1 week of age developed a significant decrease in PO2 levels (P < 5.0) during the fat infusion period. There were no changes in other pulmonary function parameters. Infants less than 1 week of age also developed significantly higher peak levels of plasma triglycerides than infants 2 to 3 weeks old (P < .05). A correlation between increment in triglyceride levels and postnatal age was demonstrated (r = .75), with the younger infants presenting the higher triglyceride levels. This study demonstrates that: (1) small premature infants receiving intravenous fat are more susceptible to hyperlipemia and hypoxemia during the first week of life; (2) hypoxemia associated with intravenous fat infusion does not result from changes in lung dynamics; (3) the capacity to tolerate intravenous fats is enhanced after the first week of life.
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Patterns of parent-child interaction and family functioning were systematically examined in well-matched groups of 15 autistic and 14 dysphasic children. The measures used included the Douglas 24-hour standard day analysis, the Brown and Rutter interview measure of positive interaction, the Ittleson scales (based on a four- to six-hour period of home observation, specially developed time-sampled measures of observed mother-child interaction at home), and the Eysenck Personality Inventory. The findings from all measures agreed in showing that family life and interaction patterns were closely similar in the two groups. The results were compared with those of previous investigations; we concluded that autism is most unlikely to be due to abnormal psychogenic influences in the family.
Elevated serum levels of carcinoembryonic antigen (CEA) were found in 70% of 141 patients with advanced gastrointestinal (GI) cancers. Serial CEA measurements were performed on 70 patients before and during chemotherapy. The majority were treated with 5-FU and Methyl-CCNU (33 patients), 5-FU (19 patients), or 5-FU and mitomycin-C (8 patients). In 49 patients with colorectal carcinoma who had elevated serum CEA prior to chemotherapy, 18 had objective partial tumor remission, 16/18 (89%) showed definite decrease in CEA level, one had no change, and one had an increase CEA titer. Thirty-one patients had either stable disease (10 patients) or increasing disease (21 patients) while on chemotherapy. Of these patients four showed decrease in CEA, eight had no change, and 19 had increase in CEA levels as compared to pretreatment value. The survival of patients with a decrease in CEA during chemotherapy was statistically significant (p = .03) as compared to survival of those with no change or increasing CEA levels. In 21 patients with other GI cancers, the correlation between the clinical response and change in CEA level observed was not as definite as in patients with colorectal carcinoma.
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In the present study we used a developmental morphologic approach to fix the latest time in development at which the malformations commonly reported in infants of diabetic mothers could occur. Developmental morphologic dating shows that the significantly more common congenital malformations in infants of diabetic mothers occur before the seventh week of gestation. This suggests that any therapeutic intervention aimed at decreasing the incidence of congenital malformations must be instituted during the critical early period.
To examine why newborn infants frequently cannot maintain adequate levels of plasma glucose in the interval between delivery and the time they are first fed, circulating metabolic fuel and regulatory hormone concentrations were determined in 44 healthy infants at the end of an eight-hour postnatal fast. Plasma glucose fell below 40 mg/100 ml prior to eight hours in four of 24 term-appropriate-for-gestational-age (AGA), two of nine preterm-AGA, five of six term-small-for-gestational-age (SGA), and three of five preterm-SGA infants. Fuel and hormone patterns in the premature and SGA infants were not different from those found in term-AGA infants. Results in these neonates differed in two areas from the response to fasting seen later in life. In fasted term-AGA infants, ketones were low (beta-hydroxybutyrate 0.29 +/- 0.04 mM/liter) despite elevated concentrations of fatty acid precursors (1.4 +/- 0.07 mM/liter), and the group of infants studied failed to demonstrate the increase in plasma ketones with lower glucose levels (r = ".23, P = .07) which is found in older children. Levels of glucose precursors were two to three times higher in term-AGA infants (lactate 2.9 +/- 0.2 mM/liter; alanine 0.48 +/- 0.02 mM/liter) than levels found beyond the neonatal period and, in contrast to older children and adults, were not diminished in infants with lower plasma glucose (lactate, r = -.28, P less than .035; alanine, r = -33, P less than .02). These differences between the responses to postnatal fasting and those seen beyond the neonatal period suggest that the capacity for both hepatic ketone synthesis and gluconeogenesis is not fully developed at birth.
Regardless of the well-documented population associations between juvenile-onset diabetes (JOD) and certain HLA types, whatever haplotypes are segregating in JOD families may be followed to provide information on mode of inheritance of the disorder. It is essential to group together for analysis families with the same number of affected sibs. We assume a single locus determining susceptibility, closely linked to the HLA region, and ignore recombination, expected to be rare. Our first approach also assumes that the frequency of the susceptible genotype is so small that affected individuals may be considered to arise from only one mating type; the particular mating type depends on the mode of inheritance of susceptibility. Our sample is the result of pooling our own data with published studies of HLA haplotype segregation in families with two or more JOD offspring. Given the assumptions, we find that the data are more plausibly explained by a one-dose than by a two-dose or "recessive" hypothesis. We then develop the analysis further by adding a crude but explicit estimate of the frequency of the susceptible genotype, based on disease prevalence and penetrance of the genotype. The one-dose hypothesis is strongly supported by this analysis as well. We also consider some problems of ascertainment arising from heterogeneity of the disorder and selection against diabetics. Studies involving unaffected relatives of diabetics are suggested which might test further the conclusions drawn here.
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Islet cell adenomas are an important consideration in infants and children with hypoglycemia due to hyperinsulinism. Between 1965 and 1977, 32 patients with hyperinsulinism were seen at the Children's Hospital of Philadelphia. Sixteen of these patients underwent surgery. Eight patients had single pancreatic adenomas, 5 of whom were infants under 1 yr of age. In 3 infants an adenoma was recognized and removed at the time of surgery. In 4 patients a subtotal pancreatectomy was successful in removing the adenoma. One infant is whom the adenoma was not included in the subtotal pancreatectomy died postoperatively with intractable hypoglycemia. Of the 7 patients cured by surgery, 6 are normal and 1 has brain damage due to delay in treatment of hypoglycemia. One child has mild diabetes. A combined medical and surgical protocol has been developed that permits rapid diagnosis of hyperinsulinism and selection of candidates for surgery. Infants under 1 yr of age who fail to respond to diazoxide should be explored. In children whose hyperinsulinism appears beyond 1 yr of age, surgery is indicated, since an adenoma is likely.
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Chromomycin A3 was administered iv to 26 patients in a phase I trial. The maximum tolerated dose established in this study was 0.75 mg/m2/day iv X 5 days. The drug was then given to 48 evaluable patients with far-advanced disseminated breast cancer. Two short partial remissions and one clinical improvement were seen. Toxic manifestations consisted of frequent and usually reversible renal toxicity, nausea and vomiting, occasional thrombocytopenia, hypocalcemia, and two instances of semicoma. Drug toxicity may have contributed to the death of two patients.
In an attempt to substitute ftorafur for burdensome 5-fluorouracil (5-FU) infusions, 52 previously untreated patients were randomized to receive ftorafur with either mitomycin C or methyl-CCNU. Ftorafur was administered monthly as a 2-hour infusion daily x 5 days. Mitomycin C and methyl-CCNU were repeated every 8 weeks. A response rate of 27% (seven responses among 26 patients) was demonstrated on the mitomycin C arm compared to a response rate of 15% (four responses among 26 patients) on the methyl-CCNU arm (P = 0.25). There was no significant difference in the median survival between treatment arms. Central nervous system toxicity occurred in greater than 30% of the patients and appeared to be the limiting factor with ftorafur administration. Alternate schedules of ftorafur should be explored since there appears to be little advantage of a daily ftorafur schedule over conventional 5-FU infusions.