Robert O. Fisch

The Effects of a Pacifying Stimulus on Behavioral and Adrenocortical Responses to Circumcision in the Newborn

Eighteen, healthy male newborns, 2–5 days old, were subjects in this study of the effects of a pacifier on the newborns behavioral and adrenocortical responses to circumcision. Half of the subjects were randomly assigned to a condition in which they were encouraged to suck on a pacifier during circumcision, while half served in a no pacifier, control condition. Behavioral observations were made for 1/2 hour before, during, and after circumcision; while blood samples for serum cortisol determination were obtained immediately before circumcision and 30 min later. The results showed that stimulating the newborn with the pacifier reduced crying by about 40%. Reducing crying, however, had no significant effect on the adrenocortical response. Elevations of serum cortisol predicted average behavioral state following circumcision, whereas crying during circumcision did not. Furthermore, there was evidence that the neonatal adrenocortical system was sensitive to variations in surgical procedures. The results indicate the importance of obtaining data on both behavioral and hormonal systems in studies of stress and coping in human newborns.

Neonatal serum bilirubin levels related to cognitive development at ages 4 through 7 years.

instances of cryptococcosis. 4 Salyer et al ~ have proposed that the pathogenesis of primary pulmonary cryptococcal infection may be similar to that of primary pulmonary tuberculosis; the subpleural nodule often found at autopsy represents the initial focus of infection, and spread to the hilar lymph nodes follows. Our patient had no clinical evidence of parenchymal lung infection but did have paratracheal adenopathy. Since direct inoculation of the skin may produce cutaneous cryptococcosis2 our patients scalp lesion may represent the portal of entry, with subsequent preauricular adenopathy. There was, presumably, hematogenous spread, as evidenced by the inguinal adenopathy, subcutaneous nodules, and liver granulomata. Confronted with cryptococcal infection, the clinicians impulse is to search for a predisposing condition, such as a malignancy, prolonged steroid therapy, or diabetes. However, none will be found in about 50% of patients. 7-9 Traditionally the treatment for cryptococcosis has been amphotericin. Recently 5-fluorocytosine has been used experimentally, even for meningitis, in patients unable to tolerate amphotericin. 9- 10 Because our patient was clinically stable and ambulatory, and had no evidence of meningitis, a trial of 5-fluorocytosine was begun, to avoid amphotericin toxicity. There was rapid and definitive resolution of all evidence of cryptococcal disease. Because some patients will not respond to a relatively low dose (60 mg/kg) of 5-fluorocytosine, they may need either a higher

Psychiatric diagnosis and behavioral characteristics of phenylketonuric children

This study evaluated the psychiatric characteristics and the behavioral effects of an acute load of phenylalanine on phenylketonuric children. The sample consisted of 13 normal-IQ children who were identified at birth as having phenylketonuria and were subsequently treated with a strict low phenylalanine diet. After baseline measurements, the subjects were loaded with placebo or phenylalanine following a double-blind design. Behavioral and metabolic variables were monitored at hourly intervals for five subsequent hours. The effects of acute loading were measured by changes in a standardized Continuous Performance Task (CPT). Psychiatric symptoms were compiled through a structured psychiatric interview of the child and the parent. Results demonstrated that phenylalanine loading, compared to placebo, caused a nonsignificant decrement in performance on CPT testing. Psychiatric disorders were found in six of the 13 subjects with three currently meeting criteria for attention deficit disorder (ADD). In addition, six other subjects were diagnosed in the past as having ADD using DSM-III criteria.

Physical and Mental Status at 4 Years of Age of Survivors of the Respiratory Distress Syndrome.

Fifty-eight survivors of the respiratory distress syndrome were evaluated at age 4 years for deficits in neuromotor performance and physical growth in a prospective study. Comparisons between RDS survivors and 290 matched controls plus 35,198 unmatched controls demonstrated that for each group the neuromotor performance was adversely affected by either low birth weight or low socioeconomic standing. Except for infants with birth weights above 2.5 kg, the RDS survivors performed as well as the two control groups. Comparisons with siblings failed to reveal a decrement in the RDS survivors. Hypothyroidism was more frequent among the RDS survivors than among the control subjects in the highest category of birth weight.

Homotransplantation of the liver in a patient with hepatoma and hereditary tyrosinemia

A girl with hereditary tyrosinemia, diagnosed at 6 months of age, was treated with a diet restricted in phenylalanine and tyrosine. At 9 1/2 years of age she developed an acutely enlarged liver and spleen, and the diagnosis of hepatocarcinoma was made. The patient received a liver transplant and tyrosine metabolites became normal while she was receiving a regular diet. Three months later, an infected thrombosis of the portal vein caused her death. Liver transplant appears to be an effective method of enzyme replacement in tyrosinemia and should be considered for prevention of hepatoma.

Identification of heterozygotes with phenylketonuria on basis of blood tyrosine responses

The differences in the responses of the tyrosine concentration in the serum to a two dose oral loading test with l -phenylalanine provided good separation of heterozygotes with phenylketonuria from control subjects. Determination of a discriminating factor by a combination of either the fasting, one, and two hour tyrosine values, or only the one and two hour values, permitted separation of the two groups with approximately a 5 per cent error. The discriminating values so derived gave an expression of differences in the rate of formation of tyrosine from l -phenylalanine within the two hour period and appeared to be a critical method of measuring the presence of a partial gene-enzyme deficiency.

The development of blinking in response to impending collision in preterm, full-term, and postterm infants*

A series of studies on the development of depth perception investigated the effects of age from birth and age from conception on the frequency of defensive blinking at an approaching object. The initial study found that at 10 weeks after birth preterm infants respond less frequently to an approaching object than full-term and postterm infants. When preterm infants were tested at the same age from conception as a group of full-term infants, response rates were equal. In a second study, postterm infants at 6 weeks after birth blinked at the approaching object more frequently than 6-week-old full-term infants; this finding was replicated in a subsequent study. In a final experiment with 8-week-old full-term infants, we found that the individual infant tested on two successive days was highly consistent in his or her level of responding. These results argue that maturation plays an important role in the development of responsiveness to spatial information.

Phenylketonuria: Current dietary treatment practices in the United States and Canada

OBJECTIVE A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU. METHODS A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey. RESULTS The majority of the centers, 87%, favor life-long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one-third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0% compared to < 0.06% in the general population). Because of dietary noncompliance, 1% of the children were removed from the home. DISCUSSION The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.

Neurological status of survivors of neonatal respiratory distress syndrome: A preliminary report from the collaborative study*

The neurological development of infants who survived the respiratory distresssyndrome has been reviewed in a prospective study. The subjects for this study consisted of 34,792 live-born infants including 59 survivors of the respiratory distress syndrome. The subjects were divided on the basis of birthweight into 3 categories, and in each category comparisons were made between those who survived the respiratory distress syndrome and those who did not have it. Neurological abnormalities were more frequent during the first year of life in the survivors of the respiratory distress syndrome than in the control subjects in each of the birthweight categories.

Children with phenylketonuria: the interface of family and child functioning.

Functioning and coping of 43 families with children with phenylketonuria (PKU) was investigated. A significant positive correlation was found among perceived family cohesion, dietary adherence associated with metabolic control, and child IQ. Parental education also significantly related to dietary adherence and to higher IQ level in the child. In addition, paternal perception of family adaptability was related to the childs IQ. Child depression as perceived by the parents was related to family functioning and coping variables. Functioning of PKU families is discussed in relation to current theories of family functioning under stress.