Persistent hypertransaminasemia as the presenting finding of childhood muscle disease.

Four children with isolated, persistent elevations of serum transaminases were investigated for hepatic disease and followed for 4 to 24 months before serum creatine kinase determinations were obtained and found to be markedly elevated. Evidence of muscle disease was obtained by close questioning, retrospectively, and mild abnormalities were found on physical examination. Review of laboratory tests in our center for 6 months revealed 30 additional cases of anicteric hypertransaminasemia (20% of those with elevated enzymes), only two of which were unexplained by the admitting diagnosis.

Inner ear anomalies in two cases of trisomy 18.

Two cases of trisomy 18 in which temporal bone defects were limited to the inner ears are described. Several abnormalities were present that have not been described previously in this syndrome. The first case involved a 1-month-old female infant who died of congenital heart defects.

Genetic analysis of crossfostering data with sire and dam records.

A method is presented for the analysis of data from crossfostering experiments in which parts of litters are reciprocally interchanged at birth. Observed variances and covariances of differently related individuals are expressed as functions of theoretical causal components of phenotypic variance (additive direct, dominance direct, additive maternal, dominance maternal, direct-maternal covariance, and environmental). Causal components are estimated by weighted least squares analysis of this system of equations, including a ridge-regression procedure to examine consequences of correlation between observed components.

Sternal malformation/vascular dysplasia association.

Sternal defects associated with superficial craniofacial vascular lesions are rare. We report on two additional patients with a sternal cleft and cutaneous, craniofacial hemangiomata to emphasize that this unusual combination of findings represents a recognizable sternal malformation/vascular dysplasia association. In addition, internal vascular lesions were also identified in these individuals, in one instance involving the upper respiratory tract and in the other the viscera.

Gastric emphysema.

Gastric emphysema is a rare condition in which gas from a nonbacterial source accumulates within the wall of the stomach. Gastric distension and vomiting frequently precede the formation of the intramural air. Pulmonary disease, instrumentation of the stomach, and obstructing lesions of the antrum and pylorus are also common contributing factors.

Pseudo dominant-lethal response in female mice treated with plant oils.

Corn oil, sesame oil, peanut oil, or olive oil, injected intraperitoneally to female mice prior to insemination, increased the number of deciduomata. In many plant oil-treated females the number of implantation sites was markedly higher than corpora lutea count. These effects were not observed among females that were treated similarly with mineral oil or among females that were given corn oil via oral administration.

A "new" lethal multiple congenital anomaly syndrome: joint contractures, cerebellar hypoplasia, renal hypoplasia, urogenital anomalies, tongue cysts, shortness of limbs, eye abnormalities, defects of the heart, gallbladder agenesis, and ear malformations.

Three cases of a lethal malformation syndrome with severe visceral anomalies were seen in two families and include one pair of sibs. The predominating external manifestations are mesomelic dwarfism, micrognathia, V-shaped upper lip, microglossia, thick alveolar ridges, ambiguous genitalia, webbed neck, highly arched palate, clubfeet, fused fontanelles, inclusion cysts of the tongue, four-finger creases, digital anomalies, apparently low-set ears, widely spaced nipples, and dislocated thighs and forearms. The internal findings include oligopapillary renal hypoplasia, severe congenital heart defect, cerebellar hypoplasia, pulmonary hypoplasia, hypoplastic larynx, and hypoplastic gallbladder.

Etiologic heterogeneity of fetal hydrocephalus diagnosed by ultrasound.

Increased use of fetal ultrasound imaging by obstetricians has led to an increased rate of in utero detection of fetal malformations. Eight patients referred for level II sonography for confirmation of suspected fetal hydrocephalus were found to have affected fetuses. Examination of the resulting fetuses and infants revealed remarkable etiologic heterogeneity for the hydrocephalus.

A genetic analysis of targeted growth in mice.

Effects of normal growth regulation on components of phenotypic variance and covariance of body weight were examined in a cross-fostering study of growth between 2 and 10 wk of age in ICR randombred mice. Different early growth rates caused genetic, postnatal maternal and residual environmental variances to increase, but these variances were subsequently reduced by negative autocorrelation between early and later growth. Postnatal maternal variance continued to increase for about 1 wk after weaning but then decreased substantially.

Differential diagnosis and clinical approach to the patient with acute chest pain.

Chest pain syndromes are diverse and often enigmatic. Most chest pain is of a benign nature and poses no serious threat to life. However, when presented with the patient complaining of acute chest pain, the clinician must make decisions concerning management of potentially lethal disease while considering the emotional and financial implications of overtreatment of less serious disorders.

Fatal acute splenic sequestration at 4 months of age.

A patient with homozygous sickle cell anemia is the youngest known to have died from acute splenic sequestration crisis. A cord blood screening program might have prevented this infant's death.

Trophoblastic cell sensitivity to 8-day chemotherapy in nonmetastatic gestational trophoblastic neoplasia.

Serial radioimmunoassay determinations of serum beta hCG and methotrexate were compared in two patients with nonmetastatic gestational trophoblastic neoplasia (NMGTN) treated with Goldstein's modification of Bagshawe's intermediate-dose methotrexate-citrovorum factor rescue-treatment program. Pretreatment beta hCG levels (mIU/ml) ranged within the outer limits of the 10(3) log level. Following intravenous methotrexate, sharp serum peaks between 10(-6) and 10(-5) M were observed.

Bismuth bronze from machu picchu, peru.

The decorative bronze handle of a tumi excavated at the Inca city of Machu Picchu, Peru, contains 18 percent bismuth and appears to be the first known example of the use of bismuth with tin to make bronze. The alloy is not embrittled by the bismuth because the bismuth-rich constituent does not penetrate the grain boundaries of the matrix phase. The use of bismuth facilitates the duplex casting process by which the tumi was made and forms an alloy of unusual color.

Determination of the Thermal Death Time of Vibrio cholerae in Blue Crabs ( Callinectes sapidus ).

The D-values of Vibrio cholerae were determined in peptone water and in crab meat homogenate. In peptone water, the D-values in minutes were 1.70 at 49°C, 1.

Hypobaric hypoxia-induced intrauterine growth retardation.

2-week-pregnant New Zealand White rabbits were placed into a hypobaric chamber and maintained at 3,657 m (12,000 ft). Upon delivery (31 +/- 1 days) or at cesarean section (on day 30), all newborns were sacrificed and organ weights were compared to those of control pups maintained at 128 m (420 ft). Significant findings in the hypobaric animals included a diminished birth weight, placental weight, and liver weight per body weight.

Spontaneous occurrence of localized pulmonary interstitial and endolymphatic emphysema in infancy.

Two infants are described with persistent pulmonary interstitial and endolymphatic emphysema of the localized, bullous type, occurring apparently spontaneously. Roentgenographically, the process simulated congenital lobar emphysema in the first patient and bilateral post-infectious pneumatoceles in the second. The histologic features of the lesion were identical to those seen in localized pulmonary interstitial emphysema caused by positive pressure ventilation in newborns with hyaline membrane disease.