Biomedical subjects
Publications and source records attributed to P Merlob.
Two infants with hemolytic disease of the newborn due to Kell incompatibility are described. The peripheral blood smears revealed many spherocytes. This important hematologic sign in a neonate suggests not only ABO incompatibility, hereditary spherocytosis, or G6PD deficiency, but also the possibility of Kell incompatibility.
The case history of a baby with neonatal pemphigus vulgaris is presented. This is the 13th case of pemphigus vulgaris during pregnancy reported in the literature. The correlations between the clinical, histologic, and immunofluorescent findings are discussed and a review of all previously reported cases is presented.
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The incidence of isolated, nonsyndromatic craniosynostosis in a newborn population was found to be 0.6 per 1,000 live births. The distribution by anatomic types was metopic suture, 50%; sagittal suture, 28%; coronal suture, 16.5%; and lambdoid suture, 5.5%. We found a higher incidence of trigonocephaly than has been reported previously.
During a 2-month period all male newborns were examined prospectively to determine the normal characteristics of the penis. Of the 274 neonates examined 3 were excluded because of hypospadias with chordee. The spontaneous direction of the shaft was in the midline in 76.8 per cent, to the left side in 15.5 per cent and to the right side in 7.7 per cent. Partial absence of the prepuce was observed in 10 per cent of the patients, while an unretractable foreskin was seen in 23.2 per cent. The mean meatal aperture was 2.6 plus or minus 0.8 mm. Deviation of the median raphe was present in 10 per cent of the newborns and was associated with deviation of the meatus in 2.2 per cent. Mild torsion of the penis was noted in 1.5 per cent, while isolated torsion of 90 degrees was seen in 0.7 per cent of the patients. Early diagnosis and followup of these newborns might be helpful in determining further treatment.
A familial nevus flammeus of the forehead associated with Unna's nevus is described. The pedigree suggests an autosomal dominant mode of inheritance.
We prospectively studied 535 newborn infants who had been monitored during labour with scalp electrodes. Daily examination of scalp changes showed frequent transient mild lacerations, while severe complications were rare: seven (1.3%) had scalp ulceration and one (0.2%) developed scalp abscess.
A rare association of triphalangeal thumbs and duplication of the big toes is described. The family pedigree confirms autosomal dominant inheritance with marked penetrance. A systematic approach to the diagnosis of this anomaly is presented. The distinction between the two types of triphalangeal thumb, true (opposable) and finger-like (non-opposable), is emphasised.
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Diarrhea is often seen during phototherapy in jaundiced infants. Lactose malabsorption and reduced gut transit time (GTT) are some of the proposed explanations. However, the etiology of the diarrhea is still controversial. We investigated GTT and lactose absorption during phototherapy using the H2 breath test. Breath H2 was measured every 10 min for 150 min after feeding of jaundiced infants with and without phototherapy, and in controls. There were 12 newborns in each group. The time of increase of H2 excretion over 10 ppm was taken as the transit time. Lactose malabsorption was estimated by integrating the area under the excess H2 curve. No difference was found in GTT, lactose absorption, peak breath H2 and the time of the peak between phototherapy-treated infants and jaundiced and nonjaundiced infants. The results did not support the presence of lactose malabsorption during phototherapy and the decreased total GTT reported in the literature was not due to shortened small intestinal transit time.
Spleen tip size was determined in 470 full-term appropriate-for-gestational-age infants within the first 24 hours of life. The spleen was palpable in 17.9 percent of them. It was palpated less than 1 cm below the costal margin in 11.7 percent, between 1 and 2 cm in 3.6 percent, and 2 cm or more in 2.6 percent. No cause for spleen enlargement was found except in two infants with ABO hemolytic disease.
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To define standards for lower limb measurements in the newborn, 198 full-term and preterm infants (range, 27 to 41 gestational weeks) were examined. The gestational age was determined chronologically and clinically, and the total length of the lower limb and the leg and foot lengths were measured by two observers with the use of standard measurement techniques. Normal values were determined by plotting the mean +/- 2 SDs for each gestational week v gestational age.
The distance between the right costal margin and the liver edge at the mid-clavicular line was measured within the first 24 hours of life and before discharge (between 72 and 96 hours of life) in 367 healthy, full-term infants. No significant difference was found between the measurements during the first day of life and at discharge, or in the different groups of gestational age (range, 37 to 41 weeks). The mean +/- SD distance of the liver edge below the costal margin was 3.0 +/- 0.7 cm, with a normal range from 1.6 to 4.4 cm. According to our results, we recommend investigation for hepatomegaly when the liver is palpated more than 4.4 cm below the costal margin.
Three cases of circumscribed congenital smooth muscle hamartoma (CSMH) in the neonate are described, including typical characteristics and differential diagnosis. This condition is a clearly defined, distinct entity, separate from similar lesions that are considered in the differential diagnosis. It is possibly more common than is evident from the literature.