Biomedical subjects
Publications and source records attributed to D Ben-Amitai.
Postoperative alopecia is an uncommon complication of surgery and is reported mainly in adults who undergo prolonged anesthesia. The disorder occurred in three infants after cardiac surgery. It is easily prevented by frequently changing the position of the head during surgery and the recovery period.
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We report on a 4-month-old girl with congenital hypodipsic hypernatremia resulting from decreased sensitivity of the hypothalamic osmoreceptors with increased tonicity in association with hyperlipemia and cleft lip and cleft palate. We postulate that the link among these various derangements is hypothalamic dysfunction.
The present study establishes anthropometric standards for newborn infants, born between 26-41 weeks of gestational age. The measurement of 10 standard anthropometric traits was made within the first 72 hours of life in 224 preterm (26-36 weeks) and 190 term (37-41 weeks) infants. Multiple regression analysis was carried out for each of these anthropometric traits (dependent variables) on gestational age, birth weight and sex (independent variables). It was found that the prediction of several traits, namely, body length, body mass index, body surface area, interocular diameter, ear length and palm length, can be done solely via the information on birth weight; cephalic index is better predicted on the basis of gestational age, while for the prediction of such cranial traits as the circumference, length and breadth of head, both birth weight and gestational age are desirable.
Fluctuating asymmetry (FA) of 8 morphometric traits was studied in 113 preterm infants (26-36 wk of gestation), 103 term infants (37-41 wk), and their respective parents. With 3 different measures of FA, the highest values were obtained from extremely preterm infants (26-29 wk), and the lowest from the group of term infants. The estimates of FA values among parents, particularly mothers, showed a similar, albeit less pronounced, trend. Multiple regression analysis of individual mean FA values, calculated in infants for the 8 studied bilateral traits, documented a significant inverse correlation with gestational age and with the health status of the infants and their mothers, as well as a positive correlation with the mothers' mean FA values.
The main purpose of the present study was to evaluate the effects of factors associated with spontaneous pre-term births so that the high risk woman could be identified before or at early stages of pregnancy. For this purpose, we have compared the measurements of 21 anthropometric traits and mean fluctuating asymmetry over 8 bilateral anthropometric traits, as well as age, occupation, education, previous obstetric history, complications during pregnancy, medicines received during and after pregnancy and some others in women who delivered babies of short (26-36 wk, n = 113 ind.) and normal gestational age (n = 103). Diseases and mean fluctuating asymmetry of eight morphological traits in the newborn infants themselves were also studied. Both univariate and multivariate analyses were carried out and these were in agreement, showing a highly significant increase in the morbidity rate (especially of respiratory diseases) among pre-term infants and in complications during pregnancy in their mothers. Among other variables associated with the current pre-term birth were previous spontaneous pre-term births, suggesting their special risk value. The estimate of the sib correlation in gestational age on the liability scale was about 0.63. Spearman and Pearson correlations in gestational age for siblings were 0.34 and 0.31, respectively.
Association of prolonged neonatal cholestasis with hypoglycemia, small penis and congenital hypothalamo-hypopituitary derangement is presented. The infant's jaundice was unresponsive to thyroxine replacement therapy but resolved rapidly with hydrocortisone therapy. The time relationships between persistent jaundice and thyroxine and cortisol deficiencies are discussed.
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The case of a patient with Kaposi's sarcoma and paraproteinemia is described and the relevant literature is reviewed. It is suggested that Kaposi's sarcoma is part of the spectrum of the lymphoproliferative diseases, and that both the marked angiogenesis and the synthesis of paraproteins in this disease are different expressions of the same proliferative process.
Palmoplantar eccrine hidradenitis (PEH) is characterized by painful erythematous papules and nodules of abrupt onset on the soles of young individuals. The histologic hallmark is a predominant neutrophilic infiltrate surrounding the eccrine gland apparatus. A total of 28 cases have been published since 1988, with a broad variation in the age of patients, symptomatology, associated diseases, clinical course, and histologic features. These different histologic pictures have resulted in several names for this entity, including idiopathic plantar hidradenitis, neutrophilic eccrine hidradenitis, recurrent palmoplantar hidradenitis in children, and idiopathic palmoplantar hidradenitis. We present three cases of this disorder and review the literature on the varying symptomatology, clinical course, and histology that have led to the myriad of names for this disease.
Neonates with midline lumbar, thoracic, or occipital cutaneous lesions should be suspected of having spinal dysraphism and should undergo an imaging study. The aim of the present study was to evaluate whether sacral nevus flammeus simplex (SNFS) in neonates is associated with spinal dysraphism. During a period of 6 months, we prospectively examined all neonates in our center for the presence of SNFS. Those affected were evaluated for associated anomalies by physical and neurologic examination and ultrasound imaging of the lumbosacral area. Twenty-eight of 3623 neonates (0.77%) were diagnosed as having SNFS. In 64% of the SNFS patients, nevus flammeus simplex (NFS) was noted as well. Physical and neurologic examinations were unremarkable. Ultrasound imaging of the lumbosacral area was performed in 25 patients (89%) and revealed diastematomyelia in one. The results show that SNFS may constitute the only cutaneous sign of spinal dysraphism. In conclusion, we recommend that neonates with SNFS be investigated for occult spinal dysraphism.
Long eyelashes may be congenital, acquired in association with certain systemic diseases, or drug induced. In the past, long eyelashes were considered an external sign found in children with allergic diseases. However, this claim has never been examined in a controlled study. We compared the eyelash lengths of allergic children and adolescents with perennial allergic rhinitis, with or without bronchial asthma and atopic dermatitis (n = 60) to those of age- and sex-matched nonallergic controls (n = 80). The eyelashes of the allergic patients were found to be significantly longer than those of the controls: 9.43 +/- 1.39 mm versus 8.45 +/- 1.30 mm (p < 0.001). Eyelash length did not differ between patients with allergic rhinitis only (n = 31; 9.65 +/- 1.43 mm) and patients with allergic rhinitis and other allergic diseases (n = 29; 9.19 +/- 1.31 mm) (p = 0.196). These results indicate that children and adolescents with allergic diseases have longer eyelashes compared to nonatopic controls and that long eyelashes may be a part of the phenotype of the allergic patient.
Monilethrix is a rare autosomal dominant disease characterized by hair fragility and follicular hyperkeratosis. Mutations in the human basic hair keratins hHb1 and hHb6 have recently been reported in this disease. Twelve families and sporadic cases were clinically diagnosed with monilethrix and were available for the study. The gene segment encoding the helix termination motif region of keratin hHb6 was PCR amplified and sequenced. Mutations were recognized in 6 families. Four families had the previously described mutations, Glu413Lys and Glu413Asp. In 2 unrelated families, a novel mutation, Glu402Lys, was identified. No clear association was found between the severity of the phenotype and the mutation carried. Furthermore, heterozygous members of the same family had variable degrees of hair and skin involvement. Homozygous patients identified in one large consanguineous family were more severely affected. Other genetic or environmental factors may also play a role in monilethrix.