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Biomedical subjects

R M Ehrlich

Publications and source records attributed to R M Ehrlich.

At least 19 recordsLinked to original sources
PubMed1992

Brain tumors in children and adolescents--III. Effects of radiation and hormone status on intelligence and on working, associative and serial-order memory.

The effects on intelligence and memory of two post-surgical conditions (radiation treatment, hormone deficiency and supplementation) were explored in 46 children and adolescents with tumors in a variety of brain sites. Verbal intelligence, but not non-verbal intelligence, varied positively with age at radiation treatment. Memory for word meanings was unrelated to either radiation history or to hormone status. Severe deficits in serial position memory occurred with impaired hormone function and an older age at tumor onset. Severe deficits in working memory were associated with a history of radiation and a principal tumor site that involved thalamic/epithalamic brain regions. Radiation treatment and hormone status affect later cognitive function in children and adolescents with brain tumors. Although the greater vulnerability of the verbal intelligence of the younger radiated child and the serial order memory of the child with later tumor onset and hormone disturbances remain to be explained, and although the form of the relationship between radiation and tumor site is not fully understood, the data highlight the need to consider the cognitive consequences of pediatric brain tumors according to a set of markers that include maturational rate, hormone status, radiation history, and principal site of the tumor.

Adolescent↗
PubMed1992

Neurodevelopment in infants and preschool children with congenital hypothyroidism: etiological and treatment factors affecting outcome.

Studied global intelligence and specific abilities of children from 1 to 5 years old, 108 with congenital hypothyroidism (CH) diagnosed by newborn screening and 71 sibling controls. Intelligence levels of the CH group were within the normal range but differed significantly from controls at age 5. CH demonstrated a language deficit at age 3, which diminished with age, as well as significantly poorer visuospatial and verbal skills at age 5. Children with absent thyroid glands or evidence of hypothyroidism in utero were outperformed by other CH children in most ability areas from 1 year on. Different components of ability were correlated with specific factors reflecting timing or duration of thyroid hormone deficiency, suggesting unique critical periods of thyroid hormone sensitivity for different specific cognitive abilities and their neural substrates. It is concluded that even though screened CH children are markedly improved by neonatal diagnosis, they are still at risk for subtle irreversible deficits, which depend on factors such as type, age at onset, and duration of disease.

Age Factors↗
PubMed1991

Current status of pancreatectomy for persistent idiopathic neonatal hypoglycemia due to islet cell dysplasia.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histopathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases, 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

Female↗
PubMed1991

A case report of Drash syndrome in a 46,XX female.

Progressive renal insufficiency, male pseudohermaphrotidism, and Wilms tumor are known as the triad of Drash syndrome. The renal involvement has been reported in association with Wilms tumor with or without pseudohermaphroditism. The nephropathy progresses rapidly, is unresponsive to steroids, and eventually requires dialysis. The case we report is a 46,XX phenotypic female child who had diffuse mesangial sclerosis (DMS) and developed Wilms tumor 3 years after initiating dialysis for end-stage renal disease (ESRD). Any child presenting with DMS should be considered at risk for development of Wilms tumor and should be investigated for the presence of XY gonadal dysgenesis. Once ESRD develops in such a child, prophylactic nephrectomy and possibly gonadectomy should be undertaken.

Child, Preschool↗
PubMed1990

Growth in children with congenital hypothyroidism detected by neonatal screening.

A prospective analysis of physical growth in 56 children with congenital hypothyroidism compared the children's height, weight, and head circumference with population percentiles. Two measures of skeletal maturation and predictions of adult height were also compared with population standards. The mean percentiles for the children's height and head circumference were higher than population means. Although mean bone age scores were slightly higher, predictions of adult height did not differ significantly from midparental height (arithmetic mean of sum of parental heights) and population means. The duration of intrauterine hypothyroidism as measured by gestational bone age and the duration of postnatal hypothyroidism were inversely correlated with heights attained up to 9 years. This association suggests a possible long-term influence of early hypothyroidism on growth. In children with congenital hypothyroidism, maintenance of serum thyroxine levels in the upper half of the normal range results in normal growth patterns.

Age Determination by Skeleton↗
PubMed1990

Technical factors contributing to successful kidney transplantation in small children.

Cadaveric renal transplantation in small children has not been as successful as either cadaver transplantation in older children, or adults, or living-related transplantation in small children. We report the results of cadaver renal transplantation in 17 children 6 years or younger with only 1 graft lost within 1 year after transplantation. The important technical factors that have enabled these improved results include careful intraoperative fluid management, use of adult kidneys with arterial anastomoses to the common iliac artery or aorta, and sequential immune suppression.

Child↗
PubMed1990

The necessity of contralateral surgical exploration in Wilms tumor with modern noninvasive imaging technique: a reassessment.

Surgical exploration of the contralateral kidney in unilateral Wilms tumor has been the standard of surgical practice. During the last decade advances in noninvasive imaging techniques (ultrasound, computerized tomography and magnetic resonance imaging) have led to more accurate definition of intrarenal pathological conditions. The intuitive question presently is whether contralateral exploration remains mandatory in Wilms tumor patients. We reviewed the records of 52 consecutive children at our institutions who underwent radiological and operative staging of the Wilms tumor from 1979 to 1988. All 48 evaluable patients underwent either preoperative computerized tomography, ultrasound and/or magnetic resonance imaging. Five bilateral Wilms tumors were diagnosed preoperatively and confirmed surgically, whereas extensive operative exploration of the contralateral kidney in the other 43 cases of radiologically diagnosed unilateral disease failed to reveal any contralateral abnormality. Thus, in all patients preoperative radiological investigation was accurate to stage the disease regarding bilaterality. With modern imaging techniques and effective chemotherapy, extensive contralateral renal exploration may no longer be mandatory to manage Wilms tumor.

Child, Preschool↗
PubMed1990

Long-term effects of prepubertal testicular vessel ligation on testicular function in the rat.

To determine the effects of unilateral testicular vein and artery ligation in the immature rat on the function and final location of the testis at adulthood, 10-day-old male rats underwent either a sham operation or unilateral ligation of these vessels of the still undescended testis. Testicular location, blood flow, size and histology as well as ventral prostate weights were measured 50 days later at adulthood. At age 60 days, it was determined that all testes were descended into the scrotum, and there were no differences in testis and ventral prostate weights, intratesticular sperm counts and mean seminiferous tubular area between the control and sham operated animals. However, there was an 18% reduction in testicular blood flow (ml. per 100 gm. per minute +/- standard error of mean) in the operated animals when compared to the sham (20.43 +/- 1.10 versus 16.69 +/- 0.74, p less than 0.02). These data indicate that although there is a slight but significant reduction in testicular blood flow at adulthood when the testicular artery and vein are ligated early in life, this diminution is not sufficient to alter the ultimate location, testicular weight and spermatogenic function of the testis. This would suggest that after ligation of the main testicular vessels to the immature testis, the collateral blood supply is able to compensate with time to allow normal growth and development of the testis. These experimental observations provide additional support for the 2-staged approach to the high undescended testis whereby the testicular vessels are initially ligated and a subsequent procedure is performed to place the undescended testis into the scrotum.

Animals↗
PubMed1990

Intellectual characteristics of diabetic children at diagnosis and one year later.

Examined neurocognitive functions in 63 newly diagnosed pediatric patients with insulin-dependent diabetes mellitus (DM) at onset of illness (T0) and 1 year postdiagnosis (T1). Siblings (S) serving as controls were assessed at T0 only. Subjects were given age-appropriate tests of verbal and visuospatial abilities. In addition, DM were interviewed regularly during diabetes clinic to determine current diabetic control and different intervening glycemic-related events. Results revealed no differences between DM and S at T0, nor any specific impairment in DM predating illness. Also, DM did not demonstrate any acquired impairment after 1 year of illness. Children with early onset DM (less than 5 years) scored lower in spatial ability at T0 and T1 than children with later onset DM, who scored lower in verbal ability. Episodes of asymptomatic and mild chronic hypoglycemia correlated positively, not negatively, with improved outcome over time. There were no adverse effects of severe hypoglycemia. Ketonuria and hospitalizations were associated with lower performance IQs 1 year after onset, as was diabetic ketoacidosis at onset. Results are discussed in terms of critical periods of sensitivity of different brain regions to the effects of diabetes and the need for longer follow-up of these children.

Blood Glucose↗
PubMed1989

Spurious impotence after hypospadias repair.

A fifteen-year-old who had undergone multiple hypospadias repairs alleged erectile dysfunction as a result of his surgery. A new method of nocturnal penile tumescence and rigidity monitoring supplied objective evidence to refute this allegation. Attention is drawn to the need for and availability of written documentation when diagnosing the presence or absence of erectile ability.

Adolescent↗
PubMed1989

Effect of thyroid hormone level on temperament in infants with congenital hypothyroidism detected by screening of neonates.

To determine the effect of congenital hypothyroidism and its treatment on infant behavior, we assessed temperament in 50 six-month-old infants with congenital hypothyroidism detected by means of screening of neonates. Intelligence and temperament were also evaluated at 12, 18, 24, and 36 months. More of these children were classified as "difficult" than children in the nonhypothyroid standardization sample. Temperamental difficulty was associated with increased nervous system sensitivity, reflecting more intense responses and a lower threshold of response to external stimulation. Greater temperamental difficulty was found to persist until at least age 2 years of age and to be associated with higher circulating triiodothyronine and thyroxine levels between 1 and 3 months of age. Our results suggest that behavioral features should be considered, as well as circulating hormone levels, in determining the proper dose of thyroid hormone replacement in infants with congenital hypothyroidism.

Congenital Hypothyroidism↗
PubMed1989

Recovery of testicular blood flow following ligation of testicular vessels.

To determine whether initial ligation of the testicular vessels of the high undescended testis followed by a delayed secondary orchiopexy is a viable alternative to the classical Fowler-Stephens procedure, a series of preliminary experiments were conducted in the rat in which testicular blood flow was measured by the 133xenon washout technique before, and 1 hour and 30 days after ligation of the vessels. In addition, testicular histology, and testis and sex-accessory tissue weights were measured in 6 control, 6 sham operated and 6 testicular vessel ligated rats 54 days after vessel ligation. The data demonstrate that ligation and division of the testicular blood vessels produce an 80 per cent decrease in testicular blood flow 1 hour after ligation of the vessels. However, 30 days later testis blood flow returns to the control and pre-treatment value. There were no significant changes in testis or sex-accessory tissue weights 54 days after vessel ligation. Histologically, 4 of the surgically operated testes demonstrated necrosis of less than 25 per cent of the seminiferous tubules while 1 testis demonstrated more than 75 per cent necrosis. The rest of the tubules in all 6 testes demonstrated normal spermatogenesis. From this study we conclude that initial testicular vessel ligation produces an immediate decrease in testicular blood flow but with time the collateral vessels are able to compensate and return the testis blood flow to its normal pre-treatment value. These preliminary observations lend support for the concept that initial ligation of the testicular vessels followed by a delayed secondary orchiopexy in patients with a high undescended testis may be a possible alternative to the classical Fowler-Stephens approach.

Animals↗
PubMed1989

Complications of bladder mucosal graft.

A bladder mucosal graft for urethral reconstruction was performed on 79 patients who had complicated conditions in which local penile or preputial skin was not available. The major and minor complication rates for this procedure were 15.2 and 43 per cent, respectively, the latter representing mucosal glanular protrusion obviated by a technical alteration. Given the difficulty of these cases these complication rates are deemed acceptable in such patients.

Humans↗
PubMed1989

A report of 4 patients with the Drash syndrome and a review of the literature.

Four patients with the Drash syndrome, represented by the triad of male pseudohermaphroditism, progressive renal insufficiency and Wilms tumor are discussed. This syndrome, which some have suggested should be expanded to include patients with nephropathy, Wilms tumor and any abnormality of gonadal differentiation, appears early in life. The first sign usually is genital ambiguity. However, proteinuria, hematuria and hypertension eventually require renal biopsy, which may reveal a variety of glomerular and interstitial changes. The nephropathy progresses rapidly, is unresponsive to steroids and eventually requires dialysis. Wilms tumor may appear as a mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. Young children with gonadal dysgenesis and nephropathy are at high risk for nephroblastoma and gonadal tumors and, therefore, prophylactic nephrectomy and gonadectomy should be considered early in the course of this disease.

Disorders of Sex Development↗
PubMed1988

A technique for ureteral stump ablation.

A total of 5 children underwent successful electrofulguration of a distal ectopic ureteral stump with reflux. Electrofulguration was performed endoscopically in 4 girls and in 1 boy the stump was ablated during an open operation. The procedure, which is simple and safe, obviates the potential nidus for recurrent infection.

Choristoma↗
PubMed1988

Use of fine needle aspiration cytology for the diagnosis of testicular relapse in patients with acute lymphoblastic leukemia.

The testis frequently is the site of relapse in male patients with acute lymphoblastic leukemia. While many patients with testicular involvement by acute lymphoblastic leukemia have enlarged or firm testes, clinical examination alone is insufficient to establish or exclude the diagnosis completely. Open biopsy generally has been used to document the presence of acute lymphoblastic leukemia. However, this procedure requires general anesthesia and hospitalization. We studied 11 patients with a history and/or physical findings suspicious for testicular acute lymphoblastic leukemia relapse to determine the efficacy of fine needle aspiration cytology in the evaluation of the testes for leukemic infiltration. Of the 11 patients fine needle aspiration cytology correctly identified all 5 patients with histologically proved testicular acute lymphoblastic leukemia, it was negative in 5 with no histological evidence of leukemia and it demonstrated rare atypical cells that were not evident on subsequent histological examination in 1. No adverse effects were encountered in this series. Fine needle aspiration cytology appears to be a safe, reproducible alternative to open biopsy in the evaluation of patients for testicular relapse of acute lymphoblastic leukemia.

Adolescent↗