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Biomedical subjects

J Bernstein

Publications and source records attributed to J Bernstein.

At least 163 records · Page 9Linked to original sources
PubMed1988

Mother-daughter in vitro fertilization triplet surrogate pregnancy.

A successful triplet pregnancy has been established in a surrogate gestational mother following the transfer of five embryos fertilized in vitro. The oocytes were donated by her biological daughter, and the sperm obtained from the daughter's husband. The daughter's infertility followed a total abdominal hysterectomy performed for a postpartum hemorrhage as a result of a placenta accreta. Synchronization of both their menstrual cycles was obtained using oral contraceptive suppression for 2 months, followed by stimulation of both the surrogate gestational mother and her daughter such that embryo transfer would occur at least 48 hr after the surrogate gestational mother's own ovulation. This case raises a number of medical, social, psychological, and ethical issues.

Adult↗
PubMed1988

Ribavirin aerosol in the elderly.

Ribavirin aerosol is effective in treating respiratory syncytial virus and influenzal infections in children and young adults. It has not been studied in elderly patients. We evaluated the safety of ribavirin aerosol in eight elderly volunteers at high risk for influenza. Their mean age was 64 years; seven subjects had chronic obstructive pulmonary disease (COPD). Subjects received ribavirin aerosol for two or six hours with six hours between treatments. Regimens were continued for 96 hours. The drug was well tolerated. Subjects receiving ribavirin for 2 hours demonstrated a significant decrease in their forced expiratory volume in one second, peak expiratory flow rate and flow over the middle 50 percent of the forced vital capacity. These changes were small and the subjects' symptoms remained stable. Subjects receiving ribavirin for 6 hours had no significant changes in pulmonary function. We conclude that ribavirin aerosol is safe in elderly patients with underlying COPD.

Aerosols↗
PubMed1988

Congenital hepatic fibrosis: evolving morphology.

A clinicopathologic study of congenital hepatic fibrosis in 21 patients confirms a strong association with autosomal recessive renal polycystic disease. The liver specimens were subclassified into two groups according to the severity of fibrosis, showing typical hepatic abnormalities in young infants (mean age 0.3 years) and increased hepatic fibrosis in older patients (mean age 19.6 yr) (p less than 0.02). Apparent progression to perilobular fibrosis with parenchymal nodularity occasionally resembled cirrhosis when the nodules had a regenerative appearance because of rounded contours and inapparent central veins. Progression of fibrosis was observed in second biopsy specimens from 2 cases, but not in that of a 3rd, suggesting that factors other than the heritable disorder itself may be responsible for evolving morphology. Identifiable factors that may have contributed to increased fibrosis included localized intrahepatic biliary obstruction and biliary sepsis with suppuration. A factor possibly contributing to the pathogenesis of biliary sepsis was intrahepatic biliary ectasia, i.e., Caroli's disease, which appears to be one morphologic expression of CHF. This study shows that the hepatic abnormality evolves over time and that it may be altered by secondary complications.

Bile Duct Diseases↗
PubMed1988

Chronic occupational exposure to asbestos: more than medical effects?

One hundred and twenty-nine workers chronically exposed to asbestos were interviewed regarding their perceived health status and concerns, their health behaviors, particularly their smoking behavior, and their psychologic well-being. In contrast to a non-exposed comparison group of postal workers, asbestos workers exhibited significantly elevated levels of somatic concern (P less than .03), and significantly lower levels of mental health functioning only when experiencing high levels of stress (P less than .01). Despite feeling significantly more susceptible to developing cancer (P less than .0001), 34% of asbestos workers were cigarette smokers (compared to 32% of the postal group) and long-term mask usage was minimal. Asbestos workers' increased sensitivity to stress and changes in health status along with the lack of adaptation of health-promotive behaviors indicate the need for interventions to attend to the psychologic effects of increased risk status.

Asbestos↗
PubMed1988

Renal glomerular and tubular abnormalities in glycogen storage disease type I.

Three children had renal histopathologic findings indicative of glycogen storage disease type I. Glomerular basement membrane (GBM) alterations were present in the three patients, particularly so in the two patients with proteinuria. Thickening, lamellation, and glycogen deposition were the characteristic alterations in the GBM. Glomerulosclerosis was prominent in one patient. We suggest that the GBM alteration is related to the glomerular sclerosis and that both are related to metabolic derangements of glycogen storage disease type I.

Adolescent↗
PubMed1987

Impact of maternal drug dependency on birth weight and head circumference of offspring.

The impact of maternal opiate dependency on birth weight and head circumference of offspring was studied in 150 mother-infant pairs and 150 controls. Mean birth weight and head circumference of drug-dependent offspring were reduced symmetrically compared with drug-free controls of similar socioeconomic status. Although mean gestational ages and rates of prematurity were similar, intrauterine growth retardation occurred in 20% of passively addicted infants vs 4% of controls. A longer period of methadone maintenance was associated with longer gestation and increased birth weight, while higher methadone dosages were associated with higher birth weights and larger head circumferences. Smoking was associated with reduced birth weight by an average of 160 g per pack of cigarettes smoked per day; longer duration of smoking was associated with reductions in both birth weight and head circumference. These data suggest therapeutic strategies to improve these fetal growth characteristics and perhaps improve neurobehavioral outcome of drug-dependent newborns.

Adult↗
PubMed1987

Renal-hepatic-pancreatic dysplasia: a syndrome reconsidered.

Five infants, three dying neonatally and two later in the first year of life, had renal, hepatic, and pancreatic dysplasia, a combination of abnormalities first described by Ivemark et al [1959]. The renal malformation consisted of cystic dysplasia, with abnormally differentiated ducts, deficient nephron differentiation, and glomerular cysts. The hepatic abnormality consisted of enlarged portal areas containing numerous elongated biliary "profiles," with a tendency to perilobular fibrosis. Serial liver biopsies in one child with cholestasis from birth showed a progression from bile duct paucity at 1 1/2 wk to typical biliary "dysgenesis" at 7 mo. Four of the five children had intrahepatic ductal dilatation, diagnosed ante mortem in the two older children as Caroli disease. The pancreatic abnormality consisted of fibrosis and cysts, with a diminution of parenchymal tissue. The clinical and functional reflection of these abnormalities in the two children surviving the newborn period included renal insufficiency, chronic jaundice, and insulin-dependent diabetes mellitus. Similar renal, hepatic, and pancreatic abnormalities occur in other syndromes, including trisomy 9, Meckel syndrome, Jeune, Saldino-Noonan, and Elejalde types of chondrodysplasia, and glutaric aciduria II. After exclusion of identifiable syndromes, the remaining cases of renal-hepatic-pancreatic dysplasia do not necessarily constitute a homogeneous group.

Abnormalities, Multiple↗
PubMed1987

Renal multicystic dysplasia: an occasional manifestation of the hereditary renal adysplasia syndrome.

Renal multicystic dysplasia is commonly regarded as a sporadic anomaly, although several studies of renal agenesis have shown the occasional occurrence of multicystic dysplasia in relatives of propositi with bilateral agenesis. We report the occurrence of unilateral multicystic dysplasia in an infant whose mother and maternal aunt had unilateral renal agenesis. The aunt's daughter, a first cousin of the proposita, had unilateral megaureter and hydronephrosis secondary to obstruction at the ureteropelvic junction. These observations suggest that nonsyndromal multicystic renal dysplasia can occur as part of the spectrum of hereditary renal adysplasia, with what appears to be autosomal dominant inheritance, and that the primary abnormality may reside in the ureter, with secondary renal maldevelopment.

Female↗
PubMed1987

Pathology of renal and hepatic anomalies in Meckel syndrome.

We describe 9 cases of Meckel syndrome (dysencephalia splanchnocystica), ranging in gestational age from 17 to 39 weeks. Five were liveborn, and the longest survival was 30 hours. Six of the 9 had all 3 elements of the classically defined triad, namely occipital encephalocele, renal cystic dysplasia, and postaxial polydactyly. The remaining 3 cases had only 2 elements of the triad. The renal abnormalities in the 9 cases resembled one another closely, varying only slightly in severity. They consisted of renal cystic dysplasia with different degrees of nephronic differentiation. The 8 available livers all contained intrahepatic bile duct anomalies. Seven of them had the classic "ductal plate malformation," as defined by Jorgensen [1977]; the remaining case had a less severe form of this malformation. The livers of the Meckel syndrome fetuses were compared to those of age-matched control fetuses, and it was concluded that the hepatic abnormality represents an arrested development of the intrahepatic biliary system at the stage of biliary cylinders with varying degrees of reactive bile duct proliferation, bile duct dilatation, portal fibrosis, and portal fibrous vascular obliteration superimposed on the arrested pattern. The case showing the least severe hepatic lesion also had the least severe renal lesion. The anomalies that were present are listed. The relationship of the different lesions to each other is briefly discussed. It was concluded that the hepatic lesion appears to be a consistent manifestation in Meckel syndrome and is of diagnostic value as the renal lesion has previously been shown to be.

Abnormalities, Multiple↗
PubMed1987

Human cystic kidney diseases: epithelial hyperplasia in the pathogenesis of cysts and tumors.

Several examples of human renal cystic disease are associated with tubular epithelial hyperplasia. Micropapillary hyperplasia occurs in autosomal dominant polycystic kidney disease, in localized cystic disease, and in acquired cystic disease; neoplastic or severely dysplastic epithelial hyperplasia occurs in von Hippel-Lindau disease; a histopathologically distinctive epithelial hyperplasia occurs in tuberous sclerosis. In all of these conditions the epithelial hyperplasia appears to be responsible for cyst formation by causing tubular or ductal luminal obstruction, and in all of these conditions, save localized cystic disease (a rare condition with very few reported cases), epithelial hyperplasia imposes an increased risk of malignancy. The risk seems to be highest in patients under treatment with long-term hemodialysis for end-stage kidney disease. Some of these diseases may share common features, but it appears likely that the histopathological differences reflect different features converging on a common result.

Epithelium↗
PubMed1987

Increased glucose increases glomerular basement membrane in metanephric culture.

A model of in vitro renal development has been used to examine the effect of glucose on the glomerular basement membrane. Glomerular differentiation in this system progresses to an arborizing tuft of podocytes overlying well-formed basal lamina. The proportional amount of lamina densa and dense fibrillary matrix was increased in blastemas grown in an increased amount of glucose during the last 4 of 7 days in culture. These observations indicate that glucose itself can stimulate an accumulation of basement membrane, an excess of which is characteristic of diabetic microangiopathy.

Animals↗
PubMed1987

Emotional factors and the in vitro fertilization and embryo transfer process.

In vitro fertilization is nearly always a treatment of a last resort. This fact, along with the treatment's multiplicity of procedures and intensity, place unique emotional demands on patients. The goal of this research was to describe both the acknowledged emotional state of patients at the time they began the in vitro fertilization and embryo transfer (IVF-ET) process and the emotional experience of the actual procedures themselves. The findings can be used to develop strategies for providing emotional support. Data were collected from self-administered questionnaires returned by 94 IVF-ET patients in three Houston programs during a 6-month period. At the time of the IVF procedure, 77% of the population reported that infertility was still a painful concern, not something with which they had learned to live. The loss of control, seen by most patients as infertility's most stressful dimension, left them vulnerable to the intense stresses of in vitro fertilization, less able to handle its multiple demands. Thus for many, the IVF-ET procedures were like an emotional roller coaster on which they experienced a wide range of feelings during a brief period of time. Not surprisingly, emotional strain was a major consideration influencing the decision whether or not to repeat IVF. Patients indicated specific services which the staff could provide to reduce the stress of the procedures.

Embryo Transfer↗
PubMed1987

The glomerular basement membrane abnormality in Alport's syndrome.

The characteristic electron microscopic abnormality in Alport's syndrome is thickening of the glomerular basement membrane with splitting and lamellation of the lamina densa. It is commonly accompanied by severe attenuation of the basement membrane and sometimes by disruption of capillary walls. The ultrastructural abnormality is secondary to a more basic defect in the chemical structure of the glomerular basement membrane. Clues to chemical abnormalities have come from immunochemical studies, which suggest a lack of or alteration in that part of the noncollagenous portion of type 4 collagen related to Goodpasture antigen. Variability in the results of immunochemical studies suggest biochemical heterogeneity that still needs to be correlated with the known evidence of genetic heterogeneity in Alport's syndrome.

Autoantibodies↗
PubMed1987

Perceptions of medical school stressors: their relationship to age, year of study and trait anxiety.

It has been noted that medical school is a stressful social environment. This study proposes to determine domains of medical school stressors and to investigate to what extent the perceived stressfulness of these domains is explained by age, year of study, marital status, sex, and trait anxiety. Data were obtained by self-administered questionnaires filled out by 131 students in four classes of a six-year medical school. Four clusters of stressors were revealed by factor analysis: "off-time death," "incurable condition," "patient contact," and "medical practice demands." Marital status, sex, and year of study did not correlate with any of these stressor domains. Age was positively correlated with the perception of off-time death as stressful and negatively correlated with patient contact. Trait anxiety was directly correlated with the perceived stressfulness of patient contact and medical practice demands. No independent variable explained differences in perception of all four domains. It is suggested that there are some experiences, such as facing off-time death, that are so painful that repeated exposure to them augments, rather than decreases, the perception of their stressfulness.

Adult↗
PubMed1987

Epithelial hyperplasia in human polycystic kidney diseases. Its role in pathogenesis and risk of neoplasia.

The importance of tubular epithelial hyperplasia in polycystic kidney diseases has become apparent during the last decade. Micropapillary hyperplasia occurs in autosomal dominant polycystic kidney disease, in localized cystic disease, and in acquired cystic disease. Neoplastic or severely dysplastic epithelial hyperplasia occurs in von Hippel-Lindau disease. A histopathologically distinctive epithelial hyperplasia occurs in tuberous sclerosis. In each of these conditions, epithelial hyperplasia may be related to cyst formation and may also impose an increased risk of malignancy--a risk that seems to be highest in patients under treatment with long-term hemodialysis for end-stage kidney disease. Although hyperplasia in some of these diseases may share a common pathway of development, it is more probable that the histopathologic differences reflect different pathogenetic pathways that converge on a common endpoint.

Epithelium↗