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Biomedical subjects

W G Johnson

Publications and source records attributed to W G Johnson.

At least 55 records · Page 3Linked to original sources
PubMed1994

Binge eating disorder and the proposed DSM-IV criteria: psychometric analysis of the Questionnaire of Eating and Weight Patterns.

Proposed binge eating disorder (BED) diagnostic criteria were investigated to provide necessary psychometric characteristics and explore their utility in assessment. One hundred four subjects (52 self-referred bingers, 52 comparison subjects) completed an initial administration of the Questionnaire of Eating and Weight Patterns (QEWP). The results supported the ability of the two core BED criteria (i.e., episodic overeating, loss of control) to discriminate between clinical and nonclinical binge eaters. Thirty-nine of the self-referred and 40 of the comparison subjects completed a second QEWP administration 3 weeks later. Results indicated that the BED diagnosis was moderately stable over the 3-week interval (kappa = .58, combined sample). Using self-monitoring data completed by the self-referred subjects, predictive efficiency analyses indicated that the QEWP was able to identify both high and low probability binge eaters. Implications of the findings for the definition, assessment, and utility of the BED diagnosis are discussed.

Adult↗
PubMed1994

Body image changes over the menstrual cycle in normal women.

Changes in body image across the menstrual cycle and the relationship between these changes and menstrual distress were investigated in an effort to identify determinants of body dissatisfaction. Twenty-six normally cycling women between 18-40 years of age and 90-115% of ideal body weight, with no history of an eating disorder, completed a series of body image measures and a measure of menstrual distress during three menstrual cycle phases: perimenstrual, follicular, and luteal. These phases were identified with serum levels of ovarian hormones and basal body temperature. Results indicated that body dissatisfaction as measured by the number of body-related negative thoughts and anxiety about appearance was significantly highest during the perimenstrual phase. In contrast, measures of body size perception remained stable. Several somatosensory and psychological symptoms of menstrual distress were significantly associated with body dissatisfaction during the perimenstrual phase including, water retention, autonomic reactivity, control, negative affect, and impaired concentration. This association of body dissatisfaction and menstrual distress strongly suggests that menstrual cycle changes play a significant role in body image.

Adolescent↗
PubMed1994

Repeated binge/purge cycles in bulimia nervosa: role of glucose and insulin.

The available data indicate that over half of patients with bulimia nervosa binge and purge daily with repetitions of the binge/purge cycle being common. An understanding of the biobehavioral mechanisms associated with frequent binge/purge cycles may aid the general conceptualization of bulimia nervosa including its development and maintenance. Binging and purging have demonstrable physiological effects that may be partially responsible for the repetition of binge/purge episodes. In the present study, the cephalic phase oversecretion of insulin and high insulin levels subsequent to purging were investigated as possible mediators of repeated binging and purging. Insulin and glucose levels of bulimic and nonbulimic women were measured in response to: thinking about food, the presence of food, while eating, and for the bulimic group, after purging. Bulimic subjects displayed a dramatic reduction in both insulin and glucose after purging the test meal. When these same subjects ate a subsequent meal that was not purged, they displayed elevations in insulin and glucose similar to those of the normal controls. The hypoglycemia resulting from purging appears to be partially responsible for the continuation of repeated binge/purge episodes.

Adult↗
PubMed1994

Energy regulation over the menstrual cycle.

To investigate the influence of ovarian hormones on energy regulation, twenty-six normal weight, noneating disordered women recorded their food consumption, exercise, and eating patterns over one menstrual cycle. Serum levels of ovarian hormones were measured to determine three menstrual cycle phases, namely perimenstrual, follicular/ovulatory, and luteal. Energy intake increased 685.9 kJ/day (p < 0.05) from the follicular/ovulatory to the luteal phase. The percent of energy intake as protein and carbohydrate was relatively constant over the three menstrual cycle phases, but the intake from fat increased 2% (p < 0.05) during the luteal phase, representing a 9.2 g/day increment. Variations in energy and fat intake over the menstrual cycle were not attributable to differences in energy expenditure through exercise or dietary restraint, and appear to be related to changes in the estrogen/progesterone ratio.

Adolescent↗
PubMed1994

Study of genetics, epidemiology, and vitamin usage in familial spina bifida in the United States in the 1990s.

We analyzed family structure, genetic patterns, epidemiology, and vitamin usage in a series of families with multiple cases of spina bifida (familial SB). Among 6,491 individuals ascertained in 72 families with familial SB, we identified 180 patients--85 males and 95 females. The number of collateral cases on the maternal side (49 of 3,588), analyzed by category of kinship, were significantly higher than those on the paternal side (16 of 2,903) (p = 0.0002). Genomic imprinting or a partial mitochondrial contribution are possible mechanisms for this maternal effect. The proportion of US-born SB families reporting some Irish ancestry (49%, 34 of 70) or some German ancestry (50%, 35 of 70) were significantly higher than those for the US population at large. In contrast, the proportion of families reporting some African-American ancestry (1%, 1 of 70) was significantly lower. The elevated proportions of families with Irish and German ancestry, the high frequency of SB in Northern Ireland and in certain regions of Germany, the reduced proportion of families with African-American ancestry, and the lower prevalence of SB in African-Americans all suggest a genetic contribution to the etiology of the disorder. In our study, the proportion of mothers who used supplemental vitamins during the periconceptional period (29%, 47 of 163) was not significantly different from that in the US population at large.

Birth Order↗
PubMed1994

Familial aggregation of amyotrophic lateral sclerosis, dementia, and Parkinson's disease: evidence of shared genetic susceptibility.

Clinicians have long suspected an association of classic amyotrophic lateral sclerosis (ALS) with Parkinson's disease (PD), dementia, or both. If proven, this would raise the possibility of a shared genetic susceptibility to the three disorders. To investigate this hypothesis, we compared 151 newly diagnosed ALS patients (seven familial) with 140 controls in terms of cumulative incidence of ALS, PD, and dementia in parents, siblings, and grandparents. We used Cox proportional hazards analysis to compute rate ratios (RRs) for ALS, dementia, and PD in relatives of ALS patients versus relatives of controls. The risk for dementia was significantly higher in relatives of ALS patients than in those of controls (RR = 1.9; 95% CI 1.1-3.1) and was similar for relatives of patients with sporadic and familial ALS. The risk of PD was higher in relatives of patients with familial ALS (RR = 5.6; 95% CI 0.6-50.3) than in relatives of patients with sporadic ALS (RR = 1.8; 95% CI 0.5-6.0), but these differences were not statistically significant, probably due to insufficient statistical power with the available sample size. These findings indicate that ALS and dementia, and perhaps also PD, co-occur within families more often than expected by chance, suggesting that there may be a shared genetic susceptibility to these disorders.

Adult↗
PubMed1994

A clinical genetic study of Parkinson's disease: evidence for dominant transmission.

We used a family history questionnaire, semi-structured interview, and personal examination of secondary cases to collect data on the prevalence of Parkinson's disease (PD) in relatives of patients seen consecutively for 1 year and assessed the proportion of secondary cases of PD as a function of pedigree completeness. Survival analysis methods were applied to estimate the lifetime risk and age-at-onset distribution of PD among first-degree relatives of probands. When we considered siblings of probands with affected parents, the cumulative risk increased significantly over siblings of probands without affected parents, suggesting significant familial aggregation in a subset of randomly ascertained families. We further analyzed 80 multicase families with two or more affected individuals. Age-adjusted segregation ratios approaching 0.5 and similar proportions of affected parents and siblings, as well as the distribution of ancestral secondary cases, were compatible with an autosomal dominant mode of inheritance with reduced penetrance in a subset of PD.

Adolescent↗
PubMed1993

Do the poor sue more? A case-control study of malpractice claims and socioeconomic status.

OBJECTIVE: To evaluate whether socioeconomic status is associated with risk of malpractice claims, particularly among those who have suffered medical injury. DESIGN: Case-control study. SETTING: Fifty-one hospitals in New York State. METHODS: The presence and severity of medical injury, defined as disability at the time of discharge or prolongation of the hospitalization caused by medical treatment as opposed to the disease process, were assessed through review of approximately 31,000 hospital records in New York in 1984. These sampled records were then linked to formal malpractice claims. To estimate the risk of malpractice claims by age, gender, race, insurance status, and income, we conducted a case-control study of claimant cases matched with nonclaimant controls. The cases were all those patients who filed malpractice claims referring to alleged malpractice during a sampled hospitalization. Physician reviewers had previously judged the level of disability that resulted from the medical injury. Claimants (n = 51) were each matched with five nonclaimant controls on the basis of injury. Noninjured cases were matched with noninjured controls and injured cases were matched with injured controls. RESULTS: We found that poor patients (odds ratio [OR], 0.2; 95% confidence interval [CI], 0.03 to 0.8) and uninsured patients (OR, 0.1; 95% CI, 0.005 to 0.9) were significantly less likely to file malpractice claims, after controlling for the severity of medical injury. Among patients who suffered medical injury, the elderly (OR, 0.2; 95% CI, 0.03 to 0.9) were also less likely to file claims. Gender and race were not independently associated with risk of malpractice claims. CONCLUSIONS: Poor and uninsured patients are significantly less likely to sue for malpractice, even after controlling for the presence of medical injury. Fear of malpractice risk should not be a significant factor in the decision to serve the poor. Tort reforms that would protect physicians who serve the medically indigent from malpractice suits may not be warranted.

Case-Control Studies↗
PubMed1993

Preventing medical injury.

Although adverse events (AEs) are not uncommon in hospitalized patients, they are by no means inevitable. A review of records from a population-based study in New York revealed that nearly 4% of hospitalized patients suffered AEs. Two-thirds of those events were considered to be caused by errors in management, most of which were not because of negligence. A large part of the reason preventable AEs occur is that today's medical care is extremely complex, involving a variety of personnel, equipment, and procedures. By seeking to eliminate errors in the system of providing care and raising the awareness of health care providers about the potential for AEs, programs can be established to address and possibly remedy this serious problem.

Accident Prevention↗
PubMed1993

Waardenburg syndrome and myelomeningocele in a family.

We report the first family with Waardenburg syndrome type 1 and myelomeningocele in which more than one subject was affected with both disorders. The possible association is discussed. Prenatal screening for myelomeningocele is suggested for a family with Waardenburg syndrome type 1.

Child, Preschool↗
PubMed1993

Neuropsychiatric aspects of adult-onset Tay-Sachs disease: two case reports with several new findings.

Deficiency of hexosaminidase A causes the GM2 gangliosidosis known as Tay-Sachs disease. It is now known that this condition has several late-onset variants that cause numerous neuropsychiatric disturbances. Early recognition is important because treatment with phenothiazines and heterocyclic antidepressants may worsen the course. The authors report two cases with several new findings, including prominent psychiatric symptoms without psychosis early in the course of the illness.

Adult↗
PubMed1992

Parental sex effect in spina bifida: a role for genomic imprinting?

Fifty families (491 individuals in 137 sibships) with more than one living case of isolated, nonsyndromic spina bifida (SB) were analyzed genetically. There were twice as many gene-carrier females (56) as gene-carrier males (28) (P < 0.005). This was not an artifact of ascertainment bias because the sex ratio of gene-carriers was the same whether the pedigree was obtained through the proband's father or mother. Also, this effect was not observed in other disorders analyzed by the same method. Neither was the effect due to differential fertility because the number and sex of affected and unaffected children per gene-carrier parent were not different for male or female gene-carrier parents. There was no evidence that the missing male gene-carriers were lost by selective spontaneous abortion. There was no deficit of male-to-male or male-to-female transmission, excluding simple X-linked or simple mitochondrial inheritance. If genomic imprinting plays a role in the unequal female and male carrier frequencies in SB, penetrance should differ with parental sex. Penetrance was higher for offspring of female parents than of male parents, but the difference was not statistically significant. In addition, both male and female gene-carriers were frequently found in the same pedigree. Thus, the present data suggest a possible role for imprinting in SB.

Female↗
PubMed1992

The economic consequences of medical injuries. Implications for a no-fault insurance plan.

BACKGROUND: There has been little research into the actual economic consequences of medical injuries. This inhibits informed discussion of alternatives to malpractice litigation. For example, the cost of no-fault medical accident insurance has been thought to be prohibitive. METHOD: As part of a comprehensive analysis of medical injury and litigation, we interviewed a random sample of 794 individuals who had suffered medical adverse events in New York hospitals in 1984 and used their responses to calculate the cost of injuries. We then estimated the costs of a simulated no-fault insurance program that would operate as a second payer to direct insurance sources and would compensate for all financial losses attributed to medical injury. RESULTS: The estimated costs that would be paid by a simulated no-fault program were $161 million for medical care, $276 million for lost wages, and $441 million in lost household production, or a total of $878 million in 1989 dollars for the cohort of patients who were injured in 1984. CONCLUSION: Although our estimate does not include administrative costs, it nonetheless indicates that a no-fault program would not be notably costlier than the more than $1 billion New York physicians now spend annually on malpractice insurance.

Adult↗
PubMed1992

Hereditary Lewy-body parkinsonism and evidence for a genetic etiology of Parkinson's disease.

The first systematic genetic study of Parkinson's disease (PD) was carried out by Mjönes in 1949. His results indicated autosomal dominant transmission with 60% penetrance. These conclusions, however, were long discounted because oligosymptomatic and atypical relatives were counted as secondary cases without clear justification. Subsequent surveys of patient and twin studies failed to confirm evidence of familial concentration and the hypothesis of a genetic etiology was over-shadowed by interest in possible environmental neurotoxins. A growing accumulation of pedigrees of histologically confirmed Lewy-body PD over the past several years has refocused attention on genetic factors. Fluorodopa positron emission tomography (PET) studies in oligosymptomatic co-twins of probands and recent clinicopathologic studies of atypical cases have provided retrospective support for Mjönes' methodology. A survey of a personal series of PD patients showed that the majority of those for whom pedigree information was available were familial. This along with another recently reported series confirm Mjönes' data showing similar segregation ratios for siblings and parents, a low ratio of maternal:paternal transmission and a marked asymmetry in the distribution of ancestral secondary cases. These findings favor monogenic autosomal dominant inheritance and show reason to argue against a multifactorial etiology or heteroplasmy. The clinical evidence justifies searching for gene loci linked to PD. Available methods are briefly outlined. Preliminary investigations have examined possible allelic associations, e.g., with alleles of MAO-A and debrisoquine hydroxylase and linkage to the tyrosine hydroxylase gene on chromosome 11p15.5 has been excluded in one study of juvenile familial parkinsonism. Linkage mapping studies are presently underway.

Brain↗
PubMed1992

A 17th-century founder gives rise to a large north American pedigree of autosomal dominant spinocerebellar ataxia not linked to the SCA1 locus on chromosome 6.

There have been three reports since 1969 of members of a "W" family with autosomal dominant spinocerebellar ataxia (SCA), and various conclusions have been drawn about the nosology. This pedigree has been traced back over 300 years through 11 generations. Although phenotypically similar to the disorder in the Schut-Swier, Nino, and other kindreds, the disorder in the W family is not linked to the SCA1 locus on chromosome 6, as reported in those hereditary ataxia pedigrees. The W family represents the largest such North American kindred yet reported. We examined 33 family members of a distantly related branch of the W family, determined the cumulative age of onset, and projected the number of present-day gene carriers. Two cases illustrate the spectrum of symptoms among family members. Age of onset and presenting symptom, however, seem to correlate both in our patients and in those previously reported. Between 2,000 and 5,000 individuals are estimated to be at risk of developing the disorder within this pedigree alone. The pedigree reported here will be valuable in the identification and cloning of a gene for hereditary ataxia, designated "SCA2" at the Eleventh International Workshop on Human Gene Mapping.

Adult↗