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We are reporting what we believe to be the first case of moya moya disease (hemiplegia associated with supraclinoid carotid stenosis and multiple cerebral telangiectasia) in a child with Down's syndrome. On cerebral angiography, multiple collateral vessels and rete mirabile (anastomosis of meningeal vessels with internal cerebral vessels) were noted, in addition to the supraclinoid carotid stenosis. Computerized tomography revealed nonobstructive hydrocephalus and findings consistent with multiple vascular insults or infarcts. It is not clear whether moya moya disease represents a true disease entity (congenital arterial dysplasia) or is a syndrome caused by nonspecific vascular reaction. Since abnormal vascular morphology has previously been described in children with trisomy 21, we suggest that the presence of these two disease entities may not be coincidental. It may represent a genetic predisposition in Down's syndrome toward vascular abnormalities, with variable expressivity which manifested itself in this case by abnormalities in the cerebral circulation.

The use of biological dressing techniques accelerates recovery from radical vulvectomy combined with lymphadenectomy. Our earliest interest in the field arose as a result of the morbidity rate which ensued subsequent to surgical therapy of cancer of the vulva. The technique involved in 8 such procedures is described, and its applicability illustrated with photographic plates and review of the literature.

A study was carried out to investigate the menstrual blood loss and changes in sexual life following two methods of tubal sterilization, the Pomeroy procedure and fimbriectomy, as compared with a control group having undergone Cesarean section alone. Results support the approach that hysterectomy be performed in older women, over the age of 40, and fimbriectomy in younger patients, in whom further pregnancy or reanastomosis of the Fallopian tube is absolutely contraindicated.

Recent evidence indicates that many teenagers, despite access to birth control information and services, are choosing not to utilize them, and, therefore, are becoming intentionally pregnant. Several studies have begun to provide reasons for this new dilemma and profiles of those teenagers who become intentionally pregnant. New strategies have to be developed by health care professionals to combat this problem. Some suggestions are presented.

To determine the extent to which different sources of information are perceived to influence common medical decisions, 10 interns, 22 senior residents and 9 faculty general internists rated the degree of influence of house staff, general internists, subspecialists, conferences, journal reading and past experience on their decisions concerning primary prevention (vaccination), secondary prevention (screening) and drug therapy. Analysis of variance of their questionnaire data supports the following conclusions: physicians at different stages of training rely on different sources of information; as physicians advance in training the influence of generalists wanes while that of subspecialists increases; subspecialists and past experience are perceived as primarily affecting therapeutic decisions; primary prevention appears least subject to influence by prevailing information sources; and the preference for reading begins early and increases as physicians advance in training. These data suggest that designing effective medical education requires considering the level of the physician's training and the nature of the medical decision.

Fifty private practitioners and their office staff members were randomly assigned to one of four groups: participants received a protocol for smoking management and a lecture on the consequences and management of smoking, or in addition, had nicotine gum freely available to patients, had stickers attached to their charts, or had gum and reminders. The percentage of patients in each group who had quit smoking a year later was 7.7, 16.3, 8.6, and 16.9, respectively, indicating a significant main effect for the gum conditions. The availability of nicotine gum also significantly increased the amount of time that patients reported they received smoking cessation counseling from the dentists and office staff.

Ten cases of archenteronoma are reviewed with a follow-up between 2 mo and 7 yr. Their presentation was similar to that of rhabdomyosarcoma except for testicular tumors that may present as a hydrocele. A good prognosis was found with tumors amenable to primary resection and chemotherapy. Other tumors are best treated initially with chemotherapy before relatively conservative surgery, radiotherapy being used either pre- or postoperatively.

Periodic sleep apnea, a chronic sleep deprivation state, in which marked changes in the arterial PO2 and PCO2 tensions have been recorded, is a relatively new syndrome not previously reported in pregnancy. It is characterized by episodes of apnea, prevalently obstructive, during sleep. The majority of patients with this syndrome have snored heavily for years, suggesting a causal relationship between snoring and periodic sleep apnea. The effects of prolonged snoring on alveolar ventilation and systemic pressure(s) suggest that this snoring has physiopathological implications on maternal cardio-respiratory reserve and indirectly upon the fetus, especially as there are recordable changes in fetal heart rate and also a change in the acid-base status of the fetus. The possibility that this syndrome may have an adverse effect upon the fetus is stressed.

To examine the effects of intensive patient and/or physician diabetes education on patient health outcomes, a controlled trial was conducted in which internal medicine residents and their 532 diabetic patients were randomly assigned to: routine care; patient education; physician education; or both patient and physician education. Patient outcome data were analyzed either by analysis of covariance on post intervention values (2-hour post-prandial plasma glucose [PPG]; body weight [BW]; blood pressure [BP]; or analysis of variance conducted on change values (fasting plasma glucose [FPG] and glycosylated hemoglobin [A1Hgb]). After patient education, significant improvements were observed in FPG, A1Hgb, BW, and systolic and diastolic BP. Physician education resulted in significant decreases in FPG, A1Hgb and BW. The combination of patient plus physician education resulted in the greatest improvements in patients' health outcomes including FPG, A1Hgb, PPG, BW and diastolic BP. Adjusted systolic BPs were not significantly different in the two groups. While these physiologic improvements were statistically and probably clinically significant, hyperglycemia and obesity still persisted. Thus, achieving optimal patient outcomes for a chronic disease like diabetes mellitus may require a greater or more effective use of resources than currently estimated.

To test the utility of a qualitative chloride titrator strip in facilitating compliance with a reduced sodium intake diet, we enrolled 32 patients into a randomized crossover trial comprising two study periods of four weeks each. The study periods were begun after the patients had undergone extensive instruction in the diet and the use of the strip. A high degree of correlation between the patient's and the laboratory's interpretation of the strip result was identified in 29 of the subjects. Ability to use the strip was not related to level of education. A total of 12 patients achieved compliance with the diet when using the strips. Of these, nine were able to achieve compliance without the strips. Ten patients (30%) had significantly lower sodium intake when using the strips than when they did not use them. We conclude that the use of the chloride titrator strip can be mastered by most patients and, in conjunction with dietary counseling, can facilitate compliance with a reduced sodium intake diet.

An adolescent girl, had a grade 1 immature teratoma combined with a rare manifestation of glial peritoneal implants of grade 0. A unilateral salpingo-oophorectomy was performed. Alpha-fetoprotein levels, which were elevated before the operation, returned to normal. The patient has been symptom-free for more than three years after the surgical treatment. Repeated normal assays for alpha-fetoproteins can serve as a guide to a projected favorable course. The World Health Organization grading of the teratoma is listed and pertinent medical literature is briefly discussed.

The reconstruction of a stable anterior pelvic ring and the tension-free soft tissue closure is the key to a successful reconstructive surgery of the ectopic bladder. Instead of the commonly practised iliac osteotomy to achieve this goal we postulate the bilateral anterior osteotomy of the superior ramus of the pubic bone. The operative technique of this new method is described. The early results of four cases are discussed.

Dietary salt reduction is an important nonpharmacologic remedy for mild hypertension as well as a useful adjunct to drug treatment. However, a reduced salt intake diet is not effective in reducing the blood pressure of all hypertensive patients. Several lines of evidence indicate that some patients are salt-sensitive whereas others are salt-resistant. A series of investigations have been conducted showing that the blood pressure responses to either acute salt and volume loading or to a reduced dietary salt intake are normally distributed. Blood pressure, humoral regulators of blood pressure and renal sodium handling are each found to be influenced by genetic variance. The change in blood pressure from dietary salt reduction is influenced by genetic variance as well. Definitions of salt sensitivity and resistance were formulated, and salt sensitivity of blood pressure was found to occur significantly more often in black than in white Americans. Furthermore, preliminary data suggest that measurement of phenotypes of haptoglobin in blood may assist in identifying salt-sensitive and salt-resistant subjects. Trials of a reduced salt intake diet in pharmacologically treated hypertensive patients are currently being conducted. The data suggest that at least half of the patients are salt-sensitive and that their medications may be reduced in response to the intervention. Results of this study may be of relevance to many of the 60 million Americans with hypertension, particularly to those who are black and elderly.

The diabetogenic variant of encephalomyocarditis virus (EMC-D) induces a diabetes-like syndrome in certain strains of mice. A study was done to determine if virus-induced diabetes could be prevented by interferon (IFN). It was found that the production of diabetes by EMC-D was blocked by either IFN beta or a variety of IFN-inducers in SWR/J, but not ICR Swiss mice. The replication of EMC-D in cell culture was inhibited by IFN beta. It is concluded that the response of pancreatic beta cells to the protective effect of IFN, is probably under genetic control.

1. Steady state values of cell water, intracellular Na+ and K+ concentrations, and the electrical parameters ETr, Em, and Isc in the mucosa of isolated bullfrog small intestine were determined following immersion in sodium sulfate Ringer solutions with identical ionic composition but different osmolalities. 2. Cell water and intracellular K+ concentration were inversely related to the osmolality of the bathing medium. During 1 h immersion, intracellular Na+ concentration was not significantly affected by an increase or decrease in external osmolality. 3. Replacement of a hypotonic or an approximately isotonic (normal) medium by a medium of greater osmolality caused statistically significant decreases in ETr, Isc and the (inside negative) magnitude of Em. Conversely, when a hypertonic or a normal medium was replaced by one of lower osmolality, significant increases in the magnitude of these parameters were observed. 4. An equivalent circuit model for the epithelial cell layer, in which the resistance of a relatively highly conducting extracellular shunt pathway is assumed to be the major determinant of the electrical responses of the small intestine to external osmolality, has been shown to account satisfactorily for the observed changes in ETr and Em. In terms of this model, the experimentally observed dependence of Isc on external osmolality requires that, even when both the mucosal and the serosal sides of the tissue are bathed by identical media, isolated bullfrog small intestine maintains a finite diffusion potential across the shunt pathway. This is consistent with current views concerning transepithelial ionic transfer mechanisms.

A case of severe foetal distress is recorded and the rationale and use of a beta-sympathomimetic drug are described.

Posterior iliac osteotomy is a recognised method employed to facilitate and support anterior abdominal wall closure in patients with bladder exstrophy, but it adds considerably to the already lengthy procedure of bladder exstrophy surgery. Anterior pelvic osteotomy of the superior ramus of the pubic bone was developed to overcome this problem and to achieve a stable anterior pelvic ring with tension-free soft tissue closure. It can easily be performed after completing the bladder closure, without the need to turn the patient.

The Diabetes Education Study (DIABEDS) was a randomized, controlled trial of the effects of patient and physician education. This article describes a systematic education program for diabetes patients and its effects on patient knowledge, skills, self-care behaviors, and relevant physiologic outcomes. The original sample consisted of 532 diabetes patients from the general medicine clinic at an urban medical center. Patients were predominantly elderly, black women with non-insulin-dependent diabetes mellitus of long duration. Patients randomly assigned to experimental groups (N = 263) were offered up to seven modules of patient education. Each content area module contained didactic instruction (lecture, discussion, audio-visual presentation), skill exercises (demonstration, practice, feedback), and behavioral modification techniques (goal setting, contracting, regular follow-up). Two hundred seventy-five patients remained in the study throughout baseline, intervention, and postintervention periods (August 1978 to July 1982). Despite the requirement that patients demonstrate mastery of educational objectives for each module, postintervention assessment 11-14 mo after instruction showed only rare differences between experimental and control patients in diabetes knowledge. However, statistically significant group differences in self-care skills and compliance behaviors were relatively more numerous. Experimental group patients experienced significantly greater reductions in fasting blood glucose (-27.5 mg/dl versus -2.8 mg/dl, P less than 0.05) and glycosylated hemoglobin (-0.43% versus + 0.35%, P less than 0.05) as compared with control subjects. Patient education also had similar effects on body weight, blood pressure, and serum creatinine. Continued follow-up is planned for DIABEDS patients to determine the longevity of effects and subsequent impact on emergency room visits and hospitalization.

The reverse latissimus dorsi muscle flap was successfully used for the repair of recurrent congenital diaphragmatic hernia in three neonates. This muscle flap can be rapidly raised on a reliable blood supply, and provides sufficient vascularized tissue to replace the whole hemidiaphragm without tension. It should be considered at the first reparative operation when insufficient tissue is available for safe repair by direct suture.

A combination of propranolol and hydrallazine was administered to 13 patients with longstanding hypertension during 15 pregnancies. Hydrallazine was continued through labour and delivery in all patients, while in eight patients propranolol was discontinued 2 to 15 days before delivery. Blood pressure control was uniformly good and superimposed pre-eclampsia did not occur during combined therapy. There were 14 livebirths and one unexplained stillbirth. Except for two cases of milk hypoglycemia, there were no neonatal complications.

Calcinosis circumscripta, usually associated with scleroderma, Raynaud's phenomenon, and telangiectasia, usually affects middle aged persons and is more common in females than males in a ratio of 6 to 1. The etiology is unknown, and onset of the lesion is slow and insidious. It occurs in the periarticular area as a painful mass with surrounding erythema and edema. It is visible in x-rays as localized, scattered calcification.

The Diabetes Education Study was a controlled trial of the effects of physician and patient education. This article describes an educational program for internal medicine residents and its effects on ambulatory diabetes management practices. Forty-five of 86 residents practicing in the general medicine clinic of a university-affiliated city/county hospital were assigned randomly to receive a multifaceted program intended to 1) provide specific care recommendations, 2) teach necessary skills, and 3) make the professional and institutional environment more supportive. During the subsequent 11 months, 323 diabetic patients were interviewed and their records audited for evidence of changes in care. Experimental residents utilized fasting blood glucose determinations more often than controls (i.e., during 40% of visits vs. 31%, p = 0.004). Experimental residents also engaged more frequently in a variety of recommended dietary management recommendations. Isolated differences in monitoring/management of chronic complications also were found (e.g., lipid screening: 70% of experimental residents' patients vs. 58%, p = 0.016). Intensive, multifaceted programs of this nature are concluded to result in improvements in diabetes care, over and above that which is attainable through routine methods of clinical training for residents.

This study evaluates the impact of a pipeline assessment (salivary cotinine determination) on the accuracy of self-reported use of cigarettes and smokeless tobacco by 160 rural seventh- and eighth-grade males ranging in age from 12 to 16. Half of them were randomly assigned to complete a questionnaire on tobacco use prior to revealing the biochemical validation materials and collecting samples. The others had the samples collected prior to receiving and completing the questionnaires. The questionnaire-first group reported significantly greater smokeless tobacco use than did the pipeline-first group. Only for the latter were their self-reports significantly corroborated by the cotinine results. These results suggest that the utilization of a biochemical pipeline not only can improve self-reported tobacco use but also may help identify perceptions about the social desirability of using harmful substances.

We examined the role of physicians' knowledge and attitudes in effective diabetes management among internal medicine house staff. We examined the extent to which (1) knowledge of common diabetes management strategies and (2) beliefs about the efficacy of, and intentions to engage in, such strategies predict success in their management of patients with diabetes. Successful managers are those house staff whose ambulatory diabetes patients' fasting blood sugar levels were lower than the clinic median. Knowledge was assessed with a written case simulation of an obese noninsulin-dependent woman; beliefs and intentions were surveyed through a self-administered questionnaire. The data suggest that knowledge was unable to discriminate physicians who are relatively successful in controlling their patient's blood sugar levels from those who are less successful. However using intentions related to initial and follow-up management strategies and beliefs about the benefits of achieving euglycemia, there was discrimination between the two physician groups (P less than 0.001). These findings suggest that beliefs are important in understanding why some physicians are successful managers. Further, if beliefs are mutable, continuing medical education courses could be more effective by fostering critical beliefs rather than depending solely upon the transfer of new medical information.