The cerebroplacental Doppler ratio predicts postnatal outcome in fetuses with congenital heart block.

OBJECTIVE: Autoimmune-mediated congenital complete heart block (CCHB) is associated with significant perinatal morbidity and mortality, and prenatal monitoring of these fetuses is a significant challenge. The cerebroplacental Doppler ratio (CPR), defined as the ratio of the middle cerebral artery pulsatility index (MCA-PI) to the umbilical artery pulsatility index (UA-PI), is predictive of perinatal outcome in growth-restricted fetuses. This study tests the hypothesis that the CPR would be useful in monitoring fetal well-being in fetuses with CCHB. STUDY DESIGN: We reviewed data on all patients diagnosed prenatally with autoimmune-mediated CCHB at our institution over the past 2 years. The fetal echocardiograms from each patient were analyzed for: CPR, UA-PI, MCA-PI, cardiothoracic ratio, degree of tricuspid regurgitation, ventricular function and presence of effusions. We compared hemodynamic data from the fetal echocardiogram before delivery in patients who required urgent pacemaker placement postnatally to those patients that did not require an urgent pacemaker. RESULT: Five patients with autoimmune-mediated CCHB were identified, and two patients required urgent pacemaker placement. The CPR before delivery was significantly lower in the patients who required urgent pacemaker placement (0.97+/-0.06 versus 1.45+/-0.03; P=0.003), but there was no difference in other fetal echocardiographic markers between groups. Both patients requiring urgent pacemaker placement had a CPR less than 1.08. CONCLUSION: The CPR is predictive of postnatal outcome in fetuses with CCHB and is useful in prenatal monitoring of these patients.

A case of an infant with flail tricuspid valve due to spontaneous papillary muscle rupture: was neonatal lupus the culprit?

A 3-month-old infant presented in extremis with a flail tricuspid valve. The authors theorized that the genesis of her papillary muscle rupture was perinatal ischemia compounded by worsening pulmonary valvular stenosis leading to excessive fiber tension. Her underlying diagnosis of autoimmune-mediated heart block with endocardial fibroelastosis and prenatal glucocorticoid steroid treatment represents potentiating factors.

Reengineering the medical staff. Thirteen equals two.

Managed care forces were putting pressure on Athens Regional Medical Center, Inc., and its medical staff to sustain quality of care, and simultaneously tighten the budget, achieve continued improvements in efficiency, and retrain employees. To ensure its leadership position in Northeast Georgia, ARMC decided to restructure the organization to avoid a "slash and burn" crusade later. This article addresses the medical staff restructuring and how it came about. The strategy eliminated five committees and reduced 13 Medical Staff departments to two.

Benign intracranial hypertension in children with growth hormone deficiency treated with growth hormone.

We report 13 cases of benign intracranial hypertension (IH) in children with growth hormone (GH) deficiency treated with GH in the United States. The group consisted of eight boys and five girls, 3 to 16 years of age (median, 9 years). The interval from starting GH therapy to diagnosis of IH was 2 weeks or less in six patients, between 2 and 12 weeks in four, 8 months in one, 5 years in one, and unknown in one. Seven patients were not known to have previously described IH risk factors; the other six had at least one factor each. All patients but one had headache, nausea, vomiting, and visual changes. All had papilledema, and cerebrospinal fluid pressures were elevated (> 250 mm H2O) in all nine patients tested. The GH dosage range was 0.17 to 0.35 mg per kilogram body weight per week (median, 0.30 mg/kg per week) for the 11 patients with dosage data. After discontinuation of GH and treatment with lumbar punctures and/or medications, signs and symptoms resolved in eight children; in two of these children signs and symptoms reappeared when GH therapy was restarted. In four patients signs and symptoms resolved while GH therapy was continued; one child was treated with a ventriculoperitoneal shunt because of an arachnoid cyst, after which GH was restarted without subsequent IH. In the 12 patients with idiopathic GH deficiency the course of IH was benign, with complete resolution of all signs and symptoms. Because doses and scheduling of GH administration have changed since the introduction of recombinant GH, higher doses and increased frequency of administration may be contributing to the development of IH in some patients. We suggest beginning therapy at the lowest recommended dose, with gradual titration to higher doses, and the performance of routine funduscopic examinations during initiation of GH therapy and whenever signs or symptoms of IH develop.

Reporting quality of health care to the board.

The reporting of quality of health care to the governing board has long been an enigma. Now we are in the midst of a revolution in health care, as we shift our focus from solely the clinical performance of individuals to a broader scope of assessing and improving all activities around patient services and patient care--i.e., management outcomes integrated with clinical outcomes to help identify opportunities to improve patient care. In addition, apprised of corporate liability for the quality of care provided in health care organizations, governing boards are raising questions and demanding more information. To maintain this high degree of interest in quality of health care, information should be restricted to what the board needs to know. This article will be confined to the hospital's organizationwide quality system of monitoring and evaluating. While medical staff credentialing and privileging are also board responsibilities and quality management activities should be used in the privileging and credentialing process, they will not be addressed in this article.

Implementing a quality assessment program.

It is difficult to imagine a more stressed organization than today's hospital. If the scope of change is not a sufficient challenge, the rate of the change is staggering, especially in quality assessment. Now we are poised for continuous quality improvement, whereby outcomes identified by quality assessment become the focus for system and process review and modification. It is imperative that a good quality assessment program be in place before implementing and integrating a continuous quality improvement process. The purpose of this article is to show how a quality assessment system can be implemented in a community hospital, regardless of size or scope of services. Key to the process is making all staff members part of the system's development and operation.

The relationship between medical students' locus of control and promotion of breast self-examination.

There is general agreement that medical school curricula should involve the teaching of preventive health behaviours. Locus of control is a personality variable that has been associated with health practices and may be associated with tendencies to promote health practices. Few significant relationships have been found correlating health locus of control beliefs with preventive health behaviours. This study investigated the health locus of control of medical students and their tendencies to talk about and teach breast self-examination to women patients. Women students' health locus of control and breast self-examination practices were also investigated. The authors conclude that health locus of control has little influence on the tendency of medical students to promote breast self-examination. Other factors apparently have more effect on the student doctor's tendencies to promote health practices.

Type II hyperprolinaemia in a pedigree of Irish travellers (nomads).

We describe a study of 312 subjects in 71 families near related to a proband with type II hyperprolinaemia. The subjects were Irish travellers (nomads) among whom consanguineous marriage and high fertility are common. Thirteen additional cases of type II hyperprolinaemia were discovered; all were offspring of consanguineous unions. A further 50 subjects were found to have mild hyperprolinaemia. We found a strong association between type II hyperprolinaemia and seizures during childhood but no significant association with mental handicap. Most adults with type II hyperprolinaemia enjoyed normal health and there was no evidence that maternal hyperprolinaemia compromised fetal development. The documented association between type II hyperprolinaemia and seizures may be related to the neuromodulatory or reducing-oxidising effects of proline and pyrroline-5-carboxylate, respectively, that has been shown in vitro. Alternatively, another genetic defect closely linked to the type II hyperprolinaemia allele could be the explanation.

Fluctuations in plasma pyrroline-5-carboxylate concentrations during feeding and fasting.

Pyrroline-5-carboxylate (P5C) is the oxidized metabolite of proline. In cultured cells redox-sensitive metabolic pathways are influenced by these two amino acids. To determine whether plasma P5C levels fluctuate enough physiologically to regulate such pathways in vivo, we measured venous plasma P5C concentrations hourly for 48 h in five normal subjects while eating and in four subjects while fasting. Two fed subjects had one or more plasma P5C excursions per day exceeding 10 times the baseline level; the other fed subjects had lesser variations. These patterns suggest a relationship of plasma P5C peaks to meals. This relationship is supported by the finding of no significant P5C elevations above baseline levels in four subjects during fasting for 24 h. Despite the changes in plasma P5C concentrations, those of proline and other metabolically related amino acids were constant. The variation among subjects in the degree of plasma P5C fluctuation could not be explained by differences in total protein and caloric intake or body weight. Among plasma constituents, the large peak to basal plasma P5C excursions in some of the normal subjects we studied are matched only by those of peptide hormones.

Teachers' perceptions concerning the relative values of personal and clinical characteristics and their influence on the assignment of students' clinical grades.

Twenty senior teachers were asked to rank, in order of influence, the seven clinical and five personal characteristics used to grade third-year medicine clerks. Seventeen perceived themselves to be more influenced by clinical characteristics when assigning grades. Independently, the actual ratings completed over a 3-year period by these same teachers were analysed to measure the congruency between their perceived and actual grading behaviour. When actually rating students only nine raters were more influenced by clinical characteristics and just one half of the teachers displayed a congruency between their perceived and actual rating behaviour. The implications of these findings are discussed.

Increased pro-opiomelanocortin-derived peptide release in myotonic dystrophy.

The response of plasma immunoreactive (IR)-ACTH, IR-beta-endorphin (beta-END) and IR-cortisol to insulin-induced hypoglycaemia, an acute stimulus to the pituitary corticotrophs through the central nervous system, and to synthetic ovine corticotrophin-releasing hormone (CRH), a direct corticotroph stimulator, were studied in normal males and males with myotonic dystrophy. Myotonics had an increased IR-ACTH and IR-beta-END response to hypoglycaemia and an increased IR-ACTH response to CRH compared with normals. Plasma IR-cortisol response were not different in either group of subjects to both stimuli. This neuroendocrine abnormality in myotonic dystrophy may represent a manifestation of the purported specific cell membrane defect underlying the disease. This is the first report of an abnormality in proopiomelanocortin peptide release in myotonic dystrophy.

The aqueous humor of rabbit contains high concentrations of pyrroline-5-carboxylate.

Accumulating evidence suggests that the interconversion of proline and pyrroline-5-carboxylate (P5C) transfers oxidizing or reducing potential within or between cells. We report here that rabbit aqueous humor contains P5C at a concentration at least 20 times that found in rabbit plasma or in biologic fluids from other animals, and, in isolated lens, P5C stimulates the activity of the pentose phosphate shunt. However, aqueous humor in other species contains P5C in the same range of concentrations as their respective plasma. The high P5C concentration in rabbit aqueous humor may be due to comparatively low levels of P5C reductase activity in ocular tissues, especially the cornea. The levels in lens epithelium, though lower in the rabbit, nevertheless could mediate the transfer of oxidizing potential from P5C and stimulate the pentose-phosphate shunt. These results suggest that the eye may serve as a convenient model for the study of proline and P5C-related transfer of reducing or oxidizing potential between tissues.

Curriculum change in the 1980s. A report of 40 southern US medical schools.

Forty southern medical schools were surveyed to evaluate the nature and mechanisms of curricular change during 1980 to 1983. Ninety percent of schools experienced change in some aspect of curriculum. Faculty, curriculum committees, the dean, and external forces were stimuli for change. Internal self-review or self-criticism seemed to be the most important reasons for change. Proposals were considered by curriculum committees and the dean, but veto power often rested with the dean, although departmental chairmen and faculty occasionally had veto power. Types of change were divided equally between content, timing of courses, and the educational process. Half the schools stated that they had evaluated the changes or planned to do so. Thirteen of 38 changes in curriculum were in the direction of curricular innovations of the 1960s and 1970s and 25 were in the opposite direction.