Secretory dysfunction in parathyroid cells from a neonate with severe primary hyperparathyroidism.

We report a patient with neonatal severe primary hyperparathyroidism whose parathyroid cells were markedly refractory to regulation by calcium in vitro. He showed life-threatening hypercalcemia (4.8-5.2 mM vs. normal of 2.1-2.7 mM). A sibling had been treated previously for an identical disorder. At age 6.5 months, four hyperplastic parathyroid glands were removed, and portions of one were immediately grafted into the forearm. Serum calcium again became elevated post-operatively and then fall to the normal range after excision of grafted parathyroid tissue. Dispersed parathyroid cells from the first operation showed no suppression of PTH secretion by 2 mM calcium; however, there was normal maximal suppressibility at 4 mM calcium with half-maximal suppression at 2.53 mM (the calcium set point). This contrasts with much lower set points previously established for suppressible cells from normal (1.02 +/- 0.10 mM, mean +/- 1 SD), from primary hyperplastic (1.10 +/- 0.14 mM), or from adenomatous (1.26 +/- 0.14 mM) parathyroid glands. The strikingly high set point may not be unique because a small number of glands previously classified as nonsuppressible (by the criterion of failing to suppress below 50% maximum at calcium concentration up to 2-3 mM) might have shown similarly high set points if tested at higher calcium concentrations. We conclude that an unusual abnormality of PTH secretory control accounts, in large part, for both the marked hypercalcemia and for its refractoriness to surgical treatment in this patient.

Weighing the evidence: the Canadian experience.

The Canadian Task Force on the Periodic Health Examination created a hierarchy of evidence that has been used for the past 18 y to evaluate the scientific evidence for and against the preventability of each condition reviewed. The methodology developed by the task force may be applicable to study of the preventive aspects of dietary sodium and health. This paper describes the history and modus operandi of the task force.

Impact of preadmission evaluations on elective hospitalization of children.

It was hypothesized that a well-organized evaluation of children scheduled for elective medical admission would: (1) significantly reduce average length of stay; (2) meet with parent satisfaction; and (3) significantly reduce health care costs. To test this hypothesis, a system of preadmission procedures was established for patients scheduled for elective medical admission to a 332-bed tertiary care pediatric hospital, and the impact of the system on these three parameters was evaluated. Compared with 167 concurrent control patients and 379 historical matched control patients, a highly significant shortening of length of hospital stay was associated with the preadmission program. Preadmission evaluation was associated with a reduction of 2.31 days in mean hospital stay and of three days in median stay as compared with number of days for concurrent control patients (P less than .01). Patient and staff satisfaction was high. The extension of such a program to most elective medical admissions should result in significant savings in health care costs and reduction in family inconvenience.

Controlled trial of a home and ambulatory program for asthmatic children.

Care of asthmatic children is often episodic and more therapeutic than preventive. A 2-year randomized, controlled trial involving 95 children measured the impact of a comprehensive home and ambulatory program for pediatric asthma management using objective outcome measures. Interventions for the study group during the first year included 3-month clinic visits, education, and home visits by a specially trained research nurse. Control subjects continued to receive regular care from a family physician or pediatrician. Eight-nine subjects (93%) completed the study. Study subjects had less school absenteeism than control subjects (10.7 vs. 16.0 days, P = .04) and showed significantly better small airway function after 1 year. Asthma severity improved in 13 study subjects and worsened in 5. The reverse was true for control subjects. Study subjects exhibited better metered aerosol technique than control subjects (P = .0005). Fewer days were spent in hospital by the study subjects admitted compared with control subjects (3.67 vs 11.2 days, P = .02). After 1 year, more study than control families (72.1% vs 33.1%, P = .006) reported that their asthmatic child took responsibility for the asthma management. The intervention failed to reduce exposure to secondhand smoke or to household pets. There were no significant differences in medical visits, theophylline levels, or records of asthma symptoms. One year after discontinuing the intervention, a marked "washout" effect was observed. Comprehensive ambulatory programs of childhood asthma management can improve objective measures of illness severity but must be sustained.

Failure to thrive.

Reviewing the chronological evolution of our understanding of why some children fail to thrive without obvious organic cause permits us to develop a rational contemporary approach to diagnosis and management. Originally recognized as a phenomenon of children living in institutions, it later became clear that it could occur in children living with their families. While emotional deprivation was at first emphasized as the principal determinant of growth failure, it is now apparent that the major mediating mechanism is insufficient caloric intake. Thus prior to nutritional recovery it may be difficult to segregate whether emotional symptoms in the child reflect the causes or effects of malnutrition. With rare exceptions, an etiologic diagnosis can be suspected on the basis of history, physical examination, and family assessment. Nonspecific investigative screening of such children has proved to be without value. Successful management depends on recognition of the supernormal caloric intake required to induce rapid catch-up growth. Aggressive hyperalimentation combined with intensive emotional stimulation and often with active family participation offer the best chances of a successful outcome.

Design and reliability of pediatric HealthQuiz: preliminary report of a comprehensive, computerized, self-administered child health assessment.

The time available for pediatric ambulatory visits is rarely sufficient to permit a truly comprehensive health assessment. We hypothesized that a reliable, computerized, self-administered questionnaire could be designed to screen for a full range of pediatric health issues and provide a comprehensive health database for pediatric patients. An age- and gender-specific pediatric questionnaire of 478 questions was formatted to elicit only a "Yes," "No," or "Not Sure" response and structured in a branched, decision-tree format. The initial draft was reviewed for content by pediatric experts in Canada and the United States and revised in accordance with their suggestions. The questionnaire was divided into two modules, Medical Peds, covering biomedical issues and Prevent Peds, covering prevention, psychosocial, educational, and safety topics. Cognitive interviews were carried out with 132 parents in pediatric ambulatory care centers in Chicago and Halifax, with use of scripted and nonscripted probe questions, to ensure comprehensibility among patients with widely varying educational levels and health knowledge. Reliability was tested in 100 parents of children aged 1 month to 12 years, through use of five different test-retest sequences. Respondents' impressions were surveyed on completion of the procedure. Following content reviews, and cognitive and reliability testing, the total bank of questions was reduced to 375. As a result of the use of branching logic, individual parents answered an average of 111 Prevent Peds and 144 Medical Peds questions. Average time required to complete the entire questionnaire was 13 minutes for Prevent Peds and 19 minutes for Med Peds. Retesting within 36 hours showed an overall 97% concordance of response pairs in the Medical Peds and Prevent Peds questionnaires. There were no statistically significant differences in test-retest reliability between different sequence formats used, (e.g., HealthQuiz followed by personal interview, or HealthQuiz vs. HealthQuiz). A few questions that frequently elicited "Not Sure" responses were eliminated. As a result, the majority of questions elicited either a "Yes" or "No" response. Pediatric HealthQuiz identified a wide spectrum of child health problems that are often overlooked in routine health visits. Parents completing Pediatric HealthQuiz indicated a high degree of satisfaction with the procedure. Most reported that they believed the information would improve their child's health care.

The role of a tutorial system in undergraduate medical education.

A tutorial system for first- and second-year medical students is now in its third year of operation at McGill University. The program was designed to meet the specific needs of McGill students. The aims and objectives were intentionally defined in a general way, application of detail being the responsibility of individual tutors and their groups of students. The main purpose of the program is the repeated exposure of students to the individual competence of outstanding teachers. A large majority of students and tutors favour the program. It has provided faculty members with insight into some of the problems of present-day medical education. The consensus is that, in the Faculty of Medicine at McGill University, the tutorial system serves an important role, complementing the normal curriculum.

Idiopathic periosteal hyperostosis with dysproteinemia. A new clinical entity.

Two unrelated children, suffering from a transient illness characterized by fever, bone pain and tenderness, inability to walk, x-ray evidence of subperiosteal new bone formation and dysproteinemia, are described. In both children the onset followed a respiratory infection. The fever lasted for about three weeks in 1 patient and two months in the other. As fever subsided, the x-ray evidence of periosteal hyperostosis gradually disappeared, and the electrophoretic pattern of the serum proteins returned to normal. Tibial biopsy in 1 patient showed subperiosteal new bone formation of the right-angled type, with no inflammation and with the presence of mucinous edema. Plasma cells were unusually abundant in the bone marrow. The serum protein disturbance was characterized by hypoalbuminemia and striking elevation of the alpha2 and gamma globulins. Detailed serum protein studies performed in 1 patient showed the hypergammaglobulinemia to be due primarily to an increase in the IgG fraction. No etiologic agent was identified, and recovery was complete in both patients. The entity does not appear to have been described previously.