Idiopathic dilatation of the aorta with dissection in a family without Marfan syndrome.

Dissection of the aorta is very rare in children, but classically occurs in the presence of Marfan syndrome or other connective tissue disorder. We present a case of spontaneous dissection in a 12-year-old boy whose half brother has an idiopathic dilated aorta and whose mother has also required surgery for dissection of a dilated aorta. No features of connective tissue disorder were present in any family member.

Closing wedges: a technique for repair of midline craniosynostosis.

Description of a surgical technique for sagittal and metopic synostosis utilizing closing skull wedges. The technique and results are outlined. The technique has proven effective when done by 4 months of age.

Syncope in childhood: a case control clinical study of the familial tendency to faint.

We investigated the possibility of an inherited tendency to faint by studying 30 consecutively referred well children with vasodepressor or vasovagal syncope. The family history of each patient was reviewed for syncope and for 24 cases was compared with the family history of the child's best friend. None of the best friends had syncope.

The efficacy of pulse-dosed antibiotic therapy in the management of persistent otitis media with effusion.

Consensus has been achieved supporting the efficacy of antimicrobials in the management of acute otitis media with effusion (OME). No such agreement has been reached into medical management of the patient in whom middle ear effusion (MEE) persists beyond the conventional 10- to 14-day treatment cycle. This study is designed to demonstrate the efficacy of antibiotics administered in a single daily dose in the management of persistent otitis media with effusion (POME).

An epidemiological study on increased length of stay.

The epidemiologic triad of causation states that all illness results from a disequilibrium between host, agent and environmental factors. The "illness" investigated in this report--increased LOS--resulted from a combination of: patient factors--the increased prevalence of chronic diseases in childhood, a revolution in neonatal survival and an increase in survivorship in general for severe diseases, such as congenital anomalies and genetic diseases; agent factors--the transition from agents of infectious disease to agents of chronic disease as well as iatrogenesis; and health care environmental factors--equity issues involving the ethics of treatment, changes in medical technology and patterns of medical practice. The use of preadmission testing, increased participation by parents in the care of their children, an investigation of the appropriate venue for care of chronically ill children and the back transfer of recovering children to their home hospitals were recommended and considered by the hospital's administration and board of governors.

Large volumes of apple juice preoperatively do not affect gastric pH and volume in children.

The effect on gastric pH and volume of 0, 6 and 10 ml.kg-1, of apple juice given 2.5 hours before surgery to children aged five to ten years was investigated in this prospective, randomized, single-blind study.

Infantile hypertrophic cardiomyopathy with QT prolongation.

A neonate presented with neonatal myocardial dysfunction and long QT interval with subsequent appearance of hypertrophic cardiomyopathy at one week of age. Maternal glucose metabolism was normal. By 15 months of age the hypertrophy had largely resolved but the long QT persisted.

Routine screening of blood and urine for severe reactions to anticonvulsant drugs in asymptomatic patients is of doubtful value.

Severe or fatal reactions to anticonvulsant agents are fortunately rare. We examined the value of routine screening of blood and urine to detect early signs of such reactions in asymptomatic patients. The basic assumptions of this type of screening program have been faulty or unproven, and the results of studies, although not definitive, have not supported the value of such programs.

Bacterial tracheitis in children.

Bacterial tracheitis is a relatively rare cause of respiratory distress in children and is associated with a high morbidity and mortality rate. Three children who have survived this affliction are presented: one developed toxic shock syndrome in the acute phase and two developed late complications. The authors review the pediatric literature and postulate possible etiological factors.

Sinus arrhythmia in children with atrial septal defect: an analysis of heart rate variability before and after surgical repair.

The expression of sinus arrhythmia depends on separation of the systemic and pulmonary venous return to the heart as well as on normal autonomic control mechanisms. Patients with atrial septal defect provide a naturally occurring experiment of communication between the two venous systems. In adults with atrial septal defect sinus arrhythmia is minimal or absent.

The effect of preoperative apple juice on gastric contents, thirst, and hunger in children.

The effect of 3 ml.kg-1 of apple juice given 2.6 +/- 0.

Acute scrotal pain in children: prospective study of diagnosis and management.

Forty-eight boys were assessed for an acutely painful scrotum. Thirty-six (75%) of them underwent radionuclide scanning of the scrotum; the average age of this group was 11 years. The scan revealed epididymitis in 19 cases, spermatic cord torsion in 9, appendix testis torsion in 7 and acute hernia-hydrocele in 1.

Epidemiology of severe eye injuries in childhood.

The authors conducted a cross-sectional study of all ocular injury cases admitted to a children's hospital between January 1978 and December 1984. Of 222 injuries reviewed, 77 (35%) resulted in some visual deficit. Males were significantly overrepresented in all age groups with an average male:female ratio of 3.

Clobazam for refractory childhood seizure disorders--a valuable supplementary drug.

Clobazam is a new benzodiazepine recently introduced in Canada on an experimental basis. We report our experience with 27 children (average age 9.1 years) with severe intractable seizure disorders.

Isolated infundibuloarterial inversion (S,D,I): a newly recognized form of congenital heart disease.

A newly recognized form of congenital heart disease is presented that is characterized by viscero-atrial situs solitus (S), D-loop ventricles (D), and inverted normally related great arteries (I), the segmental combination being (S,D,I). This anomaly may be called isolated infundibuloarterial inversion because only the subsemilunar infundibulum and the great arteries are inverted, whereas the atrial and the ventricles are not. All three patients had atrioventricular concordance, ventriculoatrial concordance, dextrocardia, superoinferior ventricles, crisscross atrioventricular relations, underdevelopment of the right ventricle, a large ventricular septal defect, and an inverted tetralogy of Fallot type of malformation of the infundibulum and great arteries.

Obese hypoventilation syndrome of early childhood requiring ventilatory support.

In December 1986 a 30-month-old female child with morbid obesity and respiratory failure was admitted to the Izaak Walton Killam Hospital for Children in Halifax. The etiology of the obesity was found to be dietary in origin after ruling out genetic, neurological and metabolic causes. This patient exhibited somnolence and cyanosis in association with hypercapnia and right ventricular overload.

Teaching child and family assessment skills to medical students during brief outpatient clinic experience.

This paper describes a teaching process in which two 4th year medical students learn a family approach to problem solving during a short clerkship of twelve hours spread over four weekly sessions. A clinical-educational process is described, alternating "content" and "process" roles are defined, and the use of a dual earphone communication system is explained. It is suggested that this teaching system could have a wide field of application.

The recruitment of lymphocytes into the skin by T cell lymphokines: the role of gamma-interferon.

Numerous lymphocytes are recruited from the blood into cutaneous DTH reactions. Alpha/beta-interferon (IFN) and its inducers can recruit lymphocytes into the skin after i.d.

Levels of cytomegalovirus seropositivity in homosexual and heterosexual men.

The prevalence of antibodies to cytomegalovirus (CMV) among 57 homosexual men (77.2%) was significantly higher than that among 155 heterosexual men (32.3%) attending a sexually transmitted disease clinic.

Observations on the efficacy of intranasal beclomethasone dipropionate in cystic fibrosis patients.

The authors report the subjective results on the use of intranasal beclomethasone dipropionate in patients with cystic fibrosis. This medication has demonstrated positive results for these patients, whether polyps are present or not, and its use should be considered whenever symptoms of obstruction are present.

Thrombosis of the umbilical cord: analysis of 52 cases and literature review.

Fifty-two cases of umbilical cord thrombosis from 3 patients populations are analyzed and compared with 68 cases from the literature. The incidence of cord thrombosis is approximately 1/1300 deliveries, 1/1000 perinatal autopsies, and 1/250 high-risk gestations. There is a slight male predominance.

Heart-rate variability in children. Spectral analysis of developmental changes between 5 and 24 years.

The variation in instantaneous heart rate is most prominent in infants and younger subjects. In a preliminary study of the effects of maturation on heart rate, we compared the heart rate variations of 29 children and young adults in three groups between 5 and 24 years of age. We used spectral analysis to determine the intensity of the variations in each of the two main frequency bands in which variations occur: HF, 0.

Neonatal seizures: a commentary on selected aspects.

Despite the immaturity of the newborn brain, the neonatal period is reported to have a very high frequency of seizures. This review concludes that many of the neonatal events that are called seizures probably originate from subcortical structures, have little in common with cortical seizures seen in older individuals, and may not benefit from conventional anticonvulsant treatment. Many studies of anticonvulsants in the newborn have important methodologic problems, compounded by the fact that the seizures tend to spontaneously remit with the resolution of the acute hypoxic-ischemic encephalopathy that is most often the cause.