Should routine screening of neonates for deafness be introduced in South Africa?
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Biomedical subjects
Publications and source records attributed to C A Prescott.
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Clinical case reports of 3 children with recurrent respiratory papillomatosis who developed lung dissemination of the disease are presented. The radiological features of the primary pulmonary disease included papillomata in the distal airway and multiple, nodular lung lesions, both solid and cavitated. Changes secondary to pulmonary papillomatosis included atelectasis, infection and bronchiectasis. Respiratory papillomatosis involving pulmonary parenchyma is associated with a poor prognostic outcome. Early detection of such involvement and aggressive treatment of secondary infection are required to limit parenchymal damage.
The process to decannulation requires attention to details from the time of initial tracheostomy, through the pre- and peri-operative period up until the decannulation event. Important points to consider during this process are: a formal tracheostomy rather than a tracheotomy; other potential sites of obstruction than the laryngeal stenosis; gastroesophageal reflux; prevention and control of infection and the use of prophylactic antibiotics; method and type of suture material; stenting; movement and method of feeding. Decannulation itself requires attention to removal of granulation tissue and control of tracheomalacia and tracheostenosis.
Bifid epiglottis is a rare congenital laryngeal anomaly, usually associated with multiple congenital anomalies which include digital anomalies and hypothalamic pituitary dysfunction manifesting as growth retardation. A case is presented of a 2-year-old child with this picture in whom the bifid epiglottis prolapsed over the laryngeal inlet to cause inspiratory airway obstruction. Corrective surgery was not successful and eventually a partial epiglottectomy was required.
OBJECTIVE: The authors reviewed the combined interventional radiologic and surgical management of 54 patients with intrahepatic stones at the Johns Hopkins Hospital. The team approach used large-bore transhepatic stents to access the intrahepatic ducts until they were stone free. SUMMARY BACKGROUND DATA: Intrahepatic stones are uncommon in western countries. As a result, few American institutions have had much experience, and multiple management algorithms have been suggested. Nonoperative, operative, and combination surgical and nonoperative approaches have been advocated. At Johns Hopkins, combined surgical and percutaneous management has been used for 18 years. METHODS: This team approach includes (1) percutaneous placement of transhepatic access catheters, (2) surgery for underlying biliary disease and stone removal, and, when necessary (3) postoperative percutaneous choledochoscopy and stone removal through the transhepatic stents. RESULTS: The median age of the 54 patients was 50 years, and 32 were men. Biliary disease included 27 benign strictures, 7 sclerosing cholangitis, 5 choledochal cysts, 5 parasitic infections, 5 choledocholithiasis, and 5 biliary tumors. Fourteen patients (26%) were treated exclusively with percutaneous techniques. Forty patients (74%) had surgery, including 36 Roux-en-Y hepatico- or choledochojejunostomies with large-bore transhepatic stents. Eighteen of these 40 patients (45%) with multiple intrahepatic stones, strictures, or both required additional procedures after operation. No hospital deaths occurred after any of the percutaneous or surgical procedures. With a mean follow-up of 60 months, 94% of patients were stone free, 87% of patients were symptom free, and 73% have had their transhepatic stents removed. CONCLUSIONS: A combined radiologic and surgical approach with transhepatic stents is a safe and effective method for managing intrahepatic stones.
The authors explored the factors influencing the agreement of diagnoses of alcoholism obtained by a best-estimate (BE) procedure versus those obtained by family history (FH) only, based on data from the Roscommon Family Study. The participants were first-degree relatives of either schizophrenic subjects, subjects with affective disorders, or matched community controls. The FH information was obtained from first-degree relatives of the participants, whereas the BE diagnoses included personal interview and medical records as well as FH information. Two types of error were distinguished: false-negative FHs, who were diagnosed with alcoholism by BE but not by FH; and false-positive FHs, who were diagnosed with alcoholism by FH but not by BE. The risk of false-negative FHs was increased by young age of the subject and male gender of the informant, and decreased by a history of previous hospitalization of the subject. Conversely, the risk for false-positive FHs was increased by older age of the subject, male gender of the subject, female gender of the informant, and informant's diagnosis of alcoholism. Comorbid diagnosis of nonaffective psychosis increased the risk of both types of error. It is concluded that when validated against a BE diagnosis, the FH diagnosis of alcoholism is subject to several biases and that the FH method is not a satisfactory substitute for BE diagnoses.
A growing literature supports genetic contributions to familial resemblance for alcohol use characteristics, but few studies have focused on the mechanisms underlying alcohol use among older persons. We report patterns of alcohol use in a U.S. volunteer sample of 3,049 female and 1,070 male twins aged 50 to 96. Significant gender and age effects were found for self-report measures of current and lifetime alcohol use, with greater intake among males and current and lifetime abstinence more common among older participants. Comparisons with data obtained 4 years previously revealed high stability for quantity and frequency of alcohol consumption. Twin pairs with more frequent social contact tended to be more similar for lifetime and current alcohol use. Biometrical genetic modeling results indicate that use of alcohol is highly familial, with both genetic and shared environmental factors contributing to initiation of alcohol use among men and women. Among drinkers, however, the degree of twin resemblance for consumption behaviors is low to moderate and appears to be regulated by shared genes rather than shared environments. These data are consistent with a multidimensional process, suggesting that the determinants of whether one drinks in older age differ from those underlying how much or how often alcohol is consumed.
Few studies have employed genetically informative designs to study the causes of alcohol-related problems in nonclinical populations. We report patterns of alcohol abuse in a community-based U.S. volunteer sample of 3,049 female and 1,070 male twins aged 50 to 96. Significant gender and age effects were found for self-report measures of current and lifetime alcohol-related problems, with higher prevalence among males and lower frequency among older birth cohorts. Significant associations were found between severity of alcohol abuse (adapted from Feighner criteria) and age of drinking onset, parental history of alcohol problems and, among males, lower educational attainment. Model-fitting analyses based on data from 650 identical and 479 fraternal twin pairs indicate substantial family resemblance for a variety of definitions of lifetime alcohol abuse and alcohol problems. The median estimate of genetic variance across several definitions of alcohol problems was 38.5%, while that for shared environmental influence was 15.5%. Gender heterogeneity was not found for magnitude of genetic and environmental influences, but these comparisons were limited by low statistical power. Findings are discussed with reference to the literature on alcohol abuse among older adults and the genetic epidemiology of alcoholism.
Ambulatory surgery has become an important component in the provision of surgical care. In 1987 a day-case surgical unit was established at Red Cross War Memorial Children's Hospital, Cape Town. Experience over a 6-year period (1987-1992) is reviewed in relation to the nature and total number of surgical procedures, pre-operative preparation, daily utilisation of the facility, postoperative care and complications. During this period 16,538 patients (mean age 3 years) were operated on in the unit. Nine surgical disciplines participated. Pre-operative assessment and preparation reduced the rate of cancellations on the day of surgery to less than 6%, with a 90% effective use of schedules operating time. Scarce nursing resources were also maximally utilised. Most procedures took less than 30 minutes and only 0.8% exceeded 1 hour. The average ward stay was 6 hours. No major complications were encountered and acceptance by patients and parents was excellent. Only 16 children who were not scheduled to do so needed to stay in hospital overnight. Recommendations to establish and improve day-stay surgical services are presented. It is concluded that a practical, efficient service can be established and that the authorities should be encouraged to expand appropriate facilities.
During the period 1982-1988, 20 new cases of laryngeal papillomatosis in children were seen at The Red Cross War Memorial Children's Hospital. HPV typing of biopsy specimens was performed. Their clinical course was reviewed and age at presentation ranged from 1 to 10 years. There was no correlation between age and either aggression or a prolonged clinical course. HPV DNA was identified in all 20 cases (100%). Fifteen (75%) were HPV Type 11 and 5 (25%) were HPV Type 6. There were no mixed infections. HPV Type 6 infection was more clinically aggressive than HPV Type 11 infection. The intensity of the virus signal in the biopsy specimens was not in general indicative of the clinical behavior of the disease, although two children with particularly strong intensity exhibited aggressive disease. It is suggested that identification of HPV type 6 infection has prognostic significance.
The design of an adaptor for use with fibre-optic flexible endoscopes with a single instrument channel is described that enables them to be used for oesophagoscopy. The adaptor enables both aspiration of secretions as well as gas insufflation to expand the oesophagus distal to the tip of the endoscope down the single channel. When the adaptor is used on a pediatric bronchoscope it is possible to perform oesophagoscopy in an outpatient setting on the awake child. This application is particularly useful for the assessment of children who have ingested corrosive agents and enables rational management decisions to be based on precise knowledge of the extent of the mucosal injury.
Cartilage grafts or biopsies of such grafts were obtained from six children who had undergone laryngo-tracheoplasty surgery at times ranging from 10 to 38 months after surgery. The histological appearance of the grafted cartilage was of gradual degeneration with chondrocyte necrosis and replacement of cartilage matrix by fibrous tissue.
Ascaris lumbricoides has rarely been implicated in the causation of middle ear disease. An infant who developed acute suppurative otitis media while on antibiotics for an upper respiratory tract infection and who extruded an ascaris worm from the ear is presented. The otitis media subsequently resolved. Our understanding of the pathophysiology of ascariasis in the eustachian tube and middle ear is discussed.
A simple commercially available compression device allowed intermittent ultrasound scanning of the compression site during compression repair of a femoral artery pseudoaneurysm in six patients. All six of the pseudoaneurysms (five superficial femoral and one common femoral) were compressed without compression of the underlying vessels. The procedure was successful in four of the six patients, without complications. Use of this device may decrease operator fatigue during compression repair of pseudoaneurysm.
Family, twin, and adoption studies have firmly established the genetic basis for a majority of cases of schizophrenia. Several other psychiatric disorders probably represent alternative manifestations of this genetic liability. The underlying genetic mechanisms are not yet well understood, but the development of schizophrenia appears to involve a system of genes that interact with pre- and postnatal stressors. Recent reports of genetic linkage in schizophrenia have not been replicated, but the potential of molecular genetic technology makes us optimistic that the bases of this complex disorder will be uncovered in the near future.
Failure of decannulation after paediatric tracheostomy, once the underlying disorder has resolved, is almost always due to peristomal complications. Granulation tissue formation in the raw tissue of the stoma and its subsequent fibrosis requires removal (50 of the 293 tracheostomies from the Red Cross War Memorial Children's Hospital). It is suggested that this can be avoided by creating a formal skin-to-trachea stoma at the time of tracheostomy. Suprastomal depression of the anterior wall of the trachea (52/293) appears to be unavoidable when using standard tracheostomy tubes. Localised stomal site tracheomalacia and stenosis (numbers of this complication are unknown) results from damage to cartilage of the trachea either by incision or by necrosis from pressure of the tracheostomy tube. Trauma to the cartilage needs to be minimised by careful design of the tracheal incision. It is suggested that consideration should be given to creating a formal tracheostomy stoma for any paediatric tracheostomy that is likely to be required for more than a short period of time.
Methods of evaluating adenoidal size pre-operatively are controversial. Symptoms and clinical evaluation have been both advocated and condemned. Depending on the methodology, measurement of the radiological adenoidal shadow has been difficult to correlate with the clinical patency of the nasopharyngeal airway and to the actual size of the removed adenoid. Four different methods were used to measure the adenoidal size on 74 radiographs. The methods were evaluated against the degree to which the adenoids obstructed the nasopharynx on flexible endoscopy of the postnasal space. Obstructive symptomatology was also evaluated against the degree of obstruction. The radiological method that best correlated to the endoscopic findings was that of Cohen and Konak (1985). However, obstructive symptomatology had the overall highest correlation to endoscopic findings.
To investigate the pathogenesis of respiratory lesions caused by the facultative intracellular pathogen, Rhodococcus equi, pulmonary clearance was compared in four groups of genetically defined mice, chosen for their specific deficits in immune and inflammatory responses. Complement-deficient A/J, immunodeficient nu/nu (nude), scid/scid.bg/bg (SCID/beige), C57BI/6J.bg/bg (beige) and normal Swiss mice (SW) received approximately 10(7) R. equi intranasally on day 0. Bacterial clearance was assessed in lung, liver and spleen on days 1, 4, 7 and 14. Pulmonary clearance was not significantly different between SW and A/J mice. Beige mice cleared R. equi more rapidly and completely than A/J and SW, indicating that deficits in phagocytic and NK cell function associated with the bg/bg gene did not compromise clearance. Pulmonary clearance in immunodeficient SCID/beige mice paralleled that of the SW and A/J mice initially but bacterial proliferation produced significant differences from SW mice at day 14. Nude mice were unable to clear R. equi from day 1, resulting in the death of two nude mice at day 11. Both SCID/beige and nude mice developed severe pyogranulomatous bronchopneumonia, whereas A/J and SW mice developed transient pulmonary lesions. Beige mice developed minimal lung lesions. Significant systemic bacterial proliferation occurred only in nude and SCID/beige mice. We conclude that deficiencies in complement components, phagocytic and NK cells do not impair the pulmonary clearance of R. equi but that a competent cellular immune system is required to prevent pneumonia and death. The difference in early phase pulmonary clearance in nude and SCID/beige mice indicates two phases are important for clearance. An acapsular mutant of R. equi was completely cleared from the lungs of SCID/beige mice suggesting an important role for the capsule in virulence for mice.