Congenital diaphragmatic defects that present late.

Twenty two patients (age range: 1 month to 11 years) were treated for congenital diaphragmatic defects (excluding hiatus hernia) in the six year period 1983-8. Presenting features were failure to thrive (n = 7), abdominal pain and vomiting (n = 4), chronic respiratory symptoms (n = 3), and inability to wean from ventilatory support (n = 3). The defect was an incidental finding in five patients. Operative repair was performed with no mortality or serious postoperative morbidity. Dramatic improvement occurred in 15 of the 17 symptomatic patients. Awareness of the differential diagnosis should avoid delay in diagnosis or inappropriate treatment. Surgical correction is strongly recommended in all cases.     

Results of Salmonella typhi culture in patients with suspected typhoid fever, treated in the Department of Child Health Medical School, Padjadjaran University, Hasan Sadikin General Hospital Bandung

From October to December 1984, examination has been carried out on Salmonella-typhi cultures from blood, bone marrow, faeces and urine of 43 patients suffering from suspected typhoid fever treated in the Department of Child Health, Medical School/Hasan Sadikin General Hospital, Bandung. It was apparent that among these 43 patients, 51.2% were males and 48.8% females. The greater number of them were more than 5 years old (64.4%). The results of Salmonella-typhi cultures were obtained from 65% of the patients, consisting of 65% positive cultures from bone-marrow and 40% positive cultures from blood that showed a statistically significant difference (p less than 0.05). Likewise, the time for matter-sampling for the examinations was based on the culture results, both on blood-culture and that of bone-marrow, statistically a significant was found (p less than 0.05), whereas the results of cultures based on previous vaccination history and the administration of chloramphenicol prior to treatment did not differ significantly. It was obviously clear that the results of bone-marrow culture were more successful compared to those from blood culture, so that it should necessarily be emphasized, as to these examinations on culture from each patient with suspected typhoid fever, especially those patients hospitalized during the third week of their illness.     

Neonatal vocal cord paralysis following extracorporeal membrane oxygenation

Five cases of unilateral vocal cord paralysis/paresis were diagnosed following extracorporeal membrane oxygenation for newborn respiratory failure. All were right sided and transient in nature. None of the five patients had other findings commonly associated with vocal cord palsy. The extracorporeal membrane oxygenation procedure requires surgical dissection in the carotid sheath on the right side of the neck, an area immediately adjacent to both the vagus and recurrent laryngeal nerve. It is speculated that vocal cord paralysis in these infants was acquired as a result of the extracorporeal membrane oxygenation cannulation. Although the vocal cord paralysis resolved in all cases, two patients had difficult courses after extracorporeal membrane oxygenation. Therefore, laryngoscopic examination should be considered for patients after extracorporeal membrane oxygenation.     

Acute infectious epiglottitis in children and adults: annual incidence and mortality

The incidence and mortality of acute epiglottitis were retrospectively analysed covering a 12-year period, 1975-1987. The diagnosis was made by the laryngoscopic findings in 95% of patients. Based on 902 hospitalized patients, a mean annual incidence of 4.9 cases per 100,000 children and adults was found. Although adult patients predominated (60%), the incidence was far higher in children, 13.8 per 100,000 compared with 3.9 per 100,000 in adults. Two peaks in incidence were identified, one for the youngest children, and the second for young adults (15-29 years). Over the years the incidence varied little in adults, but decreased significantly in children over the last 6 years studied. The data presented indicate acute epiglottitis to be a more frequent disorder than previously believed but with less risk of a fatal outcome. The mortality rate was below 1% in children and adults and the annual incidence of death from acute epiglottitis was estimated at 0.5 cases per million. The primary admission of these patients to ENT units seems to be the single factor having the most powerful impact on mortality.     

Transplantability of human head and neck squamous cell carcinomas in athymic nude mice

Tumor material from 91 patients with squamous cell carcinoma of the head and neck was transplanted subcutaneously in athymic nude mice. In the first (man to mouse) passage, the calculated mean probability of tumor take in a single mouse was 11%. The probability of growth in the first passage was significantly better for moderately and poorly differentiated tumors than for well-differentiated tumors. Also, the implantation of lymph node material resulted in a significantly better tumor take rate than material taken from a primary tumor. Transplantability was not dependent on the following characteristics: localization, T or N stage of the tumor, or the sex of the patients. Once growth was established, all variables studied had no influence on the probability of growth in the subsequent mouse passages. A relationship between tumor growth in nude mice and patient prognosis could not be found. When transplanting head and neck squamous cell carcinoma in nude mice, it has to be recognized that some tumor characteristics will influence the success of tumor growth.     

Risk of infectious complications in well-appearing children with transient neutropenia

To determine whether well-appearing children found incidentally to be neutropenic are at risk for an infectious complication, 44 consecutive months of hematology laboratory records were reviewed. One hundred nineteen patients had medical record documentation regarding clinical course, serial white blood cell counts, and the absence of serious infections, chronic illnesses, or a family history known to be associated with neutropenia. The median duration of documented neutropenia was 13 days (range, 1 to 491 days). Infectious complications occurred in 4 of the 36 patients who had neutropenia for more than 30 days (2 with stomatitis, 1 with cellulitis, and 1 with pneumonia) but in none with shorter durations of neutropenia. There were no significant associations between the development of an infectious complication and either the initial absolute neutrophil count or the lowest documented absolute neutrophil count, nor was there a correlation between the initial absolute neutrophil count and the duration of neutropenia. These data indicate that infectious complications occur in otherwise well children with unexplained neutropenia that persists, but these infections are infrequent and usually are superficial.     

Usher syndrome: clinical findings and gene localization studies

The issue of genetic heterogeneity is a critical problem in the localization of the gene(s) for Usher syndrome. Based on the data obtained on families studied to date, the differences between type I and type II Usher syndrome appear quite distinct with regard to auditory and vestibular function. Although the majority of families can be confidently diagnosed as typical type I or type II, clinical investigations revealed four families with findings that did not fit into either of the two more common subtypes. These findings emphasize the critical importance of an in-depth clinical analysis concomitant with the linkage investigation to assure accurate subtyping of Usher syndrome. Based on an analysis of only those families with definite type I or type II Usher syndrome, approximately 17% of the genome can be excluded as a potential site of the gene for type I, and 14% can be excluded as the site for the type II gene. This study will continue until the Usher gene(s) is successfully localized.