Association between liver transplantation for Langerhans cell histiocytosis, rejection, and development of posttransplant lymphoproliferative disease in children.

OBJECTIVE: Langerhans cell histiocytosis (LCH) is an unusual indication for orthotopic liver transplantation in children. Data from limited case reports suggest that orthotopic liver transplantation for LCH is associated with excellent survival rates and a low incidence of disease recurrence. However, in our experience, children who have transplantation for LCH appeared to experience a high incidence of refractory rejection and posttransplant lymphoproliferative disease (PTLD). STUDY DESIGN: Data from 398 liver transplants performed in 298 children younger than 16 years of age were reviewed to determine the presence of risk factors for PTLD in patients with LCH and other causes of liver failure. RESULTS: The incidence of PTLD was significantly higher in children who received transplants for LCH compared with all indications (p < 0.001) and specific indications that were associated with the development of PTLD (p < 0.002). Among patients in whom PTLD developed, there was no significant difference in the incidence of primary Epstein-Barr virus infections in patients who receive transplantation for LCH (4/4, 100%) versus all other indications (12/14, 86%). Children who had transplantation for LCH were older than those who had transplantation for other indications (LCH median age 3.1 years, other indications 1 year). The incidence of rejection, especially refractory rejection, was greater in patients who had transplantation for LCH (100% and 50%, respectively) compared with those who had transplantation for other indications (70% and 10%, p < 0.02 for refractory rejection). CONCLUSIONS: Patients who had transplantation for liver disease related to LCH experienced a 67% long-term survival (median follow up 5.8 years, range 2.1 to 7.5 years). Recurrent LCH occurred in only 33% of patients and was easily managed. However, PTLD developed in two thirds of these patients, perhaps in part because of the high incidence of refractory rejection. This series therefore demonstrates an association between a primary disease process and the development of PTLD. Although the data indicate that children with LCH-induced liver failure benefit from transplantation, special care must be exercised in screening for and preemptive treatment of PTLD.

Practical screening priorities for hearing impairment among children in developing countries.

Routine screening for hearing impairment in childhood is now widespread in industrial countries, although there is considerable controversy over the most efficient techniques and procedures. In most developing countries, however, routine screening programmes for hearing impairment do not currently exist. The problems involved in implementing screening programmes in developing and industrial countries are very different, and in selecting screening procedures for a particular population the following factors have to be taken into consideration: the environmental test conditions; the availability of resources for equipment and the training of testers; the local attitudes towards disability; the level of hearing impairment that may cause handicaps; and the major types of pathology causing hearing impairment. We suggest that in developing countries children should be screened at school entry using a simple field audiometer and that the external ear be inspected for the presence of a discharge. There is an urgent need to develop reliable and simple screening procedures for infants and young children; where possible, all children should be screened for severe or significant hearing impairment before the age of 2 years. No screening should, however, be implemented until appropriate follow-up services are available.

PIP: Routine screening for hearing impairment in childhood is now widespread in industrial countries, although there is considerable controversy over the most efficient techniques and procedures. In most developing countries, however, routine screening programs for hearing impairment do not currently exist. The problems involved in implementing screening programs in developing and industrial countries are very different, and in selecting screening procedures for a particular population the following factors have to be taken into consideration: the environmental test conditions; the availability of resources for equipment and the training of testers; the local attitudes towards disability; the level of hearing impairment that may cause handicaps; and the major types of pathology causing hearing impairment. The author suggest that in developing countries, children should be screened at school entry using a simple field audiometer and that the external ear be inspected for the presence of a discharge. There is an urgent need to develop reliable and simple screening procedures for infants and young children; where possible, all children should be screened for severe or significant hearing impairment before the age of 2 years. No screening should, however, be implemented until appropriate follow-up services are available. (author's)

Diarrheal diseases of infancy in Cali, Colombia: study design and summary report on isolated disease agents.

For public health reasons, it is important that the etiologic agents of early childhood diarrhea be isolated and identified, and that their routes of transmission be defined. This is especially true in tropical and subtropical developing countries, where childhood patterns of exposure to diarrheal disease agents usually differ from those in developed countries, and where diarrheal illness is a frequent harbinger of death among children under five years of age. This artical describes a study designed to identify diarrheal disease agents and transmission patterns in Cali, a large city of western Colombia's fertile Cauca River Valley. The study area, composed of five working-class districts with a total population of some 40,000, appeared to provide an environment fairly similar to those of many other "average" working-class communities in Latin America. Beginning in July 1962, a cohort of 296 children being born in these districts was studied, the period of investigation starting with the date of birth and continuing until each child's second birthday or its premature withdrawal from the study. Weekly home visits were made to establish defecation patterns, feeding practices, and anthropometry. The resulting data were then analyzed in terms of defecation frequencies, occurrence of liquid stools, and the presence of blood, mucus, or pus in the stools. Differences were noted in male and female defecation patterns and in the defecation frequencies of different age groups. Stool specimens for bacteriologic, virologic, and parasitologic examination were collected monthly on a regular basis and weekly when diarrhea occurred. Numerically, viruses were isolated and identified more often than other agents. The most commonly isolated parasite species and viral and bacterial serotypes were G. lamblia (from 222 subjects), echovirus 11 (from 166 subjects), and enteropathogenic Escherichia coli 026:B6 (from 138 subjects). Compared with the findings of several studies in other countries, isolations of shigellae were relatively rare.

The serological assessment of a tetanus toxoid field trial.

To determine the effectiveness of a method for controlling tetanus neonatorum, a double-blind controlled trial involving 1 618 women was conducted between 1961 and 1966. Women in the study were given 1-3 injections (1 ml) of aluminium-phosphate-adsorbed tetanus toxoid or a placebo (influenza vaccine). At the conclusion of the trial, 5-ml samples of blood were obtained from 299 women. Sera were titrated for anti-tetanus antibodies by two methods.A comparison of the clinical and laboratory results showed a close relationship. It is suggested that the level of protection may be lower than is at present accepted. Antitoxin levels were inversely related to age and directly to the interval between injections. Two widely spaced injections (8 months or more) may be about as effective as 3 injections. One injection of specially prepared toxoid with a high immunizing potency might give significant protection.

The use of toxoid for the prevention of tetanus neonatorum. Final report of a double-blind controlled field trial.

With a view to determining the effectiveness of a method for the control of tetanus neonatorum which would be independent of medical examination or care, a double-blind field trial covering 1618 women was conducted between 1961 and 1966 in a rural area of Colombia with an estimated existing tetanus neonatorum death rate of 11.6 per 100 births. The study group was given 1-3 injections of 1 ml of an aluminium-phosphate-adsorbed tetanus toxoid more than 6 weeks apart, and the control group a similar number of injections of an influenza-virus vaccine.There was no statistically significant difference between those in the two groups given one injection. Those in the control group given 2 or 3 injections had a tetanus neonatorum death rate of 7.8 deaths per 100 births, and the corresponding subjects in the study group had none. This difference is unlikely to have occurred by chance.