Single institution outcomes of treatment of severe aplastic anaemia

Background: In severe aplastic anaemia, the treatment of choice for young patients with a human leucocyte antigen-matched sibling is now established as allogeneic bone marrow transplantation (BMT). In older patients and in those without a matched sibling donor, immunosuppressive therapy is the usual first option. 'Alternative' marrow donors are emerging as an option for those without a matched sibling donor. Aims: To review 10 years of local experience in treating severe aplastic anaemia with BMT and immunosuppressive therapy with emphasis on long-term outcomes. Methods: A retrospective analysis was performed of all patients with severe aplastic anaemia presenting to the Royal Brisbane and Royal Children's Hos- pitals between 1989 and 1999. Data were abstracted regarding patient demographics, pretreatment characteristics and outcome measures, including response rates, overall survival and long-term complications. Results: Twenty-seven consecutive patients were identified, 12 treated with immunosuppression alone and 15 with BMT. In these two groups, transfusion independence was attained in 25% and 100%, respectively, with overall survival being 36% and 100%, respectively. Those treated with immunosuppression were significantly older (median 41.5 versus 22 years, P = 0.008). Long-term survivors of either treatment had extremely low morbidity. Three patients carried pregnancies to term post-transplant. Three patients received alternative donor BMT with correspondingly excellent survival. Conclusions: Patients treated with allogeneic BMT for severe aplastic anaemia enjoyed extremely good long-term survival and minimal morbidity. Patients treated with immunosuppressive therapy had a poorer outcome reflecting their older age and different usage of therapies over the past decade. Optimal treatment strategies for severe aplastic anaemia remain to be determined.

Pediculus humanus capitis infestations in the community: A pilot study into transmission, treatment and factors affecting control

Head lice (Pediculus humanus capitis) infestations affect schoolchildren worldwide, creating social, economic and health consequences for families. Problems with self-detection, chronic infestations and classroom transmission are compounded by increasing resistance of the lice to pediculicides. Public health strategies are based on limited research and little is known about transmission dynamics. Mismanagement and transmission in the general community are blamed for control failure. The purpose of this study was to explore community head-lice experience in Brisbane, Australia, and to identify critical factors underlying control failure. A home-based pilot survey used physical examination to verify transmission and treatment patterns which were self-reported by a group of trace-contact families in addition to other unconnected participants. The survey was enlarged to further compare therapy outcomes and suspected risk factors. The findings reinforce those of previous studies - that children attending school and early childhood centres, and subsequently their families, are most at risk of contracting pediculosis capitis, and some may carry lice for years. First-line (pediculicidal) treatment and even additional physical methods of hand-picking and fine-toothed combing usually fail to eradicate lice quickly and completely (overall cure-rate 39 per cent, n = 84 cases). Failures were linked to hair characteristics. Public education alone may not control pediculosis. Accurate diagnosis requires considerable experience; a strong case exists for returning to institutional surveillance.

Motorcycle exhaust burns in children

Objectives: To document and describe motorbike exhaust burns on children. Design, Patients and Setting: Departmental database and case note review of all children with motorbike exhaust burns seen at the Stuart Pegg Paediatric Burns Centre, Brisbane between January 1996 and October 2001. Main outcome measures: Number and age of children burned, circumstances of the injury, burns sustained, treatment required and long-term sequelae. Results: Twenty-four children, median age 8 years, sustained thermal burns, most commonly to the right lower leg. Thirteen children required surgery, and 17 required chronic scar management. Conclusions: We have identified motorbike exhausts as a cause of burns in children. The injuries received resulted in significant morbidity to these children and warrants a campaign airned at reducing the incidence of such injuries. (C) 2002 Published by Elsevier Science Ltd. and ISBI.

The effectiveness of splinting as perceived by the parents of children with Juvenile Ideopathic Arthritis

Juvenile idiopathic arthritis (JIA) is an important disease of childhood with farreaching effects on the child and family. Splinting is a major treatment modality used by occupational therapists for children with JIA. Parents play a central role in whether, when and how splints are used with their children on a daily basis. This paper describes a qualitative research project, which was undertaken to evaluate an occupational therapy service for children with JIA whose treatment had involved splinting. Using semi-structured interviews, the study investigated five mothers' perceptions of the effectiveness of splinting for their children. The interviews revealed five major points. First, the informants generally believed the splinting to be effective. Secondly, the children involved generally resisted wearing splints because they were physically uncomfortable and made them feel different to other children. Thirdly, the mothers used a variety of strategies to ensure that their children wore the splints. Fourthly, the perception of having a positive and supportive relationship with the therapist enhanced the mothers' ability to adhere to splinting. Lastly, the mothers' grief at having a child with JIA influenced their ability to understand and attend to information about specific interventions such as splinting. Practical responses to these findings are outlined.

Diagnosis and management of unusual dental abscesses in children

Although the majority of dental abscesses in children originate from dental caries or trauma, a few are associated with unusual conditions which challenge diagnosis and management. Recent research findings have shed light on these unusual entities and greatly improved understanding of their clinical implications. These conditions include developmental abnormalities such as dens invaginatus in which there is an invagination of dental tissues into the pulp chamber and dens evaginatus in which a tubercle containing pulp is found on the external surface of a tooth crown. In addition, inherited conditions which show abnormal dentine such as dentine dysplasia, dentinogenesis imperfecta, and osteogenesis imperfecta predispose the dentition to abscess formation. Furthermore, 'spontaneous' dental abscesses are frequently encountered in familial hypophosphataemia, also known as vitamin D-resistant rickets, in which there is hypomineralization of dentine and enlargement of the pulp. In addition to developmental conditions, there are also acquired conditions which may cause unusual dental abscesses,. These include pre-eruptive intracoronal resorption which was previously known as 'pre-eruptive caries' or the 'fluoride bomb'. In addition, some undiagnosed infections associated with developing teeth are now thought to be the mandibular infected buccal cysts which originate from infection of the developing dental follicles. In the present paper, these relatively unknown entities Which cause unusual abscesses in children are reviewed with the aim of updating the general practitioner in their diagnosis and management.

Dysarthria and dysphagia following treatment for a fourth ventricle choroid plexus papilloma

The present case report describes the presence of a persistent dysarthria and dysphagia as a consequence of surgical intervention for a choroid plexus papilloma (CPP). WM was a nine year ten month old male who at the time of the present study was seven years post-surgery. A comprehensive perceptual and instrumental test battery was used to document the nature of the dysarthria incorporating all components of speech production including respiration, phonation, resonance, articulation, and prosody. The nature of the dysphagia was evaluated through the use of videofluoroscopic evaluation of swallowing (VFS). Assessments confirmed the presence of a LMN dysarthria, marked by deficits in phonation, respiration, and prosody. Dysphagia assessment revealed deficits in oral preparatory, oral and pharyngeal stages of the swallow. The presence of persistent dysarthria and dysphagia in this case has a number of important implications for the management of children undergoing surgery for fourth ventricle CPPs, in particular the need for appropriate treatment, as well as counselling prior to surgery of the possible negative outcomes related to speech and swallowing. (C) 2003 Elsevier Science Ltd. All rights reserved.

EMA analysis of tongue function in children with dysarthria following traumatic brain injury

Primary objective : To investigate the speed and accuracy of tongue movements exhibited by a sample of children with dysarthria following severe traumatic brain injury (TBI) during speech using electromagnetic articulography (EMA). Methods and procedures : Four children, aged between 12.75-17.17 years with dysarthria following TBI, were assessed using the AG-100 electromagnetic articulography system (Carstens Medizinelektronik). The movement trajectories of receiver coils affixed to each child's tongue were examined during consonant productions, together with a range of quantitative kinematic parameters. The children's results were individually compared against the mean values obtained by a group of eight control children (mean age of 14.67 years, SD 1.60). Main outcomes and results : All four TBI children were perceived to exhibit reduced rates of speech and increased word durations. Objective EMA analysis revealed that two of the TBI children exhibited significantly longer consonant durations compared to the control group, resulting from different underlying mechanisms relating to speed generation capabilities and distances travelled. The other two TBI children did not exhibit increased initial consonant movement durations, suggesting that the vowels and/or final consonants may have been contributing to the increased word durations. Conclusions and clinical implications : The finding of different underlying articulatory kinematic profiles has important implications for the treatment of speech rate disturbances in children with dysarthria following TBI.

Parental predictors of poor visual outcome with occlusion treatment for unilateral amblyopia

Aim: Visual acuity outcome of amblyopia treatment depends on the compliance. This study aimed to determine parental predictors of poor visual outcome with occlusion treatment in unilateral amblyopia and identify the relationship between occlusion recommendations and the patient's actual dose of occlusion reported by the parents. Methods: This study comprised three phases: refractive adaptation for a period of 18 weeks after spectacle correction; occlusion of 3 to 6 hours per day during a period of 6 months; questionnaire administration and completion by parents. Visual acuity as assessed using the Sheridan-Gardiner singles or Snellen acuity chart was used as a measure of visual outcome. Correlation analysis was used to describe the strength and direction of two variables: prescribed occlusion reported by the doctor and actual dose reported by parents. A logistic binary model was adjusted using the following variables: severity, vulnerability, self-efficacy, behaviour intentions, perceived efficacy and treatment barriers, parents' and childrens' age, and parents' level of education. Results: The study included 100 parents (mean age 38.9 years, SD approx 9.2) of 100 children (mean age 6.3 years, SD approx 2.4) with amblyopia. Twenty-eight percent of children had no improvement in visual acuity. The results showed a positive mild correlation (kappa = 0.54) between the prescribed occlusion and actual dose reported by parents. Three predictors for poor visual outcome with occlusion were identified: parents' level of education (OR = 9.28; 95%CI 1.32-65.41); treatment barriers (OR = 2.75; 95%CI 1.22-6.20); interaction between severity and vulnerability (OR = 3.64; 95%CI 1.21-10.93). Severity (OR = 0.07; 95%CI 0.00-0.72) and vulnerability (OR = 0.06; 95%CI 0.05-0.74) when considered in isolation were identified as protective factors. Conclusions: Parents frequently do not use the correct dosage of occlusion as recommended. Parents' educational level and awareness of treatment barriers were predictors of poor visual outcome. Lower levels of education represented a 9-times higher risk of having a poor visual outcome with occlusion treatment.

Assessment of a strategy for the control of respiratory diseases in children

A programme for the control of respiratory diseases in children was conceived for the State of S. Paulo, Brazil, in 1986. Its progress thereafter and the epidemiology of the diseases concerned are examined. Apart from an inquiry into the 64 existing State local health authorities, a sample of 18,255 cases of children assisted by the programme at different levels, including both in-patient and outpatient care, is analysed. Each case record included information about identification (child, doctor and health facility), reasons for calling, diagnoses made and outcome of treatment. Further data were also sought from hospitals and from State mortality records. The programme was found to be poorly implemented in the State but, where implemented, it showed itself capable of resolving problems (only 0.5% of the cases could not be handled) as also of changing ongoing trends (more than 50% reduction in hospital admission rates). Individual assessment of each item of the programme indicated its bottlenecks. Regarding the epidemiology of respiratory diseases, it is observed that the major burden to health services comes from children aged less than five, and that the most important diseases are wheezing illnesses and pneumonia. Morevoer, they were found to be significantly associated (p = 0.000) so that a child in the community presenting wheezing diseases is 5 times more likely to develop pneumonia than a child with any other respiratory diagnosis. Similarly, among the under five deaths it was found that the risk for pneumonia is 3 times greater for children who died presenting wheezing diseases than it is for children with any other sort of diagnosis. In conclusion, the programme is deemed to be efficient and effective but its efficacy is marred by administrative flaws. The successful control of respiratory problems in childhood is related to a proper appreciation of the importance of wheezing diseases.

Glucose-6-Phosphate dehydrogenase deficiency in children from 0 to 14 years hospitalized at the Pediatric Hospital David Bernardino, Luanda, Angola

The Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymatic defect in the world. The most common clinical manifestations are acute hemolytic anemia associated with drugs, infections, neonatal jaundice and hemolytic non-spherocytic chronic anemia. The main aim of this study was to determine the frequency of major genetic variants of G6PD leading to enzyme deficiency in children from 0 to 14 years at a Pediatric Hospital in Luanda, Angola. A cross-sectional and descriptive analytical study covered a total of 194 children aged from 0 to 14 years, of both genders and hospitalized at the Pediatric Hospital David Bernardino, Luanda between November and December, 2011. The G202A, A376G and C563T mutations of the G6PD gene were determined by real-time PCR with Taqman probes. The disabled A-/A- genotype was detected in 10 girls (10.9%). Among the boys, 21 (20.6%) presented the genotype A-. Considering all the samples, the A- variant was observed in 22.4% of cases. The Mediterranean mutation was not detected in the Angolan sample. Furthermore, no association was found between genotype and anemia, nutritional state and mucosa color. A significant association, however, was observed with jaundice. Based on the results obtained, there is a clear need to identify those with the disabled genotype in the Angolan population in order to avoid cases of drug-induced anemia, particularly in the treatment of malaria, so prevalent in Angola.

Tuberculosis in indigenous children in the Brazilian Amazon

OBJECTIVE: Assess the epidemiological aspects of tuberculosis in Brazilian indigenous children and actions to control it. METHODS: An epidemiological study was performed with 356 children from 0 to 14 years of age in Rondônia State, Amazon, Brazil, during the period 1997-2006. Cases of TB reported to the Notifiable Diseases Surveillance System were divided into indigenous and non-indigenous categories and analyzed according to sex, age group, place of residence, clinical form, diagnostic tests and treatment outcome. A descriptive analysis of cases and hypothesis test (χ²) was carried out to verify if there were differences in the proportions of illness between the groups investigated. RESULTS: A total of 356 TB cases were identified (125 indigenous, 231 non-indigenous) of which 51.4% of the cases were in males. In the indigenous group, 60.8% of the cases presented in children aged 0-4 years old. The incidence mean was much higher among indigenous; in 2001, 1,047.9 cases/100,000 inhabitants were reported in children aged < 5 years. Pulmonary TB was reported in more than 80% of the cases, and in both groups over 70% of the cases were cured. Cultures and histopathological exams were performed on only 10% of the patients. There were 3 cases of TB/HIV co-infection in the non-indigenous group and none in the indigenous group. The case detection rate was classified as insufficient or fair in more than 80% of the indigenous population notifications, revealing that most of the diagnoses were performed based on chest x-ray. CONCLUSIONS: The approach used in this study proved useful in demonstrating inequalities in health between indigenous and non-indigenous populations and was superior to the conventional analyses performed by the surveillance services, drawing attention to the need to improve childhood TB diagnosis among the indigenous population.

Reinfection after treatment of schistosomiasis: environment or "predisposition"?

Although very efficient for the control of morbidity due to S. mansoni in individual patients, chemotherapy has not proven successful in the management of transmission within hyperendemic areas when used alone, even if repeated at short intervals. Consequently, a great deal of effort has been expended toward immunologic investigation and development of a specific vaccine. Based upon a study of a group of children (5-14 years) from the state of Alagoas, the author demonstrates that the outcome one year after chemotherapy depends essentially on the "risk rating" of the area of domicile. A regression analysis did not reveal significant correlation to neither age, sex or initial egg counts. Although the study was not designed to reveal individual variations in the immune status, it is postulated that putative differences in genetic make-up are irrelevant in terms of large-scale intervention. Since morbidity due to S. mansoni has substantially declined during the last two or three decades, a control policy based on vaccination can only be justified if high levels of protective immunity can be attained. At any rate, such a vaccine will have to be administered in early childhood (preferably below the age of three). It can also be demonstrated that immunization in adolescence or adulthood serves no purpose whatsoever. The author is convinced that environmental intervention, usually dismissed as unrealistic in terms of the developing countries, is not only feasible, if done on a selective basis, but prioritary.

Aspergillosis in immunocompromised children acute myeloid leukemia and bone marrow aplasia.: Report of two cases

Two cases of Aspergillosis in immunocompromised children are reported. Both were caused by Aspergillns flavus. Early diagnosis and treatment led to the remission of the process. One patient had acute myeloid leukemia; the fungus was isolated from the blood. The other patient with bone marrow aplasia, presented an invasive aspergillosis of the paranasal sinuses with dissemination of fungal infection; the diagnosis was obtained by histology and culture of biopsied tissue from a palatal ulceration.

Ascariasis in the subdistrict of Cavacos, municipality of Alterosa (MG), Brazil: effect of mass treatment with albendazole on the intensity of infection

The clinical and epidemiologic aspects of infection with Ascaris lumbricoides were studied in a random stratified sample of the population of the subdistrict of Cavacos, municipality of Alterosa (Minas Gerais, Brazil). The effect of mass treatment with a single dose of albendazole on the prevalence and intensity of infection was also studied six months later in the same population. During the first phase of the study, a questionnaire was applied to 248 individuals to obtain information about the socioeconomic, sanitary and clinical conditions of the population surveyed. A total of 230 fecal samples were also examined by the Kato-Katz technique in order to determine the intensity of A. lumbricoides infection. Two hundred and two individuals were simultaneously submitted to blood counts and 70 children aged 12 years or less were evaluated for nutritional status. The presence of A. lumbricoides and other helminth eggs was also determined in 22 soil samples collected in the urban zone of Cavacos. Infection with enteroparasitic helminths was detected in 29.1% of the sample, with a predominance of A. lumbricoides (23.9%). Parasitism and/or intensity of A. lumbricoides infection were significantly correlated with age range (15 years or less), social class, sanitary and living conditions (water, sewage and domiciliary area per person), and presence of abdominal pain. However, these parameters were not correlated with nutritional status or hematocrit levels. During the second phase of the study, a slight but not statistically significant decrease in the prevalence of A. lumbricoides infection was detected after treatment with albendazole. However, an important and significant reduction in the amount of A. lumbricoides eggs eliminated through the feces was detected, indicating that the intensity of A. lumbricoides infection was lower in all the age ranges of the Cavacos population, especially among younger individuals, even six months after administration of the anthelminthic agent.

EVALUATION OF A RAPID SCREENING ASSAY FOR BACTERIAL IDENTIFICATION (DOT-ELISA) IN FECAL SAMPLES FROM CHILDREN

With the objective of standardizing a Dot Enzyme-Linked Immunosorbent Assay (Dot-ELISA) to detect antigens of fecal bacterial enteropathogens, 250 children, aged under 36 months and of both sexes, were studied; of which 162 had acute gastroenteritis. The efficacy of a rapid screening assay for bacterial enteropathogens (enteropathogenic Escherichia coli "EPEC", enteroinvasive Escherichia coli "EIEC", Salmonella spp. and Shigella spp.) was evaluated. The fecal samples were also submitted to a traditional method of stool culture for comparison. The concordance index between the two techniques, calculated using the Kappa (k) index for the above mentioned bacterial strains was 0.8859, 0.9055, 0.7932 and 0.7829 respectively. These values express an almost perfect degree of concordance for the first two and substantial concordance for the latter two, thus enabling this technique to be applied in the early diagnosis of diarrhea in infants. With a view to increasing the sensitivity and specificity of this immunological test, a study was made of the antigenic preparations obtained from two types of treatment: 1) deproteinization by heating; 2) precipitation and concentration of the lipopolysaccharide antigen (LPS) using an ethanol-acetone solution, which was then heated in the presence of sodium EDTA