Language assessment of children with severe liver disease in a public service in Brazil

OBJECTIVE: The aim of this research was to compare language development (expressive and receptive skills) in children awaiting liver transplantation with that of children who have already undergone the surgical procedure. METHODS: An observational, descriptive, cross-sectional study was conducted with 76 children divided into groups, as follows: 31 children who were candidates for liver transplantation (Group 1; G1), 45 children who had already undergone liver transplantation (Group 2; G2), and a control group (CG) of 60 healthy, normally developing children. Health status information was gathered, and the Test of Early Language Development (TELD)-3 was used to assess language skills. Family household monthly income data were also gathered using a specific questionnaire. RESULTS: G1 had poorer language performance compared with G2 and the CG. G2 had lower language performance when compared with the CG. However, when considering the TELD-3 standard scores, G2 had scores within normal limits. The regression analysis indicated age as a risk factor for language deficits in Group 1 and family income as a risk factor for language deficits in G2. CONCLUSIONS: The results suggested that children with chronic liver disease have delays in language development. Transplanted children have linguistic performance within normal limits, but their scores tended to be lower than the CG.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program / Tratamento da síndrome do intestino encurtado na criança: valor do Programa de Reabilitação Intestinal

Summary The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Resumo A principal causa da falência intestinal aguda é a síndrome do intestino encurtado, decorrente, em geral, de ressecção de extensos segmentos de intestino delgado. Em consequência, os principais sintomas são diarreia aquosa, síndrome de má absorção, desnutrição crônica e óbito, caso o paciente não seja adequadamente tratado. Se o comprimento do intestino remanescente for superior a 30 cm, poderá haver adaptação completa e o paciente poderá ficar livre da nutrição parenteral. O tratamento atualmente preconizado inclui a utilização de nutrição parenteral prolongada e de nutrição enteral, objetivando sempre o ganho ponderal constante, em paralelo a cirurgias que visem ao alongamento do intestino dilatado. Esse conjunto de procedimentos constitui o que se denomina Programa de Reabilitação Intestinal. Essa terapia foi utilizada em 16 crianças, em períodos que variaram de 8 meses a 7 anos e meio, com sobrevida em 75% dos casos. O último recurso utilizado em crianças com ressecção completa do intestino delgado é o transplante intestinal. Até o momento, não há registro de criança brasileira que tenha sobrevivido a esse procedimento, a despeito de sete pacientes terem sido submetidos a ele. Os resultados do Programa de Reabilitação Intestinal nos anima a continuar com esse tipo de tratamento e estimular a criação do programa em outras instituições de atendimento pediátrico.

Prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation

OBJECTIVE: Chronic rejection remains a major cause of graft failure with indication for re-transplantation. The incidence of chronic rejection remains high in the pediatric population. Although several risk factors have been implicated in adults, the prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation are not known. Hence, the current study aimed to determine the factors involved in the progression or reversibility of pediatric chronic rejection by evaluating a series of chronic rejection cases following liver transplantation. METHODS: Chronic rejection cases were identified by performing liver biopsies on patients based on clinical suspicion. Treatment included maintaining high levels of tacrolimus and the introduction of mofetil mycophenolate. The children were divided into 2 groups: those with favorable outcomes and those with adverse outcomes. Multivariate analysis was performed to identify potential risk factors in these groups. RESULTS: Among 537 children subjected to liver transplantation, chronic rejection occurred in 29 patients (5.4%). In 10 patients (10/29, 34.5%), remission of chronic rejection was achieved with immunosuppression (favorable outcomes group). In the remaining 19 patients (19/29, 65.5%), rejection could not be controlled (adverse outcomes group) and resulted in re-transplantation (7 patients, 24.1%) or death (12 patients, 41.4%). Statistical analysis showed that the presence of ductopenia was associated with worse outcomes (risk ratio=2.08, p=0.01). CONCLUSION: The presence of ductopenia is associated with poor prognosis in pediatric patients with chronic graft rejection.

Experience with arteriovenous fistulas for chronic hemodialysis in children: technical details and refinements

OBJETIVO: Relatar a experiência dos autores na execução de fístulas artério-venosas em crianças, com a utilização dos recursos da microcirurgia vascular, com especial ênfase aos detalhes de técnica cirúrgica. MÉTODOS: Os pacientes foram operados entre julho de 1997 e março de 2004. Foram utilizadas lupas cirúrgicas (aumento de 3,5 vezes). Após a dissecção da veia e da artéria, a anastomose veno-arterial foi realizada de forma término-lateral, com quatro suturas contínuas de fio 7/0 ou 8/0. RESULTADOS: Vinte e nove crianças foram operadas para a realização de 33 fístulas - 21 rádio-cefálicas, 6 bráquio-cefálicas, 4 bráquio-basílicas e 2 safeno-femorais. Obteve-se permeabilidade em 17/21 (80,9%) fístulas rádio-cefálicas, 5/6 (83,3%) bráquio-cefálicas, 3/4 (75,0%) bráquio-basílicas e 2/2 (100%) safeno-femorais. 2 pacientes apresentaram obstrução da fístula e da artéria radial e 2 outras fístulas rádio-cefálicas obstruíram-se entre a primeira e segunda semana de pós-operatório. Tardiamente, verificou-se que as fístulas safeno-femorais causaram edema do membro inferior correspondente e houve formação de aneurisma em uma fístula bráquio-cefálica. Quanto à permeabilidade, verificou-se que as fístulas bráquio-cefálicas e bráquio-basílicas apresentaram a mesma incidência de permeabilidade em comparação com as fístulas rádio-cefálicas (p>0,05). CONCLUSÃO: A presente casuística demonstra que as fístulas artério-venosas persistem como um bom acesso vascular para hemodiálise em crianças. A utilização de microcirurgia e alguns detalhes técnicos aqui descritos permitem a obtenção de bons índices de permeabilidade das fístulas.

Validacao de questionario para diagnostico de cefaleia em ambulatorio de complexidade terciaria / Validation of a questionnaire to the headache diagnosis in ambulatory setting

A cefaleia e um sintoma de alta prevalencia na populacao, sendo queixa frequente na pratica clinica. Cursa geralmente com exame fisico e neurologico normais. A triagem de pacientes com cefaleia facilitaria o atendimento em grandes centros medicos. O objetivo deste trabalho e verificar a validade do questionario como metodo de triagem de cefaleias primarias. Aplicou-se o questionario a 204 pacientes do Ambulatorio Geral e Didatico do HCFMUSP, sendo a metade submetida a consulta medica...