[Update for the standard procedures of diagnosis and therapy in cases of congenital rubella]. / Aggiornamento delle procedure operative standard per la diagnosi e la cura dei casi di rosolia congenita.

Congenital Rubella is the dramatic consequence of rubella during gestation. A combined strategy of Measles and Rubella universal vaccination on children and selective vaccination of susceptible women has been shown effective in the elimination of congenital rubella requiring an incidence of < 1 case of CRS per 100,000 live births. Verification processes of rubella elimination require that physicians early and appropriately diagnose all cases of congenital rubella, including those unpatent at birth. The paper highlights clinical and laboratory aspects channeling diagnosis of congenital rubella infection or syndrome even after the first year of life, and the short- and long-term management criteria.

Severe Pulmonary Arteriopathy in a Neonate with Congenital Rubella Syndrome and Patent Ductus Arteriosus.

Neonates with congenital rubella syndrome (CRS) are known to have associated congenital cardiac malformations. Patent ductus arteriosus (PDA) is one the most common cardiac anomalies associated with CRS. PDA refractory to medical management and associated with ventilatory dependence is considered for surgical ligation. However, the management of PDA can be challenging in the presence of underlying lung disease or pulmonary vascular disease. Outcomes after closure in neonates are dependent upon age, weight, nutritional status, pre-operative pulmonary arterial hypertension and presence of chronic lung disease. We present a neonate with CRS who required surgical PDA closure. The neonate developed severe pulmonary arterial hypertension which led to fatal outcome. The clinical course is corroborated with histo-pathological changes observed on the autopsy of this neonate.

Perinatal viral infections.

Among the TORCH agents, the occurrence of rubella and human T-lymphotropic virus type 1 (HTLV-1) in Japan were studied. Rubella epidemics occurred throughout Japan from 1964 to 1969 and from 1975 to 1979. Low prevalences of CRS were observed in northeastern Japan, and high prevalences in southwestern Japan, with the highest in Okinawa. These conditions could be explained by the lower rate of rubella H1 antibody in the female population of southwestern Japan. Time of maternal rubella was in the gestational age interval from 26 to 57 days for cataract, from 25 to 62 days for heart disease and from 16 to 131 days for deafness. HTLV-1 is the causative agent of adult T-cell leukemia. Main route of transmission of this virus is mother-to-child transmission, through breast milk. Among the 311 mother-child pairs in Okinawa, 65 mothers (20.9%) and 10 children (3.2%) were seropositive for HTLV-1. Ten (15.4%) of the 65 seropositive mothers had seropositive children. These children had acquired their HTLV-1 antibodies by the age of 3 years. A significant difference existed between the prevalence rate of HTLV-1 antibodies in mothers and children.

Congenital infections and the nervous system.

Despite vaccines, new antimicrobials, and improved hygienic practices, congenital infections remain an important cause of death and long-term neurologic morbidity among infants world-wide. Important agents include Toxoplasma gondii, cytomegalovirus, Treponema pallidum, herpes simplex virus types 1 and 2, and rubella virus. In addition, several other agents, such as the varicella zoster virus, human parvovirus B19, and Borrelia burgdorferi, can potentially infect the fetus and cause adverse fetal outcomes. This article provides an overview of these infectious disorders and outlines current strategies for acute treatment and long-term management.

The use of contingent water misting in the treatment of self-choking.

A 25-year-old, deaf-blind, mentally retarded male was treated for chronic self-choking using water mist treatment and positive reinforcement. During pre-treatment baseline, self-chokes occurred at a rate of 2.09 per minute. During treatment sessions each self-choke was followed by water misting of the subject's face paired with a forceful "No!". Periods of 20 sec absent of self-chokes were positively reinforced with liquids and/or social contacts. Treatment procedures were generalized from the initial therapists, location, and absence of other clients and staff to other locations, and the presence of other clients and staff. Water mist treatment was associated with at least a 10-fold and as much as a 100-fold reduction of self-choking (i.e. to 0.02-0.20 self-chokes per min) across treatment and generalization phases. Eight-month follow-up observations showed that self-choke rates were at zero.

The ocular manifestations of congenital rubella syndrome.

Congenital Rubella affects the eye in many ways. Ocular effects can be found in the lens, retina, ciliary body and cornea. Ocular manifestations of the Congenital Rubella Syndrome are congenital cataracts, microphthalmos, pigmentary retinopathy, strabismus, and glaucoma. While surgical and medical treatment is utilized for these conditions, the need for prevention is considered critical. The responsibility of health care providers to educate the public remains the key.

Síndrome da rubéola congênita / Congenital rubella syndrome

A rubéola é uma doença viral, caracterizada como autolimitada e com evolução benigna,porém o fator de maior preocupação relacionado ao vírus da rubéola ocorre quando a infecção acomete gestantes, devido à capacidade que o vírus possui de causar infecção transpondo a barreira placentária podendo gerar a síndrome da rubéola congênita (SRC). Objetivo: O estudo pesquisou dados atualizados sobre a temática para advertir a população sobre os riscos relacionados à síndrome da rubéola congênita, além de contribuir com conhecimento científico de acadêmicos, profissionais da saúde e pesquisadores. Método: Para a elaboração do artigo de atualização, foi realizada uma revisão bibliográfica na ferramenta de busca Google Acadêmico, Sistema de Informação de Agravos de Notificação (SINAN), Ministério da Saúde e alguns livros, a fim de selecionar artigos e conteúdos disponíveis e atualizados para proporcionar maior compreensão do tema. Considerações Finais: O surgimento da vacina contra o vírus da rubéola foi uma grande conquista para a humanidade, uma vez que, após sua inclusão, houve uma redução significativa no número de casos novos de síndrome da rubéola congênita. Além disso, observou-se no decorrer da pesquisa uma grande dificuldade de encontrar trabalhos recém-publicados relacionados ao tema estudado

Rubella is viral disease, characterized as self-limiting and with a benign course, however the factor of greater concern related to rubella virus occurs when the infection affects pregnant women, due to the ability of the virus to cause infection by transposing the placental barrier, cangenerate the congenital rubella syndrome (CRS). Objective: The study searched update data on the subject to warn the population on related risks congenital rubella syndrome besides contributing with scientific knowledge of academics, health professionals and researchers. Method: For the elaboration of the update article, a bibliographic review was carried out in the Google Scholar search tool, the Notification of Injury Information System (SINAN), the Ministry of Health and some books, in order to select articles and contents available and update to provide understanding of the theme. Final considerations: The emergence of the rubella virus vaccine was great achievement for mankind, since after its inclusion there was a significant a reduction in the number of new cases of congenital rubella syndrome. In addition, it was observed during the research a great difficulty to find recently published papers related to the subject studied

Congenital rubella syndrome: seeking damages to be born. Ethical, medical and public health considerations.

A case of congenital rubella syndrome has been the reason to seek damages but a Civil Court of Rome sentenced against this and in favor of sued doctors. We discussed the high level of social attention and the feeling present in our western culture behind the request for damages. Legal considerations above the Italian abortion Law is provided to understand the framework of the court decision. Ethical, medical, and public health issues are commented and compared with the Perruche's case.

Costs of congenital rubella syndrome (CRS) in Oman: evidence based on long-term follow-up of 43 children.

As part of the national plan for elimination of rubella and congenital rubella syndrome (CRS), Oman established a national registry of CRS cases. As of May 2005, the registry included 43 surviving CRS cases, with a mean age of 11.9 years. Clinical examinations found that 84% had ocular defects, 84% had auditory/speech defects, 70% had neurological manifestations, and 42% had cardiac defects. Lifetime medical, special education, and rehabilitation costs were assessed. Using a discount rate of 3%, the average direct lifetime cost per surviving CRS patient was estimated at 18,644 US dollars. When including predicted lost productivity due to CRS, the average discounted direct and indirect lifetime costs per surviving CRS patient amounted to 98,734 US dollars.

Successful closure of the arterial duct in the setting of rubella syndrome.

A 9-year-old boy, with significant left-to-right shunting across a large duct in the context of rubella syndrome, was tested during catheterization to establish the feasability of occluding the duct with a device. The testing, including temporary closure of the duct and monitoring of pulmonary vascular reactivity to vasodilative substances, lead to the decision to implant an Amplatzer occluder. Sixteen months later, there was no residual shunting across the duct, and pulmonary arterial pressures had normalised. It remains unclear why the patient had not developed irreversible pulmonary vascular disease.

Tratamiento de la rubeola congénita activa con factor de transferencia / Treatment of active congenital german measter with transference factor

La rubeola congénita activa (RCA) es un padecimiento severo con un alto índice de mortalidad, para el que no hay un tratamiento específico. Nosotros presentamos el caso de un lactante con RCA cuya madre padeció rubeola durante el tercer trimestre del embarazo. El paciente presentaba ataque al estado general, hepatoesplenomegalia, anemia, trombocitopenia profunda y anticuerpos circulantes contra el virus de la rubeola. Con estos datos se hizo el diagnóstico de rubeola congénita activa. Dado el mal pronóstico de la enfermedad y basados en nuestra experiencia previa con Factor de Transferencia Inespecífico (FTI) en el tratamiento de varicela hemorrágica y herpes en pacientes inmunocomprometidos, decidimos utilizar FTI para el manejo de este enfermo, habiéndosele administrado 6 dosis. La respuesta de la trombocitopenia fue espectacular. La hepatoesplenomegalia también disminuyó después de cada dosis sin lograrse, sin embargo, una remisión completa por lo que decidimos administrarle Factor de Transferencia Específico (FTE) de la madre que fue seguido de una remisión contínua y permanente. Ocho meses después de la administración del FT el niño tenía desarrollo normal, y a la fecha 3 años después el niño evoluciona sin problemas y con desarrollo similar al de otros niños de su edad. Así pues, el FT parece eficaz en el tratamiento de rubeola congénita activa y amerita ser evaluado ampliamente.

Rubéola congénita / Congenital rubella

Se presenta el caso clínico de un recién nacido con rubéola congénita. Se revisó la literatura de esta patología, así como las lesiones cutáneas más frecuentes y los diagnósticos diferenciales ante la presencia de eritropoyesis cutánea