Bronchopulmonary dysplasia: temporal trend from 2010 to 2019 in the Brazilian Network on Neonatal Research.

OBJECTIVE: To evaluate the temporal trend of bronchopulmonary dysplasia (BPD) in preterm infants who survived to at least 36 weeks' post-menstrual age (PMA) and BPD or death at 36 weeks' PMA, and to analyse variables associated with both outcomes. DESIGN: Retrospective cohort with data retrieved from an ongoing national registry. SETTING: 19 Brazilian university public hospitals. PATIENTS: Infants born between 2010 and 2019 with 23-31 weeks and birth weight 400-1499 g. MAIN OUTCOME MEASURES: Temporal trend was evaluated by Prais-Winsten model and variables associated with BPD in survivors or BPD or death were analysed by logistic regression. RESULTS: Of the 11 128 included infants, BPD in survivors occurred in 22%, being constant over time (annual per cent change (APC): -0.80%; 95% CI: -2.59%; 1.03%) and BPD or death in 45%, decreasing over time (APC: -1.05%; 95% CI: -1.67%; -0.43%). Being male, small for gestational age, presenting with respiratory distress syndrome, air leaks, needing longer duration of mechanical ventilation, presenting with treated patent ductus arteriosus and late-onset sepsis were associated with an increase in the chance of BPD. For the outcome BPD or death, maternal bleeding, multiple gestation, 5-minute Apgar <7, late-onset sepsis, necrotising enterocolitis and intraventricular haemorrhage were added to the variables reported above as increasing the chance of the outcome. CONCLUSION: The frequency of BPD in survivors was constant and BPD or death decreased by 1.05% at each study year. These results show some improvement in perinatal care in Brazilian units which resulted in a reduction of BPD or death, but further improvements are still needed to reduce BPD in survivors.

Monitoramento eletrofisiológico do sistema auditivo central em crianças nascidas pequenas para a idade gestacional / Electrophysiological follow-up of the central auditory system in children born small for gestational age

RESUMO Objetivo Monitorar o sistema auditivo central de crianças nascidas pequenas para a idade gestacional, por meio da avaliação eletrofisiológica da audição, para verificar a ocorrência de eventuais disfunções neurais nesse sistema. Métodos Estudo longitudinal, cuja casuística foi composta por 23 crianças distribuídas em quatro grupos: 1) grupo de quatro crianças nascidas pequenas para a idade gestacional e a termo; 2) grupo de sete crianças nascidas pequenas para a idade gestacional e pré-termo; 3) grupo de quatro crianças nascidas com peso adequado para a idade gestacional e a termo; 4) grupo de oito crianças nascidas adequadas para a idade gestacional e pré-termo, cuja idade ao final da pesquisa foi de 3 anos (variação entre 34 e 39 meses). O critério de inclusão foi presença bilateral de emissões otoacústicas transientes. Todas as crianças foram submetidas ao potencial evocado auditivo de tronco encefálico ao nascimento, aos 6 meses e aos 3 anos de idade e à pesquisa do potencial evocado auditivo de longa latência aos 3 anos. Resultados crianças nascidas pequenas para a idade gestacional e a termo tiveram maior ocorrência de alterações, em relação aos demais grupos, com aumento da latência das ondas III e V e interpicos I-III e I-V. Todas apresentaram resultados normais no potencial evocado auditivo de longa latência. Conclusão Crianças nascidas pequenas para a idade gestacional e a termo apresentam disfunções na condução neural no tronco encefálico e devem ser consideradas de risco para alterações do desenvolvimento das habilidades auditivas necessárias para garantir qualidade de processamento da informação acústica.

ABSTRACT Purpose To follow up the central auditory system of children born small for gestational age, through electrophysiological evaluation of hearing, in order to verify the occurrence of possible neural dysfunctions in this system. Methods A longitudinal study was carried out with 23 children divided into four groups: Term-born group, subdivided into small for gestational age (four children) and four children born with appropriate weight for gestational age, whose age at the end of the research was three years old. Preterm group subdivided into small for gestational age (seven children), and appropriate for gestational age (eight children), whose corrected age, at the end of the research was three years old. All children were subjected to assessment of auditory brainstem auditory evoked potentials at birth, at six months and at three years of age, and Long-Latency Auditory Evoked Potential at three years. Results children born at term and small for gestational age had a higher occurrence of hearing alterations in relation to the other groups, with increased latency of waves III and V and interpeaks I-III and I-V. All children presented normal evaluation in the Long-Latency Auditory Evoked Potential. Conclusion Children born term and small for gestational age present dysfunctions in neural conduction in the brainstem and should be considered at risk for alterations in the development of the auditory skills that are necessary to guarantee quality of acoustic information processing.

Linguistic performance in 2 years old preterm, considering chronological age and corrected age. / Desempenho linguístico de prematuros de 2 anos, considerando idade cronológica e idade corrigida.

Introduction Preterm birth causes problems that are not restricted to perinatal mortality. Some premature, even in the absence of brain damage, have negative effects on various aspects of development, such as language difficulties. Objective This study aimed to verify the linguistic performance of preterm children at 2 years old, considering the chronological age and corrected age. Methods The study included 23 preterm children and applied the Test of Early Language Development- TELD-3 to assess the language skills. Results The premature children showed the linguistic performance alterations in Teld-3 in 39.13% of cases. They were also analyzed considering the delay to the chronological and corrected ages and there was no difference in performance for both receptive subtests (p = 0.250) and significant (p = 1.000). Conclusion The group of premature children at 2 years is a population at risk for language disorders that cannot be compensated with age correction.

Desempenho linguístico de prematuros de 2 anos, considerando idade cronológica e idade corrigida / Linguistic performance in 2 years old preterm, considering chronological age and corrected age

RESUMO Introdução O nascimento prematuro acarreta problemas que não se restringem à mortalidade perinatal. Alguns prematuros, mesmo na ausência de lesões cerebrais, apresentam consequências negativas em diversos aspectos do desenvolvimento, como dificuldades em adquirir linguagem. Objetivo O objetivo deste estudo foi verificar o desempenho linguístico de prematuros, na faixa etária de 2 anos, considerando a idade cronológica e a idade corrigida. Métodos Participaram do estudo 23 sujeitos prematuros e para verificar as habilidades linguísticas foi aplicado o Test of Early Language Development – TELD-3. Resultados Os sujeitos prematuros apresentaram desempenho total alterado no TELD-3 em 39,13% dos casos. Os prematuros também foram analisados considerando o atraso para a idade cronológica e a corrigida e não houve diferença no desempenho para os subtestes receptivo (p = 0,250) e expressivo (p = 1,000). Conclusão O grupo de prematuros aos 2 anos de idade constitui população de risco para alterações de linguagem que não podem ser compensadas com a correção da idade.

ABSTRACT Introduction Preterm birth causes problems that are not restricted to perinatal mortality. Some premature, even in the absence of brain damage, have negative effects on various aspects of development, such as language difficulties. Objective This study aimed to verify the linguistic performance of preterm children at 2 years old, considering the chronological age and corrected age. Methods The study included 23 preterm children and applied the Test of Early Language Development- TELD-3 to assess the language skills. Results The premature children showed the linguistic performance alterations in Teld-3 in 39.13% of cases. They were also analyzed considering the delay to the chronological and corrected ages and there was no difference in performance for both receptive subtests (p = 0.250) and significant (p = 1.000). Conclusion The group of premature children at 2 years is a population at risk for language disorders that cannot be compensated with age correction.

A influência da proporcionalidade corporal em crianças nascidas pequenas para a idade gestacional: estudo da maturação da via auditiva / The influence of body proportionality on children born small for gestational age: a study of auditory pathway maturation

Objetivo Acompanhar a maturação da via auditiva de lactentes nascidos pequenos para a idade gestacional (PIG), de acordo com a proporcionalidade corporal, nos primeiros seis meses de vida. Métodos Potencial Evocado Auditivo de Tronco Encefálico (PEATE) realizado no período neonatal e aos seis meses de vida, em 59 lactentes, sendo 35 nascidos pequenos para a idade gestacional e assimétricos (PIG-A) e 24 simétricos (PIG-S), comparados a 59 lactentes nascidos com peso adequado (AIG), considerando a idade gestacional. Resultados Os lactentes PIG-A e PIG-S a termo e pré-termo evidenciaram processo maturacional auditivo (diminuição progressiva das latências do PEATE) significativo, do período neonatal aos seis meses de vida, o mesmo ocorrendo em relação aos AIG. Os grupos AIG a termo e pré-termo, bem como os grupos PIG-S e PIG-A não se diferenciaram do ponto de vista auditivo durante os seis meses, quando comparados entre si. Conclusão Os lactentes PIG com padrão simétrico e assimétrico evidenciaram maturação auditiva, sugerindo que o tempo de permanência no agravo intrauterino não representou risco auditivo maior. .

Purpose To monitor the auditory pathway maturation of infants born small for gestational age (SGA), according to body proportionality, in the first six months of life. Methods Brainstem auditory-evoked potential (BAEP) was performed during the neonatal period and at six months of life in 59 infants born small for gestational age; among them, 35 were born asymmetrical (SGA-A), and 24 were born symmetrical (SGA-S). The results were compared to those of 59 infants considered appropriate for gestational age (AGA). Results The term and pre-term SGA-A and SGA-S infants showed a significant auditory maturation process (progressive decrease of BAEP latencies) from the neonatal period to six months of life. A similar result was observed with the AGA infants. The term and pre-term AGA infants and the SGA-S and SGA-A infants did not differ from each other from an auditory perspective during the six-month period. Conclusion The SGA infants with symmetrical and asymmetrical patterns showed auditory maturation, suggesting that the duration of intrauterine injury did not represent a greater risk to hearing. .

Auditory pathway maturational study in small for gestational age preterm infants.

PURPOSE: To follow up the maturation of the auditory pathway in preterm infants small for gestational age (SGA), through the study of absolute and interpeak latencies of auditory brainstem response (ABR) in the first six months of age. METHODS: This multicentric prospective cross-sectional and longitudinal study assessed 76 newborn infants, 35 SGA and 41 appropriate for gestational age (AGA), born between 33 and 36 weeks in the first evaluation. The ABR was carried out in three moments (neonatal period, three months and six months). Twenty-nine SGA and 33 AGA (62 infants), between 51 and 54 weeks (corrected age), returned for the second evaluation. In the third evaluation, 49 infants (23 SGA and 26 AGA), with age range from 63 to 65 weeks (corrected age), were assessed. The bilateral presence of Transient Evoked Otoacoustic Emissions and normal tympanogram were inclusion criteria. RESULTS: It was found interaural symmetry in both groups. The comparison between the two groups throughout the three periods studied showed no significant differences in the ABR parameters, except for the latencies of wave III in the period between three and six months. As for the maturation with tone burst 0.5 and 1 kHz, it was found that the groups did not differ. CONCLUSION: The findings suggest that, in the premature infants, the maturational process of the auditory pathway occurs in a similar rate for SGA and AGA. These results also suggest that prematurity is a more relevant factor for the maturation of the auditory pathway than birth weight.

Longitudinal electrophysiological study of auditory pathway in small for gestational age infants.

PURPOSE: To follow the maturation of the auditory pathway of infants born small for gestational age term, by studying absolute and interpeak latencies of Auditory Brainstem Response (ABR) in the first six months of life. METHODS: Multicentric prospective longitudinal study. The ABR was carried out in the neonatal period in 96 newborn infants, 49 small for gestational age (SGA) and 47 appropriate for gestational age (AGA). Of these, 77 infants (39 SGA and 38 AGA) returned for a second evaluation. In the third evaluation, 70 infants (35 SGA and 35 AGA) returned. RESULTS: SGA and AGA did not present significant differences in the neonatal period and at three months of life. However, at six months, there was statistical significant difference between SGA and AGA groups for the latencies of wave III and interpeak I-III. Latencies of ABR waves decreased more rapidly in the first three months than the third to the sixth month of life for the SGA. AGA group showed progressive decrease in latency of ABR waves during the six months. CONCLUSION: The findings suggest that, for SGA infants, the maturational process of the auditory pathway occurs in different rate when compared to AGA infants. The SGA infants have faster maturation especially at the first three months of life, while in infants AGA, this process occurred in a constant and gradual way throughout the six months studied.

Longitudinal electrophysiological study of auditory pathway in small for gestational age infants / Estudo eletrofisiológico longitudinal da via auditiva em crianças nascidas a termo e pequenas para a idade gestacional

PURPOSE: To follow the maturation of the auditory pathway of infants born small for gestational age term, by studying absolute and interpeak latencies of Auditory Brainstem Response (ABR) in the first six months of life. METHODS: Multicentric prospective longitudinal study. The ABR was carried out in the neonatal period in 96 newborn infants, 49 small for gestational age (SGA) and 47 appropriate for gestational age (AGA). Of these, 77 infants (39 SGA and 38 AGA) returned for a second evaluation. In the third evaluation, 70 infants (35 SGA and 35 AGA) returned. RESULTS: SGA and AGA did not present significant differences in the neonatal period and at three months of life. However, at six months, there was statistical significant difference between SGA and AGA groups for the latencies of wave III and interpeak I-III. Latencies of ABR waves decreased more rapidly in the first three months than the third to the sixth month of life for the SGA. AGA group showed progressive decrease in latency of ABR waves during the six months. CONCLUSION: The findings suggest that, for SGA infants, the maturational process of the auditory pathway occurs in different rate when compared to AGA infants. The SGA infants have faster maturation especially at the first three months of life, while in infants AGA, this process occurred in a constant and gradual way throughout the six months studied. .

OBJETIVO: Acompanhar a maturação da via auditiva de lactentes nascidos a termo pequenos para a idade gestacional, por meio do estudo das latências absolutas e interpicos do Potencial Evocado Auditivo de Tronco Encefálico (PEATE) nos primeiros seis meses de vida. MÉTODOS: Estudo multicêntrico prospectivo longitudinal. PEATE realizado no período neonatal em 96 recém-nascidos, 49 pequenos para a idade gestacional (PIG) e 47 adequados para a idade gestacional (AIG). Destes, 77 lactentes (39 PIG e 38 AIG) retornaram para a segunda avaliação. Na terceira avaliação, retornaram 70 lactentes (35 PIG e 35 AIG). RESULTADOS: PIG e AIG não apresentaram diferenças significativas no período neonatal e aos três meses de vida. Aos seis meses, houve diferença estatística entre os grupos PIG e AIG para a onda III e interpico I-III. Latências das ondas do PEATE diminuíram mais rapidamente nos primeiros três meses que do terceiro para o sexto mês de vida para o grupo PIG. O grupo AIG mostrou diminuição progressiva da latência das ondas do PEATE durante os seis meses. CONCLUSÃO: Os resultados sugerem que a maturação da via auditiva nos PIG ocorre em ritmo diferente quando comparada aos AIG. Os PIG têm maturação mais rápida, especialmente nos três primeiros meses de vida; nas crianças AIG, esse processo ocorreu de modo constante e gradual ao longo dos seis meses estudados. .

Auditory pathway maturational study in small for gestational age preterm infants / Estudo maturacional da via auditiva em prematuros nascidos pequenos para a idade gestacional

PURPOSE: To follow up the maturation of the auditory pathway in preterm infants small for gestational age (SGA), through the study of absolute and interpeak latencies of auditory brainstem response (ABR) in the first six months of age. METHODS: This multicentric prospective cross-sectional and longitudinal study assessed 76 newborn infants, 35 SGA and 41 appropriate for gestational age (AGA), born between 33 and 36 weeks in the first evaluation. The ABR was carried out in three moments (neonatal period, three months and six months). Twenty-nine SGA and 33 AGA (62 infants), between 51 and 54 weeks (corrected age), returned for the second evaluation. In the third evaluation, 49 infants (23 SGA and 26 AGA), with age range from 63 to 65 weeks (corrected age), were assessed. The bilateral presence of Transient Evoked Otoacoustic Emissions and normal tympanogram were inclusion criteria. RESULTS: It was found interaural symmetry in both groups. The comparison between the two groups throughout the three periods studied showed no significant differences in the ABR parameters, except for the latencies of wave III in the period between three and six months. As for the maturation with tone burst 0.5 and 1 kHz, it was found that the groups did not differ. CONCLUSION: The findings suggest that, in the premature infants, the maturational process of the auditory pathway occurs in a similar rate for SGA and AGA. These results also suggest that prematurity is a more relevant factor for the maturation of the auditory pathway than birth weight. .

OBJETIVO: Acompanhar a maturação da via auditiva em recém-nascidos prematuros pequenos para a idade gestacional (PIG), por meio do estudo das latências absolutas e interpicos do potencial evocado auditivo de tronco encefálico (PEATE) nos primeiros seis meses de idade. MÉTODOS: Estudo transversal e longitudinal prospectivo multicêntrico, que avaliou 76 recém-nascidos, 35 PIG e 41 adequados para a idade gestacional (AIG), nascidos entre 27 e 36 semanas de gestação na primeira avaliação. O PEATE foi realizado em três momentos (período neonatal, três meses e seis meses). Retornaram para a segunda avaliação 29 PIG e 33 AIG (62 lactentes), entre 51 e 54 semanas (idade corrigida). Na terceira, retornaram 49 lactentes (23 PIG e 26 AIG), com faixa etária de 63 a 65 semanas (idade corrigida). Foi critério de inclusão a presença bilateral de emissões otoacústicas evocadas por estímulo transiente e curva timpanométrica normal. RESULTADOS: Verificou-se simetria interaural nos dois grupos. A comparação entre os dois grupos ao longo dos três períodos estudados não mostrou diferenças relevantes nos parâmetros do PEATE, exceto para as latências da onda III no período entre os três e seis meses. Quanto ao processo maturacional com tone burst 0,5 e 1 kHz, verificou-se que os grupos não se diferenciaram. CONCLUSÃO: Os resultados sugerem que, nos prematuros, o processo de maturação da via auditiva ocorre em tempo similar em PIG e AIG. Também sugerem que a prematuridade é um fator de maior relevância para a maturação da via auditiva que o fator peso ao nascer. .

Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p.F508del, mutation at the CFTR gene in newborns and patients.

Cystic Fibrosis (CF) is one of the most common single-gene defects in European descent populations with an incidence of about 1 in every 2500 live births and carrier frequency of approximately 1 in 25. The most common mutation at the CF transmembrane conductance regulator (CFTR) gene is a deletion (p.F508del) of the phenylalanine codon 508; its frequency, however, is not the same throughout the world. The purpose of this paper is to document an application of a two-tier survey design in different states of Brazil, from which regional differences of the incidence of CF and frequency of CF-causing mutation(s) carriers can be for the first time estimated. We present data on genotype distributions in reference to p.F508del mutation in samples of newborns, adult controls and CF patients from five Brazilian states, in which a total of 2683 newborns born to Brazilian white parents and 500 African-Brazilians adult controls were screened, as well as 300 CF patients (262 European descents and 38 African descents) were genotyped. Our results suggest that the CF-incidence in different parts of Brazil may differ by almost 20-fold. For the five different states as a whole, nearly 48% of the CF-alleles carry the p.F508del mutation, which places the estimates of disease incidence and carrier frequencies for the Brazilian European descents as 1 in 7576 live births and 2.3%, respectively. The implications for prevention of CF and other rare Mendelian diseases through such surveys of mutation screening are discussed.

Tomografia computadorizada de alta resolução (TCAR) no diagnóstico radiológico e seguimento da displasia broncopulmonar: relato de casos / High-resolution computed tomography (HRCT) in the radiological diagnosis and follow-up of bronchopulmonary dysplasia: case reports

Objetivo: avaliar a utilizacao da tomografia computadorizada de alto resolucao (TCAR) em pacientes com displasia broncopulmonar, para diferencia-la...

Hemangioendotelioma hepatico associado a galactosemia em um recem-nascido: relato de caso / Hepatic hemangioendothelioma associated to galactosemia in a newborn: case report

Objetivo: Descrever um recem-nascido portador de galactosemia e de hemangioendotelioma hepatico (HH). Descricao: um RN pre-termo nascido de parto normal apresentou ao nascimento hidropisia, hepato-esplenomegalia e ictericia. Os exames laboratoriais...

Circulação extracorpórea por membrana (ECMO) em recém-nascido com insuficiência respiratória por síndrome de aspiração meconial efeitos da administração de surfactante exógeno / Extracorporeal membrane oxygenation (ECMO) in a neonate with respiratory distress due to meconium aspiration syndrome: Effect of the administration of exogenous surfactant

Objetivo: apresentar a evolução clínica de recém-nascido portador de insuficiência respiratória grave neonatal secundária à sindrome de aspiração meconial tratado por circulação extracorpórea por membrana, ou conforme o termo consagrado em língua inglesa, ECMO (Extracorporeal Membrane Oxygenation), o efeito do uso de surfactante exógeno neste caso e os custos do procedimento. Método: descrição de um caso de síndrome de aspiração meconial, tratado na UCINE (Unidade de Cuidados Intensivos Neonatais) do instituto da criança Prof. Pedro Alcantara, Hospital das Clínicas da Universidade de São Paulo. Resultados: o suporte extracorporeo teve a duração de 5 dias, sem complicações clínicas ou mecânicas. Surfactante exógeno de origem porcina foi administrado no 4§ dia, após o quê observamos uma melhora significativa na complacência pulmonar. O recém-nascido pôde então ser rapidamente decanulado. Os custos do tratamento foram compatíveis com a realidade nacional em relação a um recém-nascido criticamente enfermo. Conclusões: a ECMO é indicada em casos de insuficiência respiratória que não respondam a outros tratamentos existentes. Deve ser disponível em Unidades de Tratamento Intensivo (UTIs) neonatais de hospitais terciários e ser empregada conforme critérios bem estabelecidos. A utilização de surfactante exógeno aparentemente antecipou a retirada da ECMO e, portanto, deve ser considerada em casos semelhantes. Os custos do tratamento justificam a organização de Equipes de ECMO nessas UTIs

Monitoring the treatment of sepsis with vancomycin in term newborn infants

A prospective study was conducted to determine if standardized vancomycin doses could produce adequate serum concentrations in 25 term newborn infants with sepsis. Purpose: The therapeutic response of neonatal sepsis by Staphylococcus sp. treated with vancomycin was evaluated through serum concentrations of vancomycin, serum bactericidal titers (SBT), and minimum inhibitory concentration (MIC). METHOD: Vancomycin serum concentrations were determined by the fluorescence polarization immunoassay technique , SBT by the macro-broth dilution method, and MIC by diffusion test in agar . RESULTS: Thirteen newborn infants (59.1 percent) had adequate peak vancomycin serum concentrations (20--40 mg/mL) and one had peak concentration with potential ototoxicity risk (>40 æg/mL). Only 48 percent had adequate trough concentrations (5--10 mg/mL), and seven (28 percent) had a potential nephrotoxicity risk (>10 æg/mL). There was no significant agreement regarding normality for peak and trough vancomycin method (McNemar test : p = 0.7905). Peak serum vancomycin concentrations were compared with the clinical evaluation (good or bad clinical evolution) of the infants, with no significant difference found (U=51.5; p=0.1947). There was also no significant difference between the patients' trough concentrations and good or bad clinical evolution (U = 77.0; p=0.1710). All Staphylococcus isolates were sensitive to vancomycin according to the MIC. Half of the patients with adequate trough SBT (1/8), also had adequate trough vancomycin concentrations and satisfactory clinical evolution. CONCLUSIONS: Recommended vancomycin schedules for term newborn infants with neonatal sepsis should be based on the weight and postconceptual age only to start antimicrobial therapy. There is no ideal pattern of vancomycin dosing; vancomycin dosages must be individualized. SBT interpretation should be made in conjunction with the patient's clinical presentation and vancomycin serum concentrations. Those laboratory and clinical data favor elucidation of the probable cause of patient's bad evolution, which would facilitate drug adjustment and reduce the risk of toxicity or failing to achieve therapeutic doses

Leite humano: um pouco de sua história / Human milk: a bit of history

Até o ínicio do século 20 praticamente todas as crianças, nos primeiros anos de suas vidas, alimentavam-se ao seio materno ou ao da ama de leite. A partir da descoberta da pasteurizaçäo e do leite em pó teve início a era do aleitamento artificial. A valorizaçäo do leite materno, para a promoçäo da saúde dos lactentes, originou a retomada da amamentaçäo e dos bancos de leite humanos

Síndrome de Edwards com aplasia radial - relato de dois casos e revisäo das anomalias esqueléticas na síndrome / Edward's syndrome with radial aplasia - report of two cases and review of skeletal abnormalities associated with this syndrome

A síndrome de Edwards ou trissomia do cromossomo 18 é a segunda trissomia autossômica mais freqüente entre os nativivos. Os pacientes afetados apresentam múltiplas malformaçöes, atraso grave de crescimento e desenvolvimento neuropsicomotor e prognóstico reservado. A aplasia radial é uma anomalia esquelética raramente descrita na literatura. Os autores estudaram dois pacientes com trissomia 18 associada à aplasia radial e descrevem os achados clínicos e radiológicos e os diagnósticos diferenciais, com revisäo das anomalias esqueléticas na síndrome de Edwards.

Historia natural de 24 pacientes com trissomia 18 (sindrome de Edwards) e de 20 pacientes com trissomia 13 (sindrome de Patau) / Natural history of 24 patients with trisomy 18 (Edwards syndrome) and 20 patients with trisomy 13 (Pataus syndrome)

As trissomias 18 e 13 constituem a segunda e a terceira trissomia autossomica mais frequentes entre os nativivos. Os pacientes afetados cursam com multiplas malformacoes, atraso grave de crescimento e desenvolvimento e prognostico...

Comportamento imunológico (IgG, IgM, IgA) e complemento total (CH50) de recém-nascidos com fatores de risco para sepse precose : análise comparativa entre recém-nascidos com e sem infecçäo / Immunological behavior (IgG, IgM, IgA) and total complement (CH50) of newborns infants with risk factors for early onset sepsis : comparative analysis between newborns with and without infection

Com objetivo de verificar o comportamento imunologico do recem-nascido (RN) frente a um agravo infeccioso, estudamos 60 RN que apresentavam fatores de risco para infeccao precoce (ruptura prematura de membranas, amnionite clinica, ou infeccao do trato urinario) do ponto de vista infeccioso e imunologico. Todos foram classificados em tres grupos de idade gestacional : inferior a 34 semanas, entre 34 e 36 6/7 semanas e > 37 semanas. O diagnostico de sepse foi realizado atraves de criterios clinicos e laboratorias, incluindo-se entre os exames as dosagens de imunoglobulinas (IgG, IgM, IgA) e complemento total (CH50), obtidos do RN ao nascimento e no quinto dia de vida...