Sotalol no Tratamento da Taquicardia Fetal

A taquicardia fetal é uma situação rara, que, quando mantida coloca em risco a vida do feto. O modo de tratamento não é consensual, existindo várias modalidades farmacológicas. O objectivo deste estudo foi avaliar a eficácia e segurança do sotalol no tratamento de taquicardias fetais. Material e métodos: Estudo retrospectivo, com base nos registos de consulta e entrevista às mães dos fetos com taquicardia supraventricular, referenciados ao Serviço de Cardiologia Pediátrica do Hospital de Santa Marta, durante um período de dez anos. Resultados: Foram diagnosticados oito fetos com taquicardia supraventricular, dos quais seis foram tratados com sotalol. A idade média de gestação na apresentação foi de 30 semanas. Nenhum feto apresentava cardiopatia estrutural, em dois verificou-se hidropisia fetal e outro apresentou hidrocefalia. A taquicardia era supraventricular em todos, sendo em dois por flutter auricular. Em todos os casos, excepto um, houve conversão a ritmo sinusal, não se registando efeitos secundários nas mães nem mortalidade fetal. No período neonatal em três crianças foram registados episódios de taquicardia supraventricular paroxística. Conclusão: O sotalol mostrou-se seguro e eficaz no tratamento das taquicardias fetais, mas, dada a pequenez da amostra, outros estudos mais alargados são necessários para se tirarem conclusões válidas.

Prospective Risk of Intrauterine Death of Monochorionic-Diamniotic Twins

OBJECTIVE: The purpose of this study was to calculate the prospective risk of fetal death in monochorionic-diamniotic twins. STUDY DESIGN: We evaluated 193 monochorionic diamniotic twin pregnancies that were followed and delivered after 24 weeks. Surveillance included cardiotocography and sonography performed at least once weekly. The prospective risk of fetal death was calculated as the total number of deaths at the beginning of the gestational period divided by the number of continuing pregnancies at or beyond that period. RESULTS: The fetal death rate was 5 of 193 pregnancies (2.6%; 95% CI, 1.1, 5.9); the prospective risk of stillbirth per pregnancy after 32 weeks of gestation was 1.2% (95% CI, 0.3% - 4.2%). CONCLUSION: Under intensive surveillance, the prospective risk of fetal death in monochorionic-diamniotic pregnancies after 32 weeks of gestation is much lower than reported and does not support a policy of elective preterm delivery.

Community-Associated Methicillin-Resistant Staphylococcus Aureus Lacking PVL, as a Cause of Severe Invasive Infection Treated with Linezolid

Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) is an emerging public health problem worldwide. Severe invasive infections have been described, mostly associated with the presence of Panton-Valentine leukocidin (PVL). In Portugal limited information exists regarding CA-MRSA infections. In this study we describe the case of a previously healthy 12-year-old female, sport athlete, who presented to the hospital with acetabulofemoral septic arthritis, myositis, fasciitis, acetabulum osteomyelitis, and pneumonia.The MRSA isolated from blood and synovial fluid was PVL negative and staphylococcal enterotoxin type P (SEP) and type L (SEL) positive, with a vancomycin MIC of 1.0mg/L and resistant to clindamycin and ciprofloxacin. The patient was submitted to multiple surgical drainages and started on vancomycin, rifampicin, and gentamycin. Due to persistence of fever and no microbiological clearance, linezolid was started with improvement. This is one of the few reported cases of severe invasive infection caused by CA-MRSA in Portugal,which was successfully treated with linezolid. In spite of the severity of infection, the MRSA isolate did not produce PVL.

Restrição do Crescimento Fetal - Casuística de 4 Anos

Overview and aims: Fetal growth restriction (FGR) affects 15% of pregnancies and is associated with both increased perinatal and neonatal morbidity and mortality and long-term effects in adult life. Our aim was to describe cases and outcomes of FGR from a tertiary perinatal care centre and identify the predictors of neonatal morbidity and mortality. Study design: retrospective cohort. Population: pregnancies with early or late FGR caused by placental factors followed from 2006 to 2009 in a tertiary perinatal care centre. Methods: we collected data from clinical records on demographics, clinical history and fetal ultrasound parameters. Perinatal and neonatal outcomes were stratiied according to gestational age (above or below 28 weeks) and we used bivariate analysis to identify any associations with clinical and imaging indings. Results: we included 246 pregnancies; hypertension was the most prevalent maternal risk factor (16%). There were 15 cases of early FGR, 11 of which had cesarean delivery due to deterioration of fetal Doppler parameters. Outcomes in this group included one fetal and three neonatal deaths. Of 231 cases of late FGR, 64% were delivered early given a non-reassuring fetal status i.e. due to changes in Doppler evaluation or altered Manning biophysical proile. There were four cases of perinatal death in this group, three of which delivered at 28 weeks. Neonatal morbidity was associated with lower gestational age, lower birthweight and progressive placental dysfunction (p<0.01). Conclusion: there was an association between neonatal morbidity and gestational age, birthweight and Doppler deterioration, particularly for deliveries below 28 weeks. The assessment of vascular changes through Doppler analysis allows anticipation of fetal deterioration and is a helpful tool in deciding the optimum timing of delivery.

Neuropsychiatric Features of a Cohort of Patients with Systemic Lupus Erythematosus

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non-NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS(-) (0.3 ± 1 years) than in the APS(+) (5 ± 7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS(-) group. Overall cumulative mortality was highest in NPSLE and in APS(+) patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities.

Congenital Complete Atrioventricular Block. Case Report and Review of the Literature

Introduction: Congenital complete atrioventricular block (AVB) without cardiac malformation is a rare and potentially fatal condition. In most cases it is associated with maternal systemic lupus erythematosus through transplacental passage of antibodies anti-SSA/Ro and/or anti-SSB/La. Antenatal fluorinated-steroids have been successful in reversing first and second degree congenital AVB but inconsistent in third degree block. Case Report:The authors report a case of fetal bradycardia diagnosed at 24 weeks of gestation. The fetal echocardiogram revealed a second/third degree AVB without structural heart disease. Maternal anti-SSA/Ro antibodies were detected. There was no blockage improvement with maternal oral fluorinated-steroids. An elective cesarean section was performed at term with the delivery of a healthy girl that required an epicardical pacemaker on the 8th day of life. Conclusion: In this case, treatment with maternal fluorinated corticosteroids was not effective in preventing progression of the heart block.

Clinical and Genetic Characterization of Portuguese Patients with Pseudohypoparathyroidism Type Ib

Patients with pseudohypoparathyroidism type Ib (PHP-Ib) present hypocalcemia and hyperphosphatemia, as a consequence of a resistance to PTH action, through its G-protein-coupled receptor, in the renal tubules. This resistance results from tissue-specific silencing of the G-protein alpha-subunit (G(s)α), due to imprinting disruption of its encoding locus--GNAS. In familial PHP-Ib, maternally inherited deletions at the STX16 gene are associated to a regional GNAS methylation defect. In sporadic PHP-Ib, broad methylation changes at GNAS arise from unknown genetic causes. In this study, we describe the clinical presentation of PHP-Ib in four Portuguese patients (two of whom were siblings), and provide further insight for the management of patients with this disease. The diagnosis of PHP-Ib was made after detection of GNAS imprinting defects in each of the cases. In the siblings, a regional GNAS methylation change resulted from a known 3.0 kb STX16 deletion. In the other two patients, the broad methylation defects at GNAS, which were absent in their relatives, resulted from genetic alterations that remain to be identified. We report the first clinical and genetic study of Portuguese patients with PHP-Ib. The genetic identification of a hereditary form of this rare disease allowed an early diagnosis, and may prevent hypocalcemia-related complications.