Lupus Myelopathy in a Child

A 5-year-old female developed, after a 7-month period of fever, anorexia, weight loss, and a transitory cutaneous erythematous eruption, a severe acute transverse myelopathy, with a partial recovery of motor and sensory function. She had positive antinuclear and antidouble-stranded DNA antibodies but no antiphospholipid antibodies. Six months later she had massive proteinuria and restarted treatment with steroids and cyclophosphamide. Our patient is one of the youngest reported with lupus myelopathy. We discuss the clinical presentation, the magnetic resonance imaging findings, and other relevant laboratory studies of this rare but serious complication of systemic lupus erythematosus.

Anéis de Ferrara: - 4 Anos Depois

Os anéis intraestromais, nomeadamente o anel de Ferrara, constitui uma importante opção terapêutica das doenças ectásicas da córnea, de origem não inflamatória como o Queratocone. Os autores analisaram os primeiros 30 casos operados no Serviço de Oftalmologia do CHLC relativamente à eficácia, estabilidade e segurança deste procedimento ao longo dos 4 anos. Verificaram estabilidade refractiva, diminuição queratométrica e do equivalente esférico e boa tolerância ao material implantado. Os autores concluem que esta opção terapêutica para o queratocone é segura, reversível, com resultados estáveis que permitem adiar ou evitar a queratoplastia.

Slipped Capital Femoral Epiphysis. A Report of 4 Cases Occurring in One Family

We describe slipped capital femoral epiphysis in 4 members of a black, obese family, who were all first-degree relatives. The aetiology of slipped capital femoral epiphysis is unknown, although it is thought to be multifactorial. Genetic predisposition and environmental factors have been associated with the condition. A familial incidence with at least two cases in the same family has been reported. In epidemiological studies, this incidence ranges from 3% to 35%. Our cases were investigated in an attempt to find a possible aetiological genetic factor. A genetic predisposition with an autosomal dominant pattern of transmission is suggested, although environmental variables must be considered as provocative factors.

The Risks of Self - Medication: Case Report of Familial Misuse of AM3 (Immunoferon®)

Over the last decades extended medical knowledge has been an important health care benefit in terms of disease prevention and management. However, probably with no exception, most pharmaceutical products are not devoid of adverse consequences. Immunomodulators are commonly considered a “benign” drug whose advantages bypass consequences. The immunomodulator AM3 (Immunoferon®) is a clinically used, orally administered compound whose active principle is stabilised in an inorganic matrix of calcium. We report the misuse of AM3 in three members of a family; father and two children. The drug was prescribed to the father who subsequently administered it to the children without seeking medical advice. Two months later, all subjects developed abdominal and/or flank colicky pain. Hypercalciuria was diagnosed in the children with different degrees of severity. It is likely that the calcium content of the inorganic matrix played an important role in the onset of symptoms. No adverse side effects related to the inorganic matrix of calcium of immunoferon® have been documented so far. This family case report calls attention to the risks of self -medication in a susceptible family. Paediatric patients are vulnerable as they rely on adults for the supply of medications. Concerning the use of drugs in family, especially nonprescription drugs, the quality of health care provided to the children depends on the health literacy of their parents.

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.

Morte Fetal de um Gémeo. Que Problemas para o Gémeo Sobrevivente?

Na gravidez bigemelar, a morte de um dos fetos no segundo e terceiro trimestre é uma complicação rara, variando a taxa de incidência entre 2,6 e 6,8%. Este acontecimento determina um aumento das taxas de morbilidade e mortalidade perinatal para o gémeo sobrevivente, especialmente em gravidezes monocoriónicas, quando a morte fetal é devida à síndroma de tranfusão feto-fetal. Para alguns autores o atraso de crescimento intrauterino e a prematuridade são os principais factores de risco para o aumento da morbilidade e mortalidade do gémeo sobrevivente. A patofisiologia de instalação dos distúrbios cerebrais no gémeo sobrevivente após a morte do feto irmão não está definida, nem o intervalo de tempo que medeia entre a morte e o estabelecimento das lesões. Também não existe um protocolo definitivo de seguimento destas gravidezes e, posteriormente, do gémeo sobrevivente. No período de 1 de Setembro de 1994 a 31 de Dezembro de 1998, foram seguidas, na consulta de Gravidez Múltipla da Maternidade Dr. Alfredo da Costa, 235 gravidezes bigemelares. Em nove casos (3,8%) ocorreu morte de um dos fetos com idade gestacional acima das 13 semanas. Em cinco das nove gravidezes foi conhecida a causa de morte, quatro das quais foram atribuídas à síndroma de tranfusão feto-fetal. A taxa de prematuridade do gémeo sobrevivente foi de 44,4% (4/9) e a de mortalidade de 11,1%(1/9). A taxa de morbilidade neonatal foi de 62,5% (5/8), na maioria dos casos por complicações inerentes à prematuridade. A taxa de morbilidade neurológica foi de 37,5% (3/8). A taxa de lesões neurológicas major foi de 25% (2/8) e ocorreu em recém-nascidos de termo. A síndroma de transfusão feto-fetal, como causa de morte fetal, associou-se aos casos com pior prognóstico no que se referiu ao gémeo sobrevivente.O crescimento do gémeo sobrevivente parece depender das lesões provocadas pela morte do feto irmão. Os autores finalizam com uma proposta de atitudes obstétricas e pediátricas em relação ao gémeo sobrevivente.