Browsing by Author "Gomes, J"
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- Accuracy of prenatal culture in predicting intrapartum group B streptococcus colonization statusPublication . Florindo, C; Damião, V; Lima, J; Nogueira, I; Rocha, I; Caetano, P; Ribeiro, L; Viegas, S; Gomes, J; Borrego, MJOBJECTIVE: To evaluate the positive predictive value (PPV) of group B Streptococcus (GBS) cultures at 35-37 weeks of gestation relative to GBS colonization status at delivery. METHODS: Rectovaginal swabs from 221 women at labor in four Lisbon hospitals were collected for GBS screening according to the CDC guidelines. RESULTS: The PPV was 24.4%. IAP was administered to 100% of prenatally GBS positive women. There was no case of early onset GBS disease (EOD). CONCLUSIONS: Poor accuracy of prenatal cultures in identifying true candidates for IAP highlights the need for Portuguese clinical and laboratory guidelines to prevent EOD and antibiotic overtreatment of pregnant women.
- Juvenile systemic lupus erythematosus in Portugal: clinical and immunological patterns of disease expression in a cohort of 56 patientsPublication . Cabral, M; Escobal, C; Conde, M; Ramos, M; Gomes, JObjective: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. Methods: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. Results: A cohort of 56 patients with a mean age at di- sease onset of 12.6±4.04 years (mean±1SD) (range, 1.0- -17.0 years) and a mean period of follow-up of 5.5±5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular ne - phritis in all cases. Neuropsychiatric manifestations occurred in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respec- tively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were treated with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being active disease and severe infection the causes of death. Conclusions: This study suggests that in our patients the clinical and laboratory features observed were similar to juvenile systemic lupus erythematosus patients from other series. Clinical outcome was favourable in the present study. Complications from therapy were frequent. Objective: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. Methods: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. Results: A cohort of 56 patients with a mean age at di- sease onset of 12.6±4.04 years (mean±1SD) (range, 1.0-17.0 years) and a mean period of follow-up of 5.5±5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular nephritis in all cases. Neuropsychiatric manifestations occur red in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respectively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were trea- ted with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being active disease and severe infection the causes of death. Conclusions: This study suggests that in our patients the clinical and laboratory features observed were similar to juvenile systemic lupus erythematosus patients from other series. Clinical outcome was favourable in the present study. Complications from therapy were frequent.
- Parvovirus B19 Associated Systemic Lupus Erythematosus in a Child with Sickle Cell Disease; a Diagnostic and Therapeutic ChallengePublication . Cabral, M; Dias, A; Abadesso, C; Conde, M; Ferreira, M; Gomes, J; Carreiro, HAlthough sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two distinct and relatively common chronic diseases, coexistence of these two conditions in the same patient appears to be rare. The authors report an eight-year-old child with SCD who developed a severe form of parvovirus B19-associated SLE, with secondary severe immune hemolytic anemia related to drugs, Libman-Sacks endocarditis complicated by severe aortic regurgitation, dilated left ventricle with impaired function and myocardial ischemia, with further decompensation culminating in cardiac arrest during an infectious intercurrence, which led inevitably to death. This patient displayed a broad spectrum of musculoskeletal, hematologic and cardiovascular complications, which could be associated with either SCD or SLE. Conclusion: Because of a substantial overlap between the clinical manifestations of these two disorders, the diagnosis of SLE in a patient with a previous known diagnosis of SCD may be difficult and is often delayed. Our report illustrates the importance of considering other disease processes, like autoimmune diseases when clinical features or its evolution are atypical of SCD and emphasizes some of the diagnostic difficulties encountered during the diagnosis and management of these patients.
- Protracted Febrile Myalgia Syndrome with Henoch-Schönlein Purpura: an atypical presentation of Familial Mediterranean FeverPublication . Cabral, M; Conde, M; Brito, MJ; Almeida, HI; Gomes, JA Febre Mediterrânica Familiar (FMF) é uma doença hereditária autossómica recessiva caracterizada por episódios de febre recorrente, artrite e poliserosite – peritonite, pleurite e/ou pericardite. A sua principal complicação é a amiloidose AA sistémica. Menina de 8 anos, origem africana, com febre recorrente desde os 5 anos e três internamentos com febre, dor abdominal e elevação dos reagentes de fase aguda. No primeiro episódio foi sujeita a apendicectomia e no terceiro o quadro clínico acompanhou-se de mialgias, púrpura e proteinúria não nefrótica. A biopsia renal foi compatível com nefrite de Henoch-Schönlein. Durante os episódios de febre e dor abdominal registou-se um nível sérico de amilóide A - 92 mg/L (VR < 6.8) que levantou a suspeita de FMF. Posteriormente o diagnóstico foi confirmado por estudo genético (homozigotia para M694V no gene MEFV). Iniciou colchicina e actualmente encontra-se em remissão completa. A FMF deve ser considerada no diagnóstico diferencial de febre e dor abdominal recorrente na criança, mesmo quando a forma de apresentação é atípica (p.e. Protracted Febrile Myalgia Syndrome). O estudo genético permite confirmar o diagnóstico e tem valor em termos de prognóstico