DIA - Departamento da Infância e da Adolescência
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- [Transient hypertrophic neonatal myocardiopathy after acute fetal distress]Publication . GUIMARÃES, P.; TEIXEIRA, F.; MORAIS, L.; ALVARES, S.Rev Port Cardiol. 1998 Jan;17(1):89-92. [Transient hypertrophic neonatal myocardiopathy after acute fetal distress]. [Article in Portuguese] Guimarães P, Teixeira F, Morais L, Alvares S. Unidade de Cardiologia Pediátrica, Hospital de Crianças Maria Pia. Abstract Hypertrophic cardiomyopathy is rare in neonates. The causes include metabolic disease, maternal diabetes, dexamethasone therapy in premature newborns for bronchopulmonary dysplasia and idiopathic hypertrophic cardiomyopathy. The authors present a case report regarding a neonate with transient hypertrophic cardiomyopathy, after acute fetal distress. Signs of congestive cardiac failure appeared at day 1. Echocardiography showed signs of mild pulmonary hypertension and normal ventricular wall thickness. Echocardiography on day 2 showed hypertrophy of myocardial walls affecting first the right ventricular anterior wall and the interventricular septum, followed by the left ventricular posterior wall. With therapy, clinical improvement was noted on day 4. The ventricular wall thickness was almost normal at three weeks. The authors alert physicians to the importance of echocardiographic examination of these neonates. PMID: 9580509 [PubMed - indexed for MEDLINE]
- Study on COgnition and Prognosis in the Elderly (SCOPE)Publication . HANSSON, L.; LITHELL, H.; SKOOG, I.; BARO, F; BANKI, C.M.; BRETELER, M.; CARBONIN, P.U.; CASTAIGNE, A.; CORREIA, M.; DEGAUTE, J.P.; ELMFELDT, D.; ENGEDAL, K.; FARSANG, C.; FERRO, J.; HACHINSKI, V.; HOFMAN, A.; JAMES, O.F.; KRISIN, E.; LEEMAN, M.; DE LEEUW, P.W.; LEYS, D.; LOBO, A.; NORDBY, G.; OLOFSSON, B.; ZANCHETTI, A.; ET ALBlood Press. 1999;8(3):177-83. Study on COgnition and Prognosis in the Elderly (SCOPE). Hansson L, Lithell H, Skoog I, Baro F, Bánki CM, Breteler M, Carbonin PU, Castaigne A, Correia M, Degaute JP, Elmfeldt D, Engedal K, Farsang C, Ferro J, Hachinski V, Hofman A, James OF, Krisin E, Leeman M, de Leeuw PW, Leys D, Lobo A, Nordby G, Olofsson B, Zanchetti A, et al. University of Uppsala, Department of Public Health, Sweden. Abstract The Study on COgnition and Prognosis in the Elderly (SCOPE) is a multicentre, prospective, randomized, double-blind, parallel-group study designed to compare the effects of candesartan cilexetil and placebo in elderly patients with mild hypertension. The primary objective of the study is to assess the effect of candesartan cilexetil on major cardiovascular events. The secondary objectives of the study are to assess the effect of candesartan cilexetil on cognitive function and on total mortality, cardiovascular mortality, myocardial infarction, stroke, renal function, hospitalization, quality of life and health economics. Male and female patients aged between 70 and 89 years, with a sitting systolic blood pressure (SBP) of 160-179 mmHg and/or diastolic blood pressure (DBP) of 90-99 mmHg, and a Mini-Mental State Examination (MMSE) score of 24 or above, are eligible for the study. The overall target study population is 4000 patients, at least 1000 of whom are also to be assessed for quality of life and health economics data. After an open run-in period lasting 1-3 months, during which patients are assessed for eligibility and those who are already on antihypertensive therapy at enrolment are switched to hydrochlorothiazide 12.5 mg o.d., patients are randomized to receive either candesartan cilexetil 8 mg once daily (o.d.) or matching placebo o.d. At subsequent study visits, if SBP remains >160 mmHg, or has decreased by <10 mmHg since the randomization visit, or DBP is >85 mmHg, study treatment is doubled to candesartan cilexetil 16 mg o.d. or two placebo tablets o.d. Recruitment was completed in January 1999. At that time 4964 patients had been randomized. All randomized patients will be followed for an additional 2 years. If the event rate is lower than anticipated, the follow-up will be prolonged. PMID: 10595696 [PubMed - indexed for MEDLINE]
- Cardiac involvement in metabolic diseasesPublication . PEREIRA, A.; MARTINS, E.; CARVALHO, C.; ALVARES, S.Rev Port Cardiol. 1999 Jan;18(1):53-7. [Cardiac involvement in metabolic diseases] [Article in Portuguese] Pereira A, Martins E, Carvalho C, Alvares S. Unidade de Cardiologia Pediátrica, Hospital de Crianças Maria Pia. Erratum in: Rev Port Cardiol 1999 Feb;18(2):131. Abstract OBJECTIVE: To evaluate cardiac involvement in children with metabolic disease in the out patient clinic of the Pediatric Cardiology Unit of Maria Pia Children's Hospital and their follow-up. MATERIAL AND METHODS: Twenty-nine medical records belonging to out patients with metabolic disease in consultation at our unit were reviewed. The following data from each record was analyzed: sex, metabolic disease diagnosis, age and motive for referral to a pediatric cardiology unit, cardiology diagnosis, therapy and evolution. RESULTS: Seventeen patients were boys and 12 girls. The average age of referral was 7.2 years (SD 4.8). The motives for referral were: screening for heart disease, 16; heart murmur, 7; congestive heart failure, 3; heart murmur and fatigue, 2; poor weight gain, 1. The following metabolic diagnoses were made: lysosomal diseases, 21; mitochondrial citopathies, 5; disorder of beta-oxidation of fatty acids, 2; carbohydrate deficient glycoprotein syndrome (CDG syndrome), 1. The cardiologic evaluation was normal in ten patients (4 with lysosomal disease, 4 with mitochondrial citopathy, one disorder of beta-oxidation of fatty acids, the CDG syndrome). Mitral and aortic valve lesions predominated in lysosomal diseases (12/21); myocardial involvement alone was present in two patients, and both myocardial and valvular lesions were present in three. Dilated cardiomyopathy was the presented manifestation in two patients-one with mitochondrial citopathy and one with a disorder of beta-oxidation of fatty acids. Three patients died and 26 remain out-patients. One patient was submitted to valve surgery. The average duration of follow-up was 21 months (SD 24). COMMENTS: Lysosomal diseases were the most representative in our patients, as described in the literature. Heart valve disease was the most frequent alteration. Indication for heart valve surgery is dependent on systemic involvement of the primary disease. All children with a metabolic disease with eventual heart involvement should be evaluated periodically by a cardiology unit. On the other hand, it is mandatory to screen a cardiomyopathy of unknown cause for a metabolic disease. The authors draw attention to the importance of infectious endocarditis prophylaxis in this group of patients. PMID: 10091525 [PubMed - indexed for MEDLINE
- Haemolytic uraemic syndrome, cardiomyopathy, cutaneous vasculopathy and anti-phospholipid activityPublication . Faria, M.; Mota, C.; Barbot, J.; Alvares, S.; Jardim, H.; Vilarinho, A.; Pereira, E.
- Cefalea racimos en una niña de 3 añosPublication . Garrido, C.; Tuna, A.; Ramo, S.; Temudo, T.Summary. Introduction. Cluster headache is a rare disorder in childhood. We identified, in the literature, 64 cases of cluster headache starting at or before 18 years (only 17 of them began before 10 years old). All patients met the criteria of the International Headache Society. Russell et al demonstrated recently that the cluster headache is an inherited disorder in some families. They conclude that the gene is present in 3 to 4% of males and 7 to 10% of females with cluster headache and that it has an autossomal dominant transmission. Clinical case. The authors report the clinical case of a five-year-old child with cluster headache starting at three years. This paper reviews the differential diagnosis and the treatment of cluster headache
- Dysgenetic male pseudohermaphroditismPublication . Proença, E.; Freitas, S.; Fonseca, M.; Figueiredo, S.; Rodrigues, C.Acta Med Port. 2001 Sep-Dec;14(5-6):511-4. [Dysgenetic male pseudohermaphroditism] [Article in Portuguese] Proença E, Freitas S, Fonseca M, Figueiredo S, Rodrigues C. Serviço de Cirurgia Pediátrica, Hospital Maria Pia, Porto. Abstract Dysgenetic male pseudohermaphroditism is the result of a defect of testis development that encompasses a large clinical heterogeneity. It is characterized by bilateral dysgenetic testis, absence of mullerian regression, ambiguous genitalia and/or stigmata of Turner's syndrome in the majority of the cases. Typically, these individuals have either a 46,XY or 45,X/46,XY karyotype. The authors present four cases of dysgenetic male psudohermaphroditism, with ages of diagnosis between 1 month and 17 years old. The first had a male phenotype with stigmata of Turner's syndrome and the others ambiguous genitalia. Two patients were 45,X/46,XY and 45X/47,XYY mosaics and the other two were 46,XY. Gonadal karyotyping showed mosaicism (45,X/46,XY) in all four cases. In the first case was programed orquidectomy; all the others assigned a male gender, with regular follow-up until the puberty. PMID: 11878163 [PubMed - indexed for MEDLINE]
- Comparative study of the degree of patient satisfaction in intermittent catheterization with Lofric and polyvinyl chloride cathetersPublication . Pereira, P.; Urrutia, M.; Lobato, L.; Rivas, S.; Monereo, E.Actas Urol Esp. 2001 Nov-Dec;25(10):725-30. [Comparative study of the degree of patient satisfaction in intermittent catheterization with Lofric and polyvinyl chloride catheters]. [Article in Spanish] López Pereira P, Martínez Urrutia MJ, Lobato L, Rivas S, Jaureguizar Monereo E. SourceUnidad de Urología Infantil, Hospital Universitario La Paz, Madrid. Abstract PURPOSE: To assess the grade of satisfaction in children on intermittent catheterization with the use of LoFric and PVC conventional catheters. MATERIAL AND METHODS: A total of 40 p with experience in CIC were included in this study. An anonymous questionnaire was sent to all patients after 2-months using the LoFric catheter. Patients were divided in 3 groups (bladder augmentation, artificial sphincter, Mitrofanoff) because of major differences in CIC discomfort between these groups. RESULTS: The questionnaire was completed by 87.5% of the patients (35 p). In 86% (30 p) LoFric catheter training was easy or very easy but in 14% (5 p) it was difficult. Four patients had some difficulty during conventional catheter insertion, in 3 (75%) the difficulty disappeared with the use of LoFric catheter. Of the 51% (18 p) who reported some discomfort during the insertion of conventional catheter, 72% said it was eliminated when the LoFric catheter was used. Of 6 p with some discomfort when removing the conventional catheter, 5 (83%) said it disappeared with the new catheter. Th LoFric catheter was favored by 70% of patients because it reduced the discomfort caused by conventional catheters, bladder insertion was easier and smoother, and gel lubrication was not needed. The 17% of patients reported some difficulty dealing with this slippery catheter. CONCLUSIONS: The use of the LoFric catheter could be justified in patients who report with conventional catheters have some discomfort. It can also be recommended in patients with artificial sphincter, bladder augmentation and Mitrofanoff procedure, in whom any complication related to CIC would have serious consequences.
- Other indications for surfactant].Publication . PROENÇA FERNANDES, E.; CARVALHO, C.; SILVA, A.; FERREIRA, P.; ALEGRIA, A.; LOPES, L.; AREIAS, M.A.An Esp Pediatr. 2002 Jan;56(1):45-8. [Other indications for surfactant] [Article in Spanish] Proença Fernandes E, Carvalho C, Silva A, Ferreira P, Alegria A, Lopes L, Areias MA. Unidades de Cuidados Intensivos Neonatales y Pediátricos, Hospital Maria Pia, Spain. Abstract OBJECTIVE: The introduction of surfactant replacement therapy in the management of respiratory distress syndrome in the premature infant was a remarkable advance in neonatal intensive care. In the last few years, recognition of the role played by surfactant inactivation in the pathogenesis of other respiratory diseases of the newborn has justified new therapeutic applications. The aim of this study was to evaluate the efficacy of treatment with natural exogenous surfactant in situations with secondary surfactant deficiency. METHODS: We performed a retrospective analysis of the evolution of 15 newborn infants treated with natural exogenous surfactant. Nine infants had meconium aspiration syndrome, five had congenital pneumonia and one had adult respiratory distress syndrome. Oxygenation indexes before and after surfactant treatment as well as clinical and radiographic evolution were compared. RESULTS: The 12 surviving infants showed improvement in oxygenation and radiographic alterations after surfactant administration. Three patients with meconium aspiration syndrome died. CONCLUSIONS: These results support the therapeutic use of exogenous surfactant in severe respiratory diseases of the newborn causing secondary surfactant deficiency. PMID: 11792244 [PubMed - indexed for MEDLINE
- Clinical trial of lamivudine in children with chronic hepatitis B.Publication . Jonas, M.M.; Mizerski, J.; Badia, I.B.; Areias, J.A.; Schwarz, K.B.; Little, N.R.; Greensmith, M.J.; Gardner, S.D.; Bell, M.S.; Sokal, E.M.; International Pediatric Lamivudine Investigator Group.N Engl J Med. 2002 May 30;346(22):1706-13. Clinical trial of lamivudine in children with chronic hepatitis B. Jonas MM, Mizerski J, Badia IB, Areias JA, Schwarz KB, Little NR, Greensmith MJ, Gardner SD, Bell MS, Sokal EM; International Pediatric Lamivudine Investigator Group. of Gastroenterology, Children's Hospital, Boston, MA 02115, USA. Erratum in: N Engl J Med 2002 Sep 19;347(12):955. Kelley, Deirdre [corrected to Kelly, Deirdre]. Comment in: J Hepatol. 2003 May;38(5):698-9. N Engl J Med. 2002 May 30;346(22):1682-3. Abstract BACKGROUND: Lamivudine therapy is effective for chronic hepatitis B infection in adults. We evaluated the efficacy and tolerability of lamivudine as a treatment for chronic infection with hepatitis B virus (HBV) in children. METHODS: Children with chronic hepatitis B were randomly assigned in a 2:1 ratio to receive either oral lamivudine (3 mg per kilogram of body weight; maximum, 100 mg) or placebo once daily for 52 weeks. The primary end point was virologic response (defined by the absence of serum hepatitis B e antigen and serum HBV DNA) at week 52 of treatment. RESULTS: Of the 403 children screened, 191 were randomly assigned to receive lamivudine and 97 to receive placebo. The rate of virologic response at week 52 was higher among children who received lamivudine than among those who received placebo (23 percent vs. 13 percent, P=0.04). Lamivudine therapy was well tolerated and was also associated with higher rates of seroconversion from hepatitis B e antigen to hepatitis B e antibody, normalization of alanine aminotransferase levels, and suppression of HBV DNA. CONCLUSIONS: In children with chronic hepatitis B, 52 weeks of treatment with lamivudine was associated with a significantly higher rate of virologic response than was placebo. PMID: 12037150 [PubMed - indexed for MEDLINE]
- [Infection due to Mycoplasma pneumoniae: three cases with neurological complications].Publication . Cunha, J.; Madalena, C.; Guimarães, P.; Sousa, A.; Temudo, T.Summary. Introduction. Mycoplasma pneumoniae infection has been associated with severe central nervous system diseases. The pathogenesis of these disorders is unknown and the treatment uncertain. Case reports. The authors present three cases of central nervous system diseases: acute transverse myelitis, cerebellitis and encephalomyelitis associated with M. pneumoniae infection. Conclusions. M. pneumoniae infection should be considered in all cases of severe acute central nervous system symptomatology. El Mycoplasma pneumoniae es un agente implicado frecuentemente en infecciones respiratorias de niños y adultos [1,2]. Se pueden producir complicaciones extrarrespiratorias básicamente mucocutáneas (eritema multiforme, eritema nudoso, síndrome de StevenJohnson), cardíacas (miocarditis, pericarditis), articulares (artritis), hematológicas (anemia hemolítica, trombocitopenia, coagulación vascular diseminada), pancreatitis, salpingitis y complicaciones neurológicas [13]. La implicación del sistema nervioso central (SNC) se estima en aproximadamente un 0,1% del total de infecciones producidas por M. pneumoniae, y puede afectar al 7% de los pacientes hospitalizados a causa de una infección producida por este agente [2,3]. Las complicaciones neurológicas incluyen: encefalitis, meningoencefalitis, encefalomielitis, polirradiculoneuropatía (como el síndrome de GuillainBarré), cerebelitis, psicosis, mielitis transversa y coma [14]. Presentamos tres casos clínicos con complicaciones neurológicas en el contexto de una infección por M. pneumoniae (mielitis transversa, cerebelitis, encefalomielitis), cuyo diagnóstico se estableció a partir de los análisis clínicos y los exámenes auxiliares de diagnóstico efectuados, principalmente las serologías seriadas. A infecção por Mycoplasma pneumoniae tem sido associada a múltiplas complicações neurológicas. A patogénese destas permanece incerta e o seu tratamento controverso. Casos clínicos. Os autores apresentam três casos de complicação neurológica em contexto de infecção pelo M. pneumoniae: mielite transversa, cerebelite e encefalomielite. Conclusão. A infecção por M. pneumoniae deve ser considerada em todos os casos de sintomatologia severa aguda do sistema nervoso central