SESARAM - Pedi - Artigos
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- A evolução do refluxo vesico-ureteral com diagnóstico perinatalPublication . Leitão, Henrique; Correia, António Jorge; Gomes, Clara; Oliveira, Oliveira; Rolo, Vitor; Mimoso, Gabriela; Ramos, ConceiçãoResumo.Avaliar a evolução e tratamento do refluxo vesico--ureteral congénito (RVU) em recém-nascido ou lactente com diagnóstico pré-natal de anomalia nefro-urológica. Métodos. Estudo de coortes históricas de crianças com o diagnóstico perinatal de RVU, nascidas numa maternidade de apoio perinatal diferenciado da Região Centro do País, entre 1993 e 2002, e posteriormente acompanhadas no hospital pediátrico de referência da mesma Região. Dividimos as crianças em duas coortes, aquelas que efectuaram tratamento cirúrgico e aquelas em que foi decidido manter vigilância. Para cada coorte (se aplicável) avaliámos: sexo, o tipo e grau de RVU, tipo de cirurgia, complicações, frequência e tempo de evolução até à cura espontânea. Na coorte das crianças em vigilância comparámos a proporção de crianças que mantiveram RVU com aquelas em que se verificou cura espontânea, relativamente ao sexo e ao grau de RVU. Resultados. Oitenta e duas crianças cumpriam os critérios de inclusão. O RVU congénito foi mais frequente no sexo masculino (77%) e era maioritariamente unilateral (65%). O tratamento cirúrgico foi efectuado em 35% dos casos, sobretudo nas crianças com RVU bilateral, com unidades refluxivas (UR) de grau elevado – IV ou V - (80% vs. 12% na coorte de crianças em vigilância) e em crianças com lesão/malformação renal (ipsilateral ou contralateral) e/ou assimetria funcional renal (58% vs 24% na coorte de crianças em vigilância). Neste último grupo, verificámos uma frequência de resolução do RVU de 72% (76% no subgrupo que foi acompanhado pelos menos 48 meses). O período entre os 24 e os 36 meses foi aquele em que ocorreu o maior número de resolução de casos. Não encontrámos diferenças significativas entre sexo e grau de RVU no que respeita à cura espontânea do RVU. Conclusões. O refluxo vesico-ureteral tende a resolver-se nos primeiros anos de vida, parecendo a profilaxia e vigilância clínica medidas suficientes e seguras no casos de refluxo de baixo grau e, provavelmente, também nos casos mais graves.
- Congenital medium sternal cleft with partial ectopia cordis repairPublication . Sousa, P. R.; Antunes, S.; Couto, A.; Santos, G. C.; Leal, L. G.; Magalhaes, M. P.; Madeira Island, Região Autónoma da Madeira; PortugalCongenital sternal malformation is a rare anomaly often diagnosed as an asymptomatic condition at birth. The authors report a clinical case of a full-term female neonate with congenital sternal cleft and partial ectopia cordis. Successful surgical repair was accomplished at 6 days of age. When surgery is performed shortly after birth, the procedure is easier and better results are achieved.
- Idiopathic congenital chylothorax treated with octreotidePublication . Sousa, P. R.; Leitao, H.; Camacho, M. C.; Nunes, J. L.Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects.
- Spontaneous spinal epidural haematoma due to arteriovenous malformation in a childPublication . Cabral, A. J.; Barros, A.; Aveiro, C.; Vasconcelos, R.Spontaneous spinal epidural haematoma (SSEH) is a rare clinical entity, especially in infants, in whom only a few cases have been reported. In a paediatric emergency setting, SSEH should be considered as part of the differential diagnosis for acute extremity weakness and paraesthesia. Epidural vascular malformations are often suspected in these cases but have rarely been demonstrated. The authors report herein a case of SSEH in a 9-year-old boy arising from an epidural vascular malformation. He initially presented with sudden intense cervicodorsal pain followed by hypotonic lower extremities and progressive motor weakness, with no sensory change. The MRI showed an acute extradural haematoma extending from C7 to T4 with compression of the spinal cord. After submission to decompression surgery, he presented full recovery in 1 month. The histopathological analysis revealed a vascular malformation.
- Unusual association of diseases/symptoms - Campomelic dysplasia and malignant hyperthermiaPublication . Barros, A.; Teixeira, F.; Camacho, M. C.; Alves, C.Campomelic dysplasia (CD) is a rare clinical entity, usually fatal in the first year of life. It is characterised by bowing and angulations of long bones, along with other congenital anomalies. The occurrence of malignant hyperthermia is rare, but it has been associated with skeletal dysplasias. The authors present the case of a boy, born at 40 weeks of gestational age, with multiple congenital anomalies and subsequently diagnosed with CD, who died at 16 months of age as a consequence of malignant hyperthermia.
- Giant placental chorioangioma: a rare cause of fetal hydrops - Rare diseasePublication . Barros, A.; Freitas, A. C.; Cabral, A. J.; Camacho, M. C.; Costa, E.; Leitao, H.; Nunes, J. L.Giant choriangiomas are rare placental tumours, associated with a high prevalence of pregnancy complications and a poor perinatal outcome. Neonatal consequences include severe microangiopathic haemolytic anaemia, thrombocytopaenia and hydrops. The associated high perinatal death rate (30-40%) has led to a number of prenatal therapeutic interventions with limited success in most cases. The authors present a case of non-immune fetal hydrops caused by a giant chorioangioma, diagnosed at 27 weeks of gestational age. Despite tocolytic therapy, the baby was born prematurely (28 weeks of gestational age) and required transfusion of blood derivatives, intensive phototherapy and exchange transfusion. She had an uncomplicated recovery and was discharged home in the second month of life. The authors emphasise the need to consider chorioangioma as a cause of non-immune fetal hydrops and microangiopathic haemolytic anaemia.
- Genes, crianças e pediatrasPublication . Cardoso, Cátia; Bandeira, Anabela; Martins, Márcia; Martins, EsmeraldaVACTERL association is characterized for vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. In this patient we found a also a poor weight gain and microcephaly associated with normochromic and normocytic, anemia, uncontrolled glycemia (hypoglycemia and hyperglycemia) and glycosuria with tubulopathy, nephrotic proteinuria and metabolic acidosis with hyperlactacidemia with led us to suspect and confirm a mitochondrial respiratory chain defect.
- Neonatal stroke associated with de novo antiphospholipid antibody and homozygous 1298C/C methylenetetrahydrofolate reductase mutationPublication . Rego Sousa, P.; Figueira, R.; Vasconcellos, R.Antiphospholipid antibodies are a recognised prothrombotic risk factor associated with acute ischaemic infarction. Most autoimmune diseases are rare in infants, and in the neonatal period, autoimmunity is related to transplacental passage of maternal immunoglobulin G autoantibodies. Distinguishing between de novo and acquired autoimmunity has important therapeutic implications and is crucial for determining the prognosis. We present a case of a neonatal thrombotic stroke associated with de novo synthesis of antiphospholipid antibodies, a homozygous 1298C/C methylene-tetrahydrofolate reductase mutation and a double-homozygous plasminogen activator inhibitor 1 polymorphism (PAI-1 844A/A and 675 4G/4G), which may have increased the final thrombotic risk. Her mother was not positive for antiphospholipid antibodies. The authors highlight an unequivocal evidence of a de novo case of paediatric antiphospholipid antibody syndrome and emphasise the need for a thorough investigation in cases of neonatal stroke including molecular thrombophilia study.
- Paediatric acute basilar thrombosis successfully treated with intravenous alteplasePublication . Rego Sousa, P.; Vasconcellos, R.Acute ischaemic stroke has significant attendant morbidity and is one of the top ten causes of childhood death. It requires prompt investigation and management, however little is known about the safety and efficacy of acute thrombolytic therapies in childhood arterial ischaemic stroke. The authors report a case of a 13-year-old girl with an acute basilar thrombosis, successfully treated with intravenous recombinant tissue plasminogen activator and discuss the management of paediatric arterial ischaemic stroke.
- Paediatric acute basilar thrombosis successfully treated with intravenous alteplasePublication . Rego de Sousa, Paulo; Vasconcellos, RuiAcute ischaemic stroke has significant attendant morbidity and is one of the top ten causes of childhood death. It requires prompt investigation and management, however little is known about the safety and efficacy of acute thrombolytic therapies in childhood arterial ischaemic stroke. The authors report a case of a 13-year-old girl with an acute basilar thrombosis, successfully treated with intravenous recombinant tissue plasminogen activator and discuss the management of paediatric arterial ischaemic stroke.
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