SCir.P - Serviço de Cirurgia Pediátrica
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- A case report of a 4-year-old child with glucose-6-phosphate dehydrogenase deficiency: An evidence based approach to nutritional managementPublication . Pinto, Al.; MacDonald, A.; Cleto, E.; Almeida, M.; Ramos, P.; Rocha, J.Pinto A, MacDonald A, Cleto E, Almeida MF, Ramos PC, Rocha JC. A case report of a 4-year-old child with glucose-6-phosphate dehydrogenase deficiency: An evidence based approach to nutritional management. Turk J Pediatr 2017; 59: 189-192. The objective was to describe the nutritional management of a 4-year-old child with glucose-6-phosphate dehydrogenase (G6PD) deficiency. A 4-year-old male child, African descent, born from non-consanguineous parents presented with a clinical history of frequent respiratory infections, usually treated with antibiotics. At 30 months of age, G6PD diagnosis was made after eating one portion (40 - 60 g) of fava beans, resulting in severe hemolytic anemia hospitalization for 5 days. Diagnosis was confirmed by G6PD activity measurement. Nutritional counseling was given to avoid dietary oxidative stressors particularly the exclusion of fava beans and accidental ingestion of other similar beans. Dietary intake of high vitamin C containing foods was discouraged and adequate hydration advised. Nutritional management is crucial in preventing acute stress events in patients with G6PD deficiency.
- 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.
- 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]
- A experência da criança no perioperatório de cirurgia programadaPublication . Teixeira, E.; Figueiredo, M.A experiência da criança submetida a cirurgia programada é sempre uma vivência individual, não descorando o binómio criança/família. A ansiedade, o medo, o choro, e outras reacções são conhecidas da nossa prática como manifestações à separação familiar e ao ambiente hospitalar. Numa tentativa de compreender a experiência da criança, em idade escolar, no perioperatório de cirurgia programada, levamos a cabo um estudo qualitativo de intervenção. A amostra incluiu 30 crianças admitidas no Serviço de Cirurgia da Instituição. O primeiro grupo (A), seguiu os procedimentos habituais da instituição. O segundo grupo (B), foi submetido a uma intervenção de enfermagem no pré-operatório, realizada na véspera do acto cirúrgico. Após análise das entrevistas, constatou-se a existência de oito categorias: experiência hospitalar, experiência cirúrgica, experiência de aprendizagem, experiência no bloco operatório, sensações, resultados da experiência, papel do enfermeiro e hospital pediátrico. Estas categorias foram alvo de análise de conteúdo para compreensão das mensagens das crianças. As conclusões deste estudo, revelam-nos que as crianças submetidas a intervenção de enfermagem pré-operatória, quando comparadas com as crianças que seguiram o procedimento habitual da instituição, expressam maior entendimento, aceitação e reconhecimento dos procedimentos cirúrgicos; estabelecem, com maior facilidade, uma relação terapêutica com a equipa de enfermagem; relatam menos medo e encaram a experiência como fonte de aprendizagem, e a presença do familiar como o garante tranquilizador.
- In utero topographic analysis of astrocytes and neuronal cells in the spinal cord of mutant mice with myelomeningocele.Publication . Reis, J.L.; Correia-Pinto, J.; Monteiro, M.P.; Hutchins, G.M.Abstract OBJECT: Myelomeningocele (MMC) is the most severe form of spina bifida causing severe neurological deficits. Injury to the placode has been attributed to in utero aggression. In this study, glial and neuronal cell changes in both number and topography in mice with MMC were investigated during gestation. METHODS: The curly tail/loop-tail mice model of MMC was used, and fetuses were harvested using caesarean surgery at Days 14.5, 16.5, and 18.5 (full gestation at 19 days). Immunohistochemical analyses of the MMC placodes and the normal spinal cords from the control group were performed using anti-glial fibrillary acidic protein (astrocytes) and mouse anti-neuronal nuclear (neurons) antibodies. Light microscopy was used along with computer-assisted morphometric evaluation. Progressive increases in astrocytes in the spinal cord of all mouse fetuses were found between Days 14.5 and 18.5 of gestation. This increase was significantly higher in the placodes of mice with MMC than in those of normal mice, particularly in the posterior region. Neuronal labeling at Day 14.5 of gestation was similar between mice with MMC and control mice. At Day 16.5 of gestation there was a deterioration of neural tissue in MMC fetuses, mainly in the posterior region, progressing until the end of gestation with a marked loss of neurons in the entire MMC placode. CONCLUSIONS: This study delineated the quantitative changes in astrocytes and neurons associated with MMC development during the late stages of gestation. The detailed topographic analysis of the MMC defines the timing of the intrauterine insult and how the placode lesions progress. This study supports the current concept of placode protection through in utero surgery for fetuses with MMC. PMID: 17566405 [PubMed - indexed for MEDLINE]
- Minimal invasive surgical of intersex abnormalities: our experiencePublication . Recamán, M.; Bonet, B.; Enes, C.; Cidade-Rodrígues, J.
- Nefrectomia laparoscópica retroperitoneal em crianças com idade inferior a nove anos: Estado da ArtePublication . Moreira-Pinto, J.; Osório, A.; Pereira, J.; Enes, C.; Ribeiro-Castro, J.; Reis, A.Introdução e Objectivos: Em Portugal, a experiência em retroperitoneoscopia pediátrica é escassa. Os autores apresentam a primeira série portuguesa de nefrectomias realizadas por esta via, em crianças com idade inferior a nove anos, e uma revisão da literatura sobre o tema. Material e Métodos: Análise retrospectiva dos processos das crianças submetidas a nefrectomia laparoscópica retroperitoneal (NLR), de Janeiro de 2009 e Dezembro de 2009, num Departamento de Cirurgia Pediátrica. A revisão da literatura foi realizada através de uma pesquisa na base de dados Medline. Resultados: Foram realizadas oito NLR. A média de idades dos doentes operados foi 4,5 anos (mínimo = 11 meses, máximo = 8,6 anos). As indicações cirúrgicas encontradas foram: quatro rins multiquísticos, três nefropatias de refluxo, uma nefropatia obstructiva. Quatro NLR foram realizadas à esquerda. O tempo cirúrgico médio foi 99 minutos (mínimo = 50 minutos, máximo 180 minutos), notando-se um encurtamento do mesmo à medida que aumenta a experiência da equipa. Não houve nenhuma conversão para lombotomia. A média de tempo de internamento foi 1,5 dias (mínimo = 1 dia, máximo = 2 dias). Não se registaram complicações intra-operatórias nem pós-operatórias. Conclusão: A NLR é exequível em crianças de idade inferior a nove anos e deve ser considerado tratamento de eleição na idade pediátrica.
- Reconstruction of velopharyngeal sphincter in secondary Cleft Palate: surgical alternativePublication . Recamán, M.; Bonet, B.; Leitão, J.; Mesquita, A.Cir Pediatr. 2006 Apr;19(2):106-10. [Reconstruction of velopharyngeal sphincter in secondary Cleft Palate: surgical alternative] [Article in Spanish] Recamán M, Bonet B, Leitão J, Mesquita A. Hospital Central e Especializado de Crianças Maria Pia, Serviço de Cirurgía Plástica. monicarec@iol.pt Abstract There are numerous surgical procedures for the repair of Cleft Palate (CP). Since 1998, in children with CP we use a modified Wardill-Kilner technique, with a large section of the nasal mucous layer at the level of the muscular insertion on the hard palate and lateral nasopharingeal wall, obtaining a push-back and reorientation of the muscular fibres without dissection, diminishing this way the risks of haemorrhages and fibrosis, simplifying the intervention. It allows a lower operative time and a short internment. The aim of our study was to evaluate the results of this operative procedure specially in the development of the speech in 73 children operated on from 1998 until 2000 in our hospital. We verify a competence of the velopharingeal sphincter with ideal results in speech in 88,8% of the cases. PMID: 16846134 [PubMed - indexed for MEDLINE
- Testículos no palpables: experiencia del Hospital Central Especializado de Crianças Maria PiaPublication . Bonet, B.; Recaman, M.; Ferreira-Sousa, J.; Carvalho, F.; Enes, C.; Cidade-Rodrigues, J.La incidencia de criptorquidia es del 1% en niños de 1 año de edad, siendo en el 20% de los casos el testículo no palpable. El objetivo de este trabajo fue estudiar la contribución de la cirugía mínimamente invasiva en el diagnóstico y tratamiento de estos pacientes. Los autores realizaron un estudio prospectivo, analítico y longitudinal durante el período de enero de 2001 a diciembre de 2004, englobando 89 niños portadores de 98 testículos no palpables sometidos a laparoscopia. Los autores analizaron los siguientes parámetros: localización del testículo no palpable, examen objetivo bajo anestesia general ecografía inguinoescrotal, edad de la intervención, hallazgos laparoscópicos, tratamiento efectuado, resultados anatomopatológicos de las piezas extirpadas y seguimiento después de la cirugía.
- TRANSESOPHAGEAL RIGHT UPPER PULMONARY LOBECTOMY - IN VIVO PORCINE EXPERIMENTAL STUDYPublication . Moreira-Pinto, J.; Ferreira, A.; Miranda, A.; Rolanda, C.; Correia-Pinto, J.TRANSESOPHAGEAL RIGHT UPPER PULMONARY LOBECTOMY - IN VIVO PORCINE EXPERIMENTAL STUDY João Moreira-Pinto, MD1,2,3; Aníbal Ferreira, MD1,2,4; Alice Miranda, DVM1,2; Carla Rolanda, MD, PhD1,2,4; Jorge Correia-Pinto, MD, PhD1,2,5 1Surgical Sciences Research Domain, Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, Braga, Portugal; 2ICVS/3B’s - PT Government Associate Laboratory, Braga/Guimarães, Portugal; 3Pediatric Surgery division, Centro Hospitalar do Porto, Porto, Portugal; 4Department of Gastroenterology, Hospital de Braga, Braga, Portugal; 5Pediatric Surgery division, Hospital de Braga, Braga, Portugal. Background and Study Aims Video-assisted thoracoscopic surgery (VATS) has been widespread as the better approach to carry out pulmonary lobectomy. Natural Orifice Transluminal Endoscopic Surgery (NOTES) is being assessed as an alternative to the transthoracic endoscopic surgery. We designed this study to test the feasibility of peroral transesophageal right upper pulmonary lobectomy with the assistance of a single transthoracic trocar. Material and Methods In ten anesthetized pigs (35-45 Kg), we performed right upper pulmonary lobectomy using a forward viewing single-channel gastroscope (introduced perorally) and an operative thoracoscope with a 5 mm working channel (introduced through a single-transthoracic 10 mm port) (Karl Storz). After introducing the gastroscope throughout an oroesophageal overtube into the esophagus, a 1 cm transverse esophagotomy was carried out in the upper third using an ESD-knife under thoracoscope control. Anatomic dissection of the right upper hilum was performed using flexible (gastroscope) and rigid (thoracoscope) instruments. After individual dissection, right upper pulmonary arteries, veins and correspondent bronchus were independently stapled using a 45-mm long, linear endostapler (EndoPath®, Ethicon Endo-Surgery) introduced through the oroesophageal overtube. After completing the lobe resection using an endoscopic snare with cautery, the specimen was extracted retrogradely through the mouth. The esophagotomy was stitched and tied using Endo Stitch™ (Covidien) and a long knot-pusher, which were handled through the oroesophageal overtube. Results Esophagotomy was performed safely in all animals. Dissection of the right upper lobe hilum elements (arteries, veins and bronchus) was also carried out in all animals without significant problems. Oroesophageal handling of the endostapplers for independent ligation of the hilum elements under transthoracic imaging was surprisingly feasible, reasonably easy to perform and reliable in 7 cases. In two cases ligation of the vessels was en bloc. In one case, severe hemorrhage occurred from incomplete vein ligation, although we could control it using electrocoagulation. Esophagotomy closure was feasible but its reliability was not tested in survival studies. All but one animal were kept alive until the end of the acute experiment when they were sacrificed. Conclusions Transesophageal right upper pulmonary lobectomy using single transthoracic trocar assistance is feasible and it may represent a step towards scarless pulmonary lobectomy. Additional survival studies are necessary to test the reliability of this procedure. Apresentador: João Moreira-Pinto, Médico Interno de Cirurgia Pediátrica, CHP.