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[PMID]:29339865
[Au] Autor:Altiok H; Riordan A; Graf A; Krzak J; Hassani S
[Ad] Endereço:Shriners Hospitals for Children Chicago.
[Ti] Título:Response of Scoliosis in Children with Myelomeningocele to Surgical Release of Tethered Spinal Cord.
[So] Source:Top Spinal Cord Inj Rehabil;22(4):247-252, 2016.
[Is] ISSN:1945-5763
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:To examine the effect of surgical tethered cord release (TCR) on scoliosis in children with myelomeningocele. A retrospective review of 65 pediatric patients with myelomeningocele and TCR. The final sample consisted of 20 patients with scoliosis who were managed conservatively after TCR. Average age at TCR was 6.2 years with average follow-up of 3.8 years. Scoliosis of 1 (5%) patient improved, 7 (35%) were stable, and 12 (60%) worsened (≥10°). Fifty percent of patients ultimately required definitive spinal surgery. TCR release delayed definitive spine surgery for an average of 3.2 years. Sixty-four percent of patients with curves less than or equal to 45° had progression of their curves compared to 50% with curves greater than 45°. For patients with curves less than or equal to 45°, curves progressed in 80% of those younger than 10 years as compared to 25% of those older than 10 years. For patients with curves less than or equal to 45°, 43% required definitive spine surgery as opposed to 83% with curves greater than 45°. Level of neurological involvement (ie, lumbar versus thoracic) and age at untethering emerged as factors influencing the effects of TCR for patients with curves less than or equal to 45°. Lumbar curves had more favorable results. Pediatric patients with myelomeningocele and scoliosis should be closely assessed and monitored. A selective approach for youth with lumbosacral level myelomeningocele and progressive curves less than or equal to 45° may result in scoliosis stabilization and avoidance of definitive surgery.
[Mh] Termos MeSH primário: Meningomielocele/cirurgia
Escoliose/cirurgia
[Mh] Termos MeSH secundário: Criança
Feminino
Seres Humanos
Masculino
Defeitos do Tubo Neural
Estudos Retrospectivos
Vértebras Torácicas
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180220
[Lr] Data última revisão:
180220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180118
[St] Status:MEDLINE
[do] DOI:10.1310/sci2204-247


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[PMID]:29215525
[Au] Autor:Belfort MA; Whitehead WE; Bednov A; Shamshirsaz AA
[Ad] Endereço:Texas Children's Fetal Center and the Departments of Obstetrics and Gynecology and Neurosurgery, Baylor College of Medicine, Houston, Texas.
[Ti] Título:Low-Fidelity Simulator for the Standardized Training of Fetoscopic Meningomyelocele Repair.
[So] Source:Obstet Gynecol;131(1):125-129, 2018 Jan.
[Is] ISSN:1873-233X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Fetoscopic meningomyelocele repair in a gas-filled uterus is a new technique performed in very few centers. There are few opportunities as well as ethical prohibitions on the initial development and subsequent refinement of innovative fetal surgery techniques in humans, and using an animal model is both very expensive and logistically difficult. METHOD: We developed a low-fidelity endoscopic fetal surgery simulation using a plastic doll and pieces of chicken breast to simulate a fetal meningomyelocele, and a polyurethane ball to simulate a gas-filled uterus, along with a standard endoscopy system and instruments. EXPERIENCE: A unique two-port technique with significant differences from the standard laparoscopic surgery procedure was developed and refined through an iterative phase into a standardized methodology, and the simulator was then used to train three other teams to perform standardized fetoscopic meningomyelocele repair. CONCLUSION: A low-fidelity fetoscopic surgery simulator is a useful tool for developing new fetoscopic operations and for training multidisciplinary fetal surgery teams without the need for extensive use of an animal model. This simulator may be used to further explore the human uterus as a new surgical space for additional fetal surgeries.
[Mh] Termos MeSH primário: Competência Clínica
Fetoscopia/educação
Meningomielocele/cirurgia
Procedimentos Neurocirúrgicos/educação
Treinamento por Simulação/métodos
[Mh] Termos MeSH secundário: Simulação por Computador
Feminino
Fetoscopia/métodos
Seres Humanos
Modelos Anatômicos
Procedimentos Neurocirúrgicos/métodos
Gravidez
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180108
[Lr] Data última revisão:
180108
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171208
[St] Status:MEDLINE
[do] DOI:10.1097/AOG.0000000000002406


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[PMID]:29190654
[Au] Autor:Kancherla V; Ibne Hasan MOS; Hamid R; Paul L; Selhub J; Oakley G; Quamruzzaman Q; Mazumdar M
[Ad] Endereço:Department of Epidemiology, Rollins School of Public Health, Emory University, Atlanta, Georgia, United States of America.
[Ti] Título:Prenatal folic acid use associated with decreased risk of myelomeningocele: A case-control study offers further support for folic acid fortification in Bangladesh.
[So] Source:PLoS One;12(11):e0188726, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Neural tube defects contribute to severe morbidity and mortality in children and adults; however, they are largely preventable through maternal intake of folic acid before and during early pregnancy. We examined the association between maternal prenatal folic acid supplement intake and risk of myelomeningocele (a severe and common type of neural tube defect) in the offspring. We performed secondary analysis using data from a case-control study conducted at Dhaka Community Hospital, Bangladesh between April and November of 2013. Cases and controls included children with and without myelomeningocele, respectively, and their mothers. Cases were identified from local hospitals and rural health clinics served by Dhaka Community Hospital. Controls were selected from pregnancy registries located in the same region as the cases, and matched (1:1) to cases by age and sex. Myelomeningocele in the offspring was confirmed by a pediatrician with expertise in classifying neural tube defects. Maternal prenatal folic acid supplement intake was the main exposure of interest. We estimated crude and adjusted odds ratios (OR) and 95% confidence intervals (CI) using conditional logistic regression analysis. There were 53 pairs of matched cases and controls in our study. Overall, 51% of case mothers reported using folic acid supplements during pregnancy compared to 72% of control mothers (p = 0.03). Median plasma folate concentrations at the time of study visit were 2.79 ng/mL and 2.86 ng/mL among case and control mothers, respectively (p = 0.85). Maternal prenatal folic acid use significantly decreased the odds of myelomeningocele in the offspring (unadjusted OR = 0.42, 95% CI = 0.18-0.96). The association was slightly attenuated after adjusting for maternal age at the time of pregnancy (adjusted OR = 0.43, 95% CI = 0.18-1.02). Our study confirms the protective association between maternal prenatal folic acid supplement use and myelomeningocele among children born in Bangladesh. Our findings point to an overall low folic acid supplement use and low plasma folate concentrations among women of reproductive age in Bangladesh. Mandatory fortification of staple foods with folic acid can address low folate status among women of child-bearing age, and prevent child morbidity and mortality associated with myelomeningocele in Bangladesh.
[Mh] Termos MeSH primário: Ácido Fólico/administração & dosagem
Meningomielocele/prevenção & controle
Cuidado Pré-Natal
[Mh] Termos MeSH secundário: Adolescente
Adulto
Bangladesh
Estudos de Casos e Controles
Feminino
Seres Humanos
Recém-Nascido
Gravidez
Fatores de Risco
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
935E97BOY8 (Folic Acid)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171201
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0188726


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[PMID]:28832491
[Au] Autor:Committee on Obstetric Practice, Society for Maternal­Fetal Medicine
[Ti] Título:Committee Opinion No. 720: Maternal-Fetal Surgery for Myelomeningocele.
[So] Source:Obstet Gynecol;130(3):e164-e167, 2017 09.
[Is] ISSN:1873-233X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. Women with pregnancies complicated by fetal myelomeningocele who meet established criteria for in utero repair should be counseled in a nondirective fashion regarding all management options, including the possibility of open maternal-fetal surgery. Maternal-fetal surgery for myelomeningocele repair should be offered only to carefully selected patients at facilities with an appropriate level of personnel and resources.
[Mh] Termos MeSH primário: Meningomielocele/cirurgia
Procedimentos Cirúrgicos Obstétricos/normas
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Obstetrícia
Gravidez
Resultado da Gravidez
Sociedades Médicas
Estados Unidos
[Pt] Tipo de publicação:JOURNAL ARTICLE; PRACTICE GUIDELINE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170908
[Lr] Data última revisão:
170908
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170824
[St] Status:MEDLINE
[do] DOI:10.1097/AOG.0000000000002303


  5 / 3574 MEDLINE  
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[PMID]:28832482
[Ti] Título:Committee Opinion No. 720 Summary: Maternal-Fetal Surgery for Myelomeningocele.
[So] Source:Obstet Gynecol;130(3):672-673, 2017 Sep.
[Is] ISSN:1873-233X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. Women with pregnancies complicated by fetal myelomeningocele who meet established criteria for in utero repair should be counseled in a nondirective fashion regarding all management options, including the possibility of open maternal-fetal surgery. Maternal-fetal surgery for myelomeningocele repair should be offered only to carefully selected patients at facilities with an appropriate level of personnel and resources.
[Mh] Termos MeSH primário: Meningomielocele/cirurgia
Procedimentos Cirúrgicos Obstétricos/normas
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Obstetrícia
Gravidez
Resultado da Gravidez
Sociedades Médicas
Estados Unidos
[Pt] Tipo de publicação:JOURNAL ARTICLE; PRACTICE GUIDELINE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170908
[Lr] Data última revisão:
170908
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170824
[St] Status:MEDLINE
[do] DOI:10.1097/AOG.0000000000002294


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[PMID]:28716320
[Au] Autor:Cardona-Grau D; Chiang G
[Ad] Endereço:Pediatric Urology, University of California San Diego, Rady Children's Hospital, 3020 Children's Way MC 5120, San Diego, CA 92123, USA.
[Ti] Título:Evaluation and Lifetime Management of the Urinary Tract in Patients with Myelomeningocele.
[So] Source:Urol Clin North Am;44(3):391-401, 2017 Aug.
[Is] ISSN:1558-318X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:In the United States, there are an estimated 25,000 children ages 0 to 19 years and about 166,000 of all ages currently affected by spina bifida. Management is multimodal and can be complex. Management techniques vary throughout a child's lifetime, but the goals remain the same: prevention of urinary tract infections and establishing acceptable continence. Continence is addressed as the child reaches school age. Additional considerations such as development of urolithiasis and the associated burden are highlighted in adolescence and into adulthood. These complex medical needs become more challenging as patients age and need to transition to adult providers.
[Mh] Termos MeSH primário: Meningomielocele/complicações
Doenças Urológicas/diagnóstico
Doenças Urológicas/terapia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Fatores Etários
Criança
Pré-Escolar
Seres Humanos
Lactente
Recém-Nascido
Vigilância da População
Transição para Assistência do Adulto
Doenças Urológicas/etiologia
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170803
[Lr] Data última revisão:
170803
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170719
[St] Status:MEDLINE


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[PMID]:28559064
[Au] Autor:Rei J; Pereira J; Reis C; Salvador S; Vaz R
[Ad] Endereço:Faculty of Medicine of the University of Porto, Oporto, Portugal. Electronic address: rei.joana13@gmail.com.
[Ti] Título:Endoscopic Third Ventriculostomy for the Treatment of Hydrocephalus in a Pediatric Population with Myelomeningocele.
[So] Source:World Neurosurg;105:163-169, 2017 Sep.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although endoscopic third ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiologic outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 pediatric patients with myelomeningocele treated with ETV from 1998 to 2015 at the Centro Hospitalar São João (Porto, Portugal) were reviewed retrospectively. Patients' caregivers were contacted to evaluate their clinical manifestations before and after surgery regarding signs and symptoms of hydrocephalus and Chiari malformation. Control neuroradiologic imaging of 9 patients was obtained and analyzed. Success of ETV was defined by clinical resolution and radiologic confirmation. RESULTS: ETV was successful in 8 of 18 cases (44.4%). Groups of patients were compared according to age at the time of surgery, with a 40% (2/5) success rate in newborns and a 50% success rate (3/6) in children older than 1 year. Eight patients underwent ETV as a first option, with a 37.5% success rate. Ten patients underwent the procedure after previous ventriculoperitoneal shunt (VPS), 5 for malfunction and 5 for VPS infection with 60% and 40% success rates, respectively. Early postoperative complications occurred in 2 patients. CONCLUSIONS: ETV can be performed in patients with myelomeningocele and hydrocephalus with success rates of almost 50%. Prior VPS or VPS malfunction or infection do not contraindicate ETV. If possible, the procedure should be delayed until the patient is at least 1 month old.
[Mh] Termos MeSH primário: Hidrocefalia/etiologia
Hidrocefalia/cirurgia
Meningomielocele/complicações
Terceiro Ventrículo/cirurgia
Ventriculostomia/métodos
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Endoscópios
Feminino
Seres Humanos
Hidrocefalia/diagnóstico por imagem
Lactente
Recém-Nascido
Estudos Longitudinais
Imagem por Ressonância Magnética
Masculino
Meningomielocele/diagnóstico por imagem
Estudos Retrospectivos
Terceiro Ventrículo/diagnóstico por imagem
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170929
[Lr] Data última revisão:
170929
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170601
[St] Status:MEDLINE


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[PMID]:28436679
[Au] Autor:Li B; Pu T; Liu Y; Xu Y; Xu R
[Ad] Endereço:1 Department of Pediatric Cardiology, Shanghai Jiaotong University School of Medicine Xinhua Hospital , Shanghai, China .
[Ti] Título:CITED2 Mutations in Conserved Regions Contribute to Conotruncal Heart Defects in Chinese Children.
[So] Source:DNA Cell Biol;36(7):589-595, 2017 Jul.
[Is] ISSN:1557-7430
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Conotruncal heart defects (CTDs) are severe malformations of outflow tract with heterogeneous morphology. Several missense variants of CITED2 have been identified to cause CTDs in recent researches. In this study, we screened the coding regions of CITED2 in 605 Chinese children with CTDs and found two possible pathogenic mutant sites: p.Q117L and p.T257A, both located in the conserved regions of CITED2. Then, we investigated the biological and functional alterations of them. Western blotting showed low level of protein expression of mutant Q117 and T257A compared with wild-type CITED2. Dual-luciferase reporter assay demonstrated that mutant Q117 and T257A decreased the ability of CITED2 to modulate the expression of paired-like homeodomain transcription factor 2 gamma (PITX2C), which are closely related to cardiac growth and left-right patterning. Meanwhile, T257A also exhibited impaired ability to mediate vascular endothelial growth factor expression, another gene closely associated with the normal development of cardiovascular system. Three-dimensional molecular conformation showed reduced hydrogen bond between Asp254 and mutant Thr257, indicating the weakened stability and binding ability of CITED2. All these results suggest that CITED2 mutations in conserved regions lead to disease-causing biological and functional changes and may contribute to the occurrence of CTDs.
[Mh] Termos MeSH primário: Anormalidades Múltiplas/genética
Cardiopatias Congênitas/genética
Proteínas de Homeodomínio/genética
Meningomielocele/genética
Mutação de Sentido Incorreto
Proteínas Repressoras/genética
Transativadores/genética
Fatores de Transcrição/genética
Fator A de Crescimento do Endotélio Vascular/genética
[Mh] Termos MeSH secundário: Anormalidades Múltiplas/classificação
Anormalidades Múltiplas/etnologia
Anormalidades Múltiplas/patologia
Sequência de Aminoácidos
Animais
Grupo com Ancestrais do Continente Asiático
Linhagem Celular
Criança
Sequência Conservada
Regulação da Expressão Gênica no Desenvolvimento
Cardiopatias Congênitas/classificação
Cardiopatias Congênitas/etnologia
Cardiopatias Congênitas/patologia
Proteínas de Homeodomínio/metabolismo
Seres Humanos
Ligações de Hidrogênio
Meningomielocele/classificação
Meningomielocele/etnologia
Meningomielocele/patologia
Camundongos
Modelos Moleculares
Mioblastos/citologia
Mioblastos/metabolismo
Fases de Leitura Aberta
Conformação Proteica
Estabilidade Proteica
Proteínas Repressoras/química
Proteínas Repressoras/metabolismo
Alinhamento de Sequência
Homologia de Sequência de Aminoácidos
Transativadores/química
Transativadores/metabolismo
Fatores de Transcrição/metabolismo
Fator A de Crescimento do Endotélio Vascular/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (CITED2 protein, human); 0 (Homeodomain Proteins); 0 (Repressor Proteins); 0 (Trans-Activators); 0 (Transcription Factors); 0 (VEGFA protein, human); 0 (Vascular Endothelial Growth Factor A); 184787-43-7 (homeobox protein PITX2)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170920
[Lr] Data última revisão:
170920
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170425
[St] Status:MEDLINE
[do] DOI:10.1089/dna.2017.3701


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[PMID]:28433844
[Au] Autor:Matsuda S; Yamaguchi S; Kajihara Y; Takeda M; Kolakshyapati M; Kurisu K
[Ad] Endereço:Department of Neurosurgery, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan. Electronic address: 1108shingo@gmail.com.
[Ti] Título:Neurologic Decline in an Older Patient with Repaired Myelomeningocele Complicated with Lumbar Canal Stenosis.
[So] Source:World Neurosurg;103:952.e1-952.e4, 2017 Jul.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Tethered cord syndrome is a well-known complication after myelomeningocele (MMC) repair in childhood. However, late complications in adults with a repaired MMC are not well understood. In particular, the influence of a degenerative spinal deformity on a sustained tethered cord is still unclear. CASE DESCRIPTION: A 63-year-old man with a repaired MMC exhibited a progressive gait disturbance and numbness in both lower limbs. Magnetic resonance imaging demonstrated that the tethered spinal cord was compressed by severe canal stenosis along the entire lumbar spine. After a multi-level lumbar decompression surgery, the patient recovered to baseline neurologic status. CONCLUSION: In adults with a repaired MMC, lumbar canal stenosis should be investigated as a possible cause of late neurologic decline. Clinical manifestations may be complicated by the coexistence of both the original and subsequent neurologic disorders. Because these additional disorders result from compressive myelopathy, early surgical decompression is indicated to avoid irreversible spinal cord dysfunction.
[Mh] Termos MeSH primário: Vértebras Lombares/cirurgia
Meningomielocele/cirurgia
Defeitos do Tubo Neural/diagnóstico por imagem
Complicações Pós-Operatórias/diagnóstico por imagem
Compressão da Medula Espinal/diagnóstico por imagem
Estenose Espinal/diagnóstico por imagem
[Mh] Termos MeSH secundário: Descompressão Cirúrgica
Transtornos Neurológicos da Marcha/etiologia
Seres Humanos
Hipestesia/etiologia
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Mielografia
Defeitos do Tubo Neural/complicações
Defeitos do Tubo Neural/cirurgia
Complicações Pós-Operatórias/cirurgia
Compressão da Medula Espinal/complicações
Compressão da Medula Espinal/cirurgia
Estenose Espinal/complicações
Estenose Espinal/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170424
[St] Status:MEDLINE


  10 / 3574 MEDLINE  
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[PMID]:28432425
[Au] Autor:Nair RP; Kongwad LI
[Ad] Endereço:Department of Neurosurgery, Kasturba Medical College, Manipal University, Manipal, India. rajeshnair39@yahoo.com.
[Ti] Título:The problem with myelomeningoceles, hydrocephalus, and progressive neurological deficits-what do we know?
[So] Source:Childs Nerv Syst;33(6):891-892, 2017 06.
[Is] ISSN:1433-0350
[Cp] País de publicação:Germany
[La] Idioma:eng
[Mh] Termos MeSH primário: Hidrocefalia/cirurgia
Meningomielocele/cirurgia
[Mh] Termos MeSH secundário: Seres Humanos
[Pt] Tipo de publicação:LETTER; COMMENT
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170926
[Lr] Data última revisão:
170926
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170423
[St] Status:MEDLINE
[do] DOI:10.1007/s00381-017-3420-3



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