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Neurogenic bowel dysfunction in patients with spinal cord injury, myelomeningocele, multiple sclerosis and Parkinson’s disease

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World Journal of Gastroenterology 2011年第46期17卷 5035-5048页

作者： Richard A Awad Experimental Medicine and Motility Unit Gastroenterology Service U-107Mexico City General Hospital

Exciting new features have been described concerning neurogenic bowel dysfunction,including interactions between the central nervous system,the enteric nervous system,axonal injury,neuronal loss,neurotransmission of noxious and non-noxious stimuli,and the fields of gastroenterology and *** with spinal cord injury,myelomeningocele,multiple sclerosis and Parkinson's disease present with serious upper and lower bowel dysfunctions characterized by constipation,incontinence,gastrointestinal motor dysfunction and altered visceral *** cord injury is associated with severe autonomic dysfunction,and bowel dysfunction is a major physical and psychological burden for these *** adult myelomeningocele patient commonly has multiple problems reflecting the multisystemic nature of the *** sclerosis is a neurodegenerative disorder in which axonal injury,neuronal loss,and atrophy of the central nervous system can lead to permanent neurological damage and clinical ***'s disease is a multisystem disorder involving dopaminergic,noradrenergic,serotoninergic and cholinergic systems,characterizedby motor and non-motor ***'s disease affects several neuronal structures outside the substantia nigra,among which is the enteric nervous *** reports have shown that the lesions in the enteric nervous system occur in very early stages of the disease,even before the involvement of the central nervous *** has led to the postulation that the enteric nervous system could be critical in the pathophysiology of Parkinson's disease,as it could represent the point of entry for a putative environmental factor to initiate the pathological *** review covers the data related to the etiology,epidemiology,clinical expression,pathophysiology,genetic aspects,gastrointestinal motor dysfunction,visceral sensitivity,management,prevention and prognosis of neurogenic bowel dysfunction patients with these neurolog

关键词： Neurogenic bowel dysfunction Spinal cordinjury myelomeningocele Multiple sclerosis Parkin-son's disease Central nervous system Enteric nervoussystem

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Lethal Consequences in an Infant with myelomeningocele Following an Inadvertent Treatment

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Journal of Forensic Science and Medicine 2016年第3期2卷 164-166页

作者： Xuemei Wu Libing Yun Min Liu Xufu Yi Department of Forensic Pathology West China School of Preclinical and Forensic MedicineSichuan UniversityChengduSichuanChina

myelomeningocele(MMC)is a protrusion of spinal cord contents and meninges through a vertebral *** deaths of patients with MMC are rarely encountered in forensic *** our case,a 3‑month‑old female was born with a lumbosacral cyst,the size of which had been increasing gradually over *** was no neurological,orthopedic,or urologic *** the day of her death,she received a repetitive and rapid lumbosacral cyst puncture drainage procedure,performed by an illegal medical *** autopsy findings confirmed a diagnosis of MMC and the cause of death to be cerebellar tonsillar *** is a pathetic case of preventable infant *** report suggests that the possibility of MMC should be considered in infants born with a lumbosacral cyst,and aspiration is ***,forensic autopsy has a valuable role in determining the exact cause of death,identifying,or excluding iatrogenic factors that may be relevant to death following a medical procedure.A final point is that prevention programs should be developed,especially by the health care sectors to reduce such tragedy.

关键词： Autopsy cerebellar tonsillar herniation iatrogenic death inadvertent treatment myelomeningocele

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Recto-Anal Biofeedback Treatment and Quality of Life in Children with myelomeningocele

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Open Journal of Epidemiology 2019年第3期9卷 202-212页

作者： C. E. Zubiri S. W. Miculan A. Zosi G. I. Nanfito L. Guzman P. Borobia R. Bigliardi C. Arregui V. Bernedo C. Otegui V. Valdiviezo M. Allende M. A. Savia L. Menendez A. Besga S. Morales R. Drut S. G. Tobia Gonzalez J. M. Dominguez A. G. Tortarolo H. A. Longuinho Y. R. Gomez T. Gonzalez Pediatric Hospital “Sor Maria Ludovica” La Plata Buenos Aires Argentina Posadas Hospital Buenos Aires Argentina

Introduction: myelomeningocele is one of the most complex birth defects that cause physical disability, with consequent fecal incontinence and therefore difficulty in social integration of these patients. Objective: To improve the quality of life and manometric values after biofeedback therapy. Method: Longitudinal, prospective, analytical and experimental study. Patients with myelomeningocele and fecal incontinence who were between 5 to 15 years old and their parents were included in the study. Child and parent reports of PedsQMtm generic questionnaire were collected after obtaining informed consent and assent. Anorectal manometry and first biofeedback sessions were held with each child. Following treatment completion, the PedsQMtm was applied again. Results: 17 children and their parents were included in the study. All the patients presented fecal incontinence and an inability to voluntarily evacuate rectal contents. After biofeedback, the totality of patients improved their fecal incontinence. Nine of them stop using diaper. All reported successful use of the toilet. Statistically significant differences were observed when comparing the quality of life of children and parents at the beginning and at the end of treatment. There was an improvement in quality of life after the treatment with biofeedback for both children and parents, which was more perceived by these. Conclusions: It is highly significant for the improvement both in clinic and manometric values. The improvement in quality of life is more evident in the parents.

关键词： Biofeedback Fecal Incontinence Manometry myelomeningocele Quality of Life

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The Timing of Primary Neurosurgical Repair and Wound-Site Infection in Children with myelomeningocele

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Open Journal of Modern Neurosurgery 2024年第2期14卷 137-148页

作者： Joseph O. Obande Paul T. Bitrus Elizabeth I. Obande Division of Neurosurgery Department of Surgery University of Abuja Teaching Hospital Abuja Nigeria Post-Basic School of Critical Care Nursing University of Abuja Teaching Hospital Abuja Nigeria

Background: The optimal time to closure of a newborn with a myelomeningocele has been the focus of a number of evaluations. The Timing of primary surgery has received significant attention due to its relationship to repair-site infection that can lead to increased morbidity and prolonged hospital stays. It is on this basis that recommendations have utilized 48 - 72 hours post birth as ideal time of closure. This is not only prevent infection at the site but also prevent ventriculitis and neural structure damage. We therefore, hypothesized an increase in wound infection rates in those patients with delays in myelomeningocele repair. Methods: We retrospectively reviewed the records of 103 children with myelomeningocele treated between 2016 and 2023. At discharge the patients were followed up at the post-operative clinic visit 2 weeks later. Children were assigned to 1 of 2 groups, those who underwent primary neurosurgical repair within 72 hours of delivery (Group 1) and those undergoing repair after 72 hours (Group 2). We compared the infection rates. Results: 103 children who underwent myelomeningocele repair were identified, with a median time from birth to treatment of 1 day. Eight (7.8 %) patients were noted to have post-repair surgical site complications. There was no significant difference in rates of infection between Group 1 and Group 2 repair times. The presence of infection was associated increased length of stay when compared to neonates without infection. Conclusion: In children with myelomeningocele, the timing of primary neurosurgical repair appears not to have a significant impact on surgical site infection. Closure of the spinal lesion within the first 72 hours of life may be more favorable for neural damage prevention. These results suggest that early myelomeningocele repair may not impart significantly on the rate of wound-site infection.

关键词： Spina Bifida Surgical Timing Excision and Repair Surgical Site Infection myelomeningocele

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Demographic and Clinical Characteristics of 63 Children with myelomeningoceles

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Open Journal of Modern Neurosurgery 2021年第2期11卷 59-64页

作者： Youssouf Sogoba Seybou Hassane Diallo Issa Amadou Boubacar Sogoba Moussa Diallo Benoi Kamaté Daouda Mariko Coulibaly Oumar Quenum Kisito Hawa Diall Leonie Diakité Salimata Diallo Belco Maiga Fousseyni Traoré Karamoko Sacko Oumar Diallo Drissa Kanikomo Youssoufa Maiga Department of Neurosurgery H&#244 pital Gabriel Touré Bamako Mali Department of Neurology H&#244 pital Gabriel Touré Bamako Mali Department of Pediatric Surgery H&#244 pital Gabriel Touré Bamako Mali Department of Neurosurgery H&#244 pital du Mali Bamako Mali Department of Neurosurgery University Hospital of Parakou Parakou Benin Department of Pediatrics H&#244 pital Gabriel Touré Bamako Mali

Background: myelomeningocele (MMC) is the most common neural tube defect (NTD) characterized by the extrusion of the spinal cord into a sac filled with cerebrospinal fluid, resulting in lifelong disability. In the general population, the incidence of MMC ranges from 0.3 to 4.5/10,000 births. Live born infants with myelomeningocele have a death rate of approximately 10%. Many factors may play a role in the development of MMC such as environmental and genetic factors. In this study, we present our experience with a group of 63 children afflicted with MMC. Methods: This study was a retrospective analysis of 63 patients with MMC admitted to the neurosurgical department of Gabriel Touré Hospital from September 2017 to August 2018. A detailed history was obtained from the family at presentation. The family history and medical information before and during the pregnancy were compiled. Patients underwent complete physical and neurological examinations. Forty-seven (74.60%) patients underwent repair of the MMC and a ventriculoperitoneal shunt was placed in 12 (19%) patients with accompanying hydrocephalus. The risk factors, neurological status, and surgical results have been analyzed. Results: Of 63 children with MMC admitted to our neurosurgical department, 34 (54%) were boys and 29 (46%) were girls. Forty (63.49%) patients were the children of marriages of second cousins or closer. The mean age of the fathers was 34 years (16 - 65), while that of mothers was 26 years (16 - 38). The pregnancy was unplanned in all cases. Fourteen (22.22%) mothers had genitourinary infections, 9 (14.3%) had malaria and 57 (90.47%) mothers used analgesics and antibiotics during the pregnancy. Fifty-nine (93.65%) children were born at term, 58 (92%) were delivered via normal spontaneous vaginal delivery, and 5 (8%) via cesarean section. Lumbosacral lesions were the most frequent in 27 (42.86%) patients. Forty-seven (74.60%) patients underwent r

关键词： myelomeningocele Neural Tube Defects Hydrocephalus

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Assessment on self-care, mobility and social function of children with spina bifida in Turkey

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Neural Regeneration Research 2014年第12期9卷 1234-1240页

作者： Hulya Sirzai Beril Dogu Selamet Demir Figen Yilmaz Banu Kuran Ankara Physical Medicine and Rehabilitation Education and Research Hospital Department of Physical Medicine and Rehabilitation Sisli Etfal Education and Research Hospital

The aim of the study was to investigate the functional performance in children with spina bifida, using the Pediatric Evaluation of Disability Inventory （PEDI） to look into capacity of twen- ty-eight children with spina bifida with lesions at different levels in different dimensions of self- care, mobility and social function. Mean age of the patients was 3.5 ± 2.3 （1-10） years. In the muscle test carried out, 13 patients （44.8%） had no movements including pelvic elevation in lower extremity muscles and they were at level 5. Sixteen patients （54%） were non-ambulatory according to the Hoofer ambulation classification. Raw and scale scores in the self-care, mobil- ity and social function domains both in the functional skill scale and in the caregiver scale were found to be lower compared to the data of the normal population. A statistically significant correlation was observed in the self-care values of the Functional Skills Scales and the Caregiver Assistance Scale measurements, which was positive for age and negative for Functional Ambu- lation Scale and muscle test （P 〈 0.05）. A positive relation was found between the Functional Skills Scales-mobility area and age while a negative relation was observed between Functional Ambulation Scale and muscle test （P 〈 0.005）. A negative relation was also found between Care- giver Assistance Scale-mobility and Functional Ambulation Scale and muscle test （P 〈 0.005）. In our study, the functional performance of the children was found to be low. Low-level lesions, encouraging muscular strength and independence in mobility are all very important factors for functional independence.

关键词： nerve regeneration spina bifida myelomeningocele disability evalutatton self-care mobility muscular energy child social function neural regeneration

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LL-37 induced cystitis and the receptor for advanced glycation end-products (RAGE) pathway

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Advances in Bioscience and Biotechnology 2013年第8期4卷 1-8页

作者： Lindsi McCoard Roundy Wanjian Jia Jianxing Zhang Xiangyang Ye Glenn D. Prestwich Siam OottamasathienQ Department of Medicinal Chemistry and Center for Therapeutic Biomaterials University of Utah Salt Lake City USA Department of Pharmacotherapy Primary Children’s Medical Center University of Utah Salt Lake City USA Department of Surgery and Division of Pediatric Urology Primary Children’s Medical Center Salt Lake City USA

To elucidate pathways in bladder inflammation, we employed our physiologically relevant LL-37 induced cystitis model. Based on inflammatory studies involving other organ systems implicating the receptor for advanced glycation end-products (RAGE), we first hypothesized that RAGE is critically involved in LL-37 induced cystitis. We further hypothesized that?a common RAGE ligand high mobility group box 1 (HMGB1) is up-regulated in bladders challenged with LL-37. Finally, we hypothesized that NF-κB dependent inflammatory genes are activated in LL-37 induced cystitis. Testing our first hypothesis, C57Bl/6 mice were challenged with either saline (control) or 320 μM of LL-37 intravesically for 1 hr. After 12 or 24 hours, tissues were examined with immunohistochemistry (IHC) for RAGE, and both mRNA and protein isolation for respective qRT-PCR and Western Blot analysis. Our second hypothesis was tested by employing HMGB1 IHC. Testing our final hypothesis, qRT-PCR was performed investigating five genes: TNFα, IL-6, IL-1β, GM-CSF, COX-2. In control and LL-37 challenged tissues, IHC for RAGE revealed similar qualitative expression. Evaluation with qRT-PCR and Western Blot for RAGE revealed diminished expression at the mRNA and protein level within LL-37 challenged bladders. IHC for HMGB1 revealed a moderate qualitative increase within LL-37 challenged tissues. Finally, with the exception of TNFα, all NF-κB dependent inflammatory genes yielded substantial up-regulation. We have employed our LL-37 induced cystitis model to gain insight to wards a possible mechanistic pathway involved in bladder inflammation. This work provides data for future studies involving the inflammatory ligand HMGB1, RAGE, and receptor pathways that activate NF-κB.

关键词： LL-37 Cathelicidin Bladder Inflammation Bladder Fibrosis Spina Bifida myelomeningocele Interstitial Cystitis RAGE HMGB1 NF-Kappa B

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Bone Mineral Density and Vitamin D Status in Children with Chronic Neurological Syndromes—Clinical Observations

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Case Reports in Clinical Medicine 2018年第5期7卷 315-323页

作者： Elzbieta Jakubowska-Pietkiewicz Gabriela Orzechowska Elzbieta Wozniak Joanna Kudzin Ewa Toporowska-Kowalska Department of Paediatric Propaedeutics and Bone Metabolism Diseases Medical University of LodzLodzPoland Department of Paediatric Allergology Gastroenterology and NutritionMedical University of LodzLodzPoland

Background: Some of the risk factors for osteoporosis population include: chronic immobilisation, insufficient dietary supply of calcium and vitamin D, decreased physical activity and long-term pharmacological treatment (glucocorticoids, anticonvulsant drugs). In disabled children and adolescents, the negative impact of these factors may cumulate to considerably impair the quality of life. Objectives: The aim of our study was to assess the vitamin D status and bone mineral density in children with chronic neurological syndromes. Material and Methods: A total of 34 children between 3 and 18 years of age were examined: 9 children with muscular dystrophy, 17 with cerebral palsy and 8 with lumbar myelomeningocele. All the subjects underwent the following assessments: measurement of the concentration of the hepatic metabolite of vitamin D and total body less head and/or lumbar spine densitometry by dual energy X-ray absorptiometry (DEXA). Low bone mass or low bone mineral density was diagnosed if the Z-score value was found to be equal to or below −2.0. Results: Indications for the above tests were chronic immobilisation or motor activity restriction, and—in 10/34 children—femoral or vertebral fracture. Vitamin D deficiency (81%). Low bone mass on densitometry was demonstrated in 27/34 and osteoporosis in 10/34 subjects (Z-score ≤−2.0). Conclusion: Bone densitometry should be included in the standard of care for children with chronic neurological syndromes, and early detection of low bone mass should be an indication for treatment with calcium and vitamin D.

关键词： Children Densitometry Cerebral Palsy Muscular Dystrophy myelomeningocele

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In utero and exo utero fetal surgery on histogenesis of organs in animals

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World Journal of Surgical Procedures 2015年第2期5卷 198-207页

作者： Esrat Jahan Ashiq Mahmood Rafiq Hiroki Otani Department of Developmental Biology Faculty of Medicine Shimane University

Until recently, fetal surgery was only used for fetuses with very poor prognosis who were likely to die without intervention. With advances in imaging, endoscopic techniques, anesthesia and novel interventions, fetal surgery is becoming a realistic option for conditions with less severe prognoses, where the aim is now to improve quality of life rather than simply allow survival. Until forty years ago, the uterus shielded the fetus from observation and therapy. Rapid changes in the diagnosis and treatment of human fetal anatomical abnormalities are due to improved fetal imaging studies, fetal sampling techniques(e.g., amniocentesis and chorionic villus sampling), and a better understanding of fetal pathophysiology derived from laboratory animals. Fetal therapy is the logical culmination of progress in fetal diagnosis. In other words, the fetus is now a patient. Now-a-days, in utero(IU) and exo utero(EU) surgical methods are popular for experimental analyses of the histogenesis of organ development. Using these surgical methods, developmental anomalies can be created and then repaired. By applying microinjection and/or fetal surgery with these methods, models of developmental anomalies such as neural tube defects, temporomandibular joint defects, hip joint defects, digit amputation, limb and digit development and regeneration, and tooth germ transplantation in the jaw could be created and later observed. After observing different types of anomalies, novel IU and EU surgical techniques would be the best approach for repairing or treating those anomalies or diseases. This review will focus on the rationale for the IU and EU creation of animal models of different organ defects or anomalies and their repair, based on analyses of organ histogenesis and pathologic observations. It will also focus in detail on the surgical techniques of both IU and EU methods.

关键词： myelomeningocele Microinjection Rodent Sheep Neural tube defect Temporomandibular joint Fetal surgery In utero Exo utero

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Pattern and Management of Neural Tube Defect in Cameroon

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Open Journal of Modern Neurosurgery 2017年第3期7卷 87-102页

作者： Mathieu Motah Mireille Moumi Aurélien Ndoumbe Clerence Ntieafac Vincent De Paul Djienctheu Department of Surgery Faculty of Medicine and pharmaceutical sciences University of Douala Douala Cameroon Neurosurgical Unit General Hospital of Douala Douala Cameroon Faculty of Medicine and Biomedical Sciences University of Yaoundé Yaounde Cameroon Service of Neurosurgery Central Hospital of Yaoundé Yaoundé Cameroon

Objectives: The aim of study was to determine the pattern and management of neural tube defects (NTD). Methodology: It was a hospital based descriptive cross-sectional retrospective study on patients who consulted and/or were admitted at the Douala General hospital for neural tube defects from January 2005 to April 2015. Results: A total of forty-nine (49) patients were enrolled. Males constituted 59.8% and females 40.2% giving a sex ratio of 1.5 in favour of males. Most of the parents of the patients (71.5%) had a low socio-economic status. myelomeningocele was the most common type (80.4%) followed by 17.4% cases of meningocele and 2.2% cases of lipomeningocele. Three cases (3) of encephaloceles were seen during this period. The commonest site of these defects was the lumbosacral region (47.8%). Other sites included lumbar (19;41.3%), sacral (3;6.5%) and thoracolumbar (2;4.3%) ones. About half of the patients (24;48.9%) presented with ruptured lesions. Hydrocephalus was also recorded in 65.3% of patients. Talipes equinovarus and talipes calcaneovalgus were the most common associated orthopedic birth defects found. Surgical closure was done for 44 (89.9%) patients. Ventriculoperitoneal shunting was done in 78.1% of those who presented with hydrocephalus. Post-operative complications were more frequent in patients with ruptured lesions (P = 0.001). The most common post-operative complications were wound infections (22;44.9%) and wound dehiscence (20;40.8%). Conclusion: Lumbosacral myelomeningocele was the most common type of NTD in our region. Low socio-economic status was a common risk factor.

关键词： Neural Tube Defects myelomeningocele Cameroon

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