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 n...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 neurology.Patients 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 sensitivity.Spinal cord injury is associated with severe autonomic dysfunction,and bowel dysfunction is a major physical and psychological burden for these patients.An adult myelomeningocele patient commonly has multiple problems reflecting the multisystemic nature of the disease.Multiple 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 disability.Parkinson's disease is a multisystem disorder involving dopaminergic,noradrenergic,serotoninergic and cholinergic systems,characterizedby motor and non-motor symptoms.Parkinson's disease affects several neuronal structures outside the substantia nigra,among which is the enteric nervous system.Recent 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 system.This 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 process.This 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 neurological diseases.Embryological,morphological and ex展开更多
Background: Spinal dysraphism represents a wide spectrum of congenital abnormalities of the spine. Myelomeningocele is considered the most common malformation and the most common we saw in our community, with its morb...Background: Spinal dysraphism represents a wide spectrum of congenital abnormalities of the spine. Myelomeningocele is considered the most common malformation and the most common we saw in our community, with its morbidity problems seen commonly in the postoperative period. ASQ-3<sup>TM</sup> Scores are the ages and stages questionnaire, third edition, and represent a tool to assess the development progress, especially in toddlers. Objectives: Evaluation of neurodevelopmental outcome among Sudanese toddlers with spinal dysraphism after surgical closure with or without a VP shunt using ASQ-3<sup>TM</sup> Scores. Methodology: This is a retrospective hospital-based study of 84 patients who underwent myelomeningocele repair at the National Center for Neurological Sciences (NCNS) during the period from 2017 up to 2019. Data were collected through a constructed questionnaire, including ASQ-3<sup>TM</sup> Scores. Data were processed and analyzed using the Statistical Package for Social Science (SPSS) computer program. Version 25. Results: 84 patients were included in this study;all patients were diagnosed with spinal dysraphism. Out of them, 51 (60.7%) were 2 years old, 33 (39.3%) were 3 years old, 45 (53.6%) were male, 45 (53.6%) of patients mothers attended ANC irregularly, and 54 (64.3%) their mothers didn’t receive folate supplements. 44 (52.3%) of patients underwent MMC repair only, while 40 (47.7%) underwent MMC repair and VP shunt. The commonest postoperative complication was infection, reported in 12 (14.3%) of patients, followed by VP shunt revision in 9 (10.7%) of patients. Neurological assessment showed that the majority of patients need further assessment with a professional, 57 (67.9%) of children don’t walk, run, or climb like other toddlers as their parent’s state;also, half of patients (42, 50%) had medical problems, and 27 (32.1%) of their parent’s state that they do not talk like other toddlers their age. There was a statistically significant association between post-operative complications and展开更多
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 r...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.展开更多
A child born with untreatable birth defect encounters constant challenges in lifetime. Spina bifida is such type of defect mainly affecting neural tube. As a result, a child with spina bifida faces abnormal physical a...A child born with untreatable birth defect encounters constant challenges in lifetime. Spina bifida is such type of defect mainly affecting neural tube. As a result, a child with spina bifida faces abnormal physical appearance to neurological dysfunctions. The incident rate of such birth defect is relatively common compared to other birth defects, therefore, an awareness among people is necessary to avoid such vulnerability. Therefore, this article provides a general outline of symptoms, types, risk factors, pathophysiology, preventive and therapeutic strategies of spina bifida which will help the general people for better understanding of the disease and be able to take precautions to combat such defect.展开更多
目的探讨脊髓脊膜膨出(myelomeningocele,MMC)修补术后迟发性脊柱畸形的影像学特征与矫形策略。方法回顾性分析2006年1月至2019年12月收治的MMC修补术后迟发性脊柱畸形的患者23例,男16例、女7例,年龄(15.4±5.9)岁(范围6~28岁),均...目的探讨脊髓脊膜膨出(myelomeningocele,MMC)修补术后迟发性脊柱畸形的影像学特征与矫形策略。方法回顾性分析2006年1月至2019年12月收治的MMC修补术后迟发性脊柱畸形的患者23例,男16例、女7例,年龄(15.4±5.9)岁(范围6~28岁),均在幼儿期(0~4岁)接受MMC切除修补术。对脊髓脊膜膨出的合并症、脊柱畸形的影像学特征(侧凸Cobb角、冠状面平衡、局部后凸角)、矫形方法、临床疗效及并发症的发生率进行统计学分析。采用脊柱侧凸研究学会-22项问卷(Scoliosis Research Society,SRS-22)评分和Oswestry功能障碍指数(Oswestry disability index,ODI)评估患者的生活质量。结果所有患者均获得随访,随访时间(2.4±0.8)年(范围1~4年)。23例患者中MMC发生于上胸段3例、胸段1例、胸腰段13例,腰骶段6例。16例患者脊柱侧凸或后凸的顶点与MMC病损在同一节段内。MMC位于胸腰段的13例患者中12例伴有侧凸畸形、9例伴有后凸畸形,MMC位于腰骶段的6例患者中3例伴有骨盆倾斜。椎体畸形包括椎弓根间距增宽21例、椎管扩大19例、棘突缺如17例、分节不良17例、半椎体畸形9例。髓内病变包括脊髓纵裂6例、脊髓拴系9例。总体植入物密度57.2%±17.0%(范围16.6%~100%)。末次随访时侧凸Cobb角为40.9°±19.1°,小于术前的71.5°±28.2°,差异有统计学意义(P<0.001);局部后凸角为26.7°±12.9°,小于术前的40.4°±21.5°,差异有统计学意义(P<0.001);冠状面平衡为(16.1±13.6)mm,小于术前的(28.5±23.7)mm,差异有统计学意义(P<0.001)。末次随访时SRS-22总分为(18.7±0.7)分,高于术前的(17.7±0.9)分,差异有统计学意义(t=-9.74,P<0.001);术后ODI为25.5%±6.2%,小于术前的44.8%±10.1%,差异有统计学意义(t=13.66,P<0.001)。4例患者发生硬脊膜破裂,其中2例发生脑脊液漏;1例术后发生胸腔积液;2例患者出现螺钉位置不良。末次随访时3例患者出现断棒,1例出现深部感染。结论MMC患者在�展开更多
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...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.展开更多
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 ...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.展开更多
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: T...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.展开更多
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 g...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.展开更多
<strong>Background:</strong> 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 li...<strong>Background:</strong> 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. <strong>Methods:</strong> 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. <strong>Results:</strong> 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 repair of the MMC and a ventriculoperitoneal shu展开更多
文摘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 neurology.Patients 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 sensitivity.Spinal cord injury is associated with severe autonomic dysfunction,and bowel dysfunction is a major physical and psychological burden for these patients.An adult myelomeningocele patient commonly has multiple problems reflecting the multisystemic nature of the disease.Multiple 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 disability.Parkinson's disease is a multisystem disorder involving dopaminergic,noradrenergic,serotoninergic and cholinergic systems,characterizedby motor and non-motor symptoms.Parkinson's disease affects several neuronal structures outside the substantia nigra,among which is the enteric nervous system.Recent 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 system.This 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 process.This 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 neurological diseases.Embryological,morphological and ex
文摘Background: Spinal dysraphism represents a wide spectrum of congenital abnormalities of the spine. Myelomeningocele is considered the most common malformation and the most common we saw in our community, with its morbidity problems seen commonly in the postoperative period. ASQ-3<sup>TM</sup> Scores are the ages and stages questionnaire, third edition, and represent a tool to assess the development progress, especially in toddlers. Objectives: Evaluation of neurodevelopmental outcome among Sudanese toddlers with spinal dysraphism after surgical closure with or without a VP shunt using ASQ-3<sup>TM</sup> Scores. Methodology: This is a retrospective hospital-based study of 84 patients who underwent myelomeningocele repair at the National Center for Neurological Sciences (NCNS) during the period from 2017 up to 2019. Data were collected through a constructed questionnaire, including ASQ-3<sup>TM</sup> Scores. Data were processed and analyzed using the Statistical Package for Social Science (SPSS) computer program. Version 25. Results: 84 patients were included in this study;all patients were diagnosed with spinal dysraphism. Out of them, 51 (60.7%) were 2 years old, 33 (39.3%) were 3 years old, 45 (53.6%) were male, 45 (53.6%) of patients mothers attended ANC irregularly, and 54 (64.3%) their mothers didn’t receive folate supplements. 44 (52.3%) of patients underwent MMC repair only, while 40 (47.7%) underwent MMC repair and VP shunt. The commonest postoperative complication was infection, reported in 12 (14.3%) of patients, followed by VP shunt revision in 9 (10.7%) of patients. Neurological assessment showed that the majority of patients need further assessment with a professional, 57 (67.9%) of children don’t walk, run, or climb like other toddlers as their parent’s state;also, half of patients (42, 50%) had medical problems, and 27 (32.1%) of their parent’s state that they do not talk like other toddlers their age. There was a statistically significant association between post-operative complications and
文摘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.
文摘A child born with untreatable birth defect encounters constant challenges in lifetime. Spina bifida is such type of defect mainly affecting neural tube. As a result, a child with spina bifida faces abnormal physical appearance to neurological dysfunctions. The incident rate of such birth defect is relatively common compared to other birth defects, therefore, an awareness among people is necessary to avoid such vulnerability. Therefore, this article provides a general outline of symptoms, types, risk factors, pathophysiology, preventive and therapeutic strategies of spina bifida which will help the general people for better understanding of the disease and be able to take precautions to combat such defect.
文摘目的探讨脊髓脊膜膨出(myelomeningocele,MMC)修补术后迟发性脊柱畸形的影像学特征与矫形策略。方法回顾性分析2006年1月至2019年12月收治的MMC修补术后迟发性脊柱畸形的患者23例,男16例、女7例,年龄(15.4±5.9)岁(范围6~28岁),均在幼儿期(0~4岁)接受MMC切除修补术。对脊髓脊膜膨出的合并症、脊柱畸形的影像学特征(侧凸Cobb角、冠状面平衡、局部后凸角)、矫形方法、临床疗效及并发症的发生率进行统计学分析。采用脊柱侧凸研究学会-22项问卷(Scoliosis Research Society,SRS-22)评分和Oswestry功能障碍指数(Oswestry disability index,ODI)评估患者的生活质量。结果所有患者均获得随访,随访时间(2.4±0.8)年(范围1~4年)。23例患者中MMC发生于上胸段3例、胸段1例、胸腰段13例,腰骶段6例。16例患者脊柱侧凸或后凸的顶点与MMC病损在同一节段内。MMC位于胸腰段的13例患者中12例伴有侧凸畸形、9例伴有后凸畸形,MMC位于腰骶段的6例患者中3例伴有骨盆倾斜。椎体畸形包括椎弓根间距增宽21例、椎管扩大19例、棘突缺如17例、分节不良17例、半椎体畸形9例。髓内病变包括脊髓纵裂6例、脊髓拴系9例。总体植入物密度57.2%±17.0%(范围16.6%~100%)。末次随访时侧凸Cobb角为40.9°±19.1°,小于术前的71.5°±28.2°,差异有统计学意义(P<0.001);局部后凸角为26.7°±12.9°,小于术前的40.4°±21.5°,差异有统计学意义(P<0.001);冠状面平衡为(16.1±13.6)mm,小于术前的(28.5±23.7)mm,差异有统计学意义(P<0.001)。末次随访时SRS-22总分为(18.7±0.7)分,高于术前的(17.7±0.9)分,差异有统计学意义(t=-9.74,P<0.001);术后ODI为25.5%±6.2%,小于术前的44.8%±10.1%,差异有统计学意义(t=13.66,P<0.001)。4例患者发生硬脊膜破裂,其中2例发生脑脊液漏;1例术后发生胸腔积液;2例患者出现螺钉位置不良。末次随访时3例患者出现断棒,1例出现深部感染。结论MMC患者在�
文摘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.
文摘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.
文摘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.
文摘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.
文摘<strong>Background:</strong> 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. <strong>Methods:</strong> 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. <strong>Results:</strong> 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 repair of the MMC and a ventriculoperitoneal shu
