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Keith E Georgeson - One of the best experts on this subject based on the ideXlab platform.

  • laparoscopic cecostomy button placement for the management of fecal incontinence in children with hirschsprung s disease and Anorectal Anomalies
    Surgical Endoscopy and Other Interventional Techniques, 2006
    Co-Authors: A Yagmurlu, Carroll M Harmon, Keith E Georgeson
    Abstract:

    Background Antegrade colonic enemas offer a surgical solution for many children with chronic constipation and encopresis associated with Hirschsprung’s disease and Anorectal malformations. This study demonstrated the feasibility of a new laparoscopic technique for cecostomy button placement (LCBP) to allow antegrade enema treatment.

  • laparoscopic cecostomy button placement for the management of fecal incontinence in children with hirschsprung s disease and Anorectal Anomalies
    Surgical Endoscopy and Other Interventional Techniques, 2006
    Co-Authors: A Yagmurlu, Carroll M Harmon, Keith E Georgeson
    Abstract:

    Antegrade colonic enemas offer a surgical solution for many children with chronic constipation and encopresis associated with Hirschsprung’s disease and Anorectal malformations. This study demonstrated the feasibility of a new laparoscopic technique for cecostomy button placement (LCBP) to allow antegrade enema treatment. Charts of children with encopresis who underwent LCBP between 1999 and 2001 were reviewed. The age, weight, primary diagnosis, operative time, hospital stay, associated complications, follow-up duration, and outcome of the patients were recorded. The surgical technique used a “U-stitch” method and a chait tube or a standard gastrostomy button. A follow-up telephone survey was conducted to assess parental satisfaction and overall success in continence. Seven patients ages 4 to 12 years (mean, 7.3 ± 1.3 years) and weighing 15 to 44 kg (mean, 24.5 ± 4 kg) underwent LCBP over a 2-year period. The mean follow-up period was 15 ± 4 months (range, 6–33 months). Four patients had Anorectal malformations, and three patients had Hirschsprung’s disease. For all the patients, LCBP was accomplished without any intraoperative complications. The mean operative time was 33 ± 2 min, and the hospital stay was 2 to 5 days (mean, 3.8 ± 0.5 days). The patients received one or two daily antegrade enemas, and none had accidental bowel movements. Episodes of soiling at night once or twice a week were observed with two children. Two patients had hypertrophic granulation tissue formation, which responded to topical therapy. The button was uneventfully changed twice in one patient because of mechanical malfunction. To manage overflow incontinence of children with Anorectal malformations and Hirschsprung’s disease, LCBP is a technically straightforward, effective, and reversible method for the placement of a cecostomy button.

Valdemiro Amaro Da ,silva Júnior - One of the best experts on this subject based on the ideXlab platform.

  • Abnormalities of digestive tract innervation in rat fetus treated with ethylenethiourea
    Sociedade Brasileira para o Desenvolvimento da Pesquisa em Cirurgia, 2012
    Co-Authors: Lemos,sidney Pereira Pinto, Martins,josé Luiz, Lemos,patrícia Veruska Ribeiro Barbosa, Silva,silvio Romero Gonçalves E, Santos,fernando Leandro Dos, Valdemiro Amaro Da ,silva Júnior
    Abstract:

    PURPOSE: The pathophysiology of abnormalities associated with myenteric plexus lesions remains imperfectly understood. Such abnormalities have been correlated with subocclusive intestinal conditions in children with Hirschsprung's disease, cases of chronic constipation and, postoperatively, in cases of Anorectal Anomalies. This study evaluated abnormalities of the myenteric plexus in fetus from female rats that received ethylenethiourea. METHODS: Female rats were exposed to ethylenethiourea on the 11th day of pregnancy (experimental group) or to 0.9% physiological solution (control group). Abnormalities were only found in the experimental group. The digestive tract muscle layer was analyzed morphometrically and changes to the frequencies of nerve plexus cells and interstitial cells of Cajal were evaluated, using hematoxylin-eosin, S-100 protein, neuron-specific enolase and C-Kit, respectively. RESULTS: Muscle and skeletal abnormalities were observed in 100%, Anorectal Anomalies in 86%, absent tail in 71%, short tail in 29%, duodenal atresia in 5%, esophageal atresia in 5% and persistent omphalomesenteric duct in 5%. Histopathological analysis showed a thinner muscle layer associated with lower frequencies of ganglion cells and interstitial cells of Cajal, in all gastrointestinal tract. CONCLUSION: Severe nerve plexus abnormalities associated with muscle layer atrophy were observed throughout the gastrointestinal tract in newborn rats exposed to ethylenethiourea

  • Anomalias da inervação do trato digestório de fetos de ratas expostas à etilenotioureia
    'FapUNIFESP (SciELO)', 2012
    Co-Authors: Lemos,sidney Pereira Pinto, Martins,josé Luiz, Lemos,patrícia Veruska Ribeiro Barbosa, Silva,silvio Romero Gonçalves E, Santos,fernando Leandro Dos, Valdemiro Amaro Da ,silva Júnior
    Abstract:

    PURPOSE: The pathophysiology of abnormalities associated with myenteric plexus lesions remains imperfectly understood. Such abnormalities have been correlated with subocclusive intestinal conditions in children with Hirschsprung's disease, cases of chronic constipation and, postoperatively, in cases of Anorectal Anomalies. This study evaluated abnormalities of the myenteric plexus in fetus from female rats that received ethylenethiourea. METHODS: Female rats were exposed to ethylenethiourea on the 11th day of pregnancy (experimental group) or to 0.9% physiological solution (control group). Abnormalities were only found in the experimental group. The digestive tract muscle layer was analyzed morphometrically and changes to the frequencies of nerve plexus cells and interstitial cells of Cajal were evaluated, using hematoxylin-eosin, S-100 protein, neuron-specific enolase and C-Kit, respectively. RESULTS: Muscle and skeletal abnormalities were observed in 100%, Anorectal Anomalies in 86%, absent tail in 71%, short tail in 29%, duodenal atresia in 5%, esophageal atresia in 5% and persistent omphalomesenteric duct in 5%. Histopathological analysis showed a thinner muscle layer associated with lower frequencies of ganglion cells and interstitial cells of Cajal, in all gastrointestinal tract. CONCLUSION: Severe nerve plexus abnormalities associated with muscle layer atrophy were observed throughout the gastrointestinal tract in newborn rats exposed to ethylenethiourea.OBJETIVO: As anomalias associadas a lesões dos plexos mioentéricos permanecem sem plena compreensão da sua fisiopatologia. Alterações nos plexos nervosos têm sido correlacionadas com quadros suboclusivos intestinais em crianças portadoras de doença de Hirschsprung, em constipação crônica e no pós-operatório de anomalias anorretais. Este estudo avaliou as anomalias do plexo mioentérico em fetos de ratos fêmea que ingeriram etilenotioureia (ETU). MÉTODOS: Ratos fêmea foram expostos no 11º dia de gestação a ETU 1% no Grupo Experimento e a solução fisiológica 0,9% no Grupo Controle. Foram observadas anomalias apenas no Grupo experimento, sendo realizada morfometria da camada muscular e avaliadas alterações da frequência celular nos gânglios do plexo mioentérico e nas células intersticiais de Cajal (CIC) utilizando hematoxilina-eosina, P S-100, Enolase Neurônio Específica e C-KIT. RESULTADOS: Foram observadas anomalias musculoesqueléticas (100%), anorretais (86%), ausência de cauda (71%), cauda curta (29%), atresia duodenal (5%), atresia esofágica (5%) e conduto onfalomesentérico persistente (5%). A análise histopatológica mostrou adelgaçamento da camada muscular associada às alterações da frequência das células ganglionares e das CIC em todos os segmentos do trato gastrointestinal. CONCLUSÃO: Foram observadas alterações graves nos plexos nervosos associadas ao adelgaçamento da camada muscular de todo o trato gastrointestinal nos fetos expostos a ETU.UNIVASFUniversidade Federal de São Paulo (UNIFESP) Department of Surgery Pediatric Surgery DivisionFederal Rural University Department of Veterinary MedicineFederal Rural University Department of Animal Morphology and PhysiologyUNIFESP, Department of Surgery Pediatric Surgery DivisionSciEL

  • Anomalias da inervação do trato digestório de fetos de ratas expostas à etilenotioureia
    Sociedade Brasileira para o Desenvolvimento da Pesquisa em Cirurgia, 2012
    Co-Authors: Lemos, Sidney Pereira Pinto [unifesp], Lemos,patrícia Veruska Ribeiro Barbosa, Santos,fernando Leandro Dos, Martins, Jose Luiz [unifesp], Silva, Silvio Romero Gonçalves E [unifesp], Valdemiro Amaro Da ,silva Júnior
    Abstract:

    PURPOSE: The pathophysiology of abnormalities associated with myenteric plexus lesions remains imperfectly understood. Such abnormalities have been correlated with subocclusive intestinal conditions in children with Hirschsprung's disease, cases of chronic constipation and, postoperatively, in cases of Anorectal Anomalies. This study evaluated abnormalities of the myenteric plexus in fetus from female rats that received ethylenethiourea. METHODS: Female rats were exposed to ethylenethiourea on the 11th day of pregnancy (experimental group) or to 0.9% physiological solution (control group). Abnormalities were only found in the experimental group. The digestive tract muscle layer was analyzed morphometrically and changes to the frequencies of nerve plexus cells and interstitial cells of Cajal were evaluated, using hematoxylin-eosin, S-100 protein, neuron-specific enolase and C-Kit, respectively. RESULTS: Muscle and skeletal abnormalities were observed in 100%, Anorectal Anomalies in 86%, absent tail in 71%, short tail in 29%, duodenal atresia in 5%, esophageal atresia in 5% and persistent omphalomesenteric duct in 5%. Histopathological analysis showed a thinner muscle layer associated with lower frequencies of ganglion cells and interstitial cells of Cajal, in all gastrointestinal tract. CONCLUSION: Severe nerve plexus abnormalities associated with muscle layer atrophy were observed throughout the gastrointestinal tract in newborn rats exposed to ethylenethiourea.OBJETIVO: As anomalias associadas a lesões dos plexos mioentéricos permanecem sem plena compreensão da sua fisiopatologia. Alterações nos plexos nervosos têm sido correlacionadas com quadros suboclusivos intestinais em crianças portadoras de doença de Hirschsprung, em constipação crônica e no pós-operatório de anomalias anorretais. Este estudo avaliou as anomalias do plexo mioentérico em fetos de ratos fêmea que ingeriram etilenotioureia (ETU). MÉTODOS: Ratos fêmea foram expostos no 11º dia de gestação a ETU 1% no Grupo Experimento e a solução fisiológica 0,9% no Grupo Controle. Foram observadas anomalias apenas no Grupo experimento, sendo realizada morfometria da camada muscular e avaliadas alterações da frequência celular nos gânglios do plexo mioentérico e nas células intersticiais de Cajal (CIC) utilizando hematoxilina-eosina, P S-100, Enolase Neurônio Específica e C-KIT. RESULTADOS: Foram observadas anomalias musculoesqueléticas (100%), anorretais (86%), ausência de cauda (71%), cauda curta (29%), atresia duodenal (5%), atresia esofágica (5%) e conduto onfalomesentérico persistente (5%). A análise histopatológica mostrou adelgaçamento da camada muscular associada às alterações da frequência das células ganglionares e das CIC em todos os segmentos do trato gastrointestinal. CONCLUSÃO: Foram observadas alterações graves nos plexos nervosos associadas ao adelgaçamento da camada muscular de todo o trato gastrointestinal nos fetos expostos a ETU

  • Abnormalities of digestive tract innervation in rat fetus treated with ethylenethiourea Anomalias da inervação do trato digestório de fetos de ratas expostas à etilenotioureia
    Sociedade Brasileira para o Desenvolvimento da Pesquisa em Cirurgia, 2012
    Co-Authors: Sidney Pereira Pinto Lemos, Jose Luiz Martins, Patrícia Veruska Ribeiro Barbosa Lemos, Silvio Romero Gonçalves E Silva, Fernando Leandro Dos Santos, Valdemiro Amaro Da ,silva Júnior
    Abstract:

    PURPOSE: The pathophysiology of abnormalities associated with myenteric plexus lesions remains imperfectly understood. Such abnormalities have been correlated with subocclusive intestinal conditions in children with Hirschsprung's disease, cases of chronic constipation and, postoperatively, in cases of Anorectal Anomalies. This study evaluated abnormalities of the myenteric plexus in fetus from female rats that received ethylenethiourea. METHODS: Female rats were exposed to ethylenethiourea on the 11th day of pregnancy (experimental group) or to 0.9% physiological solution (control group). Abnormalities were only found in the experimental group. The digestive tract muscle layer was analyzed morphometrically and changes to the frequencies of nerve plexus cells and interstitial cells of Cajal were evaluated, using hematoxylin-eosin, S-100 protein, neuron-specific enolase and C-Kit, respectively. RESULTS: Muscle and skeletal abnormalities were observed in 100%, Anorectal Anomalies in 86%, absent tail in 71%, short tail in 29%, duodenal atresia in 5%, esophageal atresia in 5% and persistent omphalomesenteric duct in 5%. Histopathological analysis showed a thinner muscle layer associated with lower frequencies of ganglion cells and interstitial cells of Cajal, in all gastrointestinal tract. CONCLUSION: Severe nerve plexus abnormalities associated with muscle layer atrophy were observed throughout the gastrointestinal tract in newborn rats exposed to ethylenethiourea.OBJETIVO: As anomalias associadas a lesões dos plexos mioentéricos permanecem sem plena compreensão da sua fisiopatologia. Alterações nos plexos nervosos têm sido correlacionadas com quadros suboclusivos intestinais em crianças portadoras de doença de Hirschsprung, em constipação crônica e no pós-operatório de anomalias anorretais. Este estudo avaliou as anomalias do plexo mioentérico em fetos de ratos fêmea que ingeriram etilenotioureia (ETU). MÉTODOS: Ratos fêmea foram expostos no 11º dia de gestação a ETU 1% no Grupo Experimento e a solução fisiológica 0,9% no Grupo Controle. Foram observadas anomalias apenas no Grupo experimento, sendo realizada morfometria da camada muscular e avaliadas alterações da frequência celular nos gânglios do plexo mioentérico e nas células intersticiais de Cajal (CIC) utilizando hematoxilina-eosina, P S-100, Enolase Neurônio Específica e C-KIT. RESULTADOS: Foram observadas anomalias musculoesqueléticas (100%), anorretais (86%), ausência de cauda (71%), cauda curta (29%), atresia duodenal (5%), atresia esofágica (5%) e conduto onfalomesentérico persistente (5%). A análise histopatológica mostrou adelgaçamento da camada muscular associada às alterações da frequência das células ganglionares e das CIC em todos os segmentos do trato gastrointestinal. CONCLUSÃO: Foram observadas alterações graves nos plexos nervosos associadas ao adelgaçamento da camada muscular de todo o trato gastrointestinal nos fetos expostos a ETU

A Yagmurlu - One of the best experts on this subject based on the ideXlab platform.

  • laparoscopic cecostomy button placement for the management of fecal incontinence in children with hirschsprung s disease and Anorectal Anomalies
    Surgical Endoscopy and Other Interventional Techniques, 2006
    Co-Authors: A Yagmurlu, Carroll M Harmon, Keith E Georgeson
    Abstract:

    Background Antegrade colonic enemas offer a surgical solution for many children with chronic constipation and encopresis associated with Hirschsprung’s disease and Anorectal malformations. This study demonstrated the feasibility of a new laparoscopic technique for cecostomy button placement (LCBP) to allow antegrade enema treatment.

  • laparoscopic cecostomy button placement for the management of fecal incontinence in children with hirschsprung s disease and Anorectal Anomalies
    Surgical Endoscopy and Other Interventional Techniques, 2006
    Co-Authors: A Yagmurlu, Carroll M Harmon, Keith E Georgeson
    Abstract:

    Antegrade colonic enemas offer a surgical solution for many children with chronic constipation and encopresis associated with Hirschsprung’s disease and Anorectal malformations. This study demonstrated the feasibility of a new laparoscopic technique for cecostomy button placement (LCBP) to allow antegrade enema treatment. Charts of children with encopresis who underwent LCBP between 1999 and 2001 were reviewed. The age, weight, primary diagnosis, operative time, hospital stay, associated complications, follow-up duration, and outcome of the patients were recorded. The surgical technique used a “U-stitch” method and a chait tube or a standard gastrostomy button. A follow-up telephone survey was conducted to assess parental satisfaction and overall success in continence. Seven patients ages 4 to 12 years (mean, 7.3 ± 1.3 years) and weighing 15 to 44 kg (mean, 24.5 ± 4 kg) underwent LCBP over a 2-year period. The mean follow-up period was 15 ± 4 months (range, 6–33 months). Four patients had Anorectal malformations, and three patients had Hirschsprung’s disease. For all the patients, LCBP was accomplished without any intraoperative complications. The mean operative time was 33 ± 2 min, and the hospital stay was 2 to 5 days (mean, 3.8 ± 0.5 days). The patients received one or two daily antegrade enemas, and none had accidental bowel movements. Episodes of soiling at night once or twice a week were observed with two children. Two patients had hypertrophic granulation tissue formation, which responded to topical therapy. The button was uneventfully changed twice in one patient because of mechanical malfunction. To manage overflow incontinence of children with Anorectal malformations and Hirschsprung’s disease, LCBP is a technically straightforward, effective, and reversible method for the placement of a cecostomy button.

Charlotte Schramm - One of the best experts on this subject based on the ideXlab platform.

  • autosomal dominant non syndromic anal atresia sequencing of candidate genes array based molecular karyotyping and review of the literature
    European Journal of Pediatrics, 2011
    Co-Authors: Charlotte Schramm, Enrika Bartels, Markus Draaken, Eberhard Schmiedeke, Gabriel Tewes, Stefanie Marzheuser, Sabine Grasshoffderr, Stuart Hosie, Stefan Hollandcunz, Lutz Priebe
    Abstract:

    Introduction Anorectal malformations (ARM) range from mild anal to severe Anorectal Anomalies. Approximately 50% are estimated to be non-syndromic with multiple familial cases reported that suggest underlying genetic factors. These, however, still await identification.

  • de novo partial trisomy 18p and partial monosomy 18q in a patient with Anorectal malformation
    Cytogenetic and Genome Research, 2011
    Co-Authors: Enrika Bartels, Markus Draaken, B Kazmierczak, S Spranger, Charlotte Schramm, Friederike Baudisch, Markus M Nothen, Eberhard Schmiedeke, Michael Ludwig, Heiko Reutter
    Abstract:

    Anorectal malformations (ARM) encompass a broad clinical spectrum which ranges from mild anal stenosis to severe Anorectal Anomalies such as complex cloacal malformations. The overall incidence of ARM

  • de novo partial trisomy 18p and partial monosomy 18q in a patient with Anorectal malformation
    Cytogenetic and Genome Research, 2011
    Co-Authors: Enrika Bartels, Markus Draaken, B Kazmierczak, S Spranger, Charlotte Schramm, Friederike Baudisch, Markus M Nothen, Eberhard Schmiedeke, Michael Ludwig, Heiko Reutter
    Abstract:

    Anorectal malformations (ARM) encompass a broad clinical spectrum which ranges from mild anal stenosis to severe Anorectal Anomalies such as complex cloacal malformations. The overall incidence of ARM is around 1 in every 2,500 live births. Although causative genes for a few syndromic forms have been identified, the molecular genetic background of most ARM remains unknown. The present report describes a patient with a de novo 13.2-Mb deletion of chromosome 18q22.3-qter and a 2.2-Mb de novo duplication of chromosomal region 18pter-p11.32 located at the telomeric end of chromosome 18q. The patient presented with ARM and the typical features of 18q- syndrome (De-Grouchy syndrome). The combination of a partial duplication of the short arm and a partial deletion of the long arm of chromosome 18 has been described in 16 previous cases. However, this is the first report of an association between this complex chromosomal rearrangement and ARM.

Enrika Bartels - One of the best experts on this subject based on the ideXlab platform.

  • autosomal dominant non syndromic anal atresia sequencing of candidate genes array based molecular karyotyping and review of the literature
    European Journal of Pediatrics, 2011
    Co-Authors: Charlotte Schramm, Enrika Bartels, Markus Draaken, Eberhard Schmiedeke, Gabriel Tewes, Stefanie Marzheuser, Sabine Grasshoffderr, Stuart Hosie, Stefan Hollandcunz, Lutz Priebe
    Abstract:

    Introduction Anorectal malformations (ARM) range from mild anal to severe Anorectal Anomalies. Approximately 50% are estimated to be non-syndromic with multiple familial cases reported that suggest underlying genetic factors. These, however, still await identification.

  • de novo partial trisomy 18p and partial monosomy 18q in a patient with Anorectal malformation
    Cytogenetic and Genome Research, 2011
    Co-Authors: Enrika Bartels, Markus Draaken, B Kazmierczak, S Spranger, Charlotte Schramm, Friederike Baudisch, Markus M Nothen, Eberhard Schmiedeke, Michael Ludwig, Heiko Reutter
    Abstract:

    Anorectal malformations (ARM) encompass a broad clinical spectrum which ranges from mild anal stenosis to severe Anorectal Anomalies such as complex cloacal malformations. The overall incidence of ARM

  • de novo partial trisomy 18p and partial monosomy 18q in a patient with Anorectal malformation
    Cytogenetic and Genome Research, 2011
    Co-Authors: Enrika Bartels, Markus Draaken, B Kazmierczak, S Spranger, Charlotte Schramm, Friederike Baudisch, Markus M Nothen, Eberhard Schmiedeke, Michael Ludwig, Heiko Reutter
    Abstract:

    Anorectal malformations (ARM) encompass a broad clinical spectrum which ranges from mild anal stenosis to severe Anorectal Anomalies such as complex cloacal malformations. The overall incidence of ARM is around 1 in every 2,500 live births. Although causative genes for a few syndromic forms have been identified, the molecular genetic background of most ARM remains unknown. The present report describes a patient with a de novo 13.2-Mb deletion of chromosome 18q22.3-qter and a 2.2-Mb de novo duplication of chromosomal region 18pter-p11.32 located at the telomeric end of chromosome 18q. The patient presented with ARM and the typical features of 18q- syndrome (De-Grouchy syndrome). The combination of a partial duplication of the short arm and a partial deletion of the long arm of chromosome 18 has been described in 16 previous cases. However, this is the first report of an association between this complex chromosomal rearrangement and ARM.