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2.
Transplant Proc ; 55(2): 459-465, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37059668

RESUMO

INTRODUCTION: Patients undergoing lung transplantation (LT) are at high risk of developing serious abdominal complications, which can lead to higher rates of morbidity and mortality. The aim of this study was to investigate the incidence and spectrum of these complications when they develop during the first 30 days after LT, as well as their possible association with possible risk factors. METHODS: A retrospective study of 552 patients undergoing LT between 01/02/2006 and 06/03/2021 was carried out. A descriptive and analytical evaluation of the patients who experienced complications and those who did not was performed comparatively. Data related to patient characteristics and the lung transplantation procedure were collected. RESULTS: Overall, 8.2% of patients developed severe abdominal complications during the first 30 days; paralytic ileus was the most frequent (31.1%), closely followed by visceral perforation (26.7%). The percentage of patients who required an invasive procedure to manage post-transplant complications was 57.8%. Surgical intervention was required in 39.8%. The variables that showed a significant relationship with the development of severe short-term abdominal complications in the univariate analysis were the time of surgery, the use of ECMO/ ECC and red blood cell transfusion during or after surgery. In the multivariate study, however, only duration of surgery remained significant (p=0.03). CONCLUSION: The incidence of severe short-term abdominal complications after LT period was 8%. The commonest complications were paralytic ileus and intestinal perforation. Most patients did not require surgery. The only risk factor found associated with these complications was the duration of the surgical intervention.


Assuntos
Pseudo-Obstrução Intestinal , Complicações Pós-Operatórias , Humanos , Estudos Retrospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Transplantados , Fatores de Risco , Pulmão , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia
3.
Artigo em Inglês | MEDLINE | ID: mdl-36011537

RESUMO

Background: In the United States, ileus accounts for USD 750 million of healthcare expenditures annually and significantly contributes to morbidity and mortality. Despite its significance, the complete picture of mortality risk factors for these patients have yet to be fully elucidated; therefore, the aim of this study is to identify mortality risk factors in patients emergently admitted with paralytic ileus. Methods: Adult and elderly patients emergently admitted with paralytic ileus between 2005−2014 were investigated using the National Inpatient Sample Database. Clinical outcomes, therapeutic management, demographics and comorbidities were collected. Associations between mortality and all other variables were established via univariable and multivariable logistic regression models. Results: A total of 81,674 patients were included, of which 45.2% were adults, 54.8% elderly patients, 45.8% male and 54.2% female. The average adult and elderly ages were 48.3 and 78.8 years, respectively. Elderly patients displayed a significantly (p < 0.01) higher mortality rate (3.0%) than adults (0.7%). The final multivariable logistic regression model showed that for every one-day delay in operation, the odds of mortality for adult and elderly patients increased by 4.1% (p = 0.002) and 3.2% (p = 0.014), respectively. Every additional year of age corresponded to 3.8% and 2.6% increases in mortality for operatively managed adult (p = 0.026) and elderly (p = 0.015) patients. Similarly, non-operatively treated adult and elderly patients displayed associations between mortality and advanced age (p = 0.001). The modified frailty index exhibited associations with mortality in operatively treated adults, conservatively managed adults and conservatively managed elderly patients (p = 0.001). Every additional day of hospitalization increased the odds of mortality in non-operative adult and elderly patients by 7.6% and 5.8%, respectively. Female sex correlated to lower mortality rates in non-operatively managed adult patients (odds ratio = 0.71, p = 0.028). Undergoing invasive diagnostic procedures in non-operatively managed elderly patients related to reduced mortality (odds ratio = 0.78, p = 0.026). Conclusions: Patients emergently admitted for paralytic ileus with increased hospital length of stay, longer time to operation, advanced age or higher modified frailty index displayed higher mortality rates. Female sex and invasive diagnostic procedures were negatively correlated with death in nonoperatively managed patients with paralytic ileus.


Assuntos
Fragilidade , Pseudo-Obstrução Intestinal , Adulto , Idoso , Feminino , Fragilidade/complicações , Fragilidade/epidemiologia , Mortalidade Hospitalar , Hospitais , Humanos , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/terapia , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Estados Unidos
4.
Dig Dis Sci ; 67(10): 4834-4840, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35001241

RESUMO

BACKGROUND: Chronic intestinal pseudo-obstruction (CIP) is a rare motility disorder characterized by dilated small bowel in the absence of mechanical obstruction. CIP has a known association with small intestinal bacterial overgrowth (SIBO); however, data regarding association with specific subtypes such as methane-positive (M+) and hydrogen-positive (H+) SIBO are limited. Therefore, we conducted this study to characterize subtypes of SIBO in CIP and compare them with non-CIP patients. AIMS: The aim is to explore the association and prevalence of hydrogen and methane subtypes of SIBO in patients with CIP. METHODS: A retrospective chart review was conducted for 494 patients who underwent glucose breath tests (GBT) in 2019. CIP was diagnosed based on clinical suspicion and after ruling out mechanical obstruction. We also reviewed demographic data, including age, gender, body mass index, tobacco and alcohol history, medical comorbidities, use of proton pump inhibitors, and history of colectomy. RESULTS: Among 494 patients, 7.7% (38) had CIP. The prevalence of M+ GBT in CIP patients was higher compared with non-CIP patients, and it was significant [52.6% (20/38) versus 11.8% (54/456), p < 0.001]. The prevalence of H+ GBT in our cohort of CIP patients was similar to that of non-CIP patients [23.7% (9/38) versus 25.7% (117/456), p = 0.941]. CONCLUSION: The prevalence of methane-positive GBT was higher in CIP patients than in patients without CIP. This finding further strengthens the hypothesis that the relationship between motility disorders and methanogen overgrowth is facilitative.


Assuntos
Euryarchaeota , Pseudo-Obstrução Intestinal , Testes Respiratórios , Glucose , Humanos , Hidrogênio , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/epidemiologia , Metano , Inibidores da Bomba de Prótons , Estudos Retrospectivos
5.
Surgery ; 170(6): 1618-1626, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34497027

RESUMO

BACKGROUND: Postoperative paralytic ileus prolongs hospitalization duration, increases medical expenses, and is even associated with postoperative mortality; however, effective prevention of postoperative paralytic ileus is not yet available. This trial aimed to assess the preventative effectiveness of transcutaneous electrical acupoint stimulation applied in the lower limbs on postoperative paralytic ileus incidence after colorectal surgery. METHODS: After ethics approval and written informed consent, 610 patients from 10 hospitals who were scheduled for colorectal surgery between May 2018 and September 2019 were enrolled. Patients were randomly allocated into the transcutaneous electrical acupoint stimulation (stimulated on bilateral Zusanli, Shangjuxu, and Sanyinjiao acupoints in lower limbs for 30 minutes each time, total 4 times) or sham (without currents delivered) group with 1:1 ratio. The primary outcome was postoperative paralytic ileus incidence, defined as no flatus for >72 hours after surgery. RESULTS: Compared to the sham treatment, transcutaneous electrical acupoint stimulation lowered the postoperative paralytic ileus incidence by 8.7% (32.3% vs 41.0%, P = .026) and decreased the risk of postoperative paralytic ileus by 32% (OR, 0.68; P = .029). Transcutaneous electrical acupoint stimulation also shortened the recovery time to flatus, defecation, normal diet, and bowel sounds. Transcutaneous electrical acupoint stimulation treatment significantly increased median serum acetylcholine by 55% (P = .007) and interleukin-10 by 88% (P < .001), but decreased interleukin-6 by 47% (P < .001) and inducible nitric oxide synthase by 42% (P = .002) at 72 hours postoperatively. CONCLUSION: Transcutaneous electrical acupoint stimulation attenuated the postoperative paralytic ileus incidence and enhanced gastrointestinal functional recovery, which may be associated with increasing parasympathetic nerve tone and its anti-inflammatory actions.


Assuntos
Pontos de Acupuntura , Neoplasias Colorretais/cirurgia , Pseudo-Obstrução Intestinal/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Estimulação Elétrica Nervosa Transcutânea/métodos , Idoso , Colo/inervação , Colo/fisiopatologia , Colo/cirurgia , Feminino , Humanos , Incidência , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/fisiopatologia , Pseudo-Obstrução Intestinal/prevenção & controle , Extremidade Inferior , Masculino , Pessoa de Meia-Idade , Sistema Nervoso Parassimpático/fisiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/prevenção & controle , Reto/inervação , Reto/fisiopatologia , Reto/cirurgia , Resultado do Tratamento
6.
Clin Transl Gastroenterol ; 11(8): e00206, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32931184

RESUMO

INTRODUCTION: Intestinal pseudo-obstruction is characterized by impaired transit and luminal dilation in the absence of mechanical obstruction. Our study aims to describe the clinical, radiographic, and physiological findings in pseudo-obstruction associated with systemic sclerosis (SSc), amyloidosis, and paraneoplastic syndrome. METHODS: A retrospective cohort of patients evaluated at our institution between January 1, 2008, and August 1, 2018, was assembled. Clinical, imaging, and physiological characteristics were abstracted from electronic medical records. RESULTS: We identified 100 cases of pseudo-obstruction (55 SSc, 27 amyloidosis, and 18 paraneoplastic). Female population predominance was seen in SSc (71%) vs male population in amyloidosis (74%). Most common symptom was abdominal bloating in all 3 groups. Vomiting was more common in SSc than amyloidosis (73% vs 46%, P = 0.02). Diarrhea was more common in amyloidosis and SSc compared with paraneoplastic (81% and 67% vs 28%, P < 0.01). Weight loss (>5%) was more common in SSc compared with amyloidosis and paraneoplastic (78% vs 31% and 17%, P < 0.0001). Only small bowel dilation was seen in 79%, 40%, and 44% and only large bowel dilation in 2%, 44%, and 44% of patients in SSc, amyloidosis, and paraneoplastic, respectively. Five of 8 SSc patients had myopathic and 3 of 5 paraneoplastic had neuropathic involvement on gastroduodenal manometry. DISCUSSION: SSc-associated pseudo-obstruction demonstrates female population predominance and presents with vomiting, diarrhea, and weight loss. Amyloidosis-associated pseudo-obstruction shows male population predominance. Small bowel is more commonly involved than large bowel on both imaging and transit studies in SSc. Myopathic involvement was more common in SSc, contrary to neuropathic in paraneoplastic syndrome.


Assuntos
Amiloidose/complicações , Pseudo-Obstrução Intestinal/diagnóstico , Síndromes Paraneoplásicas/complicações , Escleroderma Sistêmico/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Amiloidose/epidemiologia , Diarreia/epidemiologia , Diarreia/etiologia , Feminino , Trânsito Gastrointestinal/fisiologia , Humanos , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/fisiopatologia , Intestino Delgado/diagnóstico por imagem , Masculino , Manometria , Pessoa de Meia-Idade , Síndromes Paraneoplásicas/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Escleroderma Sistêmico/epidemiologia , Fatores Sexuais , Vômito/epidemiologia , Vômito/etiologia , Redução de Peso , Adulto Jovem
7.
Neurogastroenterol Motil ; 32(4): e13781, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31885159

RESUMO

BACKGROUND: Pediatric intestinal pseudo-obstruction is a rare disorder affecting gastrointestinal motility leading to chronic symptoms and hospitalizations. There is limited understanding of the epidemiology and healthcare burden. METHODS: We analyzed data from Kids' Inpatient Database from 2016, which includes inpatient discharge records from US hospitals. ICD-10 codes were used to identify patients 0-18 years with pediatric intestinal pseudo-obstruction and comorbid conditions. Multivariable logistic regression and Wilcoxon rank-sum test were used. RESULTS: In 2016, there were 1671 inpatient discharges from US hospitals for patients 0-18 years of age with this diagnosis. The incidence of inpatient admission was 29/100 000 patients. After controlling for age, race, income status, and insurance, males vs females (adjusted odds ratio, aOR: 1.10; 95% CI: 0.94-1.28; P = .241) and caucasians vs other races (aOR: 1.55; 95% CI: 1.27-1.88; P < .001) were more likely to be admitted. Inpatient admissions incurred significant healthcare burden; median (inter quartile range IQR) cost of hospitalization of US$ 52 079 (US$ 23 530-120 961) and a median (IQR) length of stay of 6 days (3-14 days). Gastrostomy (32%) and ileostomy (12.6%) status appeared to incur lower healthcare burden. Parenteral nutrition, malnutrition, and central line/bloodstream infections resulted in higher healthcare burden. CONCLUSIONS: Pediatric intestinal pseudo-obstruction is a rare diagnosis with a high incidence of inpatient admissions and healthcare burden. An aggressive multidisciplinary management is crucial in reducing inpatient admissions in this cohort.


Assuntos
Efeitos Psicossociais da Doença , Hospitalização/estatística & dados numéricos , Pacientes Internados/estatística & dados numéricos , Pseudo-Obstrução Intestinal/epidemiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estados Unidos/epidemiologia
8.
Scand J Gastroenterol ; 54(12): 1441-1447, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31814461

RESUMO

Background: Intestinal degenerative neuropathy without extra-intestinal involvement occurs as familial forms (FIDN) but the genetics behind is unknown. We studied a Swedish family with autosomal dominant disease and several cases of chronic intestinal pseudo-obstruction (CIP).Methods: We included 33 members of a family sharing a male ancestor. Chronic intestinal symptoms including diarrhoea occurred in 11, four had severe CIP. DNA was analysed with SNP-microarray (Affymetrix), linkage (Allegro Software) and gene dosage (CNAG 3.0).Results: Genetic linkage was found to the short arm of Ch9 to a 9.7 Mb region with 45 protein-coding genes, 22 of which were duplicated (1.2 Mb duplication) (dup(9)(p21.3) with breaking point in the FOCAD-gene. Lod score for the region was 3.4. Fourteen subjects were duplication carriers including all 11 subjects having severe chronic symptoms/CIP. Nineteen subjects had no duplication. The occurrence of gastrointestinal symptoms in the family was strongly linked to duplication carrier-ship (p = .0005). The two branches of the family had separate maternal ancestors (A and B). Including the previous generation, severe disease (overt CIP and/or death from intestinal failure) was assessed to occur in 100% (5/5) of duplication carriers in branch A and in 21% (3/14) in branch B (p = .005). In branch B the onset of symptoms was later (median 38 vs. 24 yrs) and three duplication carriers were symptom-free.Conclusions: In this family with autosomal dominant hereditary intestinal neuropathy, the disorder is linked to a 9.7 Mb region in Ch9 including a 1.2 Mb duplication. There is a significant difference in disease expressivity between family branches, seemingly related to separate maternal ancestors.


Assuntos
Cromossomos Humanos Par 9 , Diarreia , Transtornos Heredodegenerativos do Sistema Nervoso , Pseudo-Obstrução Intestinal , Intestinos , Proteínas do Tecido Nervoso/genética , Adulto , Doença Crônica , Diarreia/diagnóstico , Diarreia/etiologia , Família , Feminino , Duplicação Gênica , Loci Gênicos , Transtornos Heredodegenerativos do Sistema Nervoso/diagnóstico , Transtornos Heredodegenerativos do Sistema Nervoso/epidemiologia , Transtornos Heredodegenerativos do Sistema Nervoso/genética , Humanos , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/fisiopatologia , Intestinos/inervação , Intestinos/fisiopatologia , Masculino , Linhagem , Índice de Gravidade de Doença , Suécia/epidemiologia
9.
Gastroenterol Clin North Am ; 48(4): 513-524, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31668180

RESUMO

Chronic intestinal pseudo-obstruction (CIPO) is a severe form of intestinal dysmotility disorder, characterized by the impairment of gastrointestinal propulsion of the gut content in the absence of fixed occluding lesions. CIPO is a rare disease that can develop in both children and adults. CIPO is classified as primary/idiopathic, when no underlying disorder is demonstrated, or secondary, when related to systemic diseases. Diagnosis relies on the finding of chronic/recurrent obstructive type symptoms with radiological features of dilated intestine with air/fluid levels without any lumen occluding lesion. Therapy is based on nutrition, pharmacologic and surgical intervention and requires a multidisciplinary approach.


Assuntos
Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/terapia , Síndromes de Malabsorção/terapia , Doença Crônica , Transplante de Microbiota Fecal , Fármacos Gastrointestinais/uso terapêutico , Transplante de Células-Tronco Hematopoéticas , Humanos , Pseudo-Obstrução Intestinal/classificação , Pseudo-Obstrução Intestinal/epidemiologia , Intestinos/transplante , Transplante de Fígado , Síndromes de Malabsorção/etiologia , Nutrição Parenteral no Domicílio , Oligoelementos/administração & dosagem , Vitaminas/administração & dosagem
10.
Semin Arthritis Rheum ; 49(3): 405-410, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31202479

RESUMO

OBJECTIVE: Although up to 90% of systemic sclerosis (SSc) patients are affected by gastrointestinal (GI) dysmotility, the clinical phenotype of patients with pseudo-obstruction is not well-defined. We sought to identify this phenotype by studying a large cohort of SSc patients with and without pseudo-obstruction. METHODS: We performed a retrospective analysis of patients seen at the Johns Hopkins Scleroderma Center between February 2003 and September 2017. All SSc patients had clinical data prospectively collected in a longitudinal database. Cross-sectional analyses were performed comparing autoantibody status and clinical and demographic features of patients with and without pseudo-obstruction. Cox proportional hazards regression was used to identify risk factors for pseudo-obstruction. RESULTS: 175 patients with SSc had a history of pseudo-obstruction, and 2,637 SSc patients did not. After adjusting for significant variables from the univariate analysis and potential confounders, the Cox proportional hazards multivariable analysis demonstrated that older age (HR 1.02; 95%CI 1.00-1.04), male sex (HR 1.75; 95%CI 1.42-2.43), diffuse cutaneous disease (HR 2.52; 95%CI 1.59-3.99), myopathy (HR 1.83, 95%CI 1.09-3.08), and opioid use (HR 2.38; 95%CI 1.50-3.78) were predictive of pseudo-obstruction. Autoantibodies to RNA polymerase-3 were negatively associated with pseudo-obstruction (HR 0.34; 95%CI 0.17-0.66). CONCLUSION: We identified clinical features associated with pseudo-obstruction in a large US SSc cohort. This study identifies characteristics of patients with SSc who are at a higher risk of developing pseudo-obstruction and suggests that opioids may be a modifiable risk factor. These clinical features may allow for earlier diagnostic evaluation and/or therapeutic intervention for patients at risk for pseudo-obstruction.


Assuntos
Autoanticorpos/imunologia , Pseudo-Obstrução Intestinal/etiologia , Medição de Risco/métodos , Escleroderma Sistêmico/complicações , Adulto , Biópsia , Estudos Transversais , Eletromiografia , Feminino , Seguimentos , Humanos , Incidência , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/imunologia , Estados Unidos/epidemiologia
11.
Surg Oncol ; 28: 201-207, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30851901

RESUMO

BACKGROUND: Enhanced recovery after surgery (ERAS) protocols have been developed and implemented as of 2001 and may have significantly reduced several complication types including paralytic ileus. However, no formal analyses targeted paralytic ileus rates after contemporary major surgical oncology procedures. We examined temporal trends of paralytic ileus following ten major oncological surgical procedures. The effect of paralytic ileus on length of stay (LOS) and total hospital charges was examined. Univariable and multivariable linear and logistic regression analyses were used. METHODS: Between 2003 and 2013, we retrospectively identified patients, who underwent prostatectomy, colectomy, cystectomy, mastectomy, gastrectomy, hysterectomy, nephrectomy, oophorectomy, lung resection or pancreatectomy within the Nationwide Inpatient Sample. A total of 3 431 602 patients were included in our analyses. Annual paralytic ileus rate differences after major oncological surgical procedures were evaluated using linear regression. Multivariable logistic regression analyses were used to test for paralytic ileus rates determinants, as well as on the effect of paralytic ileus rates on LOS and hospital charges. RESULTS: Paralytic ileus rates ranged from 0.1% (mastectomy) to 23.2% (cystectomy) after ten examined major oncological surgical procedures. Overall annual paralytic ileus rates did not change [estimated annual percentage change (EAPC)+0.1%, p = 0.7]. Multivariable logistic regression derived predicted probabilities (PP) of paralytic ileus were highest for cystectomy (PP: 26.1%) and colectomy (PP: 17.15%) and were lowest for lung resection (PP: 2.22%) and mastectomy (PP: 0.16%). In analyses predicting LOS above the 75th percentile, paralytic ileus effect after mastectomy (OR: 14.66) and prostatectomy (OR: 13.21) ranked, as highest and second highest respectively. In analyses predicting hospital charges above the 75th percentile, paralytic ileus effect after mastectomy (OR: 2.21) and oophorectomy (OR: 1.99) ranked as highest and second highest respectively. CONCLUSIONS: Despite implementation of ERAS protocols paralytic ileus rates have not decreased over time. Gastrointestinal procedures are among the highest contributors of paralytic ileus. Moreover, procedures with short LOS represent the strongest relative contributors to LOS increases and increases in hospitalization costs.


Assuntos
Pseudo-Obstrução Intestinal/etiologia , Tempo de Internação/estatística & dados numéricos , Neoplasias/cirurgia , Complicações Pós-Operatórias , Procedimentos Cirúrgicos Operatórios/efeitos adversos , Idoso , Feminino , Seguimentos , Humanos , Pseudo-Obstrução Intestinal/epidemiologia , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasias/patologia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
12.
Pediatr Surg Int ; 35(4): 439-442, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30430282

RESUMO

INTRODUCTION: Visceral myopathies remain difficult and frustrating clinical entities, a distinctive form of acquired degenerative visceral myopathy, African degenerative leiomyopathy, a myogenic functional intestinal obstruction without aganglionosis which affects smooth muscle of the intestine, in young indigenous African children. The Actin G2 gene is the main gene encoding smooth muscle actin found in enteric tissues. Recent research has identified Actin G2 alpha gene variation as an important causative biomarker in visceral myopathies and megacystis microcolon. This study of the Actin G2 gene (ACTG2) in an African population explores a possible molecular basis abnormal muscle function in a visceral myopathy. PATIENTS AND METHODS: Following ethical permission and informed consent, DNA was extracted from whole blood samples in five patients with histologically proven African degenerative leiomyopathy. PCR amplification of ACTG2 alpha gene products by semi-automated bi-directional sequencing analysis. Results were analysed using FinchTV Sequence Alignment Software and predicting bioinformatic investigation by PolyPhen 2 software. RESULTS: Five new patients with the ADL phenotypes were prospectively investigated for variation in the Actin G2 gamma gene (ACTG2). ACTG2 gene variation occurred in exon 5 (c.463 A>G K119R), in three (60%). In addition, intronic variation t > c-IVS3 was identified in three with the K119 mutation plus further g > c -IVS12 and t > c + IVS16(2), suggesting a possible haplotype. Bioinformatic modelling showed that these ACTG2 gene variations are highly non-conservative altering protein expression. CONCLUSIONS: Recurrent Actin G2 smooth muscle gene variation in African degenerative visceral leiomyopathy is associated with abnormal muscle actin development.


Assuntos
Actinas/genética , DNA/genética , Pseudo-Obstrução Intestinal/genética , Mutação , Actinas/metabolismo , Criança , Análise Mutacional de DNA , Feminino , Variação Genética , Doença de Hirschsprung , Humanos , Incidência , Pseudo-Obstrução Intestinal/epidemiologia , Masculino , Fenótipo , Reação em Cadeia da Polimerase , África do Sul/epidemiologia
13.
Lupus ; 27(10): 1661-1669, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30028259

RESUMO

This study was performed to investigate the clinical characteristics of lupus enteritis in Japanese patients with systemic lupus erythematosus (SLE). A total of 481 patients with SLE admitted to our hospital between 2001 and 2015 were retrospectively reviewed. Diagnosis of lupus enteritis was based on the following three criteria: (1) abdominal symptoms, (2) diffuse long-segment bowel thickening and (3) a requirement for glucocorticoid therapy. Lupus enteritis was identified in 17 patients (3.5%) and there were two distinct types: small intestine-dominant and large intestine-dominant. Significant differences between the two types were noted with respect to the age, frequency of biopsy-proven lupus nephritis, frequency of rectal involvement, maximum bowel wall thickness, and requirement for steroid pulse therapy. Among patients with large intestine-dominant lupus enteritis, 60% had extra-intestinal symptoms (hydroureter, bladder wall thickening, and bile duct dilatation) that are known complications of intestinal pseudo-obstruction. Two patients with large intestine-dominant lupus enteritis developed intestinal pseudo-obstruction either before or after diagnosis of lupus enteritis. Five patients (29%) developed recurrence during a median observation period of 7.2 years (1.4-14.4 years). In conclusion, large intestine-dominant lupus enteritis resembles intestinal pseudo-obstruction and these two diseases may have a common pathogenesis.


Assuntos
Enterite/diagnóstico , Pseudo-Obstrução Intestinal/diagnóstico , Intestino Grosso/patologia , Intestino Delgado/patologia , Lúpus Eritematoso Sistêmico/diagnóstico , Adolescente , Adulto , Povo Asiático , Biópsia , Enterite/tratamento farmacológico , Enterite/epidemiologia , Enterite/patologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Incidência , Pseudo-Obstrução Intestinal/tratamento farmacológico , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/patologia , Intestino Grosso/diagnóstico por imagem , Intestino Grosso/efeitos dos fármacos , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/efeitos dos fármacos , Japão/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem
14.
Rev Med Interne ; 39(10): 792-799, 2018 Oct.
Artigo em Francês | MEDLINE | ID: mdl-29937298

RESUMO

Chronic intestinal pseudo-obstruction is a rare disease stemming from numerous causes characterized by disturbances in gastrointestinal motility. Symptomatology is often misleading and topography is variable, thus putting the clinician in serious difficulty. Diagnosis is based on a body of arguments, ranging from the clinical examination to surgical biopsies in expert centers. Treatment is non-consensual and mostly symptomatic. It is based on the use of prokinetics and optimal nutritional support. In the most serious cases, surgery can be required. The etiological treatment should be that of the causal disease when it exists and when the etiology is identified. Results of such treatment are variable. Chronic intestinal pseudo-obstruction is a disease which remains poorly understood. Progress had been made in terms of diagnosis and treatment but it seems obvious that a better comprehension of physiopathological mechanisms is necessary in order to improve our practice.


Assuntos
Pseudo-Obstrução Intestinal , Adulto , Idade de Início , Doença Crônica , Motilidade Gastrointestinal/fisiologia , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/terapia
15.
World J Urol ; 36(2): 221-229, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29167985

RESUMO

PURPOSE: Cystectomy for bladder cancer is associated with a high risk of postoperative complications. Standardized perioperative protocols, such as enhanced recovery after surgery (ERAS) protocols, aim to improve postoperative outcome. Postoperative feeding strategies are an important part of these protocols. In this two-centre study, we compared complications and length of hospital stay (LOS) between an ERAS protocol with early oral nutrition and a protocol with early enteral feeding with a Bengmark nasojejunal tube. METHODS: We retrospectively reviewed 154 consecutive patients who underwent cystectomy for bladder cancer in two hospitals (Hospital A and B) between 2014 and 2016. Hospital A uses an ERAS protocol (n = 45), which encourages early introduction of an oral diet. Hospital B uses a fast-track protocol comprising feeding with a Bengmark nasojejunal tube (Bengmark-protocol, n = 109). LOS and complications according to Clavien classification were compared between protocols. RESULTS: Overall 30-day complication rates in the ERAS and Bengmark protocol were similar (64.4 and 67.0%, respectively; p = 0.463). The rate of postoperative ileus (POI) was significantly lower in the Bengmark protocol (11.9% vs. 34.4% in the ERAS protocol, p = 0.009). This association remained significant after adjustment for other variables (odds ratio 0.32, 95% confidence interval 0.11-0.96; p = 0.042). Median LOS did not differ significantly between protocols (10 days vs. 11 days in the ERAS and Bengmark protocols, respectively; p = 0.861). CONCLUSIONS: Early oral nutrition in Hospital A was well tolerated. However, the Bengmark protocol was superior with respect to occurrence of POI. A prospective study may clarify whether the lower rate of POI was due to the use of early nasojejunal tube feeding or other reasons.


Assuntos
Carcinoma de Células de Transição/cirurgia , Cistectomia/métodos , Nutrição Enteral/métodos , Intubação Gastrointestinal/métodos , Tempo de Internação/estatística & dados numéricos , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/epidemiologia , Neoplasias da Bexiga Urinária/cirurgia , Idoso , Protocolos Clínicos , Feminino , Humanos , Pseudo-Obstrução Intestinal/epidemiologia , Masculino , Pessoa de Meia-Idade , Razão de Chances , Assistência Perioperatória/métodos , Estudos Retrospectivos
16.
Asian J Surg ; 40(1): 29-34, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26216257

RESUMO

BACKGROUND: Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. METHODS: The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. RESULTS: In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6%) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. CONCLUSION: Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.


Assuntos
Anormalidades Múltiplas/epidemiologia , Colo/anormalidades , Sistema Nervoso Entérico/anormalidades , Gânglios/anormalidades , Doença de Hirschsprung/epidemiologia , Pseudo-Obstrução Intestinal/epidemiologia , Bexiga Urinária/anormalidades , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/patologia , Colo/patologia , Diagnóstico Diferencial , Seguimentos , Inquéritos Epidemiológicos , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/patologia , Humanos , Incidência , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/patologia , Japão/epidemiologia , Prognóstico , Estudos Retrospectivos , Bexiga Urinária/patologia
17.
World Neurosurg ; 99: 302-307, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27923757

RESUMO

BACKGROUND: Ogilvie's syndrome (OS) is a relatively uncommon pathology characterized by significant colonic dilation in the absence of mechanical obstruction. If unrecognized and untreated, cecal perforation resulting in a mortality rate of 25%-71% may occur. It is a potentially underdiagnosed condition in the lateral transpsoas approach population because of its uncommon nature and imitation of other well-known pathologies. METHODS: Two thousand nine hundred and thirty patients from 6 separate institutions were retrospectively reviewed since 2007 and screened for OS. All patients underwent a minimum of single-level lateral transpsoas fusion. Diagnostic criteria included signs of a postoperative paralytic ileus combined with abdominal computed tomography showing a cecal diameter greater than 9 cm. Treatment modalities and outcomes were recorded. RESULTS: Eight cases (0.22%) of OS were diagnosed at 6 separate institutions. Most institutions recorded more than 350 lateral access procedures. Four cases were initially diagnosed as a routine postoperative ileus; however, they failed conservative therapy and underwent abdominal CT imaging. Neostigmine treatment was required for 1 patient in the intensive care unit setting, and 3 patients were managed conservatively without complications. Four other patients demonstrated bowel perforation at least 48 hours after surgery and required laparotomy with diversion ileostomy. CONCLUSION: Ogilvie's syndrome is a rare but potentially fatal complication that can mimic a postoperative ileus. It is likely underdiagnosed in the lateral transpsoas approach population because of its uncommon nature and a high index of suspicion should remain. Neostigmine can be administered safely under close observation with immediate and successful outcomes. Patients with perforation require urgent laparotomy and primary repair.


Assuntos
Pseudo-Obstrução do Colo/epidemiologia , Perfuração Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Fusão Vertebral , Idoso , Idoso de 80 Anos ou mais , Ceco/diagnóstico por imagem , Ceco/patologia , Inibidores da Colinesterase/uso terapêutico , Pseudo-Obstrução do Colo/diagnóstico por imagem , Pseudo-Obstrução do Colo/terapia , Feminino , Humanos , Ileostomia , Perfuração Intestinal/diagnóstico por imagem , Perfuração Intestinal/cirurgia , Pseudo-Obstrução Intestinal/diagnóstico por imagem , Pseudo-Obstrução Intestinal/terapia , Laparotomia , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Neostigmina/uso terapêutico , Tamanho do Órgão , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/terapia , Radiografia Abdominal , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
18.
Clin Nutr ; 36(4): 1165-1169, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-27624996

RESUMO

BACKGROUND & AIMS: Home parenteral nutrition (HPN) improves survival and quality of life in patients with chronic intestinal failure (IF). Few cases of pregnancy on HPN have been published. The aim of this study was to report pregnancy cases on long-term HPN in benign IF. METHODS: This retrospective study included all pregnant patients on HPN from 4 HPN referral centers. Data on demographics, ongoing pathology, HPN type, maternal and newborn complications were collected. RESULTS: From 1984 to 2014, 21 pregnancies occurred in 15 patients (short bowel syndrome (n = 11), motility disorders (n = 3), mucosal disease (n = 1)) of whom 14 occurred after 2010. Median follow-up was 12 years. Median HPN duration before pregnancy was 8 years. HPN was adapted monthly during pregnancy, with close monitoring and supplementations. Energy intake was regularly increased and median maternal weight gain was 10 kg. Median age at the first pregnancy was 27 years. In 55% of cases, the newborn was preterm. Maternal complications occurred in 67% of cases (mainly due to underlying disease or HPN complications). There were 3 post-partum hemorrhages and 6 hypotrophic newborns. Eighteen infants were healthy and 2 chronic intestinal pseudo-obstruction (CIPO) were suspected. CONCLUSION: Our series, the largest reported to date, shows that pregnancy is possible in HPN patients but the complication rate is high. A specific support is necessary, particularly in CIPO patients. As pregnancies have increased over the last 15 years, physicians practicing in HPN referral centers should be aware of the need for implementing a specific multidisciplinary monitoring in HPN patients considering pregnancy.


Assuntos
Enteropatias/terapia , Fenômenos Fisiológicos da Nutrição Materna , Nutrição Parenteral Total no Domicílio/efeitos adversos , Complicações na Gravidez/terapia , Gravidez de Alto Risco , Adulto , Estudos de Coortes , Feminino , Retardo do Crescimento Fetal/epidemiologia , Retardo do Crescimento Fetal/etiologia , Seguimentos , França/epidemiologia , Humanos , Recém-Nascido , Enteropatias/fisiopatologia , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Masculino , Hemorragia Pós-Parto/epidemiologia , Hemorragia Pós-Parto/etiologia , Gravidez , Complicações na Gravidez/epidemiologia , Complicações na Gravidez/etiologia , Complicações na Gravidez/fisiopatologia , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia , Estudos Retrospectivos , Risco , Índice de Gravidade de Doença , Síndrome do Intestino Curto/fisiopatologia , Síndrome do Intestino Curto/terapia
19.
Surgery ; 159(5): 1333-41, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26747224

RESUMO

BACKGROUND: We assessed the efficacy of TJ-100 taken perioperatively to recovery among patients with periampullary tumor or tumor of the head of the pancreas who underwent pancreaticoduodenectomy (PD). PATIENTS AND METHODS: In this multicenter, randomized, double-blinded, placebo-controlled, phase II trial (JAPAN-PD Study), patients were assigned randomly in a 1:1 ratio to receive TJ-100 or placebo. The coprimary endpoints were (1) incidence of postoperative paralytic ileus lasting >72 hours after surgery and (2) time to occurrence of postoperative paralytic ileus. This trial is registered at the UMIN Clinical Trials Registry (000007975) and at ClinicalTrials.gov (NCT01607307). RESULTS: From August 2012 through July 2013, we assessed 273 patients for eligibility, and 224 underwent randomization; 112 patients received TJ-100, and 112 patients received placebo. The population for analysis consisted of 104 patients who received TJ-100 and 103 who received placebo. Paralytic ileus occurred 35 (33.7%) in the TJ-100 group and 38 (36.9%) in the placebo group (P = .626). Time to first flatus was 2.25 (2.00-2.50) days in the TJ-100 group and 2.50 (1.50-2.50) days in the placebo group (P = .343). Among 23 patients who underwent a pylorus ring-preserving PD, time to first flatus was lower in the TJ-100 group than in the placebo group: 0.50 (0.50-1.00) days versus 1.50 (0.50-3.00) days (P = .034). CONCLUSION: Our findings suggest that use of TJ-100 did not improve recovery from paralytic ileus after PD, and may preclude the routine use of TJ-100 in clinical practice after PD operation.


Assuntos
Fármacos Gastrointestinais/uso terapêutico , Pseudo-Obstrução Intestinal/prevenção & controle , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia , Extratos Vegetais/uso terapêutico , Complicações Pós-Operatórias/prevenção & controle , Adulto , Idoso , Idoso de 80 Anos ou mais , Método Duplo-Cego , Esquema de Medicação , Feminino , Humanos , Incidência , Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Panax , Assistência Perioperatória , Complicações Pós-Operatórias/epidemiologia , Modelos de Riscos Proporcionais , Resultado do Tratamento , Zanthoxylum , Zingiberaceae
20.
J Rheumatol ; 43(3): 559-64, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26773109

RESUMO

OBJECTIVE: To analyze the epidemiology, clinical characteristics, and risk factors for systemic lupus erythematosus-related intestinal pseudo-obstruction (SLE-IPO). METHODS: We retrospectively examined 85 patients with SLE with IPO as the case group and 255 randomly matched patients with SLE without any gastrointestinal manifestations as the control group, out of 4331 inpatients at the Peking Union Medical College Hospital (PUMCH) from 2003 to 2014. RESULTS: Over the last 11 years at PUMCH, the prevalence of IPO in patients with SLE was 1.96% and the in-hospital fatality rate was 7.1%. Of these patients, 57.6% presented with IPO as the initial affected system of SLE, and the rate of misdiagnosis was about 78%. Pyeloureterectasis was the most common complication (58.9%) in patients with SLE-IPO and the incidence of biliary tract dilation was 7.1%. Patients with SLE with IPO were always diagnosed at an earlier stage of SLE with a higher frequency of hematological disturbance, polyserositis, and hypocomplementemia. Pyeloureterectasis, hypocomplementemia, and elevated C-reactive protein levels in serum were independent risk factors for IPO in SLE disease. Patients with SLE-IPO with long IPO duration and those diagnosed during late stages of SLE or concurrent with pyeloureterectasis and megacholedochus always had an unfavorable outcome. CONCLUSION: IPO is a rare complication, but commonly presents as the initial affected system of SLE, which can lead to a difficult diagnosis and delayed treatment. SLE-IPO occurrence concomitantly with pyeloureterectasis and megacholedochus showed a severe clinical situation in our cohort. Thus, patients with SLE-IPO with systemic smooth muscular involvement should be diagnosed early and treated aggressively.


Assuntos
Pseudo-Obstrução Intestinal/epidemiologia , Pseudo-Obstrução Intestinal/etiologia , Lúpus Eritematoso Sistêmico/complicações , Adulto , Estudos de Casos e Controles , Erros de Diagnóstico , Feminino , Glomerulonefrite Membranoproliferativa/diagnóstico , Glomerulonefrite Membranoproliferativa/etiologia , Mortalidade Hospitalar , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Masculino , Prevalência , Estudos Retrospectivos , Fatores de Risco
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