Mitochondrial neurogastrointestinal encephalomyopathy(MNGIE),a rare mitochondrial disorder caused by TYMP gene mutations,is characterized by severe gastrointestinal dysmotility,peripheral neuropathy,and leukodystrophy.This article summarizes the clinical data and intestinal ultrasound findings of a MNGIE case,aiming to provide insights for clinical diagnosis and treatment.
Humans ; Mitochondrial Encephalomyopathies/diagnostic imaging* ; Ultrasonography ; Intestines/diagnostic imaging* ; Male ; Female ; Intestinal Pseudo-Obstruction/diagnostic imaging* ; Ophthalmoplegia/congenital* ; Muscular Dystrophy, Oculopharyngeal

Bromadiolone, commonly known as super warfarin, is a long-acting coumarin dicoumarin rodenticide. The mechanism of bromadiolone is mainly to inhibit vitamin K1 epoxide reductase and affect the synthesis of coagulation factors Ⅱ, Ⅶ, Ⅸ and Ⅹ, which causes blood coagulation dysfunction and systemic multiple organ hemorrhage. Here, we report of a case of bromadiolone poisoning patient who had digestive tract, abdominal hemorrhage, as well as secondary paralytic ileus. After blood product transfusion and vitamin K1 supplementation, the patient was discharged after the physical condition was improved. It's suggestied that clinicians should pay attention to rare complications to prevent missed diagnosis when treating other bromadiolone poisoning.
4-Hydroxycoumarins ; Blood Coagulation Factors ; Dicumarol ; Hemorrhage ; Humans ; Intestinal Pseudo-Obstruction/chemically induced* ; Oxidoreductases ; Rodenticides ; Vitamin K 1 ; Warfarin

Humans ; Intestinal Obstruction/complications* ; Ileus/etiology* ; Abdominal Injuries/complications*

OBJECTIVE: To observe the effect of electroacupuncture (EA) on postoperative ileus after laparotomy for gastrointestinal cancer. METHODS: A total of 90 patients with postoperative ileus after laparotomy for gastrointestinal cancer were randomized into an EA group and a conventional treatment group, 45 cases in each one. In the conventional treatment group, the postoperative fast track surgical regimen was accepted. In the EA group, on the base of the treatment as the conventional treatment group, acupuncture was applied to Zusanli (ST 36), Shangjuxu (ST 37), Yinlingquan (SP 9) and Taichong (LR 3) and electric stimulation was attached on Zusanli (ST 36) and Yinlingquan (SP 9), with continuous wave, 2 Hz in frequency and 3-5 mA in intensity. Acupuncture was provided once daily till the onset of postoperative exhaust and defecation. The first postoperative exhaust time, the first postoperative defecation time, the postoperative hospital stay and the wound pain under standing on the next morning after entering group were compared in the patients between the two groups. The impact of the EA expectation was analyzed on the first postoperative exhaust time, the first postoperative defecation time and the postoperative hospital stay separately. RESULTS: The first postoperative exhaust time and the first postoperative defecation time in the EA group were earlier than the conventional treatment group (P<0.05), the postoperative hospital stay was shorter than the conventional treatment group (P<0.05), and the rate of wound pain in the postoperative standing was lower than the conventional treatment group (P<0.05). EA expectation had no obvious correlation with the clinical therapeutic effect (P>0.05). CONCLUSION EA can relieve postoperative ileus symptoms, alleviate pain and shorten hospital stay in the patients after laparotomy for gastrointestinal cancer.
Acupuncture Points ; Electroacupuncture ; Gastrointestinal Neoplasms ; Humans ; Ileus/therapy* ; Laparotomy/adverse effects*

OBJECTIVE: In an aging society, the number of patients with symptomatic degenerative spondylolisthesis (DS) is increasing and there is an emerging need for fusion surgery. However, few studies have compared transforaminal lumbar interbody fusion (TLIF) and lateral lumbar interbody fusion (LLIF) for the treatment of patients with DS. The purpose of this study was to investigate the clinical and radiological outcomes between TLIF and LLIF in DS.METHODS: We enrolled patients with symptomatic DS at L4–5 who underwent TLIF with open pedicle screw fixation (TLIF group, n=41) or minimally invasive LLIF with percutaneous pedicle screw fixation (LLIF group, n=39) and were followed-up for more than one year. Clinical (visual analog scale and Oswestry disability index) and radiological outcomes (spondylolisthesis rate, segmental sagittal angle [SSA], mean disc height [MDH], intervertebral foramen height [FH], cage subsidence, and fusion rate) were assessed. And we assessed the changes in radiological parameters between the postoperative and the last follow-up periods.RESULTS: Preoperative radiological parameters were not significantly different between the two groups. LLIF was significantly superior to TLIF in immediate postoperative radiological results, including reduction of spondylolisthesis rate (3.8% and 7.2%), increase in MDH (13.9 mm and 10.3 mm) and FH (21.9 mm and 19.4 mm), and correction of SSA (18.9° and 15.6°) (p<0.01), and the changes were more stable from the postoperative period to the last follow-up (p<0.01). Cage subsidence was observed significantly less in LLIF (n=6) than TLIF (n=21). Fusion rate was not different between the two groups. The clinical outcomes did not differ significantly at any time point between the two groups. Complications were not statistically significant. However, TLIF showed chronic mechanical problems with screw loosening in four patients and LLIF showed temporary symptoms associated with the surgical approach, such as psoas and ileus muscle symptoms in three and two cases, respectively.CONCLUSION: LLIF was more effective than TLIF for spondylolisthesis reduction, likely due to the higher profile cage and ligamentotactic effect. In addition, LLIF showed mechanical stability of the reduction level by using a cage with a larger footprint. Therefore, LLIF should be considered a surgical option before TLIF for patients with unstable DS.
Aging ; Follow-Up Studies ; Humans ; Ileus ; Minimally Invasive Surgical Procedures ; Pedicle Screws ; Postoperative Period ; Spinal Fusion ; Spondylolisthesis

BACKGROUND/AIMS: Previous studies from Korea have described chronic intestinal pseudo-obstruction (CIPO) patients with transition zone (TZ) in the colon. In this study, we evaluated the pathological characteristics and their association with long-term outcomes in Korean colonic pseudo-obstruction (CPO) patients with TZ. METHODS: We enrolled 39 CPO patients who were refractory to medical treatment and underwent colectomy between November 1989 and April 2016 (median age at symptoms onset: 45 [interquartile range, 29–57] years, males 46.2%). The TZ was defined as a colonic segment connecting a proximally dilated and distally non-dilated segment. Detailed pathologic analysis was performed. RESULTS: Among the 39 patients, 37 (94.9%) presented with TZ and 2 (5.1%) showed no definitive TZ. Median ganglion cell density in the TZ adjusted for the colonic circumference was significantly decreased compared to that in proximal dilated and distal non-dilated segments in TZ (+) patients (9.2 vs 254.3 and 150.5, P < 0.001). Among the TZ (+) patients, 6 showed additional pathologic findings including eosinophilic ganglionitis (n = 2), ulcers with combined cytomegalovirus infection (n = 2), diffuse ischemic changes (n = 1), and heterotropic myenteric plexus (n = 1). During follow-up (median, 61 months), 32 (82.1%) TZ (+) patients recovered without symptom recurrence after surgery. The presence of pathological features other than hypoganglionosis was an independent predictor of symptom recurrence after surgery (P = 0.046). CONCLUSIONS: Hypoganglionosis can be identified in the TZ of most Korean CPO patients. Detection of other pathological features in addition to TZ-associated hypoganglionosis was associated with poor post-operative outcomes.
Cell Count ; Colectomy ; Colon ; Colonic Pseudo-Obstruction ; Cytomegalovirus Infections ; Eosinophils ; Follow-Up Studies ; Ganglion Cysts ; Humans ; Intestinal Pseudo-Obstruction ; Korea ; Male ; Myenteric Plexus ; Pathology ; Recurrence ; Ulcer

BACKGROUND: To evaluate the efficacy and safety of fentanyl for sedation therapy in mechanically ventilated children. METHODS: This was a double-blind, randomized controlled trial of mechanically ventilated patients between 2 months and 18 years of age. Patients were randomly divided into two groups; the control group with midazolam alone, and the combination group with both fentanyl and midazolam. The sedation level was evaluated using the Comfort Behavior Scale (CBS), and the infusion rates were adjusted according to the difference between the measured and the target CBS score. RESULTS: Forty-four patients were recruited and randomly allocated, with 22 patients in both groups. The time ratio of cumulative hours with a difference in CBS score (measured CBS–target CBS) of ≥ 4 points (i.e., under-sedation) was lower in the combination group (median, 0.06; interquartile range [IQR], 0–0.2) than in the control group (median, 0.15; IQR, 0.04–0.29) (P < 0.001). The time ratio of cumulative hours with a difference in CBS score of ≥ 8 points (serious under-sedation) was also lower in the combination group (P < 0.001). The cumulative amount of midazolam used in the control group (0.11 mg/kg/hr; 0.07–0.14 mg/kg/hr) was greater than in the combination group (0.07 mg/kg/hr; 0.06–0.11 mg/kg/hr) (P < 0.001). Two cases of hypotension in each group were detected but coma and ileus, the major known adverse reactions to fentanyl, did not occur. CONCLUSION: Fentanyl combined with midazolam is safe and more effective than midazolam alone for sedation therapy in mechanically ventilated children. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02172014
Child* ; Coma ; Fentanyl* ; Humans ; Hypotension ; Ileus ; Midazolam* ; Respiration, Artificial*

OBJECTIVE: To evaluate the feasibility of robotic single-site myomectomy (RSSM). METHODS: Medical records of 355 consecutive women who underwent robotic-assisted laparoscopic myomectomy were retrospectively reviewed. Clinical characteristics were compared between multi-site and single-site systems. After 1:1 propensity score matching for the total myoma number, largest myoma size, and total tumor weight (105 women in each group), surgical outcomes were also compared between the 2 systems. RESULTS: A total of 105 (29.6%) and 250 (70.4%) women underwent RSSM and robotic multi-site myomectomy (RMSM), respectively. RSSM was more commonly performed in women with lower body mass index (21.6 vs. 22.5 kg/m2, P=0.014), without peritoneal adhesions (7.6% vs. 24.8%, P < 0.001), and less (2.6 vs. 4.6, P < 0.001) and smaller (6.3 vs. 7.7 cm, P < 0.001) myomas compared to RMSM. After propensity score matching, the largest myoma size (P=0.143), total myoma number (P=0.671), and tumor weight (P=0.510) were not significantly different between the 2 groups. Although the docking time was significantly longer in the RSSM group (5.1 vs. 3.8 minutes, P=0.005), total operation time was similar between RSSM and RMSM groups (145.9 vs. 147.3 minutes, P=0.856). Additionally, hemoglobin decrement was lower in the RSSM group than in the RMSM group (1.4 vs. 1.8 g/dL, P=0.009). No surgical complication was observed after RSSM, while 1 ileus and 2 febrile complications occurred in women that underwent RMSM (0% vs. 2.9%, P=0.246). CONCLUSION: Although RMSM is preferred for women with multiple large myomas in real clinical practice, RSSM seems to be a feasible surgical method for less complicated cases, and is associated with minimal surgical morbidity.
Body Mass Index ; Feasibility Studies ; Female ; Humans ; Ileus ; Medical Records ; Methods ; Myoma ; Propensity Score ; Retrospective Studies ; Robotic Surgical Procedures ; Tumor Burden

PURPOSE: To assess the feasibility of transanal total mesorectal excision in difficult cases including obese patients or patients with bulky tumors or threatened mesorectal fascias. METHODS: We performed laparoscopy-assisted transanal total mesorectal excision in patients with biopsy-proven rectal adenocarcinoma located 3–12 cm from the anal verge as part of a prospective, single arm, pilot trial. The primary endpoint was resection quality and circumferential resection margin involvement. Secondary endpoints included the number of harvested lymph nodes and 30-day postoperative complications. RESULTS: A total of 12 patients (9 men and 3 women) were enrolled: one obese patient, 7 with large tumors and 8 with threatened mesorectal fascias (4 patients had multiple indications). Tumors were located a median of 5.5 cm from the anal verge, and all patients received preoperative chemoradiotherapy. Median operating time was 191 minutes, and there were no intraoperative complications. One patient needed conversion to open surgery for ureterocystostomy after en bloc resection. Complete or near-complete excision and negative circumferential resection margins were achieved in all cases. The median number of harvested lymph nodes was 15.5. There was no postoperative mortality and 3 cases of postoperative morbidity (1 postoperative ileus, 1 wound problem near the stoma site, and 1 anastomotic dehiscence). CONCLUSION: This pilot study showed that transanal total mesorectal excision is also feasible in difficult laparoscopic cases such as in obese patients or those with bulky tumors or tumors threatening the mesorectal fascia. Additional larger studies are needed.
Adenocarcinoma ; Arm ; Chemoradiotherapy ; Conversion to Open Surgery ; Fascia ; Humans ; Ileus ; Intraoperative Complications ; Laparoscopy ; Lymph Nodes ; Male ; Mortality ; Pilot Projects ; Postoperative Complications ; Prospective Studies ; Rectal Neoplasms ; Transanal Endoscopic Surgery ; Wounds and Injuries

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting “nutritional failure” which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.
Bone Diseases ; Child ; Citrulline ; Enterocytes ; Humans ; Intestinal Diseases ; Intestinal Mucosa ; Intestinal Pseudo-Obstruction ; Liver Diseases ; Micronutrients ; Parenteral Nutrition ; Parenteral Nutrition, Home ; Prognosis ; Rehabilitation ; Short Bowel Syndrome