Probiotics may alleviate intestinal damage induced by cardiopulmonary bypass in children.

OBJECTIVES: Intestinal ischaemia-reperfusion injury induced by cardiopulmonary bypass causes intestinal epithelial barrier dysfunction, leading to dysbiosis and bacterial translocation. We conducted a randomized prospective study with 2 objectives: (i) to investigate epithelial barrier dysfunction and bacterial translocation induced by cardiopulmonary bypass and changes in the gut microbiota and (ii) to verify whether probiotics can improve these conditions. METHODS: Between 2019 and 2020, patients 0-15 years old scheduled to undergo cardiac surgery using cardiopulmonary bypass were enrolled and randomly allocated to 2 groups: the intervention group received probiotics and the control group did not receive probiotics. We analysed the microbiota in faeces and blood, organic acid concentrations in faeces, plasma intestinal fatty acid-binding protein and immunological responses. RESULTS: Eighty-two patients were enrolled in this study. The characteristics of the patients were similar in both groups. The total number of obligate anaerobes was higher in the intervention group than in the control group after postoperative day 7. We identified 4 clusters within the perioperative gut microbiota, and cluster changes showed a corrective effect of probiotics on dysbiosis after postoperative day 7. Organic acid concentrations in faeces, incidence of bacterial translocation, intestinal fatty acid-binding protein levels and immunological responses, except for interleukin -17A, were not markedly different between the 2 groups. CONCLUSIONS: Administration of probiotics was able to correct dysbiosis but did not sufficiently alleviate the intestinal damage induced by cardiopulmonary bypass. More effective methods should be examined to prevent disturbances induced by cardiac surgery using cardiopulmonary bypass. CLINICAL TRIAL REGISTRATION NUMBER: https://center6.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000037174 UMIN000035556.

Surgical tricks of handling a needle and grasping forceps during laparoscopic percutaneous extraperitoneal closure (LPEC) for pediatric inguinal hernia.

INTRODUCTION: Laparoscopic percutaneous extraperitoneal closure (LPEC) is an alternative to open repair for pediatric inguinal hernias; however, its application for boys remains controversial. In this study, we developed a technique to enhance the safety and feasibility of LPEC. MATERIAL AND SURGICAL TECHNIQUE: In our technique, forceps are used to pull up the peritoneum ahead on the route, creating a space between the peritoneum and structures, including gonadal vessels and vas deferens. This potentially decreases the risk of perioperative injury of these structures. This technique also allows the needle to pass on the shortest course around the inguinal ring without crossing the vas deferens, possibly lowering the likelihood of injury and preventing excessively high ligation of the vaginalis process. DISCUSSION: Our technique diversifies the LPEC methods, thereby augmenting the feasibility and safety of the procedure.

Occult bacteremia in living donor liver transplantation: a prospective observational study of recipients and donors.

PURPOSE: To investigate the incidence and clinical impact of occult bacteremia in liver transplantation (LT). METHODS: This prospective observational study involved a fixed-point observation for up to 2 weeks after living donor LT in 20 recipients, with 20 donors as comparison subjects. Bacteria in the blood samples were detected using the ribosomal RNA-targeted reverse-transcription quantitative polymerase chain reaction method. To identify the causality with the gut microbiota (GM), fecal samples were collected and analyzed simultaneously. RESULTS: Occult bacteremia was identified in four recipients (20%) and three donors (15%) before the operation, and in seven recipients (35%) and five donors (25%) after the operation. Clostridium leptum subgroup, Prevotella, Colinesella, Enterobacteriaceae, and Streptococcus were the main pathogens responsible. Although it did not negatively affect the donor post-hepatectomy outcomes, the recipients with occult bacteremia had a higher rate of infectious complications post-LT. The GM analyses showed fewer post-LT predominant obligate anaerobes in both the recipients and donors with occult bacteremia. CONCLUSIONS: Occult bacteremia is a common condition that occurs in both donors and recipients. While occult bacteremia generally remains subclinical in the healthy population, there is potential risk of the development of an apparent post-LT infection in recipients who are highly immunosuppressed.

Efficacy of adhesive strapping on umbilical hernia in children: a systematic review and meta-analysis of cohort studies.

Background: Although adhesive strapping (AS) for pediatric umbilical hernia (UH), which was once obsolete, has been reconsidered as a common practice in Japan, its efficacy is still unclear. This study aimed to evaluate its efficacy by reviewing related articles. Methods: A comprehensive literature search of PubMed, Cochrane, Google Scholar, and Igaku Chuo Zasshi via Ichushi-Web was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Cohort studies reporting on the UH closure rate after AS compared with observation-only management were included. Results: A total of 10 cohort studies were included, and the overall UH closure rate was not statistically significant (p=0.31, risk ratio (RR)=0.76, 95% confidence interval (CI) 0.45 to 1.28). However, there were significant differences in the UH closure rate at the age of 6 months (p<0.01, RR=0.55, 95% CI 0.41 to 0.75) and the efficacy of preventing protruding umbilici with redundant skin (p=0.049, RR=0.16, 95% CI 0.03 to 0.99). Conclusions: Although the efficacy of AS on UH compared with observation-only management did not differ in terms of the UH closure rate, the application of AS may be effective for faster UH closure and the prevention of protruding umbilici. However, due to the high heterogeneity of the study, further large-scale studies, particularly randomized controlled trials, are warranted to reach a conclusion. PROSPERO registration number: CRD42022314417.

Gut microbiota associated with the mitigation effect of synbiotics on adverse events of neoadjuvant chemotherapy in patients with esophageal cancer: A retrospective exploratory study.

Introduction. Our synbiotics (Lacticaseibacillus paracasei strain Shirota, Bifidobacterium breve strain Yakult, and galacto-oligosaccharides: LBG) helps mitigate serious adverse events such as febrile neutropenia (FN) and diarrhoea in oesophageal cancer patients receiving neoadjuvant chemotherapy (NAC). Unfortunately, LBG therapy does not benefit all patients.Hypothesis/Gap Statement. Identification of the gut microbiota species involved in adverse events during chemotherapy could help predict the onset of adverse events. Identification of the gut microbiota that influence the efficacy of LBG could also help establish a diagnostic method to identify patients who will respond to LBG before the initiation of therapy.Aim. To identify the gut microbiota involved in adverse events during NAC and that affect the efficacy of LBG therapy.Methodology. This study was ancillary to a parent randomized controlled trial in which 81 oesophageal cancer patients were recruited and administered either prophylactic antibiotics or LBG combined with enteral nutrition (LBG+EN). The study included 73 of 81 patients from whom faecal samples were collected both before and after NAC. The gut microbiota was analysed using 16S rRNA gene amplicon sequencing and compared based on the degree of NAC-associated adverse events. Furthermore, the association between the counts of identified bacteria and adverse events and the mitigation effect of LBG+EN was also analysed.Results. The abundance of Anaerostipes hadrus and Bifidobacterium pseudocatenulatum in patients with no FN or only mild diarrhoea was significantly higher (P<0.05) compared to those with FN or severe diarrhoea. Moreover, subgroup analyses of patients receiving LBG+EN showed that the faecal A. hadrus count before NAC was significantly associated with a risk of developing FN (OR, 0.11; 95 % CI, 0.01-0.60, P=0.019). The faecal A. hadrus count after NAC was positively correlated with intestinal concentrations of acetic acid (P=0.0007) and butyric acid (P=0.00005).Conclusion. Anaerostipes hadrus and B. pseudocatenulatum may be involved in the ameliorating adverse events and can thus be used to identify beforehand patients that would benefit from LBG+EN during NAC. These results also suggest that LBG+EN would be useful in the development of measures to prevent adverse events during NAC.

Do heel-unloading orthoses improve clinical outcomes in patients after surgical treatment of calcaneal fracture? A propensity-matched, multicenter analysis of the TRON database.

BACKGROUND: Postoperative protocols after surgical treatment of calcaneal fracture have not been standardized to date. There are only a few reports on the efficacy of heel-unloading orthoses (HUOs; Mars shoe, Graffin orthosis), and thier efficacy is uncertain. OBJECTIVES: The purpose of this study was to compare postoperative radiologic and clinical outcomes in patients with calcaneal fractures who used Graffin orthosis. STUDY DESIGN: Multicenter retrospective study. METHODS: We finally extracted 182 patients from a database of the Trauma Research Group of Nagoya and divided them into two groups: group C (underwent casting or splinting only) and group O (Graffin orthosis was used). A propensity score algorithm was used to match group C to group O in a 1:1 ratio. We evaluated American Orthopaedic Foot and Ankle Society (AOFAS) score at three and six months after surgery and at final follow-up. Differences in reduction of the Böhler angle between the two groups were evaluated radiographically. All data were analyzed with a t-test or Fisher's exact test. P < .05 was considered statistically significant. RESULTS: The AOFAS score 3 months after surgery in group O was significantly higher than that in group C (69.57 vs. 77.22; P = .004). However, there were no statistically significant differences between group C vs. group O in AOFAS scores at 6 months after surgery and at final follow-up (81.92 vs. 85.67 and 89.18 vs. 88.13; P = .087 and 0.597, respectively). There was no significant statistical difference in the reduction of the Böhler angle (5.07 vs. 5.89; P = .529). CONCLUSIONS: At 3 months postoperatively, the orthosis group showed predominantly better functional results. We believe that heel-unloading orthoses are useful for patients who require an early return to work and to daily life.

Comparison of the risk of appendiceal tumors in uncomplicated and complicated appendicitis.

BACKGROUND: Interval appendectomy or non-operative management is commonly performed for complicated appendicitis in adult patients. However, these treatments are still controversial because the incidence rate of appendiceal tumors recognized after interval appendectomy is reportedly higher than that after emergency appendectomy. Thus, this study aimed to compare the appendiceal tumor rates between uncomplicated and complicated appendicitis. METHODS: This study was a retrospective review of patients with appendicitis who underwent surgical removal at a single institution over 7.5 years. The primary objective was the comparison of the incidence rate of appendiceal tumors using propensity score matching, and the secondary objective was the same comparison among older patients, defined as patients aged ≥ 60 years. RESULTS: A total of 1277 patients were included. Of these patients, 297 (23.3%) were preoperatively diagnosed with complicated appendicitis. Moreover, 22 (1.7%) patients, including 14 cases of complicated appendicitis and 8 cases of uncomplicated appendicitis, were diagnosed with appendiceal tumors based on pathological examination. No significant difference was found in the incidence rate of appendiceal tumors between the two groups after matching for patients' background, including age, sex, and history of appendicitis by propensity score matching (P = 0.073). However, among patients aged ≥ 60 years, the incidence of appendiceal tumors was significantly higher in complicated than in uncomplicated appendicitis (P = 0.006). CONCLUSIONS: Although the overall risk of appendiceal tumors did not differ between complicated and uncomplicated appendicitis when analyzed by the propensity score matching, in older patients aged ≥ 60 years, the risk increased among those with complicated appendicitis. Therefore, although the incidence is low, complicated appendicitis, particularly, among older patients, should be examined carefully and be performed IA when unusual findings exist.

Comparison of synbiotics combined with enteral nutrition and prophylactic antibiotics as supportive care in patients with esophageal cancer undergoing neoadjuvant chemotherapy: A multicenter randomized study.

BACKGROUND & AIMS: Established supportive care to reduce the toxicity of neoadjuvant chemotherapy (NAC) is lacking. This multicenter randomized study compared the administration of synbiotics combined with enteral nutrition (EN) versus that of prophylactic antibiotics as supportive care treatment for patients with esophageal cancer undergoing NAC. METHODS: Patients with advanced esophageal cancer scheduled to receive NAC were randomly administered either prophylactic antibiotics (antibiotic group) or synbiotics combined with EN (Syn + EN group). The primary endpoint was the febrile neutropenia (FN) incidence during the first course, and the secondary endpoints were other adverse events, changes in intestinal environment, including fecal microbiota, organic acid concentrations, pH, and chemotherapy tolerability. RESULTS: Eighty-one patients were enrolled. The FN incidence was nonsignificantly lower (P = 0.088) in the Syn + EN group. The incidences of grade 4 neutropenia and grades 2-4 diarrhea were significantly lower in the Syn + EN group (P = 0.014 and 0.033, respectively). Relative dose intensity was significantly higher in the Syn + EN group (92.0 ± 10.9%) than in the antibiotic group (83.2 ± 18.2%) (P = 0.01). Alpfa diversity was significantly higher in the Syn + EN group than in the antibiotic after chemotherapy (P = 0.002). The numbers of Bifidobacterium (P < 0.05), Lacticaseibacillus (P < 0.001), and Enterobacteriaceae (P < 0.001) and the concentration of acetic acid (P < 0.001) were significantly higher in the Syn + EN group than in the antibiotic group after chemotherapy. The severity of diarrhea and occurrence of FN were significantly correlated with Clostridioides difficile abundance and were significantly inversely correlated with acetic acid concentration after chemotherapy. CONCLUSIONS: Synbiotics combined with EN may be an alternative supportive care treatment to prophylactic antibiotics in patients with cancer undergoing toxic chemotherapy (https://jrct.niph.go.jp; jRCTs051180153).

Two-Stage Portal Vein Ligation Facilitates Liver Regeneration Safely in Rats with Liver Cirrhosis.

BACKGROUND: Portal vein (PV) embolization is performed prior to extended hepatectomy for the damaged liver to increase future remnant liver volume and prevent postoperative liver failure. This study examined whether two-stage PV ligation (PVL) increased regeneration and hypertrophy of the future remnant liver compared to conventional PVL, and whether two-stage PVL was safe for damaged liver. METHOD: We produced a cirrhotic liver rat model with perioperatively maintained fibrosis. Rats were divided into: Group A (70%PVL), ligation of left branch of PV; Group B (90%PVL), ligation of right and left branches of PV; and Group C (two-stage 90%PVL), two-stage PVL with left branch ligation of PV followed by right branch ligation 7 days later. To evaluate liver regeneration, liver weight ratios, proliferating cell nuclear antigen (PCNA) labeling index (LI), mitotic index (MI), and TdT-mediated dUTP-biotin nick end labeling (TUNEL) LI in the non-ligated caudate lobe were measured. RESULTS: Fourteen-day survival rate was 20% in Group B but 100% in Group C. TUNEL LI differed significantly between Groups A and B at 2 and 7 days postoperatively. Weight ratios were significantly higher in Group C than in Groups A and B at 14 days postoperatively. PCNA LI and MI in the non-ligated caudate lobe decreased to preoperative levels by 7 days postoperatively in Groups A and B, but remained elevated until 14 days postoperatively in Group C. CONCLUSION: In cirrhotic liver rats, two-stage PVL avoided the lethal liver failure seen with one-stage PVL, and significantly facilitated liver regeneration more than one-stage PVL.

Plate fixation through the lateral extensile approach versus cannulated screw fixation through the sinus tarsi approach for calcaneal fracture: a multicenter, propensity score-matched TRON study.

PURPOSE: For calcaneal fracture, plate fixation through lateral extensive approach (LEP) is the most common procedure performed to achieve anatomic reduction. However, wound complications sometimes occur after LEP. To reduce complications, minimally invasive operative methods with cannulated screw fixation through sinus tarsi approach (STS) were developed. The aim of this multicenter propensity-matched study was to compare the clinical and radiographic outcomes of LEP to those of STS for calcaneal fracture and to evaluate the incidence of postoperative complications including surgical site infection (SSI). METHODS: We extracted 271 patients with calcaneal fracture undergoing surgery between January 2014 and March 2019 from our multicenter TRON database. We assessed the American Orthopedic Foot and Ankle Society (AOFAS) score at the final follow-up as the clinical outcome. We obtained the Bohler and Preis angles as radiographic parameters and also recorded the complications. We divided the subjects into two groups: LEP group and STS group. To adjust for baseline differences between the groups, a propensity score matching algorithm was used in a 1:1 ratio. RESULTS: After matching, there were 32 fractures in each group. There was no significant difference between the LEP versus STS group in AOFAS score at final follow-up (90 vs 90 points, p = 0.98) and in the Bohler and Pries angles (19.2 vs. 18.0 degrees, p = 0.74 and 16.0 vs. 17.5 degrees, p = 0.47). The rate of SSI in the LEP group was higher than that in the STS group (21.9% vs. 0.0%, p = 0.01). CONCLUSION: For calcaneal fracture, STS provides similar fixation effectiveness and functional outcomes as LEP while reducing the likelihood of infection.

The Association of Bohler's Angle With Postoperative Pain and Gender for Displaced Intra-Articular Calcaneal Fracture, Multicenter Retrospective Study-TRON Study.

A relationship between Böhler angle (BA) before or after surgery and clinical outcomes remains unclear. This retrospective multicenter cohort study aimed to compare pain and functional outcomes between a group in which the reduction angle was preserved and a group in which the reduction angle was lost during follow-up, and to clarify the risk factors leading to loss of last follow-up BA. From 2014 to 2018, 271 cases of calcaneal fractures were surgically treated at ten facilities. We divided patients into Group L (lost reduction of fracture) and Group P (preserved reduction of fracture). We matched subjects between the 2 groups according to age, sex and BA before surgery and compared American Orthopedic Foot and Ankle Society (AOFAS) score between the groups. We investigated the correlation between the amount of BA loss and postoperative pain. The factors leading to loss of last follow-up BA were examined by logistic regression analysis. Ultimately, 112 patients were eligible. After matching, each group included 38 patients. There was no difference between the 2 groups in total AOFAS score. However, the pain component of AOFAS score at 6 months and 12 months were worse in group L than in group P (p = .011, p = .031, respectively). We also showed a weak correlation between the amount of BA loss and postoperative pain. Logistic regression analysis revealed that female and BA before surgery independently predicted loss of reduction (odds ratios: 4.66, 95% CI: 1.15-18.9 and odds ratios: 0.90, 95% CI: 0.82-0.99, respectively). We clarified that reduction and preservation of BA within its normal range should lead to decrease postoperative pain. Female and lower pre-BA were risk factors leading to loss of reduction of BA in operative treatment of calcaneal fractures.

Impact of synbiotics treatment on bacteremia induced during neoadjuvant chemotherapy for esophageal cancer: A randomised controlled trial.

BACKGROUND & AIMS: To elucidate the impact of synbiotics on bacterial translocation and subsequent bacteremia during neoadjuvant chemotherapy for esophageal cancer. METHODS: Patients requiring neoadjuvant chemotherapy for esophageal cancer were randomized to receive synbiotics (synbiotics group) or no synbiotics (control group) during chemotherapy. Blood and fecal samples were taken before and after every chemotherapy cycle, and 1 day before surgery. Mesenteric lymph nodes (MLNs) were harvested at laparotomy (MLN-1) and after resection of the tumor (MLN-2). Bacteria in each sample were detected. Fecal microbiota and organic acid concentrations were also determined. The primary endpoint was the detection of bacteria in the blood samples, as well as the incidence of side effects during chemotherapy. The secondary endpoint was the detection rate of bacteria in the MLN samples collected during surgery. RESULTS: The study recruited a total of 42 patients (22 in the control group, 20 in the synbiotics group). Bacteria were detected in 16 of 101 blood samples in the control group, whereas those were detected only 2 of 100 blood samples in the synbiotics group (p < 0.001) during neoadjuvant chemotherapy. Additionally, bacteria were detected in 12 of 34 MLN samples in the control group, whereas no bacteria were detected in 38 MLN samples in the synbiotics group (p < 0.001). Suppression of bacterial translocation was at least partly associated with an increased fecal acetic acid concentration as well as a lowered fecal pH by synbiotics. The incidence rate of grade 3 gastrointestinal toxicity during chemotherapy was lower in the synbiotics group compared to the control group (8/22 vs. 1/20, p = 0.022). CONCLUSIONS: Neoadjuvant chemotherapy for esophageal cancer may induce bacterial translocation and subsequent bacteremia, which can be prevented by synbiotics administration. TRIAL REGISTRATION: The University Hospital Medical Information Network (http://www.umin.ac.jp; registration number ID 000007651).

An incarcerated internal hernia of the sigmoid colon through a defect in the broad ligament: A case report.

INTRODUCTION AND IMPORTANCE: Hernias through a defect of the broad ligament are rare, accounting for only 1.6-5% of internal herniations [1]. This report describes a rare case of sigmoid colon obstruction due to hernia through a defect of the broad ligament, which was diagnosed before surgery. CASE PRESENTATION: A 78-year-old multiparous woman presented with lower abdominal pain and nausea. Contrast-enhanced multi-detector CT (MDCT) demonstrated a dilated sigmoid colon and edematous mesentery of the sigmoid colon in the left Douglas' fossa, the uterus was compressed dorsally to the right and the left ovary was compressed ventrally. We diagnosed an internal broad ligament defect hernia with incarceration of the sigmoid colon, and performed emergency laparotomy. The necrotic sigmoid colon was resected and anastomosis was performed by the double stapling technique. The postoperative course was uneventful. CLINICAL DISCUSSION: We consider the treatment of hernia of sigmoid colon through a broad ligament defect. CONCLUSION: We recognize that there is a possibility that, in addition to the small intestine, proximally located organs may be incarcerated. In the case of the colon, we should choose the treatment method carefully according to whether or not the colon is expected to be necrotic.

Ruptured mesenteric desmoid-type fibromatosis without emergency surgery: A rare case report.

INTRODUCTION: Desmoid-type fibromatosis (DF) is a rare tumor that develops in the limbs, abdominal wall, and abdominal cavity. It is accounting for less than 3% of soft-tissue sarcomas and less than 0.03% of all neoplasms. PRESENTATION OF CASE: A 57-year-old man was diagnosed as acute peritonitis due to intra-abdominal tumor rupture. Since his systematic symptoms were relatively stable, gastrointestinal perforation was ruled out, the differential diagnosis of the tumor itself was difficult, and it was unclear resectable by emergency surgery, we started conservative treatment. After examinations, ileocolectomy was performed. Histopathological examination revealed spindle cells with collagenous fiber hyperplasia and immunohistochemical staining for ß-catenin was positive, so we made a diagnosis of mesenteric desmoid-type fibromatosis (MDF). DISCUSSION: The mechanism of DF development is suggested to be associated with hereditary diseases, mechanical stimuli, and a history of exposure to radiation appear to be involved as pathogenetic factors in sporadic development. Surgical resection is the first-line treatment for MDF, but the postoperative high local recurrence rate is problematic. Drug therapy and radiation therapy are selected for cases in which radical resection is not possible or for recurrent cases. However, the number of examined cases is small and sufficient evidence has not been accumulated for most treatment strategies, it is expected that the optimal treatment at the time of recurrence will be further verified by the accumulation of MDF. CONCLUSION: There are few reports of peritonitis caused by MDF rupture; emergency surgery can be avoided.

Ab initio molecular simulations for proposing novel peptide inhibitors blocking the ligand-binding pocket of urokinase receptor.

Recent biochemical experiments have revealed that a variety of proteases play important roles in cancer invasion and metastasis. Among these proteases, urokinase-type plasminogen activator (uPA) is particularly important, since its specific binding to the receptor (uPAR) existing on the surface of a cancer cell is considered to be a trigger for cancer invasion. It is thus expected that the blocking of the binding can inhibit cancer invasion in the cancer patients and improve their prognosis dramatically. To develop a potent inhibitor for the binding, many types of peptides of amino acids were produced and their effect on the cancer invasion was investigated in the previous biochemical experiments. On the other hand, our previous ab initio molecular simulations have clarified that some amino acid residues of uPA play important roles in the specific binding between uPA and uPAR. In the present study, we propose some peptides composed of these important residues and investigate the specific interactions and the binding affinity between uPAR and the peptides at an electronic level, using ab initio molecular simulations. Base on the results simulated, we elucidate which peptide can bind more strongly to uPAR and propose a novel potent peptide which can inhibit the binding between uPAR and uPA efficiently.

Early features in neuroimaging of two siblings with molybdenum cofactor deficiency.

We report the features of neuroimaging within 24 hours after birth in 2 siblings with molybdenum cofactor deficiency. The first sibling was delivered by emergency cesarean section because of fetal distress and showed pedaling and crawling seizures soon after birth. Brain ultrasound revealed subcortical multicystic lesions in the frontal white matter, and brain MRI at 4 hours after birth showed restricted diffusion in the entire cortex, except for the area adjacent to the subcortical cysts. The second sibling was delivered by elective cesarean section. Cystic lesions were seen in the frontal white matter on ultrasound, and brain MRI showed low signal intensity on T1-weighted image and high signal intensity on T2-weighted image in bifrontal white matter within 24 hours after birth, at which time the infant sucked sluggishly. Clonic spasm appeared at 29 hours after birth. The corpus callosum could not be seen clearly on ultrasound or MRI in both infants. Cortical atrophy and white matter cystic lesions spread to the entire hemisphere and resulted in severe brain atrophy within ~1 month in both infants. Subcortical multicystic lesions on ultrasound and a cortex with nonuniform, widespread, restricted diffusion on diffusion-weighted images are early features of neuroimaging in patients with molybdenum cofactor deficiency type A.

Small bowel metastasis of uterine cervical adenocarcinoma.

A few cases of small bowel metastasis from uterine cervical cancer have been previously reported. All reported cases were connected to squamous cell carcinoma, while none were associated with cervical adenocarcinoma. This report is of a rare case of cervical adenocarcinoma that haematogenously metastasised to the small intestine, and which caused a perforation and small bowel obstruction metachronously. An 84-year-old woman was admitted to our hospital with vaginal bleeding. She was diagnosed with FIGO stage III cervical adenocarcinoma by imaging and pathohistological examinations. Three months after receiving radiation therapy to control the bleeding, surgery was performed twice; the first operation for small bowel perforation and the second for small bowel obstruction. She was then diagnosed with haematogenous metastasis of cervical adenocarcinoma to the ileum according to the operative, histopathological and immunopathological findings.

Anal function after ligation of the intersphincteric fistula tract.

BACKGROUND: Although the ligation of the intersphincteric fistula tract is a promising anal sphincter-saving procedure for fistula-in-ano, the objective assessment of the sphincter preservation remains unknown. OBJECTIVE: The primary end point was to measure the anal function before and after this procedure. The secondary end point measured was cure of the disease. DESIGN: This study is a prospective observational study. SETTING: This study was conducted at the Department of Surgery, Kameda Medical Center, Japan, from March 2010 to August 2012. PATIENTS: Twenty patients with transsphincteric or complex fistulas were evaluated. INTERVENTIONS: All patients underwent the ligation of the intersphincteric fistula tract with a loose seton for anal fistulas. MAIN OUTCOME MEASURES: Anal manometric study was performed before and 3 months after the procedure. Fecal incontinence was evaluated by using the fecal incontinence severity index. Failure was defined as nonhealing of the surgical wound or fistula. RESULTS: The median operation time was 42 minutes. No intraoperative complications were documented. The median follow-up duration was 18 (3-32) months. No patients reported any incontinence postoperatively. The median score of the fecal incontinence severity index before and 3 months after the procedure was 0. The median maximum resting pressure measured before and after operation were 125 (71-175) cm H2O and 133 (95-169) cm H2O. The median maximum squeeze pressure measured before and after operation were 390 (170-815) cm H2O and 432 (200-902) cm H2O. There were no significant postoperative changes in either the resting pressure or the squeeze pressure. Primary healing was observed in 19 (95%) patients, and the median healing time was 7 weeks; 1 wound remained incompletely healed. LIMITATIONS: Short-term follow-up may not justify the use of the term definitive cure. CONCLUSION: The ligation of the intersphincteric fistula tract with a loose seton showed no postoperative deterioration on anal sphincter function with favorable healing rates.

A randomized, prospective, double-blind, placebo-controlled trial of the effect of diltiazem gel on pain after hemorrhoidectomy.

BACKGROUND: Spasm of the internal anal sphincter is considered to be one of the causes of pain in anal diseases. We have evaluated the effects of topical diltiazem on postoperative pain after hemorrhoidectomy. METHODS: Sixty-two patients were randomly assigned to receive a 2 % diltiazem gel (n = 32) or a placebo gel (n = 30) after hemorrhoidectomy. Patients applied the gel to the anal region three times per day for 14 days. Pain both in the resting state and on defecation ranged from 0 to 10 on a numerical rating scale, and the number of prescribed loxoprofen tablets (Loxonin) were recorded and confirmed daily by telephone. Any morbidity during the follow-up period was recorded. RESULTS: Both pain scores during defecation and the number of analgesic tablets consumed tended to be lower in the diltiazem group, although they did not reach statistical significance (P = 0.09, P = 0.12, respectively). Total number of complications was significantly higher in the diltiazem group, but each incidence of complications, including itching sensation, headache, and dizziness was not statistically different. CONCLUSIONS: Perianal application of 2 % diltiazem gel after hemorrhoidectomy has the potential to reduce postoperative pain during defecation.

Exome sequencing identifies a novel INPPL1 mutation in opsismodysplasia.

Opsismodysplasia is an autosomal recessive skeletal disorder characterized by facial dysmorphism, micromelia, platyspondyly and retarded bone maturation. Recently, mutations in the gene encoding inositol polyphosphate phosphatase-like 1 (INPPL1) are found in several families with opsismodysplasia by a homozygosity mapping, followed by whole genome sequencing. We performed an exome sequencing in two unrelated Japanese families with opsismodysplasia and identified a novel INPPL1 mutation, c.1960_1962delGAG, in one family. The mutation is predicted to result in an in-frame deletion (p.E654del) within the central catalytic 5-phosphate domain. Our results further support that INPPL1 is the disease gene for opsismodysplasia and that opsismodysplasia has genetic heterogeneity.