Young Patient with X-linked Agammaglobulinemia Presents with Advanced Gastric Cancer and Extensive Atrophic Gastritis.

X-linked agammaglobulinemia (XLA) is associated with an increased risk of gastrointestinal cancers including gastric cancer (GC). We herein report the case of a 30-year-old male patient with XLA who developed GC and extensive atrophic gastritis. He tested positive in the urea breath test, thus indicating the presence of Helicobacter pylori. Distal gastrectomy and chemotherapy were performed without any complications; however, the died two years after this diagnosis. Immunoglobulin deficiency makes these patients susceptible to progressive atrophic gastritis and the associated risk of GC. Therefore, patients with XLA are advised to undergo an evaluation for Helicobacter pylori infection as well as monitoring for GC.

Long-term course of untreated asymptomatic esophageal eosinophilia and minimally symptomatic eosinophilic esophagitis.

Background and study aims The long-term course of untreated asymptomatic esophageal eosinophilia (aEE) and minimally symptomatic eosinophilic esophagitis (mEoE) are not well understood. This study aimed to clarify this course. Patients and methods A total of 36 patients with EE who were endoscopically followed up for more than 5 years, and who underwent more than one endoscopy evaluation after the first diagnosis, were investigated. These patients were divided into two groups according to the presence or absence of the continuous treatment: no treatment group (NT group, n=22) and proton pump inhibitor/potassium competitive acid blocker group (Tx group, n=14). Symptoms and endoscopic and histological findings were retrospectively reviewed according to endoscopic phenotypes. Endoscopic assessment was performed using the EoE endoscopic reference score (EREFS). Results The median follow-up period was 84.5 months in the Tx group and 92 months in the NT group. During the follow-up period, about half of the patients in the Tx-diffuse group persisted EREFS >3, while the remaining half had EREFS ≤2. The total EREFS in the NT-diffuse group remained almost unchanged (median: 2-4) without apparent exacerbation. In contrast, EREFS in the NT-localized group exhibited an unchanged or gradually decreasing trend, with statistical significance from the first diagnosis to 72 to 83 months after. Conclusions Untreated aEE and mEoE are not likely to worsen even without treatment at least for a median follow-up of 7 years. Instead, the localized type may spontaneously improve, implying a different pathogenesis in the presence of the diffuse type. Further studies should clarify the long-term prognosis.

An algorithm for the pre-operative differentiation of benign ovarian tumours based on magnetic resonance imaging interpretation in a regional core hospital: A retrospective study.

Objective: For selecting minimally invasive surgery (i.e. laparoscopic ovarian cystectomy) for treating ovarian tumours (OTs) in premenopausal patients, the pre-operative differentiation of benign ovarian tumours (Be-OTs) based on magnetic resonance imaging (MRI) interpretation is important. This paper describes the authors' 8-year experience of approximately 1000 OT cases, and provides information about a diagnostic algorithm to help other hospitals. Study design: The medical records of 901 patients aged < 50 years with OTs from 1 January 2015-31 March 31 2023 were reviewed. First, the accuracy of pre-operative differentiation between Be-OTs and borderline/malignant ovarian tumours (Bo/Ma-OTs) was compared in each type of OT. Second, to identify the factors influencing differentiation between Be-OTs and Bo/Ma-OTs in 164 serous/mucinous ovarian tumours (SM-OTs), a multi-variate logistic regression analysis was performed to assess the effect of 13 factors, including MRI findings, OT size and tumour markers. Results: In the comparison of diagnostic accuracy of pre-operative MRI for each OT type, accuracy was found to be notably high for ovarian endometrial cyst (OEC) (n = 409), ovarian mature cystic teratoma (OMCT) (n = 308), ovarian endometrioid adenocarcinoma (OEA) (n = 6) and ovarian clear cell adenocarcinoma (OCCA) (n = 14). On the other hand, discrepancies between MRI and pathological findings often occurred in SM-OTs, including ovarian serous cystadenoma (n = 86), ovarian mucinous adenocarcinoma (n = 61), ovarian serous adenocarcinoma (n = 12) and ovarian mucinous adenocarcinoma (n = 5). In the multi-variate logistic regression analysis of the latter 164 patients, in addition to MRI findings, OT size and carbohydrate antigen 125 also had an effect to some extent. The combination of MRI interpretation and OT size may enhance differentiation of Be-OTs and Bo/Ma-OTs. Conclusions: Among four types of OTs (OEC, OMCT, OEA and OCCA), MRI interpretation was able to differentiate between Be-OTs and Bo/Ma-OTs almost perfectly. Additionally, to mitigate the difficulty in differentiating SM-OTs, OT size may be useful in combination with MRI findings, although further accumulation and analysis of OT cases is needed.

Obvious advantage of vaginal natural orifice transluminal endoscopic surgery hysterectomy against total laparoscopic hysterectomy in small uterus patients and the future prospects at a regional core institution: A retrospective study.

Objective: Owing to the combination of benefits, including permanent visual guidance and no abdominal skin incision, vaginal natural orifice transluminal endoscopic surgery hysterectomy (vNOTES-H) is currently widely used. However, the introduction of vNOTES-H has been delayed in many Japanese regional core hospitals because of its specific device and skill requirements. Therefore, the characteristics and advantages should be explained for the widespread use of this technique. Study design: We reviewed the medical records of 17 patients with vNOTES-H and 94 patients with total laparoscopic hysterectomy (TLH) from January 1, 2015 to December 31, 2022. In this analysis, to compare the results of vNOTES-H to TLH, we excluded certain patients with a relatively heavy uterus (>255 g) and the presence of abdominal adhesions. In this report, first, the characteristics of the vNOTES-H procedures using a transvaginal access platform are explained by referring to one representative patient. Second, the patient characteristics of the vNOTES-H and TLH groups, including operation time and blood loss amount, were compared. Then, to detect the influence of vNOTES-H on the difficulty of operation among all 111 patients, we performed a multivariate logistic regression analysis to assess the influence of each of 9 factors, including "vNOTES-H", "Advanced age", "High BMI", "3 parity", "Gynaecological operation history", "Adenomyoma", "Large leiomyoma", "Heavy uterus" and "Large uterus", on the two indexes, including "Short time operation" and "Massive blood loss". Results: In the simple comparison between the groups with vNOTES-H and TLH, the operation time in the former group was significantly shorter than in the latter group, although other factors did not show significant differences, including blood loss amount. Moreover, in the multivariate logistic regression analysis of all 111 patients, the "vNOTES-H" factor showed a significantly high possibility of "short time operation", although no factor, including "vNOTES-H", showed a significant influence on "massive blood loss". Conclusions: vNOTES-H showed advantages in terms of operation time without increasing blood loss for patients with a relatively small uterus. However, to expand the selection for vNOTES-H, we should accumulate further patients and perform more analyses.

Desmoid Tumor Mimicking Port Site Metastasis after Laparoscopic Surgery for Endometrial Cancer.

Desmoid tumors are rare; however, they sometimes form in the abdominal wall after surgery or trauma. We report a case of desmoid tumors in the abdominal wall mimicking port-site metastasis after laparoscopic surgery for endometrial cancer. A 53-year-old woman with familial adenomatous polyposis presented to our hospital with vaginal bleeding and was diagnosed with endometrial cancer. We performed a total laparoscopic hysterectomy and began observation. Two years after surgery, follow-up computed tomography revealed three nodules with a size of approximately 15 mm in the abdominal wall at the trocar sites. Tumorectomy was performed because endometrial cancer recurrence was suspected, but desmoid fibromatosis was finally diagnosed. This is the first report of desmoid tumors at the trocar site after laparoscopic surgery for uterine endometrial cancer. Gynecologists should be aware of this disease because differentiating it from metastatic recurrence is challenging.

Preventive effect of metronidazole vaginal tablets on vaginal bacteria-related postoperative complications with total laparoscopic hysterectomy.

BACKGROUND: The use of total laparoscopic hysterectomy is increasing. However, as with conventional abdominal hysterectomy, vaginal bacteria-related postoperative complications need to be managed in total laparoscopic hysterectomy. Therefore, we started to combine metronidazole vaginal tablets with intravenous administration of cephem antibiotics immediately before starting surgery to reduce complications. To evaluate the effect of this combination, and to determine the risk factors for these complications, we retrospectively collected medical records from our hospital and performed a multivariate analysis. METHODS: We reviewed the medical records of 520 patients who underwent total laparoscopic hysterectomy from 1 January 2015 to 31 December 2021. Among these cases, we identified 16 cases as having vaginal bacteria-related postoperative complications, defined as needing more than one additional day for treatment of postoperative complications, namely postoperative infection (10 cases) and vaginal dehiscence (6 cases). First, we evaluate the effect of metronidazole vaginal tablets by dividing the patients into two groups according to whether metronidazole vaginal tablets were used, and comparing the vaginal bacteria-related postoperative complication rates and other indices. Second, we performed a multivariate logistic regression analysis to assess the influence of each of 17 representative factors, including patient characteristics and symptoms, uterus and leiomyoma sizes, concomitant procedures such as laparoscopic cystectomy and pelvic lymphadenectomy, and others. RESULTS: In the multivariate analysis of the 520 cases, we confirmed that the use of metronidazole vaginal tablets could reduce the vaginal bacteria-related postoperative complications rate by more than half (odds ratio, 0.36). In addition to metronidazole vaginal tablets use, concomitant laparoscopic cystectomy and blood transfusion were associated with significant increases in the vaginal bacteria-related postoperative complication rate. CONCLUSIONS: The effect of the addition of metronidazole vaginal tablets to pre- and postsurgical treatment on the reduction of vaginal bacteria-related postoperative complications was confirmed. This easy, safe, and low-cost method may improve the management of total laparoscopic hysterectomy.

Unilateral uterine artery embolization and Bakri tamponade balloon insertion in the treatment of acute puerperal uterine inversion: a case report.

BACKGROUND: Acute puerperal uterine inversion is rare but may cause massive postpartum blood loss due to uterine atony. Therefore, these patients must be diagnosed, and uterine replacement must be performed as soon as possible. However, in some cases, active bleeding due to uterine atony becomes uncontrollable, even though the uterine inversion itself is treated. In these cases, additional treatments, including surgical procedures, are needed. CASE PRESENTATION: A 41-year-old Japanese woman, gravida 1, para 0, was hospitalized for labor induction at 40 weeks and 3 days of gestational age. She had a vacuum-assisted delivery after 3 days of oxytocin administration, but acute uterine inversion occurred. Although replacement of the inverted uterus was successful by manual repositioning and Bakri balloon tamponade insertion, massive postpartum hemorrhage caused by uterine atony became uncontrollable. In this situation, since disseminated intravascular coagulation had developed, we used uterine artery embolization to stop the bleeding. After detecting the pseudo-aneurysmal sac and tortuous vessels of the right uterine artery, transcatheter right-sided uterine artery embolization was performed. Thirteen days after uterine artery embolization, she was discharged with no complications. CONCLUSIONS: In cases of disseminated intravascular coagulation caused by massive postpartum bleeding, uterine artery embolization may often be selected. In our case, since we performed angiography to detect the main bleeding site, the hemorrhage could be stopped with unilateral uterine artery embolization alone, without hysterectomy.

Successful Management of a Noncommunicating Rudimentary Uterine Horn Pregnancy by Laparoscopic Surgery: A Case Report and Literature Review.

Pregnancy in a noncommunicating rudimentary horn is extremely rare but can cause serious clinical complications, such as uterine rupture. The standard treatment is excision of the rudimentary horn, and recently, in some cases, laparoscopic resection has been performed in the first trimester of gestation. Herein, we present a case of noncommunicating rudimentary horn pregnancy (NCRHP), which was diagnosed by magnetic resonance imaging at 6 weeks of gestation and treated by laparoscopic surgery. However, we have also found some rare cases in which patients could obtain live newborn babies. Since management is affected by the different levels of obstetric medical care and diagnostic tools, we also performed a review and analysis of NCRHP. A PubMed search yielded 103 cases reported in the English literature. Correct diagnosis and laparoscopic treatment were achieved more frequently in developed countries, especially in the first trimester of gestation. On the other hand, symptoms, including abdominal pain and hypovolemic shock, tended to occur in the second trimester of gestation. This period was also found to be a risk factor for uterine rupture. Among 18 patients at the third trimester of gestation, 13 obtained live neonatal infants. Therefore, detailed information about this disease is crucial for proper treatments.

Predictive factors of spontaneously regressed uterine endometrial polyps during the waiting period before hysteroscopic polypectomy.

BACKGROUND: The presence of uterine endometrial polyps is associated with not only abnormal uterine bleeding but also infertility, so the use of hysteroscopic polypectomy has been increasing. This operation is considered to increase cost-effectiveness when performed prior to infertility treatments. However, there are typical problems to consider, including the possibility of spontaneous regression of the polyp and the duration of complete endometrial wound healing after surgery. Meaningless interventions must be avoided, when possible. Therefore, data acquisition and analysis of various findings obtained from surgery have become important for improving treatment procedures and patient selection. To estimate the spontaneous regression rates and contributions of multiple factors to uterine endometrial polyps during the waiting period (approximately 2-3 months) before hysteroscopic polypectomy, we performed a multivariate analysis of data from the records in our hospital. METHODS: The medical records of 450 cases from September 2014 to April 2021 in our hospital were retrospectively reviewed under the approval of our Institutional Review Board. We included all cases of hysteroscopic polypectomy with postoperative pathological diagnosis. We defined cases as having a "spontaneously regressed polyp" when the target polyp was not detected by postoperative pathological examination. We extracted data on the following ten factors: "Advanced age" (≥ 42 years), "Small polyp" (< 10 mm), "High body mass index" (≥ 25 kg/m2), "Nulliparity," "Single polyp," "Infertility," "Hypermenorrhea," "Abnormal bleeding," "No symptom," and "Hormonal drug use." We also classified cases into five groups according to the size of the polyp (≤ 4.9 mm, 5.0-9.9 mm, 10.0-14.9 mm, 15.0-19.9 mm, and ≥ 20.0 mm) and determined the frequency of spontaneously regressed polyp in each group. RESULTS: After exclusion of cases with insufficient data or other diseases, such as submucosal leiomyoma, 424 cases were analyzed. Among them, 28 spontaneously regressed polyps were identified, and the highest frequency of spontaneously regressed polyp was detected among the cases with polyps measuring 5.0-9.9 mm (16.4%). On multivariate analysis of the ten factors, "Small polyp" and "Hormonal drug use" were found to significantly impact the frequency of spontaneously regressed polyp. CONCLUSIONS: On the basis of the factors identified in this analysis, the indications for observation or medical therapy adapted to small polyps might be expanded.

A retrospective study of 323 total laparoscopic hysterectomy cases for various indications and a case report treating caesarean scar pregnancy.

BACKGROUND: The application of laparoscopic surgeries has been increasing, and various uterine diseases in addition to leiomyoma/adenomyoma have become indications for total laparoscopic hysterectomy (TLH). Therefore, data acquisition and analysis of TLH procedures, including TLH for rare uterine diseases, have become important for improving surgical procedures and patient selection. To determine the prevalence of and risk factors for the occurrence of intraoperative and postoperative complications of TLH, we performed a multivariate analysis of the records in our hospital. METHODS: We retrospectively reviewed the medical records of 323 patients who underwent TLH for the treatment of leiomyoma/adenomyoma (278 cases), low-grade (pre)malignant uterine tumours (40 cases) and other rare uterine diseases (5 cases) from January 1, 2015, to December 31, 2019. Of the rare uterine diseases, one case of caesarean scar pregnancy for which TLH was performed is introduced as a case report. To assess the effects of 11 representative factors, including patient characteristics, uterus and leiomyoma sizes, indications for TLH and others, we performed a multivariate logistic regression analysis. RESULTS: Among the 323 cases, 20 intraoperative complications and 15 postoperative complications were reported. In the multivariate analysis, "ovarian tumour" and "heavy uterus" were positively associated and "nulliparity" was negatively associated with intraoperative complications. There were no significant risk factors for postoperative complications. The only risk factor for operative complications directly related to the resected uterus was "heavy uterus". Therefore, we could perform TLH relatively safely for patients with other indications besides leiomyoma/adenomyoma. CONCLUSIONS: Considering the factors detected in this analysis, the indications for TLH may be expanded. Owing to the increase in TLH for indications other than leiomyoma/adenomyoma, a more accurate determination of the treatment approach can be achieved.

Formation of Calcified Nodule as a Cause of Early In-Stent Restenosis in Patients Undergoing Dialysis.

Background Dialysis is an independent risk factor for in-stent restenosis (ISR) after stent implantation in coronary arteries. However, the characteristics of ISR in patients undergoing dialysis remain unclear, as there are no histological studies evaluating the causes of this condition. The aim of the present study was to investigate the causes of ISR between patients who are undergoing dialysis and those who are not by evaluating tissues obtained from ISR lesions using directional coronary atherectomy. Methods and Results A total of 29 ISR lesions from 29 patients included in a multicenter directional coronary atherectomy registry of 128 patients were selected for analysis and divided into a dialysis group (n=8) and a nondialysis group (n=21). Histopathological evaluation demonstrated that an in-stent calcified nodule was a major histological characteristic of ISR lesions in the dialysis group and the prevalence of an in-stent calcified nodule was significantly higher in the dialysis group compared with the nondialysis group (75% versus 5%, respectively; P<0.01). On the other hand, the prevalence of an in-stent lipid-rich plaque was significantly lower in the dialysis group compared with the nondialysis group (0% versus 43%, respectively; P=0.03). In all cases with an in-stent calcified nodule, the underlying calcification before stent implantation was moderate to severe. When tissue characteristics were stratified according to duration post-stent implantation, an in-stent calcified nodule in the dialysis group was mainly observed within 1 year after stent implantation. Conclusions In-stent calcified nodules are a common cause of ISR in patients undergoing dialysis and are observed within 1 year after stent implantation, suggesting different causes of ISR between patients undergoing dialysis and those who are not.

[A case of fibrinogen storage disease with primary biliary cholangitis].

A 66-year-old woman was diagnosed as primary biliary cholangitis (PBC) and was previously hospitalized for ascites and jaundice. She came to our hospital for further examination of the liver by needle biopsy, which showed interface hepatitis that mainly comprised lymphocytes and inflammatory infiltrates in the bile duct in the portal area. On the other hand, numerous intracytoplasmic inclusions that were positive for fibrinogen immunostaining were seen in the lobular area. Finally, we histologically diagnosed as PBC with fibrinogen storage disease (FSD). FSD is rare disease that leads to liver damage caused by abnormal fibrinogen storage in the endoplasmic reticulum of hepatocytes, with only four cases reported in Japan until now.

Significant risk factors for malignant transformation of ovarian endometrioma during dienogest treatment: a case report and retrospective study.

BACKGROUND: To determine the prevalence of and risk factors for malignant transformation of ovarian endometrioma during dienogest therapy, which is very rare, we examined multiple cases of malignant transformation of ovarian endometrioma during dienogest therapy and performed a multivariate analysis of the records in our hospital. METHODS: The medical records of 174 patients who underwent DNGT for the treatment of OMA from June 1, 2011, to May 31, 2018, were reviewed retrospectively with the approval of the Human Ethical Committee of the University of Teikyo Hospital. And we provided one representative case of MT with obtaining written informed consent. To assess the effects of six representative factors, including advanced age, parity, surgical history, and endometrial cyst characteristics (including 3 factors), on the possibility of malignant transformation, we performed a multivariate logistic regression analysis. RESULTS: Of the 174 cases, 4 were diagnosed with malignant transformation, and these cases are reported. In the multivariate analysis, advanced age (P = 0.0064), nullipara (P = 0.0322), and enlargement (P = 0.0079) showed significant differences for malignant transformation occurrence. All 4 malignant transformation cases were among the 19 patients who had all of these 3 factors. CONCLUSIONS: For a more accurate determination of the treatment approach, a larger sample size will be needed to determine the risk factors for malignant transformation during dienogest therapy.

Intraoperative and Postoperative Clinical Evaluation of the Hysteroscopic Morcellator System for Endometrial Polypectomy: A Prospective, Randomized, Single-blind, Parallel Group Comparison Study.

OBJECTIVE: To evaluate the TRUCLEAR™ system (Smith and Nephew Inc., London, UK), a hysteroscopic system that morcellates and aspirates masses, in terms of the operating time, surgeon's convenience, and effect on patients compared with conventional electrosurgical resection. METHODS: Patients undergoing hysteroscopic resection of endometrial polyps were randomly allocated to undergo hysteroscopic morcellation or electrosurgical resection (UMIN-CTR identifier: UMIN000019649). The primary outcome was the operating time. Secondary outcomes were the removal success, fluid deficit, convenience with the technique, insertion time, number of insertions during the operation, visibility of the operative field, recurrence of the patient's chief complaint, and adverse events. RESULTS: Sixty-seven women were randomly allocated to the morcellation arm (n = 34) or electrosurgical resection arm (n = 33) from November 2015 to November 2016. The polyps were completely removed, and no adverse events were observed in all 67 patients. The average operating time (8.3 min vs. 12.0 min, P = 0.014), insertion time (5.0 min vs. 9.0 min, P < 0.001), and number of insertions (1.0 vs. 8.2, P < 0.001) were significantly lower in the morcellation arm than in the electrosurgical resection arm. Surgeons' subjective evaluation measured on a 10-cm visual analog scale was higher in the morcellation arm than in the electrosurgical resection arm in terms of easiness of removal (8.4 vs. 6.5, P < 0.001) and visibility of the operative field (7.8 vs. 6.4, P < 0.001). CONCLUSION: Surgeons gave the hysteroscopic morcellator system a better evaluation compared than electrosurgical resection, and the system shortened the operating time.

[Anesthetic Management under Spinal Anesthesia in a Patient with Erythropoietic Protoporphyria].

Erythropoietic protoporphyria (EPP) is a hereditary disease resulting from a deficiency in ferrochelatase required for haem synthesis system. We describe the anesthetic management of a 51-year-old man with EPP undergoing open reduction and internal fixation of patella fracture. Spinal anesthesia was induced with bupivacaine. The surgery was performed without any complications. No skin symptom was observed periop- eratively. Spinal anesthesia with bupivacaine can be safe for an EPP patient.

Pulmonary hypertension with extensive calcification in small pulmonary vessels and alveolar capillary wall in a chronic hemodialysis patient.

We report a case of a 41-year-old male with end-stage renal disease receiving chronic hemodialysis who was referred to this hospital because of dyspnea. He had been on a regular dialysis for 20 years due to chronic glomerulonephritis. His transthoracic echocardiography revealed severe pulmonary hypertension (PH), and cardiac catheterization confirmed this diagnosis. From clinical examination and review of the chest X-ray and computed tomography images, we thought PH was due to multifactorial mechanisms typical of hemodialysis patients. However, microscopic examination of lung tissue from autopsy specimen revealed extensive calcium deposits not only in alveolar septal wall but also in alveolar capillaries and small vessels, which had diffuse intimal thickening causing the narrowing of the lumens. These pathological findings suggest that pulmonary vascular calcification contributed to the PH in this patient. .

Myomectomy reduces endometrial T2 relaxation times.

Magnetic resonance imaging was used to measure the endometrial T2 relaxation times of patients with infertility with fibroma. Although the location of fibromas did not influence the T2 relaxation times, we did observe a significant decrease in endometrial T2 relaxation times after myomectomy.