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Ultrasonography is an essential part of the diagnostic process of biliary atresia (BA). The characteristic findings of BA include a hilar hyperechoic zone, the triangular cord sign (TCS), an absence of gallbladder contraction after feeding, and gallbladder atrophy. However, approximately 10% of patients with BA have a normal gallbladder. We herein present two cases of BA with normal morphology of the gallbladder as shown by ultrasonography. In the first case, the patient was positive for the TCS, negative for gallbladder atrophy, and positive for contraction of the gallbladder after feeding; the final diagnosis was hilar obstructive BA complicated by pancreaticobiliary maljunction. In the second case, the patient was positive for the TCS, negative for gallbladder atrophy, and negative for contraction of the gallbladder after feeding; the patient also had common bile duct obstruction and stenosis of the hepatic duct in the hilar region. Based on these two cases, we conclude that gallbladder findings are not diagnostic for BA because in some types, the gallbladder may be normal in morphology and even have the capacity for contraction after feeding.

Background/purpose: As the occurrence of second primary cancers (SPCs) is strongly related to the survival rate of patients with oral and pharyngeal cancers, early detection and treatment are important. Therefore, this study aimed to clarify the incidence of SPCs and their risk factors in patients with oral and pharyngeal cancer. Materials and methods: This observational study was conducted using data from the administrative claims database of 21,736 participants with oral and pharyngeal cancer from January 2005 to December 2020. We evaluated the cumulative incidence of SPCs among patients with oral and pharyngeal cancers using the Kaplan–Meier method. The Cox proportional-hazard model was used for multivariate analysis. Results: Of the 1633 patients with oral and pharyngeal cancer who qualified for analysis, 388 developed SPCs (incidence rate, 7.994/1000 person-months). The multivariate analysis showed that the risk of developing SPCs was affected by age at diagnosis of oral and pharyngeal cancer, cancer treatment, and anatomical site of the primary cancer. Conclusion: Patients with oral and pharyngeal cancers are at a high risk of developing SPCs. The data from this study may be useful in providing accurate information to patients with oral and oropharyngeal cancer.

Background: Congenital duodenal obstruction occur with other anomalies in 50% of cases. Although predictable, its association with biliary/pancreatic anomalies is rarely reported. All cases of duodenal atresia (DA) and stenosis (DS) associated with biliary and/or pancreatic abnormalities were analyzed. Methods: A systematic review searched all studies reporting association between DA/DS and biliary/pancreatic abnormalities. The study was performed according to PRISMA guidelines. Results: Of 522 studies reviewed, 285 were analyzed after removing the duplicates, and 104 (249 patients) meeting the inclusion criteria, were included. Anomalous bifurcated bile duct was described in 24 cases (20 DA, 4 DS): 6 at necropsy, and 20 at perinatal surgery. Choledochal cyst was found in 7 patients (5 DA, 2 DS): 1 perinatally, 2 in the first year, 4 at median age of 6.5 years (range 4–27 years). Biliary atresia was diagnosed perinatally in 13 patients with DA and in 1 at 9 months. Annular pancreas was discovered in 146 patients (15 DA, 113 DS, 18 not specified): 113 perinatally, 4 children, and 28 adults (median age 33 years, range 4–72 years). In the remaining 58 cases, a combination of biliary and pancreatic abnormalities was diagnosed in 41 DA, 16 DS, 1 not specified duodenal obstruction, in 35 patients perinatally, in 4 infants, and in 19 at median age of 3 years (range 1–40 years). Conclusions: Presence of DA/DS should rise the alert for a possible association with biliary/pancreatic abnormalities. Therefore, a careful intraoperative approach should be adopted when repairing DA/DS to avoid damaging biliary and pancreatic ducts. In addition, appropriate imaging looking for biliary/pancreatic anomalies should be considered in symptomatic patients.

Abstract Background One-dose packaging is frequently used in Japan for elderly patients who are prescribed several medications. It is useful for easy administration and the prevention of misuse or missed medications. Hygroscopic medications are not suitable for one-dose packaging because moisture absorption may alter their properties. Plastic bags with desiccating agents are sometimes used to store hygroscopic medicines in one-dose packaging. However, the relationship between the quantity of desiccating agents and their safety in the storage of hygroscopic medications is poorly understood. Furthermore, older adults might accidentally consume desiccating agents used in food preservation. In this study, we developed a bag that suppresses the moisture absorption of hygroscopic medications without the use of desiccating agents. Methods The bag was manufactured using polyethylene terephthalate, polyethylene, and aluminum film on the outside, and unified with a desiccating film on the inside. Results The relative humidity (RH) in the bag was maintained at approximately 30–40% when the bag was stored at 75% RH and 35 °C. The manufactured bag’s moisture suppressing effect was better than that of plastic bags with desiccating agents when the hygroscopic medications, potassium aspartate and sodium valproate tablets, were stored at 75% RH and 35 °C for 4 weeks. Conclusions The moisture-suppression bag effectively stored and preserved hygroscopic medications and was more effective in inhibiting moisture absorption than plastic bags with desiccating agents under high temperature and humidity conditions. The moisture-suppression bags are expected to be useful for elderly patients who are prescribed several medications in one-dose packaging.

Abstract Background This study aims to evaluate the association between smoking habits and dental care utilization and cost in individuals registered with the Japan Health Insurance Association, Osaka branch. Methods We used the administrative claims database and specific medical check-up data and included 226,359 participants, who visited dental institutions, underwent dental examinations, and underwent specific medical checkups, with smoking data from April 2016 to March 2017. We calculated propensity scores with age, gender, exercise, eating habits, alcohol intake, and sleep. We also compared dental care utilization with the total cost of each procedure. Results According to propensity score matching, 62,692 participants were selected for each group. Compared to non-smokers, smokers were younger, and a higher proportion were men. Smokers tended to skip breakfast, have dinner just before bed, and drink alcohol. After adjusting for potential confounding factors with propensity score matching, the mean annual dental cost among smokers was significantly higher than non-smokers. The prevalence of pulpitis, missing teeth, and apical periodontitis were higher among smokers than non-smokers, while inlay detachment, caries, and dentine hypersensitivity were higher among non-smokers. Conclusion This study suggests that smokers have higher dental cost consisted of progressive dental caries, missing teeth, and uncontrolled acute inflammation that necessitated the use of medications. It is suggested that smokers tend to visit the dentist after their symptoms become severe.

Heterotopic pancreas (HP) is a rare entity. HP is rarely diagnosed during the neonatal period, and only a few neonatal cases of HP causing gastrointestinal obstruction have been reported. We herein describe a rare case of HP causing jejunal obstruction in the early neonatal period and discuss the association of an inflammatory process in this case. Keywords: Heterotopic pancreas, Neonate, Gastrointestinal obstruction, Jejunum

Introduction Given an increasing use of dipeptidyl peptidase-4 (DPP-4) inhibitors to treat patients with type 2 diabetes mellitus in the real-world setting, we conducted a prospective observational study (Japan-based Clinical Research Network for Diabetes Registry: J-BRAND Registry) to elucidate the safety and efficacy profile of long-term usage of alogliptin.Research design and methods We registered 5969 patients from April 2012 through September 2014, who started receiving alogliptin (group A) or other classes of oral hypoglycemic agents (OHAs; group B), and were followed for 3 years at 239 sites nationwide. Safety was the primary outcome. Symptomatic hypoglycemia, pancreatitis, skin disorders of non-extrinsic origin, severe infections, and cancer were collected as major adverse events (AEs). Efficacy assessment was the secondary outcome and included changes in hemoglobin A1c (HbA1c), fasting blood glucose, fasting insulin and urinary albumin.Results Of the registered, 5150 (group A: 3395 and group B: 1755) and 5096 (3358 and 1738) were included for safety and efficacy analysis, respectively. Group A patients mostly (>90%) continued to use alogliptin. In group B, biguanides were the primary agents, while DPP-4 inhibitors were added in up to ~36% of patients. The overall incidence of AEs was similar between the two groups (42.7% vs 42.2%). Kaplan-Meier analysis revealed the incidence of cancer was significantly higher in group A than in group B (7.4% vs 4.8%, p=0.040), while no significant incidence difference was observed in the individual cancer. Multivariate Cox regression analysis revealed that the imbalanced patient distribution (more elderly patients in group A than in group B), but not alogliptin usage per se, contributed to cancer development. The incidence of other major AE categories was with no between-group difference. Between-group difference was not detected, either, in the incidence of microvascular and macrovascular complications. HbA1c and fasting glucose decreased significantly at the 0.5-year visit and nearly plateaued thereafter in both groups.Conclusions Alogliptin as a representative of DPP-4 inhibitors was safe and durably efficacious when used alone or with other OHAs for patients with type 2 diabetes in the real world setting.

An omphalocele containing the entire liver inside the umbilical cord through a small abdominal wall defect is a rare condition. The surgical management of an omphalocele with extensive liver herniation through a small abdominal wall is extremely challenging due to not only a limited abdominal cavity but also a narrowed umbilical ring. Herein, we report a case of omphalocele with extensive liver herniation through a small abdominal wall treated successfully with a delayed external silo reduction technique, by preserving the amniotic membrane.

Abscess formation of the falciform or round ligament of the liver is rare. A 15-year-old boy was diagnosed with Hirschsprung's disease after birth and underwent surgery for transverse colostomy at the site of the left upper abdomen in the neonatal period. A radical operation for Hirschsprung's disease was performed with a widely extended incision of the colostomy at 3 months of age. When he was 14 years old, he presented with lower abdominal pain due to a patent urachus. Six months after excision of the patent urachus, he returned to our outpatient clinic, showing pain and a palpable mass at the middle upper abdomen. We report herein a case of abscess formation of the round ligament of the liver after surgical resection of a patent urachus. We also review previous literature and discuss the possible pathogenesis and mechanisms of this phenomenon.

Colonic atresia (CA) is one of the rarest causes of neonatal intestinal obstruction. The combination of jejunoileal atresia (JIA) and CA is particularly rare. To the best of our knowledge, only 21 cases of JIA associated with CA have been described in the English-language literature. We herein present a case involving a 34-week preterm female infant with bilious vomiting by the first day of life. Laparotomy revealed jejunal gap atresia and ileal gap atresia. In addition, colonic gap atresia was found at the hepatic flexure with a wide mesenteric defect. The JIA and CA appeared to have been caused by a thromboembolic event in the superior mesenteric artery. We also review the 21 previously published cases and discuss the etiologies of the association between JIA and CA.

Purpose: We compared laparoscopic redo fundoplications performed for failed laparoscopic Toupet fundoplication (LTF) and failed laparoscopic Nissen fundoplications (LNFs). Methods: Redo LTF (R-LTF; n = 4) and redo LNF (R-LNF; n = 6) performed between 2007 and 2014 were assessed retrospectively for severity of intraperitoneal adhesions on a scale of 0–3, identification/preservation of the anterior/posterior/hepatic branches of the vagus nerve (VN), complications, and outcome. Results: Redos were performed after a mean of 34 months in R-LTF and 32 months in R-LNF (P = ns) indicated for sliding hernia (n = 3; 2 with partial wrap dehiscence) and partial wrap dehiscence (n = 1) in R-LTF and sliding hernia (n = 6; 4 with partial wrap dehiscence) in R-LNF. The mean adhesion severity score was 1.5 in R-LTF and 2.5 in R-LNF (P < 0.05). The mean number of VN branches identified/preserved was 2.0 in R-LTF and 0.8 in R-LNF (P < 0.05). Mean operative times and mean blood loss were similar. Intraoperative complications were accidental local trauma (n = 1 in R-LTF and n = 3 in R-LNF, one requiring conversion to open repair) (P = ns). Gastric outlet obstruction developed in two R-LNF cases; both were managed conservatively. There have been no further recurrences to date. Conclusion: Although our series is small, adhesions were less, and identification/preservation of VN was easier during R-LTF.

Adherence to medications is an important challenge while treating chronic disease such as resistant hypertension, which is defined as uncontrolled blood pressure (BP) despite treatment with more than 3 antihypertensive drugs to achieve targets. It is possible that poor adherence is the most significant contributor to rates of pseudo-resistance among treated hypertensive patients. In this report, we describe 4 patients with apparent treatment-resistant hypertension, who received intervention to promote adherence by pharmacists who set the prescribed medicines in a weekly medication calendar and conducted a weekly pill count. The results showed that the intervention of pharmacists to medication adherence improved systolic BP in patients with apparent treatment-resistant hypertension; however, further controlled trials are required to strengthen supporting evidence.

Congenital biliary dilatation (CBD) associated with intestinal malrotation (IM) has rarely been reported. We herein report three cases of pediatric CBD associated with IM that was incidentally found at surgery. The patients underwent total resection of the extrahepatic bile duct followed by hepaticoduodenostomy at the hepatic hilum or hepaticoduodenostomy as the definitive surgery for CBD. Ladd's procedure was simultaneously performed for asymptomatic IM. Postoperative adhesive intestinal obstruction occurred in all three patients, and conservative treatment was successful. Duodenogastric regurgitation was also observed as a biliary reconstruction-related complication, and conversion to hepaticojejunostomy at the hepatic hilum was performed in two of the three patients. We herein present the clinical course of these three patients and discuss the clinical significance of the association between CBD and IM in terms of the surgical strategy and in comparison with 12 previously reported cases. Keywords: Congenital biliary dilatation, Intestinal malrotation, Postoperative adhesive intestinal obstruction

We report here two cases of Kasai type IIIa biliary atresia (BA), associated with pancreaticobiliary maljunction (PBM). Both cases were referred to our hospital because of hyperbilirubinemia and diagnosed with BA preoperatively by abdominal ultrasonography and biliary scintigraphy. They underwent Kasai portojejunostomy at 49 and 37 days old, respectively. Intraoperative cholangiographies performed from their gallbladders clarified Kasai type IIIa BA with PBM. Laboratory test of colorless fluid from the gallbladder of one patient showed high level of lipase (460 U/L). Reported cases of BA associated with PBM are rare and this is a second report showed elevated level of a pancreatic enzyme in bile. The findings in our cases indicate the pathogenesis of biliary anomaly in association with PBM. Keywords: Kasai type IIIa biliary atresia, Pancreaticobiliary maljunction, Infantile obstructive cholangiopathy

Background: Congenital urethroperineal fistula is an extremely rare anomaly in which a second channel rises from the prostatic urethra and opens onto the perineum. This entity resembles the hypospadiac type of urethral duplication, but resection of the ventral channel is simple and curative. Case: A 4-year-old healthy boy had a small pimple on his perineum that yielded a small amount of whity discharge. Discharge occurred every one month for three years. The patient had no history of incontinence or urinary tract infection. Physical examination revealed a small opening to the median perineal raphe below the scrotum. Voiding cystourethrogram showed a normal urethra but no fistula was demonstrated. Fistulography failed due to closure of external orifice at the small pimple. MRI revealed abnormal sagittal tract from the pimple to the prostatic urethra. A circular incision was made around the pimple and the dissection was performed along the fistula. The fistula was ligated. Histopathologic examination of the fistula revealed that it was lined with squamous epithelium. Two months after surgery, incision of the abscess was performed. He has been well with 8 months postoperatively. Conclusion: Simple resection of the ventral channel of the urethroperineal fistula is curative. While both are rare entities, differentiation to the hypospadiac type of urethral duplication is crucial for definite treatment.

There is a risk for high-output congestive heart failure to be associated with an extra-lobar pulmonary sequestration (ELS) when there is left-to-left shunting caused by a large systemic arterial supply to a sequestration and venous drainage via the pulmonary veins into the left atrium. We present a 2-year-old boy who underwent thoracoscopic resection of an unusually hypervascular right ELS with a high output left-to-left shunt between the aorta and the left atrium via a pulmonary vein. This case is of particular interest because computed tomographic angiography identified a hypervascular nidus with indistinct borders between arterial and venous vessels suggestive of an arteriovenous malformation.

Background We previously reported a pilot study of temporary umbilical loop colostomy for neonates with intermediate-type anorectal malformations (ARM) and recommended this technique because of its cosmetic excellence. We herein report the postoperative complications of umbilical stomas (US) compared with traditional abdominal stomas (AS).Methods From our institutional prospective database, we analyzed the patients with ARMs who underwent stoma creation at Kansai Medical University Hospital from January 1995 to November 2016. The surgical technique used to create the US had been performed since 2004.Results US and AS were made for 12 and 27 patients with ARMs, respectively. The postoperative complication rates in patients who underwent US and AS had no significant difference (17% and 11%, p=0.6). The complications comprised a wound infection (one case of US), ileus (one case each of US and AS), mucosal prolapse (one case of AS), and depression (one case of AS). No emergency surgery was required for these complications.Conclusion For patients with ARMs, the umbilicus appears to be a safe alternative site for temporary loop colostomy.

Late-onset congenital diaphragmatic hernia (CDH) is an uncommon subset of CDH and distinct from neonatal CDH with respect to presenting symptoms, diagnosis, management, and prognosis. In particular, CDH diagnosed after 30 days of age (late-onset CDH) is uncommon and has an atypical presentation and a more favorable prognosis. In the present report, an infantile late-onset CDH case that presented with bloody stool and had a severe clinical course is described. In previous reports, no late-onset CDH case developed bloody stool. After diagnosis with image inspections, emergency surgery was performed. At operation, via the hernia orifice, the jejunum was seen to have prolapsed into the thoracic cavity with focally significant intestinal and mesenteric congestion, but no intestinal necrosis. In general, other disorders such as intussusception may be considered in the differential diagnosis of acute abdomen with bloody stool in patients of this age. However, in such late-onset CDH cases, immediate differentiation from other causes of acute abdomen that present with bloody stool is life-saving.

Background and objectives: Wheelchair basketball players are classified into four functional classes according to the player’s “volume of action”, characterized by trunk movement and stability. As this classification is based on a kinematic point of view, test items might be differentially affected by the functional classification level. We aimed to clarify field-based skill test items closely related to the functional classification level. Materials and Methods: Twenty-six female wheelchair basketball athletes (Japan National Team candidates) completed seven field-based skill tests (20 m sprint, agility T-test, figure-eight with a ball test, the Yo-Yo 10 m recovery test, and three types of maximal passes), and anthropometric measurements were applied. Results: Significant differences among the classification levels were found for one-hand maximal passes (baseball and hook passes) and the figure-eight with a ball test. Furthermore, performance in the 20 m sprint and 10 m Yo-Yo recovery tests significantly differed between classes 1 and 4. Conclusions: The test items most influenced by the classification levels were one-hand passes, which require trunk stability and balance not only in the horizontal plane, but also in the sagittal and frontal planes. Coaches should consider which test items are strongly affected by the functional classification level when assessing a player’s performance.

Although thoracoscopic lobectomy for severe bronchiectasis has been reported in children, this is the first report of double lobectomy of the right middle and lower lobes performed using the video-assisted thoracoscopy in a 9-year-old girl. The post-operative course was uneventful and she is currently well after 18 months′ follow-up.

Interstitial lung disease (ILD) can be separated into two groups: (i) that in which the causes are known; and (ii) that in which the etiologies have not been determined. Meanwhile, complement is essential for the inflammatory response and complement systems have been recognized as factors involved in organizing ILD. In the present study we investigated whether there were any differences in complement-related membrane proteins on lymphocytes and alveolar macrophages in bronchoalveolar lavage fluids (BALF) between different ILD. Bronchoalveolar lavage fluid samples were obtained from 12 patients and eight healthy individuals. Bronchoalveolar lavage fluid cells were incubated with monoclonal antibodies (mAbs) against C3b/C4b receptor (CR1), C3bi receptor (CR3), membrane cofactor protein (MCP) and decay accelerating factor (DAF) and were then labeled with fluorescein isothio-cyanate-conjugated anti-mouse IgG. Cells were analyzed using a flow cytometer.The results demonstrate for the first time that CR3 and DAF are elevated on alveolar macrophages, and that MCP and DAF are increased on BALF lymphocytes in sarcoidosis. The possible roles of complement-related membrane proteins in the pathogenesis of immune processes that are ongoing in sarcoidosis are still obscure, but our results may provide some useful information on the mechanisms underlying sarcoidosis.