Direct health care cost of treatment and medication of biliary atresia patients using the National Database of Health Insurance Claims and Specific Health Checkups.

BACKGROUND: Treatment of biliary atresia (BA), which typically requires an initial surgical intervention called the Kasai procedure (KP) and possible liver transplant (LT) afterwards, is quite resource-intensive and would affect patients and families for a lifetime; yet a comprehensive view of the economic burden has not been reported. We estimated direct health care costs from the public payer perspective using the National Database of Health Insurance Claims and Specific Health Checkups of Japan. METHODS: Children newly diagnosed at ages 0 days to 4 years between April 2010 and September 2019 were identified. Costs of treatment were estimated for six phases of care: prediagnosis, KP and inpatient hospitalization, follow-up after KP, pre-transplant checkup, LT and inpatient hospitalization, and follow-up after LT. RESULTS: Mean total prediagnosis medical cost was $6847 (USD) and KP and inpatient hospitalization was $42,157 per year. Follow-up after KP was $15,499, and pre-transplant checkup after KP was $36,015 per year. Mean cost for LT and inpatient hospitalization was $105,334, and follow-up after liver transplant was $25,459 per year. CONCLUSIONS: Treatment of BA requires extensive medical resource consumption. The use of the comprehensive national database allowed us to estimate the costs which will be useful for health service planning and cost-effectiveness analysis.

Laparoscopic cholecystectomy for gallbladder torsion in a 3-year-old child.

BACKGROUND: Gallbladder torsion is a congenital abnormality of the gallbladder and is a rare cause of acute abdomen. Although gallbladder torsion is a life-threatening condition requiring emergency surgery, laparoscopic cholecystectomy for gallbladder torsion in pediatric patients has seldom been reported. CASE PRESENTATION: A 3-year-old boy with abdominal pain and vomiting was admitted to our hospital. He was diagnosed with acute cholecystitis through abdominal ultrasound and computed tomography, and he received antibiotics. Two days after admission, a repeat abdominal ultrasound revealed exacerbation of gallbladder wall thickness and lack of intramural blood flow, which led to a diagnosis of gallbladder torsion. Emergency laparoscopic cholecystectomy was performed, and the gallbladder was twisted counterclockwise at 270°. After an uneventful course of intraoperative and postoperative treatment, the patient was discharged on postoperative day 6. CONCLUSION: Laparoscopic cholecystectomy for gallbladder torsion was safely performed in a pediatric patient.

Initial experience with a 3D printed model for preoperative simulation of the Nuss procedure for pectus excavatum.

The incidence of pectus excavatum has been estimated to be between 0.1% and 0.8% though a large autopsy series reports. After publication of the Nuss procedure for pectus excavatum, it became widely accepted. However, there are still some complications, such as over-correction and recurrence. To reduce differences in the procedure due to surgeons' experience level, preoperative simulation may be useful. Thus, we performed simulated surgery using a specific patient's three-dimensional (3D) chest wall model made by a 3D printer before operation. A 13-year-old male patient with a severe deformity of the chest underwent the Nuss procedure. As in the simulation, bars were inserted into the 5th and 7th intercostal spaces (ICS), leading to improvement of the chest wall. This simulation can increase surgeons' confidence to improve the deformity by determination of the number and insertion sites of bars.

An iPhone application using a novel stool color detection algorithm for biliary atresia screening.

BACKGROUND: The stool color card has been the primary tool for identifying acholic stools in infants with biliary atresia (BA), in several countries. However, BA stools are not always acholic, as obliteration of the bile duct occurs gradually. This study aims to introduce Baby Poop (Baby unchi in Japanese), a free iPhone application, employing a detection algorithm to capture subtle differences in colors, even with non-acholic BA stools. METHODS: The application is designed for use by caregivers of infants aged approximately 2 weeks-1 month. Baseline analysis to determine optimal color parameters predicting BA stools was performed using logistic regression (n = 50). Pattern recognition and machine learning processes were performed using 30 BA and 34 non-BA images. Additional 5 BA and 35 non-BA pictures were used to test accuracy. RESULTS: Hue, saturation, and value (HSV) were the preferred parameter for BA stool identification. A sensitivity and specificity were 100% (95% confidence interval 0.48-1.00 and 0.90-1.00, respectively) even among a collection of visually non-acholic, i.e., pigmented BA stools and relatively pale-colored non-BA stools. CONCLUSIONS: Results suggest that an iPhone mobile application integrated with a detection algorithm is an effective and convenient modality for early detection of BA, and potentially for other related diseases.

Evaluation of a trastuzumab-containing treatment regimen for patients with unresectable advanced or recurrent gastric cancer.

The present study aimed to evaluate the efficacy and safety of trastuzumab plus chemotherapy for patients with unresectable advanced or recurrent gastric cancer. A retrospective analysis of 213 patients with unresectable advanced or recurrent gastric cancer who received systemic chemotherapy, including 15 patients who were also administered trastuzumab, at Kochi Medical School between 2007 and 2013 was performed. The overall survival was compared between patients who received trastuzumab plus chemotherapy and patients who received chemotherapy alone, and the safety and efficacy of the trastuzumab-containing regimen was evaluated. Human epidermal growth factor receptor (HER)2 status was examined in 86 patients, of whom 15 (17.4%) exhibited strong positive HER2 expression. The rate of strong positive HER2 expression was significantly higher for intestinal type tumors compared with diffuse type tumors [23.6 (13/55) vs. 6.5% (2/31); P=0.044]. The median overall survival of the patients treated with trastuzumab was significantly longer compared with that for patients who were not treated with trastuzumab (22.9 vs. 11.6 months; P=0.014). The objective response rate and disease control rate for trastuzumab plus chemotherapy were 46.7 and 86.7%, respectively. Frequently encountered grade 3-4 toxicities included neutropenia (26.7%; 4/15), anemia (13.3%; 2/15) and fatigue (13.3%; 2/15). Trastuzumab plus chemotherapy is effective for patients with HER2-positive advanced or recurrent gastric cancer, and the frequencies of hematological and non-hematological toxicities experienced by patients in the present study indicated that it can be safely administered clinically.

Synchronous Large Gastrointestinal Stromal Tumor and Adenocarcinoma in the Stomach Treated with Imatinib Mesylate Followed by Total Gastrectomy.

Herein we report on a case of synchronous large gastrointestinal stromal tumor (GIST) and adenocarcinoma of the stomach treated with radical surgery following neoadjuvant therapy with imatinib mesylate. A 58-year-old man was referred to our hospital with a large mass in the peritoneal cavity. Abdominal computed tomography showed a large mass measuring 21×20×14 cm in the left upper peritoneal cavity. Esophagogastroduodenoscopy revealed a large elevated lesion in the upper body and a depressed lesion in the lower gastric body near the lesser curvature. Biopsy specimens revealed GIST in the large elevated lesion and signet-ring cell carcinoma in the depressed lesion. Because of the large size of the GIST, the patient was treated with neoadjuvant therapy with imatinib mesylate (400 mg/day) for 5 months. After confirmation of a marked decrease in tumor size following imatinib mesylate therapy, the patient underwent total gastrectomy and regional lymph-node dissection with distal pancreatectomy and splenectomy. Pathological examination confirmed the diagnosis of high-risk GIST and signet-ring cell carcinoma invading the muscularis propria with one lymph-node metastasis. At the time of writing, the patient was receiving postoperative chemotherapy using oral fluoropyrimidine (S-1) without evidence of disease recurrence for 4 months after surgery. In addition to the present case, we provide a retrospective review of another 15 patients who were diagnosed with synchronous GIST in the stomach and primary gastric adenocarcinoma.

Impact of an external lengthening procedure on the outcome of long-gap esophageal atresia at our hospitals.

PURPOSE: To demonstrate the outcome of external lengthening for long-gap esophageal atresia (LGEA) at our hospitals. METHODS: Five patients with LGEA underwent external lengthening between 2010 and 2014 (group A), and 11 patients with LGEA underwent other lengthening techniques between 1990 and 2011 (group B). We compared the procedure and outcome between these two groups. RESULTS: The mean birth weight was 2001 g in group A and 2485 g in group B (p = 0.06). The mean age at esophageal reconstruction was 28 days in group A and 227 days in group B (p = 0.03). Although primary esophageal anastomosis without myotomy was feasible in all patients in group A, a myotomy was needed for primary esophageal anastomosis in half of the patients in group B. Anastomotic leakage occurred in none in group A and in six patients in group B (p = 0.03). The mean age at the establishment of full oral feeding was 76 days in group A and 686 days in group B (p = 0.009). CONCLUSION: External traction for LGEA can effectively lengthen the esophagus to enable primary anastomosis at an earlier age. This may facilitate oral intake.

Video-assisted thoracoscopic lobectomy with the patient in the semi-prone position: initial experience and benefits of lymph node dissection.

OBJECTIVES: Recently, the prone position has been used for thoracoscopic oesophagectomy for oesophageal cancer because it is known to facilitate mediastinal dissection. We hypothesized that this advantage of the prone position could apply to video-assisted thoracoscopic surgery, which has been commonly performed with the patient in the lateral position. METHODS: Forty-six patients with clinical stage I, right-sided, non-small cell lung cancer were enrolled in this study. They were classified into three groups: conventional thoracotomy using rib retractors (n = 17), and the lateral (n = 15) and semi-prone (n = 14) positions in video-assisted thoracoscopic surgery. Surgical parameters, such as operation time, the amount of blood loss, and the number of dissected lymph nodes, of each group of patients were compared. RESULTS: There were three (6.5 %) minor complications in this study, chylothorax and heart failure in the thoracotomy group and atelectasis with the video-assisted thoracoscopic surgery in the lateral position group. No complications were seen in the semi-prone group. The number of dissected lymph nodes was significantly higher in the video-assisted thoracoscopic surgery in the semi-prone position group than in the conventional thoracotomy group for inferior mediastinal nodes (subcarinal, paraeosophageal, and pulmonary ligament, average 8.9 and 5.9 lymph nodes, p = 0.04). CONCLUSIONS: To the best of our knowledge, this is the first report that has described video-assisted thoracoscopic lobectomy with the patient in the semi-prone position. Although the number of cases was limited, the results of this study show that the semi-prone position could be attempted especially for right lower lobe lung cancer.

Intratracheal Administration of Recombinant Human Keratinocyte Growth Factor Promotes Alveolar Epithelial Cell Proliferation during Compensatory Lung Growth in Rat.

Keratinocyte growth factor (KGF) is considered to be one of the most important mitogens for lung epithelial cells. The objectives of this study were to confirm the effectiveness of intratracheal injection of recombinant human KGF (rhKGF) during compensatory lung growth and to optimize the instillation protocol. Here, trilobectomy in adult rat was performed, followed by intratracheal rhKGF instillation with low (0.4 mg/kg) and high (4 mg/kg) doses at various time-points. The proliferation of alveolar cells was assessed by the immunostaining for proliferating cell nuclear antigen (PCNA) in the residual lung. We also investigated other immunohistochemical parameters such as KGF, KGF receptor and surfactant protein A as well as terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling. Consequently, intratracheal single injection of rhKGF in high dose group significantly increased PCNA labeling index (LI) of alveolar cells in the remaining lung. Surprisingly, there was no difference in PCNA LI between low and high doses of rhKGF with daily injection, and PCNA LI reached a plateau level with 2 days-consecutive administration (about 60%). Our results indicate that even at low dose, daily intratracheal injection is effective to maintain high proliferative states during the early phase of compensatory lung growth.

Yolk sac tumor of the vulva: a case report with recurrence after long-term follow-up.

A yolk sac tumor (YST) of the vulva is extremely rare and highly malignant with recurrence frequently occurring within a year. This report presents the 13th known case of vulvar YST, with recurrence occurring after the longest known follow-up period so far reported in the literature.

Pancreatic head resection preserving the main pancreatic duct for congenital hyperinsulinism of infancy.

Surgical intervention for congenital hyperinsulinism with an unclear focal lesion in the pancreatic head sometimes require the resection of most of the pancreas head and pancreaticojejunotomy. This report presents the case of a patient that underwent pancreatic head resection preserving the main pancreatic duct to avoid pancreaticojejunostomy.

A modified Foker's technique for long gap esophageal atresia.

External traction using the Foker's technique enables elongation in the esophageal segments within days, and allows the primary repair of the long gap. This article presents our modified Foker's technique which was easily applicable for long-gap esophageal atresia.

The usefulness of a high-speed 3D-image analysis system in pediatric living donor liver transplantation.

BACKGROUND: Since March 2010, we have used a high-speed 3D-image analysis system (SYNAPSE VINCENT) to calculate the graft volume in living donor liver transplantation (LDLT) to replace CT volumetry. The SYNAPSE VINCENT is capable of extracting each vessel territory in the liver and displaying 3D images simply, quickly, and accurately. Therefore, we report here the usefulness of the SYNAPSE VINCENT in pediatric LDLTs in overcoming issues with perfusion area of hepatic venous tributaries in monosegmental grafts. MATERIAL/METHODS: The SYNAPSE VINCENT was used in three pediatric patients. In two of these cases, the possibility of monosegmental grafts was assessed when calculating graft volumetry of segment III. RESULTS: The graft recipient weight ratio (GRWR) with graft volumetry measurements of the left lateral segment were 1.8-5.6%. GRWR of segment III were 2.3 and 2.0%. Since donor V2, venous branch to segment II and V3, venous branch to segment III were independently branching in one case, the monosegmental graft could be evaluated preoperatively according to the venous perfusion. CONCLUSIONS: Graft volumetry using the SYNAPSE VINCENT was useful for planning the LDLT operative procedures, especially in infants possibly in need of monosegmental graft.

A gallstone in the Roux-en Y limb during pregnancy in a female patient with biliary atresia.

A 28-year-old female developed liver dysfunction at the 13th gestation week of her second pregnancy. A postpartum radiological examination revealed a stone in the Roux-en Y (RY) limb. She underwent surgical removal of the gallstone in the RY limb. Bile congestion during pregnancy led to the development of a gallstone.

Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1.

We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa require further investigation.

Prophylactic ligation of the innominate artery and creation of tracheostomy in a neurologically impaired girl: a case report.

Tracheoinnominate artery fistula is known as a potentially fatal complication for patients who depend on tracheostomy or tracheoesophageal diversion. Since the bleeding from a TIF is often difficult to control, preventative procedures are recommended to avoid this complication. An 11-year-old girl with hypoxic-ischemic encephalopathy and scoliosis developed tracheal stenosis caused by compression from the innominate artery. Respiratory control with intubation through the tracheal stenosis was needed, and the patient was at high risk for developing a TIF. She underwent ligation of the innominate artery at tracheostomy. Subsequent tracheostomy revealed a widened tracheal lumen and no further complications. Prophylactic ligation of the innominate artery and creation of tracheostomy might be considered as a valid option for patients at high risk of developing TIF.

Endoscopic balloon dilatation for congenital membranous stenosis in the jejunum in an infant.

INTRODUCTION: As endoscopic equipment and instruments have improved, the indications for endoscopic treatment have also been extended. This report presents an applicable procedure of endoscopic balloon dilatation for an infant patient with congenital membranous stenosis in the jejunum. METHODS: We used a 9-mm flexible endoscope and a through-the-scope multidiameter balloon catheter in the endoscopic treatment. RESULTS: Dilatation was performed for dilatation diameters 10, 12, and 15 mm each for 2 min. After carrying out balloon dilatation, the endoscope could be smoothly inserted through the opening. CONCLUSION: In upper jejunal stenosis, endoscopic balloon dilatation was minimally invasive and effective as a treatment modality.

Urinary sulfated bile acid analysis for the early detection of biliary atresia in infants.

BACKGROUND: Measurement of urinary sulfated bile acid (USBA) is a non-invasive method to detect bile congestion. Our aim was to evaluate the feasibility of USBA analysis for the early detection of biliary atresia (BA). METHODS: We determined the USBA-to-creatinine ratio (USBA/cr) in 1148 infants at 10-40 days after birth. All infants were followed until the 3- to 4-month postnatal routine health check. The cutoff value for USBA/cr was 55.0 µmol/g creatinine. RESULTS: Among the infants tested, 47 (4.10%) had USBA/cr ratios that exceeded the cutoff value. Two of these 47 infants had liver disease; one was diagnosed with neonatal hepatitis syndrome, and the other was diagnosed with BA. The BA patient underwent USBA analysis for the first time on day 18 after birth and hepatoportoenterostomy on day 49. No other infants were diagnosed with hepatobiliary disease during the follow-up period. CONCLUSION: This USBA analysis provided the correct assessment without fail and identified a case of BA. This approach could be used for the screening and early detection of BA when the false-positive rate is decreased by improving the methods for sample collection and urine storage.

Long-term results of chest wall reconstruction with DualMesh.

The aim of this study was to evaluate the clinical outcomes of chest wall reconstruction using a new, expanded polytetrafluoroethylene prosthesis, 'DualMesh'. Between December 2005 and March 2010, chest wall reconstruction using 2-mm DualMesh was performed in 11 patients. The indication for resection was primary lung cancer in six patients, malignant mesothelioma in one patient, recurrent lung cancer in one patient, recurrent invasive thymoma in one patient, postirradiated osteomyelitis in one patient, and chondro-hamartoma in one patient. The mean observation period was 23 months, and four cases were observed for more than three years. There were no operative deaths and no wound infections. There were two postoperative complications: prolonged air leakage occurred in a patient with pulmonary emphysema who underwent right lower lobectomy, and slight paradoxical respiration occurred in the patient who underwent resection of the entire sternal body for osteomyelitis. Follow-up chest computed tomography was performed routinely. No dehiscence occurred in any cases. Chest wall reconstruction using DualMesh demonstrated acceptable durability and biocompatibility, even after long-term follow-up. DualMesh has the potential to become an ideal prosthesis for the bony chest wall as an alternative to conventional polytetrafluoroethylene or polypropylene grafts.