Usefulness of an artificial neural network for a beginner to achieve similar interpretations to an expert when examining myocardial perfusion images.

This study examined whether using an artificial neural network (ANN) helps beginners in diagnostic cardiac imaging to achieve similar results to experts when interpreting stress myocardial perfusion imaging (MPI). One hundred and thirty-eight patients underwent stress MPI with Tc-labeled agents. An expert and a beginner interpreted stress/rest MPI with or without the ANN and the results were compared. The myocardium was divided into 5 regions (the apex; septum; anterior; lateral, and inferior regions), and the defect score of myocardial blood flow was evaluated from 0 to 4, and SSS, SRS, and SDS were calculated. The ANN effect, defined as the difference in each of these scores between with and without the ANN, was calculated to investigate the influence of ANN on the interpreters' performance. We classified 2 groups (insignificant perfusion group and significant perfusion group) and compared them. In the same way, classified 2 groups (insignificant ischemia group and significant ischemia group) and compared them. Besides, we classified 2 groups (normal vessels group and multi-vessels group) and compared them. The ANN effect was smaller for the expert than for the beginner. Besides, the ANN effect for insignificant perfusion group, insignificant ischemia group and multi-vessels group were smaller for the expert than for the beginner. On the other hand, the ANN effect for significant perfusion group, significant ischemia group and normal vessels group were no significant. When interpreting MPI, beginners may achieve similar results to experts by using an ANN. Thus, interpreting MPI with ANN may be useful for beginners. Furthermore, when beginners interpret insignificant perfusion group, insignificant ischemia group and multi-vessel group, beginners may achieve similar results to experts by using an ANN.

Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension.

Pediatric living donor liver transplantation (LDLT) in patients with advanced portopulmonary hypertension (PoPH) is associated with poor prognoses. Recently, novel oral medications, including endothelin receptor antagonists (ERAs), phosphodiesterase 5 (PDE5) inhibitors, and oral prostacyclin (PGI2) have been used to treat PoPH. Pediatric patients with PoPH who underwent LDLT from 2006 to 2016 were enrolled. Oral pulmonary hypertension (PH) medication was administered to control pulmonary arterial pressure (PAP). Four patients had PoPH. Their ages ranged from 6 to 16 years, and their original diseases were biliary atresia (n = 2), portal vein obstruction (n = 1), and intrahepatic portal systemic shunt (n = 1). For preoperative management, 2 patients received continuous intravenous PGI2 and 2 oral medications (an ERA alone or an ERA and a PDE5 inhibitor), and 2 received only oral drugs (an ERA and a PDE5 inhibitor). One patient managed only with intravenous PGI2 died. In the remaining 3 cases, intravenous PGI2 or NO was discontinued before the end of the first postoperative week. Postoperative medications were oral PGI2 alone (n = 1), an ERA alone (n = 1), or the combination of an ERA and a PDE5 inhibitor (n = 1). An ERA was the first-line therapy, and a PDE5 inhibitor was added if there was no effect. New oral PH medications were effective and safe for use in pediatric patients following LDLT. In particular, these new oral drugs prevent the need for central catheter access to infuse PGI2.

Pharmacokinetic Profile of Twice- and Once-daily Tacrolimus in Pediatric Kidney Transplant Recipients.

BACKGROUND: The aim of this study was to assess the differences in pharmacokinetic (PK) profiles after the 1:1 ratio-based conversion from a twice-daily to a once-daily tacrolimus formulation (TD-TAC and OD-TAC, respectively) in pediatric recipients of kidney transplants. METHODS: TD-TAC was initially administered to 29 pediatric patients who underwent kidney transplantations between April 2010 and September 2015 and were then subsequently switched to OD-TAC. The switch dose ratio was 1:1, and the 24-hour complete PK parameter assessment was performed before and after the regimen was changed from TD-TAC to OD-TAC. RESULTS: The mean total daily dose at baseline was 5.5 ± 2.9 mg (0.18 ± 0.10 mg/kg body weight). Consecutive PK studies revealed no significant difference in the mean time to achieve maximum concentrations and the area under the concentration-time curve from 0 to 24 hours (AUC0-24) of both drug formulations. However, the mean trough concentration (Cmin) and the maximum concentration of OD-TAC were 22% and 6% lower and higher, respectively, than those of TD-TAC. Therefore, a better correlation was observed between the AUC0-24 and Cmin of OD-TAC than between those of TD-TAC. CONCLUSIONS: After the change from TD-TAC to OD-TAC, the AUC0-24 values were equivalent despite a 22% reduction in Cmin. Cmin may therefore be an excellent predictor in the therapeutic drug monitoring of OD-TAC because of its superior correlation with AUC0-24.

Cystic lymphangioma of Retzius space manifested as acute abdomen.

The authors describe a rare pediatric case of cystic lymphangioma arising from the Retzius space. A 9-year-old boy underwent an appendectomy in a nearby hospital after a sudden onset of severe hypogastralgia. When laparotomy revealed a retroperitoneal mass, he was referred to our hospital. After diagnosis of a multicystic mass in the Retzius space, extirpation of the cystic lesion was performed. Histological evaluation of the resected specimens revealed cystic lymphangioma. The patient has been free of symptoms for 6 years since the operation.

Idiopathic gastric rupture in a 3-month-old girl.

UNLABELLED: This case reports on idiopathic gastric rupture in a 3-mo-old girl who had frequent episodes of vomiting and abdominal distension the day before admission to the clinic. Metabolic acidosis was detected and rotavirus antigen was identified in the stools. Abdominal X-rays and CT scans revealed free air and fluid in the abdominal cavity, leading to a diagnosis of gastrointestinal perforation. During surgery, idiopathic gastric rupture was detected and treated. The postoperative course was uneventful. CONCLUSION: Idiopathic gastric rupture is extremely rare in childhood beyond the neonatal period. Early diagnosis and surgery are mandatory if children with this condition are to be saved.

A case of ischemic jejunal stricture after surgical reduction of intussusception.

A 15-year-old girl presented with small bowel obstruction due to ischemic jejunal stricture which developed three weeks after successful surgical reduction of an intussusception with a Peutz-Jeghers-type polyp as a lead point. The reduced jejunum had no macroscopic injury, and the stricture caused complete obstruction requiring jejunal resection.

Benign transient non-organic ileus of neonates.

There are a substantial number of neonates who present with Hirschsprung's disease-like symptoms, but respond very well to conservative therapy. However, once Hirschsprung's disease is ruled out, little attention is paid to these infants, because of the lack of necessity for surgical treatment and their excellent prognosis. The purpose of this study was to elucidate the clinical features of functional ileus of neonates, which we named benign transient non-organic ileus of neonates (BTNIN). Out of 61 neonates referred to our institution with suspected neonatal Hirschsprung's disease (NH), 10 were diagnosed as having NH and 51 as having BTNIN. All the cases of BTNIN showed marked abdominal distension, and 12 showed explosive defecation on digital examination at the first visit. Plain X-ray demonstrated marked whole intestinal dilatation in 12 cases including cases with niveau formation and segmental dilatation. These findings were indistinguishable from those of NH. However, all had a normal anorectal reflex, and rectal suction biopsy revealed normal acetylcholinesterase activity and submucosal ganglion cells. All the cases of BTNIN were treated with periodic glycerin enemas until daily spontaneous defecation was established, which took 2 to 14 months, with an average of 5.0 +/- 2.9 months. None of them showed residual symptoms during the follow-up period.

Late onset of right Bochdalek's hernia with strangulation of the omentum.

UNLABELLED: We report a case of bloody pleural effusion and infarction of the greater omentum caused by a non-traumatic diaphragmatic hernia with a late presentation. A 15-y-old boy with Down's syndrome developed abdominal pain and vomiting, as well as an elevated serum level of C-reactive protein. Chest roentgenograms showed a right-sided pleural effusion and computed tomography revealed a right diaphragmatic hernia. Barium enema confirmed the diagnosis. An operation revealed a right Bochdalek's hernia with strangulation of the greater omentum in the right pleural cavity. CONCLUSION: Diaphragmatic hernia should be considered in patients with pleural effusion, abdominal pain and vomiting.

In vitro and in vivo inhibition of muscle lipid and protein oxidation by carnosine.

Carnosine, a beta-alanyl-L-histidine dipeptide with antioxidant properties is present at high concentrations in skeletal muscle tissue. In this study, we report on the antioxidant activity of carnosine on muscle lipid and protein stability from both in vitro and in vivo experiments. Carnosine inhibited lipid peroxidation and oxidative modification of protein in muscle tissue prepared from rat hind limb homogenates exposed to in vitro Fenton reactant (Fe2+, H2O2)-generated free radicals. The minimum effective concentrations of carnosine for lipid and protein oxidation were 2.5 and 1 mM, respectively. Histidine and beta-alanine, active components of carnosine, showed no individual effect towards inhibiting either lipid or protein oxidation. Skeletal muscle of rats fed a histidine supplemented diet for 13 days exhibited a marked increase in carnosine content with a concomitant reduction in muscle lipid peroxidation and protein carbonyl content in skeletal muscle caused by subjecting rats to a Fe-nitrilotriacetate administration treatment. This significant in vitro result confirms the in vivo antioxidant activity of carnosine for both lipid and protein constituents of muscle under physiological conditions.

The preparation of the chronic hyper-endotoxemia experimental animal model by means of a drug delivery system.

A drug delivery system (DDS) consisting of lipopolysaccharide (LPS) as a drug and 2-hydroxyethyl methacrylate (HEMA)-diethylene glycol dimethacrylate (2G) or -polyethylene glycol dimethacrylate (4G, 9G) copolymer was prepared, and used for the efficient preparation of an experimental animal model of chronic hyper-endotoxemia. The release profiles of LPS in the in-vitro test were greatly influenced by the composition of HEMA-2G, 4G, 9G in the copolymer. It was found that LPS release from the DDS continued gradually and constantly throughout 2 weeks. In the in-vivo experiment with rats, the DDS maintained a high blood concentration level of LPS for 3 days. These results strongly suggest the possibility of convenient and reproducible preparation of a chronic hyper-endotoxemia animal model.

Application of a drug delivery system in a novel rat model of chronic hyperendotoxemia.

There has not been an ideal reproducible small-animal model of chronic hyperendotoxemia to date. Our drug delivery system (DDS) is a new technology that can deliver a drug conveniently to a target organ at an optional rate. 2-Hydroxyethyl methacrylate (HEMA) was used as a carrier of lipopolysaccharide (LPS), and diethylene glycol and polyethylene glycol dimethacrylates (2G, 4G, 9G) were used as cross-linking agents. A mixed solution of HEMA and di(poly)ethylene glycol dimethacrylate was charged into a glass tube with or without LPS and polymerized by ultraviolet irradiation. This polymer was cut into DDS tablets of the same size with or without LPS. A mixture with HEMA:4G = 1:3 was the most suitable composition to release a constant concentration of LPS. We also developed a novel rat model of chronic hyperendotoxemia. Four DDS tablets, each containing 15 mg LPS, were implanted into the abdominal cavity of rats in the LPS group. The control group was implanted with four DDS tablets without LPS. Plasma levels of LPS in the study group were maintained at more than 2,000 pg/ml for 72 h after implantation. Weight gain was lower and body temperature was higher in the LPS group than in the control group. Plasma levels of inter leukin (IL)-6 in the LPS group were higher than in the control group only during the initial 12 h after implantation of DDS tablets. The white blood cell count at 24 h and platelet counts at 24, 48, and 72 h in the LPS group were lower than those in the control group. These results indicate that chronic hyperendotoxemia was maintained for 72 h by continuous release of LPS from the DDS. Moreover, the intensity of endotoxemia could be varied by varying the number of DDS tablets. It is concluded that our new rat model using LPS-DDS will be applicable and useful as a model of chronic hyperendotoxemia.

Clinicopathologic relationship of hypoganglionosis.

BACKGROUND/PURPOSE: Congenital motor dysfunction of the intestine associated with a morphologically abnormal myenteric nervous plexus (MP) is known as Hirschsprung's disease allied disorder (HAD). However, the clinicopathologic features of HAD are not well understood, partially because a standardized method of histologic evaluation of MP has not been established. To elucidate the clinicopathologic relationship of HAD the authors reviewed 6 cases of HAD using a newly devised histologic evaluation method. METHODS: Flat-mounted frozen sections of the ileum were stained for S-100 protein by fluorescent immunohistochemistry. Quantitative evaluation of MP was performed by measuring the fluorescence-positive area (MP ratio), and the results were compared with those of age-matched normal controls. RESULTS: All of 6 patients required laparotomy within 1 month after birth and enterostomy between 23 days and 10 months. Three died of intractable enteritis by the age of 2.2 years and were totally dependent on parenteral nutrition (PN) throughout their lives. The other 3 have survived for 6 to 10 years but have required PN occasionally. MP ratio in controls was more than 0.34 at all ages, whereas that in HAD was significantly lower than that in controls according to the clinical severity. CONCLUSION: MP size measured on 2-dimensional demonstration is suggested to be an indicator of clinical severity of HAD. J Pediatr Surg 36:898-900.

Surgical intervention for emphysematous pulmonary regions in a postoperative infant with congenital diaphragmatic hernia.

A postoperative infant with congenital diaphragmatic hernia (CDH) developed extrinsic obstruction of the trachea by the innominate artery that ensued from unequal expansion of the lungs followed by left mediastinal shift. Septation of the anterior mediastinum prevented unequal expansion of the lungs, and elongation of the innominate artery improved proximal airway obstruction. Prolonged artificial ventilation, however, resulted in the emphysematous bullae in the left lung. Lung volume reduction surgery (LVRS), at 3 years of age, ameliorated the respiratory distress and resulted in good weight gain. Surgical intervention, including LVRS, should be considered to improve respiratory disturbance caused by difference in compliance of the lungs in children.

Two cases of persistent hyperinsulinemic hypoglycemia that showed spontaneous regression and maturation of the Langerhans islets.

Near-total pancreatectomy has been recommended as the treatment for persistent hyperinsulinemic hypoglycemia (PHH) in infants. However, recently there has been a report described that one third of 95% pancreatectomy failed to prevent hypoglycemia and more than two thirds had diabetes ultimately. The authors experienced 2 cases of PHH, which raise a query about the extensive pancreatectomy. Case 1: A female patient who manifested PHH shortly after birth underwent less extensive pancreatectomy twice at age 2 months and 8 years. After each operation, her clinical symptoms regressed, and she became free from the disease eventually. Histologic findings showed nesidioblastosis in which the islets clearly matured. Case 2: A male infant with PHH had an absolute indication for pancreatectomy. However, after a meticulous control of the blood glucose level with parenteral nutrition followed by continuous enteral nutrition feeding combined with medication, he became free from the disease. The current cases show there exist cases of PHH in which the islets mature and symptoms regress spontaneously. Therefore, we conclude near-total pancreatectomy is not always the treatment of choice. As an alternate strategy, long-term controlled feeding and medication combined with or without less extensive pancreatectomy should be considered with the expectation of spontaneous regression.

Total parenteral nutrition-associated intrahepatic cholestasis in infants: 25 years' experience.

BACKGROUND/PURPOSE: There are few long-term chronological reviews examining the incidence of total parenteral nutrition (TPN)-associated intrahepatic cholestasis (TPNAC) in infants. The authors therefore reviewed TPNAC in their 25-year series, and also looked at the current problems associated with TPN in infants. METHODS: Two hundred seventy-three surgical neonates who received TPN for more than 2 weeks were divided into 3 groups chronologically: group A (1971 through 1982, n = 77), group B (1983 through 1987, n = 72), and group C (1992 through 1996, n = 124). TPNAC was defined as serum direct bilirubin (DB) level greater than 2.0 mg/dL during the neonatal period. RESULTS: The incidence of TPNAC in groups A, B and C was 57%, 31%, and 25% (P< .01), respectively, and the mortality rate from TPN-associated complications was 13%, 3%, and 3% (P< .05), respectively. Over the last 5 years, severe TPNAC developed in 20 patients (16%). Four of 20 died of TPN-associated sepsis with hepatic failure; 2 had hypoganglionosis with intractable stagnant enteritis and subsequent sepsis, and 2 had fatal respiratory or cardiac disease. CONCLUSIONS: The incidence of TPNAC in surgical neonates and TPN-associated mortality rates have decreased significantly. The mortality rate, however, still remains at 3%. Two of 4 fatal cases had hypoganglionosis, which were totally dependent on TPN. In patients who require long-term TPN, TPN still has unsolved problems, and small bowel transplantation may be indicated.

Congenital ileal atresia presenting as a single cyst-like lesion on prenatal sonography.

A routine prenatal sonographic examination at 37 weeks' menstrual age revealed a large sonolucent lesion with peristaltic movement in the abdomen of a fetus. After birth, the female infant showed progressive abdominal distention, and radiography showed a bubble-like dilatation of the small intestine. Exploratory laparotomy revealed ileal atresia with nearby partial torsion of the dilated small bowel. The incomplete torsion may have functioned as a check valve, inducing segmental dilatation of the ileum without proximal dilatation.

Handlebar hernia: case report and review of pediatric cases.

The authors describe a rare case of handlebar hernia in a 9-year-old-boy. All layers of his abdominal wall were disrupted by a fall on a bicycle; however, his skin and intra-abdominal organs were completely intact. Computed tomography demonstrated subcutaneous intestinal loops protruding through the rent. Surgical repair was performed, and his postoperative course was uneventful.

Subcapsular hemorrhage of the liver in a very-low-birth-weight neonate: survival after decompression laparotomy.

Subcapsular hemorrhage of the liver in a very-low-birth-weight neonate was successfully treated by decompression laparotomy. This may be the second smallest survivor after surgery in the literature.

Infantile myofibromatosis of the triceps detected by prenatal sonography.

A routine prenatal sonographic examination at 36 weeks' menstrual age revealed a solid and slightly inhomogeneous soft-tissue tumor on a fetus's left upper arm. The mass in the left triceps brachii muscle measured 8 x 7 x 5 cm at birth. Because of progressive flexion contracture of the left elbow joint, at 2 months of age the infant underwent radical resection of the tumor, sparing some muscle fibers. Light microscopic and immunohistochemical studies revealed myofibromatosis. Neither tumor nor functional disorder of the arm was evident 3 years after surgery.