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.

Epidemiologic survey of Blastocystis hominis infection in Japan.

The incidence of Blastocystis hominis in a healthy population was determined by fecal examination of 6,422 Japanese and 54 resident non-Japanese who visited the St. Luke's International Hospital Health Screening Center for a routine medical check-up during a one-year period. Of the enrolled subjects, 30 Japanese (0.5%) and four non-Japanese (7.4%) had B. hominis in their stools. These individuals were asymptomatic except for one who reported flatus and one who reported mild abdominal discomfort. Statistical analysis indicated that the prevalence in the Japanese was lower than in the non-Japanese, and lower than the prevalence reported for other countries. Colonoscopic observations on seven B. hominis-positive individuals did not reveal pathogenic intestinal lesions. Several months after the first examination, 23 of the B. hominis-positive individuals, including three non-Japanese, were re-examined. Although they had not been treated with anti-B. hominis drugs, 10 individuals were now B. hominis-negative (by stool examination) and eight were passing fewer organisms. The remaining five individuals were still discharging large numbers of B. hominis. These B. hominis-positive individuals had no reported symptoms despite passing numerous organisms. Therefore, it seems that infection with B. hominis rarely gives rise to clinical symptoms. In no instance was invasion of host tissues by the organisms detected.

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.

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.

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.

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.

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.

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.

A spigelian hernia in an infant.

The case of a two-month old Japanese girl with a left side Spigelian hernia that developed during the course of recovery from the surgical treatment of a meningomyelocele and ventriculo-peritoneal shunting (V-P shunting) is reported on. At this time, to our knowledge, 20 cases involving infants and children have been reported on in world-wide medical literature. Of these cases, 4 cases were caused by trauma, 1 by abdominal surgery, and the others had no evident causes. Our patient had a meningomyelocele, and underwent a V-P shunting. However, a cause and effect relationship between the Spigelian hernia and the treatment of the meningomyelocele followed by V-P shunting is inconceivable. We doubt any relationship between the preceding neurosurgery and the development of the hernia. This is the first case of infantile Spigelian hernia reported on in Japan.

[Ultrastructural study of interlobular bile ductal disorganization in autoimmune liver diseases].

Ultrastructures of interlobular bile ductules were examined in 7 cases of lupoid hepatitis and related disease (LH) and 8 cases of primary biliary cirrhosis (PBC). Mononuclear cell, especially lymphocyte infiltrations into bile ducts cross basement membrane were common findings found in 57.1% of LH and in 50% of PBC patients, thus, statistically, the occurrence of lymphocyte infiltration was similar for both groups. Cell contact between bile duct epithelia and infiltrated cells differed in LH and PBC. In LH, 86.7% of the cells contacted at small point, but 92.3% PBC cells had broad contact with each other. Stratification of bile duct epithelia and other visible changes in PBC epithelial cells was statistically more extensive compared with LH. Dilatation of intercellular space was often observed in basal region of LH. Destruction and degeneration of LH and PBC bile duct epithelial cells was mainly observed in basal and luminal regions, respectively. Rupture and thickening of basement membrane was seen in PBC, but rarely in LH. The average diameter of interlobular bile ducts were larger and oval shaped in patients with PBC compared to smaller circular ducts observed in LH patients. These results not only revealed that several changes in interlobular bile ducts occur but that similar changes with LH and PBC. This suggests that both distinctions and similarities exist between LH and PBC as autoimmune hepatic disease.