Interobserver variability in antroduodenal manometry.

Interobserver variability affects investigations involving assessment of complex visual data, such as histopathology, radiology and motility. This study assessed interobserver variation for interpretation of antroduodenal manometry (ADM), as this has not been previously investigated. Thirty-five ADM recordings from children aged 0.3-18 years were independently evaluated by five experienced paediatric gastroenterologists who were blinded to cases' clinical histories. Intra-class correlation (ICC) was analysed for detection and measurement of phase three of the migrating motor complex (MMC) and Cohen's kappa statistic was calculated between observer pairs for detection of specific motility features and final diagnosis. Observers were unanimous on the differentiation of normal and abnormal motility in 63% of cases. There was excellent interobserver agreement for the number of phase three of the MMC in fasting (ICC = 0.82, P < 0.0001) and for measurements of phase three of the MMC (ICC = 0.9999, P < 0.0001). Detection of other normal and abnormal motility patterns varied more. Objective findings such as the presence of phase three of the MMC correlated more closely than findings that involved the integration of several variables, such as final diagnosis. However, these data overall indicate that agreement between expert observers for the distinction of normal and abnormal antroduodenal motility compares favourably with other standard medical assessments.

Effect of formula thickened with reduced concentration of locust bean gum on gastroesophageal reflux.

AIM: Previous studies showed that HL-350, a formula thickened with a reduced concentration of locust bean gum, decreased frequent regurgitation in 4-month old infants with reflux. In this study, we investigated the effect of HL-350 in younger infants. METHODS: We studied 20 infants less than 2 months old who had three or more episodes of regurgitation or vomiting per day. Ten infants (group A) were fed with HL-350 for the first week, and with control milk, HL-00, for the following week. The other 10 infants (group B) were fed in reverse order. Mothers recorded number of regurgitation episodes, feeding volume and time and number of bowel movements. To evaluate gastric emptying we measured antral cross sectional areas ultrasonographically at various time points after feeding. RESULTS: The median number of regurgitation episodes decreased significantly with feeding of HL-350 (2.3/day) compared to feeding with control milk (5.2/day) (p = 0.00048). No significant difference was evident in feeding volume and time, body weight gain, or gastric emptying rate between HL-350 and control milk. CONCLUSION: HL-350 decreased the number of regurgitation episodes without affecting gastric emptying delay in very young infants with recurrent vomiting.

Measurement of tricuspid annular diastolic velocities by Doppler tissue imaging to assess right ventricular function in patients with congenital heart disease.

We assessed the clinical utility of using diastolic tricuspid annular velocities obtained by Doppler tissue imaging as a noninvasive index of right ventricular function in patients with congenital heart disease. Doppler tissue imaging at the tricuspid annulus and pulsed Doppler echocardiography of the right ventricular inflow were performed in 71 children with congenital heart disease, with and without elevated right ventricular pressure. Cardiac catheterization was performed in all patients with congenital heart disease, and the hemodynamic determinants of the tricuspid annular and inflow velocities were determined. In patients with congenital heart disease, the ratio of the late-to-early diastolic tricuspid annular velocity (Aa/Ea) showed a highly significant correlation with right ventricle pressure/left ventricle pressure (r = 0.79, p < 0.0001), right ventricular end diastolic pressure (r = 0.46, p < 0.0001), and the first derivatives of the change in right ventricle pressure during diastole (r = 0.72, p < 0.0001). However, the late-to-early diastolic tricuspid inflow velocity (A/E) did not correlate with any invasively measured index of right ventricular function. Aa/Ea, derived from tricuspid annular velocities as measured by Doppler tissue imaging, is a valuable, noninvasive tool for detecting an elevated right ventricular pressure in patients with congenital heart disease.

Percutaneous hydrodynamic thrombectomy for femoral arterial thrombosis after arterial catheterization.

We report on a 3-year-old boy in whom a complete occlusive thrombus in the right femoral artery was removed rapidly and successfully using a hydrodynamic thrombectomy catheter. There was no bleeding complication, reocclusion, vascular injury, or loss of motor or sensory function. Hydrodynamic thrombectomy can be an important part of the treatment of thrombosis in children.

Endosonographic diagnosis of pneumatosis cystoides intestinalis in infancy.

We report pneumatosis cystoides intestinalis in a 10-month-old girl who developed bloody diarrhea following chemotherapy for leukemia. The diagnosis was made only by colonic endoscopic ultrasonography, whereas the abdominal plain radiogram and computed tomography failed to elucidate the diagnosis. She was successfully treated with hyperbaric oxygen therapy. Wider application of endoscopic ultrasonography may lead to the more frequent detection of pneumatosis cystoides intestinalis, currently a rare disorder.

Divided right atrium associated with extensive coronary vein abnormalities.

This report describes a case of a divided right atrium associated with coronary vein abnormalities. An 18-year-old woman who had a past history of surgery for repair of a divided right atrium and atrial septal defect developed exertional dyspnea 8 years after the surgery. Selective coronary angiography showed a dilated right coronary artery with a fistulous communication to the right atrium, tortuous coronary veins draining directly into the cardiac chambers, and the absence of the coronary sinus. Embryologically, regression failure of the right venous valve is hypothesized to have created both the divided right atrium and extensive coronary vein abnormalities.

Results of bowel plication in addition to primary anastomosis in patients with jejunal atresia.

BACKGROUND/PURPOSE: Disturbed intestinal transit (DIT) associated with bowel dilation occurs in some postoperative patients with jejunal atresia. Bowel plication (BP) has been introduced to prevent the DIT, but the long-term results of BP are unclear. METHODS: The authors reviewed the preoperative and operative records and postoperative clinical courses (for 2.3 to 7.0 years; mean period, 3.7 years) of 19 jejunal atresia patients, 4 of whom had undergone additional BP with primary anastomosis after dilated bowel resection or tapering jejunoplasty at neonatal surgery. The degree of DIT was evaluated by the clinical symptoms, weight gain, whether reoperation was performed, and duration from the operation to receiving an oral feeding volume of at least 130 mL/kg/d. The patients then were assigned 4 grades (0 to 3). To determine the presence or absence of bowel dilation at the BP site, the plain abdominal x-rays were reviewed. RESULTS: (1) Within 2 months after surgery, 4 patients without BP underwent operation because of severe DIT. The degree of postoperative DIT in patients who had received additional BP at neonatal surgery was less than that in patients without BP (mean grade, 0.50 v. 2.08). (2) The preoperative clinical features, operative method, and postoperative weight gain were almost similar in patients with and without BP. (3) On abdominal x-ray the bowel dilation remained 6 to 12 months after the operation, but was not observed over 1 year after the operation. CONCLUSIONS: In this preliminary study, the addition of BP after tapering jejunoplasty or resection of dilated bowel may be effective in preventing early postoperative DIT. Further study is necessary to evaluate the long-term results of additional BP at neonatal surgery. J Pediatr Surg 36:1752-1756.

Plasmapheresis in fulminant acute disseminated encephalomyelitis.

We report an 11-year-old girl with acute disseminated encephalomyelitis (ADEM) who developed respiratory failure and coma despite the use of corticosteroid and intravenous immunoglobulin. We performed plasmapheresis four times, which improved her level of consciousness, hyperesthesia, external ophthalmoplegia and muscle weakness, and led to the normalization of brain and spinal cord MRI. Plasmapheresis might be an effective treatment in cases of fulminant ADEM.

Intestinal motility in an in vivo rat model of intestinal ischemia-reperfusion with special reference to the effects of nitric oxide on the motility changes.

BACKGROUND: To clarify the relation between intestinal ischemia-reperfusion (IR) and dysmotility, the authors investigated changes in the motility pattern in the duodenum and jejunum in an in vivo rat model of IR when artery- (and vein-) fed jejunum was clamped transiently. The authors also studied the effect of nitric oxide on the motility changes in this model by means of the administration of L-NAME (N(G)-nitro-L-arginine methyl ester) or S-methylisothiourea sulfate (SMT). MATERIALS AND METHODS: A force transducer was sutured onto the serosal side of the duodenum or jejunum. After a 3-to 4-day recovery period, contractions were recorded during periods of preischemia, ischemia (60 minutes), and reperfusion (90 minutes). An intestinal IR was produced by clamping and releasing the mesenteric artery and vein with artery forceps. RESULTS: In the jejunum, there was a prolongation in the duration of contraction and there were decreases in the number of contractions (NC) during the IR. When treated with L-NAME, no decrease in the NC was observed during the 45 to 90 minutes after reperfusion. S-methylisothiourea sulfate did not affect the IR-induced motility changes significantly. In the duodenum, there was a prolongation in the duration of contraction and a decrease in the NC and AC only during the reperfusion. L-NAME or S-methylisothiourea sulfate inhibited the decreases in the NC during the reperfusion. CONCLUSIONS: Intestinal IR causes motility changes in the ischemic site during the IR and in the nonischemic site during the reperfusion. The IR-induced motility changes partly depend on nitric oxide production.

Amatoxin poisoning from ingestion of Japanese Galerina mushrooms.

BACKGROUND: Although some Japanese Galerina species poisonings manifest as gastrointestinal symptoms followed by late-onset hepatorenal failure (phalloides syndrome), the toxin responsible for this has not been determined. CASE REPORT: We report a 6-year-old boy who developed characteristic cholera-like diarrhea and late-onset severe hepatic deterioration after eating mushrooms, later identified as a Galerina species, most likely Galerina fasciculata. A residual mushroom revealed alpha-amanitin. This account is the first known reported case of poisoning by Japanese Galerina species where an amatoxin was demonstrated to be responsible for the toxicity.

Developmental changes in cyclooxygenase mRNA expression in the kidney of rats.

Prostaglandins, synthesized by cyclooxygenase (COX), regulate renal hemodynamics and also epithelial water and solute transport. To determine whether COX mRNA expression changes with age, we studied expression in renal medulla and in cortex in developing rats at various ages. We also examined age-related changes in COX mRNA expression induced by lipopolysaccharide (LPS). COX mRNA was quantitatively analyzed in a real-time polymerase chain reaction (PCR) with dual-labeled fluorogenic probes. COX-1 mRNA expression did not change with age in cortex or medulla. COX-2 mRNA expression was highest in 1-week-old rats and lowest in 4- and 8-week-old rats. Lipopolysaccharide treatment did not alter COX-1 mRNA expression in infantile or adult rats. In adults, LPS at 1, 5, and 10 mg/kg induced COX-2 mRNA expression in renal medulla; the higher doses, 5 and 10 mg/kg, induced COX-2 expression in cortex. In infantile rats, COX-2 mRNA, already high in the unmanipulated state, was further increased by only 1 mg/kg LPS in both renal cortex and medulla. Age-related changes in the expression of COX-2 mRNA might be responsible for changing physiologic characteristics of renal function during postnatal development in rats, and may be important in renal cortical development.

Possible clinical and histologic manifestations of adult-onset type II citrullinemia in early infancy.

We describe 2 patients with adult-onset type II citrullinemia who developed transient hypoproteinemia and jaundice in early infancy. Liver histology showed a marked fatty change and fibrosis. After the patients had lived without symptoms to the ages of 5 and 16 years, respectively, the diagnosis was made by genetic analysis.

Effect of vincristine on gastric motility in conscious rats.

We investigated the possible involvement of the upper alimentary tract in vincristine (VCR)-induced dysmotility. Gastric contractions were recorded by a strain-gauge force transducer in conscious rats. Rats were injected with various doses of VCR followed by continuous recording for 12 hr. Additionally, 3-hr recordings to study the later effects were performed one and three days after injection. Gastric motility was dose-dependently increased by VCR. Post- versus preinjection motility index (MI; area under contraction waves) ratios were 0.78+/-0.12 for saline and 1.95+/-0.21 for VCR at 0.75 mg/kg. This increase in MI was completely inhibited by atropine and hexamethonium. MI one day after injection of VCR (0.75 mg/kg) was significantly less than in control (0.32+/-0.10 vs 0.92+/-0.24, respectively). The decrease was reversed by acetylcholine. In conclusion, VCR first increased gastric motility, and this was followed by a gradual decrease in conscious rats. These alterations may involve a presynaptic cholinergic pathway.

Neonatal thrombocytosis resulting from the maternal use of non-narcotic antischizophrenic drugs during pregnancy.

Neonatal thrombocytosis can result from maternal narcotic drug abuse. The case of a male infant is reported who was born to a woman with schizophrenia treated with non-narcotic psychotropic drugs during pregnancy; he developed severe prolonged thrombocytosis. The platelet count reached 1310 x 10(9)/l on day 15. This thrombocytosis persisted for three months. The patient was treated with dipyridamole. A bone marrow aspirate showed normal myeloid and erythroid precursors with an increased number of megakaryocytes. Plasma concentrations of interleukin 6 and thrombopoietin were suppressed. No obvious complications from the thrombocytosis occurred, and the platelet count fell to within the upper limit of normal after 3 months of age. This case indicates that thrombocytosis may occur in infants born to mothers treated with non-narcotic psychopharmaceutical drugs during pregnancy. The thrombocytosis in this case may have been induced by factors other than interleukin 6 or thrombopoietin.

Living-related liver transplantation for type II citrullinemia using a graft from heterozygote donor.

BACKGROUND: Type II citrullinemia (CTLN2) characterized by a liver-specific argininosuccinate synthetase deficiency is an adult onset genetical disorder caused by the mutation of SLC25A13 gene, which results in fulminant hyperammonemia often with poor prognosis. METHODS: A 16-year-old Japanese boy presented fulminant hyperammonemia and encephalopathy and recovered after aggressive medical treatment. The patient was diagnosed as CTLN2 by plasma amino acid pattern and detection of the mutated SLC25A13 gene. We performed living-related liver transplantation (LRLT) using a graft from the genetically proven heterozygote father. RESULTS: Serum amino acid concentration was normalized within a day after transplantation without protein restriction and medication. The patient's postoperative course was natural. The patient is back in school 6 months after surgery. CONCLUSIONS: Living-related liver transplantation using a graft from genetically proven heterozygote donors might be a permissible treatment modality for CTLN2. Long-term observation may be necessary to make a definite conclusion possible.

Effects of bathing immediately after birth on early neonatal adaptation and morbidity: a prospective randomized comparative study.

OBJECTIVE: Because the risks and benefits of early bathing of newborn infants are not well established, we investigated the effects of bathing immediately after birth on rectal temperature, respiratory rate, heart rate, blood pressure, percutaneous arterial blood oxygen saturation (SpO2) and early neonatal morbidity. METHODS: The study was designed as a randomized prospective comparative study in the neonatal care unit of a university hospital. A total of 187 healthy term and near-term newborn infants, who were delivered vaginally without asphyxia, between January and December 1997 were the study subjects. We compared findings in newborns who were bathed 2-5 min after birth (n = 95) with those of a control group (n = 92) who received dry care instead. Groups were comparable with respect to gestational age, birthweight, male: female ratio, Apgar score and umbilical blood pH. Rectal temperature was measured with an electronic thermometer immediately before the intervention bathing or dry care and at 30 min and 1, 2, 3, 8 and 12 h after birth. Heart rate, respiratory rate, systolic and diastolic blood pressure and SpO2 were measured at 1, 2, 8 and 12 h after birth. The incidence of early neonatal morbidity, including hyperbilirubinemia and gastrointestinal and respiratory problems, was also compared. RESULTS: Rectal temperature changed over time postnatally in both groups (P < 0.0001, ANOVA) and there was a significant difference in rectal temperature between groups (P< 0.0001, ANOVA). Mean (+/- SEM) rectal temperature at 30 min after birth (i.e. approximately within 20 min after intervention) was significantly higher in the bathed group than in the control (dry care) group (37.30 +/- 0.06 is 37.00 +/- 0.05 degrees C, respectively; P = 0.000022). Respiratory rate, heart rate, blood pressure and the ratio of the number of infants with SpO2 90-94% and 95-100% did not differ significantly between the two groups. The incidence of early neonatal morbidity, including vomiting, acute gastric mucosal lesion, polycythemia, need for tube feeding, phototherapy and oxygen therapy, also did not differ between the two groups. CONCLUSIONS: Early bathing, minutes after birth, did not appear to adversely affect the adaptation of healthy full-term and near-term newborn infants.

Doppler echocardiographic evaluation of right ventricular diastolic function in children.

Right ventricular diastolic function was evaluated by flow velocity pattern in the right ventricular inflow tract by means of pulsed Doppler echocardiography. Traditionally used to evaluate this function are peak velocities obtained during early diastole (peak E wave) and during atrial contraction (peak A wave), their ratio (peak E/A ratio), and the deceleration half-time. We conducted pulsed Doppler echocardiographic studies of right ventricular inflow and outflow patterns in 171 children (105 normal children and 66 children who were undergoing total surgical repair of congenital heart defects without sequelae). Results showed that summation flow was present in the right ventricular inflow tract in 43 (25%) of the 171 subjects, which made it difficult to separate the peak E wave from the peak A wave. We noted the presence of antegrade late diastolic flow (DW) in the right ventricular outflow tract of all subjects. DW, measured in 121 subjects in whom both E and A waves were detected in the right ventricular inflow tract, showed a highly significant correlation (p < 0.0001) with A waves in the right ventricular inflow tract. The ratio of DW to right ventricular outflow tract velocities during systole (SW) showed a highly significant (p < 0.0001) correlation with E/A ratio. When evaluating right ventricular diastolic function by pulsed Doppler, especially in children, the analysis of right ventricular outflow tract patterns is helpful in addition to that of inflow tract patterns. The DW and DW/SW ratio may present good alternatives to traditional parameters in children.

Y-27632 inhibits gastric motility in conscious rats.

Y-27632, a highly selective inhibitor of p160ROCK, desensitizes the smooth muscle to Ca2+ and inhibits smooth muscle contraction. While this drug has the potential to become a novel drug for hypertension, it might also affect other smooth muscle, including that of gastrointestinal tract. We studied the effects of Y-27632 on gastric contractions in conscious rats. Strain gauge force transducers were sutured onto the serosal side of the gastric antrum and contractions were recorded before and after the intravenous injection of Y-27632. Doses of 1.0 mg/kg to 10 mg/kg significantly decreased contraction amplitude and the motility index in a dose dependent manner. With 10 mg/kg, the mean amplitude was decreased by up to 69 +/- 14% and the motility index by up to 81 +/- 7%. The change occurred immediately after drug infusion and lasted for 3.5h. Contraction frequency showed only a slight decrease. No signs of bowel obstruction were observed. These results indicate that Rho-mediated Ca sensitization has a role in the physiologic contractions of gastric smooth muscle in rats. Y-27632 is useful to investigate the physiology of gastrointestinal motility.