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Colorimetric properties of fluorescent materials depend on the SPD of the illumination. That is why most standards for evaluating them specify the illuminations, which are often hard-to-realize daylight illuminants. The presented method using commercially available LEDs enables accurate enough colorimetric measurements of FWA-treated papers or prints on them illuminated by the specified illuminant. The total spectral radiance factor of a fluorescent specimen, from which most colorimetric values are derived, consists of the luminescent spectral radiance factor and the spectral reflectance factor. This method separately estimates those of FWA-treated paper to add up to the total spectral radiance factor. The luminescent spectral radiance factor is obtained by estimating the SPD of luminescence excited by the specified illuminant as the weighted sum of the multiple SPDs of luminescence excited by the respective narrow band LED emissions at different wavelengths. The LEDs and their weights are determined optimally for generally used papers. The spectral reflectance factor is derived from the estimated SPD of the radiation with fluorescence excluded from the paper illuminated by visible illumination. The method was applied with five different illumination systems each using two or three narrow band LEDs in the excitation range. They were evaluated by measuring the total spectral radiance factors by D50 of seven FWA-treated papers and CMYK prints on four papers. The derived colorimetric values were compared to the respective references by the ideal D50.

Numerical corrections of colorimetric errors caused by positioning error and irregular geometry in multiangle measurements of gonio-apparent coatings are presented. These corrections use a model function approximating the gonio-reflection characteristics of the coating to convert the angular displacement due to those geometric errors to reflectance factor errors to be corrected as well as to estimate the angular displacement conversely from the reflectance factors. The performance of these corrections was evaluated for 11 different gonio-apparent coatings using a multiangle geometry conforming to ASTM E2539 consisting of eight subgeometries to which artificial displacements were applied so as to present typical positioning errors or irregular geometries. The results show that both corrections significantly reduce the colorimetric errors for all specimens in all subgeometries sensitive to geometric errors. © 2015 Wiley Periodicals, Inc. Col Res Appl, 2015

The virtual fluorescent standard (VFS) method is a new approximation method for the practical measurement of the colorimetric properties of an object treated with fluorescent whitening agent (FWA). The essential requirement of the VFS method is that the bi-spectral characteristics of the VFS must be similar in curve shape to those of the object to be measured. In the case of an object printed on an FWA-treated substrate, the bi-spectral characteristics will vary depending on not only the substrate but also the printed ink films. In this study, two simplified VFS methods, one using the bi-spectral characteristics of the substrate and the other using those of typical paper as the VFS instead of those similar to each object, were evaluated. The evaluation was performed using two instrument models for the VFS method with the different illuminations with five sets of 13 samples printed in different colors by five different printer/paper combinations. In this evaluation, the total spectral radiance factor of each sample was obtained by simulated measurement, that is, it was calculated based on the bi-spectral radiance factor of the sample and the spectral power distributions of the light source of the instrument. The total spectral radiance factor of each sample under D50 obtained using both VFS models and CIE L*a*b* values derived therefrom were compared with those by the reference model with ideal D50 illumination. Although the samples are limited, the results shows that both simplified VFS methods remarkably reduce the errors due to fluorescence when compared to the conventional method. © 2011 Wiley Periodicals, Inc. Col Res Appl, 2012

The recently introduced virtual fluorescent standard (VFS) method for measuring an optical property of a sample treated by fluorescent whitening agent (FWA) was applied to a printed sample on FWA-treated paper. The primary difficulty of this application is that the bi-spectral luminescent radiance factor of the paper substrate is specifically modified by the ink films printed and each sample has the specific bi-spectral luminescent radiance factor. In this study, the difficulty is handled by two different, orthodox and abridged, approaches. In the orthodox approach, the effective bi-spectral luminescent radiance factor of a printed sample is estimated based on the effective spectral transmittance, which is also estimated, of the ink films printed. In the abridged approach, the effective spectral transmittance of the ink films is considered to be spectrally neutral and accordingly the effective bi-spectral luminescent radiance factor of the printed sample is regarded as relatively identical or close to that of the paper. The performances of the VFS method by both approaches were examined with 28 samples printed by a laser printer with 7 primaries Y, M, C, K, R, G, B at 25, 50, 75, 100% dot areas on FWA-treated paper. The results show that the performances of both approaches are remarkable at least for those samples and the abridged approach has the performance close to that of the orthodox approach for most of the samples. © 2007 Wiley Periodicals, Inc. Col Res Appl, 32, 449–462, 2007

In this article, a new method for measuring a total spectral radiance factor of a FWA-treated sample illuminated by a specific standard illuminant is introduced. The method replaces an unstable real fluorescent standard by a bi-spectral luminescent radiance factor data, which works as a virtual fluorescent standard (VFS) by knowing spectral intensity distributions of illuminations applied to the sample. The method utilizes two illuminations I1 and I2 whose relative spectral intensity distributions are different from each other and synthesizes a virtual illumination presenting the identical fluorescent spectral radiance factor to that presented by the standard illuminant with the VFS of the specific bi-spectral luminescent radiance factor by linearly combining I1 and I2 with the suitable weighting factors. The applicability of the method is examined in principle by comparing ISO brightness and CIE whiteness index of fluorescent standard paper as a test sample obtained by this new method to the assigned values. © 2007 Wiley Periodicals, Inc. Col Res Appl, 32, 195–200, 2007

A subset of patients with esophageal atresia (EA) have an associated short esophagus and require an antireflux procedure. The Collis-Nissen procedure, which consists of a combination of Collis gastroplasty and Nissen fundoplication, is considered an option in such conditions. The long-term results of EA patients undergoing this procedure have rarely been reported. The results of the Collis-Nissen procedure were examined in four EA patients with follow-up for an average of 9 years. The Collis-Nissen procedure was conducted concurrently with segmental esophageal resection for an associated congenital esophageal stenosis close to the esophagogastric junction in two postoperative Gross type C patients (7 months, 2 years), as an antireflux operation in a postoperative Gross type A patient with an unreducible sliding hiatal hernia (10 years), and with primary repair of EA in a Gross type A patient with a long gap (4 months). There were no significant complications except minor anastomotic leakage and an anastomotic stenosis that required postoperative dilation. Significant reflux was negative, as shown by an upper gastrointestinal study and scintigraphy. Limited esophagitis just above the esophagogastric junction was observed in a Gross type A patient with a long mediastinal gastric segment. Swallow-related pressure reduction at the wrapping cuff was detected by manometric examination. The Collis-Nissen procedure is a useful option for treating EA patients who require esophageal lengthening and control of gastroesophageal reflux.

Aims: To assess the efficacy of a new regimen of intravenous atropine treatment for infantile hypertrophic pyloric stenosis (IHPS) with special reference to regression of pyloric hypertrophy. Methods: Atropine was given intravenously at a dose of 0.01 mg/kg six times a day before feeding in 19 patients with IHPS diagnosed from radiographic and ultrasonographic findings. When vomiting ceased and the infants were able to ingest 150 ml/kg/day formula after stepwise increases in feeding volume, they were given 0.02 mg/kg atropine six times a day orally and the dose was decreased stepwise. Results: Of the 19 infants, 17 (89%) ceased projectile vomiting after treatment with intravenous (median seven days) and subsequent oral (median 44 days) atropine administration. The remaining two infants required surgery. No significant complications were encountered. Ultrasonography showed a significant (p < 0.05) decrease in pyloric muscle thickness, but no significant shortening of the pyloric canal after completion of the atropine treatment. The patients exhibited failure to thrive at presentation, but were thriving at 6 months of age (p < 0.01). Conclusions: This atropine therapy resulted in satisfactory clinical recovery. Pyloric muscle thickness was significantly reduced.

Background/Purpose: Periodic clusters of phasic pressure waves in the gastroduodenal junction (GDJ) have been seen in patients with infantile hypertrophic pyloric stenosis (IHPS). This study investigated the details of these pressure waves in relation to disturbed transpyloric flow in IHPS. Methods: Manometric study was performed in 11 IHPS patients before and after atropine therapy and 2 non-IHPS infants. Pressure changes in the GDJ were measured with an 8-channel sleeve or a 9-channel sidehole micromanometric assembly under fluoroscopic control for 2 hours. Results: Clusters of phasic pressure waves (365 ± 42 mm Hg) associated with an increase in basal pressure (10 ± 3 mm Hg) were intermittently observed in the GDJ in all IHPS patients. Similar observations were not made in the non-IHPS infants. Most antral pressure waves occurred simultaneously with those pressure waves in the GDJ in the IHPS patients. Atropine (0.01 mg/kg) transiently abolished the phasic and tonic pressure waves for 19 ± 10 minutes. Significantly fewer phasic pressure waves were observed after atropine therapy. Conclusions: Characteristic phasic and tonic contractile activity in the GDJ is uncoordinated with the antral contractions in IHPS patients. Such incoordination may be an important factor in the disturbed transpyloric flow in IHPS. J Pediatr Surg 36:1641-1645. Copyright © 2001 by W.B. Saunders Company.

PURPOSE The purpose of this prospective consecutive patient study was to test if perioperative respiratory system compliance of patients undergoing surgical repair of congenital diaphragmatic hernia (CDH) can predict the need for prolonged postoperative mechanical ventilation. METHODS All neonates over 35 weeks of gestation who had surgical repair of left-sided CDH between July 1994 and December 1996 ( n = 10) were included in this study. Static respiratory system compliance (C(rs)) was measured by the passive occlusion method with muscle relaxation before (C(pre)) and after (C(post)) surgical repair of left-sided CDH. We examined the relationship between respiratory system compliance and postoperative ventilator-dependent duration. RESULTS The mean birthweight-corrected C(post) was lower than that of birthweight-corrected C(pre) (0.41 +/- 0.18 vs 0.54 +/- 0.18 ml.cm H(2)O(-1).kg(-1), P = 0.04). One neonate died on postoperative day 31 from intraventricular hemorrhage, and the other nine neonates survived. The patient who died was excluded from the rest of our calculations. The ventilator-dependent duration was 16.7 +/- 12.3 days. The postoperative ventilator-dependent duration was longer when the birthweight-corrected C(post) was smaller, with a significant correlation between these parameters ( P = 0.006). CONCLUSION C(post), but not C(pre), may be useful to predict the need for prolonged postoperative mechanical ventilation in neonates with left-sided CDH.

Background/Purpose: The risks of homologous transfusion and the effectiveness of predeposit autologous transfusion have been described. The authors examined the clinical usefulness of cord-blood harvesting for autologous transfusion in newborns who had congenital anomalies antenatally diagnosed that would require surgical intervention at or near the time of delivery. Methods: Of 112 cases of antenatal diagnosis of congenital anomalies, 50 mothers gave informed consent and enrolled in this study. Cord-blood was withdrawn immediately after clamping of the umbilical cord and was used for autologous transfusion in newborns within the first 3 days postpartum. Results: A mean of 72 ± 54 mL of cord-blood was harvested (27 ± 18 mL/kg). While preserving cord-blood for 3 days at 4°C, no signs of clot formation or hemolysis were observed. The harvested cord-blood included plasma-free Hb ranging from 1 to 68 (13 ± 18) mg/dL and thrombin-antithrombin III complex ranging from 2 to 273 (18 ± 50) ng/mL. Bacteriologic examination of the stored cord-blood showed negative cultures, except for samples from 3 newborns after vaginal delivery. A mean of 46 ± 34 mL of cord-blood was used in 26 patients for autologous transfusion. No significant complications related to cord-blood transfusion were recognized clinically. Conclusions: Autologous cord-blood transfusion has the potential to be a useful alternative to homologous transfusion in newborns requiring surgery. Adequate collection and storage techniques for cord-blood must be developed. J Pediatr Surg 36:851-854. Copyright © 2001 by W.B. Saunders Company.

Sixty-eight neonates with functional ileus were reviewed. Twelve required laparotomy; in seven, histological studies revealed decreased ganglia and ganglion cells of the myenteric plexus (MP) (Group A), and in five, MP was normal (Group B). In the remaining 56 cases, obstructive symptoms were relieved following conservative therapy (Group C). All Group A cases except one had normal birth weight, while Group B and C cases showed significantly lower birth weights. A marked caliber change of the small intestine and/or small-caliber distal intestine with meconium stagnation in the proximal intestine was commonly demonstrated at operation in Group A and B, or on contrast enema in Group C. Four Group A cases died of enteritis, and three survivors suffered from prolonged obstructive symptoms. The grade of histological abnormality of MP correlated with the clinical outcome. In Group B, three died of sepsis shortly after surgery, but two survivors have been free from symptoms. Group A can be categorized as Hirschsprung's disease-allied disorders (HAD). Group B and C can be categorized as meconium-related ileus (MRI). The similarity of the macroscopic findings of HAD and MRI, and the occurrence of MRI exclusively in low birth weight neonates, strongly suggest that functional immaturity of MP plays a role in the etiology of MRI.

We examined the clinical significance of the lung-thorax transverse-area ratio (L/T) in fetuses with cystic lung disease (CLD). The transverse section of the thorax was analyzed at the level of the 4-chamber view of the heart. L/T was calculated as the bilateral normal lung area divided by the thoracic area. Within a 9-year period, ultrasonography was performed in 15 fetuses with CLD, 13 of which were histologically diagnosed: congenital cystic adenomatoid malformation of the lung (CAM; n = 8), pulmonary sequestration (n = 4), and bronchial atresia (n = 1). Although the initial L/T measurement (29.6 +/- 4.2 weeks) showed significantly lower values than in the controls, the final measurement (36.4 +/- 1. 6 weeks) indicated that the L/T in CLD excluding CAM was significantly higher than that in CAM. Six patients with a final L/T of less than 0.21 required mechanical ventilation immediately after birth. Two of these patients died, and the autopsy findings indicated pulmonary hypoplasia. In these cases, a significant correlation was observed between the maximal value of L/T and preductal alveolar-arterial oxygen difference (A-aDO(2)), although the final L/T was not correlated to A-aDO(2). These results indicate that serial measurements of L/T may be useful in the diagnosis of CLD and may help to predict postnatal respiratory conditions.

Laparoscopic resection was performed successfully in four children with left-sided adrenal tumors. Three of the cases were mass screening-positive neuroblastomas with early stages, and the other was ganglioneuroma. With the transabdominal approach, those tumors were completely resected and retrieved after isolation. Our technique was feasible and safe for the left-sided adrenal tumors ≤50 mm. Patients with noninvasive adrenal tumors, including screening-positive neuroblastomas, are potential candidates for this technique.

There are no published data of manometric studies of pyloric motor function in patients with infantile hypertropic pyloric stenosis (IHPS). The present study attempted to examine the characteristics of motor abnormality of the pylorus in five children with IHPS. Using a transducer-built-in manometric catheter cannulated through the pylorus under fluoroscopy, the pressure in the pyloric canal was recorded continuously over 3 h during fasting. Clusters of high-amplitude spastic contractions of over 300 mmHg were recorded at intervals. The frequency was 1-3/min (mean 1.7 cpm) and the duration was 7-15 s. These periodic spastic contractions were suppressed temporarily for 20-30 min after intravenous injection of 0.01 mg/kg atropine. After pyloromyotomy, these spastic contractions decreased remarkably in amplitude, but there were no changes in frequency. It is concluded that the underlying motor abnormality observed in hypertrophied pyloric muscle is clusters of high-amplitude contractions, although more precise measurements of basal pyloric pressure are needed to explore the pathophysiology of IHPS in detail. The effect of pyloromyotomy may be related to the decrease in high-amplitude contractions.

Background: The prognosis of hepatoblastoma is poor unless the tumor is completely resected. Various types of chemotherapy have been developed to increase its resectability. Recently, transcatheter arterial chemoembolization (TACE) has been developed for the treatment of unresectable adult hepatoma with favorable results. The authors applied this procedure to hepatoblastoma in infants and children. Methods: TACE was performed in eight hepatoblastoma cases. After an intraarterial catheter was inserted into the main feeding artery of the tumor, injection of adriamycin or THP-adriamycin (20 to 30 mg/m 2 ) dispersed in lipiodol and cisplatin (40 to 60 mg/m 2 ) followed by embolization using Gelfoam pieces was performed. Effects of TACE were evaluated according to shrinkage of tumor mass on imaging examinations, alpha-fetoprotein (AFP) levels, and pathological findings of the surgical specimens 4 weeks after TACE. Results: A marked reduction in tumor size associated with a decrease in AFP level occurred 1 month after the treatment. Tumor shirinkage ranged from 0.9% to 45.0% with a mean value of 25.8%. AFP levels decreased by 0.2% to 11.9% with a mean level of 4.6% from initial levels. In addition, there was no marked chemotherapeutic agent-induced toxicity noted during the observation period. Resection of the tumors was performed safely after TACE in all cases. Pathological examination showed massive necrosis in the surgical specimens, and the mean percentage of necrotic area within the tumor was 71.1%. Two patients died of extensive lung metastasis 2 months and 3 years after the operation, respectively. The remaining six were doing well and free of disease at a mean follow-up period of 50 months. Conclusion: TACE is an effective, safe, and useful method for the initial treatment of hepatoblastoma.

As with energy requirements, protein requirements are relatively much greater in infants and decline progressively with age. Amino acid metabolism in pediatric patients is characterized by the following differences. The requirement for essential amino acids in neonates is larger than that in adults. Because of low activity of phenylalanine hydroxylase and cystathionase, hyperphenylalaninemia and hypermethioninemia tend to occur, whereas tyrosine and cysteine tend to be deficient. In addition to cysteine and tyrosine, histidine, lysine, arginine and taurine are considered as semiessential amino acids. Nowadays there are different kinds of amino acid formulas to satisfy these specific requirements, and most of these formulas are intended to normalize the plasma aminogram. However, the nutritional benefit of these formulas for growth and development is still not completely proven, and the pharmacological use for specific diseases is expected with some modification of these formulas.

The outcome of fetuses with congenital diaphragmatic hernia (CDH) has been reported to be fatal when pulmonary hypoplasia (PH) is severe. As an indicator of PH, we attempted to measure the lung-thorax transverse area ratio (L/T) using ultrasonic echography. Immediate postnatal surgery was performed using high-frequency oscillatory ventilation (HFOV) and sometimes followed by extracorporeal membrane oxygenation (ECMO). Eighteen fetuses were treated and 14 survived. L/T correlated well with the best preductal arterial blood gas data before surgical reduction during manual ventilation and HFOV, while preductal PO2 and alveolar-arterial oxygen differences from patients managed with HFOV were better than those in patients with manual ventilation. Although L/T also correlated with the duration of O2 therapy and hospitalization in survivors without major anomalies, there was no significant difference between L/T in survivors and nonsurvivors. Because delayed institution of ECMO and complications related to ECMO management seemed to be a major cause of death in non-survivors, the unsalvageable L/T due to PH was estimated to be below 0.06 for HFOV and below 0.1 for conventional ventilation based on the correlation between L/T and preductal P02. These results suggest that L/T is a useful indicator of PH in patients with CDH and also that HFOV is advantageous in treating CDH with PH. The advantage of prenatal diagnosis in predicting unsalvageable L/Ts, should be considered in the therapeutic strategy.

Although respiratory management with high-frequency oscillatory ventilation (HFOV) has generally been used for neonates with congenital diaphragmatic hernia (CDH), entry criteria for extracorporeal membrane oxygenation (ECMO) based on data from patients who underwent HFOV have not yet been reported. To establish entry criteria for ECMO in such patients, we retrospectively studied 36 neonates with CDH treated by HFOV in our institutions between 1986 and 1994. From the admission records, preductal and postductal arterial blood gas data and HFOV ventilation conditions for 72 h after admission were extracted. Oxygenation index (01) and alveolar-arterial oxygen gradient (A-aD02) time interval combinations were calculated. Patients were divided into two groups: candidates for ECMO (n = 22) who underwent ECMO (n = 18) or died without ECMO (n = 4); and non-candidates (n = 14), who survived without ECMO. Blood gas data in patients placed on ECMO were comparable to those in patients who died without ECMO: mean pre- and postductal OI for 4 h > 30, postductal A-aD02 ≥620 mmHg for 4 h, postductal A-aD02 ≥580 mmHg for 8 h, and postductal A-aD02 ?550 mmHg for 12 h showed better sensitivity with a specificity of more than 90% compared to entry criteria that had previously been used in our institutions: a postductal OI >40 for 4 h and postductal A-aDO2 ≥610 mmHg for 8 h. In addition, a combination of preand postductal OI >30 for 4 h indicated a sensitivity of 95.5% and a specificity of 92.9%.

Spontaneous regression occurs in some cases of neuroblastoma, especially stage IVS. The incidence of neuroblastoma has been reported to be increasing since the mass screening program was introduced in Japan. This would indicate that the screening is detecting regressing tumors. However, the mechanism of regression is still unknown. To evaluate the hypothesis that the regression might be related to apoptosis, the authors examined apoptosis by in situ end-labeling of fragmented DNA and expression of the apoptosis-suppressing protein bcl -2. Materials and Methods: One hundred eighteen neuroblastoma cases were available for examination. Eighty (67.8%) were detected by the mass screening program. Serial sections were cut from paraffin-embedded tumors. A modified TdT-mediated dUTP nick end-labeling (TUNEL) method was performed to detect apoptosis. Immunohistochemical analysis was performed to detect bcl -2 expression. Results: In cases under 1 year of age or with a favorable clinical stage, the incidence of apoptosis was significantly high. Expression of bcl -2 was associated with N- myc amplification and unfavorable histology (Shimada classification). Tumors in patients under 1 year of age often had areas where cellularity was markedly decreased, and apoptosis was often observed while bcl -2 expression was reduced. In such cases, there was a negative correlation between occurrence of apoptosis and bcl -2 expression. This suggests that apoptosis may be related to spontaneous regression in neuroblastoma.

To examine associated anatomic anomalies of the tracheobronchial tree and structural abnormalities of the trachea, and their clinical consequences, bronchoscopic examination and follow-up studies were performed in 32 patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) over a 5-year period. To evaluate structural abnormalities of trachea, the ratio of circumferential length of cartilaginous trachea to circumferential length of membranous trachea (C:M ratio) was determined before surgical repair. In patients examined before and after surgical repair, the motion of the posterior tracheal wall was observed under spontaneous respiration. Fifteen of the 32 patients (47%) had abnormalities of the tracheobronchial tree such as ectopic right upper bronchus (12), congenital bronchial stenosis (4), and absence of the right upper bronchus (2). The C:M ratio of the 27 patients with EA (2.0 +/- 0.6) was significantly lower than that of controls (3.0 +/- 0.3); the ratio for the 10 cases with ectopic right upper bronchus (1.7 +/- 0.4) was significantly lower than that of other cases (2.2 +/- 0.5). The C:M ratio for the 12 patients who had atelectasis (1.7 +/- 0.3) was significantly lower than that of the 15 who did not have atelectasis (2.2 +/- 0.6). Of 24 infants, nine had bulging of the posterior tracheal wall synchronized with expiration, and their C:M ratio (1.8 +/- 0.2) was significantly lower than that of the 15 patients without bulging (2.2 +/- 0.6). These data show an association between anatomic anomalies of the tracheobronchial tree and structural abnormalities of the trachea in patients with EA. Because these abnormalities may be related to the occurrence of respiratory disorders such as tracheomalacia and atelectasis, early bronchoscopic examination, including measurement of the C:M ratio and observation of the motion of the posterior tracheal wall under spontaneous respiration, may be useful in the care of patients with EA.

To determine whether associated anatomic features may be useful in predicting fetal prognosis, 43 fetuses who had ultrasonographic evaluation for abdominal wall defects during a 13-year period were reviewed. Thirty-one fetuses had omphalocele and were classified into three groups according to fetal ultrasonography results: ruptured omphalocele with exposed liver (7), giant omphalocele with exposed liver (12), and small omphalocele without liver herniation (12). Twelve fetuses had gastroschisis. Ten of the 12 fetuses with gastroschisis survived; one died in utero. Nine of the 12 with a small omphalocele survived. Ten of 12 fetuses with giant omphalocele survived, but six of the seven with ruptured omphalocele died of pulmonary hypoplasia and respiratory insufficiency. Ruptured omphalocele recognized in utero was accompanied by intrauterine growth retardation and liver herniation and frequently was associated with deformity of the spine, diaphragmatic defects, vesicointestinal fissure, and meningocele. These results indicated that, together with the deformity of the spine, rupture and absence of the covering membrane with an exposed liver (noted through fetal ultrasonography) may suggest a poor prognosis because of pulmonary hypoplasia.

As a possible prognostic index, plasma levels of amino acids were serially measured for 1 week in 22 neonates with perforative peritonitis including gastric rupture and necrotizing enterocolitis and 28 neonatal surgical patients without infection as a control, who were subjected to total parenteral nutrition with hypertonic glucose and amino acids formulated for neonates. Plasma levels of branched chain amino acids significantly increased on the first day after operation in peritonitis group, whereas plasma levels of phenylalanine and histidine increased on the third postoperative day and tyrosine increased seventh postoperative day. In the peritonitis group, 5 neonates died within 2 weeks after operation (early death group) because of cardiopulmonary failure and 5 neonates died with hyperbilirubinemia from 1 month to 6 months after operation (late death group). The early death group showed increased levels of total amino acids compared with those in the late death group and survivors. The late death group also showed higher levels of total amino acids than survivors. Plasma levels of tyrosine increased in the early and late death groups compared with survivors. These results indicated that serial measurements of plasma amino acids early after operation showed the existence of liver dysfunction in addition to the derangement of amino acid metabolism in the liver and muscle caused by septic insult, which predicted the prognosis of perforative peritonitis in neonates.

We mapped the (1,1), (2,2), and (3,3) lines of NH3 toward the molecular cloud associated with the Monkey Head Nebula (MHN) with a 1. � 6 angular resolution using a Kashima 34 m telescope operated by the National Institute of Information and Communications Technology (NICT). The kinetic temperature of the molecular gas is 15–30 K in the eastern part and 30–50 K in the western part. The warmer gas is confined to a small region close to the compact Hii region S252A. The cooler gas is extended over the cloud even near the extended Hii region, the MHN. We made radio continuum observations at 8.4 GHz using the Yamaguchi 32 m radio telescope. The resultant map shows no significant extension from the Hα image. This means that the molecular cloud is less affected by the MHN, suggesting that the molecular cloud did not form by the expanding shock of the MHN. Although the spatial distribution of the Wide-field Infrared Survey Explorer and Two Micron All Sky Survey point sources suggests that triggered low- and intermediate-mass star formation took place locally around S252A, but the exciting star associated with it should be formed spontaneously in the molecular cloud.

In order to evaluate liver function in postoperative biliary atresia (BA) patients, the indocyanine green (ICG) clearance test was performed. Nineteen patients, ranging in age from 2 to 9 years, were classified into three groups. Group I were those who had no jaundice throughout the postoperative period (n = 9), Group II were those who had recurrent or intermittent jaundice (n = 7), and Group III were those who had persistent jaundice after hepatic portojejunostomy (n = 3). Forty-four volunteers of comparative age and sex served as controls to establish a normal range of ICG-K values in infants and children. The normal minimal ICG-K value in those 2-9 years of age was defined as 0.19. ICG-K values in BA patients were 0.262 +/- 0.073 (Group I), 0.150 +/- 0.060 (Group II), and 0.054 +/- 0.011 (Group III). All nine patients with ICG-K below the normal level had esophageal varices, and seven of nine had a history of gastrointestinal hemorrhage. Two patients with ICG-K values within the normal range had esophageal varices, and none had a history of gastrointestinal hemorrhage. Five of nine patients with ICG-K values below normal had low serum albumin levels, and all had low prealbumin levels. Of those with ICG-K within the normal range, none had low serum albumin or prealbumin levels. On the other hand, serum bilirubin and other liver function tests did not accurately reflect clinical findings. In conclusion, the ICG test using the ICG-K value was confirmed to be a reliable indicator of postoperative liver function in BA patients.

To assess the severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarian section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 ± 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCO 2 , preductal A-aDO 2 before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in L/T between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia.

Cystatins are protein inhibitors of cysteine proteinases, which are believed to play an important role in the pathogenesis of periodontal disease. In this study, we report a new sensitive method for the quantitative analysis of cystatin activity in a small amount of crude sample such as gingival crevicular fluid. Cystatin activity in the crude sample was determined by using active site-titrated papain, which is a cysteine proteinase from the plant Carica papaya. Crude samples usually contain endogenous cysteine proteinases. These competed with the added papain for the active sites of the cystatins. The cystatin-cysteine proteinase complex was able to be dissociated by the addition of papain. This competition and dissociation could interfere with the determination of cystatin activity, since some of the cysteine proteinases, such as cathepsin B, hydrolyzed the specific substrate for papain during titration with the papain. In order to exclude this interference and measure total cystatin activity, the crude sample must be alkalinized (pH 11.0) for 5 min at 4 degrees C followed by 10 min at 40 degrees C before titration with papain. The minimum detectable amount of cystatins was 20 fmol/assay when it was calculated per mole of papain inhibitory sites. Using this method, significant levels of cystatin activity were detected in all the samples of gingival crevicular fluid taken from periodontal disease patients. These results suggest that cystatins could regulate the cysteine proteinases in gingival crevicular fluid and that this new method could be useful to clarify the role of cystatins in the pathogenesis of periodontal disease.

The outcome of fetuses with diaphragmatic hernia (CDH) has been reported to be related to the severity of lung hypoplasia. As an index of pulmonary hypoplasia, we attempted to measure the lung-thorax transverse area ratio (L/T) using ultrasonic echography in eight fetuses with left-sided CDH. Two cases with L/T more than 0.28 (controls: 0.52±0.04) were transported postnatally and recovered after early operation without episodes of persistent fetal circulation. Elective surgical repair was performed in six infants immediately after cesarean delivery at 35–37 weeks' gestation. In three cases with L/T between 0.21 and 0.24 who recovered with no complications, surgical reduction of the abdominal organs improved arterial blood gases and high-frequency oscillation ventilation (HFOV) was fully effective for respiratory management. In three with L/T between 0.11 and 0.17, extracorporeal membrane oxygenation (ECMO) was required from the 1st to the 12th postoperative day despite HFOV. Although two infants died of combined cardiovascular anomalies and airway bleeding caused by prolonged HFOV, respectively, one infant with minimal L/T survived. Measurement of L/T may help to predict the outcome of fetuses with CDH and to determine the indications for various treatments including immediate operation after cesarean delivery, HFOV, and ECMO.

To clarify the relationship between clinical features in utero and postnatal prognosis, 20 fetuses who underwent ultrasonic (US) evaluation for meconium peritonitis (MP) over a 17-year period were reviewed. According to final US findings in utero, patients were classified into three types. Type I (massive meconium ascites) was noted is 5 cases, type II (giant pseudocyst) in 4, and the other 11 were classified as type III (calcification and/or small pseudocyst). Abdominal calcifications were identified in only 5 cases (2 type I, 1 type II, 2 type III). Seven fetuses who had associated polyhydramnios (1 Type I, 1 Type II, 2 Type III) and fetal hydrops (3 Type II) were delivered before 36 weeks' gestation. Cardiopulmonary resuscitation at birth was required in 9 cases (5 type I, 4 type II) who underwent abdominal drainage before delivery and/or immediately after birth. Although dilatation of the intestine was identified in 10 fetuses (2 type II, 8 Type III), 18 had intestinal atresia and 2 had fecal obstruction of the distal ileum. Four infants (2 type I, 1 type II, 1 type III) died of respiratory failure and postoperative complications. These results indicated that careful fetal US may be useful for perinatal management of MP.

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.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.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.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.

The purpose of this study was to evaluate whether the ambulatory level premorbid or at discharge reflected the survival rate better. Ambulatory level was retrospectively evaluated as a postoperative indicator of survival rate following operative treatment in 301 consecutive patients over 65 years old. All of the patients were followed up for a mean of 62 months. The postoperative ambulatory level at discharge reliably reflected the survival rate in the elderly after hip fracture, better than the premorbid ambulatory level. It is confirmed that the planning of the operation and rehabilitation to maintain the premorbid ambulatory level after hip fracture might be critical.

A male infant with congenital cardiac anomalies and esophageal atresia with tracheoesophageal fistula (EA-TEF) showed intractable respiratory symptoms after delayed primary repair of EA-TEF. Computed tomography demonstrated that the trachea was compressed by the enlarged aorta. Artificial ventilation was necessary even after aortopexy performed at 2 months of age. At 140 days of age, an expandable metallic stent (Palmaz stent) was inserted through a rigid bronchoscope into the trachea under fluoroscopic control. His respiratory status improved dramatically, and he was extubated in 18 hours. Although the follow-up period has been 9 months, the short-term result is satisfactory. The expandable metallic stent placement should be considered in patients with EA-TEF who show intractable respiratory symptoms caused by tracheomalacia.

Background/Purpose: Hirschsprung's disease (HD, HSCR) is one of the most common diseases in the field of pediatric surgery. It is well known that the aganglionic bowel is primarily a causative factor of dismotility of distal narrow segment. Recent studies have shown that mutations in endothelin-B receptor (EDNRB), endothelin-3, RET , glial cell line-derived neurotrophic factor (GDNF) genes are responsible for the occurrence of congenital aganglionosis. Here, the authors describe two new mutations of the EDNRB gene in Japanese patients with HD. Results: One patient had a heterozygous point mutation at the splice donor site of intron 3, leading to premature termination of translation of EDNRB mRNA. Another patient has a heterozygous missense mutation (N1041) in exon 1, but the same mutation was found in two of 50 normal individuals, so the mutation may be a noncausative polymorphism of the EDNRB gene. Conclusion: These results provide further evidence that a spectrum of different mutations within the EDNRB gene are responsible for HD.

The authors present a pair of dizygotic twins with congenital esophageal atresia with tracheoesophageal fistula who underwent successful single-stage surgical repair. To our knowledge, this is the second set of dizygotic twins with this congenital anomaly in the literature.

In our multi-epoch observation of the star-forming region NGC 2264 with the VLBI Exploration of Radio Astrometry, we detected two water maser features. We have measured the annual parallax of the maser sources for the first time in NGC 2264 and derived 1.356 ± 0.098 mas, corresponding to the distance of pc, which is consistent with its previously reported photometric observations. One of the maser features is located near a dust continuum core, CMM4S, positionally corresponding to the X-ray source FMS2-1269 listed in Flaccomio et al. Re-analyzing Chandra X-ray data, we found that FMS2-1269 is still surrounded by a dense envelope of a H2 column density, 5.4 × 1023 cm–2. We suggest that the maser detected near the CMM4S core is most likely associated with the X-ray-emitting Class 0 candidate FMS2-1269. The other maser feature is found to emerge from a high-velocity outflow of 150 km s–1 and is associated with the 3.6 cm radio continuum source NGC 2264 VLA 3 located close to IRS1.

Background Recent studies have indicated that mass screening for neuroblastoma detects tumors that otherwise would have regressed spontaneously without recognition. Therefore, we started an observation program for these patients to determine how frequently spontaneous regression occurs. Procedure Eighteen patients were detected by mass screening between June 1994 and December 1996. Eight of these cases matched the following criteria and entered the observation program: Stage I or II, less than 5 cm in diameter; no involvement of large vessels or organs; not difficult to resect; informed consent. If there were an increase in tumor size, an elevation of tumor markers, or evidence of metastasis, the tumor would be immediately resected. Results Five of the eight cases showed spontaneous regression. Although the remaining three tumors were resected 6–10 months after diagnosis, all patients survived without evidence of recurrence. Conclusions At least 60% of neuroblastoma cases who entered our observation program regressed spontaneously. Med. Pediatr. Oncol. 36:160–162, 2001. © 2001 Wiley-Liss, Inc.

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. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: Sacrococcygeal teratomas (SCT) diagnosed in utero have been reported to be large and associated with high perinatal mortality rate. However, operative management including timing of operation after birth, combined abdominal approach for devascularization, and the position of the patients during resection is not well established. Methods: A retrospective review of 14 patients with SCT between 1978 and 1999 was performed. To prevent massive bleeding during surgery, the authors used an abdominoperineal resection in the supine position after devascularization. The patients' clinical and sonographic characteristics, prenatal outcome, operative management, and postnatal outcomes were examined. Results: One fetus died in utero. Two patients died within a week, but no late death and no malignant degeneration were noted. A staged operation with devascularization was performed in 2 patients, and 1 death occurred. Surgical management was analyzed between survivors without massive bleeding at surgery (n = 9) and others (n = 4). A significant difference was observed in the subgroup of tumor resection with devascularization or supine position and that of early resection with devascularization or supine position. Conclusions: Early resection using the abdominoperineal approach supported by close antenatal sonography may be preferable for a favorable outcome. Resection in the supine position after devascularization may have advantages of respiratory management, cardiac resuscitation, and bleeding prevention. J Pediatr Surg 36:545-548. Copyright © 2001 by W.B. Saunders Company.

A developing child, with a high metabolic rate and low body stores of nutrients, is susceptible to metabolic disturbances due to surgical stress. For perioperative nutritional care of infants, the distinct physiologic features of their body fluids must be carefully considered. The postoperative metabolic response is the same as that in adults, but in children this response is more rapid. Based on the urinary excretion of 3-methylhistidine in infants, the postoperative degradation of muscle protein is thought to be twice the preoperative level. This transient increase during the early postoperative period was not suppressed by increased amino acid intake, and energy intake was sufficient. To prevent postoperative metabolic complications, the energy intake of glucose or fat should correspond to the patient—s requirements, and excess carbohydrate should be avoided. The quantity and quality of the amino acid formula used must be carefully evaluated.

Background. Congenital esophageal stenosis (CES) has been reported to be a rare association in patients with esophageal atresia or tracheoesophageal fistula, or both (EA-TEF). This study investigates the clinical characteristics of this association with special reference to its treatment. Methods. A retrospective review of medical and radiographic records of 81 patients who underwent primary repair of EA-TEF was performed. An association of CES was diagnosed when they showed histologic evidence or persistent radiographic images of esophageal narrowing since the neonatal period. Results. Eleven of 81 EA-TEF patients (14%) were identified with CES. Their symptoms were difficulty in swallowing solid food, food impaction, emesis, stridor, repeated respiratory infection, and failure to thrive. Two patients had a recurrence of TEF. Three patients were diagnosed with CES in the neonatal period—2 at the time of primary repair of EA-TEF and 1 on the initial postoperative esophagram. The remaining 8 patients were diagnosed between the ages of 2 months and 3 years. Although esophageal dilatation was attempted in 9 patients, its effectiveness was temporary in all except 2 patients and esophageal laceration occurred in 4 patients. Surgical repair including myotomy and resection of the narrow segment was performed in 7 patients, in 6 of whom Nissen/Collis-Nissen fundoplication was added. A histologic examination was performed in 5 patients, revealing that their CES was due to fibro-muscular hypertrophy (2) or tracheobronchial remnants (3). The long-term outcome was satisfactory in all patients except one who showed wrap herniation. Conclusions. The association between CES and EA-TEF is not rare. Esophageal dilatation was not universally effective and carried with it a considerable incidence of esophageal leakage. An antireflux operation concomitant with repair of CES may be useful to prevent postoperative gastroesophageal reflux in patients with a narrowing close to the esophagogastric junction. (Surgery 2001;129:29-38.)

To define the clinical role of laparoscopic Nissen fundoplication (LNF) in children with gastroesophageal reflux (GER), an appropriate understanding of its functional effects is required. The aim of this study was to investigate the motor function of the esophageal body and the lower esophageal sphincter (LES) with special reference to the effects of caloric nutrients in children undergoing LNF.Studies were performed in 12 children with GER (age, 6 months to 13 years) before and a month after LNF. Continuous manometric examination was performed with an infusion system using a sleeve sensor for an hour each before and after the administration of apple juice (AAJ; 10 mL/kg).AAJ increased postoperative basal LES pressure from 15 +/- 7 to 20 +/- 6 mm Hg, whereas it decreased the preoperative values from 13 +/- 5 to 10 +/- 4 mm Hg (P.05). Significant residual pressure was noted at the nadir of swallow-induced LES relaxation after LNF, which was increased by AAJ from 7 +/- 3 to 11 +/- 4 mm Hg. A child with a high nadir LES pressure showed postoperative dysphagia. Significant changes in the patterns of esophageal contractions were not noted after LNF.Characteristics of the effect of LNF on the LES were a postprandial increase of basal LES pressure and significant residual LES pressure at the nadir of LES relaxation. The motor function of the esophageal body was not affected by LNF.

The aim of this study was to clarify whether serum hyaluronic acid level (SHA) can reflect the degree of liver fibrosis in biliary atresia (BA).SHA was measured in 44 postoperative BA patients at 7 months to 22 years of age, with sandwich enzyme method (Hy-A 100 kit). SHA was compared with T.Bil (group 1, T Bil2; group 2, 2or = T Bil5; group 3, T Bilor = 5 mg/dL), fibrosis score (0-6, the number of abnormal values among Alb, PT, ChE, T Chol, Fischer's ratio, prealbumin), and histologic grading (0-IV).SHA was 499.8 +/- 332.5 in group 3, significantly higher than in the control, group 1, or group 2. As fibrosis score rose, SHA became higher, and SHA in Score 6 (430.1 +/- 366.1 ng/mL) and score-5 (172.9 +/- 141.8 ng/mL) was significantly higher than in the control and other scores, respectively. As the histologic grade rose, SHA became higher, and SHA in grade IV (444.8 +/- 323.5 ng/mL) and grade III (166.0 +/- 70.3 ng/mL) was significantly higher than in the control or other Grades. Serial change of SHA since before HPE was parallel to the clinical course in 8 patients.SHA may be a useful serum marker reflecting the degree of liver fibrosis in BA.

The authors report on a child with tracheal agenesis who survived for 6 years and 10 months. A female infant was born with respiratory distress and an absence of audible cry. With mask ventilation, gastrostomy and division of the esophagus was performed just after birth. She was well ventilated with an endotracheal tube inserted through the cervical esophagostomy after thoracic esophageal banding. An accidental extubation caused her anoxic encephalopathy at 9 months of age. She had been alive without artificial ventilatory support until she died of acute esophageal bleeding.

Simpson-Golabi-Behmel syndrome (SGBS) is one of the overgrowth syndromes. Microdeletions of the glypican-3 (GPC3) gene were described by Pilia et al. (1996). Glypican-3 encodes a putative extracellular proteoglycan which is expressed in embryonic mesodermal tissues and plays an important role in embryonal growth. We report a Japanese patient with SGBS who had a single base deletion in the exon 7 of the GPC3 gene. This is the first report of a single base deletion of the GPC3 gene.

Although delayed repair with preoperative stabilization using high-frequency oscillatory ventilation (HFOV) has been advocated in neonates with congenital diaphragmatic hernia (CDH), improved survival has not been reported. We compared survival between neonates undergoing delayed repair after prolonged stabilization for more than 48 h using HFOV and those undergoing immediate repair following short stabilization using HFOV in subjects with high-risk CDH. Patient selection was made by two criteria: fetuses with the lung/thorax transverse area ratio (L/T) below 0.26 and neonates who presented with respiratory distress significant enough to require intubation within 6 h after delivery. Selection of the strategies differed by era and medical center. Stabilization failed in 11 of 18 patients with delayed repair and only 7 patients (38.9%) in this group survived, but 18 of 23 patients (78.3%) in the immediate-repair group survived (P0.05). Although lower values of Apgar scores, best postductal PO2 (BPtDPO2), gestational weeks, and L/T and more frequent patch repair were observed in the delayed than in the immediate repair group, significant differences in survival among the subpopulations were also observed in the prenatally diagnosed group, including the group with L/T0.15 and the group that required ECMO, the group with the best preductal PO2 (BPrDPO2)/= 100 torr, and the group with BPtDPO2100 torr. These results indicate that prolonged stabilization for more than 48 h using HFOV does not improve survival in patients with high-risk CDH.