Heparin-binding epidermal growth factor-like growth factor (HB-EGF) protected intestinal ischemia-reperfusion injury through JNK and p38/MAPK-dependent pathway for anti-apoptosis.

BACKGROUND: Heparin-Binding Epidermal Growth Factor-Like Growth Factor (HB-EGF) is a potent cytoprotective factor in various body systems, including gastrointestinal tract. In this study, we intended to examine whether HB-EGF exerts its protective effects through MAPK dependent anti-apoptosis after intestinal I/R injury. METHODS: We randomly divided 30 laboratory 30 rats into 5 groups: (A) normal control group, (B) ischemia group with normal saline, (C) I/R group with normal saline, (D) ischemia group with HB-EGF (400 ug/kg), and (E) I/R group with HB-EGF (400 ug/kg). With Western blotting study, we determined JNK and p38/MAPK pathway and caspase-3 activity protein levels using Western analyses. RESULTS: The JNK phosphorylation protein levels increased after intestinal ischemia or intestinal reperfusion phase, and HB-EGF pre-treatment was significantly decreased in JNK phosphorylation protein levels (p < 0.01). We found that p38 protein levels was increased after intestinal reperfusion phase, and that HB-EGF pre-treatment significantly decreased p38 protein levels (p < 0.01). The expression protein level of caspase 3 was increased after intestinal ischemia or intestinal reperfusion phase. HB-EGF pre-treatment significantly decreased Caspase 3 proteins. (p < 0.01). CONCLUSION: Our study revealed that pre-treatment of HB-EGF decreased the amount of activity of JNK and p38/MAPK pathway and caspase-3 protein after intestinal I/R injury. These results may further support that the cytoprotective of HB-EGF after I/R injury could be through anti-apoptotic effect of activity of JNK and p38/MAPK pathway.

Trends in diagnostic approaches for pediatric appendicitis: nationwide population-based study.

BACKGROUND: To define the benefits of different methods for diagnosis of pediatric appendicitis in Taiwan, a nationwide cohort study was used for analysis. METHODS: We identified 44,529 patients under 18 years old who had been hospitalized with a diagnosis of acute appendicitis between 2003 and 2012. We analyzed the percentages of cases in which ultrasound (US) and/or computed tomography (CT) were performed and non-perforated and perforated appendicitis were diagnosed for each year. Multivariate logistic regression analyses were performed to evaluate risk factors for perforated appendicitis. RESULTS: There were more cases of non-perforated appendicitis (N = 32,491) than perforated appendicitis (N = 12,038). The rate of non-perforated cases decreased from 0.068% in 2003 to 0.049% in 2012; perforated cases remained relatively stable at 0.024%~0.023% from 2003 to 2012. The percentage of CT evaluation increased from 3% in 2003 to 20% in 2012; the rates of US or both US and CT evaluations were similar annually. The percentage of neither CT nor US evaluation gradually decreased from 97% in 2003, to 79% in 2012. The odds ratios of a perforated appendix for those patients diagnosed by US, CT, or both US and CT were 1.227 (95% confidence interval (CI) 0.91, 1.65; p = 0.173), 2.744 (95% CI 2.55, 2.95; p < 0.001), and 5.062 (95% CI = 3.14, 8.17; p < 0.001), respectively, compared to patients who did not receive US or CT. The odd ratios of a perforated appendix for those patients 7-12 and ≤6 years old were 1.756 (95% CI 1.67, 1.84; p < 0.001) and 3.094 (95% CI 2.87, 3.34; p < 0.001), respectively, compared to those 13-18 years old. CONCLUSIONS: Our study demonstrated that using CT scan as a diagnostic tool for acute appendicitis increased annually; most patients especially those ≤6 years old who received CT evaluation had a greater risk of having perforated appendicitis. We recommend a prompt appendectomy in those pediatric patients with typical clinical symptoms and physical findings for non-complicated appendicitis to avoid the risk of appendiceal perforation.

Therapeutic effectiveness of percutaneous drainage and factors for performing an interval appendectomy in pediatric appendiceal abscess.

BACKGROUND: In this study, we studied the therapeutic effectiveness of percutaneous drainage with antibiotics and the need for an interval appendectomy for treating appendiceal abscess in children with a research-oriented dataset released by the Bureau of National Health Insurance in Taiwan through the Collaboration Center for Health Information Application (CCHIA). METHODS: We identified 1225 patients under 18 years of age who had non-surgical treatment for an appendiceal abscess between 2007 and 2012 in a Taiwan CCHIA dataset. The treatment included percutaneous drainage with antibiotics or antibiotics alone. We also analyzed data of patient's baseline characteristics, outcomes of percutaneous drainage, and indicating factors for performing an interval appendectomy. RESULTS: Totally, 6190 children had an appendiceal abscess, an 1225 patients received non-operative treatment. Of 1225 patients, 150 patients received treatment with percutaneous drainage and antibiotics, 78 had recurrent appendicitis, 185 went on to receive an interval appendectomy, and 10 had postoperative complications after the interval appendectomy. We found that patients treated with percutaneous drainage and antibiotics had a significantly lower rate of recurrent appendicitis (p < 0.05), a significantly smaller chance of receiving an interval appendectomy (p < 0.05), and significantly fewer postoperative complications after the interval appendectomy (p < 0.05) than those without percutaneous drainage treatment. Older children (13 ~ 18 years) patients were found to have a significantly smaller need to receive an interval appendectomy than those who were ≤ 6 years of age (odd ratio (OR) = 2.071, 95 % confidence interval (CI) = 1.34-3.19, p < 0.01), and those who were 7 ~ 12 years old (OR = 1.662, 95 % CI = 1.15-2.41, p < 0.01). In addition, those treated with percutaneous drainage were significantly less indicated to receive an interval appendectomy later (OR = 2.249, 95 % CI = 1.19 ~ 4.26, p < 0.05). In addition, those with recurrent appendicitis had a significantly increased incidence of receiving an interval appendectomy later (OR = 3.231, 95 % CI = 1.95 ~ 5.35, p < 0.001). CONCLUSIONS: In this study, we used nationwide data to demonstrate therapeutic effectiveness of percutaneous drainage and antibiotics was more beneficial than only antibiotics in treating patients with an appendiceal abscess. We also found three factors that were significantly associated with receiving an interval appendectomy: recurrent appendicitis, being aged ≤ 13 years, and treatment with antibiotics only.

Calcitonin Gene-Related Peptide Downregulates Expression of Inducible Nitride Oxide Synthase and Caspase-3 after Intestinal Ischemia-Reperfusion Injury in Rats.

BACKGROUND: Various investigations have demonstrated that calcitonin gene-related peptide (CGRP) plays an important role in mediating ischemic preconditioning. CGRP has been shown to mimic the protective effects of ischemic preconditioning and mitigate ischemia-reperfusion (I/R) injury in the heart, brain, gastrointestinal system, and other tissues. This study aimed to examine whether CGRP, a proven intestinal cytoprotective molecule, exerted its protective effects through modulation of inducible nitride oxide synthase (iNOS) and apoptosis after intestinal I/R injury. METHODS: This animal study randomly divided 30 rats into the following five groups: (1) the normal control group, (2) the ischemia group with normal saline, (3) the I/R group with normal saline, (4) the ischemia group with CGRP (300 µg/kg), and (5) the I/R group with CGRP (300 µg/kg). Levels of iNOS messenger RNA (mRNA) and protein, and caspase-3 protein were determined by real-time quantitative polymerase chain reaction and Western blotting analyses, respectively. Statistical analysis was performed using analysis of variance with Dunn test. RESULTS: The mRNA levels of iNOS increased after the intestinal ischemia or intestinal reperfusion phase (p < 0.01), and CGRP pretreatment significantly decreased iNOS mRNAs and protein levels (p < 0.01). The expression protein levels of caspase-3 increased after the intestinal ischemia or intestinal reperfusion phase. CGRP pretreatment significantly decreased the levels of caspase-3 proteins. CGRP intestinal cytoprotection is mediated, in part, by downregulation of expression of iNOS and caspase-3 after intestinal I/R injury. CONCLUSION: The study indicates that the cytoprotective role of CGRP (i.e., antiapoptotic effect) after I/R injury could be via downregulation of iNOS, which may relieve I/R tissue damage by blocking iNOS activity.

National trends in therapeutic approaches and outcomes for pediatric appendicitis: a Taiwanese nationwide cohort study.

PURPOSE: To define the pattern of therapeutic approaches for pediatric appendicitis and compare their benefits in Taiwan, we analyzed a research-oriented dataset released by the Bureau of National Health Insurance in Taiwan through the Collaboration Center for Health Information Application (CCHIA) to document the impact of the rise of laparoscopic treatment on outcomes. METHODS: We identified 22,161 patients under 18 years who had been hospitalized with a diagnosis of acute appendicitis between 2007 and 2012 in the CCHIA. Statistical comparisons between the Laparoscopic appendectomy (LA) and open appendectomy (OA, control) groups were computed using a Chi squared test. The odds ratios (ORs) and 95% confidence intervals (CIs) of risk factors for intra-abdominal abscess (IAA) and postoperative bowel obstruction (PBO) were derived from multivariate logistic regression models. RESULTS: In each respective year, the incidence of LA increased from 29.17% in 2007 to 57.4% in 2012, while that of OA decreased from 70.83% in 2007 to 42.60% in 2012; incidences of non-perforated appendicitis and perforated appendicitis with LA or OA seemed similar. The length of hospitalization between an LA and OA for non-perforated appendicitis was the same, but that with an LA was shorter for perforated appendicitis. The adjusted ORs for IAA and PBO for those patients with perforated and non-perforated appendicitis were 6.30 (95% CI = 5.09-7.78; p < 0.001) and 6.49 (95% CI = 4.45-9.48; p < 0.001); while for those cases undergoing an LA and OA, they were 0.50 (95 % CI = 0.40-0.62; p < 0.001) and 2.07 (95% CI = 1.45-2.95; p < 0.001), respectively. The ORs of IAA and PBO for those patients ≤6 and 7-12 years of age were 1.67 (95% CI = 1.23-2.25; p = 0.001) and 1.20 (95% CI = 0.97-1.49; p = 0.095), and 1.88 (95% CI = 1.08-3.24; p = 0.025) and 1.47 (95% CI = 1.01-2.14; p = 0.043), respectively, compared to those aged 13-18 years. CONCLUSIONS: Our study demonstrated that young age and perforated appendicitis can affect postoperative IAA and PBO. LA appeared beneficial in reducing the length of hospitalization and postoperative IAA, but had an increasing risk of PBO. Although laparoscopic approach for pediatric appendectomy is increasing in our country, the different hospital levels and pediatric surgeon's laparoscopic experience must be evaluated in further study.

Genome-wide gene expression profiling of ischemia-reperfusion injury in rat kidney, intestine and skeletal muscle implicate a common involvement of MAPK signaling pathway.

The mechanisms of ischemia­reperfusion (I/R) injury have not been fully elucidated to date. In order to determine the genetic involvement across different organs during I/R injury, a DNA microarray approach was used to analyze the gene expression profiles of the kidney, intestine, and skeletal muscle in a rat model of I/R injury. Fifteen male Lewis rats were divided randomly into three different organ groups; a sham operation (control group), 60­min­ischemia (Is group) only, and 60­min­ischemia plus 60­min­reperfusion (I/R group), respectively. The target genes were identified by DNA microarray and studied by quantitative polymerase chain reaction (qPCR). By comparing the I/R group with the control group, a 2­fold upregulation of 467, 172, and 3932 and a 2­fold downregulation of 437, 416, and 4203 genes were identified in the kidney, small intestine, and skeletal muscle, respectively. Several commonly upregulated genes associated with mitogen­activated protein kinase (MAPK) pathways, including Jun, Atf3, junB, Fos, Adm and Dusp 1, were differentially expressed in the I/R group. The mRNA expression levels of the target genes were confirmed by qPCR. The present study hypothesized that the MAPK pathway may function in a common pathway of I/R injury and regulate the pathogenesis through activator protein 1. The findings of the present study contributed to the understanding of the molecular pathways associated with I/R injury.

Preoperative lip measurement in patients with complete unilateral cleft lip/palate and its comparison with norms.

PURPOSE: There is prominent lip asymmetry in patients with unilateral complete cleft lip and palate. Measurement of the lip on cleft and non-cleft sides provides appraisal of the lip deformity and information for planning of surgical correction. The purpose of this retrospective study is to evaluate the degree of lip deformity and to compare it with normative data. MATERIALS AND METHODS: From 1983 to 1997, data from a total of 168 patients with unilateral complete cleft lip and palate were collected. There were no other associated craniofacial anomalies in this patient group. The measurement was performed under general anaesthesia by a senior surgeon using a calliper prior to the first lip repair. Corresponding normative data were collected from 2002 to 2003 on 50 patients who had normal facial appearance prior to hernia repair. The measurements included lip height, lip width, philtrum length and vermilion thickness. Comparisons were made between the cleft side and the non-cleft side, as well as between cleft patients and norms. RESULTS: Comparisons between the cleft and the non-cleft sides revealed significantly longer lip on the non-cleft side, including lip height from alar base to Cupid's bow, lip width from Cupid's bow to commissure and the vermilion thickness. The lip measurements on the norms were longer than those on the cleft side of the lip, but were similar to the non-cleft side. CONCLUSION: A wide variety of tissue growth asymmetry is observed between the non-cleft and the cleft sides, indicating a deficiency of tissue development associated with the cleft deformity. These data can provide a fundamental basis for presurgical orthopaedic treatment, surgical planning, execution of surgery, postoperative assessment and may help to predict treatment outcome.

Risk factors for intestinal gangrene in children with small-bowel volvulus.

OBJECTIVES: Pediatric small-bowel volvulus (SBV) is a surgical emergency, and early diagnosis is difficult. We analyzed the clinical manifestations, imaging findings, and laboratory parameters in children with SBV and attempted to determine the risk factors for bowel gangrene. PATIENTS AND METHODS: Forty-nine children (35 boys and 14 girls) with SBV who were admitted to the hospital for a period of 13 years were enrolled. Clinical and laboratory parameters and evaluation measures included fever, abdominal pain, vomiting, bloody stool, peritoneal signs, severe dehydration, disease duration, white blood cell counts, sugar, C-reactive protein (CRP), sodium, potassium, metabolic acidosis, blood urea nitrogen, and creatinine. These parameters were statistically compared between patients with and without bowel gangrene. RESULTS: Thirty-six patients (73.5%) were 5 years old or younger, and nearly half were younger than 1 year old. Abdominal pain and vomiting were 2 major symptoms. Malrotation was the most common cause of SBV. In univariate analysis, nonbilious vomiting, peritoneal signs, severe dehydration, leukocytosis (WBC count >18,000 cells/mm3), elevated CRP (>50 mg/dL), and hyponatremia (<130 mmol/L) were significantly associated with bowel gangrene (P < 0.05). In multivariate analysis, nonbilious vomiting, leukocytosis, and elevated CRP were significantly (P < 0.05) associated with bowel gangrene. The resection rate for bowel gangrene was 44.9%, and no mortality was found. Seven (14.3%) patients had postoperative complications, including short-bowel syndrome (n = 2), adhesion ileus (n = 3), and intraabdominal abscess (n = 3). Seven experienced failure to thrive in later follow-up. CONCLUSIONS: Specific clinical manifestations and laboratory parameters are helpful in the identification of bowel gangrene in children with SBV.

Elimination of postoperative pyloric stricture by endoscopic electrocauterization and balloon dilatation in an infant with congenital antral web.

We, herein, report a male infant who presented with recurrent pyloric stricture after two surgeries (web excision and antropyloroplasty), which were done, respectively, at 5 days of age for congenital antral web and 6 months of age for the subsequent pyloric stricture. The patient suffered from anorexia, progressed vomiting, and weight loss gradually after the first and second surgeries, and then, endoscopy revealed severe pyloric deformity and stricture. Poor inflation was noted during endoscopic balloon dilatation because of tight pylorus; a subsequent electrocauterization and balloon dilatation were done, and the patient's clinical symptoms improved significantly 2 weeks later. A follow-up endoscopy was performed 1 month and 12 months after endoscopic therapy, showing steady regression of pyloric stricture. The patient had adequate diet intake and growth in the later 12 months.

Inguinal hernia containing uterus and uterine adnexa in female infants: report of two cases.

We herein report two female cases, aged 1 and 1.5 months, of inguinal sliding hernias containing the uterus, fallopian tube, and ovary. The diagnosis of inguinal hernia with uterus and uterine adnexa was highly suspected preoperatively by ultrasonography and was confirmed during surgical correction. Freeing the attachment of fallopian tube and uterus from the sac and with reduction of the uterus, ovary, and fallopian tube back to the peritoneal cavity, high ligation of the hernia sac was performed in these cases. In conclusion, the hernia sac containing fallopian tube, ovary, and uterus in the female is very rare. We present our experience of treatment with these rare cases and suggest that sonography be performed routinely in female infants with an inguinal hernia containing a palpable movable mass.

Heparin-binding epidermal growth factor-like growth factor downregulates expression of activator protein-1 transcription factor after intestinal ischemia-reperfusion injury.

The pathogenesis of necrotizing enterocolitis (NEC) is unknown. Ischemia and reperfusion (I/R) injury have been considered to be major contributing factors. More recent reports have noted that apoptosis is a significant and perhaps the principal contributor to cell death after I/R injury. Recent studies have revealed that activator protein 1 (AP-1) family proteins including c-Fos and c-Jun potentially induce either the proliferation or apoptosis of the cells in the brain, heart, kidney, and liver. c-Fos and c-Jun expression has also been reported to be upregulated in postischemic intestinal epithelial cells (IECs). Heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) is a potent cytoprotective factor in various pathologic conditions and plays a pivotal role in mediating the earliest cellular responses to injury. This study aims to examine whether HB-EGF, a proven intestinal cytoprotective molecule, exerts its protective effects through modulation of AP-1 transcription factor after intestinal I/R injury. Thirty rats were randomly divided into the following 5 groups: (1) normal control group; (2) ischemia group; (3) I/R group; (4) ischemia group with HB-EGF (400 µg/kg), and (5) I/R group with HG-EGF (400 µg/kg). c-Fos and c-Jun messenger RNAs and protein levels were determined by real-time quantitative polymerase chain reaction (PCR) and Western analyses, respectively. Statistical analysis was performed using ANOVA with Dunn's test. The messenger RNA levels of the c-Fos and c-Jun increased after intestinal ischemia or the intestinal reperfusion phase. HB-EGF pretreatment significantly decreased c-Fos and c-Jun messenger RNAs. The expression of protein levels of c-Fos and c-Jun were correlation with the expression of messenger RNA level. HB-EGF intestinal cytoprotection is mediated, in part, by downregulation of the expression of AP-1 transcription factor after intestinal I/R injury.

Duodenal derotation and extent tapering jejunoplasty as primary repair for neonates with high jejunal atresia.

BACKGROUND: The dilated bowel segment usually involves the entire jejunum, as well as the duodenum in patients with high jejunal atresia. Classical approaches with a limited tapering enteroplasty and anastomosis frequently fail to restore intestinal function. A novel technique using duodenal derotation and extent tapering jejunoplasty (DDETJ) is reported here. METHODS: Infants with high jejunal atresia within 10 cm of the ligament of Treitz treated with DDETJ over a 3-year period were reviewed. The entire dilated duodenum and jejunum were visualized after duodenal derotation. A longitudinal extensive tapering resection of dilated bowel with Endo GIA stapler was performed to fashion as a tube and anastomosed to the distal jejunum. RESULTS: Five female infants underwent this procedure over a 3-year period. Four infants underwent primary DDETJ, while one with associated multiple atresias had previously undergone limited tapering jejunoplasty, but was unable to tolerate oral feeding. DDETJ was performed 4 weeks later. There were no postoperative complications, and all patients tolerated feeding within 14 days. Follow-up lasted from 2 to 18 months. CONCLUSION: In very proximal high atresia, the extent of tapering is limited by the proximity of the ligament of Treitz. Duodenal derotation provides better access to the high atresia. The results of this limited experience suggest that the DDETJ procedure could provide an alternative therapy in patients with high jejunal atresia.

Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome.

OBJECTIVE: There are no available data for outcomes in children's idiopathic superior mesenteric artery syndrome (SMAS) strictly treated conservatively. The aim of the study was to evaluate clinical and nutritional outcome in children with idiopathic SMAS. PATIENTS AND METHODS: A 1-year prospective observation study of effects of treatment and outcome was performed in 27 children (8 boys, 19 girls) with idiopathic SMAS who underwent an upper gastrointestinal (UGI) series, ultrasound measurement of the aortomesenteric angle, treatment, clinical assessment, growth evaluation, and regular clinical visits for more than 12 months. RESULTS: Mean age of the patients was 11.77 +/- 2.15 years. The major clinical complaints were postprandial pain or fullness (88.9%), vomiting (55.6%), and early satiety (51.9%). Eight patients (29.6%) had weight loss. The UGI series revealed typical features of SMAS. The aortomesenteric angle on ultrasound was 10 degrees to 19 degrees. The height of most patients (92.6%) was above the 10th percentile, whereas 15 (55.6%) patients weighed below the 10th percentile. Six patients underwent surgical intervention (3 for obstruction and 3 for persistent anorexia with weight loss), and their clinical symptoms and weight status improved steadily during the follow-up months. Among the 21 patients not subject to surgical intervention, 11 (52.4%) experienced a reduction of symptoms >50% after 3 months of treatment, and weight-for-age percentile increased significantly after 6 months of treatment. Overall, a significant increase in the weight-for-age status was seen in the patients with surgical treatment or with medication only after 6 and 12 months of treatment. CONCLUSIONS: An aortomesenteric angle <20 degrees is a constant phenomenon in children with idiopathic SMAS. A duodenojejunostomy can effectively relieve the obstructive symptoms, such as anorexia, and improve nutritional status, whereas long-term medical treatment may aid in relieving the clinical symptoms, promoting appetite, and improving nutritional status in pediatric patients with idiopathic SMAS.

Ovarian tumors in the pediatric age group: 37 cases treated over an 8-year period.

BACKGROUND: Ovarian tumors have generally been considered rare in the pediatric age group. We reported our experience dealing with pediatric ovarian tumors during an 8-year period. METHODS: Between January 1998 and December 2006, 37 girls with ovarian tumors were treated at the Department of Pediatric Surgery, Chang Gung Childrenfs Medical Center. Modes of clinical presentation, pathology diagnosis, methods of treatment and clinical outcome were retrospectively analyzed. RESULTS: Twenty-nine of the 37 patients were symptomatic with abdominal pain, abdominal distention or the presence of a palpable mass, reduction in appetite or nausea and vomiting and precocious puberty. Another 8 patients were diagnosed prenatally. Thirty patients had benign disease and 7 had malignant tumors. The malignant lesions included 5 germ cell tumors (2 yolk sac tumors, 2 immature teratomas, 1 dysgerminoma), and 2 sex cord stromal tumors. Operations performed were salpingo-oophorectomy (n = 22), oophorectomy (n = 8), cystectomy (n = 3), aspiration (n = 2) and biopsy only (n = 2). A laparoscopic approach was performed in 10 cases. Patients with stage II yolk sac tumors (n = 2) or grade III immature teratomas (n = 2) had elevated alpha-fetoprotein levels, and the patient with dysgerminoma was diagnosed as stage II b. All underwent salpingo-oophorectomy and received chemotherapy following their initial operation and remained free of disease at 8 months to 6 years of follow-up. CONCLUSIONS: In our studies, most ovarian tumors were benign. Epithelial cysts and teratomas were the most common benign lesions, and germ cell tumors were the most common malignancy. A laparoscopic approach was feasible in most cases. With accurate staging, complete resection, and chemotherapy for malignant tumors, patients are expected to have excellent survival rates.

Individualized management of upper rectal atresia.

BACKGROUND/PURPOSE: Congenital colonic atresia (CA) or stenosis is an infrequent cause of low intestinal obstruction in the neonate. Atresias can occur at any level, and the management of CA is determined by the atretic site and by the presence or absence of associated anomalies. We report our experience dealing with upper rectal atresia during a 5-year period. METHODS: Between January 2004 and December 2008, 3 female newborns with upper rectal atresia with or without associated anomalies were treated. Modes of clinical presentation, methods of diagnosis, associated anomalies, alternative management techniques, and clinical outcome were retrospectively analyzed. RESULTS: All 3 patients had progressive abdominal distension, bilious vomiting, and failure to pass meconium. Contrast enema showed an atresia at the upper rectum in 2 patients. At laparotomy, case 1 was found to have type III atresia of the upper rectum. Resection of the dilated portion of the proximal colon with end sigmoid colostomy was accomplished in the neonatal period followed by a transanal mucosectomy with takedown of the colostomy and a pull-through procedure at age 3 months. Case 3 had multiple jejunoileal atresias and an upper rectal atresia. The initial management was multiple resections of atretic bowel and anastomoses and an end sigmoid colostomy. The secondary procedure was a takedown of the colostomy and transanal mucosectomy with a pull-through procedure. Case 2 had type I upper rectal atresia in association with imperforate anus complicated by colon perforation during performance of a distal colostogram leading to a complicated and protracted clinical course. All the patients are currently well with voluntary bowel movements, and one has occasional soiling with follow-up of 9 months to 3 years. CONCLUSIONS: Colon atresia, especially at the level of the upper rectum, is uncommon. Whether to proceed with an ostomy or to individualize the operative procedure according to the location of the atresia is still controversial. Transanal mucosectomy was a useful technique at the time of the definitive pull-through for the treatment of upper rectal atresia. In cases of upper CA associated with imperforate anus, delay in diagnosis and potential complications may result if the diagnosis of upper rectal atresia is missed.

Single-Center Experience: immunosuppressive therapy as frontline treatment for 33 children with acquired severe aplastic anemia.

The authors retrospectively analyzed the records of 33 children with acquired severe aplastic anemia (SAA) diagnosed from July 1998 to October 2007 and first treated by immunosuppressive therapy (IST). Serial hematologic parameters, complications, transfusion requirements, and time to response were assessed. Allogeneic hematopoietic stem cell transplantation (HSCT) was attempted in 7 patients after failure of IST (n = 6) or relapse following an initial response to IST (n = 1). One child died of post-transplant lymphoproliferative disorder. Thirty of the 33 patients are alive and well after a median follow-up of 45 months (range, 7-116 months). Overall (transfusion-independent) response to IST was 73% (24/33). The actuarial 5 years survival rate was 89.4%. In this study, all patients with SAA received IST as standard front-line therapy. Approximately three-fourths of patients with SAA have durable recovery and excellent overall survival.

Clinical manifestations in infants with symptomatic meconium peritonitis.

BACKGROUND: Meconium peritonitis (MP) develops when bowel perforation occurs in utero or soon after birth, resulting in leakage of meconium into the peritoneal cavity. The clinical features are often variable and prenatal ultrasonography plays an important role in prenatal diagnosis. METHODS: We conducted a retrospective review of neonates diagnosed with MP in our hospital from January 1998 to December 2007. Prenatal examinations, postnatal presentations, investigations, management, patient outcomes, and possible causes were analyzed. RESULTS: Ten patients (five boys, five girls) diagnosed with MP were studied. The most common prenatal ultrasonographic finding was fetal ascites (7/10, 70%), followed by polyhydramnios (3/10, 30%), bowel dilatation (3/10, 30%), intra-abdominal calcification (1/10, 10%), and hydrops fetalis (1/10, 10%). Two of our patients were completely normal on prenatal ultrasonography. Only one MP diagnosis (1/10, 10%) was definitely confirmed by prenatal ultrasonography due to the presence of ascites, bowel perforation and intra-abdominal calcification. Nine patients (90%) required surgical intervention because of bowel perforation (5), bowel atresia (2), obstruction due to ileus (1), and intussusception (1). Peritonitis was of cystic type in one case (10%), fibroadhesive in five cases (50%), and generalized in four cases (40%). All 10 patients survived without long-term gastrointestinal complications. CONCLUSION: Prenatal ultrasonography can be diagnostic for MP, which should be considered in the differential diagnosis of patients presenting with ascites or abdominal distension at birth. Close observation of postnatal clinical manifestations and timely surgical intervention resulted in a high survival rate and favorable outcome in these patients at our hospital.

An accessory labioscrotal fold associated with anorectal malformation in female neonates.

The association of perineal lipomas with anorectal malformations in the newborn has been reported previously (Shaul DB, Monforte HL, Pena A, et al. Surgical management of perineal masses in patients with anorectal malformations. J Pediatr Surg 2005;40:188-191; Wester T, Rintala RJ. Perineal lipomas associated with anorectal malformations. Pediatr Surg Int 2006;22:979-981). Only 2 cases of accessory labioscrotal fold with perineal lipoma have been reported (Redman JF, Ick KA, North PE. Perineal lipoma and an accessory labial fold in a female neonate. J Urol 2001;166:1450, Sule JD, Skoog SJ, Tank ES. Perineal lipoma and the accessory labioscrotal fold: an etiologic relationship. J Urol 1994;151:475-477). To our knowledge, no other reports of accessory labioscrotal fold with anorectal malformation in the female patient have appeared in the literature. We present our experience of treatment with these rare cases and a review of the literature.

Choledochoduodenal fistula caused by blunt abdominal trauma in a child.

We report a 15-year boy who presented with obstructive cholangiopathy and pancreatitis after blunt abdominal trauma. A magnetic resonance cholangiopancreatography showed dilated common bile duct, dilated hepatic ducts, and a suspicious choledochoduodenal fistula. An endoscopic retrograde cholangiopancreatography revealed a parapapillary choledochoduodenal fistula. An operation of choledochojejunostomy, excision of common bile duct, and cholecystectomy was done for recurrent cholangitis. Abdominal symptoms completely subsided one month later. Serum amylase, lipase and bilirubin levels turned to normal 3 months after operation.