Restoration of electric footshock-induced immunosuppression in mice by Gynostemma pentaphyllum components.

The immunomodulatory effects of the ethanol extract of Gynostemma pentaphyllum (GP-EX) were examined in electric footshock (EFS)-stressed mice. The mice were orally administered various doses of GP-EX for 7 days before exposure to EFS (duration: 3 min, interval: 10 s, intensity: 2 mA) once a day from day 8 for 14 days with continuous daily feeding of GP-EX. Oral administration of GP-EX to mice prevented EFS stress-induced immunosuppression as determined by the lymphoid organ (thymus and spleen) weight and cellularity. In addition, oral administration of GP-EX restored EFS-suppressed functional properties of mature lymphocytes in terms of concanavalin A-induced proliferation of splenocytes and lipopolysaccharide-induced cytokine production (TNF-α, IL-1ß). Furthermore, we found that mice that were orally administered with GP-EX generated much more potent ovalbumin-specific cytotoxic T lymphocyte responses upon intravenous ovalbumin injection compared to the untreated controls. These results demonstrate that oral administration of the ethanol extract of Gynostemma pentaphyllum could increase host defense in immunocompromised situations such as stress-induced immunosuppression.

Haddad syndrome with PHOX2B gene mutation in a Korean infant.

Congenital central hypoventilation syndrome with Hirschsprung's disease, also known as Haddad syndrome, is an extremely rare disorder with variable symptoms. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. We report a newborn male infant with clinical manifestations of recurrent hypoventilation with hypercapnea and bowel obstruction. These clinical manifestations were compatible with congenital central hypoventilation syndrome and Hirschsprung's disease, and polyalanine 26 repeats in the PHOX2B gene supported the diagnosis of congenital central hypoventilation. We described a first case of Haddad syndrome in Korean and its clinical and genetic characteristics were discussed.

Successful endoscopic submucosal dissection of a giant polyp in a 21-month-old female.

Endoscopic submucosal dissection (ESD) is now recognized as the preferred treatment modality for gastrointestinal epithelial lesions. A 21-month-old female was admitted with a giant hyperplastic polyp causing a gastric outlet obstruction. Successful ESD was performed with caution. The post-procedural course was uneventful without a bleeding episode. Although further study of the feasibility of ESD in early children is necessary, ESD could be applied to avoid laparotomy even in young children.

Mesenteric suture granuloma caused by retained fragments of suture material in a girl who had a laparotomy 12 years previously.

The authors report a case of a mesenteric suture granuloma in a 12 year-old-girl who had a small bowel resection for a complicated intussusception at the age of 5 months. At later exploration a whitish round tumor located on the anti-mesenteric side of the intestine was found. Several small intestinal loops also abutted on the tumor. Pathologic examination showed fibrosis and a granuloma containing linear colored braided suture material with multinucleated giant cell. As mesenteric suture granulomas have a complex appearance and mimic a soft tissue tumor during imaging, it is important for a surgeon to know about this condition and to consider the history of previous surgery when evaluating the images of patients presenting with an abdominal or pelvic mass. Suture granulomas separate from previous suture sites have not been described in the literature.

Neurotoxic effects of berberine on long-term L-DOPA administration in 6-hydroxydopamine-lesioned rat model of Parkinson's disease.

The effects of berberine on long-term administration of L-DOPA in 6-hydroxydopamine (6-OHDA)-lesioned rat model of Parkinson's disease (PD) were investigated. Rat models of PD were prepared by 6-OHDA lesions in the ipsilateral sides, and then were treated with berberine (5 and 15 mg/kg) and/or L-DOPA (10 mg/kg) once daily for 21 days. Treatments with either concentration of berberine (5 and 15 mg/kg) in 6-OHDA-lesioned groups decreased the numbers of tyrosine hydroxylase (TH)-immunopositive neurons in the substantia nigra and the levels of dopamine, norepinephrine, 3,4-dihydroxyphenylacetic acid (DOPAC) and homovanillic acid (HVA) in the striatum as compared to 6-OHDA-lesioned groups. In addition, dopaminergic neuronal cell death of the ipsilateral sides in 6-OHDA-lesioned groups was attenuated by L-DOPA administration. However, both concentrations of berberine in 6-OHDA-lesioned groups treated with L-DOPA aggravated the numbers of TH-immunopositive neurons in the substantia nigra and the levels of dopamine, norepinephrine, DOPAC and HVA in the striatum as compared to rats not treated with berberine. These results suggest that berberine leads to the degeneration of dopaminergic neuronal cells in the substantia nigra in the rat model of PD with chronic L-DOPA administration. Long-term L-DOPA therapy that may involve possibly neurotoxic isoquinoline agents including berberine should involve monitoring for adverse symptoms.

Primary segmental volvulus of the ileum mimicking meconium plug syndrome.

Primary segmental volvulus (PSV) of the ileum in neonate occurs rarely but shows an aggressive clinical course. Thus, early diagnosis is important to prevent necrosis and perforation of the intestine. We report a case of PSV of the ileum in a 2-day-old female neonate whose clinical features and radiologic findings appeared to be meconium plug syndrome.

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy.

PURPOSE: Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE. METHODS: A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors. RESULTS: The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications. CONCLUSION: The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.

Diffuse microscopic angiodysplasia and nodular lymphoid hyperplasia in an infant with obscure massive lower gastrointestinal bleeding: a diagnostic challenge.

The authors report a rare case of diffuse microscopic angiodysplasia and nodular lymphoid hyperplasia involving the distal ileum and total colon in a 13-month-old girl who had recurrent episodes of massive lower gastrointestinal bleeding of obscure origin. Colonoscopy showed multiple nodular hyperplasia and mucosal erosions, and all other diagnostic studies were negative. At laparotomy, intraoperative transluminal endoscopic transillumination was of benefit in identifying the bleeding foci but could not detect every angiodysplastic lesion.

Intestinal chondrolipoma: uncommon cause of bowel obstruction.

A case of intestinal obstruction caused by jejunal chondrolipoma in an 11-year-old boy is presented. To the best of our knowledge, this seems to be the first report of chondrolipoma arising from the small bowel in English language literatures.

An unusual urachal sinus with external opening in the midline suprapubic area.

Urachal sinuses usually manifest as umbilical discharge, but the authors experienced a case of unusual urachal sinus with external opening in the midline suprapubic area. The distal end of the sinus tract was found to extend directly to the median umbilical ligament. The histology shows a sinus tract lined with a modified transitional epithelium with surrounding inflammatory infiltrates. The authors believe that this is the first report of a urachal sinus with external opening other than the umbilicus in the English-language literature.

Anal canal duplication in infants.

BACKGROUND/PURPOSE: Anal canal duplication (ACD) is the most distal and the least frequent digestive duplication. A review of the English-language literature found 15 cases reported in the pediatric age group. METHODS: A retrospective chart review was performed for our experience from 1999 to 2001 with 6 patients who presented with a midline postanal opening. RESULTS: All of 6 patients were girls and included one set of twins. Patients were from 3 to 9 months of age at the time of diagnosis (mean, 4.5 months). The anal canal duplication was delineated clearly by contrast study of the tract. All were tubular structures, 10 to 12 mm in length. None of the patients had any other associated anomalies. Five of 6 patients underwent operation between the ages of 3 and 8 months (mean, 5.4 months). Excision of the ACD was accomplished through the posterior sagittal approach. The orifice of the ACD, measuring 1 to 2 mm in diameter located just behind anus, directed to the lumen of the anal canal by keeping in the midline and ended blindly 5 mm above the dentate line without luminal communication. Histology findings showed a squamous epithelium with smooth muscle bundles in 2 cases and pseudostratified columnar epithelium with focally squamous epithelial lining and adjacent smooth muscle bundles in 3 cases. The postoperative courses were uneventful with satisfactory anal function. One patient has not yet undergone operation and has been well on outpatient follow-up. CONCLUSIONS: ACD is a congenital developmental lesion located in the midline posterior to the anus presenting as a tubular structure without communication with the anal canal, usually discovered in early infancy, and characteristically predominant in girls. The authors recommend that all ACDs, regardness of size and length, should be removed surgically to restore the normal anatomy and to avoid delayed presentation of infection such as perianal abscess or fistula formation.

Resolution of refractory chylous ascites after Kasai portoenterostomy using octreotide.

Chylous ascites is a rare disorder with variables causes. Herein, the authors report a case of refractory chylous ascites after Kasai operation for biliary atresia, which was treated successfully with subcutaneous octreotide, a synthetic somatostatin analogue. To the best of the authors' knowledge, this is the first report in the English-language literature of post-Kasai chylous ascites being successfully treated with subcutaneous octreotide in parallel with the continuation of enteral feeding.

Congenital gastric teratoma with gastric perforation mimicking meconium peritonitis.

A case of congenital immature gastric teratoma along with spontaneous gastric perforation in a 1-day-old boy is presented. The clinical features are similar to those of meconium peritonitis. Coincidentally, the child had an arachnoid cyst at 25 months of age. To the best of the authors knowledge, this case of congenital immature gastric teratoma associated with gastric perforation mimicking meconium peritonitis, is the first description in the English-language literature.

Objective criteria of triangular cord sign in biliary atresia on US scans.

PURPOSE: To develop objective criteria for the ultrasonographic (US) appearance of the triangular cord (TC) sign for the diagnosis of biliary atresia. MATERIALS AND METHODS: US was performed in 86 infants with jaundice. Biliary atresia (n = 20) was confirmed with hepatoportoenterostomy. Neonatal hepatitis (n = 66) was diagnosed with needle biopsy (n = 5), cholescintigraphy (n = 19), or clinical findings (n = 42). Thickness of the echogenic anterior wall of the right portal vein (EARPV) was measured. The TC sign was defined as thickness of the EARPV of more than 4 mm on a longitudinal scan. Biliary atresia was diagnosed when the TC sign was present. Statistical analyses were performed to compare the thickness of the EARPV between patients with biliary atresia and those with neonatal hepatitis and to test the significance of a 4-mm thickness as the criterion for the TC sign in the differentiation of biliary atresia from neonatal hepatitis (P <.05). RESULTS: The TC sign was present in 16 (80%) of 20 patients with biliary atresia and in one of 66 patients with neonatal hepatitis. Mean thickness of the EARPV was significantly greater in patients with biliary atresia (5.39 mm) than in patients with neonatal hepatitis (2.17 mm) (P <.05). Use of 4-mm thickness as the criterion for TC sign was statistically significant (P <.05), resulting in a sensitivity of 80%, specificity of 98%, and positive and negative predictive values of 94% for the diagnosis of biliary atresia. CONCLUSION: An objective criterion of the TC sign is an EARPV thicker than 4 mm on a longitudinal scan.