Possible effect of probiotics and breast milk in short bowel syndrome: report of one case.

Primary volvulus means idiopathic volvulus without predisposing factor and is rare in children. The etiology is unknown. The incidence is relatively higher in neonates. The most common symptoms are abdominal distension and bilious vomiting. Our patient was a preterm baby at age of 89 days. Acute onset of abdominal distension and sepsis-like symptoms were noted. After operation, no anatomical anomaly was noted. Probable primary midgut volvulus was diagnosed. Early diagnosis of primary volvulus of the small intestine is difficult. Operation should be performed as soon as possible in a neonate with quick progression toward unstable hemodynamics and acidosis with ileus. Postoperative short bowel syndrome was noted. There are often sepsis, enterocolitis, and poor body weight gain noted among short bowel patients. With breast milk feeding and probiotics usage, there were few complications of short bowel syndrome noted in our patient. The duration for establishing intestinal adaptation was shorter than for other patients. The patient's body weight, body length and development caught up gradually within 18 months.

Home-made double-channel endoscope for pediatric patients.

Commercially available double-channel endoscopes are designed mainly for adults. We present our experience of using a home-made pediatric double-channel endoscope. A feeding tube was attached to the side of a single-channel endoscope by adhesive tape. The attached tube was used as an additional working channel. Fifty-nine procedures, including esophageal variceal injection sclerotherapy, gastrointestinal bleeding, polypectomy, and foreign body removal, were successfully performed using this home-made double-channel endoscope. No complications were observed. This home-made double-channel endoscope is safe, efficient, and inexpensive for use in pediatric procedures.

A comparison of engineered urinary bladder and intestinal smooth muscle for urinary bladder wall replacement in a rabbit model.

BACKGROUND/PURPOSE: The small intestine is the most common resource for bladder augmentation. Little is known whether intestinal smooth muscle cells (SMCs) may be engineered into bladder tissue. We investigated the phenotypic and functional characteristics of engineered bladder and intestinal SMCs as bladder wall replacement in a rabbit model. METHODS: One month after an initial 70% partial cystectomy, 3 autoaugmentation surgeries were performed, including traditional autoaugmentation (TA, n = 6), TA using engineered bladder SMCs (TA + B, n = 6), and TA using intestinal SMCs (TA + I, n = 6). All were followed up by bladder volume measurement and retrieved on the first, third, and sixth month. The grafts and the native bladder wall were evaluated with immunocytochemistry and electrical field stimulation (EFS). Statistical analysis was performed using analysis of variance. RESULTS: Both the TA + I and TA + B groups showed significant and similar bladder capacity increment in all time points. The engineered muscle cells demonstrated the typical "contraction-relaxation" response to supramaximal EFS. There were no statistical differences in both the TA + I and TA + B groups in contractility force. CONCLUSION: Engineered SMCs derived from urinary bladder and small intestine could retain their phenotype after implantation in vivo. Both exhibited a similar degree of contractility to EFS. These results suggest that there are no phenotypic or functional differences between muscle cells obtained from the 2 different organs. Both have the potential to be engineered into normal bladder tissues.

Bladder autoaugmentation using various biodegradable scaffolds seeded with autologous smooth muscle cells in a rabbit model.

BACKGROUND/PURPOSE: The prolapsed mucosa after bladder autoaugmentation usually collapses, and the volume increment is limited. This study is aimed at evaluating the efficacy of autoaugmentation assisted with 2 different scaffolds, polyglycolic acid (PGA) mesh and small intestinal submucosa (SIS), seeded with autologous bladder smooth muscle cells in a rabbit model. METHODS: One month after an initial 70% partial cystectomy, various autoaugmentation surgeries were performed. These procedures included traditional autoaugmentation (n = 6) and traditional autoaugmentation covered with PGA or SIS without cell seeding (N) (PGA-N, n = 6; SIS-N, n = 6) or covered with scaffolds seeded with autologous bladder smooth muscle cells (C) (PGA-C, n = 6; SIS-C, n = 6). All were followed up by bladder volume measurement and retrieved on 1, 2, 3, and 6 months. Statistical analysis was by analysis of variance. RESULTS: A normal urothelial layer was maintained in all groups. Only PGA-C group showed a significant bladder capacity increment as compared with the other groups in all time-points (P = .001, .000, .000, and .001 at first, second, third, and sixth months, respectively). The PGA-C group showed grossly normal bladder wall with scattered smooth muscle bundles. The other groups had marked graft shrinkage with only unorganized muscle fibers. CONCLUSION: Cell-seeded PGA polymer facilitates smooth muscle regeneration, offers sufficient bladder wall backup, and achieves satisfactory volume increment after the autoaugmentation with time. The collagen matrix, although seeded with cells, did not offer adequate mechanical support after the surgery.

Comparison of childhood hepatic malignancies in a hepatitis B hyper-endemic area.

AIM: To examine the differences of clinical behaviors between hepatocellular carcinomas (HCC) and hepatoblastomas (HB) in children. METHODS: From 1979 to 1997, we collected 73 HCC and 54 HB from two major medical centers in Taiwan. Demog-raphic, laboratory and radiological data, and survival curves were statistically compared. RESULTS: HCC clinically differed from HB in mean age (10.6 vs 2.5 years; P<0.001), status of hepatitis B infection (56/56 vs 4/35, P<0.001) and accompanying liver cirrhosis (26/40 vs 0/30, P<0.001), portal vein thrombi (22/56 vs 5/38, P = 0.006) and para-aortic lymphadenopathy (10/56 vs 1/38, P = 0.026). Due to a higher recurrence rate (7/12 vs 2/13, P = 0.041), stage I HCC compared poorly in survivals with stage I HB (P = 0.0183). Chemotherapy could only benefit HB as evidenced by 66.7% of resectability conversion and improve survivals for advanced HB, even with unsuccessful conversion. The survival difference between stage I HB and advanced HB with delayed complete resection was of borderline insignificance (P = 0.0507). CONCLUSION: HCC and HB were preliminarily distinguishable by some clinical clues. Delayed resection after chemotherapy was only possible for HB. However, further studies are needed to strengthen our observation that appropriate reliance upon chemotherapy to subsequently resect advanced HB could achieve the comparable survival to that of stage I HB.

Correlation of MYCN amplification with MCM7 protein expression in neuroblastomas: a chromogenic in situ hybridization study in paraffin sections.

Amplification of the MYCN oncogene in neuroblastomas is generally associated with a more aggressive clinical course. Recently, 1 of the minichromosome maintenance proteins, MCM7, was found to be a direct target of the MYCN transcription factor in neuroblastoma. To confirm this correlation, chromogenic in situ hybridization (CISH) to detect MYCN amplification and immunohistochemical staining for MCM7 protein expression were performed on paraffin tissue sections of 26 neuroblastomas cases and of 4 recurrences of these tumors. Seven of the primary tumors showed MYCN amplification, and all were stage 3 or 4 tumors. Only 4 of these showed MCM7 overexpression. However, 11 primary tumors overexpressed MCM7. The 4 patients with MCM7 expression associated with MYCN amplification all died from the tumor. In contrast, the 7 patients with MCM7 overexpression but no MYCN amplification were all younger than 1 year of age and have shown good survival. This suggests that MCM7 overexpression by itself is not related to a poorer prognosis as is MYCN amplification. In addition, the 4 pairs of primary and recurrent tumors all showed changes in MCM7 expression from negative to positive, whereas none of them had MYCN amplification. This study showed that MCM7 overexpression is not necessarily correlated with MYCN amplification or an aggressive clinical course. Interpretation of the results of CISH was quite easy and straightforward because the preparations were viewed with an ordinary light microscope with good preservation of the tissue morphology.

Translocation of coagulase-negative bacterial staphylococci in rats following intestinal ischemia-reperfusion injury.

Many patients with sepsis have bacteremia for which no septic focus is identified either clinically or by autopsy. This study was designed to determine the relationship between the ischemia-reperfusion injury (IRI) and bacterial translocation that might be involved in the pathogenesis of necrotizing enterocolitis. In the first experiment, a total of 32 Sprague-Dawley rats weighing 150-200 g were divided into four groups. The mesentery to isolated loop was occluded for 30, 60, and 90 min following 30-min reperfusion in the three groups of experimental animals with a micro-bulldog clamp. A control group involved the same technique and exposure, without occlusion of the mesentery. Two sets of blood culture were taken through a catheter in the portal vein immediately and 15 min after the reperfusion, respectively. In another experiment, bacteria isolated were fed in different doses to control rats and those after 30- or 60-min ischemia and 30-min reperfusion. Two sets of blood culture were taken following the procedure. Invasion and transcytosis of the bacteria through epithelial cells were studied in vitro using a Madin-Derby canine kidney (MDCK) cell monolayer model. PCR for delta toxin gene was performed on all bacteria isolated, using Staphylococcus epidermidis as the control. Coagulase-negative staphylococci (CoNS) were invariably isolated from mice with prolonged ischemia (90 min) and reperfusion. When bacteria were fed into mice with only 30-min ischemia, an inoculum as low as 5 x 10(5) CFU/ml could induce bacteremia. No bacterial translocation was found in control mice even fed with a higher dose of bacteria (5 x 10(8) CFU/ml). In vitro experiments showed that CoNS failed to transcytose MDCK monolayer. These isolates were not cytotoxic to MDCK cells and contained no delta toxin gene. Bacterial translocation of CoNS occurred following severe bowel ischemia and reperfusion injury. Intact mucosa integrity readily prevented bacterial translocation; however, bacterial translocation could occur in rats following mild IRI in the presence of a higher number of CoNS in the gut.

Body wall repair using small intestinal submucosa seeded with cells.

BACKGROUND/PURPOSE: Prosthetic repair of large ventral abdominal wall defects has been associated with high complication rates. This study was aimed at applying tissue engineering to body wall replacement. METHODS: Syngeneic Lewis rats underwent harvest of skeletal muscle specimens. Once expanded in vitro, skeletal muscle cells or fibroblasts were suspended in a collagen gel. All animals underwent creation of a 2.5- x 3-cm abdominal wall defect. The defect was repaired with the cell-seeded gel placed in between 2 pieces of small intestinal submucosa (SIS). The control group was repaired by SIS with acellular gel. Animals were killed at different time-points for histologic and mechanical examination. Statistical analysis was by analysis of variance (ANOVA). RESULTS: Abdominal wall hernia was present in 6 of 24 fibroblast-seeded constructs (25%), 5 of 21 skeletal muscle cell-seeded constructs (23.9%), and 16 of 21 acellular grafts (76.2%), respectively (P <.05). At harvest, cell-seeded constructs were thicker with better cellular infiltration, whereas acellular grafts were thin, low in cell density, and poor in mechanical resistance. CONCLUSIONS: Unlike acellular collagen matrices, engineered cellular constructs have better cell infiltration and mechanical performance. Tissue engineering may be a viable alternative for body-wall replacement.

A new variant of esophageal atresia with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus.

Esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is the most common anatomic pattern within congenital anomalies of the esophagus. Also, more than 50% of the infants with esophageal atresia have other congenital malformations. To our knowledge, this is the first case report of EA with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus (SI).

Adenocarcinoma of the jejunum: a pediatric case report.

The clinical history and surgical findings of an adenocarcinoma of the jejunum in a 15-year-old boy are reported. The diagnosis of small bowel carcinoma remains difficult, primarily because of the rarity of the disease and the ambiguity of its symptoms. We reviewed the literature on this subject and found a total of seven cases of adenocarcinima of the jejunum and ileum in patients under 20 years of age. We report this case for its rarity and the findings which should alert the pediatricians including intermittent abdominal pain, severe loss of body weight, and stools positive for guaiac.

Pathological analysis of congenital cervical cysts in children: 20 years of experience at Chang Gung Memorial Hospital.

BACKGROUND: Congenital cervical cysts are frequently encountered in pediatric populations, and constitute one of the most intriguing areas of pediatric pathology. This report analyzes cervical cysts in Taiwanese children diagnosed at Chang Gung Memorial Hospital (CGMH) over the past 20 years. The pathologic and clinical findings are reviewed. METHODS: Files on 331 patients under the age of 18 years, with a diagnosis of congenital cervical cyst at CGMH from January 1, 1983 to June 30, 2002, were retrieved from the Department of Pathology. There were 204 boys and 127 girls. We reviewed the histology of all cases and correlated it with clinical information in the medical records. RESULTS: Thyroglossal duct cysts, the most common congenital neck cyst, accounted for 54.68% of all cases, followed by cystic hygromas (25.08%), branchial cleft cysts (16.31%), bronchogenic cysts (0.91%), and thymic cysts (0.30%). Nine cases (2.72%) remained unclassified. CONCLUSIONS: This is the largest series regarding pediatric cervical cysts in the literature to date. Thyroglossal duct cysts were the most common congenital cervical cyst encountered. Our experience indicates that each type of cyst has its unique location in the neck and is highly associated with its embryonic origin. Complete and precise clinical information is a prerequisite in order for pathologists to make accurate diagnoses of congenital cervical cysts.

Pheochromocytoma presenting as stroke in two Taiwanese children.

Pheochromocytoma is a rare tumor and accounts for about 1% of pediatric hypertension. Its diagnosis is important because the hypertension is usually curable by eradication of the tumor. We report two Taiwanese children with pheochromocytoma presenting as stroke. Patient 1 developed a cerebral infarction and patient 2 had an intracranial hemorrhage. Abdominal MRI of patient 1 showed an extraadrenal tumor 5.1 x 4 x 4.7 cm in size and abdominal CT of patient 2 revealed a left adrenal mass 4 x 2.9 x 4 cm in size. 24-h urinary excretion of norepinephrine for patients 1 and 2 were 1480.8 and 3279 microg/day, respectively. Preoperative alpha- and beta-adrenergic blockade was used for hypertension control. Both patients underwent successful tumor resection, upon which blood pressure and 24-h urinary excretion of catecholamines returned to normal without any further medication. They have been free from hypertension for 4 years (patient 1) and 2 years (patient 2).

Evaluation of oesophageal atresia without fistula by three-dimensional computed tomography.

UNLABELLED: During the last decade, an increasing number of paediatric surgeons have chosen to perform a delayed oesophageal anastomosis for oesophageal atresia (OA) without tracheo-oesophageal fistula (TOF). The gap between the two oesophageal pouches is an important determinant in the surgical management of these patients. We describe a new method using spiral computed tomography (CT) to evaluate the gap between the proximal and distal oesophageal pouches. In our last four cases of OA without TOF, Stamm gastrostomy was performed soon after birth. At about 4 weeks of age, these patients underwent spiral CT with air inflation via the gastrostomy feeding tube with 3 mm slice thickness, pitch 1, and reconstruction interval every 1.5 mm. A three-dimensional reconstruction was done and measurements of the gap between the two segments were recorded. The procedure was repeated at monthly intervals until the optimal conditions for surgery were observed. All the babies thrived well on gastrostomy feeding. The gap distances were 2.4, 3.8, 4.2 and 5.9 cm respectively. Delayed primary oesophageal anastomosis was accomplished in three cases and gastric transposition was performed in another case at 12, 13, 14 and 16 weeks of age. The exact anatomical position the operation correlated well with the pre-operative three-dimensional CT results. CONCLUSION: this new technique is an easy and non-invasive method to assess the long gap in babies with oesophageal atresia without tracheo-oesophageal fistula. The radiological findings are also consistent with surgical anatomical situation during surgery suggesting that this investigation is useful in the pre-operative planning of oesophageal anastomosis or replacement.

Undifferentiated sarcoma of the liver.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare primary hepatic malignancy principally affecting patients of pediatric age. It is believed to be a primitive mesenchymal neoplasm, which usually behaves in a highly malignant fashion. The median survival has been less than a year. The only chance for cure appears to be radical excision of the tumor. However, some patients may develop recurrent disease despite complete surgical resection of the tumor. Recently, long-term disease-free survival has been achieved in cases which underwent aggressive multimodal treatment. Herein we report on a 12-year-old girl who underwent surgical excision of USL. Histologically, it was composed of pleomorphic stellate or spindle-shaped cells in a myxoid stroma. Characteristic periodic acid Schiff-positive, diastase-resistant intracytoplasmic hyaline globules were seen. Immunohistochemically, the tumor cells were positive for vimentin, alpha1-antitrypsin, and alpha1-antichymotrypsin. In addition, p53 protein was expressed in 40% of tumor cells, and Ki-67 was demonstrated in 45% of tumor cells. Postoperative chemotherapy was recommended, but was refused by the patient's family. A recurrent liver mass was found 171 days after the operation. Chemotherapy was refused once again by the patient's family, and she was lost to follow-up for 5 months. The patient was brought to the outpatient clinic again because of abdominal fullness and back pain. The recurrent tumor was 20 cm in size with compression of the inferior vena cava. She was admitted and received chemotherapy with vincristine, ifosfamide, and cisplatin.

Infrequent p53 gene mutations and lack of p53 protein expression in clear cell sarcoma of the kidney: immunohistochemical study and mutation analysis of p53 in renal tumors of unfavorable prognosis.

A high prevalence of p53 gene mutation and protein expression has been found in the anaplastic variant of Wilms' tumor (WT), known to be associated with poor outcome. However, there are very few studies of p53 alterations in the other two rare and highly malignant renal tumors in childhood, in other words, clear cell sarcoma of the kidney (CCSK) and malignant rhabdoid tumor of the kidney (MRTK). Overexpression of p53 protein has been detected in eight CCSKs in one study, and in two in another, yet no molecular correlation with p53 gene mutations has been carried out. Our study is the first molecular analysis concerning p53 in CCSK. We investigated eight cases of CCSK and one case of MRTK for p53 protein expression by immunohistochemical staining. All were analyzed for p53 mutations in the region of exons 4 to 8 by polymerase chain reaction-single-strand conformational polymorphism (PCR-SSCP) method and DNA sequencing analysis. By histological study, no CCSK showed anaplastic features. None expressed p53 protein, but two harbored p53 mutations. One was in exon 5, with a base pair insertion between codons 162 to 163 causing frameshift alteration in amino acid. Another was a silent CTC-->CTT transversion in codon 289 of exon 8. The case of MRTK did not show any alterations of p53 protein or gene. Our result indicates that p53 alterations are infrequent in CCSK and do not seem to be primary genetic events in the pathogenesis of CCSK.

Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature.

UNLABELLED: Malignant rhabdoid tumour of the kidney (MRTK), an uncommon aggressive neoplasm of children, is now recognised as a separate entity from Wilms' tumour with distinct clinical and pathological features. MRTK is unique in its significant association with primary brain tumours or brain metastases. We report two cases, aged 2 and 6 months, of MRTK occurring concurrently with a brain tumour. Radical nephrectomy and ventriculo-peritoneal shunting were performed. Both patients expired 2 and 6 months later despite receiving aggressive post-operative chemotherapy and radiotherapy. CONCLUSION: malignant rhabdoid tumour of the kidney is an uncommon neoplasm of early childhood with a poor prognosis. Due to its significant association with brain tumours or early brain metastases, concurrent brain computer tomographic examination is essential for all patients with this disease.

Hepatoblastoma in a child of extremely low birth weight.

Hepatoblastoma is a rare childhood embryonal malignancy. Recent reports suggested a linkage between premature birth and later development of hepatoblastoma. In August 2000, we encountered a 24-month-old boy with extremely low birth weight who developed hepatoblastoma with an unfavorable histology. His birth weight was only 584 g. Radiological evaluation of the liver revealed a hypoattenuated tumor occupying the left lobe. His initial serum alpha-fetoprotein level was 4032 ng/mL. The patient underwent successful left hepatectomy. The histological examination showed that the tumor, weighing 210 g, was a hepatoblastoma of epithelial type in two different patterns. One was composed purely of fetal type of tumor cells, whereas another was made up of embryonal type of cells in a macrotrabecular pattern. Four courses of adjuvant chemotherapy were given after surgery and were well tolerated. The patient remained in clinical remission 18 months after diagnosis.

Spontaneous intracranial hemorrhage caused by pheochromocytoma in a child.

We report a 6-year-old boy who presented with left occipital spontaneous lobar hematoma and hypertension. Cerebral angiography failed to demonstrate a vascular malformation. Subsequently, a left adrenal tumor was found that proved to be a pheochromocytoma. This is the first report in the literature of a lobar hematoma in a child caused by a concomitant pheochromocytoma. In children with hypertension and resulting intracerebral hematoma, a pheochromocytoma should be considered in the differential diagnosis.