Kiminobu Sugito

Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision

We describe here three cases of pancreatitis after congenital choledochal cyst excision. In these three cases, the choledochal remnant in the pancreas head was markedly dilated, probably because of an incomplete resection of the cyst at the primary operation, and an increase in intraluminal pressure of the pancreatic duct caused by a dynamic obstruction by a protein plug or a pancreatic calculus. Complete cyst excision, including the choledochal wall in the pancreas, is therefore strongly recomended.

Long-term follow-up of nutritional status, pancreatic function, and morphological changes of the pancreatic remnant after pancreatic tumor resection in children.

Objectives The objectives of the present study were to determine nutritional status, pancreatic function, and morphological changes of the pancreatic remnant after pancreatic tumor resection in children. Methods The nutritional status was evaluated by the patterns of growth. Pancreatic function was evaluated by using a questionnaire, the Bristol stool form chart, the serum levels of fasting blood glucose, and hemoglobin A1c (HbA1c). Morphological changes of the pancreatic remnant were evaluated by computed tomography, magnetic resonance image, or magnetic resonance cholangiopancreatography. Results The present study consisted of 6 patients with pancreatic tumor (5 solid pseudopapillary tumors of the pancreas and 1 pancreatoblastoma) who underwent the following operations: tumor enucleation (3), distal pancreatectomy with splenectomy (1), and pylorus-preserving pancreatoduodenectomy (PPPD [2]). The serum levels of HbA1c have been gradually elevated in 2 patients with PPPD. A significant decrease in pancreatic parenchymal thickness and dilatation of the main pancreatic duct were observed in 2 patients with PPPD. Conclusion Endocrine pancreatic insufficiency after PPPD may be explainable by obstructive pancreatitis after operation. Taking together the results of pancreatic endocrine function and morphological changes of pancreatic remnant after PPPD, tumor enucleation should be considered as surgical approach in children with pancreas head tumor whenever possible.

The clinical course in pediatric solid tumor patients with focal nodular hyperplasia of the liver

BackgroundFocal nodular hyperplasia (FNH) of the liver is a rare benign lesion that may be related to the vascular and hepatic damage induced by completion of tumor therapy and a reaction to localized vascular abnormality. The aim of this study was to analyze the clinical course in pediatric solid tumor patients with FNH.MethodsWe analyzed thirty-two patients with pediatric solid tumors who received multiagent chemotherapy (15 advanced neuroblastomas, 7 hepatoblastomas, 5 rhabdomyosarcomas, 2 nephroblastomas, 1 rhabdoid tumor of the kidney, 1 clear cell sarcoma of the kidney and 1 pancreatoblastoma). All of them had been previously treated at our hospital, and have been alive for over 3 years without recurrence.ResultsFNH lesions were discovered in three (9.4%) of 32 patients, and were neuroblastoma (NB) stage 4. All 3 patients received induction chemotherapy and high-dose alkylating agents, and developed grade 3 (National Cancer Institute Common Toxicity Criteria; NCI-CTC) liver dysfunction during completion of tumor therapy without veno-occlusive disease. Two of the 3 patients received the same induction chemotherapy and high doses of alkylating agents with total body irradiation for cytoreductive agents prior to peripheral blood cell transplantation. FNH lesions in both female patients who received estrogen replacement therapy after completion of tumor therapy have expanded and are increasing.ConclusionFNH appears to be a late complication of iatrogenic disease in NB stage 4 patients. The therapeutic agents for NB stage 4 and estrogen replacement therapy should be considered as risk factors for the development of FNH.

Study of 24 cases with congenital esophageal atresia: What are the risk factors?

Background: Recovery from esophageal atresia (EA) and tracheoesophageal fistula (TEF) has improved markedly over the years. But postoperative complications, however, have remained. This study evaluates recovery, preoperative, and postoperative status of patients with EA/TEF.

Spontaneous localized intestinal perforation and intestinal dilatation in very-low-birthweight infants.

Aim: To elucidate how spontaneous localized intestinal perforation (SLIP) is related to intestinal morphological features such as dilatation in very‐low‐birthweight (VLBW) infants. Methods: The medical records of 13 VLBW infants (<1500 g) undergoing laparotomy between 1983 and 2003 for presumed SLIP were retrospectively reviewed. Clinical findings including maternal, prenatal and perinatal factors were analysed, and the clinical and surgical findings upon laparotomy were compared. Results: Postnatal pathological conditions included patent ductus arteriosus (n= 7), sepsis (n= 2), respiratory distress syndrome (n= 7), intraventricular haemorrhage (n= 2), an indwelling catheter via the umbilical vein (n= 1) and pneumonia (n= 1). Indomethacin was used in seven neonates with patent ductus arteriosus, and dexamethasone preventive therapy was employed in one neonate for bronchopulmonary dysplasia. Operative findings revealed a localized small punched‐out perforation in the ileum. Five patients had intestinal dilatation: two with a perforation in the middle of the dilated intestine, and three with a perforation proximal to the region of dilatation. The muscularis propria was absent in the dilated intestine of four patients.

The effect of a novel immunosuppressant, FTY720, in mice without secondary lymphoid organs.

PurposeFTY720 is a novel immunosuppressive agent that is thought to reduce the number of peripheral blood lymphocytes (PBL) by directing them toward secondary lymphoid organs such as the lymph nodes and Peyer’s patches. We studied the effects of FTY720 on aly/aly mice that do not have either lymph nodes or Peyer’s patches, as well as on splenectomized aly/aly mice.MethodsFTY720 was orally administered by gavage (1 mg/kg) to aly/aly mice as well as to aly/+ mice with and without a splenectomy on 14 consecutive days. The number of lymphocytes was then counted using True Cell beads and flow cytometry. The number of B220-, CD3-, and CD4-positive cells was also determined. In addition, skin grafts from C3H donor mice were performed on these mice.ResultsFTY720 was effective in significantly reducing the total lymphocyte count as well as the B220-, CD3-, and CD4-positive subtypes in the peripheral blood of aly/+ mice as well as in aly/aly mice with and without a splenectomy. While we did observe allograft skin graft rejection in both the aly/+ mice as well as the aly/aly mice recipients and splenectomized aly/aly mice, the graft survival was prolonged in all groups. The skin allografts treated by FTY720 thus demonstrated fewer lymphocytic cells and less infiltration of CD4-positive cells.ConclusionsThe administration of FTY720 to mice without lymph nodes, Peyer’s patches, or spleens still results in peripheral lymphopenia. In all groups, FTY720 was found to prevent the infiltration of CD4-positive cells in skin allografts while also prolonging skin allograft survival. The fate of these lymphocytes, however, is unclear.

Successful treatment for hepatoblastoma in a 1‐year‐old boy with trisomy 18

type GCT, relapses may occur even decades after diagnosis, although the survival rate is 95% if diagnosed in the early stages. The presented case indicates the need to emphasize the possibility of occurrence of endocrinological disorders as a symptom of hormone-secreting tumors in children. GnRh-independent precocious puberty may be the first symptom of hormone-secreting ovarian tumors in children, even if tumors are not detectable in preliminary radiologic examinations. In such cases, profound diagnostics with systematic ultrasound examination are essential.

Therapeutic strategies of meconium obstruction of the small bowel in very‐low‐birthweight neonates

Background:  Meconium obstruction without cystic fibrosis in low‐birthweight neonates is a distinct clinical entity. We aimed to determine what therapeutic strategies work best in very‐low‐birthweight neonates with meconium obstruction of the small bowel under varied clinical conditions caused by the associated diseases of prematurity.

Mesenchymal hamartoma of the liver originating in the caudate lobe with t(11;19)(q13;q13.4): Report of a case

We herein report the case of a 35-month-old female child presenting with mesenchymal hamartoma of the liver (MHL), with t(11;19)(q13;q13.4) originating in the caudate lobe. This case is the eighth known description of a cytogenetic abnormality in mesenchymal hamartoma of the liver. It is similar to the seven cases previously reported, in that one of the breakpoints involves the chromosome band 19q13.3 or 19q13.4, but it is the first report of an abnormality originating in the caudate lobe.

Ano-neorectal function using manometry on patients after restorative proctocolectomy and ileal J-pouch anal anastomosis for ulcerative colitis in children.

BACKGROUND/AIMS The purpose of this study was to clarify the ano-neorectal functions in pediatric patients with soiling at a short period and without soiling at a long period after restorative colectomy and ileal J-pouch anal anastomosis (IPAA) for ulcerative colitis (UC). METHODOLOGY Ten patients after IPAA for UC in childhood were mamometrically studied, aged 10 to 16 years (mean, 13.9 years). Patients after IPAA with ileostomy closure were studied at 6 months (Group A; all patients had soiling) and 3 years after ileostomy closure (Group B; all patients showed continence). Group C served as controls and consisted of 12 subjects (aged 12 to 16 years, mean, 14.8). RESULTS Maximum anal sphincter pressure at rest and maximum anal sphincter pressure during voluntary contraction were significantly lower in group A than in groups B and C. Minimum neorectal sensory threshold volume in group A was significantly higher than in groups B and C (p<0.01). Maximum neorectal tolerated threshold volumes and neorectal compliances, and positive rates of neorectoanal inhibitory reflex, showed no significant difference among the groups. CONCLUSIONS Patients with soiling at 6 months after IPAA showed anal sphincter dysfunction and neorectal sensory dysfunction. The IPAA may cause damage to the ano-neorectal apparatus during rectal mobilization due to the short rectal cuff and mucosectomy.