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Biomedical subjects

Sachiyo Suita

Publications and source records attributed to Sachiyo Suita.

At least 55 records · Page 3Linked to original sources

Peutz-Jeghers syndrome in children: high recurrence rate in short-term follow-up.

We have managed four cases of Peutz-Jeghers syndrome (PJS) in children. Fathers of three of these patients had PJS. There was also a family history of cancer in three cases, with pancreatic cancer in a father, colonic and laryngeal cancers in a grandfather, and hepatic and gastric cancers in a grandmother. It is presumed that in each of the cases, the largest polyp was responsible for initial symptoms. Preoperative examination revealed additional small polyps in the whole alimentary tract except for the oesophagus. Patients underwent laparotomy to remove the largest polyps and subsequent intraoperative endoscopic polypectomy for other small polyps, to minimize intestinal resection. Follow-up gastrointestinal examinations, including upper gastrointestinal series, small intestinal contrast study, and barium enema, were repeated about once a year. Three of four cases showed recurrent small intestinal polyps, and one required a second laparotomy because of recurrent abdominal pain. In conclusion, patients with PJS occurring in childhood have a strong hereditary family history of cancer and a high incidence of recurrence. Careful follow-up examination is mandatory for the gastrointestinal tract, as well as other solid organs that are susceptible to malignant change, throughout a patient's life.

Adolescent↗

Prenatally diagnosed cystic neuroblastoma: a report of two cases.

We report two cases of prenatally diagnosed cystic neuroblastoma (PDCN). In the first case, prenatal ultrasonography (US) at 33 weeks' gestation showed a 30 x 20 mm cyst at the upper pole of the right kidney. The size and content of the mass demonstrated no change during pregnancy. Postnatal US showed no change in the cystic mass 4 weeks after birth compared to the prenatal findings. The infant underwent total resection of the tumour at 28 days of age. In the second case, a left cystic mass measuring 50 x 40 mm was detected in a fetus in the 37th week of pregnancy. Postnatal US showed a cystic mass in the left adrenal gland. The US findings showed no change 18 days after birth and the infant underwent total resection of the tumour at 19 days of age. In both cases, pathological examination revealed a neuroblastoma and all of the biological prognostic factors were favourable. Surgical intervention was necessary for a final histological and biological diagnosis to be made. We recommend that prenatally suspected neuroblastomas should normally undergo surgical intervention, unless tumour size decreases within about 1 month after birth.

Adrenal Gland Neoplasms↗

Hepatic foreign body - a sewing needle - in a child.

We report a case of a 1-year-old boy with a needle-like foreign body embedded in the liver. The foreign body was incidentally found in the right hypochondrium on routine chest X-ray during a periodic medical examination. He was asymptomatic and there was neither a history of swallowing a needle nor a puncture wound on his body. The results of blood tests and physical examination were entirely within normal limits. Computed tomography scan showed that the needle was completely buried in the liver. At laparotomy, some fibrous tissue and a scar were recognized between the surface of the left lobe of the liver and the parietal peritoneum of the upper abdominal wall. The end of the sewing needle was manually squeezed out and extracted from the liver. From this operative finding, it was assumed that the needle had penetrated the liver through his skin. His postoperative course was uneventful and he was discharged on postoperative day 8.

Child, Preschool↗

Adrenal cytomegaly: two cases detected by prenatal diagnosis.

We report our experience with two cases of adrenal cytomegaly, both of which were detected as cystic adrenal masses during prenatal ultrasonographic examinations. In Case 1, a left suprarenal cystic mass was detected in the fetus at 25 weeks of gestation. The mass, measuring 7 cm in diameter, did not show any change in size and was resected 26 days after birth. In Case 2, a right suprarenal lesion was found at 30 weeks of gestation. The cystic lesion, measuring 2 cm x 1.5 cm, did not change in size and was resected 3 months after birth. Adrenal cytomegaly is still not well known. It is characterized by the presence of large polyhedral cells with eosinophilic granular cytoplasm and enlarged nuclei in the adrenal cortex. This condition is thought to be a degenerative process but not a malignancy. Adrenal cytomegaly rarely forms cysts. It seemed to be impossible to diagnose preoperatively in our cases. Because of the difficulty of differentiating between cystic adrenal cytomegaly and other cystic diseases such as neuroblastoma, operative intervention is required in cases where the cysts do not decrease in size. Further study of a larger number of cases is needed to establish an optimal treatment protocol for these tumours.

Adrenal Gland Diseases↗

The occurrence of unusual smooth muscle bundles expressing alpha-smooth muscle actin in human intestinal atresia.

BACKGROUND/PURPOSE: Intestinal dysmotility is an important problem in the postoperative management of patients with intestinal atresia (IA). Changes in the intramural components have so far been histochemically and immunohistochemically examined in both the proximal and distal segments of IA, but no detailed analysis of the muscular elements has been performed. The aim of this study was to carefully examine any alterations in the muscular elements in the intestines from patients with IA. METHODS: Resected intestines were obtained from 6 patients with ileal atresia, 4 patients with jejunal atresia, and 3 controls without gastrointestinal diseases obtained by autopsy (congenital diaphragmatic hernia). All specimens were immunochemically stained with a monoclonal antibody to alpha-smooth muscle actin (alpha-SMA) as a smooth muscle marker. RESULTS: In the normal small intestine, almost all the enteric smooth musculature were positive for alpha-SMA antiserum, except for the bulk of the circular musculature. In the proximal segments of all cases with IA, a reduced staining intensity for alpha-SMA was observed mainly in the severely hypertrophic muscle layers. In addition, some bundles of smooth muscle also were located in the submucosal connective tissue near the border of the innermost layer of the circular musculature, in which large amounts of smooth muscle fibers extended occasionally from the innermost layer of the circular musculature to the muscularis mucosae in the proximal segments of 4 cases. In the distal segments of IA, the distribution of alpha-SMA-positive smooth muscle fibers was similar to that in the control intestines, excluding mild to moderate hypertrophy of the muscular layers. CONCLUSIONS: Both severe hypertrophy and a reduced immunoreactivity for alpha-SMA were observed in the circular muscle layer of the proximal segments. In addition, the occurrence of alpha-SMA-positive abnormal smooth muscle fibers was recognized in the submucosal layers of the proximal segments, thus, suggesting a delay in the intestinal muscular formation or a regressive reaction secondary to dilatation. These muscular alterations in the proximal segments might be considered to contribute to the postoperative intestinal dysmotility in IA cases.

Actins↗

The efficacy of autologous cord-blood transfusions in neonatal surgical patients.

PURPOSE: Allogenic blood transfusions have a risk of infection owing to unknown organisms, graft-versus-host reaction, and immunosupression; however, the use of autologous blood has been reported to be safe. Cord blood has been reported to be useful as a source of stem cell transplantation for the treatment of leukemia and genetic disease. Furthermore, autologous cord-blood transfusions (ACBT) have been reported to be effective for the treatment of anemia in premature infants. The authors examined the efficacy of ACBT in neonatal surgical patients. METHODS: Autologous cord-blood was stored from 12 infants at delivery, including 2 transvaginal and 10 cesarean section deliveries, from 1998 to 2001. All infants had surgically correctable malformations diagnosed antenatally. The mean gestational age was 37.2 +/- 1.6 weeks, and the birth weight was 2,597 +/- 1.6 g. The results of the blood count, serum electrolyte, and liver function tests of the patients who underwent ACBT only (group 1, n = 7) were compared with those of the 7 neonates who underwent an allogenic transfusion during the same period (group 2, n = 7). RESULTS: The mean volume of the stored blood was 64 +/- 35.6 g (range, 20 to 100). Eleven of the 12 patients underwent transfusions. Ten of 11 patients received autologous cord blood. A mean of 44.1 +/- 37.3 g of cord blood was used. Three of 10 cases also required an allotransfusion because of ECMO circuit preparation and a shortage of the stored blood. One patient underwent allotransfusion only. As a result, 7 of 11 babies (64%) who required transfusion were able to avoid an allotransfusion. The blood potassium levels were lower in group 1 than in group 2. No significant complications were recognized clinically. CONCLUSIONS: ACBT is considered beneficial because it enables neonatal surgical patients to avoid allotransfusions. Therefore, autologous cord-blood storage should be considered in the patients antenatally diagnosed to have surgical malformations. However, the storage volume varies for each case. Improved techniques to obtain an adequate amount of blood also should be developed.

Blood Loss, Surgical↗

[Effects of prenatal exposure to phthalate ester on both testicular descent and urogenital development in rats].

Mono-n-butyl phthalate (MBP) was administered to pregnant rats to investigate the effect of phthalate ester on both testicular descent and urogenital development in prenatal rats. Ten pregnant rats were separated into two groups. In-group 1; rats were fed with special rat chow containing 1% of MBP from the 14th to the 19th gestational days. In group 2; rats fed with normal rat chow were used as control. At birth, reduced anogenital distance was seen in MBP-treated male offspring, and additional adverse effects on androgen-dependent organs were seen at the age of 70-80 days. Undescended testis, hypospadias, short prepuce, prostatic hypoplasia and hypolastic seminal vesicles were seen in mature male offspring. The results suggest that prenatal administration of MBP may act as an antiandrogenic chemical and thereby inhibit testicular descent and urogenital development in rats.

Animals↗

Roles of nucleosides and nucleotide mixture in small bowel transplantation.

OBJECTIVES: We investigated the effect of nucleosides (NSs) and nucleotides (NTs) on the intestine and intestinal graft in a model of syngenic small bowel transplantation, with the fetal rat intestine as a graft. METHODS: Two-centimeter jejunal segments from Lewis rats at 19 d of gestation were transplanted into the abdominal walls of 5-wk-old Lewis rats by using a non-vascular anastomotic technique. After transplantation, the rats were assigned to one of two groups: group 1 did not receive NS or NT and group 2 was supplemented with NS and NT. The grafts and graft recipients were examined morphologically 14 d after transplantation according to conventional histologic and immunohistochemical studies of neurons and smooth muscles. RESULTS: Group 1 gained little body weight, even though both groups received similar amounts of food. The grafts in group 1 showed poor development in length, diameter, and wet weight. They also showed poor villi development, abnormalities in nerve distribution, and degeneration of muscle layer structure on histologic and immunohistochemical studies. CONCLUSIONS: We found that NS and NT are essential nutrients for intestinal growth and maintenance of structures in fetal small bowel transplantation.

Animals↗

Mutations in the sulfonylurea receptor gene in relation to the long-term outcome of persistent hyperinsulinemic hypoglycemia of infancy.

BACKGROUND: A 95% pancreatectomy has become the mainstay of surgical therapy for patients with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) who did not respond to medical therapy. However, a high incidence of diabetes recently has been reported after a 95% pancreatectomy. Mutations of the SUR1 (sulfonylurea receptor) or Kir 6.2 (inwardly rectifying potassium channel) genes also have been detected in some patients with nesidioblastosis. METHODS: Six infants underwent a subtotal pancreatectomy (about 80%) for the initial surgical treatment of PHHI between 1 and 6 months of age. The clinical follow-up ranged from 2 years to 23 years (mean, 14 years). Mutations of the SUR1 and Kir 6.2 genes were examined in whole exons by the PCR-SSPC method using DNA extracted from white blood cells. RESULTS: SUR1 mutations were found in 5 of the 6 cases (83.3%), whereas no Kir 6.2 mutations were detected. Four of the 5 cases were found to have hetero-type mutations. These 4 cases and the 1 case without mutation were a pathologically focal type (head, 1; body, 2; tail, 2) and showed euglycemia after the operation. The other case was found to have a homo-type mutation and was pathologically diffuse. This case showed hypoglycemia and required medical treatment for several years. Diabetes developed 10 years after surgery. CONCLUSIONS: In the patients with either a hetero-type mutation or no mutation of the SUR1 gene, a focal type is suspected, whereas a homo-type mutation is considered to be associated with a diffuse type and also is a predictor of poor blood sugar control and a tendency toward diabetes. A genetic analysis of the SUR1 gene using peripheral white blood cells is considered a useful parameter to determine the optimal surgical strategy for the treatment of PHHI.

ATP-Binding Cassette Transporters↗

Assessment of sphincter muscle function before and after posterior sagittal anorectoplasty using a magnetic spinal stimulation technique.

PURPOSE: The authors used a magnetic spinal stimulation technique to assess both the preoperative sphincter function and the effect of operative intervention on the sphincter muscle in patients with anorectal malformations. METHODS: The authors examined 5 patients with an anorectal malformation consisting of 4 high-type cases and 1 intermediate-type case, that all had undergone posterior sagittal anorectoplasty during the previous 2-year period. Control data also were obtained from 11 age-matched healthy children. A contraction of the external anal sphincter (EAS) was evoked by magnetic stimulation of the sacral roots with a figure-of-eight coil at the level of the lumber and sacral segments. EAS electromyograms then were recorded at the anal verge. RESULTS: The preoperative motor latencies at L4 stimulation increased significantly more in the patients than in the controls (5.9 +/- 0.5 msec v 4.1 +/- 0.7 msec during left side recording). In the postoperative examination (1 to 5 months after operation), the mean maximum amplitude decreased slightly without any significant difference, where as the latency was almost identical to the preoperative data. CONCLUSION: These results suggest that the neurologic function of EAS was congenitally disturbed, and the current operative technique had no significant effect on the sphincter function.

Anal Canal↗

Stephen L. Gans overseas lecture. Mass screening for neuroblastoma in Japan: lessons learned and future directions.

BACKGROUND/PURPOSE: Since 1985, a nationwide mass screening program (MS) for neuroblastoma has been conducted for 6-month-old infants throughout Japan, resulting in the detection of more than 1,900 cases of neuroblastoma. The outcome of these patients has been excellent: more than 97% of them are alive. Yet, several reports suggest that the number of advanced-stage neuroblastoma patients over 1 year of age has not changed substantially. The current report focuses on the 15-year experience with MS of the Kyushu Pediatric Oncology Study Group. METHODS: The clinical and biological features of neuroblastoms detected (n = 320) and not detected by MS (n = 245) were compared. Regional and national statistics for neuroblastoma before and after 1985 were analyzed using standard epidemiologic measures for the occurrence of disease. RESULTS: The majority of the MS-positive cases were biologically favorable and had an excellent outcome. In contrast, the majority of non-MS patients in whom neuroblastoma later developed had advanced-stage, unfavorable-prognosis tumors. The overall mortality rate of neuroblastoma in the Kyushu area was not improved by MS. CONCLUSIONS: The optimal time for screening is the point at which neuroblastomas regressing spontaneously can no longer be detected, but more aggressive disease can be found. A birth cohort study could determine the optimal timing for a second screening. Identification of other new prognostic factors may be required.

Adrenal Gland Neoplasms↗

Effects of the prenatal administration of cimetidine on testicular descent and genital differentiation in rats.

BACKGROUND: Cimetidine has been shown to induce gonadal and sexual dysfunction in men. This study aimed to clarify the effect of cimetidine on testicular descent and genital development in utero. METHODS: Pregnant rats (280 to 330 g) were separated into 4 groups. In group 1, the rats were injected subcutaneously with a dose of 600 mg/kg of cimetidine twice on days 15, 16, 17, and 18 of gestation. In group 2, the rats were injected with a dose of 300 mg/kg of cimetidine twice in the same way as in group 1. In group 3, the rats were injected with a dose of 150 mg/kg of ranitidine twice in the same manner as in the above groups. In group 4, the rats were injected with the same volume of saline. At birth the anogenital distance was measured, and the incidence of testicular descent was examined in male offspring at 90 days of age. Next, the testes were weighed and histologically examined. The chi-square test and Student t test were used to perform statistical analyses of the results. RESULTS: At birth, the anogenital distance (AGD) index in male offspring decreased significantly in groups 1 and 2 in comparison with groups 3 or 4. However, there was no significant difference in the AGD index between groups 3 and 4. At 90 days of age, the incidence of cryptorchidism was 55.3% in group 1 and 25.9% in group 2. In contrast, all testes descended into the scrotum in both groups 3 and 4. The incidence of cryptorchidism was significantly higher in group 1 than in groups 2 (P <.05), 3, or 4 (P <.01), while the incidence of cryptorchidism was also significantly higher in group 2 than in groups 3 or 4 (P <.01). The weight of the testis decreased significantly more in the undescended testis than in the scrotal testis (P <.01); however, there was no significant difference in the weight of the scrotal testes among the 4 groups. The histologic development of the undescended testes showed an absence of germ cells and smaller tubes in comparison with those of scrotal testes, which showed an advanced differentiation of germ cells and larger tubes in all 4 groups. CONCLUSIONS: These findings suggest that the antiandrogenic effect of cimetidine inhibits both the testicular descent and genital differentiation in fetuses and also causes cryptorchidism in postnatal rats. These same events might also occur in human fetuses when high doses of cimetidine are administered to pregnant women around the end of the first trimester.

Age Factors↗

Segmental small-intestinal transplantation: a comparison of jejunal and ileal grafts.

Strong immune responses, such as rejection and graft-versus-host disease, have been major obstacles to achieving a successful intestinal transplantation. Segmental small-intestinal transplantation is considered to result in a weaker response than total length grafting. Problems relating to organ harvesting from a living donor and spacial constraints of the recipient's abdominal cavity are the other reasons why a segmental small intestinal transplantation is required. It is also important to select the most suitable part of the intestine to be used in transplantation; therefore, the jejunal and ileal grafts were comparatively reviewed from the aspects of native bowel characteristics, function, preservation, and immunological response. Immunologically, the jejunum is considered to have a slight advantage over the ileum. However, the control of rejection does not become inordinately more difficult for ileal grafts. Functionally, the ileum is considered to be better with regard to several parameters, such as greater absorptive capacities of fat, bile acids, and vitamins, and adaptation potential. Anatomically, an ileal graft is feasible for living-related transplantation. However, there is no conclusive factor for choosing the ileum as a suitable segmental graft. Further extensive studies are still needed to clarify various experimental as well as clinical aspects regarding intestinal transplantation.

Animals↗

Electrophysiological properties of the aganglionic segment in Hirschsprung's disease.

BACKGROUND: In Hirschsprung's disease, the severity of bowel obstruction varies among those patients who have the affected colon of a similar length, suggesting that there is more than a simple aperistaltic obstruction in the pathophysiology of Hirschsprung's disease. METHODS: A series of our electrophysiological studies of the aganglionic segments from human specimens and rat models were reviewed to obtain an overview of Hirschsprung's disease. RESULTS: In human studies, a generation of regular spontaneous activity was recorded in both the dilated ganglionic segment and transitional aganglionic region, while the smooth muscle cells of the narrow aganglionic segment were electrically quiescent. According to a pattern of innervation, in the dilated ganglionic segment inhibitory junction potentials associated with or without excitatory junction potentials were observed in all of the examined cells, and these intrinsic nervous inputs were gradually decreased in the transitional region. In the narrow aganglionic segment, only excitatory junction potentials of the extrinsic nervous origin were found in about 20% of the examined cells In rat models, distally increasing tendency of the excitatory nervous inputs was observed in the narrow aganglionic segment. CONCLUSION: A bowel obstruction in Hirschsprung's disease might be generated due to complex mechanisms involving myogenic and neurogenic abnormalities.

Animals↗

Evaluation of genetic heterogeneity in neuroblastoma.

BACKGROUND AND METHODS: The prognosis in neuroblastoma, which is the most common solid tumor in children, tends to vary greatly, and many studies have demonstrated both clinical and biological factors to be closely correlated with the outcome. In order to select the optimal treatment according to the degree of malignancy of neuroblastoma, it is essential to accurately and rapidly identify any genetic heterogeneity associated with the prognosis. We assessed the status of some genetic abnormalities (MYCN amplification, deletion of the short arm of chromosome 1, DNA ploidy, and a gain of the chromosome 17q region) associated with the prognosis using several molecular biological methods. RESULTS AND CONCLUSIONS: The combination of several molecular biological techniques is thus considered to be useful for elucidating the degree of malignancy of neuroblastoma. In particular, diagnostic analyses based on a combination of the fluorescence in situ hybridization (FISH) method and the quantitative polymerase chain reaction (PCR) method may be considered to be the most effective methods for quickly and accurately evaluating any aberrations in the gene dosages associated with the patients' outcomes.

Chromosomes, Human, Pair 1↗

Changing profile of parenteral nutrition in pediatric surgery: a 30-year experience at one institute.

BACKGROUND: Due to technical refinements and steady advances in the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients, parenteral nutrition (PN) is now playing an important role in patient management. However, some PN-associated complications, such as catheter-related sepsis (CRS) and cholestasis, continue at high incidence, particularly in neonates. The objective of this study was to investigate the changing profiles of PN over the past 30 years in our department. METHODS: The medical records of 893 children (225 neonates, 245 infants, 261 preschool-age children, and 162 school-age children) who were placed on PN for >7 days in our department were reviewed, and the following data were extracted: birth weight, underlying disease, indications for PN, PN delivery route, type of catheter used, duration of PN, substrate and energy intake, type of amino acid solution used, and incidence of complications including CRS and liver dysfunction. The results were analyzed by dividing the patients into 3 groups according to their basic stages in management of PN and consisted of group 1 (1970 to 1979), group 2 (1980 to 1989), and group 3 (1990 to 1999). The parameters were compared in each group. RESULTS: The total number of patients in each group showed no significant difference; however, the percentage of low birth-weight neonates increased in group 3. In group 1, 85% of PN was administered through the peripheral vein; in group 2, 51.2%; and in group 3, 9.7%. The total calorie and nutrient intake decreased in groups 2 and 3 compared with group 1, particularly regarding fat intake. In groups 1 and 2, commercially available amino acid solution based on the Food and Agriculture Organization/World Health Organization formula was usually used as the nitrogen source, but in group 3, it was changed to an amino acid solution for children. CRS decreased significantly, particularly in neonates, and occurred at a rate of 45.4% in group 1, 10.7% in group 2, and 1.5% in group 3. The incidence of liver dysfunction also showed a decrease: 35.7% in group 1, 22.3% in group 2, and 18.0% in group 3. A multivariate analysis showed a strong relationship between PN-related liver dysfunction and the duration of PN, the presence of infection, and the type of amino acid solution used. CONCLUSIONS: PN via central venous catheters has been regarded as safe and effective treatment in pediatric surgical patients. Over the past 30 years, the incidence of CRS has decreased. However, PN-related liver dysfunction remains a problem, particularly in patients receiving long-term PN.

Amino Acids↗

Phase I study of irinotecan in pediatric patients with malignant solid tumors.

PURPOSE: To determine the dose-limiting toxicity, maximum tolerated dose, and potential efficacy of irinotecan in children with refractory malignant solid tumors. PATIENTS AND METHODS: In the present phase I clinical trial, 28 patients received irinotecan 50 to 200 mg/m2 per day by intravenous 2-hour infusion over the course of 3 days, repeated once after an interval of 25 days. Fifty-one treatment courses were administered to these patients. RESULTS: Dose-limiting toxicities were observed at the dose of 200 mg/m2 per day for 3 days. Diarrhea and hematopoietic toxicities were the dose-limiting factors, and the former required support with intravenous fluid administration. The occurrence of vomiting was variable. Decreases in clinical tumor marker levels were observed in the majority of patients who received two cycles of irinotecan 80 mg/m2 per day to 200 mg/m2 per day over the course of 3 days, and partial response was attained in four patients who received irinotecan in two cycles of 140 mg/m2 per day to 200 mg/m2 per day over the course of 3 days. Pharmacokinetic studies showed that the plasma concentration of irinotecan and its active metabolite SN-38 ranged from 93 to 2,820 ng/mL and 5.2 to 34.8 ng/mL, respectively, during 3-day infusions of irinotecan 200 mg/m2 per day. The mean clearance of irinotecan was 14.54 L/h per m2 (range 8.45-20.83 L/h per m2). CONCLUSION: The maximum tolerated dose was determined to be a dose of irinotecan between 160 mg/m2 per day and 180 mg/m2 per day administered over the course of 3 consecutive days on an inpatient basis, repeated once after 25 days off, and our results indicate that irinotecan is a promising anticancer agent that is worthy of phase II trials in pediatric solid tumors.

Adolescent↗