池田 太郎

Background. Vasoactive intestinal peptide (VIP) is released by stimulation of nonadrenergic noncholinergic (NANC) inhibitory nerves. In order to evaluate the function of VIP in jejunal isografts, we examined the enteric nerve responses in isografted rat jejunum compared with normal jejunum. Methods. Orthotopic entire small bowel transplantation (SBT) with portocaval drainage was performed from Lewis rats to Lewis rats. Grafted tissue specimens were obtained 130 days after SBT (n = 8). As controls, normal segments of the jejunum were obtained from nontransplanted Lewis rats (n = 20). A mechanograph was used to evaluate in vitro jejunal responses to electrical field stimulation (EFS) of the adrenergic and cholinergic nerves before and after treatment with various autonomic nerve blockers and VIP. Results. The isografted jejunum was more strongly innervated by excitatory nerves, especially NANC excitatory nerves, than the normal jejunum (P < .05). VIP mediated relaxation reactions of NANC inhibitory nerves in the normal but to a lesser extent in the isografted jejunum (P < .05). Conclusions. The increased NANC excitatory nerves and the decreased effects of VIP in mediating NANC inhibitory nerves may largely relate to the peristaltic abnormalities seen in the isografted LEW rat jejunum.

【目的】当院で経験した食道閉鎖根治術を行った24例について検討し,術後成績に影響する因子を明らかにすることを目的として検討した.【対象と方法】1975年1月から2003年9月までに当科で経験した先天性食道閉鎖症36例中,根治術を行った24例を生存例17例(A群)と死亡例7例(B群)の2群に分けて,Waterston分類,Montreal分類,Spitz分類,IUGRの有無,羊水過多の有無,妊娠週数,分娩法,出生体重,Apgar score, Retraction score(Silverman-Anderson),盲端に終わる上下食道間の距離(以下GAP)/出生身長,合併奇形ならびに術前の合併症,術後合併症,死亡時期や死因等について検討を加えた.【結果】根治術を行った24例全例がGross C型で,A群は17例(70.8%),観察期間は,術後1年から28年で平均15.0年.B群は7例(29.2%),術後4日目から382日目,平均135日目で死亡にいたった.B群の直接死因においては,呼吸器系合併症の占める割合が高かった.出生前では,IUGR,羊水過多,妊娠週数,分娩法においてA群とB群に有意差は認めなかった.出生後では,出生体重,Apgar score, Retraction score,呼吸窮迫症候群(以下RDS)の有無に有意差を認めた.手術時では,GAP/出生身長はB群において有意に高かった.B群の7例中5例の直接死因は術後の呼吸器系合併症であり,肺炎を高頻度に合併していた.われわれの症例をWaterston分類,Montreal分類,Spitz分類と比較すると,Waterston分類とSpitz分類においてその妥当性に有意差を認めた.【結論】先天性食道閉鎖症の手術症例において,出生体重と術前呼吸器合併症がその予後に強く関係し,Waterston分類,Spitz分類による危険度分類が適切であると思われた.術前術後を通じて,呼吸器系合併症をコントロールすることが予後の向上に重要であることが示唆された.

VATER associationとは胎生期の中胚葉系分化異常による先天奇形で,椎骨異常,鎖肛,気管食道瘻,橈骨あるいは腎の異形成のうち3要素以上を1個体に認める症例をいう.われわれの調べた限りでは,本邦におけるVATER associationの報告は65例であり,1個体に4要素のすべてが合併した完全型としての報告例は比較的少ない.当施設で経験したVATER associationの完全型2例について文献的考察を加えて報告する.いずれの症例も先天性食道閉鎖症Gross分類でC型の患児で,症例1は仙骨形成異常,鎖肛,橈骨欠損および総動脈幹症を合併し,日給1に胃瘻造設術および人工肛門造設術を施行したが,心奇形による心不全により根治術を施行することなく日給155に死亡した.症例2は二分脊椎,仙骨形成異常,鎖肛,左腎無形成を合併し,日齢0に胃瘻造設術,人工肛門造設術および一期的根治術(気管食道痩切離閉鎖術,食道食道端々吻合術)を施行し,現在も入院加療中である.

We describe a case of cord blood harvest for autologous transfusion in a neonate weighing 3,992 g with a giant sacrococcygeal teratoma. The umbilical vein was pierced with an 18-gauge needle, and placental blood was withdrawn into two 50-ml syringes filled with 4 ml of citratephosphatedextrose solution. Resection of the sacrococcygeal teratoma was performed on day one. During the operation the infant lost 46 ml of whole blood, more than 15% of the estimated total blood volume, and thus underwent autologous transfusion with 27.8 ml of packed red cells obtained from autologous cord blood. Consequently, she could avoid homologous blood transfusion during the hospital stay. This case highlights the safety of this procedure, with no evidence of consumption coagulopathy, hemolysis or bacterial infection.

Background/Aims: To clarify the significance of upper gut motility for Hirschsprung's disease and its allied disorders in adults, we studied the upper esophagogastroduodenal motility of adult patients with Hirschsprung's disease and its allied disorders such as hypoganglionosis and intestinal neuronal dysplasia. Methodology: Twelve patients (7 men and 5 women, aged between 20 and 55 years with a mean age of 39.6 years) with Hirschsprung's disease (2 cases) or its allied disorders (8 cases of hypoganglionosis and 2 cases of intestinal neuronal dysplasia) were studied. As a control, 15 healthy volunteers (8 men and 7 women aged between 27 and 69 years with a mean age of 49.0 years) were also examined. To obtain the upper gut motility in Hirschsprung's disease, hypoganglionosis, and intestinal neuronal dysplasia, we performed gastrointestinal transit time study, esophageal manometry, and gastroduodenal manometry. Results: On gastrointestinal transit time, barium stagnated in the upper jejunum in 2 cases of hypoganglionosis, in the terminal ileum in one case of hypoganglionosis and intestinal neuronal dysplasia, and in the colon in the remaining patients. In two of the 12 cases of Hirschsprung's disease and its allied disorders such as hypoganglionosis and intestinal neuronal dysplasia, abnormal esophageal motilities, and absence of interdigestive migrating motor complex, phase III from the stomach were observed. These findings suggested that the entire digestive tract might have been affected in these two cases, i.e., these 2 patients had total gut involvement type of hypoganglionosis. Conclusions: Gastrointestinal transit time and upper esophagogastroduodenal manometry should be performed because of the relatively frequent association of upper gut dysmotilities with these disorders.

Background/Aims: To accurately diagnose for Hirschsprung's disease and its allied disorders in adults, we studied the histology and clinical future of 12 adult patients with prolonged, refractory constipation with abdominal distension and pain. I Methodology: Based on clinical signs and symptoms noted on admission, all of 114 patients (12 males and 104 females, aged 20-74 years with a mean age 4 56.6 years) were suspected to have refractory chronic constipation. To obtain an accurate diagnosis, we performed rectal biopsy. Tissue samples were frozen and 12-mum sections were stained with hematoxylin-eosin, with acetylcholinesterase by the method of. Karnovsky and Roots, and with NADPH-diaphorase by the modified Scherer-Singler's method. Results: 1) Histological examinations; On the basis histological studies (rectal biopsies), 8 were diagnosed with hypoganglionosis, 2 with Hirschsprung's disease, and 2 with intestinal neuronal dysplasia. It was possible to, diagnose Hirschsprung's disease and intestinal neuronal dysplasia using rectal mucosal biopsies with hematoxylin-eosin and acetylcholinesterase, staining. However, accurate diagnosis of hypoganglionosis could be made only through examination of the myenteric plexus by NADPH-diaphorase staining in full-thickness rectal. specimens. 2) Clinical symptoms; All patients had refractory chronic constipation with abdominal pain and distension. Two patients with Hirschsprung's disease had constipation neonatally, Of the 8 patients with hypoganglionosis, one had constipation neonatally at sucking age, 2 as infants, 2 at school age, and 2 after operation as adults. Two patients with intestinal neuronal dysplasia had constipation while infants. Onset of signs and symptoms before school age was significantly revealed than that found after. operation as adults (P<0.01). Frequency of bowel movements was 1/7-10 days for Hirschsprung's disease, 1/7-14 days for hypoganglionosis, and 1/7-30 days for intestinal neuronal dysplasia. Conclusions: We were able obtain accurate histological diagnosis of patients with Hirschsprung's disease and intestinal neuronal dysplasia by rectal mucosal biopsy with hematoxylin-eosin and acetylcholinesterase staining. Patients with hypoganglionosis obtained accurate histological diagnosis by fall-thickness rectal biopsy with NADPH-diaphorase staining. Onset of symptoms of disease occurred predominantly before school age. In all of the patients, bowel movements occurred less than once per week.

Background/Aims: To accurately diagnose for Hirschsprung's disease and its allied disorders in adults, we studied the histology and clinical future of 12 adult patients with prolonged, refractory constipation with abdominal distension and pain. I Methodology: Based on clinical signs and symptoms noted on admission, all of 114 patients (12 males and 104 females, aged 20-74 years with a mean age 4 56.6 years) were suspected to have refractory chronic constipation. To obtain an accurate diagnosis, we performed rectal biopsy. Tissue samples were frozen and 12-mum sections were stained with hematoxylin-eosin, with acetylcholinesterase by the method of. Karnovsky and Roots, and with NADPH-diaphorase by the modified Scherer-Singler's method. Results: 1) Histological examinations; On the basis histological studies (rectal biopsies), 8 were diagnosed with hypoganglionosis, 2 with Hirschsprung's disease, and 2 with intestinal neuronal dysplasia. It was possible to, diagnose Hirschsprung's disease and intestinal neuronal dysplasia using rectal mucosal biopsies with hematoxylin-eosin and acetylcholinesterase, staining. However, accurate diagnosis of hypoganglionosis could be made only through examination of the myenteric plexus by NADPH-diaphorase staining in full-thickness rectal. specimens. 2) Clinical symptoms; All patients had refractory chronic constipation with abdominal pain and distension. Two patients with Hirschsprung's disease had constipation neonatally, Of the 8 patients with hypoganglionosis, one had constipation neonatally at sucking age, 2 as infants, 2 at school age, and 2 after operation as adults. Two patients with intestinal neuronal dysplasia had constipation while infants. Onset of signs and symptoms before school age was significantly revealed than that found after. operation as adults (P<0.01). Frequency of bowel movements was 1/7-10 days for Hirschsprung's disease, 1/7-14 days for hypoganglionosis, and 1/7-30 days for intestinal neuronal dysplasia. Conclusions: We were able obtain accurate histological diagnosis of patients with Hirschsprung's disease and intestinal neuronal dysplasia by rectal mucosal biopsy with hematoxylin-eosin and acetylcholinesterase staining. Patients with hypoganglionosis obtained accurate histological diagnosis by fall-thickness rectal biopsy with NADPH-diaphorase staining. Onset of symptoms of disease occurred predominantly before school age. In all of the patients, bowel movements occurred less than once per week.

全大腸炎型活動期の潰瘍性大腸炎(UC)では,上部消化管運動異常があり,下痢を伴う頻便は胃排出機能の充進により,増悪される可能性が報告されている.そこで,全大腸炎型活動期重症UC5例(男性4例,女性1例,29～49歳,平均38.6歳)について,健常人14例(男性10例,女性4例,24～46歳,平均35.8歳)を対照に,半固形食胃排出機能(全粥に^99mTc標識ラジオアイソトープ法)と液体食胃排出機能(オレンジジュースを用いたアセトアミノフェン法)を検討した.その結果,UCでの半固形食および液体食の胃排出曲線はともに,対照と有意差なく類似の排出パターンを示した.以上より,全大腸炎型活動期重症UCの胃排出機能は正常に機能しており,頻便には関与していないものと思われた.

Background/Purpose: Although angiogenic factors may play an important role in the biology of neuroblastoma, which frequently spreads hematogenously, the mechanism remains unclear. The authors studied tumor progression and invasion from the perspective of angiogenesis and sought to understand the features of this type of tumor. Methods: Thirty-one specimens were resected from patients with neuroblastoma and the expression of vascular endothelial growth factor (VEGF), and its receptor (Flk-1) was examined using immunohistochemistry. The authors looked for correlations among the expressions of VEGF and its receptor with various clinicopathologic factors. In addition, they examined the expression and location of VEGF and Flk-1 mRNA in 10 primary neuroblastoma using in situ hybridization. Results: Both in situ hybridization and immunohistochemistry showed the presence of VEGF expression within the neuroblastoma cells. We found VEGF mRNA in neuroblastoma cells but not vascular endothelial cells according to in situ hybridization. Further, Flk-1 mRNA was present both in neuroblastoma cells and vascular endothelial cells. The level of VEGF expression was higher in unfavorable histology, using the criteria of Shimada, than in favorable histology. Conclusion: The authors suggest that paracrine and autocrine systems are involved in the angiogenesis of neuroblastoma, and the expression of VEGF correlates with the prognosis in neuroblastoma. Copyright 2002, Elsevier Science (USA). All rights reserved.

Background/Aims: The cause of impaired motility in patients with slow transit constipation is unknown. To clarify the physiological significance of cholinergic, adrenergic, non-adrenergic non-cholinergic inhibitory nerves in the colon of patients with slow transit constipation, we investigated the enteric nerve responses on lesional and normal bowel segments derived from patients with slow transit constipation and patients who underwent colon resection for colonic cancers. Methodology: Twenty preparations were taken from the lesional colon of 6 patients with slow transit constipation (2 men and 4 women, aged 23 to 68 years, with a mean age of 44.0 years). Thirty-six preparations were taken from the normal colon of 12 patients with colonic cancer (6 men and 6 women, aged 40 to 60 years, with a mean age of 52.2 years). A mechanographic technique was used to evaluate in vitro muscle responses to acetylcholine, adrenalin, electrical field stimulation of adrenergic and cholinergic nerves before and after treatment with various autonomic nerve blockers. Results: The contraction reaction to acetylcholine in the colon with slow transit constipation was significantly weaker than in the normal colon (P<0.01). The relaxation reaction to adrenalin in the colon with slow transit constipation was stronger than in the normal colon. The colon with slow transit constipation was more strongly innervated by non-adrenergic non-cholinergic inhibitory nerves than the normal colon, significantly (P<0.05). Conclusions: These findings suggest that a decrease of cholinergic nerve and an increase of nonadrenergic non-cholinergic inhibitory nerve play an important role in the impaired motility observed in the colon of patients with slow transit constipation.

小児期に分流手術を施行した先天性胆道拡張症で術後10年以上を経過した40例を対象に,肝門部の左右肝管,肝内胆管の拡張形態を検討し,その問題点を考察した.その結果,肝門部の左右肝管,肝内胆管に拡張を認める症例では,左右肝管合流部の狭窄がみられるものが多く,拡張形態がcyst typeのものでは全例に左右肝管合流部狭窄が認められた.肝内胆管の拡張と狭窄は5例に認められ,そのうち1例は術後肝内結石が認められた.肝門部の左右肝管,肝内胆管がcyst typeの場合,拡張及び狭窄により胆汁うっ滞を招来し易い.治療にあたっては,胆汁流出路に狭窄部が残存せぬように注意することが重要である

PURPOSE: The cause of dysmotility, in patients with slow-transit constipation is unknown. Nitric oxide has recently been shown to be a neurotransmitter in the nonadrenergic, noncholinergic inhibitory nerves of the human gut. To clarify the physiologic significance of nitric oxide in the colon of patients with slow-transit constipation, we investigated the enteric nerve responses in lesional and normal bowel segments derived from patients with slow-transit constipation and patients who underwent colon resection for colonic cancers. METHODS: Twenty-six preparations were taken from colonic lesions in eight patients with slow-transit constipation (2 men; age, 23 to 69 (mean, 44.8) years). Forty-two preparations were taken from the normal colons of 14 patients with colonic cancer (8 men; age, 40 to 66 (mean, 52.4) years). A mechanographic technique was used to evaluate in vitro muscle responses to electric field stimulation before and after treatment with various autonomic nerve blockers, N-G-nitro-L-arginine, and L-arginine. RESULTS: The colons of patients with slow-transit constipation were more strongly innervated by nonadrenergic, noncholinergic inhibitory nerves than were normal colons (P < 0.05). Nitric oxide was found to act on both normal and slow-transit constipation colons. The colons of patients with slow-transit constipation were more strongly innervated by nitric oxide nerves than were normal colons (P < 0.01). Responses to electric field stimulation were the same in each case among the normal colons and were also the same in each case among the slow-transit constipation colons. CONCLUSION: These findings suggest that an increase of nitric oxide mediates nonadrenergic, nonchotinergic inhibitory nerves and plays an important role in the dysmotility observed in the colons of patients with slow-transit constipation.