池田 太郎

症例は3歳女児.1歳時に十二指腸球部後壁に潰瘍を認め,内服加療にて症状改善し,外来にて経過観察となった.3歳時に腹痛が再燃し,初回時と同部位に潰瘍認め,同時に急性膵炎の所見も認めた.血清ガストリン値は2,800pg/mLと高値を示した.CT・MRI検査にて膵頭部前方に嚢胞性腫瘤を認め,^<99m>Tc-pertechnetateシンチグラフィにて同部位に集積を認めたために十二指腸重複症の診断にて手術を施行した.十二指腸球部と膵臓との間に4cmの球状の腫瘤を認め,腫瘤の前壁は全層切除し,十二指腸との共通壁は腫瘤側の粘膜切除を施行した.病理組織診断では十二指腸粘膜を認め,異所性胃粘膜と膵組織を認めた.さらに重複腸管粘膜上皮にガストリン陽性細胞を認めた.血清ガストリン値は術翌日に正常値となった.本症例のように十二指腸潰瘍の症状が先行して認められた報告例は少なく,高ガストリン血症を伴った報告例は無い.

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.

症例は14歳,男児.4歳頃より上腹壁腫瘤を自覚するも放置していた.思春期になり,腫瘤が気になり,違和感を訴えるようになり当科初診した.腫瘤は剣状突起と臍の中間点に認め.超音波検査にて上腹壁ヘルニアの診断となり,手術を行う方針となった.修復術は臍sliding-window法にてアプローチしてヘルニア門の直接縫合閉鎖を行った.術後経過は良好であり,上腹壁ヘルニアに対する臍sliding-window法は安全で,整容性にも優れており良い適応と思われた.

超低出生体重児にWilson-Mikity症候群とBeckwith-Wiedemann症候群(BWS)を伴った肝芽腫の1例を報告する.症例は,8か月の女児.BWSに伴う腫瘍のスクリーニング検査で,肝腫瘤と血清alpha-fetoprotein (AFP)高値を指摘され紹介となった.Wilson-Mikity症候群による肺気腫と肺高血圧症により呼吸循環動態が不安定であり,開腹腫瘍生検は行わずに化学療法を開始した.CITA療法2コースの後に肝部分切除を施行した.術後にlow CITA療法4コース行い治療を終了した.本症例は,腫瘍完全切除後もAFP高値が遷延し,また,一過性の上昇を伴ったことより,血清lectin-reactive alpha-fetoprotein (AFP-L3)と合わせて腫瘍切除後の経過を観察した.超低出生体重児にBeckwith-Wiedemann症候群(BWS)を伴った肝芽腫の腫瘍切除後におけるAFPとAFP-L3の推移について報告する.

陰嚢水瘤と診断され経過観察された精巣成熟奇形腫の1例を報告する.症例は,1歳10か月時に右陰嚢水瘤の診断で腹膜鞘状突起高位結紮術と水瘤開放術を施行した.術中に,右陰嚢内に充実性の腫瘤を認め,生検にて成熟奇形腫の診断であった.1歳11か月時に右高位精巣摘除術を施行した.

Differentiation of human neuroblastoma recapitulates neural crest development. In our whole genome DNA methylation screening of tissue-specific differentially methylated regions (T-DMRs) and developmental stage specific differentially methylated regions (DS-DMRs) we reported that the exon 5 CpG island (CpGi) of Zfp206 (human: ZNF206), which was required to maintain embryonic stem cells in a pluripotent state, was one of potent brain and testis-specific T-DMRs in mice. In this study methylation level of the CpG sites at Zfp206-exon 5 CpGi in mouse brain samples at three different developmental stages (15-day-old embryo; E15, new born; NB, 12-week adult; AD) were quantitatively analyzed and it was identified that Zfp206-exon 5 CpGi was the DS-DMRs in mouse brain. In AD brains, ZfP206-exon 5 CpGi was significantly hypomethylated and Zfp206 expression was repressed, compared with E15 and NB brains. Hence, mehtylation level of human 5'-end of CpGi at ZNF206-exon 5, which is homologous CpGi to mice, was analyzed in neuroblastomas. Although all four adrenal samples showed complete methylation at the homologous region, we found the hypomethylation in 7 out of 26 neuroblastomas and a significant association between the hypomethylation and poor prognosis. In neuroblastoma cell lines and specimens, the hypomethylation was also associated with ZNF206 expression. These data indicated that the changes in DNA methylation levels at the Zfp206-exon 5 might be one of the important factors during neuronal development in mice and that the hypomethylation of the homologous region induced ZNF206 expression in humans and was associated with human neuroblastomagenesis. Even though the function of ZNF206 and its expression regulation in neuroblastoma remain elusive. ZNF206 might be a candidate differentiation suppressor and prognosis marker in neuroblastoma.

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&lt;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.

Abdominoscrotal hydrocele (以下，ASH) は陰嚢水腫が鼠径管を介し，腹腔内に交通しているまれな疾患であり，その発生機序と治療法は未だ一定の見解は得られていない．今回，我々は ASH を鼠径管アプローチに腹腔鏡を併用することで完全切除した症例を経験した．症例は 11 ヵ月の男児．左陰嚢腫大を主訴に来院した．超音波検査にて腹腔内に連続する陰嚢水腫を認め，ASH を疑い，MRI にて診断した．手術は腹腔鏡を併用して鼠径法による水腫摘出を行った．術後経過は良好で現在まで再発は認めていない．腹腔鏡を併用することは ASH の形態的評価が可能であり，安全かつ確実に切除することが可能であるため非常に有用である．