熊谷 秀規

BackgroundThe prevalence of occult hepatitis B virus (HBV) infection (OBI) in children due to mother-to-child transmission (MTCT) despite immunoprophylaxis remains controversial and is still unknown in Japan. The aim of this study was to determine the OBI prevalence in such children in Japan and identify the genomic mutations that might be associated with the pathogenesis of OBI in children. MethodsThe data on 158 children born to HBV carrier mothers and who received complete passive-active immunoprophylaxis after birth in 2002-2014 were reviewed. HBV markers were detected using commercial enzyme-linked immunosorbent assay kits. HBV-DNA was detected using real-time and nested polymerase chain reaction. Complete genomic sequences were determined. ResultsAmong the 158 children studied, three had HBV MTCT: two had OBI, and one had resolved HBV infection (RBI). The prevalence of OBI and RBI was estimated to be 1.3% and 0.6%, respectively. The HBV genomes of the two OBI children were wild type and 100% identical to those of their mothers. Of these two children, one received repeated hepatitis B immunoglobulin (HBIG) and developed overt HBV infection. Her HBV genome had a G145R mutation in the S gene that might have been induced by HBIG treatment. The RBI child was persistently positive for antibody to HBV core antigen (10-12 signal/cut-off ratio; S/CO). ConclusionsA low prevalence of OBI was observed in children who received immunoprophylaxis for preventing MTCT in Japan. The development of overt HBV infection in infants with OBI indicates the necessity of close and long-term monitoring.

PURPOSE: Because the true prevalence of food allergy (FA), as based on the results of an oral food challenge test (OFC), is unknown, it is likely that children with suspected FA unnecessarily eliminate potentially causative foods. This study aimed to identify the prevalence of FA and to determine the proportion of children who unnecessarily eliminate food. METHODS: To identify children with FA, a primary survey was conducted via a questionnaire with all children aged 0-18 years in Niijima village (remote islands of Japan). In the secondary survey, a detailed medical interview was conducted by doctors with children who currently did not eat some foods. The third survey involved serum food-specific immunoglobulin E (IgE) tests and an OFC for children with suspected FA. RESULTS: Of 376 enrolled children, 374 (99.5%) completed the questionnaire. Some foods were eliminated by 18.6% and 13.0% of all children and those ≥6 years old, respectively. The target population for the secondary survey included 69 children who all completed the medical interview. The target population for the third survey consisted of 35 children, of whom 26 (74.3%) underwent the blood test. An OFC was performed 35 times with 20 children. As a result, the prevalence of FA was 4.9% in children of all ages and 4.7% in those ≥6 years old. Moreover, 55.0% children were able to cease eliminating food intake. CONCLUSIONS: It is possible that a considerable number of children unnecessarily eliminate food because of suspected FA.

5-Aminosalicylic acid preparations have been used as first-line drugs for treatment of ulcerative colitis (UC). However, some patients with UC present with exacerbation of symptoms because of allergy to mesalazine. Diagnosis of mesalazine allergy in active UC may be challenging because its symptoms mimic those of UC. Here we describe a 13-year-old boy with mesalazine allergy who achieved remission when his medication was changed from mesalazine to salazosulfapyridine. During his clinical course mesalazine was prescribed twice, and on each occasion exacerbation of the symptoms occurred. We considered a diagnosis of mesalazine allergy, and this was confirmed by a drug lymphocyte stimulation test; the result for salazosulfapyridine was negative. On the basis of criteria involving simple mucosal biopsy combined with endoscopy for predicting patients with UC who would ultimately require surgery, we considered that the UC in this case might be susceptible to steroid treatment, and we therefore treated the patient with salazosulfapyridine and prednisolone. Shortly afterwards, remission was achieved and the patient has remained in good condition on salazosulfapyridine alone. When treating patients with mesalazine, the possibility of allergy should always be borne in mind, especially when the clinical course is inconsistent with the results of biopsy.

BackgroundOnly a handful of studies have investigated children with functional dyspepsia (FD) and irritable bowel syndrome (IBS) classified according to the Rome III criteria, and limited information is available on the lifestyle of affected patients. MethodsWe conducted an Internet questionnaire survey of 2060 parents among the general public in Japan who lived with their children aged 10-15, who were screened for FD and IBS. ResultsThe prevalence of FD and IBS was 2.8% and 6.1%, respectively, and 1.4% of the subjects met the criteria for both FD and IBS. The lifestyles of 155 subjects who met the criteria for FD, IBS, or both were compared with those of 1745 control subjects. In comparison with the controls, a significantly higher percentage of subjects with FD, IBS, or both thought that their sleep was insufficient, ate meals irregularly, were susceptible to stress and to dizziness on standing, had difficulty in getting out of bed or felt sluggish in the morning, had a tendency to faint when standing, and had migraine/chronic headache. ConclusionsChildren with FD and IBS are susceptible to stress, have impaired sleep and eating habits, and have more frequent symptoms of comorbid orthostatic dysregulation and headache.

Objectives: The safety and efficacy of double-balloon enteroscopy (DBE) in pediatric patients has not been well documented. We aimed to evaluate the clinical efficacy and safety of DBE in children, especially those under 10. Methods: We retrospectively analyzed our database of DBE procedures performed between September 2000 and September 2013. Procedures performed in pediatric patients (under 18) were selected from a total of 3980, including double-balloon endoscopic retrograde cholangioscopy (DBERC). Results: Two hundred fifty-seven DBE procedures were performed in 117 pediatric patients (median age 12.5 years). Antegrade (oral-route) DBE was performed in 166 procedures including 104 DBERC procedures (lowest body weight 13.5 kg, youngest age 3 years), and retrograde (anal-route) DBE in 91 (lowest body weight 12.0 kg, youngest age 2 years). The overall diagnostic yield for obscure gastrointestinal bleeding and abdominal pain was 58.8%. The purpose of DBERC was achieved in 76.9% of procedures. The overall complication rate in our series was 5.4% (1.9% with the DBERC cases removed); in patients under 10, it was 10.4% (7/67). No severe complications associated with enteroscope insertion and sedation were observed. Serum amylase levels tended to be elevated in patients who underwent oral-route DBE. Conclusions: DBE is safe and feasible for diagnostic evaluation of small bowel disorders in pediatric patients, even those younger than 10 years. Special attention for possible complications must, however, be paid during therapeutic DBE procedures, including DBERC, especially for patients under 10.

Fecal microbiota transplantation (FMT) is a treatment designed to correct gut dysbiosis by administration of feces from a healthy volunteer. It is still unclear whether FMT for children with ulcerative colitis (UC) is effective or hazardous. Here we describe a young patient to have received FMT for UC. A three-year-old girl was admitted to our hospital with severe active UC, and treated with aminosalicylates and various immunosuppressive drugs. As remission was not achieved, we decided to try FMT before colectomy. We administered donor fecal material a total of six times by retention enema (×2) and via a nasoduodenal tube (×4) within 10 days. The patient developed abdominal pain and pyrexia after each FMT session. Analyses revealed the transferred donor fecal microbiota had not been retained by the patient, who ultimately underwent colectomy. The severity of the UC and/or timing of FMT may have partly accounted for the poor outcome.

BACKGROUND: The Ogasawara Islands, away from mainland Japan, belong to a subtropical area. Although the daily eating habits and food are relatively similar to that on the mainland, the living environment is quite different. The prevalence of allergic diseases in the Ogasawara Islands is unknown. This study aimed to identify the prevalence of allergic diseases in the Ogasawara Islands. METHODS: A survey was conducted among all children belonging to preschool, elementary school, and junior high school in the Ogasawara Islands. A questionnaire was prepared in accordance with the International Study of Asthma and Allergies in Childhood (ISAAC) core written questionnaire in bronchial asthma (BA) and the West Japan Study of Asthma and Allergies in Childhood core written questionnaire for atopic dermatitis (AD), allergic rhinitis (AR), and food allergy (FA). At the same time, height, weight, duration of dwelling on the island, home environment, lifestyle, and exercise habits were also asked. RESULTS: The target population comprised 352 children, of whom 284 (80.6%) completed the questionnaires. The current prevalence was 9.3% for BA, 4.3% for AD, 17.8% for AR, and 3.0% for FA. Significantly lower rates of current BA and AD were observed compared to previous reports from Japan. The percentage of children belonging to sports clubs, and exercising more than 3 times per week at the Ogasawara Islands is higher compared with the national average. CONCLUSIONS: The lower prevalence of BA and AD in the Ogasawara Islands implies the influence of differences in the living environment and exercise habits.

Introduction: Patients with severe mycoplasma pneumonia having very high serum interleukin-18 levels may require systemic corticosteroid treatment. However, we know of no laboratory markers that have been identified to assess the precise severity of Mycoplasma pneumoniae pneumonia. Thus, we investigated the usefulness of four clinical laboratory tests as severity indicators and surrogate markers for initiation of steroid therapy in these patients. Patients and methods: For 22 Japanese children (including 3 patients who needed systemic corticosteroid therapy) diagnosed with Mycoplasma pneumoniae pneumonia, white blood cell counts and serum concentrations of interleukin-18, C-reactive protein, lactate dehydrogenase, and ferritin were determined in the acute and recovery phases. Results: In total, 8 and 14 patients were classified as moderate and mild pneumonia, respectively, according to clinical manifestations. The serum interleukin-18 level in the acute phase of the pneumonia group was significantly higher than that of age-matched controls. Furthermore, serum interleukin-18, lactate dehydrogenase and ferritin levels in the acute phase increased in parallel with the severity of the pneumonia. The serum ferritin level was also higher in the acute phase than in the recovery phase. Positive correlations between the levels of serum interleukin-18, lactate dehydrogenase and ferritin were observed in the acute phase. Conclusions: Serum lactate dehydrogenase and ferritin levels may be useful as indicators of the severity of pediatric Mycoplasma pneumoniae pneumonia for initiation of corticosteroid therapy. (C) 2015, Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Measles is a highly contagious viral infection associated with clinical symptoms such as fever, cough, conjunctivitis, coryza, eruption and increased serum immunoglobulin M (IgM) antibodies. A clinical diagnosis is easily established when the chain of infection can be followed. However, Japan is currently experiencing sporadic measles outbreaks, which complicate the establishment of diagnosis. Furthermore, other exanthematous infections such as rubella, human parvovirus B19, human herpesvirus 6 (HHV-6) and HHV-7 present with clinical symptoms and IgM antibody levels similar to those in measles. Therefore, real-time polymerase chain reaction virogene testing has been part of Japan's standard diagnostic protocol for measles since 2010. This report presents two pediatric cases clinically resembling measles that were diagnosed as HHV-6 based on a virogene detection test. This underscores the importance of performing pathogen testing to confirm a diagnosis when measles is suspected.

我々は、頻回に再発したステロイド依存性潰瘍性大腸炎(UC)の小児に対して、タクロリムス(TAC)による寛解導入を行い、引き続き同薬で約4年間にわたり寛解維持療法を継続している。本例は7歳時にUCと診断し、サラゾスルファピリジンで寛解導入後、維持治療を行った。8歳時に再燃したため顆粒球吸着療法を行い一旦軽快したが、間もなく再燃したためプレドニゾロン(PSL)を用いて再度寛解導入を行った。その後、アザチオプリンを併用したがPSL減量中に2回再燃したため、10歳時にはシクロスポリン持続静注を施行して寛解導入した。11歳時、PSLを減量中止後に6回目の再燃をした。この時点でステロイド総使用量はPSL換算で約10gとなったため、TACで寛解導入を行い、そのまま維持療法を施行している。TACの血中トラフ濃度は3〜5ng/ml程度で臨床的寛解及び大腸粘膜治癒が得られており、現在まで有害事象は認めない。TACは小児の難治性UCに対する寛解維持療法のひとつとして期待できる可能性がある。(著者抄録)

The speed of sound correlates well with the fat content of the liver. Therefore, non-invasive quantification of sound speed in the liver might be of diagnostic value. Here we describe a new non-invasive method that would be clinically applicable for measurement of sound speed in the liver. Sprague-Dawley rats were divided into two groups: a control group and a fatty liver group prepared by keeping the rats on a choline-deficient diet for 6 wk. The livers were subjected to pathologic and biochemical analysis; the speed of sound through the liver tissue was measured using our proposed method and a pulser-receiver as standard. Our results indicated that use of the proposed method makes it feasible to diagnose fatty liver with good accuracy on the basis of sound speed. This approach would have considerable potential for non-invasive diagnosis of fatty liver and would be a valuable adjunct to conventional liver diagnostic procedures. (C) 2014 World Federation for Ultrasound in Medicine & Biology.

トスフロキサシン(TFLX)、ミノサイクリン(MINO)で治療したマイコプラズマ肺炎47例について、その臨床効果を後方視的に検討した。肺炎重症度は、TFLX治療群とMINO治療群の間に有意差を認めなかったが、要解熱日数はTFLX治療群で平均2.5±1.3日とMINO治療群の1.3±0.5日に比べ延長しており、TFLX治療群のうち5例(18%)では解熱に4日以上を要していた。一方でLDH値はTFLX治療群(302±61IU/L)ではMINO治療群(257±61IU/L)よりも有意に高値であった。マイコプラズマ肺炎の治療経過には、治療薬剤の感受性や薬物体内動態のほか、宿主免疫応答が影響すると考えられ、TFLXで治療開始したマイコプラズマ肺炎症例では、発熱や気道症状に対する慎重な経過観察が必要と考えられた。(著者抄録)

目的:本論文では,細径音響導波路を用いて組織の高精細な超音波画像を直接観察可能とする研究について述べる.方法:まず,反射法を用いて石英ファイバ中を伝搬する超音波の挿入損失と周波数特性を測定する.次に,透過法を用いて石英ファイバ中を伝搬する超音波の挿入損失および周波数依存性減衰を調べるために,受信トランスデューサをファイバ端面の近くに設置し,音響カップリング材を媒体として伝搬した超音波の特性を測定する.最後に,上記の測定結果を用いて測定条件を最適化し,水中に置かれた反射体(硬貨)を超音波ビームで走査することによってCモード像を得る.結果:ファイバ端面から反射波が得られ,その周波数特性において最大値を示す周波数は約220MHzであった.音響カップリング媒体を伝搬した超音波の周波数は約125-170MHzの帯域で検出され,超音波の最大検出距離は100MHz周波数帯で約1.2mmであった.最終的には,テーパー状石英ファイバを用いて,水中の硬貨の高周波Cモード像を得ることができた.結論:実験結果から,微細組織像を得るためには,信号対ノイズ比(S/N)を改善し,挿入損失を減少させる必要があることが示唆された.(著者抄録)

症例は4歳男児で、発熱、黄疸、腎機能障害を主訴に、精査加療目的で当科紹介転院となった。検査所見では溶血性貧血、血小板の軽度減少、FDPおよびD-dimerの著明高値、腎機能障害、肉眼的血尿、尿潜血3+、β2MG高値を認めた。直接クームス試験は抗C3d血清で陽性、間接クームス試験は陰性で、寒冷凝集素価は256倍であった。以上より、急性腎障害を合併した低力価寒冷凝集素症と診断し、保温と寒冷刺激回避で対応し、水溶性プレドニンの静注を3日間行い、漸減中止した。さらに輸血、ハプトグロビンの静注、輸液、フロセミド静注、炭酸水素ナトリウム静注などの保存的治療を行ったところ軽快が得られ、腎機能も正常化し、転院20日目に退院となった。

We present the youngest patient reported to date with chronic nonspecific multiple ulcers of the small intestine (CNSU) diagnosed by double-balloon endoscopy (DBE). A 3-year-old girl was referred to our department with a 2-year history of iron-deficiency anemia. Failure to thrive and hypoproteinemia were also noted, and stool occult blood tests had been persistently positive. However, the C-reactive protein level and erythrocyte sedimentation rate were not elevated. Esophagogastroduodenoscopy and double-contrast enema revealed no abnormality in the colon and terminal ileum. Retrograde DBE was performed when the patient was 4 years old. Linear ulcerations arranged in an oblique or circular pattern were present at 3 sites between 55 and 65 cm from the ileocecal valve. Microscopic examination showed nonspecific inflammatory changes, and no granuloma was present. Based on the clinical and endoscopic findings, the patient was diagnosed as having CNSU. The youngest previously reported patient with CNSU was 7 years old, whereas our present patient was diagnosed at the age of 4 years. In pediatric cases of obscure gastrointestinal bleeding, it may be necessary to be aware of small bowel disease. © 2014 Springer.

9歳の女児。学校腎臓病検診で血尿と蛋白尿を指摘され来院した。腹部超音波検査とMR cholangiopancreatographyで、胆嚢は同定できず総胆管が拡張しており、先天性胆嚢欠損症と診断した。無症状ではあるが、総胆管結石症を来す可能性があるため、定期的に腹部超音波検査を施行して経過を観察する方針とした。(著者抄録)

Episodes of blood-streaked stools are not uncommon in exclusively breast-fed infants under 6 months of age. Such bleeding is thought to be associated with food protein-induced proctocolitis, however the pathomechanism remains unclear. The aim of this study was to investigate intestinal microbiota and secretory immunoglobulin A in the feces of exclusively breast-fed infants with blood-streaked stools. Fecal specimens from 15 full-term infants with blood-streaked stools and 15 breast-fed healthy infants were studied and the results compared. All infants had been delivered vaginally and exclusively breast-fed. The fecal microbiota were investigated by phylogenetic analysis combined with culture methods for some bacterial species, and feces were assessed for the presence of fecal secretory immunoglobulin A by enzyme-linked immunosorbent assay. Phylogenetic cluster analysis revealed four major clusters of fecal bacteria, cluster A being found only in healthy infants. The Bacteroides fragilis group was observed more frequently in controls than in patients (P < 0.05). In the controls, the predominant species belonging to the Enterobacteriaceae group was Escherichia coli, whereas in the patients it was Klebsiella (P < 0.05). Concentrations of secretory immunoglobulin A were high in one third of the healthy controls. In conclusion, the pathomechanism of rectal bleeding in exclusively breast-fed infants may be related to differences in the composition of their intestinal flora.

12歳の慢性C型肝炎(HCV-RNA 6.2 logIU/ml:TaqMan法)の日本人女児から分離されたHCV株(HC10-0804)を解析したところ、5'UTR/Core領域はgenotype 2b、NS5B領域はgenotype 1bと高い相同性を示した。3'末端のpoly(U)トラクトを除いた全長9,423塩基を決定し解析したところ、2b/1b recombinant株であることが判明した。crossover pointは非構造領域であるNS2/NS3 junction直後のNS3領域の上流(nucleotide position 3,443/3,444、amino acid position 1,034/1,035)であると推定された。NS5A領域がgenotype 1bであることから、genotype 1b/高ウイルス量に準じてPEG-IFN/RBV併用療法で48週投与を選択した。投与終了後6ヵ月の時点でHCV-RNAの陰性を維持している。一般的に行われている1領域のみの解析による遺伝子型判定では、recombinant株の検出はできないため、recombinationについては過小評価されている可能性もある。抗ウイルス療法を行う症例、抗ウイルス療法が奏功しなかった症例については、両末端側をターゲットにした2領域の解析による遺伝子型の判定も考慮すべきと考えられた。(著者抄録)

進行する歩行障害のためGuillai-Barre症候群(GBS)の診断で入院した3歳の男児。第4病日から免疫グロブリン大量静注療法を行い早期に歩行可能となった。抗ガングリオシド抗体は通常の抗体測定法(ELISA)では陰性だったが、フォスファチジン酸(PA)の添加で陽性化した。GBSの診断において、PA添加による抗ガングリオシド抗体検査は小児でも有用であり、また、電気生理学的検査が困難な場合でも確定診断の一助となりえる。(著者抄録)

Cyclic vomiting syndrome is a disorder characterized by recurrent, discrete, stereotypical episodes of nausea and vomiting. Although chronic or recurrent appendicitis may be a cause of recurrent abdominal pain, it rarely meets the diagnostic criteria for cyclic vomiting syndrome. Furthermore, mast cell counts are histologically high in chronic appendicitis. We report a 10-year-old Japanese girl with a cyclic pattern of vomiting that met the stringent diagnostic criteria for cyclic vomiting syndrome, and which was resolved after appendectomy. Histopathologic examination of the resected appendix showed not only acute mucosal inflammation but also chronic inflammatory changes. Because we speculated that recurrent or chronic appendicitis may have been related to the patient's vomiting bouts, we also immunohistochemically investigated the density of mast cells in the specimen. We found that the mast cell density was markedly high in the lamina propria of the appendix. In our patient, a type I hypersensitivity reaction with release of mediators by mast cells could have been the initial factor triggering the recurrent appendicitis, and this stressor could have initiated the vomiting cascade. In conclusion, chronic or recurrent appendicitis could be one of the causes of cyclic vomiting, and must be considered in the differential diagnosis of cyclic vomiting syndrome. (C) 2011 Elsevier Masson SAS. All rights reserved.

This paper describes an investigation into direct observation of microscopic images of tissue using a thin acoustic wave guide. First, the characteristics of the ultrasonic wave propagated in a fused quartz fiber were measured using the reflection method in order to study the insertion loss and the frequency shift of the ultrasonic wave transmitted from the transducer. Next, a receiving transducer was placed close to the end of the fiber, and the characteristics of the ultrasonic waves propagated through the acoustic coupling medium were measured using the penetration method in order to study the insertion loss and the frequency-dependent attenuation of the penetrated waves. Finally, a C-mode image was obtained by optimizing the measuring conditions using the results of the above measurements and scanning the ultrasonic beams on a target (coin) in water. A reflected wave with a peak frequency of approximately 220 MHz was obtained from the end of the fiber. The transmitted ultrasonic waves propagated through the acoustic coupling medium were detected with a frequency range of approximately 125-170 MHz, and the maximum detectable distance of the waves was approximately 1.2 mm within the 100-MHz frequency range. Finally, a high-frequency C-mode image of a coin in water was obtained using a tapered fused quartz fiber. The results suggest that it is necessary to improve the signal-to-noise ratio and reduce the insertion loss in the experimental system in order to make it possible to obtain microscopic images of tissue.

We report here the first published case of a pediatric patient with Gitelman's syndrome (GS) in whom hypokalemia-associated rhabdomyolysis developed. A 13-year-old girl was admitted with weakness of the extremities, walking difficulty and calf pain. Laboratory data showed a serum potassium level of 2.1 mmol/l and a serum creatinine phosphokinase level of 1,248 IU/l plus myoglobinemia. The presence of normomagnesemia was the basis for a genetic analysis of the thiazide-sensitive sodium chloride cotransporter gene, which revealed compound heterozygous mutations in this gene. Prompt fluid expansion and potassium supplementation led to regression of the muscle symptoms. Hypokalemia can be a rare cause of rhabdomyolysis in patients with GS, even in childhood. We emphasize that genetic analysis is advisable to determine whether the suspicion of GS is warranted.

We report the first documented case of pancreatitis associated with rotavirus infection in an infant. Estimation of amylase level is important in infants with severe rotavirus gastroenteritis, hyperamylasemia should alert one to the presence of overt pancreatitis which should be investigated by lipase estimation and/or imaging.

小型ユビキタス超音波診断装置のへき地・地域医療における有効性を検討するため、診療所受診患者を対象にZ-one(Zonar medical system社製)、Sono site 180、Micro-max(Sono site社製)を用い、B-modeによるリアルタイム白黒画像とカラードプラ画像を比較した。その結果、診断情報の内容に差はなかったが、Z-oneは小型ではあるが画像の質が良好であり、保存性にも優れ、大型の検査診断装置と大差なかった。在宅診療でのさまざまな疾患の発見にも役立った。病院などへの画像送信については、3機種ともwireless communicationはできないが、Z-oneでは電話回線を利用した送信が可能である。携帯電話による胆嚢癌や肝硬変の画像送信を行ったところ、劣化が強く診断に影響が出る可能性が示唆された。災害出動時などにおける電源確保については、携帯型のソーラー充電がSono site 180には使用可能であった。

激しい運動後にヘモグロビン尿やミオグロビン尿が出現し、尿色素異常を示すことがあるが、両者が合併したexertional myohemoglobinuria(EMH)の報告は少ない。【症例】14歳男子。剣道の練習後に尿色素異常が連日みられるようになり、下腹部痛も出現したため当科を受診した。血清ハプトグロビン(Hp)の減少が認められたが、発作性夜間血色素尿症は否定でき、行軍ヘモグロビン尿症と診断した。その後も同様のエピソードがあり、再度Hpの低下がみられたほか、CPKの上昇と尿中ミオグロビンの異常高値が認められたことからEMHと診断した。【考察】EMHは潜在的なものも含めると稀ではないとされ、一過性の腎機能障害も示唆されていることから、剣道の際はかかとにサポーターを装着して再発を防止すべきと考える。一方、画像診断で腹痛の部位に尿膜管遺残が認められたが、感染徴候はなく手術適応はなかった。またEMHとの因果関係も明らかでなかった。(著者抄録)

原発性硬化性胆管炎(primary sclerosing cholangitis:以下PSCと略す)は、肝内外の胆管の線維性狭窄をきたす進行性の慢性胆汁うっ滞性疾患である。原因は不明であり、症例数も少ないが、なかでも新生児期発症のPSCは非常に稀である。新生児期より胆汁うっ滞をきたし5ヵ月時に診断した新生児期発症のPSC症例を経験したので報告する。胆道閉鎖症との鑑別が困難で、二度の開腹下胆道造影、肝(楔状)生検を施行して診断に至った。胆道閉鎖症と診断される症例の中に少なからず存在することが予想される。胆道閉鎖症とPSCには重複する部分も多く、何らかの共通の病因が存在することが示唆される。新生児PSCにおいてはステロイド薬やウルソデオキシコール酸の効果は限定的であり、本症例では肝移植を視野に準備を進めている。(著者抄録)

無呼吸が修正37週以降も遷延した早産児7症例を対象に、胃食道逆流(GER)に有効とされた増粘ミルクを使用し、有用性を検討した。観察は増粘ミルク開始前後に各3日間行い、増粘ミルクは「はぐくみ」に増粘剤として0.35% ローカストビーンガム含有のHL-350を使用した。その結果、平均無呼吸回数は10.9回から6.1回に有意に減少した。脳室周囲白質軟化症、軽度脳室内出血を合併した2症例では、無呼吸回数に変化がなかった。24時間食道pHモニタリングを行えた5症例では、pH4.0未満の逆流時間率は2例で著明に低下した。このことから、遷延する無呼吸はGERによるものが少なからず混在していると考えられた。増粘ミルクによる有害事象は観察されなかった。増粘ミルクは離乳食開始後の生後5〜6ヵ月頃に中止し、この間の体重は順調に増加した。

1歳3ヶ月女.患者は1歳2ヶ月時に胃食道逆流症の診断で,Nissen手術が施行された.そして術後2日と10日に,経口ブドウ糖負荷試験(GTT)が行われた.後期ダンピング症候群(DS)を起こすリスクが高いと考えられたため,食事指導を行った後,術後12日目に退院となった.しかし,1ヵ月後にカステラ入りパンを食べた2時間後にぐったりとなり,約1分間の全般性強直間代けいれんが出現した.食事指導により低血糖が再発しないことを確認して,2日後に退となったが,2歳10ヶ月現在,低血糖症状は認められていない

軽症下痢に伴ったけいれんの原因検索中にビタミンD(VD)欠乏性くる病が判明した事例(10ヵ月の男児)を報告した.VD欠乏の要因は,母乳栄養と,蕁麻疹が出たことに対する離乳食の偏り(卵と肉の除去食など),および母親の社会的ひきこもりによる紫外線(UV)照射不足と考えられた.けいれんについてはカルバマゼピンを投与し,くる病には活性型VDを使用するとともに,母親に離乳食とUV照射について指導を行った

以前は障害児の胃食道逆流症(GERD)に対して外科治療は行っていなかったが,2002年4月からは方針を変更し,患児のQOL向上を目的とし,積極的に外科治療を行うこととした.方針変更後,2年6ヵ月間の外科治療成績を検証した.逆流防止術は11例中9例で腹腔鏡下,1例で開腹下に行い,うち7例に胃瘻造設,3例に腸瘻造設,3例に幽門形成術を付加した.呼吸器感染は11例全例で消失し,術前5例で認められた吐血も全て消失した.現在,術後4ヵ月〜2年2ヵ月間経過しているが,死亡例はない.全例で退院先の介護者から「吸引回数が減少した」,「肺炎による入院加療がなくなった」「熱を出さなくなった」など「明らかにQOLが改善した」との評価を得ている

12歳女児.右側腹部痛と右上腹部のX線不透過像から胆嚢結石と診断され入院となった.画像所見から胆石はカルシウム成分を多く含むことから手術適応と判断し腹腔鏡下胆嚢摘出術を施行した.胆嚢摘出時に胆嚢壁の一部を損傷してクリーム色の練乳状懸濁液が流出した.摘出胆嚢は瓢箪型を呈し,胆嚢頸部に18×10×10mm大の白色結石が嵌頓し,嵌頓部だけが黒色であり,さらに胆嚢底部には38×16×14mm大の白色固形物が認められた.胆嚢の組織学的検索では胆嚢壁の線維増生とリンパ球浸潤を伴う軽度の慢性炎症所見が認められた.胆石の一部と練乳状懸濁液の赤外線スペクトル分析では,95%以上が炭酸カルシウムであり,他の成分は検出されなかったことから,本症例は炭酸カルシウム胆石と石灰乳胆汁と診断された

48歳男.腹痛と嘔吐を主訴とし,重症急性膵炎Stage2と判断した.習慣性飲酒歴があり44歳時に高脂血症を指摘されたが放置し,46歳時に膵炎の既往がある.入院時検査でトリグリセリド(TG)が高値であったが,入院直後は絶食とし,その後の脂肪制限食により第9病日に正常化した.血中リポ蛋白-TG水解に関与しているヘパリン静注後血漿リポプロテインリパーゼ(LPL)は一過性に減少したが,LPLは遺伝子解析で異常を認めず他に由来すると判断した.本症例の病態と成因は,習慣性過量飲酒により高TG血症と膵炎をきたし,炎症性サイトカインによりLPLが減少したため強度の高TG血症となり,さらに膵炎を増悪したと考察した

乳児遷延性下痢症は乳児期の難治性下痢症である.病態として小腸粘膜に対する多因子的な障害が示唆され,病態解明には小腸生検が必要とされてきた.しかし,小腸生検は手技が煩雑でX線透視下で行うため放射線被曝が問題となる.小腸生検の代わりに,本症患児に内視鏡的手技で経時的に直腸生検を行ったところ,発症時期から治癒期にかけて直腸粘膜病変の変化を認めた.本症に対する直腸生検は非常に有用であると考えられたので,それについての手技,及びその組織学的評価法を述べ,若干の文献的考察を加えた

Background: Histololgic studies of rectosigmoidal mucosal biopsies of infants with isolated blood-streaked stool have shown many eosinophils and revealed aggregates of small dark granules (nuclear dust). However, no description of the nuclear dust has been made for this condition and the nature of the nuclear dust has not been thoroughly investigated. We determined the characteristics of these particles in biopsies from infants with streaked rectal bleeding. Methods: Nineteen infants who were younger than 6 months old and had isolated rectal bleeding were studied, as were six age-matched control infants. Rectosigmoidal mucosal biopsies were immunohistochemically assessed using anticarcinoembryonic antigen and macrophage-associated antibodies and examined for apoptotic cells by modified in situ TdT-mediated dUTP-biotin nick-end labelling. The number of apoptotic epithelial cells was compared between rectal bleeding and control groups. Results: Immunohistochemistry showed that at least some of the nuclear dust consisted of apoptotic epithelial cells. Infants with rectal bleeding also showed nodular lymphoid hyperplasia (n = 16), abundant eosinophils (> 20/high power field, n = 14) in the mucosa, and a significantly high number of apoptotic epithelial cells relative to the control group, Rectal bleeding disappeared at 6-month follow-up in 14 of 18 infants tone was lost to follow-up) who were fed a different milk formula or breast-fed (their mothers were restricted from having cow's milk and eggs). Conclusions: The high number of apoptotic epithelial cells in rectosigmoidal mucosal biopsies of infants with streaked rectal bleeding is probably caused by accelerated epithelial cell turnover and apoptosis. (C) 2001 Lippincott Williams & Wilkins, Inc.

Focal nodular hyperplasia of the liver is a lesion characterized by a well-circumscribed region of hyperplastic liver parenchyma and contains a stellate fibrous scar. The lesion is thought to be because of liver-cell hyperplasia that is caused by focal circulatory disturbances. We describe here a pediatric case of this lesion that provided direct histopathologic evidence of circulatory disturbances. We identified arterial and portal thrombi, as well as recanalization of arteries in the nodule. Hepatic necrosis was also seen in the lesion. We speculate that thrombosis of the hepatic artery and/or portal vein was the cause of hepatic necrosis and that reperfusion following hepatic arterial recanalization resulted in nodule formation. Although there was no stellate scar present in our case, the presence of bile ductular proliferation at the periphery of the nodule was helpful in distinguishing this lesion from adenoma and hepatocellular carcinoma. The early stage of nodular formation may explain the lack of a stellate scar in our case. The patient was treated earlier with actinomycin D and vincristine following surgical excision of Wilms' tumor. It is possible that such chemotherapy contributed to thrombosis in our case. (C) 2000 Blackwell Science Asia Pty Ltd.

乳児遷延性下痢症患児10例(月齢2から8ヵ月)について臨床病態と直腸組織病変の相関を知る目的で発症時,回復期,体重増加期,再発時,治癒期の直腸粘膜病変の変化を比較検討した. 1)発症時は上皮内に白血球浸潤がみられ,特に血便例は好中球浸潤が認められた.又,9例に好酸球浸潤が認められた. 2)回復期には上皮内の白血球浸潤が軽減し,特にリンパ球浸潤の消退が顕著に認められた. 3)体重増加期は上皮内に再び好酸球やリンパ球浸潤が認められる傾向にあり,この時再発が4例にみられ,全例に好酸球浸潤が認められた. 4)治癒期にはアトピー性皮膚炎の例を除き異常所見は消失した.以上から,発症機転に何らかのアレルギーの関与が示唆された

1歳7ヵ月男児。朝起床時に右片麻痺を認め入院,頭部CTで被殻に低吸収域を認めた。発症4日目のMRIで被殻から放線冠にかけてT 2強調像で高信号を示し脳梗塞と診断した。脳血管造影で内頸動脈,中大脳動脈,穿痛枝に狭窄・閉塞はなかった。麻痺は順調に改善するも発症後3ヵ月でまだ麻痺は残っている。本症例で原因は特定できなかったが,1ヵ月前に転倒し門歯を1本折った時の頭部外傷が関与していた可能性がある

1) 1991年夏,盛岡地区にて,37例の無菌性髄膜炎を経験し,ウイルス学的検索によりEV 30が検出された.2)従来の報告と異なり,乳児の症例がなく,CRP陽性例が多く,また,髄液細胞分類では,多核球優位を示す例が多かった.3)現在のところ,後遺症は認められていない