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An 8-month-old girl with Diamond Blackfan syndrome and idiopathic neutropenia needed steroid therapy for anemia, but the left multicystic dysplastic kidney was often infected. We performed laparoscopic nephrectomy for infection control without any complications. Neutrocytes increased and the infection decreased after nephrectomy even with steroid therapy. Finally, she underwent bone marrow transplantation, as the anemia was not responding to steroid therapy.

58例(平均68歳)の患者を対象として塩酸プロピベリン経口投与による治療を行い, 治療前, 治療4週後および8週後において, 国際尿失禁会議質問票(ICIQ-SF)・キング健康調査票(KHQ)・排尿日誌および尿意切迫感に関する問診票により症状・QOLスコア, 自覚症状の改善を評価した。1日の平均排尿回数, 平均失禁回数, 平均尿意切迫感回数および尿意切迫感程度はいずれも治療前と比較して治療4週後に有意に減少し8週後も効果は持続した。また, ICIQ-SF総スコアおよびKHQスコアも治療後有意な改善を認めた。安全性評価症例42例中10例(23.8%)に有害事象を認めたが, いずれも重篤ではなく投与中止あるいは減量した例はなかった。, The present study was prospectively conducted for female patients with wet overactive bladder to assess the efficacy of propiverine hydrochloride in improving their symptoms and quality of life (QOL). Propiverine hydrochloride was administered orally to 58 patients for 8 weeks. Prior to administration and at 4 and 8 weeks after administration, symptoms and QOL were assessed by the micturition diary, the International Consultation on Incontinence-Short Form (ICIQ-SF), and the King's Health Questionnaire (KHQ). After administration, numbers of daily micturitions, incontinence episodes, urgency episodes and severity of urgency which were assessed based on the micturition diary and urgency scale questionnaire showed significant improvement when compared to baseline values. Furthermore, both ICIQ-SF and KHQ scores improved significantly after administration. The incidence of adverse events was 23.8% and none were serious. Propiverine hydrochloride was shown to contribute not only to the improvement of symptoms in female patients with wet overactive bladder but also to their QOL.

症例1:21歳女.主訴は左腰部痛.中学3年から拒食があり, 神経性食思不振症と診断された.また, 発病当初から市販の下剤スルーラックを1日60～100錠, ピサコジルとして300～500mg(通常用量10mg/日)常用していた.腹部単純写真で左上部尿管に淡い結石陰影を認め, 体外衝撃波砕石術(ESWL)を3回施行し, 良好な結石片の排泄を得て退院した.その後, 左右の腎尿管結石が頻回に再発し, ESWLを9回行った.神経性食思不振症, 下剤乱用について母親から情報があり, 看護師の説得で下剤を中止し, 精神科にコンタクトをとり, 母子で定期的に受診するようになった.神経性食思不振症は改善傾向にあり, 下剤乱用, 尿路結石の再発は現在まで認めていない.症例2:18歳女.主訴は左下腹部痛.高校1年より摂食障害, 市販の下剤常用があった.点滴腎盂造影(DIP)で左腎盂の放射線透過性結石と軽度の左水腎症を認め, ESWLを施行し, 結石片の排泄をみた.細かな残石の排出が続き, DIPを併用して残石にESWLを行った.やせ著明であったことから改めて問診し, 下剤乱用と尿路結石の関連, 治療の必要性について説明する間に治療意欲が増し, 下剤は医師処方の酸化マグネシウムに限られるようになった.経過観察で結石再発はみられていない, We report two cases of urolithiasis related to anorexia nervosa and laxative abuse. Case 1: A 21-year-old woman was referred to our hospital because of left flank pain. A left ureteral stone, 10 x 6 mm in size, was successfully fragmented by extracorporeal shock-wave lithotripsy (ESWL), but she experienced repetitive formation of bilateral urinary stones and double J stent encrustation which required 13 sessions of ESWL, one session of transurethral ureterolithotripsy and one session of cystolithotripsy over a period of 5 years. All stones were comprised of pure ammonium acid urate. It was later revealed that she was diagnosed with anorexia nervosa at 15 years old and had suffered from laxative abuse (bisacodyl, 300-500 mg/day) ever since. Case 2: A 18-year-old woman was referred to our hospital because of left lower abdominal pain. A left renal stone, 15 x 10 mm in size, was successfully fragmented by ESWL, but she had double J stent encrustation which was managed by cystolithotripsy. All stones were comprised of pure ammonium acid urate. She was later diagnosed with anorexia nervosa and it turned out that she had suffered from an eating disorder and laxative abuse (bisacodyl, 200 mg/day) since the age of 15 years. Both patients had marked decrease in urine volume, hyponatremia and hypokalemia. Anorexia nervosa and laxative abuse should be suspected whenever a woman has an ammonium acid urate stone in sterile urine because the treatment of these disorders is crucial to the prevention of repetitive formation of urinary stones.

長期臥床高齢者で, 膀胱結石が下降して嵌頓した女性尿道結石を2例経験した.1例は婦人科の内診の際に前膣壁から腫瘤を触れ, 1例は寮母がオムツ内の肉眼的な血尿に気づいたことが発見の端緒であった.痴呆, 精神障害, 麻痺があると症状を自覚できなかったり訴えることができず, 診断の遅れが膀胱結石の成長, 女性では珍しい尿道への嵌頓につながったものと推測された.長期臥床者の尿路感染症や血尿を診る際, 結石の可能性を念頭に置くことが早期診断のために必要と考えられた, A 78-year-old female suffering from a cerebral infarction and subdural hematoma was referred to us due to a hard mass in the anterior vaginal wall which was disclosed during gynecological examination. An abdominal X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) showed that a large spindle-shaped stone, 60 x 42 mm in size, was impacting the urethra. It was impossible to catheterize the urethra. The stone gradually projected through the external urethral meatus and was removed by grasping and drawing with forceps. Another 83-year-old female with senile dementia was referred to us because of macrohematuria. An abdominal X-ray and CT showed the presence of two oval bladder stones, 32 x 24 mm and 30 x 21 mm in size. During a follow-up, one of the stones projected partially through the external urethral meatus and was removed by drawing with forceps. After a week, the other stone impacted the urethra and was removed in the same way. Both women were frail, bedridden institutionalized elderly with severe dementia, and their urination had been managed with diapers for years. As the proportion of elderly people in Japan rapidly increases, female urethral stones migrating from the urinary bladder, once very rare, may increase in number, to which we must pay attention.

67歳男.40年前の尿管切石術時の遺残ガーゼによる傍膀胱異物肉芽腫例を報告した.ガーゼオーマは医原性であるため報告されにくいが, 手術歴のある患者の腹部腫瘤の鑑別診断において忘れてならないものである.既往歴をよく確認し, 遺残ガーゼの可能性を念頭に置いて画像の特徴を検討することが, 正しい診断につながると考えられた, A 67-year-old man complained of vague pain in the lower abdomen persisting over 10 years. A hard mass was palpable just under an operative scar (right Gibson incision for ureterolithotomy performed 40 years previously). In addition to the patient's history and physical examination, abdominal echography, computerized tomography and nuclear magnetic resonance imaging (MRI) were performed and resulted in a preoperative diagnosis of retroperitoneal gauzeoma. The paravesical mass, 7 x 6 x 6 cm in size, was surgically removed, which was an encapsulated granuloma surrounding a surgical sponge. The possibility of foreign body granulomas due to a retained surgical sponge (so-called gauzeomas) should be considered in patients who have a previous history of abdominal operations.

70歳代男。患者は入院半年前から腰痛が出現, 腹部CTでも異常がみられた。更に精査を計画中に肉眼的血尿が始まり, 膀胱鏡では多数の凝血塊と左壁の乳頭状腫瘍を認めた。凝血塊の除去を行った後に, 即日入院となったが, 腹部CTでは肝臓のLDAと大動脈周囲のリンパ節腫脹を認め, 腹部MRIでは肝S5とS6に充実性腫瘤が認められた。また, 胃・膵頭部・腹腔動脈・SMA・傍大動脈周囲には多数のリンパ節腫脹が認められた。治療として脊椎麻酔下にTUR-Btを施行し, 肝のLDAに対しては超音波下肝生検が行われた。その結果, 膀胱は表在性尿路上皮癌と悪性リンパ腫の衝突癌, 悪性リンパ腫はNHL, B細胞型, stage IVと診断された。以後, 化学療法を1コース行い, 終了3週後からは肉眼的血尿は消失した。現在, 3年経過で体調は良好, 外来通院中である。, A man in his 70's visited the Department of Internal Medicine due to lumbago that had first appeared two months previously. Abdominal computed tomography showed a low-density area in the liver and swelling of lymph nodes surrounding the abdominal aorta. Four months later, he was hospitalized on an emergency basis in a urology ward in order to control bladder tamponade. Cystoscopy revealed massive blood clots and a papillary tumor at the left wall of the urinary bladder. He underwent transurethral resection of a bladder tumor, and the pathological diagnosis was a collision tumor between urothelial carcinoma (G2, pTa) and malignant lymphoma (B cell type). He underwent a liver biopsy soon thereafter, and the pathological diagnosis was malignant lymphoma (as for the one found in the urinary bladder). Bladder tamponade was repeated, which was relieved after one course of chemotherapy for malignant lymphoma. He underwent six courses of chemotherapy (THP-CO), and he was well without recurrence of either malignant lymphoma or urothelial carcinoma with 3 years' follow-up. To our knowledge, this is the 14th reported case of a collision tumor in the urinary tract.

Symposium 3 entitled "Female Urology in the Future" is very timely as female urology, a urological subspecialty mainly managing stress urinary incontinence (SUI), pelvic organ prolapse (POP) and overactive bladder (OAB), is now beginning to blossom in Japan. In 1986, the first Japanese female incontinence clinic was opened at the Department of Urology, Nagoya University. In 1999, the tension-free vaginal tape (TVT) procedure using polypropylene mesh tape to support the mid-urethra was introduced into Japan, and it dramatically changed the SUI treatment with less era"). invasiveness and durability ("mesh Recently, a variation of the TVT procedure, transobuturator tape (TOT) procedure, and POP repair using polypropylene mesh such as tension-free vaginal mesh (TVM) procedure, are the focus of concern among Japanese urologists and gynecologists practicing female pelvic floor medicine. The International Continence Society derived a new symptomatic definition of overactive bladder (OAB) in 2002. The Neurogenic Bladder Society in Japan published the OAB guideline aiming at public education and shared care in 2005. Five symposists discuss the advantages and pitfalls of the new mesh procedures for SUI and POP, conservation of the uterus in POP repair, gender specificity of OAB, basic research to develop OAB drugs other than anticholinergics, collaboration between urologists and gynecologists, and pediatric female urology.

症例:17歳男.左陰嚢内容の腫大を主訴とした.左陰嚢内, 左精巣上極に約2cm大の表面不整, 硬い腫瘤を触知した.超音波検査では腫瘤は充実性腫瘍であった.悪性腫瘍の可能性を考えて, 手術を施行した.精巣と精巣上体との剥離は困難と考え, 左高位精巣摘除術を施行した.病理組織学的に精巣上体原発の非ホジキンリンパ腫, 瀰漫性混合細胞型, B細胞型の悪性リンパ腫と診断された.術後化学療法を3コース施行した.現在治療開始26ヵ月であるが, 全身状態は良好で再発や転移を認めず生存中である, A 17-year-old man visited our hospital with the chief complaint of painless swelling of the left scrotal content. An elastic hard mass was palpable at the upper pole of the left testis. Left radical orchiectomy was performed. The tumor originated from the epididymis and did not involve the testis or the spermatic cord. Histologically, the tumor was diagnosed as a malignant lymphoma (non-Hodgkin's lymphoma, diffuse mixed cell type, B-cell type). No abnormalities were found in other organs. After establishment of the diagnosis of primary epididymal malignant lymphoma, 3 courses of chemotherapy (adriamycin, vincristine, cyclophosphamide, prednisolone) were performed. No evidence of recurrence or metastasis was found 26 months after surgery.

Bladder dysfunction is one of the major symptomatologies characteristic to HAM (HTLV-I associated myelopathy). Four patients, 3 females and 1 male, were diagnosed by neurologists to have HAM with spastic gait disturbance and increased titer of antibody to HTLV-I. They complained of urge incontinence, bed wetting, difficulty in micturition and/or pollakisuria. Urodynamically, in 3 of them severe uninhibited detrusor contractions were observed. On the other hand, in one case detrusor contractility was lost completely at voiding. In all patients, bladder sensation was well preserved. Corticosteroids and interferon could not improve their urological symptoms. Clean intermittent catheterization (CIC) put on 3 patients who had a significant amount of residue relieved them of urinary incontinence. We believe that HAM in patients suffering from severe difficulty in micturition is a good indication for CIC.

A 70-year-old man, who had undergone total gastrectomy for Borrmann III gastric cancer one year previously, was referred to us with a complaint of left scrotal swelling. Physical examination showed left scrotal hydrocele and a hard nodule in the ipsilateral inguinal area. Excretory pyelography and abdominal computed tomography showed mild left hydronephrosis and no stone. A left orchiectomy was performed. The mass in the spermatic cord was 2.5 x 1.0 x 1.0 cm in size, and the pathological diagnosis was tubular adenocarcinoma, identical to that of the previous gastric cancer. Five months after orchiectomy, the patient underwent percutaneous nephrostomy to manage postrenal renal failure caused by massive metastasis of retroperitoneal lymph nodes. He died one month later. We found a total of 84 cases of metastatic tumors of the spermatic cord reported in Japan, approximately half of which were metastases from gastric cancer. In 10 cases, including ours, the tumor was accompanied by hydrocele of the scrotum or spermatic cord. Although this association is rare, cancerous lesions should be considered in the management of hydrocele.

A seventeen-year-old girl, who had had an ileal conduit urinary diversion at 5 years of age, underwent a urinary undiversion 12 years after the initial operation. She has been suffering from recurrent episodes of urinary tract infection with fever. The preoperative evaluation revealed dilatation of the bilateral pelvis and ureters on an excretory urography. Ileal conduitgraphy revealed urinary reflux from the ileal loop to the bilateral upper urinary tracts. Cyclic bladder hydrodilatation through a suprapubic cystostomy was performed and a precise urodynamic study was done on the lower urinary tract. The vesical capacity increased from 40 ml to 300 ml in a month. Cystometry and urethral pressure profile revealed a stable bladder with good detrusor contraction and preserved urethral continence. Voiding status was satisfactory with only a small amount of residue. The undiversion was undertaken as follows. The ureters were once isolated from the ileal loop and their distal ends were everted in length of 5 mm to be nipples in shape. The ureters were reanastomosed to the proximal end of the ileal loop with submucosal tunnels of 2 cm. The ileal loop was then tapered and implanted to the bladder with a submucosal tunnel of 5 cm. Eight months after the undiversion, she enjoys good urination, free from urinary tract infection. Although moderate hydronephrosis remains bilaterally, renal function is stable.

Urge incontinence and frequency, persisting despite oral anticholinergics and antibiotics for four months, brought a 72-year-old woman to our hospital. Plain abdominal X-ray followed by cystoscopy demonstrated a large stone (43 x 37 mm) in the bladder, and the patient underwent suprapubic cystotomy to remove the stone. The stone contained a surgical sponge, which was apparently left in situ at transvaginal hysterectomy two years previously. The sponge had most likely eroded the bladder wall and migrated into the cavity. We found a total of 17 cases reported in Japan of a surgical sponge migrating into the bladder. In particular, our case was associated with transvaginal surgery, while the other 16 cases involved transabdominal surgery.

We experienced a case of giant vesical diverticulum with a malignant neoplasm in which the preoperative diagnosis was difficult. It simultaneously occurred with a vesical tumor. The patient, a 68-year-old man, visited our hospital complaining of pollakisuria, nycturia and microscopic hematuria. He had urinary retention and his vesical urine volume was 1, 450 ml. X-ray examination and ultrasound examination revealed a giant vesical diverticulum and moderate hypertrophy of the prostate. A vesical tumor was suspected. Cystometrogram revealed a flaccid pattern with vesical capacity over 900 ml. Diverticulectomy and resection for a vesical tumor were performed. A malignant neoplasm in the vesical diverticulum was found during the operation. Transurethral resection of the prostate was performed after two months. There has been no evidence of recurrence for 3 years postoperatively.

We report two cases of perivesical abscess associated with neurogenic bladders. One patient suffered from diabetic neurogenic bladder, and the other spinal cord injury. Both patients had an uninhibited contraction at the low capacity on cystometry. They complained of difficulty in micturition and presented with large lower abdominal tumors. Although the extravasation of contrast medium was not confirmed on cystography, we believe that the perivesical abscess was the consequence of urinary leakage out of the bladder. The abscesses disappeared after drainage. We have discussed the possible mechanism involved in the abscess formation which occurred in the neurogenic bladders.

We evaluated the reliability of serum beta 2-microglobulin (beta 2-MG) in comparison with serum creatinine, blood urea nitrogen and creatinine clearance in 164 patients with spinal cord injury. The value of serum beta 2-MG demonstrated the highest correlation coefficient (r = -0.927) in relation to creatinine clearance. The beta 2-MG value reflected the severity of hydronephrosis on excretory urography, with very few false negative values. The value of beta 2-MG is concluded to be one of the best screening tests in evaluating renal function in spinal cord injury patients.

Since necrotizing fasciitis of the genitalia was first described by Fournier in 1883, approximately 400 cases have been reported. It has been seldom reported in Japan. Because its mortality rate is still high, the importance of early diagnosis and the subsequent vigorous treatment has been emphasized. A 73-year-old man who had poor controlled diabetes mellitus was admitted to our hospital with painful swelling of scrotum. Chemotherapy using broad spectrum antibiotics and debridement of scrotal skin was performed combined with insulin therapy. As culture of pus and excised tissue from the gangrenous patches yielded the growth of candida, we used 8 g/day of 5-fluorocytosine. Because the gangrene was not healed, we performed bilateral orchiectomy. After the operation, the gangrene was healed and the wound was closed. Diabetes mellitus was controlled well and his general condition was improved.

Clinical effects and therapeutic usefulness of oxybutynin hydrochloride were evaluated in a long-term clinical trial on patients with unstable bladders and neurogenic bladders. Of the 46 patients entered into the trial, 37 were those diagnosed with an unstable bladder and 9 with a neurogenic bladder with overactive detrusor. In 37 of the cases (80%), the period of drug administration reached up to 12 weeks and in 16 cases (34%) the drugs were administered for more than 24 weeks. The average administration period was 165.9 days. The average total given dose was 1776.9 mg and average dose per day was 10.7 mg. Excellent and good responses were obtained in 76.3, 88.9 and 69.6% at 12 and 24 weeks after start of administration and at the time of discontinuing the drug, respectively. The cystometric changes at pre- and post-administration were evaluated on 23 cases and revealed a significant increase in volume at first sensation and maximum desire to void. Maximum resting intravesical pressure was significantly declined and uninhibited detrusor contractions were significantly suppressed. Side effects were noted in 11 of the 46 cases (23.9%), most of which were well tolerated by the patients. In 4 cases the drug had to be discontinued because of the side effects. Dry mouth was the most common side effect, occupying almost half of the incidents. No significant abnormality was noted on blood laboratory data, blood pressure or heart rate, following the drug administration. In one case slight increase in serum glutamic-oxalacetic transaminase and glutamic-pyruvic transaminase was encountered, but its relationship with the drug was obscure. The clinical usefulness of this drug (excellent and good) was 78.9, 88.9 and 69.6% at 12 and 24 weeks after start of administration, and at the time of drug discontinuation, respectively. The present long-term trial proved that oxybutynin hydrochloride is an exceedingly effective and safe agent for clinical management of unstable bladder and overactive neurogenic bladder.