Long-term prognosis of IgA nephropathy presenting with minimal or no proteinuria: A single center experience.

The long-term prognosis of patients with IgA nephropathy (IgAN) who present with preserved renal function and minimal proteinuria is not well described. We investigated the long-term outcomes of IgAN patients with an apparently benign presentation and evaluated prognostic factors for renal survival and clinical remission. We studied Japanese patients with biopsy-proven IgAN who had an estimated glomerular filtration rate (eGFR) ≥60 mL/min/1.73 m(2) and proteinuria <0.5 g/day at the time of renal biopsy. The renal biopsies were reviewed using the Oxford classification. Twenty patients met the inclusion criteria. At diagnosis, the median eGFR (interquartile range) was 76.8 (65.2-91.1) mL/min/1.73 m(2), and the median proteinuria level was 0.31 (0.16-0.39) g/day. Only one patient had an increase in serum creatinine of over 50% and no patient progressed to end-stage renal disease. The 15-year renal survival rate was 93.8%. Clinical remission was observed in 9 (45%) patients. Baseline proteinuria was the only factor significantly associated with the absence of clinical remission. The long-term prognosis of Japanese patients with IgAN who presents with minor urinary abnormalities and preserved renal function is excellent.

Usefulness of mini-tracheostomy and torque controlled insertion of applicator in fractionated endobronchial brachytherapy.

Endobronchial brachytherapy was developed as effective treatment of endobronchial cancer and fractionated schedule is applied to decrease late toxicity. However, repeated bronchofiberscopy is onerous to the patient and restricts the treatment schedule itself. We applied mini-tracheostomy for a ready access route, and a torque controlled technique for easy insertion of the endobronchial applicator. Eight patients with tracheobronchial cancer invasion were treated with endobronchial brachytherapy of 18-30 Gy/3-5 fractions/1.5-2.5 weeks (median 24 Gy/4 fractions/2 weeks) at reference points of 5 mm from the bronchial surface. The averaged individual irradiation and single session times were 4 min and 24 min, respectively. There were no procedure-related complications. These technical improvements may facilitate flexible fractionated dose prescriptions.

Brachytherapy technique for abdominal wall metastases of colorectal cancer: ultrasound-guided insertion of applicator needle and a skin preservation method.

PURPOSE: To report a technique of interstitial brachytherapy for the treatment of subcutaneous metastatic abdominal wall tumors. MATERIAL AND METHODS: We developed a brachytherapy technique consisting of ultrasound-guided insertion of applicator needles to avoid the organs at risk, such as intestines, and saline injection into the subcutaneous tissue between the tumor and the skin to decrease the skin dose. We encountered three patients with painful metastases from rectal carcinoma in the abdominal wall refractory to external radiotherapy. They were subjected to this brachytherapy with a single dose of 20 Gy. RESULTS: The procedure was safely achieved in all three patients. Long-lasting pain reduction and tumor shrinkage was obtained without early or late complications. CONCLUSION: This interstitial brachytherapy technique seems to be feasible in the treatment of metastatic abdominal wall tumors.

Selective conformal radiotherapy for arteriovenous malformation involving the spinal cord.

Radiotherapy has seldom been used in the treatment of arteriovenous malformations (AVM) involving the spinal cord because of the cord's limited radiation tolerance. A 57-year-old woman with progressive myelopathy due to AVM was treated with a selective conformal radiation therapy (55.6 Gy/20 days) on the nidus region preserving the spinal cord, drainer veins and involved bones. The drainers and symptoms were minimized at the end of treatment and disappeared over 2 years. This case illustrates the usefulness of selective conformal radiation therapy.

[A case of small cell lung cancer with paraneoplastic cerebellar degeneration and anti-voltage-gated calcium channel antibody].

A 55-year-old man presenting with 4 weeks of progressive dysarthria, gait ataxia and vertigo was admitted to our hospital. Chest X-ray films revealed a mass shadow in the right upper lobe of the lung, and transbronchial brushing specimens showed small-cell carcinoma. Extensive examination revealed metastatic lesions in the mediastinal lymph nodes and liver, but brain MRI showed no findings suggestive of metastasis or atrophy. A diagnosis of PCD associated with SCLC was made, and the patient had a high titer of anti-P/Q-type VGCC antibody. He was treated by chemotherapy and radiation therapy, which resulted in a transient improvement in the PCD symptoms.

Ocular and systemic features of Peters' anomaly.

BACKGROUND: To clarify the relationship between associated systemic anomalies and ocular manifestations in patients with Peters' anomaly, a retrospective study was conducted. METHODS: We classified 37 patients with Peters' anomaly into two groups, one with (+) and one without (-) systemic anomalies. RESULTS: The systemic anomaly (+) group consisted of 13 patients, eight males and five females, with mean age of 2.3 months. Peters' anomaly was bilateral in six cases and unilateral in seven. Corneolenticular adhesion was observed in 11 cases. Associated ocular anomalies were seen in 12 cases, and developmental glaucoma was present in eight cases. The systemic anomaly (-) group comprised 24 patients, 13 males and 11 females, with mean age of 28.3 months. Peters' anomaly was bilateral in 11 cases and unilateral in 13. Corneolenticular adhesion was observed in five cases. The associated ocular anomalies were observed in 10 cases, and developmental glaucoma was accompanied in six cases. The incidences of cases with corneolenticular adhesion, those with other ocular anomalies, and those with glaucoma were significantly higher in the systemic anomaly (+) group than in the systemic anomaly (-) group. CONCLUSIONS: Peters' anomaly accompanying corneolenticular adhesion and/or other ocular anomalies should be evaluated for the presence of systemic anomalies.

Anophthalmia in litters of female rats treated with the food-derived carcinogen, 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine.

Anophthalmia in litters of pregnant rats treated with 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine (PhIP), a food-derived carcinogen, was incidentally encountered in a risk-assessment study with 2-generation exposure to PhIP. Female Fischer 344 animals were given 200 ppm PhIP in the diet for 4 wk before mating with nontreated males and also during gestation and lactation periods. Mean numbers of newborn rats per litter in control and PhIP-treated groups were 7.9 +/- 2.9 and 7.1 +/- 1.6 in trial 1 and 8.3 +/- 1.9 and 6.1 +/- 2.4 in trial 2. Among 49 (trial 1) and 63 (trial 2) offspring from PhIP-treated dams, 9 (18.4%) and 32 (50.8%) demonstrated anophthalmia, and 1 (2.0%) and 8 (12.7%) demonstrated hydrocephaly. Five of 7 (71.4%) and 13 of 14 (92.9%) dams delivered pups with malformations in trials 1 and 2, respectively. Also, in a previous study that was carried out with the same protocol and that used the Sprague-Dawley strain of rats, anophthalmia and hydrocephaly were observed in 2 and 1 out of 175 pups, respectively, from 100 ppm PhIP-treated dams. No congenital malformations were found in control groups of the same size in either experiment. In addition to having been previously identified as a cause of carcinogenic activity, our findings suggest that PhIP is capable of causing anophthalmia in rats when administered during the gestational period.

[Sarcomatoid carcinoma of the urinary bladder in a hemodialysis patient: a case report].

The following is a case report bladder of sarcomatoid carcinoma in a Japanese 65-year old female patient treated with hemodialysis. She developed chronic renal failure due to chronic glomerulonephritis. Fifteen months after the beginning of the hemodialysis, continuous gross hematuria was noticed, and cystoscopy revealed a broad-based bladder tumor spreading from the right lateral wall to the posterior wall. The histopathologic diagnosis of the TUR-Bt specimens was sarcoma. Radical cystectomy was performed under the diagnosis clinical stage III, T3bN0M0. The post-operative histopathologic diagnosis of the tumor was sarcomatoid carcinoma, composed of nests of transitional cell carcinoma (G 3) and predominant areas of spindle cell sarcomatoid transformation. Sarcomatoid carcinoma of the bladder in a hemodialysis patient is extremely rare, and to date this may be only the second case in Japanese medical literature.

Anomalies associated with Axenfeld-Rieger syndrome.

BACKGROUND: To detect the associated anomalies in patients with Axenfeld-Rieger syndrome is clinically important, because early treatment for such anomalies is crucial to both visual and systemic development. This study was conducted to clarify the associated anomalies in the syndrome. METHODS: We evaluated 21 patients with Axenfeld-Rieger syndrome encountered at Nagoya City University Hospital over a 16-year period. Patients who presented with a prominent Schwalbe's line accompanying the iris strands were diagnosed as having Axenfeld-Rieger syndrome. RESULTS: The series consisted of 9 males and 12 females, ranging in age from 1 month to 41 years, mean 15.4+/-12.7 (SD) years. The syndrome was bilateral in 17 cases and unilateral in 4 cases. Hypoplasia of the iris was observed in 10 eyes of 6 patients. The associated ocular anomalies included sclerocornea in 6 eyes of 3 patients, developmental glaucoma in 5 eyes of 3 patients, persistent pupillary membrane in 4 eyes of 2 patients, microphthalmos in 3 eyes of 2 patients, and typical iris coloboma in 1 eye. Of 10 eyes with hypoplasia of the iris, 5 exhibited glaucoma. The accompanying systemic anomalies included 9 cases of dental anomalies, 5 of facial anomalies, and 3 of Alagille syndrome. CONCLUSIONS: All of the associated ocular and systemic anomalies appeared to arise from the maldevelopment of the neural crest cells. Patients with Axenfeld-Rieger syndrome should therefore be examined for the presence of anomalies in the tissues of neural crest origin. Patients with hypoplasia of the iris should be checked for glaucoma.

A case of an intraocular foreign body due to graphite pencil lead complicated by endophthalmitis.

We report a case of an 8-year-old boy who presented with an intraocular foreign body composed of graphite pencil lead. The patient had been accidentally poked in the right eye with a graphite pencil. Primary care consisted of corneal suturing and lens extraction. Two pieces of the pencil lead remained in the vitreous cavity following surgery, and 2 days later the patient developed endophthalmitis. Pars plana vitrectomy was performed immediately and the intraocular foreign bodies were removed through the scleral wound. Cultures of the vitreous fluid revealed no bacterial organisms. X-ray fluoroscopic analysis of the vitreous detected 1 ppm of aluminum (a constituent of the pencil lead). Although the clinical presentation indicated probable bacterial endophthalmitis, the detection of elemental aluminum within the vitreous cavity also suggested the possibility of further retinal toxicity due to some dissolving of the pencil lead.

Facial spasm and paroxysmal tinnitus associated with an arachnoid cyst of the cerebellopontine angle--case report.

A 59-year-old female presented with a 3-year history of hemifacial spasm and paroxysmal tinnitus associated with an arachnoid cyst of the cerebellopontine angle, which was identified preoperatively by magnetic resonance imaging. Surgical decompression of the cyst and arterial decompression of the facial and acoustic nerves at their root exit zones resulted in complete resolution of the symptoms.

[Four cases of persistent hyperplastic primary vitreous].

We evaluated four cases of persistent hyperplastic primary vitreous (PHPV) encountered at Nagoya City University Hospital in 1995. PHPV was seen unilaterally in three cases and bilaterally in one. The series comprised two males and two females, ranging in age from three to eight months, with an average of 4.8 months. Case 1 had a white strand running from the optic disc to the posterior surface of the lens in the left eye. Case 2 showed leukocoria in the right eye and central corneal opacity in the left. Magnetic resonance imaging (MRI) revealed total retinal detachment in both eyes. Case 3 exhibited retinal folds running from the optic disc to the posterior surface of the lens in the left eye. Case 4 showed elongation of the ciliary processes and leukocoria in the right eye. Ipsilateral total retinal detachment was seen in MRI. Three eyes of two cases were microphthalmic. Associated ocular anomalies included, posterior embryotoxon, sclerocornea, hypoplasia of the iris stroma and peripapillary staphyloma. There were accompanying systemic anomalies such as arachnoidal cyst, syndactyly, microcephalus, heart anomalies, pulmonary atresia and asplenia. Patients with PHPV should be carefully examined for the possible presence of other ocular and systemic anomalies caused by neural crest disorders.

Clinical evaluation of posterior embryotoxon in one institution.

To elucidate the pathogenesis of posterior embryotoxon, we estimated its incidence in our clinic and evaluated its associated ocular and systemic anomalies. Slit-lamp and gonioscopic examinations were performed on 440 randomly selected patients at Nagoya City University Hospital over a 10-month period. Posterior embryotoxon was detected in 107, 50 bilateral and 57 unilateral, cases (24.3%). Twelve (11.2%) of the 107 cases had open-angle glaucoma. Accompanying ocular anomalies included six cases of sclerocornea, two each of persistent pupillary membrane and familial exudative vitreoretinopathy, and 1 each of melanocytoma of the optic nervehead, choroidal nevus and subconjunctival dermoid cyst. Associated systemic anomalies included three cases of Alagille syndrome, two of congenital biliary atresia, and one each of congenital facial palsy with microtia, congenital adrenal hyperplasia, empty sella syndrome, Hirschsprung disease and Wilson disease. Many of these ocular and systemic anomalies were caused by the maldevelopment of neural crest cells. Patients with posterior embryotoxon should be examined for the possible presence of open-angle glucoma and for ocular and systemic anomalies related to maldevelopment of neural crest cells.

[A case report of angiomyolipoma arising from the renal capsule].

A case of angiomyolipoma arising from the renal capsule is reported. A 49-year-old female was admitted to our hospital with a complaint of abdominal mass pointed out by ultrasonography during her yearly health check. The tumor was 10 cm in size. On abdominal enhanced CT, the tumor was existed behind the left kidney. The capsule of the tumor was enhanced and the septums were seen inside of the tumor. MRI revealed a hemorrhage in the tumor and selective renal angiography showed the tumor was fed by the renal capsular artery. A tumor of the renal capsule was suspected and a laparotomy was done. Pathological results by the frozen section suggested malignancy and radical nephrectomy was performed. Angiomyolipoma was diagnosed histologically. Tumors of the renal capsule are uncommon and angiomyolipoma of the renal capsule is extremely rare. We have found only two cases in Japanese medical literature.

[A case of nonrhegmatogenous retinal detachment in Dandy-Walker Syndrome].

A 2-month-old female presented with nonrhegmatogenous retinal detachment in Dandy-Walker syndrome. At the fist examination, coloboma involving the optic disc in both eyes was detected. The left eye showed microphthalmos with sclerocornea, persistent pupillary membrane, hypoplasia of the iris stroma, and bullous retinal detachment near the optic disc. Chromosomal analysis revealed a mosaic pattern: 46, XX/47, XXX. Increased intracranial pressure associated with Dandy-Walker syndrome was detected by a neurosurgeon at the age of 3 months. The patient was followed for several weeks, and then nonrhegmatogenous retinal detachment appeared in the right eye. Subretinal fluid alternately increased and decreased in both eyes. A ventriculo-peritoneal shunt was performed at the age of 6 months, and the retinal detachment was remarkably reduced in both eyes after lowering of intracranial pressure. Coloboma involving the optic disc, sclerocornea, persistent pupillary membrane, hypoplasia of iris stroma, and Dandy-Walker syndrome were thought to be caused by the abnormal development of neural crest cells. We surmised that the retinal detachment in this case might have resulted from a communicating pathway between the subarachnoid space and the subretinal space. We concluded that the etiology of retinal detachment associated with optic disc anomaly should be investigated to determine adequate treatment.

Histology of fetal eyes with oculocutaneous albinism.

The diagnosis of tyrosinase-negative oculocutaneous albinism (OCA) was made in a 19-week-old fetus by skin biopsy. Because the parents had an 11-year-old son with tyrosinase-negative OCA, they requested that the fetus be aborted at the 20th week of gestation. A histological analysis of the eyes was performed. Throughout the retina, the ganglion cell layer was separated from the inner neuroblastic layer by the inner plexiform layer. However, the number of ganglion cells was decreased and the nerve fiber layer was immature. Bipolar and horizontal cells had begun to segregate into the inner nuclear layer. Rods and cones were identifiable in the posterior, but not peripheral, retina. Cones were more numerous in the center of the retina, and no rod-free area was identifiable. In addition, the ciliary body (epithelial folds, blood vessels in the mesodermal connective tissue core, and ciliary muscle) was less developed than in a normal fetus. Melanosomes in the retinal pigment epithelium only contained filaments without melanization and were therefore classified as stage I or II melanosomes. However, the ciliary epithelium also contained some stage III melanosomes with melanin adherent to the filaments.

Enhancement of chemotherapeutic effects with focused shock waves: extracorporeal shock wave chemotherapy (ESWC).

The effects of shock waves in combination with various anti-cancer agents i.e. Bleomycin (BLM), Cisplatin (CDDP) and 5-fluorouracil (5-FU) on tumor cells suspended in media containing these agents were examined. GCIY cells derived from human gastric cancer and LS 174T and SW480 cells derived from human colon cancers were used for in vitro experiments; GCIY and SW480 cells were also transplanted into nude mice for in vivo study. It was only with BLM that enhancement was evident in all three cell lines, with a degree of chemotherapeutic enhancement proportional to the amount of shock wave energy applied. Ladder formation of DNA in GCIY cells was observed only when treated with both BLM and shock waves in combination. When SW480 and GCIY cells transplanted into the backs of nude mice were treated with a combination of intravenously (i.v.) injected BLM and regional exposure to shock waves, a significant enhancement of chemotherapeutic effects was observed in terms of the tumor growth curve.