Chemotherapy in the treatment of advanced or recurrent olfactory neuroblastoma

Background: Olfactory neuroblastoma is a rare sino‐nasal tumor arising from the olfactory epithelium and is often characterized by local invasion or metastasis. The role of chemotherapy in the treatment of this tumor is unclear. The purpose of this study was to review our institution’s experience of chemotherapy for advanced or recurrent olfactory neuroblastoma. Methods: Twenty‐one patients with histologically proven olfactory neuroblastoma were treated at our institution between 1992 and 2002. Twelve of these patients received chemotherapy in the setting of unresectable or recurrent disease and were retrospectively reviewed for clinical characteristics, treatment outcome or survival. Results: Eight patients of the 12 patients received cisplatin‐based chemotherapy and the remaining four patients received chemotherapy consisting of docetaxel plus irinotecan (three patients) or cyclophosphamide, doxorubicin, and vincristine (1 patient). A partial response was achieved in five patients, with an overall response rate of 42%, although the chemotherapeutic regimens were heterogeneous. Two partial responses were obtained among the three patients who received docetaxel plus irinotecan. The response rate to chemotherapy was 83% in the younger age group (<40 years), as opposed to 0% in the older age group (≥40 years), and the difference between the two groups was statistically significant (P = 0.02). Conclusion: Our study indicated that olfactory neuroblastoma would be sensitive to chemotherapy, especially with young patients. Docetaxel plus irinotecan has the possibility of showing favorable response, and warrants further investigation.     

HYPOURICAEMIA WITH ACUTE VIRAL HEPATITIS

We report five female cases of hypouricaemia accompanied byacute viral hepatitis (serum urate 101 ± 12 µmol/1,mean ± SD). Their urate clearance was increased to 14.2±3.4 ml/min during hyperbilirubinaemia but 24-h urateexcretion was not elevated (2.09 ± 0.64 mmol/24 h). Noother renal tubular abnormalities were detected. Comparing uratemetabolism with that of four cases of inborn renal hypouricaemia,the degree of uricosuria was lower. One patient showed elevationof serum and urinary oxypurine, which normalized with returnof a normal blood uric acid level. In all cases, the serum uratereturned to normal after improvement of liver function. We suggestthat renal uricosuria due to an isolated renal defect of uratetransport might contribute to hypouricaemia in these cases butthat inhibition of xanthine oxidase activity might also contributeto this phenomenon. KEY WORDS: Liver disease, Purine synthesisPurine synthesis, Purine synthesis, Kidney, Tubular function, Xanthine oxidase     

Interactions of Endotoxin with Cortisol and Acute Phase Proteins in Septic Shock Neonates

ABSTRACT. CRP, α1-acid glycoprotein and haptoglobin were studied in 13 septic shock neonates. Endotoxin was recovered from eight infants. Serum Cortisol concentration from infants with en-dotoxemia (917 ± 596 ng/ml) was significantly higher than that from infants without en-dotoxemia (398 ± 239 ng/ml). Serum Cortisol correlated well with immature neutrophil counts denned as the unit "band/neutrophil". Increased Cortisol level and immature neutrophil counts preceded the elevation of CRP, α1-acid glycoprotein and haptoglobin in four extremely premature neonates. We conclude that positive interactions between endotoxin, Cortisol and acute phase protein synthesis are present in the initial period of infection, and delayed acute phase protein synthesis is suspected in extremely premature neonates.     

A case of four coronary artery fistulae originating from three vessels associated with aneurysm

A case of an aneurysm associated with four coronary artery fistulaeoriginating from three vessels is reported. The patient, a 52-year-oldwoman, had chest heaviness and palpitations. Coronary arteriographyreveaed the four fistulae originating from three coronary vesselswith an aneurysm draining into the left ventricle and the mainpulmonary artery.The patient's symptoms were relieved afterfistulectomy and the aneurysmectomy, suggesting that a coronarysteal phenomenon through the fistulae was the cause of her symptoms.This case is of interest because of its rarity, since this isthe first case report of an aneurysm associated with four coronaryartery fistulae from three vessels.     

An immunohistochemical study of copper, zinc-containing superoxide dismutase detected by a monoclonal antibody in gastric mucosa and gastric cancer

The immunohistochemical localization of copper, zinc-superoxide dismutase (Cu,Zn-SOD) in human gastric mucosa and gastric cancer was studied using a monoclonal antibody. In gastric mucosa, parietal cells, pyloric glandular cells and foci of intestinal metaplasia showed positive staining in the cytoplasm and/or nucleus. The wide distribution of Cu, Zn-SOD in the gastric mucosa suggests cell function may be vulnerable to active oxygen species. In gastric cancer, 34 of 70 cases showed a positive reaction for Cu, Zn-SOD. There was a relationship between the grade of Cu,Zn-SOD immunoreactivity and the histological type of gastric cancer, well-differentiated types of gastric cancer being more frequently positive. The positive cases of poorly-differentiated adenocarcinoma were characterized by a pattern of diffusely infiltrative invasion. These results suggest that some types of gastric cancer are resistant to active oxygen species.     

No linkage to the COL4A3 gene locus in Japanese thin basement membrane disease families

Summary: Patients with thin basement membrane disease (TBMD) exhibit persistent haematuria with a diffuse thinning of the glomerular basement membrane (GBM), especially of the lamina densa. It appears to be an autosomal dominant trait. It has been reported that the Goodpasture epitope, which is located in the non-collagenous domain of type IV collagen α 3 chain, may be reduced in patients with TBMD. We speculated that the candidate gene for TBMD could be the type IV collagen α 3 chain gene ( COL4A3 ), which is present closely to type IV collagen α 4 chain gene ( COL4A4 ) on chromosome 2q35–37. We conducted a linkage analysis to investigate the relationship between familial TBMD and COL4A3 gene, using COL4A3 cDNA polymorphism and a (CA)_n microsatellite marker located in the COL4A3 gene. We examined 32 individuals from four Japanese families with TBMD. There were no associations between the patients with haematuria and certain alleles of the two markers in the pedigrees of three families. It has been reported that type IV collagen α 1 chain gene ( COL4A1 ) and α 2 chain gene ( COL4A2 ) are not involved in TBMD, and that α 5 chain gene ( COL4A5 ) and a 6 chain gene ( COL4A6 ) map to chromosome X. In conclusion, our findings suggested that familial TBMD is not caused by the genetic abnormalities of type IV collagen genes isolated thus far.     

Chemical modification of superoxide dismutase Extension of plasma half life of the enzyme through its reversible binding to the circulating albumin

Protection of organisms from oxidative stress is one of the major prerequisites for aerobic life. Since intravenously injected Cu⁺⁺/Zn⁺⁺ -type superoxide dismutase (SOD) rapidly undergoes renal glomerular filtration and appears in urine in its intact form, its clinical use as a scavenger for superoxide radicals has been highly limited. To test whether reversible interaction of SOD with plasma albumin might decrease the rate of disappearance of the enzyme from the circulation, the lysyl residues of the human erythrocyte-type enzyme were covalently linked with poly-(styrene-co-maleic acid) butyl ester (SMA) via amide linkage. Affinity chromatographic analysis by an albumin-Sepharose column revealed that the enzyme samples labeled with SMA (SMA-SOD) tightly bound to the column, while unmodified SOD was eluted in the unbound fractions. SMA-SOD bound to the column could be eluted by the buffer solution containing 0.1% sodium dodecylsulfate. In vivo analysis revealed that intravenously administered SMA-SOD circulated bound to albumin with an extremely long half-life (6 h), while unmodified SOD rapidly underwent renal glomerular filtration with a plasma half-life of 4min. Thus, SMA-SOD may effectively dismutate superoxide radicals in the circulation.     

A Clinical and Experimental Study on Longitudinal Deficiencies in Lower Limbs in Relation to Pathogenesis of Cleft Foot

Abstract In order to determine whether cleft foot is caused by the same mechanism as tibial and fibular ray deficiencies, clinical cases of these anomalies and those in rat fetuses induced by myleran were analyzed. In tibial and fibular ray deficiencies, arrest of tibia or fibula was closely related to the missing of toes and tibial and fibular ray deficiencies can be accepted as an occurrence of so called longitudinal deficiency. On the other hand, cleft feet were frequently associated with central Polydactyly and syndactyly. In our experimental study, the critical period of formation of cleft foot was different from that of tibial ray deficiency, but it was similar to those of central Polydactyly and syndactyly. It seems that cleft foot is caused by the abnormal induction of toe rays as in cleft hand and does not belong to the same group as tibial and fibular ray deficiencies.     

Clinical Features of Congenital Ankylosis of the Digital Joints of the Hand

ABSTRACT: Clinical features and roentgenographic findings of 37 patients (65 hands) with congenital ankylosis of the digital joints, including symphalangism and other types of congenital ankylosis of the joints, were analyzed. Congenital ankylosis of the digital joints was divided into four types according to the clinical features as follows; Type A (typical symphalangism), 13 cases, Type B (symphalangism without associated anomalies), 6 cases, Type C (symphalangism associated with hypoplasia of the affected digit), 10 cases and Type D (symphalangism as a part of syndrome), 8 cases. Roentgenographic findings of the affected joints were divided into 4 types, such as normal type (Type I), narrow type (Type 2), flat type (Type 3) and bony ankylosis (Type 4). There seems to be 4 types of joint development in congenital ankylosis of the digital joints. In Types 1 and 2, the joint space of the affected joint and the secondary ossification center looks normal. The joint seems to develop normally in Type 1, but the condyle of the affected joint becomes flat in Type 2. In Type 3, the joint space is narrow in infancy, the secondary ossification center fuses with the proximally located phalanx and finally the affected joint develops bony ankylosis. In Type 4, there is osseous fusion of the affected joint at birth. Key words: hand, joint, symphalangism, tarsal coalition, carpal coalition     

Minute Rectal Cancer Associated with Nodular Involvement —A Case Study—

Abstract: This study presents the case of a patient with minute type Ha rectal cancer with a diameter at its largest of only 5 mm, with infiltration as far as the submucosal layer (sm) and positive parietal lymph node metastasis. The patient was a 54 year-old male who visited Yasuda Medical Hospital because of diarrhea which appeared in early May 1988. During sigmoidscopy, a small protruding lesion was seen in the rectum (Rs) about 12 cm from the margin of the anus, and the patient was referred to the authors' surgical service for an endoscopic polypectomy because of a biopsy diagnosis of adenocarcinoma. The polyp had a smooth, shiny surface, and had a well demarcated hemispherical shape. A histopathological examination of the polypectomized specimen, showed that it was an invasive carcinoma extending into the submucosal layer without any adenoma component. Since the cut end of the specimen strongly suggested positive cancer cells and lymphatic permeation was also confirmed from the polypectomized specimen, a low anterior resection was performed on August 4, 1988. The postoperative histological examination revealed a small amount of residual cancer cells in the submucosal layer which appeared to be at the cut end of the resected polyp. One metastatic focal point was seen in the pararectal lymphnode, and this patient's case provided valuable suggestions for deciding upon therapeutic policies for early cancer of the large intestine.