摘要:

AbstractUrinary excretion of cystathionine and dopa metabolites was analyzed in 61 patients with active neuroblastoma before, and at regular intervals during treatment. Thirty‐seven patients with clinical evidence of active neuroblastoma excreted elevated levels of cystathionine before treatment was initiated; six other patients showed cystathioninuria at some time during treatment with chemo‐ or radiotherapy. The cause of the cystathioninuria remains unidentified. No relationship between excessive cystathionine excretion and liver impairment or liver metastases was established; nor was there evidence to support a consistent correlation with the ratio of the sum of the excretion values for vanilglycolic acid and vanilglycol to the excretion of vanilacetic acid. Our results indicate that absence of cystathioninuria correlates with an early clinical staging and thus with a favorable prognosis. Isolated cystathioninuria does occasionally occur in patients with neuroblastoma, permitting a presumptive diagnosis until later evidence can be obtained. Determination of cystathionine excretion is essential for an extensive biochemical evaluation of patients with neuroblast

ISSN:0098-1532    

DOI:10.1002/mpo.2950120203

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractA 4‐year‐old boy had a pulmonary blastoma removed from the site of a persistent pulmonary cystic lesion present since 6 weeks of age. Bone metastasis appeared 4 months after diagnosis. Following radiation therapy to the bone and after 15 months of VAC chemotherapy, he developed further metastases which showed little response to adriamycin or radiation therapy. He died 2 years and 2 months after the initial operation. A review of other pulmonary blastomas associated with cystic lesions in children is presen

ISSN:0098-1532    

DOI:10.1002/mpo.2950120204

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractSerum lactate dehydrogenase activity (LDH) was examined in 66 children with acute lymphoblastic leukemia (ALL), 26 with acute non‐lymphocytic leukemia (ANLL), and 116 with non‐Hodgkin's lymphoma (NHL). The mean serum LDH value for the ALL and ANLL groups was not significantly different: 970 ± 105 units/L (mean ± standard error of the mean) and 817 ± 161 units/L, respectively. The difference between the LDH values in patients with ALL (970 ± 105 units/L) and NHL (551 ± 51 units/L) was significant (P= .001). In 32% of the patients with ALL and 23% of the patients with ANLL, serum LDH values were above 1000 units/L, whereas only 13% of the cases with NHL had values above 1000 units/L. In patients with ALL the LDH levels were correlated with white blood cell counts at the time of diagnosis. In NHL, there was no difference in serum LDH levels among the various histologic subtypes. Values of LDH in stage IV NHL and in ALL were

ISSN:0098-1532    

DOI:10.1002/mpo.2950120205

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractNeural crest tumors can be complicated by secretory diarrhea mediated by vasoactive intestinal peptide (VIP). An eight‐month‐old male with a several‐month history of secretory diarrhea is described. Elevated urine vanillylmandelic adic (VMA), total urine catecholamines, and plasma VIP indicated that a neural crest tumor was responsible for his protracted diarrhea. An extensive search for the tumor including CT scans of his head, neck, thorax, abdomen, and pelvis was unre vealing. A selective vena caval catheterization showed elevated catecholamines in a sample obtained above the renal veins. Subsequent laparotomy disclosed a benign ganglioneuroma arising from the left adrenal; the diarrhea resolved after its removal. Selective venous sampling proved useful in establishing the tumor's location where other techniques had been unsucce

ISSN:0098-1532    

DOI:10.1002/mpo.2950120206

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractTesticular open‐wedge biopsy was performed in 35 children in complete remission from acute lymphoblastic leukemia without clinical signs of leukemic testicular infiltration at the time of treatment discontinuation. Histological investigation showed thickening of the tunica propria of the seminiferous tubules in 13 of 35 patients. In 5 of 35 patients, the tubular fertility index was markedly reduced; in 5 of 6 pubertal patients, decreased spermatogenesis or aplasia of germinal epithelium was observed. Histologic damage was found mainly in the germinal cells both in patients treated with cytosine arabinoside and cyclophosphamide and in those treated with antiblastic drugs not considered damaging to the gonads. The extent of impairment was independent of age at start of treatment. On the other hand, endocrinological investigation carried out at the crucial moment of treatment suspension showed normal hypothalamic‐hypophyseal‐gonadal function as well as normal anthropometric data, bone age, and pubertal stage in the majority of patients. Testicular leukemia was found in only one patient (2.8%) whereas three children with negative testicular biopsies had testicular relapses within 7 months. Therefore, in view of the limits of light microscopy in diagnosing leukemic infiltration at treatment discontinuation, we propose the use of more sophisticated techniques, possibly within 6 months of suspension of th

ISSN:0098-1532    

DOI:10.1002/mpo.2950120207

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractSince 1974, we have been mass screening for neuroblastoma in 6‐month‐old infants using a VMA (vanilmandelic acid) spot test in Kyoto City. Fifteen (48.4%) of the 31 childhood neuroblastoma cases registered in this city during the recent 8‐year‐period, 1974‐1982, were under 1 year of age. The annual number of cases per year was 3.9 cases under 15 years with 1.9 cases under 1 year of age. As the number of children under 15 years of age in this city was 292,000 according to the National Census of the Human Population in 1975, the annual incidence of this tumor in childhood was 3.9/292,000 (13.3/million). And as the number of live births was 161,153, the annual detection rate for this tumor in infancy increased to 15/161,153 (93/million) from 1974 to 1982. We found that many neuroblastomas could be detected in infancy by a VMA spot test and/or a careful physical ex

ISSN:0098-1532    

DOI:10.1002/mpo.2950120208

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractNineteen patients with advanced malignant tumors, less than 20 years old were treated with intensive chemotherapy (vincristine 2 mg/m2i.v. and adriamycin 60 mg/m2i.v. on day −7; cyclophosphamide 45 mg/kg i.v. on days −6 to −3), total body irradiation (TBI, 600 rads on day −1) and autologous bone marrow transplantation (ABMT, day 0). Prior to this procedure induction of complete or partial remission by conventional therapy was attempted. Ten patients had intra‐abdominal non‐Hodgkin's lymphoma (NHL); three, yolk sac tumor; three, Ewing's sarcoma; and three, neuroblastoma. The supportive care included reverse isolation, immunoglobulin 400 mg/kg i.v. q 2 weeks, cotrimoxazole per os, and cell support as needed. No correlation between the bone marrow dose and the time of hematological reconstitution could be established. Five of seven patients with intraabdominal NHL stage III (transplanted in first remission) are surviving disease‐free for 5 +, 5 +, 20 +, 23 +, and 35 + months after ABMT. None of three patients with intra‐abdominal NHL stage IV is surviving (two of them were transplanted in second remission). One of three patients with yolk sac tumor is surviving disease‐free for 27+ months. There are no survivors among the patients with Ewing's sarcoma and neuroblastoma. Only one of 19 patients was lost due to therapeutic complications, while 12 died due to tumor. Regarding treatment results for advanced intra‐abdominal NHL, the procedure described here is comparable to the best conventional regimens. In vitro methods for tumor cell eradication in the collected bone marrow might further improve

ISSN:0098-1532    

DOI:10.1002/mpo.2950120209

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractA 9‐year‐old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which reresulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported bef

ISSN:0098-1532    

DOI:10.1002/mpo.2950120210

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

ISSN:0098-1532    

DOI:10.1002/mpo.2950120211

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY

摘要:

AbstractAn 11‐year‐old white girl was admitted to The Children's Hospital of Philadelphia in July 1983 for evaluation of metastatic tumor. She had been well until July 1982, when a mass developed over the right scapula. Treatment with warm compresses and antibiotics resulted in no improvement, and incision and drainage were unproductive. A biopsy at another hospital was interpreted as showing a primitive neuroectodermal tumor. There was no evidence of metastatic disease. She then underwent excision of the tumor with the underlying scapula. No further treatment was administe

ISSN:0098-1532    

DOI:10.1002/mpo.2950120212

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1984

数据来源: WILEY