Sialoblastoma.

Tumours of the salivary gland are very uncommon in children. We present a case of sialoblastoma, a very rare congenital salivary-gland tumour, initially recognised on antenatal ultrasound. After histological diagnosis, a superficial parotidectomy was performed at 21 days of age with preservation of the facial nerve. There is no recurrence at2 years.

A study of clinical opinion and practice regarding circumcision.

AIM: To establish clinical opinion regarding appropriate indications for circumcision and to examine actual clinical practice. METHODS: A questionnaire was sent to all NHS hospital consultants in the Yorkshire region of the UK identified as having a role to play in the management of boys (under 16 years of age) requiring circumcision. Retrospective data on actual clinical practice during a three month study period were also collected via a simple proforma. RESULTS: Of 153 questionnaires sent, 64 were returned. Responses revealed varying opinions regarding appropriate indications for circumcision within each consultant group, and between paediatricians and surgeons. Surgeons were generally more inclined to recommend circumcision for each of the indications listed in the questionnaire. Analysis of clinical practice revealed that almost two thirds of procedures were carried out for phimosis, and nearly half of these children were under the age of 5 years. CONCLUSION: There are differences in the clinical opinions of surgeons and paediatricians on what constitutes an appropriate indication for circumcision. Paediatricians' opinions are generally more in line with current evidence than those of surgeons, possibly resulting in many unnecessary circumcisions.

Venous patency after open central-venous cannulation.

To investigate the value of Doppler ultrasound scan (USS) assessment of internal jugular vein (IJV) patency after previous open central-venous cannulation (CVC), a prospective study of 66 consecutive children (median age 4.5 years; range 4 months-17 years) who had previously undergone open insertion of at least one indwelling IJV line and required further CVC for completion of therapy was undertaken. All underwent Doppler USS examination prior to surgery. Where patency of the previously cannulated vein was suggested ultrasonographically, the accuracy of this finding was confined at open surgical exploration. Initial CVCs were in situ for a median of 9 months (1 month-4 years) prior to removal. The median interval to repeated CVC was 11 months (3 weeks-45 months). In 79 Doppler USS, 70 (88.6%) veins appeared patent, 3 (4.2%) stenosed, and 6 (7.6%) obliterated. Of the 70 "USS patent" veins, 66 were explored. Patency was confirmed surgically in 59 (89.4%) and a new CVC successfully inserted. Seven (10.6%) apparently patent veins on USS were found to be obliterated at open exploration. Review of USS images in these cases suggested that enlarged collateral veins were usually responsible. Overall, successful recannulation was possible in 74.6% of all previously accessed veins. In children requiring repeated CVC, Doppler USS of neck veins is a valuable but not entirely reliable guide to the presence of underlying vessel patency and should be interpreted with caution. At least three-fourths of previously cannulated IJVs remain patent after catheter removal and can be reused for CVC.

Extraosseous osteogenic sarcoma--a rare pediatric malignancy: case report and review of the literature.

BACKGROUND: Osteogenic sarcoma rarely occurs in soft tissues and generally affects individuals beyond the second decade of life. METHODS: The authors report a rare case of an extra osseous osteogenic sarcoma arising in the retroperitoneum of an adolescent, review the literature, and outline the diagnostic and therapeutic dilemmas. The role of adjuvant chemotherapy, using drugs used in managing bony osteosarcomas, is discussed. CONCLUSIONS: Retroperitoneal sarcomas may simulate ovarian teratomas. Careful consideration of the differential diagnosis of large cystic abdominal masses in adolescent females when size precludes adequate assessment of tumor mobility and imaging fails to demonstrate the ovaries is essential if these rare tumors are to be managed effectively.

Cytogenetics and the surgeon: an invaluable tool in diagnosis, prognosis and counselling of patients with solid tumours.

BACKGROUND: Cytogenetic studies have become an important part of tumour characterization. In a minority of tumours cytogenetic and/or molecular studies may be of help for diagnosis, selection of treatment protocols and predicting outcome. METHODS: This article reviews the literature on solid tumour translocations and established family cancer syndromes. In addition, the potential importance of genetics in management of patients with breast, ovarian and pancreatic carcinoma is reviewed. RESULTS AND CONCLUSION: Cytogenetic analysis of tumours may help confirm the diagnosis when conventional histology demonstrates no evidence of differentiation along a particular tissue pathway. For patients with well defined cancer syndromes, cytogenetic and molecular analysis offers the prospect of screening to reduce the risk of malignant disease. For many malignancies the underlying genetic anomalies are gradually being elucidated. Further studies designed to demonstrate the significance of these findings can only be undertaken if tumour material is collected and stored in optimal conditions for cytogenetic and/or molecular biological studies.

Participação do estado contrátil e do relaxamento miocárdico na disfunção ventricular durante a transição hipertrofia-falência cardíaca / Myocardial function during the transition from compensated left ventricular hypertrophy to failure

OBJETIVO - Avaliar a participação do estado contrátil e do relaxamento miocárdico na disfunção do músculo cardíaco durante a transição hipertrofia-falência cardíaca em ratos espontaneamente hipertensos (SHR). MÉTODOS - Músculos papilares isolados do ventrículo esquerdo de SHR com insuficiência cardíaca (SHR-IC) e sem falência (SHR) e de ratos normotensos controle Wistar-Kyoto (WKY) foram estudados em contraçöes isométrica e isotônica, em solução de Krebs-Henseleit (1,25 mM Ca 'elevado a +2', 28 'graus Celsius'). RESULTADOS - Os valores da tensão máxima desenvolvida (TD) e da velocidade máxima de encurtamenton (V máx) foram menores nos SHR-IC e SHR, em relação aos WKY (p<0,05). TD e V máx foram semelhantes nos SHR-IC e SHR (p>0,05). A rigidez passiva do músculo aumentou significantemente nos SHR-IC (p<0,05 vs WKY e SHR); esta variável não diferiu entre WKY e SHR (p>0,05). CONCLUSÄO - Os dados obtidos mostram que a transição da fase de hipertrofia estável para insuficiência cardíaca nos ratos espontaneamente hipertensos está associado ao aumento da rigidez passiva do miocárdio e näo à piora da funçäo contrátil do músculo cardíaco.

Mesenchymal tumors of the mouse urinary bladder with vascular and smooth muscle differentiation.

Bifenthrin, a synthetic pyrethroid insecticide/miticide, has been fed to male and female Swiss Webster mice at levels of 0, 50, 200, 500, and 600 ppm in the diet for between 604 and 644 days. Tumors of the urinary bladder were observed and initially reported as leiomyosarcomas. Subsequently, the bladders were reviewed and the tumors showed a pattern of both epithelioid cells and spindle cells forming irregular vascular channels. The tumors appeared to arise from the trigone of the bladder and, in some cases, invaded the bladder wall. No metastases were recorded. The tumor is usually considered rare; however, in this study, it was commonly observed in all groups but predominantly in males. The histogenesis of the tumor is uncertain, but from its pleomorphic histological features, including smooth muscle and vascularity, it is probably derived from vascular mesenchyme.

The use of 212Pb-labeled monoclonal antibody in the treatment of murine erythroleukemia.

PURPOSE: The goals of this study were to learn whether the DOTA chelator was useful for targeting lead radionuclides (203,212 Pb) to cells and tissues invaded by the Rauscher leukemia virus (RVB3) and to investigate the therapeutic efficacy of targeted 212Pb in treating the murine leukemia. METHODS AND MATERIALS: Five to 6-week-old BALB/c mice were inoculated i.v. with RVB3. This virus causes marked splenomegaly and death by day 13 and day 70 postinfection, respectively. Biodistribution, tumor targeting, and toxicity studies were performed using varying doses of 212Pb-DOTA-103A. A heavy metal chelator, DMPS, was administered orally and parenterally in two phases of the toxicity study. RESULTS: Biodistribution studies showed marked tumor targeting (58% ID/g spleen) in mice treated with 203Pb-103A as compared with mice treated with control antibody B3 (4.6% ID/g spleen). Histologic cure was achieved in all leukemic mice treated with 20 muCi212Pb-103A; however, all of the mice died with leukopenia and secondary++ bacterial infections due to severe bone marrow toxicity. Nonleukemic mice and mice treated with 20 muCi212Pb-B3 experienced less marrow toxicity and longer survival. Coadministration of the heavy metal chelator did not diminish the bone marrow toxicity. CONCLUSION: An effective, nonlethal dose could not be established to treat this tumor. The severe bone marrow toxicity associated with this radionuclide may limit its usefulness in systemic radioimmunotherapy.

Confounded carcinogenicity study of sodium fluoride in CD-1 mice.

To determine its carcinogenic potential, sodium fluoride (NaF) was fed to CD-1 mice for up to 97 weeks. Mice given NaF at a dose of 4, 10, or 25 mg/kg of body weight per day added to a low-fluoride diet were compared to controls given either an unsupplemented low-fluoride diet or laboratory chow. Nonneoplastic changes consistent with those previously recognized from fluoride toxicity were observed in teeth, bones, and joints. Unexpectedly, osteomas occurred in all groups. The incidence of osteomas was similar in groups given the low-fluoride control diet, laboratory chow, or NaF doses of 4 or 10 mg/kg per day. The incidence of osteomas in these groups was increased over that historically experienced at the laboratory and reported in the literature for CD-1 mice. The incidence of osteomas in the mice given 25 mg NaF/kg per day added to a low-fluoride diet was increased over that in the other groups. Osteomas were first observed at Week 55. No malignant bone tumors were observed during the course of the study. The locations, multiplicity, and morphologic features of the osteomas in all groups were similar to those associated with virus-induced bone tumors. Electron microscopic examination revealed abundant retrovirus particles in all osteomas examined from control and test mice. It was concluded that the study was confounded by a retrovirus which contributed to the induction of the osteomas. Because the study was confounded, it cannot be considered a valid bioassay to be used for risk assessment.

The clinical application of the Malone antegrade colonic enema.

Continent, catheterizable enterostomies (Malone stomas) have been formed in 25 patients. A reversed appendicocecostomy was used for the majority, although experience has led to modifications of this procedure. Antegrade enemas are regularly administered via these stomas in the treatment of fecal incontinence and intractable constipation. There have been only two important surgical complications, and the technique has proved to be highly successful. Follow-up of the patients has shown that individual requirements can be met with variations in the administration of the enemas.

Usefulness of synthetic Mimetic ligands in establishing an affinity chromatography procedure for purifying estradiol antibody in sheep serum: potential application in regulating fertility of the ram.

Recently developed synthetic Mimetic affinity ligands were screened for their ability to purify estradiol antibody from sheep serum. One of these ligands (color coded Yellow 1) effectively separated immunoglobulin from other serum protein by negative affinity. When 1 ml antiserum diluted 70% (protein content approximately 25 mg) was passed through 1 ml gel with Yellow 1 ligand, estradiol antibody-binding activity (% binding of E2 tracer) peaked in the effluent (62%) and decreased progressively in the subsequent three 4-ml fractions of the wash (34%, 6% and 2%, respectively). As long as the total amount of serum protein applied remained below the saturable-binding capacity of the ligand (determined to be approximately 28 mg), the relative purity of estradiol antibody in the effluent (E2 binding per unit protein) remained very high with only 3% (0.78 mg) of applied protein appearing in the effluent. Purity of antibody in the effluent was confirmed by electrophoresis carried out on SDS polyacrylamide gel. Flow rate of serum diluted 42% (1 ml, protein content approximately 48 mg) was constant and acceptable (0.37 ml/min) and uncoupling of nonimmunoglobulin serum protein from the ligand was complete by the time the column was reequilibrated. We conclude that of the different Mimetic ligands evaluated Yellow 1 has properties that would be useful in a large-scale affinity chromatography procedure for purifying immunoglobulin from sheep serum.

Effective alpha-particle-mediated radioimmunotherapy of murine leukemia.

The specificity, toxicity, and efficacy of alpha-particle-mediated radioimmunotherapy of murine erythroleukemia was assessed by use of tumor-specific monoclonal antibody 103A labeled with 212Bi. Forty % of the injected dose/g tissue targeted to neoplastic spleens within 1 h after i.v. injection. When 212Bi-103A was injected on day 13 of disease, a dose-dependent response was achieved, as measured by a reduction in splenomegaly and absence of liver metastasis. Mice treated with 212Bi-103A on day 8 of disease showed no histological evidence of erythroleukemia on day 22 and survived significantly longer (median, 118 days) than mice treated with 212Bi-control IgG (78 days) or untreated mice (63 days), indicating successful specific radioimmunotherapy.

Intravascular haemolysis in association with necrotising enterocolitis.

Severe haemolysis is an unusual occurrence in association with necrotising enterocolitis (NEC). Activation of the Thomsen-Friedenreich (T) antigen on the neonatal erythrocytes is proposed as the mechanism. This haemolytic process is precipitated by transfusion with serum-containing blood products, which should therefore be avoided. Once haemolysis has become established the outcome is usually fatal. This report describes the management of four neonates who had established haemolysis complicating severe NEC. T activation was confirmed in two infants, but could not be tested for in the other two. Exchange transfusion abolished the haemolysis in two patients, who survived. Despite reduced use of blood products the other two infants died. We believe that exchange transfusion was essential in the successful management of the infants who survived, and recommend early exchange transfusion when haemolysis complicates NEC.