Cross-cultural validation of the CHO-KLAT and HAEMO-QoL-A in Canadian French.

Multi-site studies are necessary in the field of haemophilia to ensure adequate sample sizes. Quality of life (QoL) instruments need to be harmonized across languages and cultures to facilitate their inclusion. The purpose of this study was to adapt the Canadian Haemophilia Outcomes - Kids Life Assessment Tool (CHO-KLAT(©)) and HAEMO-QoL-A(©) to French for Canada. The CHO-KLAT and the HAEMO-QoL-A are haemophilia-specific measures of QoL for boys and men respectively. Both measures originated in English, were translated into Canadian French by clinicians with expertise in haemophilia care, back-translated by expert translators and harmonized by a multi-disciplinary team. The harmonized versions were evaluated through a cognitive debriefing process with 6 boys with haemophilia, their parents and 10 men with haemophilia. The final versions were validated in a sample of 19 boys with haemophilia, 19 parents, and 22 men with haemophilia along with a generic QoL scale: the PedsQL for children; and the SF-36 for adults. The translation and cognitive debriefing processes resulted in a preliminary version that maintained the intent of the original questions. The validation study estimated the mean score for the child-reported CHO-KLAT at 71.9 (SD 10.4), and the adult-reported HAEMO-QoL-A at 79.1 (SD 21.3). The CHO-KLAT correlated 0.64 with the PedsQL and the HAEMO-QoL-A correlated 0.78 with the SF-36 physical component summary score. The French-Canadian version of the CHO-KLAT and HAEMO-QoL-A are valid. These measures are available for use in multi-site haemophilia trials and clinical practices to capture QoL data from French Canadians.

Improvement of disseminated lymphangiomatosis with recombinant interferon therapy.

Disseminated lymphangiomatosis is a rare disorder with a poor prognosis. We present a case involving a 3-year-old boy who presented with pulmonary infiltrates, multiple lytic lesions of the ribcage, and small cystic lesions in the spleen. Open-lung and bone biopsies revealed disseminated lymphangiomatosis. Significant clinical and radiologic improvement were observed and persisted after 28 months of treatment with recombinant interferon alpha-2b (IFN alpha-2b). No significant toxicity has been observed.

Toxicity profile of interferon alfa-2b in children: A prospective evaluation.

The toxicity of interferon (IFN) alfa-2b therapy was prospectively evaluated in 53 children treated from 1991 to 1996 in 2 successive studies of IFN alfa therapy for severe hemangiomas at Sainte-Justine Hospital. Toxicity was generally mild and transient, with grade 1 toxicity occurring in 100% of patients, grade 2 toxicity in 89%, grade 3 toxicity in 58%, and grade 4 toxicity in 17%. Ten of 43 patients available for evaluation had an abnormal neurologic examination. Severe neurotoxicity in the form of spastic diplegia occurred in one patient. In conclusion, IFN alfa therapy is generally well tolerated in children. However, it may rarely be associated with severe toxicity and must be used with caution.

Nursing challenges in cancer pain assessment and management: setting an agenda for the CANO Pain Initiative.

The purpose of this inventory was to determine what oncology nurses perceived to be the challenges and priorities in pain assessment and management in Canada. It was recognised that there are a variety of practices in Canada, and if we are to establish an educational framework, there is a need to know what some of the issues are and what assessment tools are being used. Sixty-six survey questionnaires were sent to centres with an oncology or palliative care focus. Thirty-one surveys were returned with a great deal of information. The findings of this descriptive inventory of cancer pain assessment and management hold several key implications for future directions of the CANO Pain Initiative. The findings provide further support for ongoing efforts to educate and inform health professionals about the nature of cancer pain and associated assessment and management issues. A number of other issues have been identified with this survey which continues to support the efforts of the CANO Pain Initiative in developing educational modules, comprehensive care plans and practical ways to document pain interventions.

Renal-cell carcinoma in children: a different disorder from its adult counterpart?

BACKGROUND: Renal-cell carcinoma (RCC) is a rare tumor in children. To address whether RCC in children differs from its adult counterpart, we report a series of 16 children with RCC (5 boys, 11 girls, mean age 9.6 years, range 3-19 years) presenting between 1979 and 1996 at three pediatric centers. PROCEDURE: Pathology showed papillary RCC in five patients (31%). Nonpapillary tumors were present in 11 (69%), of which nine were clear-cell type (56%), one was chromophobe-cell type (6%), and one was granular-cell type (6%). Cytogenetic studies were performed on four. RESULTS: In two tumors, normal karyotypes (45,XX or 45,XY) were found. In another, there were translocations: t(X;1), t(X;2) and t(6;14). In the fourth, analysis revealed 46,XX/46,X,t(X;17)(p11.2;q25),t(1;12). Several features in this series differ from those reported in adults. In adults, RCC is more frequent in males, is usually nonpapillary, and is characterized cytogenetically by deletions or rearrangements in the short arm of chromosome 3. In contrast, in our series there was no male predominance and a higher proportion of papillary tumors. In addition, two of four cytogenetically analyzed tumors had translocations involving the X chromosome. Translocations involving the Xp11.2 locus have been infrequently reported in both adults and children with papillary RCC. CONCLUSIONS: The higher frequency of papillary histology and the presence of translocations involving Xp.11.2 in two cases raise the possibility of a unique subtype of RCC in children.

Phase II study of carboplatin (CBDCA) in progressive low-grade gliomas.

In this study, the authors sought to investigate the response rate and toxicity of carboplatin in patients with progressive low-grade glioma (LGG). Thirty-two patients with progressive LGG were treated with carboplatin at a dosage of 560 mg/m(2). Treatment was given at 4-week intervals and continued until the disease progressed, unacceptable toxicity supervened, or for 12 additional courses after achieving maximal response. Patients with stable disease were treated with a total of 12 cycles. All patients were treated as outpatients. Patients were evaluated for response to treatment and toxicity. All patients received a minimum of two cycles of carboplatin, and were examined for response. A partial response was achieved in nine patients (28%) and a minimal response in two (6%), for an overall response rate of 34% (11 of 32 patients). Eighteen patients (56%) had stable disease. A partial response was achieved in the nine patients after a median of six cycles (range 4-11 cycles), a minimal response was achieved in the two patients after five cycles. Glioma progression was noted in three patients after three, five, and five cycles, respectively. The 11 patients in whom some response was achieved had either an optic pathway tumor or a juvenile pilocytic astrocytoma. Twenty-six of the 32 patients had those characteristics, making the response rate in that group 42% (11 of 26 patients). Thirty-two patients received a total of 387 cycles of chemotherapy. Hematological toxicity was moderate. Twenty-one patients developed thrombocytopenia (platelet count < 50,000/microl); three patients required one platelet transfusion each. Nine patients developed neutropenia (absolute neutrophil count < 500/microl); one developed fever and required administration of antibiotic agents. One dose adjustment in each of the patients prevented further thrombocytopenia and neutropenia. Two patients with stable disease died of respiratory complications. One patient developed Grade III ototoxicity after receiving five cycles, one patient developed hypersensitivity to carboplatin, and none developed nephrotoxicity. Carboplatin given at a dosage of 560 mg/m(2) every 4 weeks has activity in patients with progressive LGG. This drug regimen is relatively simple and well tolerated. Further investigation and longer follow-up study are warranted.

Recombinant interferon alfa-2b in the treatment of vision-threatening capillary hemangiomas in childhood.

INTRODUCTION: Hemangiomas of the orbit and eyelids may cause serious ocular problems usually related to amblyopia and astigmatism. Steroids have become the accepted treatment. However, some hemangiomas are resistant to steroids or require prolonged use,with unacceptable side effects. Interferon alfa-2b, an antiangiogenic protein, was used in this prospective study to treat visually threatening hemangiomas that were unresponsive to oral or intralesional steroid treatment. METHODS: Forty patients aged 2 to 36 months with life- or organ-threatening hemangiomas were prospectively enrolled to evaluate the efficacy and safety of interferon alfa treatment for hemangiomas. Sixteen of these 40 patients had hemangiomas causing serious ocular dysfunction. The patients were treated with 3 x 10(6) U/m2 interferon alfa-2b subcutaneously daily for 3 months; treatment was then tapered or retreated according to response and protocol. Therapeutic responses were documented. RESULTS: Fifteen patients with ocular hemangiomas have finished treatment. The pretreatment volume measured by computed axial tomographywas an average of 22.3 cm3. Clinical response with eye opening was observed at an average of 6 weeks. There was a significant regression of the hemangioma in all patients, with an average 82% reduction in volume. Patients were treated with glasses and occlusion therapy as appropriate. Final visual acuities with a follow-up averaging 14 months after cessation of interferon treatment were normal, except that five of 15 patients had amblyopia; one of these patients had 20/40, two had 20/60, and two had 20/70. There were no major illnesses or serious adverse side effects. CONCLUSION: Interferon alfa-2b treatment resulted in good to excellent regression of all the hemangiomas. This regression was clinically significant,with patients able to open the affected eye an average of 6 weeks into treatment. Visual results were good, with moderate amblyopia occurring only in patients treated at a later age. Interferon alfa-2b was well tolerated by these young patients, and no significant illness or side effect has occurred.

Determinants of facial profile self-perception.

Using a simple profile-simulation device, forty-two orthodontic patients and an equal number of nonorthodontic patients indicated how they thought they appeared and how they would like to appear for comparison with actual cephalometrically determined profiles. Consistent with other studies of the self-perception of body parts, patients in both orthodontic and nonorthodontic groups underestimated the protrusiveness of their lips. For the psychological measures, the actual and perceived magnitude of the protrusiveness correlated significantly with anxiety and dissatisfaction with their profiles. Futher research is suggested to develop a more refined simulation technique for use by orthodontists and surgeons interested in obtaining reliable and valid measures of patient-perceived needs and expectations of corrective procedures.