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1.
This paper reviews a series of 70 consecutive children with rhabdomyosarcoma (RMS) diagnosed during 1971-1978 and treated in five Italian institutions. Thirteen were classified as group I, 12 as group II, 37 as group III, and 8 as group IV according to the Intergroup RMS Study staging system. Survival was influenced by tumor extension at diagnosis, primary site, and response to therapy. The 5-year-survival rate was 92% for group I patients, 67% for group II, 44% for group HI, and 0% for group IV. Thirty-four children had all therapy stopped after 12-32 months of complete remission, 7 had late recurrences, and 3 died from disease. Musculoskeletal sequelae were diagnosed in 11 children, short stature in 3, corneal opacity in 2, and cardiac failure in 1.  相似文献   

2.
Head circumference, height, bone age and weight were studied in 103 children with congenital hypothyroidism before and up to 8 years of thyroid replacement therapy. The patients were divided into 4 groups according to the age at start of treatment: group I (diagnosed by neonatal screening): less than 2 weeks (n = 55); group II: 1-3 months (n = 7); group III: 4-12 months (n = 15); group IV: greater than 1 year of age (n = 26). Before treatment, group I showed a head circumference significantly larger than normal and a delay in bone maturation in the presence of normal length and weight. In the other groups length as well as bone age were significantly lower than normal, head circumference, in contrast, was normal (groups II and III) or even increased (group IV). During therapy, head circumference and bone age of group I became normal as were length and weight from the beginning. In the other groups, therapy led to a further increase of head size resulting in a mean head circumference significantly larger than normal during 8 years of observation in group IV. There was a catch-up of height, bone age and weight in groups II, III and IV; mean height of late treated children (group IV), however, remained significantly lower than normal even after 8 years of therapy. - Our study shows that congenital hypothyroidism is associated with increased head circumference, either absolutely or in relation to stature. Thyroid hormone therapy resulted in a normalization of head growth when treatment was initiated early, and in a further increase when treatment was started late. There was a catch-up of height, bone age and weight; complete normalization, however, occurred only in those children treated before one year of age.  相似文献   

3.
Four induction regimens-prednisolone and 6- mercaptopurine (group I), prednisolone and vincristine (group II), prednisolone, vincristine & asparaginase (group III) and prednisolone, vincristine and adriamycin followed by cyclophosphamide and 1-asparaginase (group IV)- have been evaluated. Successful induction remission was achieved in 16 (69.6%) in group I, 23 (92.0%) in group II, 36 (94.7%) in group III, 31 (96.8%) in group IV. Relapses were seen in 10 (62.5%), 10 (73.8%), 25 (69.4%) and 9 (29.0%) in the four groups respectively. Relapses seen in group IV were infrequent as compared to children of group I, II & III. Adverse risk factors were similar in all the four groups. Ninety four children (61.3%) had one or more poor prognostic factors at diagnosis. Three of 43(7%) children with no poor prognostic factors died during induction therapy as compared to 18 of 94 (19.1%) children associated, with poor prognostic factors at diagnosis. Higher mortality was seen under two years of age.  相似文献   

4.
目的 分析儿童横纹肌肉瘤(rhabdomyosarcoma,RMS)的临床特点及影响预后的因素。方法 纳入2014年2月至2020年9月就诊于中南大学湘雅医院儿科血液肿瘤专科的20例RMS患儿为研究对象,回顾性分析患儿的临床资料和随访资料。结果 20例RMS患儿初次就诊时临床症状依次为无痛性肿块(13/20,65%)、眼球突出(4/20,20%)、腹痛(3/20,15%)。依据国际横纹肌肉瘤研究组(Intergroup Rhabdomyosarcoma Study Group,IRSG)的分期标准,Ⅰ期1例(5%)、Ⅱ期4例(20%)、Ⅲ期9例(45%)、Ⅳ期6例(30%)。20例患儿中位随访时间为19 (3~93)个月,2年总体生存(overall survival,OS)率为79.5%(95%CI:20.1~24.3),2年无事件生存(event-free survival,EFS)率为72.0%(95%CI:19.5~23.9)。病理类型为多形性可降低RMS的2年OS率(P<0.05),有远处转移、IRSG分期为Ⅳ期、危险度为高危组可降低RMS的2年EFS率(P<0....  相似文献   

5.
This retrospective study was undertaken to evaluate the effect of delayed resection on outcome of head and neck rms in a single institution which has experience in cranial base surgery. Since 1988, patients with primary non-orbital rms of the head and neck following treatment at the Children's Hospital of Pittsburgh, were evaluated by the Department of Otolaryngology, Eye and Ear Hospital at the University of Pittsburgh Medical Center either at the time of presentation or when response to chemotherapy and/or radiation therapy was thought to have been optimized for the possibility of definitive surgery. Medical records of patients who did or did not have delayed surgery were reviewed and compared with respect to demographics, tumor stage, response to therapy, survival, and cosmetic results. Of 16 children diagnosed with non-orbital head and neck rms from 1988–1994 and treated with chemotherapy according to IRS II–IV, 3 had group I or II disease following extensive surgery at diagnosis. Thirteen had group III or IV disease. Of these, 6 patients had delayed resection and 7 did not. Delayed resection was undertaken 3–12 months (median, 4 months) from diagnosis in 4 children who had a partial response (PR) and 2 children who had stable disease (SD) with chemotherapy and/or radiation. Delayed resection converted all children to complete responses (CR), including one child with clinical SD and one with PR who were found to have no viable tumor at surgery. The overall percentages of CRs for patients with group II or IV disease (documented any time post-diagnosis) were at least as good for patients who had undergone delayed surgery as for those who had not (100% vs. 71%, p = .465). Median survivals for patients with advanced disease were 3 ½ years and 2 years, respectively (p = .2801). Cosmetic and functional problems attributable to surgery were not severe but included facial asymmetry (n = 4), trismus (n = 1), cranial nerve deficits (n = 1), and abnormal dentition (n = 1). In locally extensive head and neck rms, cranial base surgery should be considered after initial cytoreductive therapy, since it may contribute to achievement of CR and to survival with acceptable morbidity. Med. Pediatr. Oncol. 28:294–298. © 1997 Wiley-Liss, Inc.  相似文献   

6.
One hundred and twenty nine children with chronic lead poisoning were followed from August 1985 to July 1989. Old lead paint was recognized as the contaminant source at home. Pica of paint flakes was the main mode of intoxication. Children were classified according to the Center for Disease Control 1985 as follows: class IV (39 cases), class III (45 cases), class II (30 cases), class I (15 cases). Nineteen of those in class IV had blood lead levels above 700 micrograms/l and received BAL + EDTA followed by EDTA alone for a mean of 4.6 +/- 3.5 courses. With this treatment, blood lead level decreases were 50 +/- 17%. Nine of these class IV children had an evaluation at last 3 months after the last chelation course: 5 became class I or II, and 2 class III with a negative provocative test. The remaining 20 children in class IV were given a mean of 2.7 +/- 1.4 courses of EDTA. Blood lead levels decreased by 52 +/- 15%; 11 children were evaluable at least 3 months after the last chelation course: 4 became class I, and 7 class II. Thus overall 80% of class IV moved under treatment to class I or II. Among those 45 children in class II, 30 underwent a provocative test and 24 one to three courses of EDTA: 8 were further studied: 3 became class I and 5 class II. Combination of screening, medical treatment and sociocultural approach led to avoid acute effects of severe chronic childhood lead poisoning. The efficacy of such an approach in preventing chronic effects has still to be evaluated.  相似文献   

7.
Children and adolescents who develop rhabdomyosarcoma (RMS) and related sarcomas in the orbit and treated on Intergroup Rhabdomyosarcoma protocols have had an extremely high cure rate. This study evaluates the possible relationship between their tumor morphologic subtypes and this high cure rate. The histology of tumors was re-reviewed from 229 of the 264 patients with tumors of the orbit, conjunctiva, and eyelids treated on Intergroup Rhabdomyosarcoma Studies (IRS) I, II, III, and IV pilot protocols, and followed through July, 1992. Immunohistochemistry was applied in selected cases. Clinical correlations were done on all 264 cases including both the re-reviewed cases and those reviewed only by the IRS Pathology committee. The 5-year survival rate of 24 children with alveolar RMS was 74% (p < .001). All five infants diagnosed to have an alveolar RMS died before the age of one. Two hundred and twenty-one patients (84%) had embryonal RMS. About three-fourths of the re-reviewed embryonal RMS tumors showed only minimal rhabdomyoblastic differentiation. Thirty-one had a spindle cell RMS, two were anaplastic variants. The 5-year survival rate for patients with embryonal RMS subtypes combined was 94%, and 97% for the 144 patients with poorly differentiated embryonal RMS. In contrast, 190 of 432 IRS II patients treated for poorly differentiated embryonal RMS located in extraocular sites had a 66% survival estimate. Med. Pediatr. Oncol. 29:51–60, 1997. © 1997 Wiley-Liss, Inc.  相似文献   

8.
Abstract Sixty-four children with malignant brain tumours diagnosed at less than 3 years of age were reported to the Finnish Cancer Registry from 1975 to 1993. The survival rate has improved significantly: the 5-year survival rate was 26% for all children, 13% for children diagnosed during 1975-85 ( n = 30) and 40% for those diagnosed during 1986-93 ( n = 4). Of the surviving children in 1986-93, 43% were categorized in Bloom's group I or II and could lead active lives without major disabilities. The remaining children had severe neurologic late complications, such as hemiplegia, intractable seizures, and mental retardation.  相似文献   

9.
Prophylaxis with factor concentrates in preventing hemophilic arthropathy.   总被引:5,自引:0,他引:5  
Seven children with severe hemophilia A on prophylactic substitution therapy since a mean age of 5 years (group I) were investigated in 1978 (at ages 6-12 years) and in 1988 (at ages 16-22 years). The results were compared with those of seven children aged 5-12 for whom such treatment was started at a mean age of 3 years (group II). In group I, four had each had more than 20 ankle hemarthroses at the first investigation, while in group II, only one boy had experienced such a high bleeding frequency. Radiological changes in ankles were found in one of seven in group II compared with five of seven in group I. Progression of these changes was shown in eight of 10 ankles of group I at reinvestigation. Regular prophylactic therapy must start early, at ages 1-2 years, to prevent changes in ankle joints, and parents and children must learn to recognize ankle bleeding. Modern Factor VIII concentrates must be administered to children two or three times per week in dosages of 3,000 U/kg/year in order to reduce hemarthroses to a minimum. The dosages can probably be lowered if the intervals are shortened. Children on prophylactic treatment can engage in regular sports activities.  相似文献   

10.
Fifty children with malignant diseases were vaccinated against hepatitis B. Twenty-nine children suffered from leukaemia or non-Hodgkin's lymphoma; 14 of these were on intensive chemotherapy (group I) and 15 were without intensive therapy (group II). The other 21 children had various forms of solid tumours, 14 of them were on intensive therapy (group III) and 7 were without intensive therapy (group IV). To evaluate the immune response, we determined antibody titres over a period of more than 14 weeks after the first vaccination. As 22 out of 50 patients had received passive immunisation together with either the first or the first and second vaccination, antibody titres at the 14th and 18th week (i.e. more than 10 weeks after passive immunisation) were used to evaluate the vaccination results. An antibody titre of 10 mIU/ml was considered to be a positive response. All patients of group IV, but only 4 out of 14 in group III, 4 out of 15 in group II, and 0 out of 14 in group I produced antibody titres higher than 50 mIU/ml. In contrast to the full response in group IV, two-thirds of all other patients had no immune response (<10 mIU/ml). Based on our experience we recommend vaccinating patients suffering from solid tumours and receiving no intensive therapy (group IV) against hepatitis B and protecting all the other children with malignant diseases by passive immunisation, if necessary.Abbreviations ALL acute lymphatic leukaemia - AML acute myelocytic leukaemia - CML chronic myelocytic leukaemia - NHL non-Hodgkin's lymphoma - RMS rhabdomyosarcoma - HBV hepatitis B virus - HBsAg hepatitis B surface antigen - HBeAg hepatitis B e antigen - anti-HBs antibody against HBsAg - RIA radioimmunoassay - BFM 81 protocol ALL therapy protocol 1981 (Berlin-Frankfurt-Münster)  相似文献   

11.
BACKGROUND: Precursor B-cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non-Hodgkin lymphoma (NHL). The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL-type therapy for treatment of these patients. PROCEDURE: From October, 1986, to March, 1995, 1,075 patients up to 18 years of age suffering from all kinds of NHL were registered in the two consecutive multicenter studies NHL-BFM 86 and 90. Of these, 27 patients were diagnosed with PBLL. Twenty-one PBLL patients were treated according to a BFM-ALL-type protocol: an eight-drug induction over 9 weeks was followed by an 8-week consolidation including methotrexate 5 g/m(2) x4. Patients in stages I and II continued with maintenance up to a total therapy duration of 24 months, whereas patients in stages III and IV received an additional eight-drug intensification and cranial radiotherapy (12 Gy for prophylaxis) after consolidation. Six PBLL patients were treated according to the BFM-protocol for B-NHL, stratified according to stage and tumor load and consisiting of two to six 5-day courses of chemotherapy. RESULTS: The median age of the 27 patients with PBLL (18 boys, 9 girls) was 6.2 (range 0.7-15) years. Stages (St. Jude) were: I (n = 3), II (n = 7), III (n = 9), and IV (n = 8). Twenty-one PBLL patients had nodal disease, 6 patients had subcutaneous manifestations, and 8 patients had bone marrow disease (<25% blasts). All patients achieved remission. With a median follow-up time of 4. 25 years, the estimated probability for event-free survival (pEFS) at 10 years for the total group was 0.73 (SE 0.10). Five patients (2, 1, 1, and 1 patients at stages I, II, III, and IV, respectively) relapsed: 2 of 21 patients who were treated according to the ALL strategy and 3 of 6 who were treated according to the B-NHL-protocol. CONCLUSIONS: PBLL accounts for 2.5% of childhood NHL. An ALL-type therapy strategy appears to be superior to a short-pulse B-NHL protocol.  相似文献   

12.
Extramedullary involvement (EMI) of various body sites was studied by doing CSF cytology anu FNAC of testes and lymph nodes in 82 children with acute lymphoblastic leukemia (ALL). Forty-five patients had received no therapy (group I) and 37 had been diagnosed and treated earlier and were now in remission (36) or relapse (group II). EMI was documented in 48.9% and 32.4% of patients of group I and II respectively. EMI was seen more frequently in patients with FAB-L 2 subtype (66.6%) as compared to FAB-L 1 subtype (34.6%) in group I. Lymph nodes and testes were commonly involved in group I and II patients respectively. Successful remission after complete chemotherapy was achieved in 82.5% of group I patients. However, successful remission rates were 95.2% in patients without any evidence of EMI versus 68.4% in those with EMI. These differences were also observed when response to therapy was analysed separately for patients with FAB-L1 and FAB-L2 subtypes.  相似文献   

13.
Palliative radiotherapy in children with neuroblastoma   总被引:1,自引:0,他引:1  
Limited information is available regarding the efficacy of external beam radiation therapy in the palliation of metastatic disease from neuroblastoma. From 1960 to 2000, 29 children with 53 metastatic sites received palliative radiotherapy. There were 26 soft tissue (group I), 19 bone (group II), 5 brain (group III), and 3 hepatic (group IV) treated sites. Median radiotherapy doses for groups I, II, III, and IV sites were 2000, 2000, 2400, and 450 cGy, respectively. For group I sites, complete response was complete disappearance of mass, partial response was > or =50% resolution of mass, no response was <50% resolution or < or =25% progression of mass, and progressive disease was >25% progression of mass. For group II sites, complete response was complete pain relief without medication, partial response was > or =50% pain relief with or without medication, no response was <50% change in pain with medication, and progressive disease was increase in pain and/or medication. Median survival was 2.5 months after palliative radiotherapy. For group I sites, complete response was seen in 1 (4%) while partial response was documented in 19 (73%). Duration of response was until death in 18 responders (90%); 2 patients relapsed with an increasing soft tissue mass at 5 months and 1 year after palliative radiotherapy. For group II sites, complete response was seen in 8 (42%) while partial response was documented in 7 (37%). Duration of response was until death in 14 responders (93%); 1 patient had relapse of pain 1 year after palliative radiotherapy. For the 5 group III children, the median survival was 2.5 months with a range of 2 days to 13 months. Four children had neurological improvement after cranial radiotherapy; one patient progressed and died secondary to neurological compromise 2 days after radiotherapy. For the 3 group IV sites, 2 had improvement in respiratory status after radiotherapy, whereas 1 progressed despite doxorubicin, cyclophosphamide, and radiation. The only patient who survived had a stage IV-S neuroblastoma with liver metastases and is alive 13 years after hepatic irradiation. Radiotherapy is an effective treatment for palliation of symptomatic metastatic disease in children with neuroblastoma.  相似文献   

14.
OBJECTIVE: To verify the prevalence of asthma in schoolchildren from Curitiba.METHOD: A written questionnaire of asthma symptoms (ISAAC-International Study of Asthma and Allergies in Childhood) was applied to children ages 6-7 years and adolescents 13-14 years registered in 32 public schools.RESULTS: 3,200 questionnaires were applied to 13-14 year-old adolescents, and 2,863 questionnaires to 6-7 year-old children. The questionnaire should be answered by the parents of 6-7 year-old children (group I) and by the adolescents themselves (13-14 year-old, group II). The rate of questionnaires returned was 58% for group I and 92% for group II. A presumptive diagnosis of asthma was based on having had 4 or more attacks of wheezing in the last 12 months, or 1-3 wheezing episodes in addition to night awakening for wheezing, nocturnal cough and wheezing after exercise. Under these criteria there were 15.7% asthmatics in group I and 11.6% in group II. Symptoms of rhinoconjunctivitis were found in 38% and 36%, and eczema in 11% and 10% for groups I and II, respectively. Among those with presumed asthma, over 70% denied having had asthma. The question "have you ever had asthma" although specific is not sensitive enough.CONCLUSION: The prevalence of asthma in Curitiba was 15.7% for children 6-7 years old and 11.6% for adolescents 13-14 years. The question "have you ever had asthma" did not contribute to defining cases with asthma.  相似文献   

15.
Among 68 children with severe dilated cardiomyopathy, 43 (aged 10 months to 15 years) presented with active myocarditis, diagnosed by endomyocardial biopsy. They were divided into four treatment groups: I, controls: 9 patients submitted to conventional treatment (digitalis, diuretics, and vasodilators) for 8.1±0.7 (SD) months; II, prednisone: 12 patients received conventional therapy plus prednisone; III, azathioprine: 16 patients submitted to conventional therapy plus prednisone and azathioprine; IV, cyclosporine: 13 patients treated with conventional therapy plus prednisone and cyclosporine. Immunosuppressive therapy was maintained for a mean of 8.4±1.2 months. They were submitted to noninvasive (electrocardiogram, chest radiograph, Doppler echocardiogram, and radioisotopic scintigraphy) and invasive (hemodynamic) studies. In the control group only 2 of 9 patients showed clinical and hemodynamic improvement and 1 of 4, histologic regression of the myocarditis. Among patients submitted to conventional therapy plus prednisone, 3 of 12 presented clinical and hemodynamic improvement; 2 of 5 also showed histologic regression of inflammatory process. By contrast, patients treated with azathioprine or cyclosporine associated with prednisone had significantly better results: 13 of 16 and 10 of 13 patients, respectively, had clinical and hemodynamic improvement; all 6 patients in the azathioprine group and all 4 patients in the cyclosporine group had histologic regression of the myocarditis. Two patients in the prednisone group, one in the azathioprine group, and one in the cyclosporine group died during treatment, in cardiogenic shock. In our experience immunosuppressive therapy with azathioprine or cyclosporine associated with prednisone improves the prognosis of children with active myocarditis and severe ventricular dysfunction.  相似文献   

16.
The results of 112 children with neuroblastoma treated at the Memorial Sloan-Kettering Cancer Center between 1949 and 1980 were analyzed. Of these children, 58 were 0-11 months old and 54 were 12-23 months old and there was a median follow-up of 111 months. All 10 patients with Stage I are alive, 21/27 with Stages II and III (78%) are alive, 5/67 patients (7%) with Stage IV are alive, and 7/8 patients with Stage IVS are alive. Age of the children is an independent prognostic factor. The survival of infants with Stage IV is significantly better than it is for older children of the same stage. Two of 15 infants in Stages II and III died, both of early complications, whereas 4/12 older children with the same stages died. Minimal individualized treatment is recommended for children 0-11 months old who have localized and Stage IVS neuroblastoma. Children less than 1 year old with localized and Stage IVS neuroblastoma had an extremely good prognosis (90% survival) and were usually cured without intensive chemotherapy. Surgical removal of the primary tumor was sufficient for Stage I, and partial tumor removal followed by conservative radiation or chemotherapy was sufficient in most Stage II and III patients. Gentle, individualized treatment was adequate for Stage IVS. Children less than 1 with Stage IV neuroblastoma had a significantly better prognosis than older children of the same stage, but their prognosis was still poor (18% survival).  相似文献   

17.
ABSTRACT. Growth and endocrine status of 38 Nordic children surviving from medulloblastoma were reevaluated 5–15 years after the diagnosis. Group I included children treated before the age of 10, and group II were the patients > 10 years at onset of tumour therapy. The median time interval from diagnosis to reexamination was 9 and 7 8/12 years in groups I and II ( p >0.1). The trunchal and standing height were highly affected at the follow-up. The median standing height standard deviation score (SDS) was -2.0 in group I and - 1.7 in group II. The corresponding median sitting height SDS was -2.5 in group I and +2.0 in group II. Growth hormone deficiency was found in 23 % of the patients, and only in group I. In both groups 69 96 had increased serum TSH concentrations (median 6.5 mU/I, range 1.3–30 mU/I) in spite of normal free T4 and total T3 levels. Hypogonadism and ACTH deficiency were rarely seen.
Conclusion: In patients treated for medulloblastoma, particularly when treated in early childhood, pronounced alterations appear in growth and endocrine systems. In the general clinical control of these patients anthropometric and endocrinological tests have to be included.  相似文献   

18.
Ceftriaxone is recommended in children with acute bacterial meningitis (ABM) for 10 days. However, the drug is expensive, and shorter duration of therapy, if equally effective, would cut costs of therapy and hospitalization. The aim of this study was to compare the outcome of 7 days vs. 10 days' ceftriaxone therapy in children with ABM. Seventy-three children aged 3 months to 12 years with ABM, consecutively admitted to hospital were enrolled. Ceftriaxone was given for 7 days to all. Randomization to group I (7 days) and group II (10 days) therapy was done on the seventh day. At the end of 7 days' therapy in group I and 10 days in group II, children were evaluated using a clinical scoring system. Children with a score of more than 10 were labelled as 'treatment failures' and were continued on ceftriaxone. If a score was less than 10, the antibiotic was stopped. Complications were appropriately evaluated and managed. All children were followed-up 1 month after discharge: neurodevelopmental assessment, Denver Development Screening Tests, IQ and hearing assessment were done. After excluding four patients, there were 35 children in group I and 34 in group II. The two groups were comparable with respect to age, sex, nutritional status, presenting clinical features, and CSF parameters. Organism identification was possible in 38 per cent of children: (Streptococcus pneumoniae, 21 per cent; Haemophilus influenzae, 13 per cent; meningococcus, 4 per cent). Treatment failure rate was comparable in both groups (9 in group I and 8 in group II) as was the sequelae at discharge and at 1 month (9 in group I, 15 in group II,p > 0.1). Status epilepticus and focal deficits at presentation were significantly associated with treatment failures and sequelae in both the groups (p < 0.05). Length of hospital stay was shorter in group I (10.8 +/- 6.0 days) as compared with group II (14.4 +/- 7.2 days,p < 0.05) and frequency of nosocomial infection was significantly more in group II (p < 0.05). It was concluded that clinical outcome of patients treated with 7 days' ceftriaxone therapy is similar to that of 10 days' therapy, and is associated with lesser nosocomial infection and earlier hospital discharge. Seven days ceftriaxone therapy may be recommended for uncomplicated ABM in children in developing countries.  相似文献   

19.
This study was designed to evaluate the indications for ventriculoperitoneal shunting in cases of children with tubercular meningitis, presenting with hydrocephalus. Thirty seven children (less than 18 years of age) of tubercular meningitis with hydrocephalus (TBMH) who underwent ventriculoperitoneal shunting over a three year period (1999 to 2001) were included in the study. Sixteen (43%) children were Palur stage II, 15 (40%) stage III, and 6 (16%) stage IV. Fifteen (40%) children had received antitubercular therapy for less than 4 months and 17 (46%) received therapy for more than 4 months prior to presentation. Five (14%) children had not previously received antitubercular therapy. Shunt related complications occurred in 11 (30%) children and 3 children had undergone revision of the shunt multiple times. Good outcome was seen in 16 (43%) children. Thirteen (35%) had moderate disability and 6 (16%) had severe disability at 3 months of follow up. 62% (n =10) children in grade II had a good outcome compared to 40% (n = 6) in grade III. All six children in grade IV had a poor outcome. 2 children, both having multiple infarcts, died and the remaining 4 were left with severe disability. We recommend shunt placement in all children of grade II and III TBMH as this policy has yielded the best results. For grade IV children external ventricular drainage, followed by shunting if improvement occurs remains the most cost-effective procedure.  相似文献   

20.

Background

Over 50% of patients with rhabdomyosarcoma (RMS) have intermediate risk disease, with a 3‐year failure‐free survival (FFS) of 50%–70% depending on histology. Doxorubicin is active against RMS, but its role in improving outcome remains controversial. Ifosfamide is as active as cyclophosphamide in RMS, with the Fourth Intergroup RMS Study (IRS‐IV) showing equivalent outcomes for patients treated with ifosfamide for the first 28 weeks compared to cyclophosphamide. Treatment with alternating cycles of non‐cross‐resistant chemotherapy has been used in a number of diseases with good results.

Procedure

The results of a pilot study utilizing alternating courses of vincristine, doxorubicin, cyclophosphamide, and etoposide/ifosfamide (VDC/IE) were compared for outcome and patient characteristics to a group of similar matched patients treated on IRS‐IV.

Results

The 5‐year FFS for patients with parameningeal (PM) primaries on IRS‐IV and the VDC/IE study were 72% and 82%, respectively (P = 0.26); for patients with non‐PM primaries, the estimated risk of failure for VDC/IE study versus IRS‐IV was 0.54. Combining all disease sites and performing analysis for relative risk of failure for 46 VDC/IE patients and 342 IRS‐IV patients, the relative risk of failure for the VDC/IE study compared to the IRS‐IV study is 0.5 (P = 0.06).

Conclusions

VDC/IE is as effective therapy for intermediate risk RMS as IRS‐IV therapy. It is being explored along with irinotecan in relapsed patients and newly diagnosed high‐risk patients. Pediatr Blood Cancer 2008;50:33–36. © 2006 Wiley‐Liss, Inc.  相似文献   

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