AIM: To determine the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in chil- dren. METHODS: In a pilot study of 10 children wit...AIM: To determine the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in chil- dren. METHODS: In a pilot study of 10 children with chronic liver disease, who had HPS, 20 mg/kg/d PTX was ad- ministered for 3 mo. Clinical data and arterial blood gas parameters were evaluated at baseline, the end of the treatment period, and 3 mo after drug discontinuation. RESULTS: Six patients could tolerate PTX, while four patients experienced complications, Among patients who could tolerate PTX, there was a significant increase in arterial oxygen pressure (PaO2) (P = 0,02) and oxy- gen saturation (Sa02) (P = 0.04) and alveolar-arterial oxygen gradient (P = 0.02) after 3 mo of treatment. Significant decreases in Pa02 (P = 0.02) and alveolararterial oxygen gradient (P = 0.02) were also seen after drug discontinuation. CONCLUSION: PTX may improve PaO2, Sa02 and alve- olar-arterial oxygen gradient in the early stage of HPS.展开更多
Objective:The aim of this study was to assess the differences in clinical appearance and prognosis of osteosarcoma between preadolescent and adolescent in Chinese patients and investigate whether age at diagnosis is a...Objective:The aim of this study was to assess the differences in clinical appearance and prognosis of osteosarcoma between preadolescent and adolescent in Chinese patients and investigate whether age at diagnosis is a prognostic indicator.Methods:Between May 2002 and May 2009,ninety-six children with high-grade osteosarcoma treated at our institute were stratified according to the age of 10.There were 19 preadolescents(≤ 10 years) and 77 adolescents(10 years < age ≤ 19 years),and their medical records were reviewed and compared using Fisher exact tests.Overall survival and disease-free survival was estimated by Kaplan-Meier methods and compared using log-rank tests.The prognostic significance of the various factors on survival was examined by Cox regression analysis.Results:There were no significant differences in terms of Karnofsky Performance Status(KPS) score,Enneking stage,tumor location,histologic type,pathologic fracture,tumor necrosis rate,tumor size,relapse and frequencies of adjuvant chemotherapy between the two groups.A high proportion of preadolescent patients was treated with amputation(78.9% vs.44.2%;P = 0.01).The 5-year survival of the preadolescent and adolescent groups was(38 ± 14)% and(33 ± 10)%,and the 2-year disease free survival for each group was(45 ± 12)% and(44.4 ± 6.3)%,respectively.Our study showed that age did not have any statistical significance for survival(P = 0.803).Univariate analysis indicated that KPS score;frequencies of adjuvant chemotherapy,tumor size and relapse were significantly related to overall survival.Multivariate Cox regression analysis revealed that both frequencies of adjuvant chemotherapy and relapse were independently prognostic factors for survival.Conclusion:Clinical characteristics and survival between the preadolescents and adolescents with osteosarcoma in China were compatible.So we suggested that there did not need to treat preadolescents patients by alternative and/or aggressive therapies compared with adolescent patients.展开更多
Tuberous sclerosis complex(TSC) is a neurocutaneous syndrome with serious clinical presentations, an autosomal dominant genetic disorder involving multiple organs and systems. We retrospectively investigated the clini...Tuberous sclerosis complex(TSC) is a neurocutaneous syndrome with serious clinical presentations, an autosomal dominant genetic disorder involving multiple organs and systems. We retrospectively investigated the clinical manifestations and genotypes of 20 Chinese children with TSC to enable informed diagnostic and surveillance recommendations in China. A retrospective analysis of clinical manifestations in 20 children(7.00±5.30 years old) with TSC was conducted. A genetic testing of the genes TSC1 and TSC2 was performed in 14 children.The earliest manifestations of TSC were skin lesions(80% of patients) and seizures(75%). Fourteen of the children presented with retinal hamartomas, and 2 of these underwent eye enucleation at other hospitals through misdiagnosis. On magnetic resonance imaging, 18 children exhibited subependymal nodules, and 16 ones showed cortical nodules. 5 cases of non-renal hamartomas, 5 cases of multiple renal cysts, and 5 cases of cardiac rhabdomyomas were observed.The genotyping of TSC1 and TSC2 in 14 children revealed 11 with mutations in TSC2, 2 with mutations in TSC1, and no mutations of either gene in one patient. Eight of these observed mutations are reported here in for the first time. The illness presentations of the TSC2-mutated patients were more severe than that of patients carrying TSC1 mutations.There were differences in the mutations of TSC genes in Chinese children from those reported in other countries. The described clinical characteristics and genotyping will help pediatric neurologists to understand, diagnosis, and treat TSC.展开更多
OBJECTIVE:To evaluate the common Traditional Chinese Medicine(TCM) syndromes and analyze their relationship to clinical and pathological manifestations in children with IgAnephropathy.METHODS:Forty five children diagn...OBJECTIVE:To evaluate the common Traditional Chinese Medicine(TCM) syndromes and analyze their relationship to clinical and pathological manifestations in children with IgAnephropathy.METHODS:Forty five children diagnosed as having primary IgA nephropathy by renal biopsy for the first time were enrolled in this trial,and their TCM syndromes were evaluated and the distribution of TCM syndromes was observed.All the sick children were growed? according to clinical manifestations and pathological damages,and the differences in TCM syndromes were compared between the groups.RESULTS:The first 5 TCM symptoms were common cold,hyperhidrosis,red dry throat,dark yellow urine and lassitude.In the acute nephritis group,edema and aching pain in loin and knees were significant(P=0.021 and P=0.000).In the severe pathological damage group,edema was obvious(P= 0.004),and 24 h urinary protein was positively correlated with edema(P=0.015) while negatively with common cold(P=0,007).The score of mesangial cell proliferation was correlated with edema,red dry throat and common cold(P=0.006,0.013 and 0.029 respectively).The score of segmental pathological change was positively correlated with edema(P=0.039).CONCLUSION:Common cold,hyperhidrosis,red dry throat,dark yellow urine,lassitude and other symptoms of qi deficiency of the spleen and lung mainly seen in children with IgA nephropathy may bear a close relationship to clinical manifestations and pathological damages.展开更多
基金Supported by Research Council of Mashhad University of Medical Sciences
文摘AIM: To determine the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in chil- dren. METHODS: In a pilot study of 10 children with chronic liver disease, who had HPS, 20 mg/kg/d PTX was ad- ministered for 3 mo. Clinical data and arterial blood gas parameters were evaluated at baseline, the end of the treatment period, and 3 mo after drug discontinuation. RESULTS: Six patients could tolerate PTX, while four patients experienced complications, Among patients who could tolerate PTX, there was a significant increase in arterial oxygen pressure (PaO2) (P = 0,02) and oxy- gen saturation (Sa02) (P = 0.04) and alveolar-arterial oxygen gradient (P = 0.02) after 3 mo of treatment. Significant decreases in Pa02 (P = 0.02) and alveolararterial oxygen gradient (P = 0.02) were also seen after drug discontinuation. CONCLUSION: PTX may improve PaO2, Sa02 and alve- olar-arterial oxygen gradient in the early stage of HPS.
基金Supported by grants from the National Natural Science Foundation of China (No. 81001191)Science and Technology Commission of Shanghai (No. 10PJ1408300)
文摘Objective:The aim of this study was to assess the differences in clinical appearance and prognosis of osteosarcoma between preadolescent and adolescent in Chinese patients and investigate whether age at diagnosis is a prognostic indicator.Methods:Between May 2002 and May 2009,ninety-six children with high-grade osteosarcoma treated at our institute were stratified according to the age of 10.There were 19 preadolescents(≤ 10 years) and 77 adolescents(10 years < age ≤ 19 years),and their medical records were reviewed and compared using Fisher exact tests.Overall survival and disease-free survival was estimated by Kaplan-Meier methods and compared using log-rank tests.The prognostic significance of the various factors on survival was examined by Cox regression analysis.Results:There were no significant differences in terms of Karnofsky Performance Status(KPS) score,Enneking stage,tumor location,histologic type,pathologic fracture,tumor necrosis rate,tumor size,relapse and frequencies of adjuvant chemotherapy between the two groups.A high proportion of preadolescent patients was treated with amputation(78.9% vs.44.2%;P = 0.01).The 5-year survival of the preadolescent and adolescent groups was(38 ± 14)% and(33 ± 10)%,and the 2-year disease free survival for each group was(45 ± 12)% and(44.4 ± 6.3)%,respectively.Our study showed that age did not have any statistical significance for survival(P = 0.803).Univariate analysis indicated that KPS score;frequencies of adjuvant chemotherapy,tumor size and relapse were significantly related to overall survival.Multivariate Cox regression analysis revealed that both frequencies of adjuvant chemotherapy and relapse were independently prognostic factors for survival.Conclusion:Clinical characteristics and survival between the preadolescents and adolescents with osteosarcoma in China were compatible.So we suggested that there did not need to treat preadolescents patients by alternative and/or aggressive therapies compared with adolescent patients.
基金supported by the Capital Health Research and Development of Special (2014-1-4091)
文摘Tuberous sclerosis complex(TSC) is a neurocutaneous syndrome with serious clinical presentations, an autosomal dominant genetic disorder involving multiple organs and systems. We retrospectively investigated the clinical manifestations and genotypes of 20 Chinese children with TSC to enable informed diagnostic and surveillance recommendations in China. A retrospective analysis of clinical manifestations in 20 children(7.00±5.30 years old) with TSC was conducted. A genetic testing of the genes TSC1 and TSC2 was performed in 14 children.The earliest manifestations of TSC were skin lesions(80% of patients) and seizures(75%). Fourteen of the children presented with retinal hamartomas, and 2 of these underwent eye enucleation at other hospitals through misdiagnosis. On magnetic resonance imaging, 18 children exhibited subependymal nodules, and 16 ones showed cortical nodules. 5 cases of non-renal hamartomas, 5 cases of multiple renal cysts, and 5 cases of cardiac rhabdomyomas were observed.The genotyping of TSC1 and TSC2 in 14 children revealed 11 with mutations in TSC2, 2 with mutations in TSC1, and no mutations of either gene in one patient. Eight of these observed mutations are reported here in for the first time. The illness presentations of the TSC2-mutated patients were more severe than that of patients carrying TSC1 mutations.There were differences in the mutations of TSC genes in Chinese children from those reported in other countries. The described clinical characteristics and genotyping will help pediatric neurologists to understand, diagnosis, and treat TSC.
基金Supported by the Project of Capital Medical Development Fund(TCMType,SF-2007Ⅱ -07)
文摘OBJECTIVE:To evaluate the common Traditional Chinese Medicine(TCM) syndromes and analyze their relationship to clinical and pathological manifestations in children with IgAnephropathy.METHODS:Forty five children diagnosed as having primary IgA nephropathy by renal biopsy for the first time were enrolled in this trial,and their TCM syndromes were evaluated and the distribution of TCM syndromes was observed.All the sick children were growed? according to clinical manifestations and pathological damages,and the differences in TCM syndromes were compared between the groups.RESULTS:The first 5 TCM symptoms were common cold,hyperhidrosis,red dry throat,dark yellow urine and lassitude.In the acute nephritis group,edema and aching pain in loin and knees were significant(P=0.021 and P=0.000).In the severe pathological damage group,edema was obvious(P= 0.004),and 24 h urinary protein was positively correlated with edema(P=0.015) while negatively with common cold(P=0,007).The score of mesangial cell proliferation was correlated with edema,red dry throat and common cold(P=0.006,0.013 and 0.029 respectively).The score of segmental pathological change was positively correlated with edema(P=0.039).CONCLUSION:Common cold,hyperhidrosis,red dry throat,dark yellow urine,lassitude and other symptoms of qi deficiency of the spleen and lung mainly seen in children with IgA nephropathy may bear a close relationship to clinical manifestations and pathological damages.