Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conduct...Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.展开更多
Idiopathic pneumomediastinum is rare in children. Few cases of patients with pneumomediastinum show negative findings on X-ray examination. Chest computed tomography (CT) was very useful for the diagnosis and evaluati...Idiopathic pneumomediastinum is rare in children. Few cases of patients with pneumomediastinum show negative findings on X-ray examination. Chest computed tomography (CT) was very useful for the diagnosis and evaluation of the extent of pneumomediastinum. We report here a case of idiopathic pneumomediastinum in a 15-year-old boy who exhibited no significant chest X-ray finding. The patient was referred to our institute for the further evaluation of pre-cordial pain and breathing difficulty. Precordial pain suddenly developed, when he was carrying a portable shrine on his shoulder (day of onset). He was admitted to another institute 3 days after onset because of worsening precordial pain. On admission, he presented with 98% saturation of hemoglobin in the peripheral blood under room air. Plain chest X-ray also revealed no abnormal findings. A half-dissolved gastrographin swallow showed no leakage of gastrographin from the pharynx and esophagus to the mediastinum, and no diverticulum within the esophagus. Plain chest CT revealed extensive emphysema around the trachea from the neck to the portion inferior to the carina of trachea. The patient was diagnosed with idiopathic pneumomediastinum because the cause was unclear. We decided to admit him to our institute under fasting conditions and rest. His symptoms improved 3 days after onset. The lesion had disap-peared 8 days after onset on chest CT. When young people experience precordial pain which increases on inspiration, we must consider pneumomediastinum in a differential diagnosis, and it is important to perform chest CT.展开更多
目的分析T细胞活化免疫球蛋白抑制V型结构域(V-domain Ig suppressor of T cell activation,VISTA)在幼年特发性关节炎(juvenile idiopathic arthritis,JIA)患儿外周血的表达情况,探讨其在发病中的作用。方法前瞻性收集不同亚型JIA患儿...目的分析T细胞活化免疫球蛋白抑制V型结构域(V-domain Ig suppressor of T cell activation,VISTA)在幼年特发性关节炎(juvenile idiopathic arthritis,JIA)患儿外周血的表达情况,探讨其在发病中的作用。方法前瞻性收集不同亚型JIA患儿(47例)及健康儿童(10例)的外周血,利用流式细胞术检测CD14+单核细胞、CD4+T淋巴细胞、CD8+T淋巴细胞上VISTA、干扰素-γ(interfern-γ,IFN-γ)、肿瘤坏死因子-α(tumor necrosis factor-α,TNF-α)的表达情况。结果VISTA在JIA患儿中的表达水平比健康儿童低(P<0.05);不同亚型JIA患儿VISTA表达差异有统计学意义,以全身型表达水平最低(P<0.05);不同免疫细胞表达VISTA差异有统计学意义,单核细胞表面VISTA表达水平更高(P<0.05)。相关性分析发现CD4+T细胞上VISTA与IFN-γ(r=-0.436,P<0.05)、TNF-α(r=-0.382,P<0.05)表达呈负相关,CD8+T细胞上VISTA与IFN-γ(r=-0.348,P<0.05)、TNF-α(r=-0.487,P<0.05)表达呈负相关;CD14+单核细胞上VISTA与IFN-γ(r=-0.582,P<0.05)、TNF-α(r=-0.603,P<0.05)表达呈负相关。结论VISTA表达不足可能与JIA发病相关,增强VISTA的免疫调节作用可能是未来治疗JIA的途径之一。展开更多
目的比较重组人生长激素(recombinant human growth hormone,rhGH)治疗前后特发性矮小症(idiopathic short stature,ISS)患儿血清Klotho、成纤维细胞生长因子23(fibroblast growth factor,FGF23)和胰岛素样生长因子(insulin-like growth...目的比较重组人生长激素(recombinant human growth hormone,rhGH)治疗前后特发性矮小症(idiopathic short stature,ISS)患儿血清Klotho、成纤维细胞生长因子23(fibroblast growth factor,FGF23)和胰岛素样生长因子(insulin-like growth factor,IGF)-1水平变化,探讨Klotho和FGF23与ISS患儿生长激素(growth hormone,GH)/IGF-1生长轴的关系。方法前瞻性选择2021年3月10日—2022年12月1日在河北省人民医院儿科确诊为ISS的33例儿童为ISS组,选择同期于儿童保健科就诊,年龄、性别与ISS组匹配的29例健康儿童为健康对照组。ISS组给予rhGH治疗,比较治疗前及治疗3、6、9个月血清Klotho、FGF23、IGF-1水平,并进行相关性分析。结果ISS组与健康对照组的血清IGF-1、Klotho、FGF23水平比较差异无统计学意义(P>0.05)。rhGH治疗3、6、9个月的ISS组血清Klotho、FGF23、IGF-1水平均较治疗前显著升高(均P<0.05)。ISS组治疗前Klotho、FGF23与磷酸盐水平均呈正相关(P<0.05);治疗前及治疗3、6、9个月的Klotho与IGF-1水平均呈正相关(P<0.05),FGF23与IGF-1水平均呈正相关(P<0.05),Klotho与FGF23水平均呈正相关(P<0.05),Klotho、FGF23水平与身高标准差积分无相关性(P>0.05)。结论rhGH治疗可上调Klotho、FGF23及IGF-1水平,实现ISS患儿的追赶生长。Klotho、FGF23可能并非直接促进ISS患儿线性生长,而是可能通过IGF-1及磷酸盐代谢等途径产生间接影响。Klotho、FGF23与IGF-1的一致变化表明三者在调节ISS线性生长中存在协同关系。展开更多
文摘Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.
文摘Idiopathic pneumomediastinum is rare in children. Few cases of patients with pneumomediastinum show negative findings on X-ray examination. Chest computed tomography (CT) was very useful for the diagnosis and evaluation of the extent of pneumomediastinum. We report here a case of idiopathic pneumomediastinum in a 15-year-old boy who exhibited no significant chest X-ray finding. The patient was referred to our institute for the further evaluation of pre-cordial pain and breathing difficulty. Precordial pain suddenly developed, when he was carrying a portable shrine on his shoulder (day of onset). He was admitted to another institute 3 days after onset because of worsening precordial pain. On admission, he presented with 98% saturation of hemoglobin in the peripheral blood under room air. Plain chest X-ray also revealed no abnormal findings. A half-dissolved gastrographin swallow showed no leakage of gastrographin from the pharynx and esophagus to the mediastinum, and no diverticulum within the esophagus. Plain chest CT revealed extensive emphysema around the trachea from the neck to the portion inferior to the carina of trachea. The patient was diagnosed with idiopathic pneumomediastinum because the cause was unclear. We decided to admit him to our institute under fasting conditions and rest. His symptoms improved 3 days after onset. The lesion had disap-peared 8 days after onset on chest CT. When young people experience precordial pain which increases on inspiration, we must consider pneumomediastinum in a differential diagnosis, and it is important to perform chest CT.
