姿势性蛋白尿预后的远期随访

姿势性蛋白尿，主要表现在直立位或脊柱前突时出现蛋白尿，卧位后消失。本病大多预后良好，尚有少数患者发展成慢性肾功能不全及肾脏疾病。为探讨本病预后和转归．我们自1978年以来对已确诊本病的116例患者分别随访5～18a，现将结果报告如下。临床资料一、一般资料确诊为单纯性     

热性惊厥小儿远期预后的随访调查

目的 探讨热性惊厥小儿的复发情况与远期预后。方法 对２８１例首次恨作的热怀惊厥患儿采用问卷调查、清醒脑电图描记及智力测定进行３￣１０ａ的无期随访。结果 ３９．５％复发,４５．０５％有１次以上多次复发。６２．１６％再次发作发生在首发后１ａ内,９２．７９％在２ａ内。４．２７％转为癫痫,２．１４％后遗智力低下,无１例死亡。结论 热性惊厥预后良好,其复发与首发年龄、首次复发距道发的时间、阳性家族史有关,而     

体位性蛋白尿的早期诊断及远期随访观察

体位性蛋白尿的早期诊断及远期随访观察董淑兰李堂卢愿姬长鸣季作全我们对１９７８年以来诊断为体位性蛋白尿的８３例患儿分别随访５～１５年，现将诊断标准和随访结果分析如下。临床资料：选自本院内儿科门诊、病房及青少年健康查体中确诊为单纯性蛋白尿的患儿，共８１５...     

儿童紫癜性肾炎远期随访

我院自1970年1月～1035年1月收治过敏性紫癜210例，其中42例确诊为紫癫性肾炎（SHPN）。现将随访结果及预后分析如下。临床资料一、一般资料共42例，男29例，女13例。二、临床表现全部病例均有典型的皮肤紫癜及尿改变。三、实验室检查42例均有不同程度的镜下血尿，红细胞均＞5～10个／HPg原蛋白＋～25例。同时24小时尿蛋白定量均＞（45～100）mg/kg；血清胆固醇增高9例，血清白蛋白低于正常值9例；血肌酐及BUN增高2例；血内生肌肝病除率降低1例。四、临床分型与分组根据病初时临床特点及病情轻重分为急性肾炎综合征（肾炎型）及肾病综合…     

紫癜性肾炎的远期随访

过敏性紫癜（ＡＰ）的基本病理变化是全身小血管炎,具有丰富毛细血管的肾脏是其主要受累器官。总结我科近十余年收治的紫癜性肾炎９７例,并对其中５９例进行随访,结果如下。临床资料一、一般资料　本组９７例,男５２例,女４５例,年龄５～１２ａ,平均８ａ６ｍｏ,均符合上海市儿科肾病协作组的诊断标准（草案）：１．两下肢或四肢对称性紫癜,同时伴有消化道及关节症状,血小板计数正常;２．病程中或紫癜消失后出现血尿（肉眼或镜下）,蛋白尿及管型;３．尿蛋白＞０．１ｇ／（ｋｇ·ｄ）,可能为肾病综合征;４．经治疗后临床症状消…     

18例单纯性蛋白尿患儿临床和病理特点及随访结果分析

目的观察单纯性蛋白尿(IP)患儿临床与肾脏病理变化及预后。方法对18例IP患儿进行肾脏活组织检查及检测常用评价肾功能的指标。并随访1～6年。结果18例IP患儿肾脏病理类型分别为系膜增生性肾炎8例,微小病变型2例,IgA肾病3例,局灶性硬化性肾小球肾炎3例,IgM肾病1例,肾小球无明显病变1例。与入院时相比,随访1年后各项指标差异无显著性(P>0.05);随访3年后24h尿蛋白定量增加,差异无显著性(P>0.05),但血肌酐上升,内生肌酐清除率下降,差异有显著性(P<0.05);随访6年后,除尿β2-微球蛋白与入院时相比差异无显著性外,其余指标均有显著性差异(P<0.05)。结论绝大多数IP患儿有肾脏病理改变,随病程延长,肾小球及肾小管病变呈进展趋势,应及时行肾活检,以期早期诊断。     

儿童孤立性冠状动脉瘘的治疗及中期随访分析

目的对儿童孤立性冠状动脉瘘的治疗过程及中期随访结果进行总结分析。方法回顾性分析广州市妇女儿童医疗中心2009年7月至2017年7月诊断为孤立性冠状动脉瘘的17例患儿临床资料,其中男11例,女6例,年龄40日龄至12岁(中位值23个月),体重3.8~29 kg(中位体重11.3 kg)。2例存在气促表现,2例存在生长发育落后表现,2例存在反复呼吸道感染表现,1例主诉心悸,余10例无明显临床症状。其中3例心脏检查未发现明显杂音,14例发现心脏杂音。瘘口发生于右冠状动脉8例,左冠状动脉9例。瘘入右心房5例,右心室10例,左心室2例;17例中12例合并巨大冠状动脉瘤。结果1例行经皮冠状动脉瘘封堵术,1例在非体外循环下行瘘管结扎术,其余15例在体外循环下行冠状动脉瘘矫治术;平均住院时间(11.1±4.1)d。其中2例术后3 d内出现射血分数下降(最低者降至38%),术后1个月复查时射血分数均升至50%以上。7例术后出现一过性T波改变,出院时均已恢复正常;2例术后存在1~2 mm残余瘘的患儿在近期复查中残余瘘消失。围术期无一例死亡。随访10个月至9年,所有患儿无自觉症状和阳性体征,心电图均无心肌缺血表现,复查超声心动图均提示心脏收缩功能正常,冠状动脉直径均较术前缩小。结论大多数儿童孤立性冠状动脉瘘无明显症状,但可合并巨大冠状动脉瘤,应尽早手术,中远期预后良好;但因存在冠状动脉扩张,仍需长期随访。     

肾小管性酸中毒的治疗和远期预后

分析肾小管性酸中毒患儿的治疗及远期预后。方法对17例患儿进行3～14a随访。结果死亡3例;自然痊愈1例;2例间断治疗者,现有轻微肾钙化、体格矮小、步履困难;11例仍在接受治疗,5例随访4～6a身高达正常水平,除轻度骨骼畸形外无其他发现,6例随访5～8a身高仍未正常,见不同程度骨骼畸形。结论本病早期诊断、正规治疗、终生服药可取得较好疗效。     

儿童脑脓肿37例临床特征和治疗随访

目的 阐明儿童脑脓肿临床特征,探讨脑脓肿的治疗方法。方法 对３７例经ＣＴ和ＭＲＩ确诊为脑逐月中的患儿临床特征进行分析,对疗效进行观察,对其中２３例进行病后３个月～５年的随访。结果 ３７例患儿中未查到原发感染灶的２９例（７８％）,以癫痫为首发症状者２０例（５４％）,头痛者１８例（４９％）,发热者１３例（３５％）,３０例经保守治疗１１例治愈,１２例好转,总有效率７７％;７例采取外科治疗,１例治愈,３例     

儿童肝移植术后的随访与治疗

目的评估复旦大学附属儿科医院肝移植患儿术后疗效。方法回顾性分析2002年10月至2005年5月期间8例小儿肝移植术后的临床效果和生活质量。本组男4例,女4例,肝移植年龄4—67个月,体重6—19kg。6例亲体活体肝移植,1例劈裂式肝移植,1例减体积肝移植。结果围手术期死亡1例,手术存活率87、5％;手术成功患儿中1例于术后8个月死于消化道出血,1年生存率为75．O％,其他6例存活至今,最长生存期43个月。术后并发症：急性排斥反应2例;远期感染：巨细胞病毒感染2例,Epstein—Barr病毒感染1例,乙型肝炎病毒感染1例;低蛋白血症3例。远期外科并发症：门静脉血栓1例,下腔静脉和肝静脉狭窄1例。他克莫司（FK506）的副作用：高血压3例,肾脏损害1例,肝脏损害1例,腹泻1例。所有存活患儿无心理异常;1例术前体格发育落后者,术后发生了生长追赶。结论肝移植改善了小儿终末期肝病的预后。儿童肝移植后并发症多见。周密的术后随访与管理有助于发现各种内外科并发症和保证术后生活质量。     

Quantitative determination of urinary protein components of children with postural proteinuria

Twenty-one protein components were measured in urine specimens from 12 subjects with postural proteinuria in the recumbent posture and after a period of upright lordotic posture. The lordotic posture produced an increased urinary excretion of total protein and 19 protein components, particularly of albumin, IgG globulin, and transferrin (128, 68, and 167-fold, respectively). However, 90 min after the lordotic posture, most of the protein components returned to initial values, whereas albumin, IgG globulin, and transferrin remained high. 2-Microglobulin, retinol binding protein, and lysozyme showed the smallest increases after the lordotic posture. These findings suggest that proteinuria occurring after the lordotic posture may be characterized by an increased glomerular permeability.     

Treatment and follow-up of children with cerebrotendinous xanthomatosis

The clinical spectrum and the effects of treatment over a period of 5 years in five children with cerebrotendinous xanthomatosis (CTX) are described. In all children biochemical, neuroradiological, and neurophysiological studies were done. CTX was diagnosed and effects of therapy were evaluated by determination of the serum cholestanol/cholesterol ratio (CCR) and the urinary excretion of bile alcohols. All children were treated with chenodeoxycholic acid (15 mg/kg/day) in three divided oral doses. Diarrhoea and juvenile cataract were the main clinical features. Psychomotor retardation, pyramidal and cerebellar signs were also found. After starting treatment, biochemical abnormalities normalized and diarrhoea disappeared. After 1 year of therapy there was no further delay in motor development, and in three children the intelligence quotient improved. EEG abnormalities disappeared. After 5 years of therapy the children are in a stable clinical condition. Conclusion The clinical, biochemical and neurophysiological abnormalities in five children with CTX showed a remarkable improvement after starting treatment with chenodeoxycholic acid. The early diagnosis of CTX and the start of treatment with chenodeoxycholic acid has prevented neurological deterioration for a period of 5 years. Received: 24 May 1997 / Accepted in revised form: 29 September 1997     

儿童感觉统合失调的随访研究

目的　通过 6年追踪调查 ,观察儿童感觉统合失调的动态变化 ,为早期干预提供科学依据。方法　用自评问卷儿童生活环境调查表、感觉统合诊断表、Achenbach儿童行为量表分别于 1993年和 1999年调查 310名儿童。结果　本组感觉统合失调累积发生率为 33 6 % ,其中男童为 39 1% ,女童为 2 6 7% ,性别差异有显著性 (χ2 =3 84 5 ,P <0 0 5 )。各因子失调累积发生率为 10 0 %～ 17 0 %。感觉统合失调 6年自愈率为 4 8 3% ,其中男童自愈率为 4 8 5 % ,女童为 4 8 2 % ,性别差异无显著性(χ2 =0 0 0 1,P >0 0 5 )。本体感失调自愈率为 6 9 7% ;触觉防御失调自愈率为 81 6 % ;前庭平衡失调自愈率为 72 0 %。 2 89名儿童各年龄组感觉统合失调累积发生率和自愈率差异无显著性 (P均 >0 0 5 )。 15 5名儿童 6年后行为问题累积发生率为 9 4 % ,其中男童为 12 5 % ,女童为 6 1% ,性别差异无显著性 (χ2 =1 6 73,P >0 0 5 )。学龄前有行为问题儿童的自愈率为 70 6 %。结论　男童的感觉统合失调较女童严重 ;感觉统合功能失调有自愈趋势 ;提示幼儿期可能是感觉统合失调预防的关键时期     

小儿肾病综合征的中长期随访研究

目的探讨小儿肾病综合征（ＮＳ）的预后及有关因素。方法对７２例小儿ＮＳ患儿作中、长期随访,其中１６例随访１０年,４９例随访５年,７２例随访３年。结果３组的完全缓解率分别为７５％、８４％及７９％。基本痊愈率在５年组为４９％,１０年组为６９％。死亡３例。结论肾小球的组织学改变与预后有关。小儿ＮＳ总的预后较好;微小病变、膜性肾病的预后较局灶硬化、弥漫性硬化及膜增殖型要好;轻、中度系膜增殖型预后好,严重增殖者预后较差。延长激素疗程,缓慢停药可增加缓解率,减少反复     

新生儿红斑狼疮12例临床特征和远期随访

目的 探讨新生儿红斑狼疮(Neonatal lupus erythematosus NLE)的发病机制、血清学改变、临床特征及远期预后。方法 确定NLE的条件：① 新生儿先天性心脏传导阻滞和新生儿或母亲的抗SSA/Ro、抗SSB/La抗体阳性;② 确定的和NLE相关的皮肤损害和新生儿或母亲的抗-Ro/SSA, 抗-La/SSB,或 抗-U1RNP抗体阳性。总结分析12例NLE患儿和母亲的临床表现,检测母亲和患儿的自身抗体,进行长期随访。并进行文献复习。结果 12例NLE , 男7例, 女5例, 就诊年龄1个月～5个月,起病年龄生后1d ～2个月,皮肤损害12例,血液学改变11例,肝脏受损6例,心脏受损4例,抗核抗体阳性12例,抗SSA/Ro抗 体阳性12例,抗SSB/La抗体阳性5例,抗ds-DNA抗体阳性4例,抗ENA抗体阳性4例,血沉增快6 例,血清丙氨酸转氨酶增高6例。12例母亲中孕前11例无症状,1例日光性皮炎;孕3～5个月时7例母亲出现日光性皮炎;2例发热、脱发、全血细胞减少;产后1个月 2例母亲出现皮疹、脱发;抗核抗体阳性12例,抗SSA/Ro抗体阳性12例,抗 SSB/La抗体阳性 5例,抗ds-DNA抗体阳性4例,抗ENA抗体阳性4例;血沉增快5例。12例NLE中11例随访18个月～12年,心电图完全右束支传导阻滞1例死亡。11例皮疹于3~11个月龄消退,自身抗体于6~12个月龄转阴,血液学改变、肝脏损害恢复正常,智力发育同正常同龄儿。1例3岁确诊为幼年特发性关节炎少关节炎型,1例12岁时出现反复颜面红斑皮疹,ANA阳性1：320,抗ds-DNA抗体阴性。12 例母亲中10例确诊为SLE,2例确诊为SS。结论 NLE是获得性的自身免疫疾病,临床表现主要有短暂性皮肤狼疮损害、先天性心脏传导阻滞、血细胞减少、黄疸、肝功能损伤等,肾脏受损少见。抗Ro (特别是抗-52KD SSA/Ro) 或抗-La抗体阳性的母亲是NLE的危险因素。NLE患儿需要长期随访,这些患儿存在到儿童期发展为其他自身免疫病的危险。     

Brain abscess in childhood-long-term experiences

A retrospective evaluation was performed on 28 cases of paediatric brain abscess (malefemale ratio 2.51; mean age 9.4 years; range 2.8–16 years) diagnosed between 1967 and 1987. In 46%, congenital cyanotic heart disease was identified as a predisposing factor, likewise sinusitis, otitis media or mastoiditis in 29% and immunodeficiency in 11%. Pathogenesis remained unclear in 14%. Initial symptoms and signs were predominantly nonspecific; loss of consciousness occurred in 32% of cases, neurological deficit and seizures each in 25%. Since the availability of CT, both diagnostic delay after hospital admission and mortality were substantially reduced: mean delay from 8.4 to 3.0 days, and mortality from 23% to 0%. Seventeen patients (61%) had follow up examinations 9.6 years (mean) after the acute illness (range 1–21 years). Neurological sequelae were diagnosed in 35% of cases, epilepsy in 29%, epileptic potentials during EEG in 12%, and CNS scars in 50%. Psychological testing revealed no statistically significant differences compared to normal populations. CNS scars, and epilepsy and/or epileptic potentials were more common after excision (7 patients) when compared to patients treated by aspiration and/or antibiotics alone (21 patients). It is concluded that excision of brain abscess should be avoided whenever possible. Therapy of choice consists of the administration of adequate antibiotics with or without CT-guided needle aspirations.     

漏斗胸术后心功能远期随访

目的 探讨漏斗胸术后心脏功能的变化。方法 用超声心动图测定４１例漏斗胸患儿术前、术后的心脏功能,并选择年龄、性别、身高、体重相同的正常儿童作对照。结果 漏斗胸患儿术前射血分数（ＥＦ）与平均左心以缩短率（ＭＶＣＦ）与对照组上比有降低,长期随记发现患儿术后ＥＦ、ＭＶＣＦ与每搏输出量较术前有上升,而且这些指标与对照组相比差异载显著性,结论漏斗胸术后患儿的心脏功能可恢复正常。     

歪嘴哭面容新生儿的临床特征及随访研究

目的：评估歪嘴哭面容新生儿的临床特征及近期转归,为临床医师更深刻地认识该疾病提供信息。方法：回顾性研究2010年1月至2012年2月间收治的11例歪嘴哭面容患儿的临床特点,随访纠正胎龄44周和3月龄时的体格及神经发育情况。结果：11例歪嘴哭面容患儿中,4例合并同侧耳部发育异常,2例先天性心脏病,1例多指和并指畸形。男性8例,女性3例。8例病变为左侧,3例为右侧。父亲年龄超过35岁者8例,母亲年龄超过30岁者7例;多次妊娠者8例,不良孕产史者4例,母亲妊娠期糖尿病者2例。纠正胎龄44周时1例体格发育指标在同胎龄同性别的P10以下,2例NBNA评分小于35分,2例GMs评估扭动阶段为单调（轻度异常）;生后3月时11例患儿体格发育指标均在P10~P90之间,GMs评估不安运动阶段均正常,但歪嘴哭面容均无改善。结论：歪嘴哭面容患儿易合并其他畸形;患儿预后相对较好,但仍需长期随访,适时给予多学科合作干预。     

The natural history of idiopathic toe-walking: a long-term follow-up of fourteen conservatively treated children

Aim: To determine the long-term results after conservative treatment (physiotherapy, casting, orthoses, or a combination of these) of idiopathic toe-walking (ITW). Methods: Tiptoe-walking is diagnosed as idiopathic (habitual) if no signs of neurological, orthopaedic, or psychiatric disease are detected. The diagnosis is one of exclusion. Sixteen former patients with ITW, all now at least 13 y old, were asked to participate in a follow-up investigation 7-21 y after being first diagnosed. Two cases were excluded because heel-cord lengthening had been performed later on in other hospitals. The remaining 14 patients completed a questionnaire. Eleven patients consented to a clinical examination, during which they were videotaped and their active and passive ankle-joint dorsiflexion measured. These data were compared with the assessment at the initial evaluation. In one instance, the toe-walking ceased after conservative treatment (plaster cast). In all other cases the toe-walking pattern recurred. Results: At follow-up three patients showed some toe-walking when they were unobtrusively observed. When videotaped, they did not toe-walk, although a distinct heel-strike was missing. The remaining eight patients all walked with a heel-strike. Two patients had slight symptoms possibly related to toe-walking. No fixed contracture was present at the first evaluation, and none was found at follow-up. There was no systematic change in ankle-joint dorsiflexion from initial assessment to follow-up examination.

Conclusion: Non-surgical treatment of ITW does not have a lasting effect and the long-term results in this study are considered to reflect the natural history, i.e. the toe-walking pattern eventually resolves spontaneously in the majority of children. Surgical treatment of ITW should be reserved for the few cases with a fixed ankle-joint contracture.     

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??Proteinuria occurs mainly due to increased filtration of plasma proteins through glomerular capillary wall and/or incapability of renal tubular reabsorption of small molecular proteins. Significant advances have been made in study on glomerular filtration barrier in recent years. Now endothelial surface layer and subpodocyte space have been accepted as new layers of whole glomerular filtration barrier??and more and more podocyte molecules have been found to play a causative role or participate in the occurrence of proteinuria.