Evaluation of the cases with acute disseminated encephalomyelitis

Objective  We aimed to describe the epidemiologic, clinical, laboratory features, neuroimaging, treatment, and outcome of children with acute disseminated encephalomyelitis in a cohort study. Methods  In this study, twelve children who were diagnosed as acute disseminated encephalomyelitis were reviewed retrospectively. All of the cases were reevaluated with systemic and neurological examinations, serologic tests, cerebrospinal fluid investigations, magnetic resonance imaging. Result  Their age ranged between 2.5 and 16 years. Five of the cases had initial infections. Patients presented most often with motor deficits (75%), secondly with loss of conscious (33%), and seizures (33%). Spinal fluid abnormalities occurred in 41.6%. Cranial, and spinal magnetic resonance imaging (MRI) revealed hyperintense signal changes mainly in basal ganglia and thalamus (58%), cortical and subcortical areas (33) in T2 weighted images. Myelitis was determined in two cases. Six patients were treated with steroid, and 3 were treated with intravenous immunoglobulin. Ten patients recovered completely. We observed relapse in one case and recurrence in two cases. These cases responded well to high dose intravenous prednisolone followed by oral prednisolone for 6 months. Conclusion  Outlook recovery is generally good in acute disseminated encephalomyelitis. Recurrence and neurological deficits are rarely seen. Early treatment of prednisolone is one of the most important factors to determine the prognosis in this disease.     

Early cytomegalovirus infection and the long-term outcome of biliary atresia

Aim:  To study the impact of ongoing cytomegalovirus (CMV) infection at presentation of biliary atresia (BA) on the long-term outcome after Kasai procedure.
Methods:  Twenty-eight patients with BA born 1988–1997 were included and followed-up until 2007. Eleven patients (group A) had ongoing CMV infection at presentation and were compared to the remaining 17 patients (group B). Median age at Kasai procedure was 75 days in group A and 70 days in group B (p = 0.12).
Results:  Including all patients, survival with native liver was 50% and 36% at 4 and 10 years of follow-up respectively. At the end of follow-up, it was 25% and overall survival was 68%. When comparing groups A and B, neither difference in survival with native liver (p = 0.67, log-rank test) nor in survival after liver transplantation was detected.
Conclusion:  Survival with native liver after Kasai procedure is comparable to that of other centres. CMV positive patients may present with a later onset, alternatively the detection of CMV infection could delay the referral of BA patients. No significant differences in long-term outcome were detected with regard to early CMV infection.     

造血干细胞移植后发生出血性膀胱炎危险因素的分析

目的探讨造血干细胞移植术（HSCT）后出血性膀胱炎的发病相关因素、治疗措施和预后。方法分析我科2000年3月～2008年2月126例HSCT患者出血性膀胱炎发病情况。结果126例移植受者中17例发生出血性膀胱炎,发生率13．5％,中位发病时间36天（16～150天）,中位病程25天（10～103天）,所有患者经过再次水化、利尿和（或）留置导尿管持续膀胱冲洗等治疗,均获得治愈。结论在充分预防出血性膀胱炎后,出血性膀胱炎多为迟发型。多因素分析显示,移植方式、HLA相合程度、急性移植物抗宿主病、异基因移植、清髓性预处理和CMV感染是引起出血性膀胱炎的危险因素。     

喜炎平注射液联合更昔洛韦治疗儿童巨细胞病毒肺炎疗效观察

目的:探讨喜炎平注射液联合更昔洛韦(GCV) 治疗小儿巨细胞病毒(CMV)肺炎的方法及效果。方法:选取我院2010年3月至2016年5月住院确诊的CMV肺炎患儿47例,随机分为观察组24例和对照组23例。对照组在常规治疗基础上加用GCV抗病毒治疗:诱导期每次5 mg/kg,每12 h一次静脉滴注,每次滴注时间>1 h,连用2周;维持期每次10 mg/kg,隔日一次,总疗程4周。观察组在常规治疗基础上加用喜炎平联合GCV治疗,GCV用法用量疗程同对照组,喜炎平注射液5 mg/(kg·d)静脉滴注,每天1次,疗程2周。观察两组患儿临床疗效及预后。结果:(1) 观察组在退热时间、住院时间短于对照组(P均<0.01)。治疗2周后,观察组肺部啰音及影像学改变阳性率低于对照组,血氧分压、二氧化碳分压的改善程度均优于对照组(P均<0.05);(2)治疗2周后两组总有效率比较差异无统计学意义(P>0.05),但观察组显效率高于对照组(62.5% vs 30.4%,P<0.05);(3)两组不良反应比较差异无统计学意义(P>0.05)。结论:小儿CMV肺炎在常规综合治疗、GCV抗病毒治疗同时,联合喜炎平注射液,可提高显效率、缩短疗程、改善预后,且具有较高安全性。     

Efficacy of prolonged valganciclovir therapy for congenital cytomegalovirus infection

We report prolonged valganciclovir (VGCV) treatment of a symptomatic cytomegalovirus infection case. Automated auditory brainstem evoked response performed at 5 days of age revealed severe hearing impairment. Cranial magnetic resonance (MR) imaging at 11 days of age showed abnormal findings. At 5 weeks of age, VGCV was started. The viral load in blood cells, plasma, and urine decreased during the 6-week treatment. Because of improvement of hearing level and no adverse effects, VGCV was restarted for an additional 6 weeks. Neither the patient’s hearing impairment nor results of cranial MR imaging have become worse in 6 months. It is crucial to gather information from as many cases as possible treated with VGCV to establish a standard protocol for VGCV treatment.     

Adrenomedullin plasma concentrations in patients with retinitis pigmentosa

PURPOSE: To evaluate the relationship between retinitis pigmentosa (RP) and plasma adrenomedullin (ADM) levels. METHODS: Blood samples were obtained from a group of 40 consecutive patients with RP matched with 35 healthy subjects (HS) as control. We carried out a complete ophtalmological examination. The study group included 26 patients with RP and 14 patients with syndromic RP. Plasma ADM levels were determined in duplicate with a specific radioimmunoassay method. RESULTS: In the HS plasma ADM levels were 13.7 +/- 6.1 pg/mL. The mean of plasma ADM concentrations in all patients with RP (23.4 +/- 10.7 pg/mL) was significantly (P < 0.0001) higher than that of HS. Moreover, in the syndromic RP patients, plasma ADM levels (28.6 +/- 14.35 pg/ml) were higher than those of HS and RP patients (P < 0.0017). CONCLUSION: The increase of plasma ADM levels in RP patients may be a response to photoreceptor damage.     

Gastrointestinal complications after kidney transplantation

Gastrointestinal complications are common after renal transplantation, and they have a wide clinical spectrum, varying from diarrhoea to post-transplant inflammatory bowel disease(IBD). Chronic immunosuppression may increase the risk of post-transplant infection and medication-related injury and may also be responsible for IBD in kidney transplant re-cipients despite immunosuppression. Differentiating the various forms of post-transplant colitis is challenging, since most have similar clinical and histological features. Drug-related colitis are the most frequently encountered colitis after kidney transplantation, particularly those related to the chronic use of mycophenolate mofetil, while de novo IBDs are quite rare. This review will explore colitis after kidney transplantation, with a particular focus on different clinical and histological features, attempting to clearly identify the right treatment, thereby improving the final outcome of patients.     

Outcome for bilateral cochlear implantation in patients with congenital Cytomegalovirus infection

ObjectivesTo analyze the impact of bilateral cochlear implantation (CI) on perceptual and linguistic development in hearing-impaired children with congenital Cytomegalovirus (CMV) infection.Patients and methodA retrospective study was performed for the period 1991-2016 in a pediatric CI reference center. Closed Set Word (CSW) recognition scores, Categories of Auditory Performance (CAP) and linguistic level on the MT Lenormand scale (MTL) were compared between bilateral (Bi) and unilateral (Uni) groups 12, 24 and 36 months after first CI (CI-1).Results84 patients with congenital CMV infection who underwent CI were included, in 2 groups: sequential or simultaneous bilateral CI (Bi) (N = 20), and unilateral CI (Uni) (N = 64). Twelve, 24 and 36 months after CI-1, CSW scores were 35.56%, 64.52% and 82.93% in Uni and 60.3%, 85% (P = 0.0084*), and 100% (P = 0.00085*) in Bi. CAP scores 12, 24 and 36 months after CI-1 were 2.57, 3.85 and 4.3 in Uni and 3.91 (P = 0.0068*), 5.00 (p = 0.029*) and 5.50 (P = 0.051*) in Bi. MTL linguistic level scores at 12, 24 and 36 months were 0.72, 1.25 and 1.65 in Uni, and 1.72, 3 (P = 0.033) and 3.11 (P = 0.045) in Bi. These significantly better scores in Bi at 24 and 36 months after CI-1 were also found on analysis of subgroups with no associated neurologic disorder (P = 0.046* and P = 0.032*), no associated psychiatric pathology (P = 0.0055* and P = 0.0073*), and no other associated disorder (P = 0.0018* and P = 0.035*), and for all subgroups together (P = 0.0036 and P = 0.037).ConclusionBilateral CI is a faster way than unilateral CI for patients with congenital CMV infection to achieve structured fluent oral language. 50% of the series showed cerebral abnormalities on MRI, without difference between groups. This was not in itself predictive of poor progression of oral communication, unless associated with major neurologic disorder. Some children made little or no use of their CI in the medium term.     

Circadian dysfunction in P23H rhodopsin transgenic rats: effects of exogenous melatonin

Abstract: This study focuses on the effects of retinal degeneration on the circadian patterns of P23H rats, as well as on the effect of exogenous melatonin administration. To this end, the body temperature of P23H and Sprague–Dawley rats was continuously monitored and their retinas examined at different stages of degeneration, by means of histological labeling and electroretinogram recordings. Melatonin (2 mg/kg BW/day) was supplied ad libitum throughout the experiment to a subset of animals. The body temperature recordings from wild‐type and mutant animals showed no differences in the periodogram and the pattern of the mean waveform. However, a progressive decrease in the relative amplitude of the rhythm (RA), a decline in the coupling strength of the rhythm to environmental zeitgebers (interdaily stability, IS) and increased rhythm fragmentation (intradaily variability, IV) were observed in P23H rats, when compared to wild‐type animals. The P23H animals showed a progressive decrease in light‐induced retinal responses until reaching 18 months of age. By this age, all photoreceptors had already disappeared, and no responses were found in the EGRs. Exogenous administration of melatonin improved the visual response of P23H rats. In fact, the maximum b‐wave recorded at 14 months of age was significantly higher in melatonin‐treated P23H rats than in the control animals. Furthermore, the maximum b‐wave recorded for P23H rats at the age of 14 months significantly correlated with RA, IS, and IV. This leads us to conclude that vision loss in P23H rats is correlated with a progressive fragmentation of their circadian patterns. Both effects are partially reversed by melatonin administration.     

异基因造血干细胞移植后肠道病变的内镜表现

目的探讨结肠镜检查在诊断异基因造血干细胞移植（allo-HSCT）后肠道移植物抗宿主病（GI-GVHD）和巨细胞病毒（CMV）肠炎中的作用。方法回顾性对比分析GI-GVHD、CMV肠炎以及GI-GVHD合并CMV肠炎（GC）的结肠镜检查表现及其相关问题。结果47例患者接受50例次结肠镜检查，其中GI-GVHD32例次，CMV肠炎7例次，GC11例次，他们的一般临床资料具有可比性（P〉0．1）；GI-GVHD、CMV肠炎和GC外周血CMV-DNA阳性率分别为28．1％、42．9％和27．3％，三组间差异没有统计学意义（P〉0．1）；肠镜下GI-GVHD和CMV肠炎都有结肠黏膜病变，病变表现呈多样性，除黏膜龟纹样改变和深在溃疡分别是GI-GVHD和CMV肠炎较为特异的病变外，黏膜水肿、红斑、充血、糜烂及浅表溃疡均不能区分GI-GVHD和CMV肠炎；3例GI-GVHD有伪膜形成，1例CMV肠炎有疱疹样黏膜隆起，GC患者容易发生回肠黏膜活动性渗血和回盲瓣炎症。GI-GVHD、CMV肠炎和GC分别有63．8％、70．0％和43．8％的活检标本取自直乙状结肠。结论allo-HSCT患者外周血CMV—DNA检查难以区分GI—GVHD和CMV肠炎；黏膜龟纹样改变和深在溃疡分别是GI-GVHD和CMV肠炎较为特异的改变；GC患者更容易发生回肠黏膜渗血和回盲瓣炎症。左半结肠检查及组织活检能诊断大部分GVHD和CMV感染，但最好进行全结肠检查并到达回肠末端。