Epiglottitis presenting as acute pulmonary edema

Presented is the case of a 2 1/2-year-old with acute pulmonary edema associated with epiglottitis prior to intubation. The patient complained only of odynophagia and had one brief episode of apnea and flaccid posturing. Chest radiograph demonstrated pulmonary edema. A soft tissue radiograph of the neck confirmed the diagnosis of epiglottitis. The patient was managed successfully with prompt intubation, PEEP, and antibiotics. Pulmonary edema associated with epiglottitis may be more common than previously recognized. It may occur prior to or after intubation. When pulmonary edema is clinically evident, PEEP should be administered early.     

Splenic torsion presenting as a twisted hemorrhagic ovarian cyst

Torsion of a wandering spleen is rare, usually presenting as an acute abdomen and commonly misdiagnosed. In our case, a 14-year-old girl was diagnosed preoperatively as having a twisted hemorrhagic ovarian cyst. On laparotomy, an infarcted spleen that was twisted on its pedicle was found in the lower abdomen. The patient recovered uneventfully. The clinical spectrum of wandering spleen is discussed briefly.     

57例老年人无肢体瘫痪脑梗塞临床与CT分析

目的：探讨老年人无肢体瘫痪脑梗塞临床与CT的关系。方法：对57例老年人无肢体瘫痪脑梗塞的发病诱因，临床表现及CT进行回顾性分析，结果：高血压，TIA，高血脂，冠心病仍为无肢体瘫痪脑梗塞的重要危险因素，结论：肢体瘫痪的发生与否是和传导通路，梗塞部位和病灶大小有头，而与梗塞数目无关，在无MRI条件时，早期头颅CT扫描是诊断的唯一依据。     

Premature ventricular beats in a previously healthy child

A 12-year-old girl presented to the emergency department with a chief complaint of left-sided chest pain. On physical examination she was noted to have an irregular pulse, and an electrocardiogram revealed 30 to 50 premature ventricular contractions per minute with periods of bigeminy. The patient was admitted to the intensive care unit and treated with antiarrhythmic medication without resolution of her premature ventricular contractions. A diagnostic workup, including echocardiogram, Holter monitoring, and exercise stress testing, was performed. It was determined that the cardiac dysrhythmia, consisting of unifocal premature ventricular contractions with a characteristic configuration on the electrocardiogram, was benign in nature and did not require antiarrhythmic therapy.     

急性脑卒中患者偏瘫早期康复的临床研究

目的探讨偏瘫早期康复治疗对急性脑卒中患者患肢功能恢复程度和日常生活活动能力(ADL)的影响。方法选择急性脑卒中偏瘫患者88例,随机分为两组(对照组44例,康复组44例)。康复组除了药物治疗外还进行规范化的康复训练,对照组在药物治疗的同时进行未经指导的自我锻炼。结果经康复治疗后康复组简式Fugl-Meyer评定量(FMA)值、Barthel指数积分均明显高于对照组,差异有显著性意义(P<0.01)。结论早期实施规范化康复训练能明显改善急性脑卒中偏瘫肢体功能和ADL,降低致残率,提高生存质量。     

Acute brain injury in hypoglycaemia-induced hemiplegia.

BACKGROUND: The development of hemiplegia as a result of hypoglycaemia was first described in 1928. However, the mechanism remains unclear. CASE REPORT: We report a case of a 58-year-old male with diabetes, who developed left hemiplegia during a severe hypoglycaemic event. Results Diffusion-weighted magnetic resonance imaging detected an increased signal intensity in the pons, indicating that the patient's hemiplegia resulted from acute brain injury. CONCLUSIONS: This report provides evidence that acute brain injury may be a cause of the neurological deficit.     

Alternating hemiplegia of childhood: a distinct clinical entity and ATP1A3-related disorders: A narrative review

Alternating Hemiplegia of Childhood (AHC) is a rare disorder with onset in the first 18 months of life characterized by stereotyped paroxysmal manifestations of tonic and dystonic attacks, nystagmus with other oculomotor abnormalities, respiratory and autonomic dysfunctions. AHC is often associated with epileptic seizures and developmental delay. Hemiplegic paroxysm is the most remarkable symptom, although AHC includes a large series of clinical manifestations that interfere with the disease course. No cure is available and the treatment involves many specialists and therapies. Flunarizine is the most commonly used drug for reducing the frequency and intensity of paroxysmal events. Mutations in ATP1A2, particularly in ATP1A3, are the main genes responsible for AHC. Some disorders caused by ATP1A3 variants have been defined as ATP1A3-related disorders, including rapid-onset dystonia-parkinsonism, cerebellar ataxia, pes cavus, optic atrophy, sensorineural hearing loss, early infant epileptic encephalopathy, child rapid-onset ataxia, and relapsing encephalopathy with cerebellar ataxia. Recently, the term ATP1A3 syndrome has been identified as a fever-induced paroxysmal weakness and encephalopathy, slowly progressive cerebellar ataxia, childhood–onset schizophrenia/autistic spectrum disorder, paroxysmal dyskinesia, cerebral palsy/spastic paraparesis, dystonia, dysmorphism, encephalopathy, MRI abnormalities without hemiplegia, and congenital hydrocephalus. Herewith, we discussed about historical annotations of AHC, symptoms, signs and associated morbidities, diagnosis and differential diagnosis, treatment, prognosis, and genetics. We also reported on the ATP1A3-related disorders and ATP1A3 syndrome, as 2 recently established and expanded genetic clinical entities.     

Water intoxication with seizures

Presented is the case of a normal two-month-old girl who developed seizures secondary to water intoxication. The infant had been fed 20 to 30 oz of water daily for three days, while her usual formula was withheld because of vomiting and diarrhea. On the day of admission, the infant exhibited signs of water intoxication in the form of lethargy, vomiting, and seizures. Hyponatremia, hypothermia, and hyperglycemia were noted on admission, and are common features of the syndrome. The patient responded well to fluid restriction and salt replacement. Previous reports have attributed water intoxication to feeding mismanagement, vigorous hydration, dilute formulas, and swimming lessons.     

急性脑卒中综合性治疗模式的优越性

目的　探讨综合性治疗 [卒中单元 ,(strokeunit) ]模式对急性脑卒中治疗的优越性。方法　选择在澳大利亚卒中单元治疗的 2 0 7例急性脑卒中病人为A组 ,同期在我科常规治疗的 32 6例为B组。入选标准统一为经CT或MRI确诊发病 48h内的急性脑卒中 ,比较两组主要临床指标。结果　A组 1 4d内病死率为 5 3 % ,其中死于非脑内原因占 2 7 3 % ,感染发生率 1 7 9% ,而B组分别为1 0 4%、64 7%、47 9% ,差异具有显著性 (P <0 0 1 )。 2 1d神经功能评分和随访 90dRankin积分差异也有显著性 (P <0 0 1 )。结论　在综合性治疗模式下急性脑卒中患者的预后 ,比在我科常规治疗模式下的疗效更具有优越性     

Infant with right hemiplegia due to acute encephalopathy with biphasic seizures and late reduced diffusion (AESD): A case report

Rationale:Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a condition characterized by biphasic convulsions and disturbance of consciousness. In Japan, the most common pediatric cases of acute encephalopathy are associated with infection. AESD usually occurs in early childhood, with the characteristic magnetic resonance imaging (MRI) appearance called “bright tree appearance.” The disease often has neurological sequelae and interferes with the schooling of children and their activities of daily living; however, there are few clinical case reports of hemiplegia caused by AESD.Patient concerns:A case with right-sided hemiplegia due to AESD in an 11-month-old girl who was followed up to 30 mo of age.Diagnoses:The patient was diagnosed with overlap AESD and hemiconvulsion-hemiplegia-epilepsy syndrome (HHE syndrome), based on the clinical course and imaging findings. DNA tests of her blood and cerebrospinal fluid revealed the presence of human herpesvirus 6.Interventions:Pharmacotherapy and rehabilitation therapy.Outcome:Gross motor function has recovered considerably, but she had a mild developmental delay at 30 mo old.Lessons:Hemiplegia due to AESD was extremely rare, and appropriate rehabilitation treatment resulted in recovery of physical function. However, as mild developmental delay was observed, the patient was referred to a specialized facility before entering school.     

Lung function in infancy and childhood following neonatal intensive care

Pulmonary function studies were performed in 11 neonatal intensive care survivors both during infancy and later in childhood. Lung function was compared with the respiratory support given in the neonatal period. The mean +/- SE thoracic gas volume was 96 +/- 4% predicted in infancy and rose to 122 +/- 8% predicted during childhood (P less than 0.005). The specific airway conductance (SGaw) in infancy was 57 +/- 7% predicted and rose to 90 +/- 8% predicted in childhood (P less than 0.0025). Abnormalities in SGaw were found only in ventilated infants, and there was a negative logarithmic correlation between the treatment score in the neonatal period and the SGaw in both infancy and childhood. The data indicate a long-term improvement in airway conductance of moderately affected infants with the development of mild hyperinflation in childhood possibly resulting from residual small airway abnormalities despite a symptomless clinical course. The residual abnormalities in prematurely born infants were in proportion to the intensity of treatment required in the neonatal period.     

下肢穴位按摩对急性脑卒中偏瘫病人膝过伸的影响

目的研究下肢穴位按摩对脑卒中偏瘫病人膝过伸的影响。方法 98例急性脑卒中偏瘫病人随机分为观察组(50例)和对照组(48例)。观察组病人给予45 d的规范的康复治疗,并给予下肢穴位按摩,对照组病人只给予45 d的规范康复治疗。结果入组45 d时,观察组病人膝过伸发生率为16.0%低于对照组的33.3%(P<0.05)。结论下肢穴位按摩可以有效防止膝过伸的发生。     

不同强度的中频脉冲电疗对脑梗死偏瘫患者肢体功能康复的疗效观察

目的观察不同强度的中频脉冲电疗对脑梗死偏瘫患者肢体功能康复的效果,探讨中频脉冲电疗的最佳刺激强度。方法选择急性脑梗死偏瘫患者66例,分为低频率组、中频率组、高频率组各22例。在常规治疗及康复治疗基础上,对各组均使用YKL-B型电脑中频治疗仪进行中频脉冲电疗,低频率组给予中频脉冲电刺激20~30 Hz,中频率组给予31~40 Hz,高频率组给予41~50 Hz。各组共治疗2周。治疗前及治疗后,采用美国国立卫生研究院卒中量表(NIHSS)、Brunnstrom分期评估神经功能缺损,采用简化Fugl-Meyer运动功能量表(FMA)评估运动能力,采用改良Barthel指数(MBI)评分评估日常生活能力。比较三组患者治疗前后神经功能、日常生活能力评分及其变化值(⊿NIHSS、⊿Brunnstrom、⊿FMA、⊿MBI)。结果总体患者治疗后的Brunstrom分期、FMA评分、MBI评分均高于治疗前,NIHSS均低于治疗前(P均<0.05)。治疗后,中频率组Brunstrom分期、FMA评分、MBI评分均高于低频率组和高频率组,NIHSS均低于低频率组和高频率组(P均<0.05)。结论中频脉冲电疗对脑梗死偏瘫患者的肢体功能康复有明显疗效,且31~40 Hz的脉冲电强度效果最好。     

Prospective gene expression analysis accurately subtypes acute leukaemia in children and establishes a commonality between hyperdiploidy and t(12;21) in acute lymphoblastic leukaemia

We have prospectively analysed and correlated the gene expression profiles of children presenting with acute leukaemia to the Royal London and Great Ormond Street Hospitals with morphological diagnosis, immunophenotype and karyotype. Total RNA extracted from freshly sorted blast cells was obtained from 84 lymphoblastic [acute lymphoblastic leukaemia (ALL)], 20 myeloid [acute myeloid leukaemia (AML)] and three unclassified acute leukaemias and hybridised to the high density Affymetrix U133A oligonucleotide array. Analysis of variance and significance analysis of microarrays was used to identify discriminatory genes. A novel 50-gene set accurately identified all patients with ALL and AML and predicted for a diagnosis of AML in three patients with unclassified acute leukaemia. A unique gene set was derived for each of eight subtypes of acute leukaemia within our data set. A common profile for children with ALL with an ETV6-RUNX1 fusion, amplification or deletion of ETV6, amplification of RUNX1 or hyperdiploidy with an additional chromosome 21 was identified. This suggests that these rearrangements share a commonality in biological pathways that maintains the leukaemic state. The gene TERF2 was most highly expressed in this group of patients. Our analyses demonstrate that not only is microarray analysis the single most effective tool for the diagnosis of acute leukaemias of childhood but it has the ability to identify unique biological pathways. To further evaluate its prognostic value it needs to be incorporated into the routine diagnostic analysis for large-scale clinical trials in childhood acute leukaemias.     

Streptokinase in the treatment of an acute cerebral embolus--a case report

During cerebral angiographic examination of a sixty-five-year-old white male, an acute right hemiplegia resulting from embolic occlusion of the left middle cerebral artery developed. Intravenous streptokinase therapy was initiated within forty-five minutes of symptom onset and the hemiplegia cleared in approximately one hour with no evidence of intracranial hemorrhage. This observation underscores the applicability and potential benefits of immediate intravenous thrombolytic therapy for selected patients presenting with acute cerebral thrombosis or embolism.     

急性脑梗死偏瘫患者早期康复治疗的成本-效果评价

目的探讨急性脑梗死偏瘫患者早期康复治疗的成本-效果。方法选择急性脑梗死偏瘫患者124例,按随机数分配法分为早期康复治疗组（62例）和对照组（62例）,于入院48 h及治疗3个月后,采用美国国立卫生院卒中量表（NIHSS）和日常生活活动能力量表（ADL）进行评分;并详细记录患者住院期间的护理费、药费、康复费、化验费、床位费、检查费、其他费用和总费用,对两组患者进行成本-效果评价。结果早期康复组的护理费、药费、床位费及总费用低于对照组,差异有统计学意义（P〈0.05）;以NIHSS和ADL改善百分比做标准的成本效果比,早期康复组优于对照组,差异有统计学意义（P〈0.05）。结论对急性脑梗死偏瘫患者进行早期康复,虽然有康复费用,但成本效果比好。     

磁共振扩散张量成像联合Fugl-Meyer量表对老年急性脑梗死患者疗效评估的作用

目的探讨磁共振扩散张量成像(DTI)、简化Fugl-Meyer(FM)运动功能量表在老年急性脑梗死偏瘫患者疗效评估中的应用价值。方法前瞻性纳入解放军第三○五医院神经内科2010年11月—2014年4月符合纳入标准的老年急性脑梗死偏瘫患者17例,入院后均给予药物联合偏瘫实用训练技术康复治疗。分别于入院第1、4、12周完成DTI检查和FM量表评估。结果 3次DTI检查感兴趣区(ROI)健患侧各向异性分数(FA)差值和FM量表评分差异均有统计学意义(F值分别为3.437和3.791,均P0.01)。急性脑梗死偏瘫患者DTI中,ROI健患侧FA差值第1周(0.19±0.06)与第4周(0.13±0.04)比较,差异有统计学意义(P﹤0.05),第4周与第12周(0.13±0.04)差异无统计学意义(P0.05)。经药物、康复综合治疗后FM量表评分:入院第1周[(69±9)分]与第4周[(74±6)分]、第4周与第12周[(82±10)分]比较,差异均有统计学意义(均P0.05)。入院第1次DTI中ROI对应的健患侧FA差值与第1阶段(4周)康复治疗前后FM量表评分差值变化呈线性负相关(rs=-0.389,P0.05)。结论 DTI和FM早期应用于脑梗死偏瘫患者预后评估有一定的预测价值。     

Practical aspects of psychological support to the patient with haemophilia from diagnosis in infancy through childhood and adolescence

Persons with haemophilia, living with their condition from infancy, require attention from a biopsychosocial approach, in which both the biological and the biographical dimension are addressed. These patients and their environment may benefit greatly from having professionals to help them manage, pre‐emptively if possible, to adapt to the disease, cope with the experience of suffering and overcome the difficulties caused by chronicity. The ultimate goal of the interventions was to achieve the best quality of life possible with tailored objectives throughout the patient's life, including disease control, addressing the particular difficulties, and achieving optimal empowerment. This article aims to describe the role of Health Psychology and its professionals in supporting the young patient with haemophilia and provide a brief guide that might be useful for health professionals involved in his care. From the psychological perspective, this paper focuses on communication of diagnosis, the role and support of the family, issues during infancy, childhood and adolescence and how the healthcare team can address them to provide successful support.     

Cerebral large artery occlusion in chronic graft-versus-host disease: A case report

Rationale:Cerebral large artery occlusion in chronic central nervous system graft-versus-host disease after allogeneic hematopoietic stem cell transplantation (allo-HSCT) was very scarce. We described a young patient with bilateral white matter lesions and symptomatic internal carotid artery occlusion after allo-HSCT with the history of aplastic anemia.Patient concerns:A 17-year-old girl with the history of aplastic anemia developed recurrent headache and sudden hemiplegia of right limbs 2 years after allo-HSCT.Diagnoses:She was diagnosed with skin chronic graft-versus-host disease 19 months after allo-HSCT. Brain magnetic resonance imaging showed bilateral subcortical white matter abnormal signals and hyperintensity of left fronto-parietal lobe on diffusion weighted imaging and corresponding hypointense apparent diffusion coefficients indicating acute infarction. CT angiography revealed thrombosis in left internal carotid artery. Carotid plaque high-resolution magnetic resonance imaging showed annular enhancement of vascular wall revealing signs of vasculitis.Interventions:Intravenous immunoglobulin, methylprednisolone, and anticoagulant therapy were used to treat the patient.Outcomes:The patient''s symptoms gradually resolved and she could walk with assistance after 3 weeks before returned home.Lessons:Chronic graft-versus-host disease-associated vasculitis could involve cerebral large vessels which warrants further study.