Liver cirrhosis in a child associated with Castleman's disease: A case report

BACKGROUND Castleman's disease(CD)is a lymphoproliferative disorder.TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia,anasarca(generalized edema and pleural effusion),fever(pyrexia),reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes,and organomegaly(such as hepatosplenomegaly and multiple lymphadenopathies);TAFRO syndrome is mainly reported in Japanese patients.To our knowledge,this is the first pediatric case report detailing a CD-associated disorder progressing to cirrhosis.CASE SUMMARY A 10-year old male patient presented with fever and anemia.Six months before hospitalization,he had remarkable abdominal distention.Subsequently,he visited a clinic for a fever that lasted 5 d.The physical findings were marked hepatosplenomegaly and cervical lymphadenopathy.A blood test revealed leukocytosis,microcytic anemia,aspartate aminotransferase-dominant transaminase elevation,high levels of C-reactive protein,polyclonal hypergammaglobulinemia,and high levels of interleukin-6 and vascular endothelial growth factor.Abdominal contrast computed tomography and magnetic resonance imaging suggested cirrhosis,which was confirmed by liver histology.Histological findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles,which is compatible with lymph node histology in TAFRO syndrome.Prednisolone was not effective in reducing the patient’s symptoms;therefore,the patient was prescribed tocilizumab.To date,the patient remains free of fever and continues to receive tocilizumab.CONCLUSION We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.     

Bowel Sonoelastography in Patients with Crohn's Disease: A Systematic Review

Sonoelastography is an ultrasound-based method for evaluating the biological properties of a target tissue. It is commonly used in the diagnosis of liver and thyroid disease, but recently its use for monitoring the characteristics of bowel wall in patients affected by Crohn's disease (CD) has been proposed. Our aim was a systematic review of the use of sonoelastography in patients with CD. An initial database search retrieved 32 possible articles. On initial screening, 7 articles, concerning a total of 129 patients and 154 lesions of the small and large bowel, were finally included in the review. The age range varied between 6 and 53 y, and disease duration ranged between 1 and 623?mo. We collected data on the examinations performed (type and time), bowel area considered and parameters evaluated. All authors found that elastography correlates well with the degree of fibrosis, as wall strain significantly decreases in segments affected by fibrotic stenosis. Even though the endpoints proposed in the various articles are relatively inhomogeneous and scarcely comparable, we found that sonoelastography is a promising tool in the evaluation of patients with CD as it can differentiate inflammatory and fibrotic strictures in small case series.     

New treatment modalities in Alzheimer's disease

Alzheimer’s disease (AD) is still a major public health challenge without an effective treatment to prevent or stop it. Routinely used acetylcholinesterase inhibitors and memantine seem to slow disease progression only to a limited extend. Therefore, many investigations on new drugs and other treatment modalities are ongoing in close association with increasing knowledge of the pathophysiology of the disease. Here, we review the studies about the new treatment modalities in AD with a classification based on their main targets, specifically pathologic structures of the disease, amyloid and tau, neural network dysfunction with special interest to the regulation of gamma oscillations, and attempts for the restoration of neural tissue via regenerative medicine. Additionally, we describe the evolving modalities related to gut microbiota, modulation, microglial function, and glucose metabolism.     

Role of micronutrients in Alzheimer's disease: Review of available evidence

Alzheimer's disease (AD) is one of the most common age-related neurodegenerative disorders that have been studied for more than 100 years. Although an increased level of amyloid precursor protein is considered a key contributor to the development of AD, the exact pathogenic mechanism remains known. Multiple factors are related to AD, such as genetic factors, aging, lifestyle, and nutrients. Both epidemiological and clinical evidence has shown that the levels of micronutrients, such as copper, zinc, and iron, are closely related to the development of AD. In this review, we summarize the roles of eight micronutrients, including copper, zinc, iron, selenium, silicon, manganese, arsenic, and vitamin D in AD based on recently published studies.     

Utilization of Hospice Services in a Population of Patients With Huntington's Disease

Context

Although the early and middle stages of Huntington's disease (HD) and its complications have been well described, less is known about the course of late-stage illness. In particular, little is known about the population of patients who enroll in hospice.

Role of Bowel Ultrasound in the Diagnosis and Follow-up of Patients with Crohn's Disease

Crohn's disease (CD) is an inflammatory chronic bowel disorder; it can involve the whole gastrointestinal tract, but its localization in the ileum or colon is most common. The reference standard for the diagnosis of CD is ileocolonoscopy with histologic assessment. The reference standard for the detection of any complications is surgery. However, imaging techniques have an important role both in the detection/localization of CD and in the follow-up of CD patients. In the last few years, the technical development of ultrasound equipment, the advent of new technologies such as elastography and mostly the increased expertise of sonographers have boosted the role of bowel ultrasound in assessment of the gastrointestinal tract. In fact, bowel ultrasound is particularly attractive thanks to its widespread availability, non-invasiveness, low cost and good reproducibility, as it can be easily repeated during follow-up. The aim of this article is to provide an extensive overview of the actual role of bowel ultrasound in the detection and follow-up of patients with CD.     

帕金森病伴不宁腿综合征的现况调查

目的:通过横断面调查帕金森病(PD)合并不宁腿综合征(RLS)的发病率及临床特征。方法:采用病例对照研究,连续选取自2015年10月至2016年1月我院及天坛医院帕金森专病门诊PD患者,分析RLS组、非RLS(NRLS)组的临床相关性,并根据RLS严重程度评分,分为轻、中、重度。同时收集患者发病年龄、病程、有无毒物接触史、UPDRS-Ⅲ评分、改良H-Y分级、贝克焦虑量表(BAI)等临床资料。结果:共收集294例PD患者,合并RLS共87例(29.6%),其中轻度32例、中度36例、重度19例,既往RLS诊治率仅19.5%。RLS组与NRLS组比较,在病程、改良H-Y分级、UPDRS-Ⅲ、BAI等方面差异有统计学意义(P0.05),在发病年龄、文化程度、是否有毒物接触史、PD家族史等方面差异无统计学意义(P0.05)。结论:PD合并RLS存在发病率高、漏诊率高的特点。运动症状较重、病程较长的患者,易合并RLS。     

Analysis of 24 patients with Achenbach’s syndrome

BACKGROUND Achenbach’s syndrome is a rare condition,and the etiology is unknown.It is most commonly seen in the volar plate of the hand distal interphalangeal joint.Patients diagnosed with Achenbach’s syndrome in cardiovascular surgery clinic were retrospectively compared with the literature.AIM To investigate the symptoms,findings,sociodemographic conditions,and laboratory data of patients diagnosed with Achenbach’s syndrome.METHODS The study is a retrospective review of 24 patients diagnosed with Achenbach’s syndrome at Afyonkarahisar State Hospital between March 2015 and November 2016,at Sivas Numune Hospital between November 2016 and November 2017,and at Cumhuriyet University Cardiovascular Surgery Department between November 2017 and November 2018.In the study,demographic characteristics of the patients,signs and symptoms of the disease,and laboratory data were analyzed retrospectively.RESULTS The cohort consisted of 83.33%female patients and 16.67%male patients.The disease was most commonly located in the index finger of the right hand.All of the patients complained of bruising and pain.No pathologic findings were present in the laboratory results.According to these results,it can be concluded that Achenbach syndrome is most commonly seen in the right index finger of middle-aged female patients.CONCLUSION Further research is needed to clarify Achenbach’s syndrome and to develop a diagnosis and treatment algorithm.As the awareness of this syndrome increases,large amounts of data will be obtained.According to current knowledge,Achenbach’s syndrome is not among the known causes of mortality or morbidity.However,it is unknown whether it is seen in brain or other vital organs.     

Recurrent cervical osteomyelitis after radiation therapy in a patient with oropharyngeal cancer

It is crucial to consider cervical osteomyelitis as a differential diagnosis for neck pain in patients who underwent radiotherapy for early diagnosis and management, thereby preventing the development of potentially debilitating neurologic symptoms.     

Patient Factors Associated with Failure to Diagnose Tuberculosis in the Emergency Department

Background

Emergency department (ED) presentation of pulmonary tuberculosis (TB) can be highly atypical and an ED visit might be the only health care interaction for high-risk patients.

Objective

Our objective was to identify patient factors associated with discharge without a diagnosis of TB during an infectious ED visit.

Methods

The study population consisted of 150 patients from 2000 to 2009 with 190 infectious ED visits. Patients were initially identified from the state registry of confirmed TB cases and epidemiological characteristics were identified prospectively during case investigation. A retrospective review was performed for clinical characteristics of visits dichotomized according to whether the diagnosis of TB was made during the ED visit.

Results

Analysis revealed that 77% of all infectious-patient visits ended with a diagnosis of TB. A TB diagnosis was more likely when patients presented with pulmonary or infectious chief complaints, endorsed cough, subjective fever, chills, dyspnea, previous TB infection, or had an abnormal lung examination or chest x-ray study. Patients were significantly less likely to be diagnosed with TB when they were unresponsive during clinical evaluation or when they reported a history of both homelessness and any substance abuse during the last year. In addition, these characteristics were independent predictors of nondiagnosis when traditional TB risk factors or abnormal vital signs were considered.

Conclusions

Patients with atypical presentations, as well as those who were unresponsive or reported a history of homelessness and substance abuse, were at greater risk for nondiagnosis of TB during an infectious ED visit.     

Effect of Age on Substantia Nigra Hyper-echogenicity in Parkinson's Disease Patients and Healthy Controls

Substantia nigra (SN) hyper-echogenicity (SN+) describes an enlargement (>90th percentile) of the area of echogenicity at the anatomic site of the SN in the midbrain detected by transcranial sonography. This ultrasound sign has proven to be a valuable marker supporting the clinical diagnosis of Parkinson's disease (PD). Although there is considerable variation in the extent of echogenic signals at the anatomic site of the SN among PD patients, previous work suggests that SN+ is a stable marker throughout the course of the disease. The present study focused on two aspects: (i) determining whether SN+ values differ between the sides, mirroring the asymmetric character of the disease; and (ii) determining whether age has an influence on SN echogenicity. This cross-sectional study included 300 PD patients and 200 healthy controls. SN+ was measured planimetrically by transcranial sonography. Echogenicity was analyzed separately for onset and non-onset sides, with onset side defined as the SN contralateral to the side of the body that first manifested PD-related motor impairment. Age of the patients and healthy controls at study time was used for correlation. We found that the onset SN+ contralateral to the side of initial motor symptoms was on average 17.6% larger than its counterpart. However, we also found that contrary to the control group, where an increase in age was associated with an increase in size of SN+, age of PD patients was associated with a decline in size of the onset SN+. Furthermore, SN measured at the onset side of PD patients correlated significantly with patient age and Hoehn and Yahr stage, a scale that grades PD severity, although this was not the case for the non-onset side. The present study indicates that changes in SN echogenicity have a different dynamic depending on the onset side of the disease. The age at study time had a significantly negative effect on the size of onset SN+, the effect on the non-onset side was non-significant. We conclude that for appropriate PD analysis, onset SN+ is a more important marker than the average of both sides of SN. Furthermore, we found that among healthy controls, the size of SN+ increases with age.     

Accelerated Infliximab Induction for Severe Lower Gastrointestinal Bleeding in a Young Patient with Crohn’s Disease: A Case Report

BACKGROUNDSevere lower gastrointestinal bleeding (SLGIB) is a rare complication of Crohn''s disease (CD). The treatment of these patients is a clinical challenge. Monoclonal anti-TNFα antibody (IFX) can induce relatively fast mucosal healing. It has been reported for the treatment of SLGIB, but there are few reports on accelerated IFX induction in CD patients with SLGIB.CASE SUMMARYA 16-year-old boy with a history of recurrent oral ulcers for nearly 1 year presented to the Gastroenterology Department of our hospital complaining of recurrent periumbilical pain for more than 1 mo and having bloody stool 4 times within 2 wk. Colonoscopy showed multiple areas of inflammation of the colon and a sigmoid colon ulcer with active bleeding. Hemostasis was immediately performed under endoscopy. The physical examination of the patient showed scattered small ulcers in the lower lip of the mouth and small cracks in the perianal area. Combined with his medical history, physical examination, laboratory examinations with high C-reactive protein (CRP), platelet count (PLT), erythrocyte sedimentation rate (ESR) and fecal calprotectin levels, imaging examinations and pathology, a diagnosis of CD was taken into consideration. According to the pediatric CD activity index 47.5, methylprednisolone (40 mg QD) was given intravenously. The abdominal pain disappeared, and CRP, PLT, and ESR levels decreased significantly after the treatment. Unfortunately, he had a large amount of bloody stool again after 1 wk of methylprednisolone treatment, and his hemoglobin level decreased quickly. Although infliximab (IFX) (5 mg/kg) was given as a combination therapy regimen, he still had bloody stool with his hemoglobin level decreasing from 112 g/L to 80 g/L in a short time, so-called SLGIB. With informed consent, accelerated IFX (5 mg/kg) induction was given 7 days after initial presentation. The bleeding then stopped. Eight weeks after the treatment, repeat colonoscopy showed mucosal healing; thus far, no recurrent bleeding has occurred, and the patient is symptom-free.CONCLUSIONThis case highlights the importance of accelerated IFX induction in SLGIB secondary to CD, especially after steroid hormone treatment.     

Disseminated cryptococcosis in a patient taking tocilizumab for Castleman's disease

Infections occur more frequently in patients receiving biologics. However, cryptococcal infection is uncommon in patients receiving tocilizumab, an interleukin-6 inhibitor, in contrast to patients receiving tumor necrosis factor-α inhibitors. In this report, we describe a case of disseminated cryptococcosis in a 55-year-old man who was receiving tocilizumab every 2 weeks along with daily prednisolone and cyclosporine for Castleman's disease. He initially developed cellulitis on both upper limbs, and his condition worsened despite antibacterial therapy. Chest X-ray scanning and computed tomography demonstrated bilateral pulmonary infiltration. Cryptococcus neoformans was detected in blood, skin, and sputum cultures. He was diagnosed with disseminated cryptococcosis, and successfully treated with liposomal amphotericin B for a week followed by oral fluconazole for 11 months. The findings of this study indicate that cryptococcosis should be considered during the differential diagnosis of infection in patients receiving tocilizumab, especially in the presence of other risk factors for infections or a short tocilizumab dosing interval.     

帕金森病抑郁患者双侧额叶的磁共振波谱改变及意义

目的研究帕金森病抑郁(DPD)患者额叶磁共振波谱(1H-MRS)的改变及其临床意义。方法对20例DPD患者、23例PD患者和20名正常对照者的双侧额叶进行~1H-MRS检测,分析3组双侧额叶的N-乙酰基天门冬氨酸(NAA)/肌酸复合物(Cr)和含胆碱化合物(Cho)/Cr值的变化。结果 DPD患者症状首发侧额叶的NAA/Cr值显著低于PD患者(P0.05),Cho/Cr值显著高于PD患者(P0.05)。DPD患者症状首发侧额叶的NAA/Cr和Cho/Cr值与PD患者比较无显著差异。结论~1H-MRS可以检测到DPD患者额叶的代谢改变,有助于DPD的病因诊断及风险预测。     

Predictive model for health-related quality of life in patients with Parkinson's disease

This study was conducted to develop and test a prediction model that explains health-related quality of life (HRQoL) of patients with Parkinson's disease (PD). Participants were 248 patients with PD enrolled in the neurology clinic of a university hospital in Seoul, Korea. The data were collected through structured questionnaires from March 1 to July 5, 2013. Motor fluctuations, depression, sleep disturbances, fatigue, and activities of daily living had significant direct effects on the quality of life of PD patients. Disease severity, social support, pain, sleep disturbances, fatigue, and activities of daily living had significant indirect effects on patients' quality of life. These predictive variables explained 77.4% of the total variance. The assessment of HRQoL in PD should be considered along with the variables affecting it, including social support. In addition, strategies to enhance social support may be useful for improving the quality of life for patients with PD.     

左旋多巴对早期帕金森病患者视觉诱发电位的影响

目的:探讨早期帕金森病(PD)患者接受左旋多巴治疗后视觉诱发电位(VEP)变化。方法:选择原发性PD患者31例,根据患者意愿,选择同意服用左旋多巴17例为PD-T组,不同意服用左旋多巴14例为PD组,分别行VEP检查,比较N75、P100、N135潜伏期及P100波幅变化。结果:治疗前PD-T组与PD组在N75、P100、N135潜伏期及P100波幅间比较差异无统计学意义(P0.05);治疗后PD-T组在N75、P100、N135潜伏期缩短,P100波幅升高(P0.05或P0.01)。结论:VEP检查可以用于PD患者的视觉障碍研究;早期PD患者使用小剂量左旋多巴对视觉功能有改善作用。     

Crohn's disease confined to the duodenum: A case report

Crohn’s disease (CD) can involve any part of the gastrointestinal tract from the mouth to anus. However, gastroduodenal CD is rare with a frequency reported to range between 0.5% and 4.0%. Most patients with gastroduodenal CD have concomitant lesions in the terminal ileum or colon, but isolated gastroduodenal Crohn’s disease is an extremely rare presentation of the disease accounting for less than 0.07% of all patients with CD. The symptoms of gastroduodenal CD include epigastric pain, dyspepsia, early satiety, anorexia, nausea, vomiting, and weight loss. The diagnosis of gastroduodenal CD requires a high level of clinical suspicion and can be made by comprehensive clinical evaluation. Here we report a rare case of isolated duodenal CD not confirmed by identification of granuloma on biopsy, but diagnosed by clinical evaluation.