The clinical features of combined central and peripheral demyelination and antibodies against the node of Ranvier

Background: Combined central and peripheral demyelination (CCPD) is a disease of inflammatory demyelination that affects central and peripheral nerves simultaneously or temporally separated. Objectives: This study evaluated the clinical characteristics and the existence of antinodal/paranodal antibodies in patients with CCPD. Methods: We reviewed the clinical manifestations, laboratory tests, electrophysiological examinations, neuroimaging findings, treatment, and prognosis of 31 patients with CCPD. Using a live cell–based assay, we tested antinodal/paranodal antibodies. Results: The most common symptoms were motor weakness (83.3%), hyporeflexia (63.3%), and sphincter disturbance (58.1%). In total, 16.6% of patients had impaired vision symptoms, whereas 33.3% of patients had abnormal visual-evoked potentials (VEPs). A total of 21.1% (4/19) of patients were positive for anti-AQP4 (aquaporin 4) antibodies, 20.0% (2/10) of patients were positive for anti-NF155 (neurofascin-155) antibodies, and 10.0% (1/10) of patients were positive for anti-MAG (myelin-associated glycoprotein) antibodies. The effective rates of intravenous corticosteroids, intravenous immunoglobulins, and rituximab were 72.2%, 37.5%, and 100%, respectively. At the illness peak, 75% of patients with CCPD had an mRS (modified Rankin Scale) score of 4 or greater. In remission, 37.5% had an mRS score of 4 or greater. Conclusion: The clinical manifestations of patients with CCPD are highly heterogeneous. We recommend testing antinodal/paranodal antibodies for patients with CCPD.

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Evaluating the validity of dengue clinical-epidemiological criteria for diagnosis in patients residing in a Brazilian endemic area

Transactions of the Royal Society of Tropical Medicine and Hygiene ◽

10.1093/trstmh/traa031 ◽

2020 ◽

Vol 114 (8) ◽

pp. 603-611

Author(s):

Elis Regina da Silva Ferreira ◽

Ana Carolina de Oliveira Gonçalves ◽

Alice Tobal Verro ◽

Eduardo A Undurraga ◽

Maurício Lacerda Nogueira ◽

...

Keyword(s):

Laboratory Tests ◽

Clinical Characteristics ◽

Endemic Area ◽

Clinical Manifestations ◽

Signs And Symptoms ◽

Laboratory Diagnosis ◽

Substantial Variation ◽

Logistic Regressions ◽

Criteria For Diagnosis ◽

Sensitivity Specificity

Abstract Background We evaluated the validity of clinical diagnosis compared with laboratory diagnosis of dengue in a retrospective sample of patients in São José do Rio Preto, Brazil. Methods Our sample included 148 299 clinically (56.3%) or laboratory-diagnosed (43.7%) dengue cases. We compared the sensitivity, specificity, positive and negative predictive value (PPV and NPV) of dengue patients’ demographic and clinical characteristics with laboratory-based diagnosis. We used logistic regressions to estimate the correlation between clinical and laboratory diagnosis of dengue and a full set of dengue signs and symptoms. Results We found substantial variability in sensitivity and specificity of signs and symptoms ranging from 0.8–81.1 and 21.5–99.6, respectively. Thrombocytopenia exhibited the highest PPV (92.0) and lowest NPV (42.2) and was the only symptom showing agreement with laboratory-confirmed dengue (φ = 0.38). The presence of exanthema and thrombocytopenia led to a greater likelihood of concordant clinical and laboratory diagnoses (exanthema: OR: 4.23; 95% CI: 2.09 to 8.57; thrombocytopenia: OR: 4.02; 95% CI: 1.32 to 12.27). Conclusions We found substantial variation in sensitivity, specificity, PPV and NPV of dengue signs and symptoms. For accuracy, clinical and laboratory diagnosis of dengue should be performed concurrently. When laboratory tests are not available, we suggest focusing on the clinical manifestations most associated with dengue.

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Postoperative cognitive dysfunction: progress mechanisms and clinical characteristics

Medical alphabet ◽

10.33667/2078-5631-2019-2-19(394)-29-33 ◽

2019 ◽

Vol 2 (19) ◽

pp. 29-33

Author(s):

K. B. Manysheva ◽

M. A. Akhmedov ◽

A. A. Rakhmanova ◽

S. M. Khutalieva

Keyword(s):

Cognitive Dysfunction ◽

Clinical Characteristics ◽

Cognitive Deficit ◽

Neuropsychological Testing ◽

Postoperative Cognitive Dysfunction ◽

Clinical Manifestations ◽

Postoperative Recovery ◽

Neuropsychological Rehabilitation ◽

Brain Barrier Permeability ◽

The Central Nervous System

The article is devoted to the study of postoperative cognitive dysfunction — a syndrome that is often found in the postoperative period and does not depend on the volume of surgeon. Based on the analysis of the results of modern studies, the authors cite the most likely etiological causes of the syndrome, grouped according to different categories of risk factors. The pathogenetic algorithm for cognitive dysfunction includes the appearance of systemic inflammation, improving blood-brain barrier permeability with the endothelial dysfunction, the migration of inflammatory agents into the central nervous system, and the formation of oxidative stress. The clinical manifestations of cognitive deficit in the outcome of surgeon performed under general anesthesia, the authors illustrate with their own observations of patients with a neurosurgical profile with spinal pathology operated on with the use of propofol anesthesia, comparing the results of neuropsychological testing with an assessment of the level of anxiety. In conclusion, the authors outline a strategy for the prevention of postoperative cognitive dysfunction and recommend conducting neuropsychological rehabilitation as an important component of postoperative recovery for all patients with a diagnosed cognitive deficit that occurred after surgery.

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369. Clinical Characteristics of the First 177 Patients Admitted with COVID-19 at a Bronx Community Hospital

Open Forum Infectious Diseases ◽

10.1093/ofid/ofaa439.564 ◽

2020 ◽

Vol 7 (Supplement_1) ◽

pp. S254-S254

Author(s):

Victoria Bengualid ◽

Maria Martinez ◽

Zhenisa Hysenaj ◽

Debra M Willner ◽

Judith Berger

Keyword(s):

Oxygen Saturation ◽

Clinical Characteristics ◽

Clinical Manifestations ◽

Gastrointestinal Symptoms ◽

Retrospective Chart Review ◽

Patient Characteristics ◽

Altered Mental Status ◽

Channel Blockers ◽

First Case ◽

Overall Mortality

Abstract Background The first case of COVID-19 was admitted on March 15th 2020 to our community based hospital in the Bronx, NY. The aim of this study is to describe the clinical characteristics and outcome of these first COVID-19 patients. Patient Characteristics and Outcome Methods IRB approved retrospective chart review study of all COVID-19 patients admitted during March 2020 focusing on patient characteristics, co-morbidities, clinical manifestations and outcome. Results A total of 177 patients were admitted during March 2020: 57% African American 23.1% Hispanic and 16.9% White. 44.9% female, average age 60 years, and 90% had at least one comorbidity. Outcome was available on all patients except for one who was transferred to another institution for ECMO. Overall mortality was 33%. Clinical presentation: 69.4% presented with cough or shortness of breath, 15.8% with diarrhea, nausea, vomiting or abdominal pain, and 14.6% with myalgia, dizziness or altered mental status. 6.2% presented only with fever. However 59.8% of patients presented with fever and respiratory or gastrointestinal symptoms. Mortality The table compares patients who died vs discharged (either home or to a short term facility). Those that were 65 years or older, hypertensive or presented to the ER with an oxygen saturation of 94% or lower, were more likely to die. Ventilated patients: 31.6% of patients were intubated with a mortality rate of 77%. 22% of these patients were intubated in the first 24 hours. Compared to non-intubated patients, there was no difference in BMI, diabetes, hypertension, COPD/Asthma, use of statins, aspirin or calcium channel blockers. Intubated patients older than 64 years had significantly higher mortality rates (p=0.0001). Conclusion This cohort of COVID-19 patients is unique as almost all received Hydroxychloroquine and Azithromycin. Only 9% received steroids and even fewer received an interleukin-6 inhibitor, convalescent plasma or Remdesivir. African Americans and Hispanics accounted for 80% of patients. Greater than 90% received Medicaid. Overall mortality was 33%. The most common presentation was respiratory followed by gastrointestinal symptoms. The overall mortality was 33% but increased to 77% in intubated patients. Age, hypertension, and ER oxygen saturation correlated with mortality. Disclosures All Authors: No reported disclosures

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SAT0507 CLINICAL CHARACTERISTICS AND TREATMENT RESPONSE IN JUVENILE GOUT PATIENTS: A SINGLE CENTER EXPERIENCE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4622 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1210.2-1210

Author(s):

S. Zheng ◽

P. Y. Lee ◽

Y. Huang ◽

Q. Huang ◽

S. Chen ◽

...

Keyword(s):

Renal Function ◽

Uric Acid ◽

Treatment Response ◽

Serum Uric Acid ◽

Clinical Characteristics ◽

Disease Onset ◽

Clinical Manifestations ◽

Joint Involvement ◽

Normal Body Mass Index ◽

Finger Joints

Background:The incidence of juvenile gout is increasing in China. The clinical manifestations of juvenile gout and treatment strategies to reduce uric acid levels in children are not well described due to the limited number of cases in the past.Objectives:We aim to describe the clinical characteristic of children with gout and study the treatment response to febuxostat.Methods:These studies were approved by the Institutional Review Board of Guangdong Second provincial General Hospital. We performed a retrospective analysis on 98 juvenile gout patients (age ≤ 18 years) evaluated in our hospital from Jan 2016 to Dec 2019. We analyzed clinical parameters, laboratory data and treatment response.Results:The average age of disease onset in children with gout was 15.2 ± 2.0 years and the youngest patient was 9 years old. The majority of patients were male (94/98) and mean serum uric acid (sUA) level were 705.8 ± 145.7 μmol/L (reference range <420 μmol/L). More than half of the cohort had normal body mass index (mean 24.7 ± 4.7 kg/m2; range 14.9 to 36.1 kg/m2). Renal function was generally normal in these children (serum creatinine 96.9 ± 17.8 μmol/L). In terms of joint manifestations, juvenile gout preferentially affected finger joints (29%), ankles (28%) and metatarsal joints (MTP; 20%). The most frequent sites of initial gout attack were ankles (45%), MTP (39%) and fingers (6%). In addition, tophi can occur in pediatric patients and typically develop in the finger joints (54%). Tophi was observed in about 25% of juvenile gout patients, typically within the first two years of disease onset (mean duration 1.7 ± 0. 9 years). We have found tophi in children as young as 10 years of age.For treatment for chronic hyperuricemia, 32 patients (32.7%) were started on febuxostat and 5 patients (5.1%) received allopurinol. A decrease in sUA was observed in both groups after the first month of treatment (febuxostat: baseline 690.4 ± 99.7 μmol/L to 482.7 ± 140.8 μmol/L vs. allopurinol: baseline 728.8 ±112.8 μmol/L to 565.0 ± 116.7 μmol/L, P=0.477). Serum uric acid of 6 patients in the febuxostat group (none in the allopurinol group) dropped below 360 μmol/L. There were no statistical differences in Cr, AST and ALT between the groups. During follow-up after 3 months, further decline in sUA level were observed in patients treated with febuxostat (409.5 ± 83.4, compared with baseline P<0.001).Conclusion:Juvenile gout has a different pattern of joint involvement and is less associated with elevated BMI compared to gout in adults. We show that febuxostat is effective in reducing uric acid levels in juvenile gout. These findings will help clinicians better understand the clinical manifestations and treatment response in juvenile gout.Figure 1Compared treatment response with allopurinol and febuxostatReferences:[1]Kishimoto K, Kobayashi R, Hori D, et al. Febuxostat as a Prophylaxis for Tumor Lysis Syndrome in Children with Hematological Malignancies. Anticancer Res. 2017 Oct;37(10):5845-5849.[2]Lu, C.C., et al. Clinical characteristics of and relationship between metabolic components and renal function among patients with early-onset juvenile tophaceous gout. J Rheumatol, 2014. 41(9): p. 1878-83.Disclosure of Interests:None declared

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Clinical Characteristics and Genetic Analysis of Interstitial Lung Disease in Chinese Children (Genes and Interstitial Lung Disease)

10.21203/rs.3.rs-958870/v1 ◽

2021 ◽

Author(s):

Mei-Xia Huang ◽

Lu Qin ◽

Fei-Zhou Zhang ◽

Lei Wu ◽

Jia-Hui Yu ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Clinical Characteristics ◽

Clinical Manifestations ◽

Failure To Thrive ◽

Surfactant Protein ◽

Chinese Children ◽

Common Mutation ◽

Onset Age ◽

Surfactant Dysfunction

Abstract BackgroundMutation in the surfactant protein C gene (SFTPC) is a cause of interstitial lung disease (ILD). Our objective was to investigate the clinical characteristics, outcome and influencing factors of ILD in Chinese children with SFTPC mutations.MethodA total of 8 Chinese children with ILD heterozygous for SFTPC mutations that were treated in our hospital from January 2014 to December 2020 were included in our study. Candidate genes responsible for surfactant dysfunction were sequenced by next-generation sequencing. The clinical and genetic data were reviewed retrospectively.ResultsThe children’s onset age was before the age of 2 years, and one case was just after birth. The most significant clinical manifestations were cough, tachypnea, hypoxemia and failure to thrive. The most common mutation was p. lle73Thr, which accounted for 87.5% (7/8) of our patients. Four patients whose onset was within 3 months, including 3 children with CMV infection, died. Conclusionp. lle73Thr mutation of SFTPC was an important and common cause of ILD in the Chinese children. The clinical manifestations of ILD associated with this mutation are not specific. The severity and outcome of the disease may be affected by factors such as onset age and viral infection.

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Electron tomographic analysis of cytoskeletal cross-bridges in the paranodal region of the node of Ranvier in peripheral nerves

Journal of Structural Biology ◽

10.1016/j.jsb.2007.10.005 ◽

2008 ◽

Vol 161 (3) ◽

pp. 469-480 ◽

Cited By ~ 33

Author(s):

Guy A. Perkins ◽

Gina E. Sosinsky ◽

Sassan Ghassemzadeh ◽

Alex Perez ◽

Ying Jones ◽

...

Keyword(s):

Peripheral Nerves ◽

Node Of Ranvier ◽

Cross Bridges ◽

Tomographic Analysis

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Karakteristik Klinis Pasien Trombosis Sinus Venosus Serebral (TSVS) di Ruang Rawat Inap Neurologi Rumah Sakit Hasan Sadikin Bandung

Jurnal Neuroanestesi Indonesia ◽

10.24244/jni.v9i2.244 ◽

2020 ◽

Vol 9 (2) ◽

pp. 71-7

Author(s):

Lisda Amalia

Keyword(s):

General Hospital ◽

Clinical Manifestation ◽

Clinical Characteristics ◽

Sinus Thrombosis ◽

Clinical Manifestations ◽

Transverse Sinus ◽

Sinus Venosus ◽

Cortical Vein ◽

Sinus Transversus ◽

D Dimer

Latar Belakang dan Tujuan: Trombosis sinus venosus serebral (TSVS) merupakan penyakit akibat oklusi struktur vena intrakranial, termasuk sinus serebral, vena korteks, dan bagian proksimal vena jugularis. Keterlambatan diagnosis dan terapi dapat mengakibatkan terjadinya komplikasi seperti infark berdarah bahkan kematian.Tujuan penelitian ini adalah untuk mengetahui karakteristik klinis pasien TSVS yang diagnosisnya telah dikonfirmasi melalui pemeriksaan DSA. Metode: Penelitian ini bersifat deskriptif observasional retrospektif. Pada pasien yang telah didiagnosis TSVS di Bagian Neurologi Rumah Sakit Hasan Sadikin Bandung periode Juni 2015 sampai November 2017.Hasil: Terdapat 33 subjek dengan rentang usia 40-49 tahun (24,2%) dan mayoritas adalah perempuan sebanyak 22 orang (66,7%). Faktor resiko terbanyak adalah infeksi 8 orang (24,2%), dan kondisi protrombotik sebanyak 6 orang (18,2%). Gejala klinis terbanyak adalah nyeri kepala yaitu 27 orang (81,8%). Lokasi sinus yang tersering mengalami trombosis adalah sinus transversus pada 28 orang dengan gejala mayoritas nyeri kepala. Kadar D-Dimer ditemukan meningkat dengan rata-rata 1,21 mg/L.Simpulan: Pasien TSVS terjadi pada perempuan dengan rentang usia dekade pertengahan dan faktor resiko terbanyak adalah infeksi. Nyeri kepala merupakan gejala paling sering. Lokasi trombosis mayoritas terjadi pada sinus transversus dan ditemukan peningkatan D-Dimer. Clinical Characteristics of Cerebral Venous Sinus Thrombosis (CVST) Patients in Neurology Ward Hasan Sadikin General Hospital BandungAbstractBackground and Objective: CVST is an entity caused by intracranial vein, including cerebral sinuses, cortical vein and proximal part of the jugular vein. A delay in diagnosing and treating can result in brain infarct with hemorrhagic transformation, even death. The goal of this study is to learn the clinical characteristics of patients with CVST that had been confirmed by DSA.Method: This was a retrospective observational descriptive study and subjects were hospitalized patients which had been diagnosed CVST in Neurology ward Hasan Sadikin General Hospital from June 2015 to November 2017.Results: there was 33 subjects with most (22 patient/66.7%) being women between 40-49 years old. In this study the most frequently found risk factor was infection in 8 subjects (24.2%), prothrombotic conditions in 6 subjects (18.2%). The clinical manifestations were mainly headache in 27 subjects (81,8%). The sinus most often involved was the transverse sinus (28 subjects) with the clinical manifestation being headache. D-dimer levels were also increased with mean 1.21 mg/dl.Conclusion: CVST patients are mostly in women at the midle age of decade, with infection being the most frequent risk. Headache was the most frequent clinical manifestation. Location of thrombosis were more often found on the transverse sinus and D-Dimer level was increased.

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Clinical Warning of Hemophagocytic Syndrome Caused by Epstein-barr Virus

10.21203/rs.3.rs-47979/v1 ◽

2020 ◽

Author(s):

Jinjin Shi ◽

Chu Chu ◽

Min Yu ◽

Dandan Zhang ◽

Yuqin Li ◽

...

Keyword(s):

Laboratory Tests ◽

Epstein Barr Virus ◽

Hemophagocytic Syndrome ◽

Clinical Manifestations ◽

Prognostic Indicator ◽

Barr Virus ◽

Ebv Infection ◽

Ebv Dna ◽

Epstein Barr ◽

D Dimer

Abstract Objectives This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). Methods The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively. Results Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively. Conclusion The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

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Acute Megakaryocytic Leukemia with or Without Acquired Trisomy 21 in 15 Pediatric Patients

10.21203/rs.3.rs-107199/v1 ◽

2020 ◽

Author(s):

Wenzhi ZHANG ◽

Hui LI ◽

Jingzhen LIU ◽

Jiawei XU ◽

Jinjin HAO ◽

...

Keyword(s):

Trisomy 21 ◽

Poor Prognosis ◽

Clinical Characteristics ◽

Pediatric Patients ◽

Laboratory Data ◽

Clinical Manifestations ◽

The Other ◽

Hematopoietic Stem ◽

Acute Megakaryocytic Leukemia

Abstract The knowledge of clinical characteristics and prognosis of pediatric acute megakaryocytic leukemia (AMKL) with or without acquired +21 was limited. We reported 15 AMKL pediatric patients without Down Syndrome (four cases with acquired +21 and 11 cases without acquired +21) with the clinical manifestations, laboratory data, and prognosis. The clinical features and laboratory data between patients with acquired +21 and patients without acquired +21 are similar. As for prognosis, three of the 11 cases without acquired +21 obtained complete remission (CR) after 1st induction. The median follow-up time of the 11 cases was 9 months. Among four cases with acquired +21, one case gave up treatment during 1st induction, one obtained CR after 1st induction and was still alive after 49 months of follow-up. One case obtained CR after 2nd induction and was still alive for 15 months of follow-up after bone marrow transplantation, the other patient was planning for allogeneic hematopoietic stem cell transplantation (HSCT) without CR. The median follow-up time of the four cases was 12 months. None relapsed in our study. In conclusion, acquired trisomy 21 may not be an indicator for poor prognosis. Cytogenetics analysis can help us for diagnosis stratification, prognostic judgment and individualized treatment of AMKL.

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A study of 235 cases of human brucellosis in Sana’a, Republic of Yemen

Eastern Mediterranean Health Journal ◽

10.26719/2001.7.1-2.238 ◽

2001 ◽

Vol 7 (1-2) ◽

pp. 238-246 ◽

Cited By ~ 1

Author(s):

H. A. Al Shamahy ◽

S. G. Wright

Keyword(s):

Differential Diagnosis ◽

Lymph Nodes ◽

Clinical Examination ◽

Clinical Picture ◽

Laboratory Tests ◽

Clinical Characteristics ◽

Geographical Area ◽

Human Brucellosis ◽

Adults And Children ◽

Spleen And Lymph Nodes

We studied the clinical characteristics of brucellosis among all patients with brucellosis referred to the Central Health Laboratory from the main hospitals in Sana’a during a 2-year period [1992-93] [235 adults and children]. A history was taken from each patient and clinical examination, general laboratory tests and brucellosis laboratory tests carried out. The overall clinical picture of brucellosis in this study is very similar to that reported by other workers in this geographical area. Awareness of the presenting features and the realization that brucellosis should be part of the differential diagnosis of febrile patients with enlarged liver, spleen and lymph nodes will lead to an increasing index of suspicion for this disease.

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