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Objective To summarize the clinical features of infection-associated autoimmune encephalitis (IAE) with different antibodies in Xinjiang region, and to explore the diagnostic value of relevant laboratory indicators for IAE. Methods A total of 47 patients with IAE diagnosed and treated in People's Hospital of Xinjiang Uiger Autonomous Region from January 2018 to October 2023 were enrolled, including 18 cases (38.30%) of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis, 16 cases (34.04%) of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, 8 cases (17.02%) of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated encephalitis, and 5 cases (10.64%) of anti-γ-aminobutyric acid receptor type B (GABABR) encephalitis. Social demographic data, clinical manifestations, laboratory and other examinations were collected, and receiver operating characteristic (ROC) curve was drawn to evaluate the diagnostic value of relevant laboratory indicators for IAE. Results The age of onset (χ² = 9.500, P = 0.023), myasthenia (χ² = 9.967, P = 0.019), vision loss (χ² = 9.967, P = 0.019) and seizures (χ² = 8.046, P = 0.045), cerebrospinal fluid (CSF) white blood cell count (χ² = 15.237, P = 0.002) and chloride (F = 4.156, P = 0.011) in patients with anti-LGI1 antibody-associated encephalitis, anti-NMDAR encephalitis, anti-MOG antibody-associated encephalitis and anti-GABABR encephalitis were statistically significant. The age of onset in patients with anti-LGI1 antibody-associated encephalitis was larger than that in patients with anti-NMDAR encephalitis (Z =-2.384, P = 0.017) and anti-MOG antibody-associated encephalitis (Z =-2.420, P = 0.016). The CSF white blood cell count in patients with anti-NMDAR encephalitis was higher than that in patients with anti-LGI1 antibody-associated encephalitis (Z =-3.307, P = 0.001) and anti-MOG antibody-associated encephalitis (Z =-2.835, P = 0.005). CSF chloride was higher in patients with anti-NMDAR encephalitis (t = 3.159, P = 0.007) and anti-GABABR encephalitis (t =-4.592, P = 0.007) than in patients with anti-LGI1 antibody-associated encephalitis. ROC curve showed that the area under the curve (AUC) of age of onset for diagnosis of anti-LGI1 antibody-associated encephalitis was 0.722 (95%CI: 0.569-0.875, P = 0.012), the sensitivity was 0.556, the specificity was 0.821, and the cut-off value was 54.50 years old. The AUC of CSF white blood cell count in the diagnosis of anti-LGI1 antibody-associated encephalitis was 0.706 (95%CI: 0.558-0.855, P = 0.019), the sensitivity was 0.889, the specificity was 0.571, and the cut-off value was 4.50 x 106/L. The AUC in the diagnosis of anti-NMDAR encephalitis was 0.790 (95%CI: 0.643-0.937, P = 0.002), the sensitivity was 0.600, the specificity was 0.967, and the cut-off value was 13.50 x 106/L. The AUC of CSF chloride for the diagnosis of anti-LGI1 antibody-associated encephalitis was 0.748 (95%CI: 0.598-0.898, P = 0.005), with a sensitivity of 0.722 and a specificity of 0.714, and a cut-off value of 122.70 mmol/L. Conclusions IAE with different antibodies in Xinjiang region has specific clinical features. Age of onset, CSF white blood cell count and chloride have important value in the diagnosis of anti-LGI1 antibody-associated encephalitis, and CSF white blood cell count has important value in the diagnosis of anti-NMDAR encephalitis. [ABSTRACT FROM AUTHOR]

Objective To preliminarily summarize the epidemiological and clinical characteristics of functional movement disorder (FMD) based on movement disorders clinic in China. Methods and Results A total of 593 patients with movement disorders newly diagnosed in movement disorders clinic of 22 clinical medical centers from August to September in 2023 were included, including 37 patients (6.24%) with FMD and 556 patients (93.76%) with organic movement disorder. Comparison of the epidemiological characteristics of the 2 groups, the proportions of corona virus disease (COVID-19) infection (χ² = 4.217, P = 0.040) and psychopsychological symptoms (χ² = 18.694, P = 0.000) in the FMD group were higher than those in the organic movement disorder group, while age (t = 3.757, P = 0.000), age of onset (t = 3.720, P = 0.000) and proportion of hypertension (χ² = 4.736, P = 0.030) were lower than those in the organic movement disorder group. Comparison of the clinical characteristics of the 2 groups, the FMD group had higher proportions of tremor (χ² = 3.955, P = 0.047) and myoclonus (Fisher's exact probability: P = 0.011) than the organic movement disorder group, and the proportions of parkinsonism (χ² = 8.491, P = 0.004) and gait disorder (χ² = 5.028, P = 0.025) were lower than those in the organic movement disorder group. Conclusions The age and age of onset, as well as the proportions of COVID-19 infection, hypertension, psychopsychological symptoms, tremor, myoclonus, parkinsonism and gait disorder with FMD were different from those with organic movement disorder in China. [ABSTRACT FROM AUTHOR]

Objective To analyze the clinical characteristics of 2 young patients with cerebral microhemorrhage as the main manifestation of cerebral small vessel disease (CSVD), and explore the relationship between the cerebral microhemorrhage and cognitive dysfunction. Methods and Results Both 2 patients were male, 39 years old, and had onset in their youth, diagnosed with cognitive dysfunction as the main complaint, all of them had a history of hypertension. The clinical manifestations were cognitive decline, including Case 1 with 28 score on Mini -Mental State Examination (MMSE) and 21 score on the Montreal Cognitive Assessment (MoCA). The MMSE of Case 2 was 26 score, the MoCA was 22 score, and Clinical Dementia Rating Scale (CDR) was one score. Laboratory tests ruled out other causes of cognitive dysfunction. The imaging findings of the 2 patients were mainly cerebral microhemorrhage. Among them, the cerebral microhemorrhage of the patient in Case 1 was located in the cortex and subcortex, mainly distributed in the cortex. In Case 2, cerebral microhemorrhage was mainly located in the deep brain. No genetic variation was found to be associated or partially associated with the clinical phenotype. Finally, Case 1 was diagnosed with CSVD, and the possibility of amyloid cerebrovascular disease was high. Case 2 was diagnosed with vascular dementia (VaD) related to CSVD. Conclusions The number and location of cerebral microhemorrhage are closely related to cognitive dysfunction in young people, but the cause of cerebral microhemorrhage needs to be further clarified. [ABSTRACT FROM AUTHOR]

Objective To report clinical phenotype and gene mutation characteristics of one case of early-onset Alzheimer's disease (EOAD) associated with PSEN2 V214L mutation and conduct a pedigree analysis. Methods and Results A 63-year-old male proband with no family history of dementia, onset at 58 years old, had memory loss as the first manifestation and progressive aggravation of symptoms. Neuropsychological tests showed a score of 1 on Mini-Mental State Examination (MMSE), 0 on Montreal Cognitive Assessment (MoCA), 71 on Activities of Daily Living Scale (ADL), and 3 on Hachinski Ischemic Score (HIS). Head MRI revealed extensive cortical atrophy, with prominent atrophy of bilateral hippocampal, multiple hyperintensity in deep white matter (Fazekas grade 2). Exons of high-throughput sequencing of dementia-related genes showed that both the proband and his sister had heterozygous missense mutations in exon 8 of PSEN2 gene c.640G > T (p.V214L) and heterozygous splicing mutations in exon 28 of SORL1 gene c.3815-4A > C. The proband was finally diagnosed with EOAD associated with PSEN2 V214L mutation, and the proband's sister was considered as an absolute risk group for Alzheimer's disease (AD). Conclusions The PSEN2 V214L mutation probably causes EOAD. Further investigations should be conducted to evaluate the effects on the production of amyloid β-protein 40 and 42 (Aβ40 and Aβ42), then verify its pathogenicity. [ABSTRACT FROM AUTHOR]

Copyright of Chinese Journal of Contemporary Pediatrics is the property of Xiangya Medical Periodical Press and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To summarize the clinical features, treatment options and prognosis of late - onset and very late - onset neuromyelitis optica spectrum disorders (LO - NMOSDs and VLO - NMOSDs). Methods Total 68 patients with NMOSDs admitted to Beijing Tongren Hospital, Capital Medical University from January 2015 to December 2021 were included, while 55 with LO -NMOSDs and 13 with VLO-NMOSDs. The sex, first-onset symptoms, Extended Disability Status Scale (EDSS), complications, anti- aquaporin 4 (AQP4) antibody and autoimmune antibody, imaging examinations (optic nerve and spinal cord), therapeutic schedule and prognosis were compared between 2 groups. Results Compared with LO - NMOSDs group, VLO-NMOSDs group had higher EDSS scores at nadir (Z = - 2.119, P = 0.034), but there were no significant differences in sex, first-onset symptoms, combination with other autoimmune diseases or tumors, anti - AQP4 antibody and autoimmune antibody, optic nerve and spinal cord involvement sites, therapeutic schedule and EDSS scores within 14 d after acute treatment (P > 0.05, for all). Conclusions Patients with VLO - NMOSDs and LO - NMOSDs have similar demographic characteristics, clinical characteristics, therapeutic schedule and short - term prognosis, but the VLO - NMOSDs has more severe disability at nadir, and its long-term prognosis remains to be further explored. [ABSTRACT FROM AUTHOR]

Objective To study the correlation between the time from rapid eye movement sleep behavior disorder (RBD) to disease onset and the clinical characteristics of Parkinson's disease (PD). Methods A total of 123 PD patients were recruited from Tianjin Huanhu Hospital between September 2019 and December 2021. All participants were divided into the group with RBD (41 cases) and the group without RBD (82 cases) according to the probable RBD criteria. Hoehn-Yahr staging was used to evaluate PD grade. Unified Parkinson's Disease Rating Scale III (UPDRS III) was used to evaluate the motor function of PD. Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Hamilton Anxiety Rating Scale (HAMA) and Hamilton Depression Rating Scale (HAMD), Parkinson's Disease Sleep Scale (PDSS), and Non - Motor Symptom Scale (NMSS) were used to evaluate cognitive function, anxiety, depression, sleep and total non-motor symptoms. The life quality of patients was evaluated by 39-Item Parkinson's Disease Questionnaire (PDQ-39). Spearman rank correlation analysis was used to explore the correlation between the time from RBD to disease onset and clinical characteristics in PD. Results UPDRSIII (t = - 2.703, P = 0.008) and NMSS (t = - 2.176, P = 0.032) scores of PD with RBD group were higher than those of the PD without RBD group. The time from the RBD to the onset of PD was positively correlated with NMSS score (rs = 0.547, P = 0.001). Conclusions The motor and non-motor symptoms in PD patients with RBD are more severe than those of patients without RBD. Early onset of RBD in prodromal period may indicates more severe non-motor symptoms in future. [ABSTRACT FROM AUTHOR]

Objective To explore the prevalence, influencing factors, and clinical and imaging features of cervicocerebral artery dissection (CAD) in young patients with ischemic stroke. Methods A total of 242 young patients with first ever ischemic stroke were included in this study from October 2016 to September 2020. Thirty-two patients with CAD were included in the CAD group, and 210 patients without CAD were included as controls. We collected the clinical and imaging data of all the patients. Univariate and multivariate Logistic regression analysis were used to screen the influencing factors of young ischemic stroke patients caused by CAD. And we analyzed imaging characteristics of CAD in different locations. Results Among the 242 young patients with ischemic stroke, 32 (13.22%) patients were due to CAD. Compared with the control group, the CAD group had younger onset age (t = -3.480, P = 0.001), more inducible events (infection and neck movement) before the onset of cerebral infarction (t = 8.796, P = 0.003), and higher incidence of headache or neck pain (t = 17.466, P = 0.000) and posterior circulation infarction (t = 22.705, P = 0.000). Multivariate Logistic regression analysis showed that older age (OR = 0.920, 95%CI: 0.853-0.992; P = 0.030), the infarction located in the anterior circulation (OR = 0.145, 95%CI: 0.059-0.356; P = 0.000) were protective factors for ischemic stroke due to CAD, while head and neck pain (OR = 5.428, 95%CI: 1.653-17.824; P = 0.005) was risk factor for ischemic stroke due to CAD. The artery dissection lesions were located in anterior circulation in 11 (34.37%) patients with CAD, including 9 cases of extracranial carotid artery, one case of anterior cerebral artery, and one case of middle cerebral artery, while 21 (65.63%) patients' artery dissection lesions were located in the posterior circulation, including 10 cases of extracranial vertebral artery dissection (2 patients with bilateral lesions), 6 cases of intracranial segment, 4 cases with both intracranial and extracranial segment involvement, and one case with posterior inferior cerebellar artery dissection. After discharge, the follow-up imaging examination of 9 CAD patients showed that 6 patients had varying degrees of recovery of the arterial lumen and the rest 3 patients had no change. Conclusions CAD is a common cause of cerebral infarction in young people, and younger age, posterior circulation infarction and head and neck pain are risk factors for CAD. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical and EEG features of patients with different ages of Rasmussen encephalitis to evaluate the efficacy of different surgical methods. Methods and Results According to the age of onset, the patients with Rasmussen encephalitis were divided into 2 groups, one group was under 6 years old (≤ 6 year group, n = 18), the other group was over 6 years old (> 6 year group, n = 18). The medial duration of advancing to actue phase was 5 (2, 11) months in ≤ 6 year group and 12.00 (5.50, 24.00) months in > 6 year group, respectively (P = 0.014). Nine months after onset, 15 cases advanced to acute phase in < 6 year group, and 6 cases advanced acute phase in > 6 year group (P = 0.006). EEG showed persistent/intermittent slow wave activity over the affected hemisphere (35 patients). Nine months after onset, 2 groups of patients with multi-region epileptiform discharges have advanced to acute phase from prodromal phase (10 cases in ≤ 6 year group and 2 in > 6 year group; P = 0.036). The prognosis of functional hemispherectomy was Engel I in 21/27 cases (11 cases in ≤ 6 year group and 10 in > 6 year group), anatomic hemispherectomy Engel I in 8/8 cases (4 cases in both ≤ 6 year group and > 6 year group, respectively). Conclusions The earlier the onset age of Rasmussen encephalitis patients, the faster the disease progresses. The EEG shows continuous slow wave and multi-epileptic discharge in the affected side hemisphere. Anatomic hemispherectomy and/or functional hemispherectomy still remains of be the only cure for the seizures caused by Rasmussen encephalitis. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical, pathological and genetic features of 10 patients with late-onset mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS). Methods and Results The clinical data of 10 patients with late-onset MELAS were retrospectively analyzed from January 2007 to December 2018. Muscle biopsy was performed in 8 cases. Polymerase chain reaction-fragment length polymorphism (PCR-RFLP) analysis and whole sequencing of mitochondrial DNA (mtDNA) were used to screen mtDNA mutations, and the mutation load of m.3243A > G in blood was detected by pyrophosphate sequencing. The onset age of the first stroke-like episodes were 40-67 years old in all patients. The main manifestations included epilepsy, aphasia, headache, dementia, mental disorder, limb paralysis and visual impairment. Past history revealed 5 cases with diabetes mellitus, 6 with deafness, 3 with hypertension and 2 with stroke. Six patients had a family history of maternally inherited diabetic mellitus, and 2 had a family history of MELAS. Laboratory examination revealed 6 cases with hyperlipidemia, 6 with carotid atherosclerosis, 1 with stenosis of right internal carotid artery and middle cerebral artery. Brain MRI showed cortex lesions involving one or more lobes in all patients, and 4 cases also had multiple infarctions in brainstem and basal ganglia. Muscle biopsy demonstrated ragged red fiber (RRF) and strongly succinate dehydrogenase-stained vessels (SSVs) in all of 8 patients except one. Genetic analysis identified 9 cases with m.3243A > G, and 1 with m.10191T > C mutation. The blood mutation load of m.3243A > G was 9%-33% in 7 cases. Conclusions The clinical phenotype of patients with late-onset MELAS was not significantly different from that of typical patients. However, the age of onset in late-onset MELAS was late, and it could be complicated with a variety of cerebrovascular risk factors and atherosclerosis. The hotspot mutation of this group of late-onset MELAS patients was m.3243A > G, but the mutation rate in blood was low. [ABSTRACT FROM AUTHOR]

Objective To analysis the safety and outcome of intravenous thrombolysis with recombinant tissue plasminogen activator (rt - PA) in patients with acute mild ischemic stroke. Methods From November 2017 to October 2018, 252 patients with acute mild ischemic stroke who were admitted into our hospital within 4.50 h after onset (84 patients in intravenous thrombolysis group, 108 patients in aspirin and clopidogrel group, and 60 patients in aspirin or clopidogrel group). The general condition, the level of high-sensitivity C-reactive protein (hs-CRP) and erythrocyte sedimentation (ESR) were compared among 3 groups. The modified Rankin Scale (mRS) were evaluated on 7 d after admission or at earlier discharge, and 21 and 90 d. Symptomatic intracranial hemorrhage rates were compared among 3 groups. Results There were no significant difference in general clinical data, the initial level of hs-CRP and ESR between the intravenous thrombolytic group and the 2 non-thrombolysis groups. At 7 d after admission or earlier discharge, the descending range of hs-CRP and ESR in 3 groups were significant (P = 0.000, for all); there were significant differences among the 3 groups (P = 0.000, for all); pairwise comparison of 3 groups showed that the levels of hs-CRP and ESR in intravenous thrombolytic group at 7 d were significantly lower than aspirin and/or clopidogrel group (P = 0.000, 0.000; P = 0.000, 0.010). There was no significant difference in the rate of intracranial hemorrhage between thrombolytic group and other 2 groups at 24 h after treatment [1.19% (1/84) vs. 0 (0/168); χ² = 2.008, P = 0.366]. At 7 d after admission or earlier discharge, the intravenous thrombolytic group had better prognosis than aspirin and/or dopidogrel group (P = 0.043, 0.012); At 21 and 90 d, the intravenous thrombolytic group had better prognosis than aspirin or clopidogrel group (P = 0.021, 0.037); but compared with aspirin and clopidogrel group, there was no significant difference (P = 0.070, 0.053). Conclusions Patients with mild ischemic stroke, especially that may induce disability should be treated with rt-PA intravenous thrombolysis timely. If it impossible by any causes, aspirin and clopidogrel should be given within 24 h after onset. [ABSTRACT FROM AUTHOR]

Background and objective Pneumonic-type lung carcinoma is a special type of lung cancer both clinically and radiologically. Here we present our experience on pneumonic-type lung carcinoma in an attempt to investigate the clinical, radiological and pathological features, diagnostic procedures, treatment, and prognosis of this type of tumor. Methods Pathologically confirmed lung cancer with a chest CT characterized by ground glass opacity or consolidation was defined as pneumonic-type lung carcinoma. Cases with advanced pneumonic-type lung carcinoma admitted to Peking Union Medical College Hospital (PUMCH) from January 1, 2013 to August 30, 2018 were enrolled. Retrospective analysis of clinical data and survival follow-up of these patients was conducted. Results A total of 46 cases were enrolled, all of which were adenocarcinoma. Cough (41/46, 89.1%) and expectoration (35/46, 76.1%) were the most prominent symptoms. The most frequent chest CT findings were ground glass attenuation (87.0%), patchy consolidation (84.8%), and multiple ground-glass nodules (84.8%). Multiple cystic changes (40%) and cavitation (13%) were also quite frequent. Ipsilateral and contralateral intrapul-monary metastasis were noted in 95.3% and 84.8% of cases respectively. The median duration from symptom onset to diagnosis was 214 days (95%CI: 129-298). Both surgical lung biopsy and CT-guided percutaneous lung biopsy had a diagnostic yield of 100%. Transbronchial lung biopsy (TBLB) combined with bronchoalveolar lavage (BAL) had a diagnostic yield of 80.9% (17/21). Sputum cytology had a diagnostic yield of 45% (9/20). Twenty-six cases were invasive mucinous adenocarcinoma (26/46, 56.5%) and the remainder were unable to identify pathological subtypes due to lack of adequate biopsy sample size. EGFR mutation was detected in 15.8% (6/38) of patients and ALK rearrangement was detected in 3.0% (1/33) of patients. The median overall survival for these patients was 522 d (95%CI: 424-619). In patients without EGFR mutation or ALK rearrangement, chemotherapy significantly improved survival (HR=0.155, P=0.002,2). The median overall survival was 547 d (95%CI: 492-602 d) with chemotherapy and 331 d (95%CI: 22-919) without chemotherapy. Conclusion Diagnosis of pneumonic-type carcinoma is usually delayed due to clinical and radiological features mimicking pulmonary infection. TBLB combined with BAL has a quite high diagnostic yield. The most frequent histological type is invasive mucinous adenocarcinoma. The incidence of EGFR mutation or ALK rearrangement is low in pneumonic-type carcinoma. For patients without cancer driver genes, chemotherapy is recommended to improve overall survival. [ABSTRACT FROM AUTHOR]

Background and objective The aim of this study was to investigate the clinical features of paraneoplastic limbic encephalitis (PLE) with small cell lung cancer (SCLC) and improve clinical diagnosis and treatment. Methods The clinical data of 15 patients with SCLC combined with PLE from January 1980 to May 2017 were collected from Beijing Union Hospital. Their symptoms and laboratory data were analyzed and the prognosis of the patients was followed. Results PLE is a rare disease, the incidence rate in SCLC is about 0.842%. The data may be underestimated because of misdiagnose or missed diagnosis; High incidence crowd of the disease is the middle-aged male smoker, the tumor-node-metastasis (TNM) stages of them are later than others; Typical neurological symptoms include varying degrees of short-term memory loss, seizures and varying degrees of mental disorders; neurological symptoms usually occur before the onset of cancer or respiratory symptoms appear, an average of about 2 months be taken from onset to diagnosis; Serum antibody (anti-Hu, GABA-R-Ab), cerebrospinal fluid, head magnetic resonance imaging (MRI) and electroencephalogram (EEG) of the patients has abnormalities; Videography, especially computed tomography (CT) is a good means of screening the primary tumor, pathology diagnosis mainly rely on bronchoscopy; The treatment of primary tumors can be more effective in alleviating the nervous system symptoms than immunotherapy. Conclusion Paraneoplastic limbic encephalitis is a rare paraneoplastic syndrome in nervous system caused by malignant neoplasms often characterized by facial neurological symptoms. The disease are usually associated with lung cancer (especially SCLC). Its nervous system symptoms occur earlier than the tumor diagnosis. Early diagnosis and treatment for primary tumors will increase the benefit. [ABSTRACT FROM AUTHOR]

Objective To study histological features, immunophenotype, genotype, diagnosis and differential diagnosis, treatment and prognosis of one case of mitochondrial encephalomyopathy with lactic acidemia and stroke-like episodes (MELAS), and to summarize the clinicopathological features and key points of diagnosis and differential diagnosis. Methods and Results A 55-year-old female presented language disorder with memory disorders for 2 months, and her head MRI showed space-occupying lesion of left temporal lobe. An exploratory craniotomy was performed. Histological morphology showed patchy grey and white matter structure, scattered hemorrhagic necrosis, small vascular hyperplasia, luminal hyperemia and vasodilatation, and scattered lymphocytic infiltration. By using immunohistochemical staining, the membrane of lymphocytes was focally positive for CD3 and CD20, and neurons were positive for neuronal nuclei (NeuN). Periodic acid-Schiff (PAS) staining was focally positive. In further muscle biopsy, modified Gomori trichrome (MGT) staining showed a lot of scattered ragged-red fibers (RRF), suggesting myopathologic change of mitochondrial encephalomyopathy. A m.3243A > G mutation (about 9%) was found in the direct sequencing genetic testing. The final diagnosis was MELAS. After operation, the patient received antiepileptic, hypoglycemic and neurotrophic treatment. Conclusions Adult-onset MELAS is a rare disease with various clinical manifestations. A definite diagnosis depends on clinical manifestations, histopathological characteristics, immunophenotype and genetic testing. [ABSTRACT FROM AUTHOR]

To study the efficacy and safety of hematoma puncture catheter drainage under CT real-time guide for treatment of intracerebral hemorrhage (ICH). Methods A total of 80 ICH patients with hematoma volume 15-30 ml were given conservative treatment (control group, 40 cases including 6 cases with hematoma volume 15-20 ml and 34 cases with hematoma volume > 20-30 ml) and hematoma puncture and catheter drainage under CT real-time guide (operation group, 40 cases with hematoma volume > 20-30 ml). Conscious states were evaluated by Glasgow Coma Scale (GCS) on admission and 3 d after onset. Neurological deficits of patients were evaluated by National Institutes of Health Stroke Scale (NIHSS) on admission and discharge. Hospitalization days and complications (including rebleeding, epilepsy, intracranial infection, severe pulmonary infection and bleeding caused by digestive tract stress ulcer) were recorded. Results The GCS scores 3 d after onset (P = 0.000) and NIHSS scores on discharge (P = 0.000) of 2 groups were significantly lower than those on admission. The GCS score of operation group was significantly higher (P = 0.003) and NIHSS score was significantly lower (P = 0.000) than that of control group. The hospitalization time of operation group was significantly lower than those of control group [(10.53 ± 2.64) d vs. (17.30 ± 4.92) d; t = 7.673, P = 0.000]. Complications including rebleeding, intracranial infection, severe pulmonary infection and bleeding caused by digestive tract stress ulcer did not occur in patients of 2 groups. Conclusions For supratentorial hemorrhage patients with hematoma volume 15-30 ml, in comparison with conservative treatment, operation with hematoma puncture and catheter drainage under CT real-time guide can save hospitalization days, relieve edema peak response and improve the prognosis. [ABSTRACT FROM AUTHOR]

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Copyright of Chinese Journal of Lung Cancer is the property of Chinese Journal of Lung Cancer and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Modern Laboratory Medicine is the property of Journal of Modern Laboratory Medicine Editorial Department and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To study the clinical features and gene mutations of early - onset epileptic encephalopathy (EOEE) and to explore the application in pathogenic diagnosis of EOEE by next generation sequencing. Methods The clinical data of 68 cases diagnosed with unexplained EOEE between June 2014 and December 2017 were obtained and analyzed. Specimens were collected from the selected children and their parents. Next generation sequencing was used to detect epilepsy - related genes, and Sanger sequencing was performed to verify the results and confirm the source of parents, further to identify suspected pathogenic mutations of EOEE. Results Among 68 cases with EOEE, 18 cases (26.47%) were detected with epilepsy - related genes. One was diagnosed as pyridoxine dependent epilepsy (PDE), which was caused by ALDH7A1 mutation. One was diagnosed as thiamine metabolism dysfunction syndrome 2 (THMD2), which was caused by SLC19A3 mutation. Among 13 cases of epilepsy syndrome, 6 cases were diagnosed as Dravet's syndrome (DS), 5 caused by SCN1A missense mutation and one by SCN1A nonsense mutation; 4 cases were diagnosed as infantile spasm (IS), one caused by TSC1 nonsense mutation but the pathogenicity cannot be identified while pathogenic mutations were not detected in other 3 cases; 2 cases were diagnosed as Ohtahara's syndrome (OS), one caused by STXBP1 missense mutation and pathogenic mutation was not found in the other; one case of malignant migrating partial seizures in infancy (MMPEI) was not found the pathogenic mutations. Among 53 cases with non - specific EOEE, suspected pathogenic mutations were detected in 9 cases: one case of SCN8A missense mutation, one case of KCNQ2 missense mutation, one case of KCNH5 missense mutation, one case of CACNA1A missense mutation, 2 cases of CDKL5 nonsense mutation, one case of CDKL5 frame - shift mutation, one case of PCDH19 nonsense mutation and one case of GRIN2A missense mutation. All cases were treated by 2 and more antiepileptic drugs (AEDs), one case of PDE was given vitamin B6 20 mg/(kg ⋅ d), and one case of THMD2 was given vitamin B1 25 mg/(kg⋅d) and biotin 2 mg/(kg⋅d). After followed by 6 months to 8 years, 15 cases (22.06%) were seizure free, 21 cases (30.88%) were part of control and 32 cases (47.06%) were out of control. Conclusions The clinical phenotypes of children with unexplained EOEE are varied. One case was diagnosed as PDE and one as THMD2 after gene sequencing. After treated by etiological treatment, patients with remediable genetic disease were seizure free. Some gene sites were denovo mutations which have not been reported such as missense mutation for SCN1A, STXBP1, KCNH5, CACNA1A, frame - shift mutation for CDKL5, and nonsense mutation for PCDH19, which enriched the mutation spectrum of EOEE. [ABSTRACT FROM AUTHOR]

Spinal muscular atrophy (SMA) is the most common fatal neurogenetic disease in infant period. Clinical manifestations of SMA include symmetrical and progressive weakness and atrophy of proximal limbs. Based on age of incidence, it is divided into child-onset SMA and adult-onset SMA. Child- onset SMA has the highest incidence rate. This article summarizes the research progress of SMA therapy in recent years to provide new thoughts for the treatment of SMA. [ABSTRACT FROM AUTHOR]

Objective The clinical, imaging and pathological manifestations of one patient at different stages of primary central nervous system lymphoma (PCNSL) have been analyzed to disclose its pathogenesis. Methods and Results A 29-year-old female patient showed recurrent onsets. On the first onset, the main clinical manifestation was blurred vision and visual field defect. Cranial MRI showed a patchy lesion in the right parietal and occipital lobes without obvious occupying sign. T1WI showed slight low-intensity sign, T2WI and FLAIR showed high-intensity signs. Enhanced scanning showed heterogeneous punctate enhancement. The lesion disappeared gradually after glucocorticoid impact therapy. Headache, vomiting and left limb paralysis were the main clinical manifestations on the second onset, and MRI showed a lesion in the right frontal and parietal lobes with obvious occupying sign and solid enhancement, low- intensity on T1WI, high-intensity on T2WI and FLAIR. The tumor was totally removed under microscope. Histological findings showed large tumor cells, rich cytoplasm, nuclei with various sizes and shapes, conspicuous mitosis, patchy necrosis, and interstitial small vessel hyperplasia. Immunohistochemical staining showed that membrane of tumor cells was positive for CD20, and nuclei were positive for paired box gene 5 (PAX5) and multiple myeloma oncogene 1 (MUM1). In a few tumor cells, membrane was positive for CD10 and CD30, and nuclei were positive for cyclin D1. Besides, tumor cells were negative for CD3, anaplastic lymphoma kinase (ALK) and glial fibrillary acidic protein (GFAP). Ki-67 labeling index was about 80%. EBER in situ hybridization (ISH) assay showed that mRNA coded by Epstein-Barr (EB) virus was negative. The tumor showed angiotropic characteristics, and the walls of involved blood vessels were wrapped and destructed. The final diagnosis was confirmed as diffuse large B cell lymphoma. Conclusions PCNSL has various imaging features, revealing as diffuse infiltrating or solid occupying lesions, which emerge as the result of its angiotropic characteristics. [ABSTRACT FROM AUTHOR]

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Creutzfeldt - Jakob disease (CJD) is a fatal neurodegenerative disease caused by prion protein infection. Compared with other neurodegenerative diseases, neuronal cell apoptosis in CJD occurs more rapidly. It has become increasingly evident that sleep dysfunction is commonly seen in chronic neurodegenerative conditions and may present several years before the disease onset. Therefore, understanding the relation of dyssomnia to CJD may enhance our realization of the relationship between sleep disorders and other common neurodegenerative diseases. More and more studies have shown that sleep disorders are very common in CJD patients. Thus, clinicians should regularly screen for sleep conditions in possible CJD patients to enhance the diagnosis and treatment of CJD. [ABSTRACT FROM AUTHOR]

Background China is one of the countries that have the largest number of patients suffering from Duchenne and Becker muscular dystrophy (DMD/BMD). Although the building of international DMD/BMD databases has laid a foundation for clinical drug development and clinical trials, it has not yet been carried out in China. In this study, a modified registry form of Remudy was applied to 229 DMD/BMD patients in order to establish a comprehensive database, which will lay the groundwork for international cooperation. Methods A total of 229 DMD/BMD patients diagnosed by genetic testing or muscle biopsy admitted in Children's Hospital of Fudan University (CHFU) during the period of August 2011 to December 2013 were enrolled in this study. The data included sex, age, age at diagnosis, geographic distribution of patients, DMD gene mutation types, family history, walking capability, cardiac and respiratory function, steroid treatment and rehabilitation intervention. Results There were 194 DMD and 35 BMD male patients who were diagnosed at the age of 0-18 years, and among them, most patients were diagnosed at the age of > 3-4 (16.59%, 38/229) and > 7-8 (14.85%, 34/229) years. Exon deletion was the most frequent genetic mutations for DMD/BMD [65.46% (127/194) and 74.29% (26/35)], respectively. Patients with a family history accounted for 23.14% (53/229). The rate of DMD registrants losing walking capability was 17.53% (34/194), and all the BMD registrants were able to walk. Cardiac functions were examined in 46.29% (106/229) DMD/BMD boys and respiratory functions were examined in 17.90% (41/229) DMD/BMD boys. The proportion of DMD patients receiving prednisone with dosage of 0.75 mg/(kg ⋅ d) was 26.29% (51/194). Conclusions This database describes in detail the genotype, clinical manifestation, diagnosis and treatment and rehabilitation status of 229 DMD/BMD patients in China. The database not only provides comprehensive information for DMD/BMD patient management, but also will make significant contribution to the development of clinical trials, prospective therapeutic research and better management of DMD/BMD patients and families in China. [ABSTRACT FROM AUTHOR]

Objective To investigate the morbidity and associated factors of early onset depression after acute ischemic stroke, in order to improve its diagnostic rate and cure rate. Methods The depression symptoms of 150 patients with acute ischemic stroke were evaluated by using Hamilton Depression Rating Scale - 17 (HAMD - 17) 2 weeks after onset. According to Diagnostic and Statistical Manual of Mental Disorders Fourth Edition (DSM - IV) criteria, patients with HAMD - 17 score ⩾ 7 were diagnosed as post-stroke depression (PSD). Clinical data of those patients, including gender, age, education, laboratory indexes, predisposing factors, National Institute of Health Stroke Scale (NIHSS) score, Trial of Org10172 in Acute Stroke Treatment (TOAST) type, Oxfordshire Community Stroke Project (OCSP) classification, and concurrent carotid artery stenosis were recorded. Univariate and multivariate Logistic regression analysis was used to investigate the related factors of PSD. Results The morbidity of PSD in patients 2 weeks after stroke onset was 18% (27/150). Univariate and multivariate Logistic regression analysis showed triglyceride level (P = 0.042), neural function deficiency (P = 0.001) and concurrent carotid artery stenosis (P = 0.003) were independent risk factors for early onset PSD. Further subgroup analysis indicated concurrent carotid artery stenosis was the independent risk factor for PSD in non-minor stroke patients (P = 0.014). Conclusions Stroke patients with severe neurological deficits and carotid artery stenosis are susceptible to early onset PSD. [ABSTRACT FROM AUTHOR]

Objective To discuss the clinical significance of elevated serum citrulline levels in patients with epilepsy, and to provide new idea for improving the curative effect of antiepileptic drugs (AEDs). Methods Select 159 child and adolescent patients with cryptogenic epilepsy but without brain structural damage, screen serum concentration of citrulline in these patients by liquid chromatography - tandem mass spectrometry (LC-MS/MS), and perform citrulline metabolism-related gene testing, including SLC25A13 gene and ASS1 gene, in part of those patients with a higher citrulline level, in order to identify the etiology of epilepsy. Results Among 159 patients, the citrulline levels of 18 cases were searched for 1.53-3.61 times higher than normal, and no causative mutation or novel mutation of SLC25A13 gene and ASS1 gene was found in 6 cases who were performed gene testing. AEDs were given to those 18 patients for at least one year. Seven cases (7/18) were free of clinical seizures, and 3 cases (3/18) had at least 75% reduction in seizure frequency, 3 cases (3/18) 50%-75% reduction in seizure frequency, 5 cases (5/18) seizure reduction below 50% . Compared with 30 cases with normal citrulline level (control group), the efficacy of AEDs in those 18 cases were significantly lower (Z = 1.759, P = 0.039). There was no significant difference between these 2 groups in basic features including sex, onset age, duration of seizure, seizure type, seizure frequency and kind of AEDs. Conclusions Higher citrulline level (1.53-3.61 times) may lead to a negative effect on the efficacy of AEDs of child and adolescent patients with cryptogenic epilepsy in this study. [ABSTRACT FROM AUTHOR]

Objective To study the medical care demands and utilizations in the old people left behind in rural in a county of Kunming. Methods By using random cluster sampling method, old people left behind in 10 nature villages of 5 towns in a county of Kunming were interviewed at their home with questionnaires, to learn the medical care demands and utilizations . Results The prevalence rate within two weeks of the Old People Left Behind in Rural was31.44%. The major influencing factor was age. The prevalence rate of chronic non-infectious diseases was 67.42%. The influencing factors were age and matrimony. The visiting rate with in two weeks of the investigated dwellers was 21.56%. The main reason of not visiting was economic difficulty. Conclusion The demands of old people left behind in rural for health service are high, and health service utilization is low. Special emphasis should be put on chronic disease prevention, and the rural medical security system should be improved. [ABSTRACT FROM AUTHOR]

Objective To investigate the correlation between knee osteoarthritis and osteoporosis in postmenopausal women. Methods A total of 134 postmenopausal women with knee osteoarthritis were selected. The age, weight, height, and bone mineral density (BMD) of all the patients were detected, and the body mass index (BMI) was calculated. X-ray filming of both knee joints with loading was performed. According to the Kellgren standard of the knee osteoarthritis, patients were divided into 4 degrees. And according to the clinical symptom and X-ray, patients with the 4 degree knee osteoarthritis were divided into mild group and severe group. The correlation between the knee osteoarthritis and the concurrent osteoporosis was analyzed. Results In postmenopausal women, BMD was negatively correlated with age, but positively correlated with body weight, height, and BMI. No significant difference of height, weight, and BMI between mild group and severe group was observed (P > 0. 05), but the difference of age and BMD of the femoral head was significant between 2 groups (P < 0. 01). Linear regression analysis showed that age had a great effect on BMD of the femoral neck. When ruling out the confounding factor of age, a significant difference of BMD of the femoral neck was still observed between 2 groups (P < 0. 05 ). Conclusion Osteoporosis is closely correlated with the severity of the knee osteoarthritis in postmenopausal women. The severer the knee osteoarthritis is, the lower BMD is. Osteoarthritis is probably one of the risk factors for the occurrence or the aggravation of postmenopausal osteoporosis. [ABSTRACT FROM AUTHOR]

The purpose of this article was to review the risk factors related to falling among the homedwelling elderly in Taiwan. The literature search included seven medical databases in either Chinese or English, including the Periopath index to Taiwan's periodic literature. Thirty studies that met the inclusion criteria for this study were reviewed. The results showed the risk factors related to falling included: (1) Biological factors: poor self-reported health, multiple signs and symptoms of disease, a number of diseases, poor physical function, unstable of gait, poor balance, stool or urinary incontinence, dysfunction in ADLs or IADLs, higher waistline, lower albumin, depression, fear of falling, and a history of falling. (2) Behavioral factors: lack of knowledge about ways to prevent falling and taking multiple medications. (3) Environmental factors: poor supportive environment, poor location of bedrooms and the safety of bedroom bathrooms and stairs, accumulation of debris, smooth ground, and dim lighting. Only a few studies found that social and economic factors, including marital status, level of education, and living alone were significantly associated with falling. The results may serve a reference for preventing falling among the home-dwelling elderly. [ABSTRACT FROM AUTHOR]

PURPOSE: To investigate the role of repairing tissue defects by infrahyoid myocutaneous flap after oral cancer surgery in elder patients. METHODS: From December 2005 to November 2010, we used infrahyoid myocutaneous flaps to reconstruct soft tissue defects in 19 elder patients after radical operation of oral cancer. There were 11 males and 8 females with the age range of 65 to 89 years (average: 72.8). The lesions included 10 tongue cancers, 2 buccal cancers, 6 mouth floor cancers and 1 gingival cancer. RESULTS: All wound healed uneventfully, and all the 19 infrahyoid myocutaneous flaps survived without total or partial necrosis. The results were satisfactory both in appearance and functions. The patients were followed up for 6 months to 5 years, no tumour relapse and metastasis occurred. CONCLUSIONS: The procedure of preparing the infrahyoid myocutaneous flap is simple and the result is reliable. The flap is useful for reconstruction of soft tissue defects in elder patients. [ABSTRACT FROM AUTHOR]

Objective To explore the efficacy of logistic regression modeling based on plasma amino acid profile and patient age, for diagno-sing hepatic fibrosis in patients with chronic hepatitis B ( CHB ). Methods One - hundred - ad - forty - eight patients ( 108 males ; mean age : 38. 1 ±11.9 years, range : 16-72 years) histological diagnosed with CHB at our institute between January 2009 ad June 2011 were en-rolled for study. Pathological staging of fibrosis classified patients as : significant ( = S2 ), severe ( = S3 ), or advanced ( S4 ) stages. Plasma molar concentrations of amino acids were determined by high - performance liquid chromatography, and used to establish the top six fractional ratio models according to progressive significance of correlations with pathological stages (by Spearman's rank correlation coefficient analysis). The abilities of scale models to predict fibrosis were evaluated using the area under the receiver operating characteristic ( AUROC) curve. ROC curve analysis allowed for the best cutoff for age to be determined for the pathological stages and calculation of sensitivity, specificity, and ac-curacy for the corresponding prediction probabilities (PP). Results Patient age was postively correlated with pathological stage (rs =0.262, P = 0. 001 ) . The significant correlation coefficients for the six models were ( in descending order) : ( isoleucine + leucine)/tyrosine ( rs = -0. 3S0, P =0.000), leucine/tyrosine ( rs = - 0.349, P= 0.000), ( isoleucine + leucine + valine)/tyrosine ( rs = - 0. 34S, P = 0.000), ( leucine + valine )/tyrosine ( rs = -0.342, P=0.000), (isoleucine+vaine)/tyrosine (rs = -0.336, P=0.000), valine/ tyrosine (rs = -0. 311, P =0.000). The logistic regression model based on age and the ratio models of plasma amino acids could not be established. For severe fibrosis, the PP was 0.722 (9S% CI: 0.640 -0.804), with sensitivity of 0.49, specificity of 0. 8S, ad accuracy of 0. 69. For advanced fibrosis, the PP was 0. 792 (9S% CI: 0.708 -0.876), with sensitivity of 0.73, specificity of 0.76, and accuracy of 0.7S. Conclusion Logistic regression modeling based on plasma amino acid profile and patient age could have clinical utility for sensitive, specific and accurate diagnosis of severe and advanced fibrosis in CHB patients. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the association of Body Mass Index (BMI) and waist circumference (WC) with the risk for diabetes, and to examine the ability of these measures to predict new cases of diabetes in ⩾50-year old Taiwanese. Methods: We utilized the 2003 and 2007 "Survey of Health and Living Status of the Elderly in Taiwan" datasets. We analyzed the cross-sectional associations in 4987 subjects (⩾50 years old) and the ability of BMI and WC to predict new cases of diabetes in these subjects four years later. We also analyzed the cutoffs with Receiver Operating Characteristics (ROC). Results: Both indicators were associated with a risk for diabetes but WC showed a stronger association. Both indicators significantly predicted the risk for new diabetes, but WC was a stronger predictor. For each 1 cm increment in WC, there was a 6% increase in the risk for new diabetes in men (p=0.001) and a 4% increase in women (p=0.005). The cutoffs for the risk of new onset diabetes differed by age and gender. The BMI cutoff was reduced from 24.52 to 21.82 kg/m2 for men and 24.27 to 22.88 kg/m2 for women, whereas the WC cutoff was increased from 84.5 to 89.5 cm for men and 82.5 to 91.5 cm for women. Conclusions: Both BMI and WC were significantly associated with the risk for diabetes or the risk for new diabetes, but the association was stronger with WC for both. The predicted cutoffs for diabetes varied according to age and gender and were different from the current Bureau of Health Promotion recommendations. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to investigate knowledge of cervical cancer and HPV in adult men and their acceptance of vaccination for HPV. Methods: A convenience sample of 229 men between 19 and 39 years of age, responded to self-report questionnaires on knowledge of cervical cancer and HPV, vaccination intention, and general and sexual characteristics. Data were collected during September and October, 2010 and analyzed using t-test, χ2-test, and ANOVA. Results: Mean scores for knowledge of cervical cancer and HPV were 4.88, and 7.87 respectively. Seventy-five percent stated they intended to be vaccinated. There were significant differences in knowledge of cervical cancer depending on education (F=3.04, p=.030), sexual frequency (F=4.62, p=.011), condom use (t=2.19, p=.029), and frequency of condom use (F=3.28, p=.040), and in knowledge of HPV depending on sexually activity (t=2.35, p=.020), condom use (t=0.03, p=.019), and frequency of condom use (F=4.65, p=.011). There were significant differences in vaccination intention depending on education (χ2=10.47, p=.015), but sexual characteristics were not significant for vaccination intentions. Conclusion: The results indicate a need to consider levels of education and knowledge of cervical cancer and HPV in target populations of men, when planning a cervical cancer prevention education program for men. [ABSTRACT FROM AUTHOR]

Objective To investigate the status of osteoporosis in healthy population, to analyze the causes of osteoporosis and the related protective measures, and to provide basis for the prevention and treatment of osteoporosis. Methods A total of 2093 patients, who received physical examination in the Department of Medical Care in Tianyou Hospital Affiliated to Wuhan University of Science and Technology from March 2011 to February 2012, were selected. The bone ultrasound detection of the left distal radius was performed. The results were analyzed statistically. Results Among all the healthy subjects, 613 cases had osteopenia (29.29%), including 253 men (23.94%) and 360 women (34.75%). Meanwhile, 521 cases had osteoporosis (24.89%), including 155 men (14.66%) and 366 women (35.33%). The significant difference of the incidence of osteoporosis among different age groups, as well as between males and females, was observed (P < 0. 05 ). Conclusion The incidence of osteoporosis in healthy population is pretty high, showing a positive correlation with age. And the incidence in women is significantly higher than that in men. We should strengthen the prevention and early treatment of osteoporosis. [ABSTRACT FROM AUTHOR]

Objective By changing the age of tested subjects, to understand the T score and Z score along with the change of age, and to investigate this effect on quantitative measurement of bone mineral density (BMD). Methods A total of 155 patients, who received CT quantitative measurement of BMD from August 2012 to December 2012, were selected. According to different age groups, the analysis of the change in bone mass was performed. Twenty female patients were extracted from patients between 30 and 40 years old. By manually changing their ages, the changes of T and Z score were analyzed. Results According to the different definition of T and Z score, T score was closely related to BMD, showing no change along with age. Z score was the comparison with population at the same age. The change of Z score was obvious at the same BMD. Conclusion In CT quantitative measurement of BMD, age is one of the important factors to affect the evaluation results. The usage of Z score in the evaluation of BMD and the diagnosis of osteoporosis is more valuable. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumor is often considered to be a mesenchymal tumor. We reported a case of atypical SFT of the buccal mucosa in a 56-year-old male patient. We found hypercellularity, nuclear atypia, increased number of mitotic figures(more than 4 per 10 high-power fields) microscopically. A long-term follow-up is necessary after complete excision. [ABSTRACT FROM AUTHOR]

PURPOSE: The purpose of this study is to investigate the relation between age, gender and thickening and nodules in port wine stains(PWS) patients. METHODS: The gender and age of 193 cases with port wine stains were compared between those with flat, those with thickening, those with thickening and nodules, in 0-19, 20-year age group. SAS6012 software package was used for Fisher's exact test and Kruskal-Wallis test. RESULTS: There were 165 cases with flat (85.49%), 10 cases with thickening (5.18%), 18 cases with thickening and nodules (9.33%). Kruskal-Wallis test demonstrated significant difference between 2 age groups (P<0.01). There was an increase incidence of thickening, and thickening and nodule formation in 0-19, 20-year age group (P<0.01), but there was no gender difference between thickening and nodules. The data were analyzed by SAS6.12 Fisher's exact test and Kruskal-Wallis test. CONCLUSION: The incidence of thickening and nodule formation in PWS increase with age. [ABSTRACT FROM AUTHOR]