Objective To explore the clinical characteristics of autoimmune disease with dual seropositive antibodies of leucine-rich glioma inactivated 1(LGI1)and contactin-associated protein 2(Caspr2).Methods The clinical data of seven patients with dual seropositive LGI1 and Caspr2 antibodies who were admitted to the Neurology Department of Peking Union Medical College Hospital from July 2014 to December 2017 were retrospectively analyzed.Results Central,peripheral and autonomic nervous systems were all involved in the seven cases;100%(7/7)presented with insomnia,myokymia,neuropahic pain and hyperhydrosis;71%(5/7)showed memory decline or psychiatric and behavioral symptoms;57%(4/7)had urinary hesitation or constipation;and 43%(3/7)had seizure.Electromyography showed 100%(6/6) of the patients had prolonged afterdischarges following normal M waves and/or abnormal spontaneous firing.Electroencephalography revealed slow waves or basic rhythm slowing in 71%(5/7)of patients.Electrocardiography showed sinus tachycardia,axis deviation,and prolonged QT intervals in 71%(5/7)of patients.One patient died from arrhythmia before immunotherapy.One died from pulmonary infection after immunotherapy.Improvement with immunotherapy was documented in the other five cases.No relapse was noted during the 1-2-year follow-up.Conclusions Autoimmune disease with dual seropositive antibodies of LGI1 and Caspr2 can diffusely affect the central,peripheral,and autonomic nervous systems.The possibility of this disease should be considered in patients with acute and subacute onset of neuropsychiatric symptoms,especially in patients with accompanying insomnia,myokymia,and hyperhydrosis.
Autoantibodies ; blood ; Autoimmune Diseases ; immunology ; Humans ; Membrane Proteins ; immunology ; Nerve Tissue Proteins ; immunology ; Proteins ; immunology ; Retrospective Studies

BACKGROUND: To define the exact boundary of the intrathyroid and extrathyroid aspects of a gland when determining the extent of cancer invasion, we plan to clarify the definition of sizable vascular structures, which is one of the helpful histologic clues in determining a minimal extrathyroid extension. We hypothesized that arterial wall thicknesses in extrathyroid soft tissue would be significantly different from the arteries in the thyroid parenchyma. METHODS: Twenty cases of papillary carcinoma were selected. The numbers and wall thicknesses of the arteries and arterioles in intrathyroid and extrathyroid tissue were evaluated. The absence of nerve tissue in the thyroid gland was confirmed using the S-100 protein immunohistochemical stain. RESULTS: The comparison of the mean thicknesses of the total arteries between the extrathyroid and intrathyroid tissues in the retrospective study (26.88 micrometer vs. 15.07 micrometer, respectively) and the prospective study (35.24 micrometer vs. 16.52 micrometer, respectively) revealed significant differences (p=0.000). The greatest thickness of the intrathyroid arteries was 67.93 micrometer. CONCLUSIONS: According to our results, the study showed that the extrathyroidal arteries were significantly thicker than the intrathyroidal arteries. We suggest that the sizable blood vessels of extrathyroidal arteries should be greater than 67.93 micrometer in thickness.
Arteries ; Arterioles ; Blood Vessels ; Carcinoma, Papillary ; Nerve Tissue ; Prospective Studies ; Retrospective Studies ; S100 Proteins ; Thyroid Gland

Female ; Gestational Age ; Globins ; Humans ; Infant, Newborn ; Infant, Premature ; Male ; Nerve Tissue Proteins ; blood

Animals ; Diastole ; Heart Failure ; blood ; Humans ; Myocardial Infarction ; blood ; Natriuretic Peptide, Brain ; blood ; Nerve Tissue Proteins ; blood ; Peptide Fragments ; blood ; Prognosis ; Systole

Allgrove syndrome is a rare autosomal recessive disorder. It is also known as the 3A syndrome and characterised by the triad of achalasia, alacrima and adrenal insufficiency. The AAAS gene is encoded on chromosome 12q13. We report the case of a 23-year-old woman who presented at the hospital with adrenal crisis that was triggered by infection of the urinary system and gastrointestinal bleeding. She had a known diagnosis of achalasia for eight years, and ophthalmologic examination revealed alacrima. Based on our findings, the patient was diagnosed with Allgrove syndrome.
Adrenal Insufficiency ; blood ; diagnosis ; genetics ; Adrenocorticotropic Hormone ; blood ; Diagnosis, Differential ; Diagnostic Techniques, Ophthalmological ; Endoscopy, Gastrointestinal ; Esophageal Achalasia ; blood ; diagnosis ; genetics ; Female ; Humans ; Mutation ; Nerve Tissue Proteins ; blood ; genetics ; Nuclear Pore Complex Proteins ; blood ; genetics ; Young Adult

The aim of this study was to detect the nerve growth factor (NGF) level in serum and NGF low affinity acceptor CD271 expression on bone marrow leukemic cells in acute B lymphoid leukemia (B-ALL) patients and to analyze their clinical significance. The NGF level in serum and CD271 expression on leukemic cells in bone marrow were detected by enzyme linked immunosorbent assay and flow cytometry in B-ALL patients respectively. The results indicated that compared with control group, the NGF level in serum of patient group significantly increased (t = 4.191, p < 0.05), but CD271 expression on leukemic cells in bone marrow decreased significantly (t = 4.898, p < 0.05). The complete remission (CR) rate of 25 B-ALL patients was 64% (16/25) after one course of CVAD chemotherapy. There were statistically significant differences of NGF level and CD271 expression in non-remission (NR) group and control group (t = 3.976, p < 0.05 vs t = 5.052, p < 0.05), but there were no statistically difference of NGF level and CD271 expression in CR group (t = 1.102, p > 0.05 vs t = 1.150, p > 0.05) as compared with control group. The CD271 expression before and after chemotherapy between CR and NR groups showed statistically significant differences (t = 3.889, p < 0.05; t = 3.751, p < 0.05 and t = 4.678, p < 0.05 respectively), but NGF level before and after chemotherapy showed no statistical difference between these 2 groups (t = 0.476, p > 0.05). 50% (8/16) patients relapsed during following up, and of their NGF level [(168.00 ± 61.66) pg/ml] and CD271 expression [(52.29 ± 13.00)%] showed the significantly differences, compared with those in control group (t = 5.284, p < 0.05 vs. t = 6.073, p < 0.05), but the NGF level [(81.13 ± 25.32) pg/ml] and CD271 expression [(78.45 ± 7.12)%] of other 8 patients showed no statistical difference as compared with control group (t = 1.228, p > 0.05 vs t = 1.144, p > 0.05). Compared with low NGF level and CD271 low expression groups, the survival time of B-ALL patients with high NGF level and CD271 expression was not changed significantly (p = 0.750 vs p = 0.170). It is concluded that the increased NGF level in serum and decreased CD271 expression on bone marrow leukemic cells in B-ALL patients are related with leukemia development and may be the useful indexes to evaluate curative effect and prognosis.
Adolescent ; Adult ; Aged ; Bone Marrow Cells ; metabolism ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Nerve Growth Factor ; blood ; Nerve Tissue Proteins ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; blood ; metabolism ; Receptors, Nerve Growth Factor ; metabolism ; Young Adult

Vitamin D insufficiency may be associated with cardiovascular (CV) mortality in HD patients. To test this hypothesis, we cross-sectionally measured 25-hydroxyvitamin D (25D), 1,25-dihydroxyvitamin D (1,25D), cardiac troponin T (cTnT), and N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) in chronic HD patients. Sixty-five patients (M:F=31:34, age 52.2+/-13.2 yr, DM 41.5%) were selected. Along with the expected low levels of 1,25D, 59 (90.8%) patients had 25D insufficiency (<30 ng/mL) among whom 15 (23.1%) were 25D deficient (<10 ng/mL). The 25D levels showed a negative correlation with cTnT levels (Spearman's rho=-0.44, p<0.01) but not with NT-pro-BNP levels (Spearman's rho=-0.17, p=0.17). The 1,25D levels, however, did not show any relationship with either cTnT or NT-pro-BNP. In multivariate analysis, being male and having low levels of 25D were independent risk factors associated with cTnT elevation (beta=0.44, p<0.01 and beta=-0.48, p<0.01, respectively). In conclusion, not only 1,25D but also 25D are commonly decreased in HD patients. Lower 25D levels appear to be associated with cTnT elevation, predicting worse CV outcome, and are possible to involve cardiac hypertrophy or coronary artery disease.
Biological Markers/*blood ; Coronary Artery Disease/blood/diagnosis ; Electrocardiography/methods ; Female ; Heart Diseases/*blood/diagnosis ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Nerve Tissue Proteins/blood ; Protein Precursors/blood ; Renal Dialysis/*methods ; Risk Factors ; Troponin T/blood ; Vitamin D/*blood/metabolism

OBJECTIVE: To observe the expression differences of the plasma miRNA-1, miRNA-21 between patients with coronary heart disease (CHD) and without coronary artery lesions, between patients with in-stent restenosis (ISR) and none in-stent restenosis (NISR), and to study their predictive value for ISR occurred after percutaneous coronary intervention (PCI) in patients with CHD and diabetes mellitus (DM). METHODS: The selected subjects were divided into CHD group in which patients were implemented stenting (=187), and control group in which patients were without coronary artery lesions (=195). According to the guidelines, the control group was divided into normal group (=150), simple-DM group (=45); the CHD group was divided into simple-CHD group (=119) and CHD-DM group (=68), the CHD group was also divided into ISR group (=48), NISR group (=139), and the ISR group was divided into simple-ISR group (=26) and ISR-DM group (=22) again. Plasma was collected from each group, and total RNA was extracted, the level of blood miRNA-1, miRNA-21 of each group was detected, and their level differences were analyzed. RESULTS: Compared with control group, the level of miRNA-1 and miRNA-21 of CHD group was increased (<0.05); compared with NISR group, the level of miRNA-1 and miRNA-21 of ISR group was increased (<0.05). The incidence of ISR of CHD-DM group was obviously higher than that of simple-CHD group, ISR-DM group's level of miRNA-21 was higher than that of simple-ISR group (<0.05), and there was no difference of miRNA-1 level between ISR and ISR-DM group (<0.05). In Logistics, for CHD patents, the OR of DM, miRNA-1, miRNA-21 were 2.132, 3.066, 1.924 respectively (<0.05); for CHD patents with ISR, the OR of DM, miRNA-21 were 2.123, 3.066 respectively (<0.05); especially for CHD and DM patents with ISR, the OR of miRNA-21 was 9.148 (<0.05). In ROC curve, for CHD patients with ISR, the AUC of miRNA-1, miRNA-21 were 0.854, 0.857 respectively; for CHD-DM patients with ISR, the AUC of miRNA-21 was 0.783. CONCLUSIONS To predict the occurrence of ISR for CHD patients, the plasma miRNA-1 and miRNA-21 have a relatively high specificity and sensitivity, for CHD patients with DM, miRNA-21 may have a higher clinical value.
Coronary Angiography ; Coronary Restenosis ; etiology ; surgery ; Diabetes Complications ; Diabetes Mellitus ; Humans ; MicroRNAs ; blood ; Nerve Tissue Proteins ; blood ; Percutaneous Coronary Intervention ; Stents

OBJECTIVETo investigate the new biomarkers of acute kidney injury, as well as to confirm the values of response gene to complement 32 (RGC-32) for early diagnosis of acute kidney injury by comparing the values of serum creatinine (Scr) and cystatin C (CysC) in children who had undergone cardiopulmonary bypass (CPB).

METHODSixty-seven patients who had accepted CPB were recruited from the cardiac surgery intensive care unit, Children's Hospital Affiliated to Shanghai Jiao Tong University from March to June 2013 and assigned to acute kidney injury group (group AKI) or non-acute kidney injury group (group non-AKI), on the basis of the definition by the pediatric RIFLE (pRIFLE) criteria. Also 30 healthy control children were recruited. Serum samples were taken regularly from each patient after CPB at 30 min, 2 h, 4 h, 24 h, 48 h and 72 h for RGC-32. Serum samples were tested by enzyme linked immunosorbent assay (ELISA) which was employed to determine the levels of serum RGC-32. Scr and CysC were analyzed by HITACHI 7180 automatic biochemical analyzer. All the data were analyzed by receiver operator characteristic curve (ROC) and area under curve (AUC).

RESULTThe incidence of AKI was 34% (23/67), including 15 cases with risk stage AKI, 4 cases with injury stage AKI, 3 cases with failure stage AKI, 1 cases with loss stage AKI. Three out of four subjects with Failure stage AKI and the one case with Loss stage all accepted renal replacement therapy. CPB group had a higher level of serum RGC-32 than that of pre-operation after CPB 30 minute [(2.88 ± 0.68) µg/L vs. (1.39 ± 0.31) µg/L, P < 0.05]. At the same time, comparing with the non-AKI group, the levels of serum RGC-32 were higher than that of controls 30 min, 2 h, 4 h, 24 h and 48 h after CPB (t = 2.560, 2.180, 2.818, 2.226, 3.017; P < 0.05). The values for the AUC were determined for RGC-32 as 0.770, 0.707, 0.768, 0.728,0.723 and 0.770 after CPB 30 min, 2 h, 4 h, 24 h, 48 h and 72 h. The values for sensitivity of serum RGC-32 30 min, 2 h and 4 h after CPB was 0.914, 0.824, 0.824 and the values for specificity of serum RGC-32 was 0.619, 0.667, 0.810, respectively. But the values for sensitivity of CysC was 0.625, 0.813, 0.813, and specificity 0.571, 0.619, 0.571, respectively. The values for sensitivity of Scr was 0.625, 0.625, 0.813 and specificity was 0.571, 0.571, 0.524, respectively.

CONCLUSIONThe sensitivity of serum RGC-32 for detecting AKI was much higher than that of Scr and serum CysC in children who had accepted CPB, and that RGC-32 may be a new biomarker for early detection of AKI. However, the conclusion needs to be further elucidated.

Acute Kidney Injury ; blood ; diagnosis ; etiology ; Area Under Curve ; Biomarkers ; blood ; Cardiopulmonary Bypass ; adverse effects ; Case-Control Studies ; Cell Cycle Proteins ; blood ; Creatinine ; blood ; Cystatin C ; blood ; Female ; Heart Defects, Congenital ; surgery ; Humans ; Infant ; Intensive Care Units, Pediatric ; Male ; Muscle Proteins ; blood ; Nerve Tissue Proteins ; blood ; Postoperative Complications ; Predictive Value of Tests ; Prospective Studies ; ROC Curve ; Sensitivity and Specificity

Cancer stem cells have stem cell-like features, such as the ability for self-renewal and differentiation but show unlimited growth because they have the lost normal regulation of cell growth. Cancer stem cells and normal stem cells have similar features. They show high motility, diversity of progeny, robust proliferative potential, association with blood vessels, immature expression profiles, nestin expression, epidermal growth factor (EGF)-receptor expression, phosphatase and tensin homolog (PTEN) expression, hedgehog pathway activity, telomerase activity, and Wnt pathway activity. On the other hand, with cancer cells, some of these signaling pathways are abnormally modified. In 1875, Cohnheim suggested the concept of cancer stem cells. Recently, evidence for the existence of cancer stem cells was identified. In 1994, the cancer stem cells'specific cell surface marker for leukemia was identified. Since then, other specific cell surface markers for cancer stem cells in solid tumors (e.g. breast and colon cancer) have been identified. In oral cancer, studies on cancer stem cells have been performed mainly with squamous cell carcinomas. Oral cancer specific cell surface markers, which are genes strongly expressed in oral cancer and cancer stem cell specific side populations, have been identified. Cancer stem cells are resistant to radiotherapy and chemotherapy. Therefore, to eliminate malignant tumors efficiently and reduce the recurrence rate, therapy targeting cancer stem cells needs to be performed. Currently, studies targeting the cancer stem cells'specific signaling pathways, telomerase and tumor vasculatures are being done.
Antigens, Surface ; Blood Vessels ; Breast ; Carcinoma, Squamous Cell ; Colon ; Epidermal Growth Factor ; Hand ; Hedgehogs ; Intermediate Filament Proteins ; Leukemia ; Microfilament Proteins ; Mouth Neoplasms ; Neoplastic Stem Cells ; Nerve Tissue Proteins ; Recurrence ; Signal Transduction ; Stem Cells ; Telomerase ; Wnt Signaling Pathway