Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation syndrome (ROHHADS) is a rare multi-system disease, and delayed diagnosis and treatment may lead to catastrophic cardiopulmonary complications. As far as we know, no patient with ROHHADS has been reported in China, and this article reports a child with ROHHADS to improve the awareness of this disease among clinicians. A girl, aged 3 years, had the clinical manifestations of rapid weight gain, fever, disturbance of consciousness, and convulsion. The physical examination showed a body weight of 20 kg, somnolence, irregular breathing, and stiff neck. She had increased blood levels of prolactin and follicle-stimulating hormone and hyponatremia. The lumbar puncture showed an increased intracranial pressure. The brain MRI and magnetic resonance venography showed symmetrical lesions in the periventricular region and venous thrombosis in the right transverse sinus and the superior sagittal sinus. The sleep monitoring showed hypopnea. The girl was finally diagnosed with ROHHADS and intracranial venous thrombosis. She recovered after symptomatic treatment including decreasing intracranial pressure, anticoagulation, and respiratory support. The possibility of ROHHADS should be considered for patients with unexplained obesity, fever, and hypoventilation, with or without central nervous system symptoms. Early diagnosis and standardized follow-up can improve the prognosis of children with ROHHADS.
Child ; Child, Preschool ; China ; Consciousness ; Female ; Humans ; Hypothalamic Diseases ; Hypoventilation ; Obesity

BACKGROUND: Anaplastic lymphoma kinase (ALK) rearrangement is a common driver gene of non-small cell lung cancer (NSCLC). Ceritinib is a second-generation ALK inhibitor, which can bring survival benefits to ALK-positive metastatic NSCLC. However, few studies focus on the safety and efficacy of ceritinib in China. Therefore, this study intends to investigate the safety and preliminary efficacy of ceritinib 450 mg with meals in Chinese patients with ALK-positive NSCLC through a real world study. METHODS: From October 2018 to December 2019, patients with ALK-positive NSCLC from 8 medical centers in Sichuan province were recruited in this study. All of these participants received ceritinib 450 mg/d with food. The basic characteristics, adverse effects (AEs) and responses were collected and analyzed in order to evaluate the safety and efficacy of ceritinib. RESULTS: A total of 109 patients were included in this study. Data cutoff was January 23, 2020. The median duration of treatment exposure was 5.87 mon (range: 0.4 mon-15.7 mon). Total AEs were reported in 98 (89.9%) of 109 patients and grade 3 or 4 AEs were reported in 22.9% of patients. Most common AEs (mainly grade 1 or 2) were diarrhea (60.6%), elevated alanine aminotransferase (ALT)(38.5%) and aspartate aminotransferase (AST)(37.6%). As of data cutoff, 45 patients discontinued ceritinib. The overall response rate (ORR) was 37.6% (95%CI: 28.5%-47.4%) and disease control rate (DCR) was 86.2% (95%CI: 78.3%-92.1%). CONCLUSIONS The treatment of ceritinib 450 mg with food for Chinese ALK-positive NSCLC patients had a good safety profile and favorable DCR in real-world setting. However, this conclusion needs to be further verified by large sample, prospective trials.

ObjectiveTo investigate the effect of Qilan Capsules (QLC) on the expressions of the related proteins HIF-1α, VEGF-α, EphA2 and MMP-1 in the formation of vasculogenic mimicry (VM) in prostate cancer.

METHODSProstate cancer PC-3 cells were cultured, transfected with siRNA, and divided into eight groups, blank control, HIF-1α siRNA, VEGF-α siRNA, EphA2 siRNA, QLC intervention, QLC + HIF-1α siRNA, QLC + VEGF-α siRNA, and QLC + EphA2 siRNA. The expressions of the HIF-1α, VEGF-α and EphA2 proteins in the pathway of VEGF were determined by Western blot.

RESULTSCompared with the blank control group, the expression of HIF-1α was evidently decreased in the HIF-lα siRNA and QLC + HIF-lα siRNA groups (0.624 7 ± 0.042 8 vs 0.032 8 ± 0.002 5 and 0.036 8 ± 0.018 1, P < 0.05), so were that of VEGF-α in the VEGF-α siRNA and QLC + VEGF-α siRNA groups (0.068 9 ± 0.005 1 vs 0.016 9 ± 0.000 7 and 0.010 9 ± 0.000 8, P < 0.05), that of EphA2 in the EphA2 siRNA and QLC + EphA2 siRNA groups though with no statistically significant difference (0.1684 ± 0.0126 vs 0.134 5 ± 0.028 6 and 0.165 4 ± 0.039 8, P > 0.05), and that of MMP-1 in the HIF-lα siRNA, VEGF-α siRNA and EphA2 siRNA groups (1.696 1 ± 0.152 7 vs 0.435 9 ± 0.036 9, 0.198 7 ± 0.009 0 and 0.0218 ± 0.000 7, P < 0.05).

CONCLUSIONSQilan Capsules can suppress VM formation in prostate cancer by inhibiting the expressions of HIF-1α, VEGF-α and MMP-1, which plays a role in the clinical treatment of prostate cancer by checking the growth and development of the blood supply system in the tumor tissue.

Capsules ; Drugs, Chinese Herbal ; pharmacology ; Humans ; Hypoxia-Inducible Factor 1, alpha Subunit ; metabolism ; Male ; Matrix Metalloproteinase 1 ; metabolism ; Molecular Mimicry ; Prostatic Neoplasms ; metabolism ; RNA, Small Interfering ; metabolism ; Receptor, EphA2 ; metabolism ; Transfection ; Vascular Endothelial Growth Factor A ; metabolism

Objective: To observe the synergistic effect of Qilan Capsules in the treatment of the patient with Qi-deficiency blood-stasis type of prostate cancer receiving androgen-deprivation therapy after castration. METHODS: This randomized controlled double-blind study included 246 cases of Qi-deficiency blood-stasis type of prostate cancer after castration, which were randomly divided into an experiment and a control group of equal number to be treated with Qilan Capsules + androgen-deprivation and placebo + androgen-deprivation, respectively. After 6 months of treatment, we compared the International Prostate Symptoms Scores (IPSS), TCM Symptoms Scores (TCMSS), maximal urine flow rate (Qmax), and the level of serum prostate-specific antigen (PSA) between the two groups of patients. RESULTS: Statistically significant differences were observed between the experiment and control groups in the syndrome classification-based efficacy (87.7% vs 67.9%, P <0.05) and total effectiveness rate (86.0% vs 71.6%, P <0.05). Compared with the baseline, the experiment group showed remarkable improvement after treatment in TCMSS (17.1±5.1 vs 8.3±4.0, P <0.05), IPSS (17.7±7.5 vs 11.4±4.6, P <0.05), and Qmax (［10.9±4.3］ ml/s vs ［14.7±3.7］ ml/s, P <0.05), and so did the control group (16.8±5.2 vs 11.5±5.2, 17.8±6.7 vs 14.6±5.8, and ［11.0±4.3］ ml/s vs ［12.0±4.1］ ml/s, P <0.05). The above three parameters were even more markedly improved in the former than in the latter group (P <0.05). However, there was no statistically significant difference between the two groups in the improvement of the PSA level after treatment (P >0.05). CONCLUSIONS Qilan Capsules can significantly enhance the effect of androgen-deprivation therapy in the treatment of Qi-deficiency blood-stasis type of prostate cancer after castration though cannot obviously improve the PSA level.
Androgen Antagonists ; therapeutic use ; Capsules ; Double-Blind Method ; Drug Therapy, Combination ; methods ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Male ; Orchiectomy ; Prostate-Specific Antigen ; blood ; Prostatic Neoplasms ; blood ; blood supply ; surgery ; Qi ; Quality of Life ; Treatment Outcome

BACKGROUNDAdvances in transplantation immunology show that the balance between dendritic cells (DCs) and their subsets can maintain stable immune status in the induction of tolerance after transplantation. The aim of this study was to investigate if DCs and DC subpopulations in recipient peripheral blood are effective diagnostic indicators of acute rejection following kidney transplantation.

METHODSImmunofluorescent flow cytometry was used to classify white blood cells (WBCs), the levels of mononuclear cells and DCs (including the dominant subpopulations, plasmacytoid DC (pDC) and myeloid DC (mDC)) in peripheral blood at 0, 1, 7, and 28 days and 1 year after kidney transplantation in 33 patients. In addition, the blood levels of interleukin-10 (IL-10) and IL-12 were monitored before and after surgery. Fifteen healthy volunteers served as normal controls. Patients were undertaking hemodialysis owing to uremia before surgery.

RESULTSThe total number of DCs, pDC, and mDC in peripheral blood and the pDC/mDC ratio were significantly lower in patients than controls (P < 0.05). Peripheral DCs suddenly decreased at the end of day 1, then gradually increased through day 28 but remained below normal levels. After 1 year, levels were higher than before surgery but lower than normal. The mDC levels were higher in patients with acute rejection before and 1 day after surgery (P < 0.005). There was no significant difference in IL-10 and IL-12 levels between patients with and without acute rejection.

CONCLUSIONThe changes in DCs and DC subpopulations during the acute rejection period may serve as effective markers and referral indices for monitoring the immune state, and predicting rejection and reasonably adjusting immunosuppressants.

Adolescent ; Adult ; Dendritic Cells ; immunology ; Graft Rejection ; immunology ; Humans ; Kidney Transplantation ; adverse effects ; Middle Aged ; Myeloid Cells ; immunology ; Young Adult

BACKGROUNDPlasma galectin-3, a mediator of fibrogenesis and inflammation, its potential to associate with type 2 diabetes (T2DM) is poorly investigated. Here, we explored its interaction with the serum galectin-3 and vascular complications.

METHODSWe conducted a population-based cross-sectional survey in Zhejiang, China involving 165 men and 119 women (age range, 43 - 84 years), investigating the relationship between serum galectin-3 and vascular disease in patients with T2DM.

RESULTSSerum galectin-3 was higher in subjects with T2DM than that in control participants (27.4 vs. 17.6 ng/ml, P < 0.001). Compared with subjects with galectin-3 values in the lowest quartile, those with values in the highest quartile had an increased likelihood of vascular complications (4th quartile odds ratio (OR) 2.52, 95% confidence interval (CI), 1.25 - 4.07). Increased risk of micro- or macrovascular complications correlated with serum galectin-3 concentration (ORs 11.4 and 8.5, respectively). An increased number of vascular complications was associated with high serum galectin-3 levels (P < 0.05). Patients with serum galectin-3 levels > 25 ng/ml had an elevated risk of diabetes relative to patients with levels < 10 ng/ml (OR for any vascular complication 2.64, for heart failure 3.97, for nephropathy 4.09, for peripheral arterial disease (PAD) 4.18; all P < 0.05). Complication risk was higher in patients with neurogenic, stroke, or retinopathy complications, but this difference was not significant after risk factor adjustment. Serum galectin-3 levels correlated with diabetes duration, C-reactive protein (CRP) levels, and albuminuria.

CONCLUSIONHigh galectin-3 values were associated with increased odds of developing heart failure, nephropathy, and peripheral arterial disease in patients with T2DM.

Adult ; Aged ; Aged, 80 and over ; C-Reactive Protein ; analysis ; Cross-Sectional Studies ; Diabetes Mellitus, Type 2 ; complications ; Diabetic Angiopathies ; blood ; etiology ; Female ; Galectin 3 ; blood ; Humans ; Male ; Middle Aged ; Risk Factors

Head ; surgery ; Humans ; Neck ; surgery ; United States

OBJECTIVETo explore the clinical significance of genetic detection and changes of red cell enzyme activities of pyrimidine 5' nucleotidase (P5'N), pyruvate kinase (PK) and glucose-6-phosphate dehydrogenase (G-6-PD) in patients with α-thalassaemia (α-thal).

METHODSThree α-thal patients were further processed to gene detection by PCR-trans-dot blot and gap-PCR, and red cell enzymes activities by absorbance at 260 and 280 nm (A) for P5'N and fluorescence spot test for PK and G-6-PD.

RESULTSRed cells in 3 α-thal cases were microcytic hypochromic with obvious augmented target cells and basophilic stippling erythrocytes. Two patients had anemia, splenomegaly, hyperbilirubinemia and augmented LDH. HbH was positively identified by hemoglobin electrophoresis and hemoglobin cellulose acetate membrane electrophoresis; the other patient had no such abnormalities. Genotypes of 3 patients were of (-α(3.7)/--(SEA)), (αα(QS)/--(SEA))and (--(SEA)), respectively. The activity of P5'N (but not for PK and G-6-PD) in red cell reduced.

CONCLUSIONSThis is the first documented α-thal with P5'N deficiency. Genetic detection might be clinical significant for the diagnosis and pedigree screening of α-thal.

5'-Nucleotidase ; deficiency ; Adolescent ; Adult ; Erythrocytes ; enzymology ; metabolism ; Female ; Humans ; Male ; Middle Aged ; alpha-Thalassemia ; enzymology ; genetics

BACKGROUNDSome studies found that cholinesterase (ChE) can be an independent risk factor for patients with multiple organ dysfunction syndrome. To assess aged patients with systemic inflammatory response syndrome (SIRS) early and predict their prognosis, the predictive value of ChE for the prognosis of aged patients with SIRS was analyzed.

METHODSFrom September 2009 to September 2010, all aged patients with SIRS in the ICU of the Second Affiliated Hospital of Zhejiang University School of Medicine were retrospectively analyzed if they met inclusion criteria: patients aged ≥ 65 years and met American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria for SIRS. Serum ChE, albumin, D-dimer, lactic acid and C-reactive protein (CRP) were measured, and the Acute Physiology and Chronic Health Evaluation (APACHE) II and Glasgow Coma Scale (GCS) scores were evaluated within the first 24 hours in the ICU. Fisher's exact test was used for comparison of the primary disease between the deceased group and surviving group. For comparison of study variables between the two groups, the Student's t test or Mann-Whitney U test was used. Multivariate significance was tested with binary Logistic regression analysis.

RESULTSThe clinical data of 124 aged patients with SIRS were collected and analyzed. Sixty-six patients (46 male, 20 female, mean age (78.70 ± 8.08) years) who died were included in the deceased group and 58 patients (34 male, 24 female, mean age (76.02 ± 6.57) years) who survived were included in the surviving group. There were no significant differences in age, gender, APACHE II score and GCS score between the deceased group and surviving group (all P > 0.05), but there were significant differences in lactic acid (P = 0.011), D-dimer (P = 0.011), albumin (P = 0.007), CRP (P = 0.008), and ChE (P < 0.0001). The correlation analysis showed that the APACHE II score and CRP were not correlated with ChE (both P < 0.05). D-dimer and albumin were correlated with ChE (Spearman's rho correlation coefficients were -0.206 and 0.324, the corresponding P values were 0.022 and < 0.0001). Multiple Logistic regression analysis showed that age, gender, lactic acid, D-dimer, albumin, CRP, APACHE II score, and GCS score were not independent risk factors for prognosis of aged patients with SIRS, but that ChE was (P < 0.0001). The receiver operating characteristic curve of ChE had an area under the curve of 0.797 (standard error = 0.04; P < 0.0001), and a ChE of 103.00 U/L was the cut-off value with sensitivity = 0.793, specificity = 0.742.

CONCLUSIONSerum ChE might be a predictive marker for the prognosis of aged patients with SIRS, with low serum ChE levels indicating poor prognosis.

Aged ; Aged, 80 and over ; Cholinesterases ; blood ; Female ; Humans ; Male ; Prognosis ; Systemic Inflammatory Response Syndrome ; blood ; enzymology ; pathology