OBJECTIVEThis study explored the subjective quality of life in children with Tourette syndrome (TS) in order to provide a basis for more effective interference of TS.

METHODSA total of 174 children with TS (≥ 8 years old) and 186 aged-matched healthy children as controls were enrolled. The subjective quality of life was investigated by a case-control study.

RESULTSThe total score of subjective quality of life in the TS group (156.6 ± 21.1) was lower than that in the control group (164.2 ± 21.2; P<0.01). The scores of family life, school life, cognitive component, anxiety experience and depression experience (19.1 ± 3.5 vs 20.7 ± 3.0, 24.1 ± 4.4 vs 26.6 ± 3.2, 90.6 ± 13.3 vs 97.9 ± 15.3, 24.0 ± 4.6 vs 25.1 ± 3.1 and 23.8 ± 4.4 vs 24.7 ± 3.5) in the TS group were lower than those in the control group (P<0.05). The correlation analysis showed that the total score of subjective quality of life in children with TS was negatively related to the age, the course of disease, the severity of symptoms, the total score of child behavior problem and family conflict (r=-0.432, -0.213, -0.869, -0.137, -0.257; P<0.01), while it was positively related to family active-cultural orientation (r=0.084, P<0.01). The multiple step regression analysis indicated that the factors influencing the subjective quality of life in children with TS included the severity of symptoms, age, family conflict and family active-cultural orientation (β'=-0.787, -0.171, -0.109, 0.106; P<0.01).

CONCLUSIONSThe subjective quality of life is not well in children with TS. It is important to control clinical symptoms and improve family environment for the improvement of the subjective quality of life in children with TS.

Adolescent ; Case-Control Studies ; Child ; Family ; Female ; Humans ; Male ; Quality of Life ; Tourette Syndrome ; psychology

OBJECTIVETo summarize the clinical characteristics, laboratory findings and prognosis of patients with Down syndrome-related acute leukemia (DS-AL).

METHODSThe clinical data, laboratory findings, chemotherapy and prognosis of 21 children with DS-AL were analyzed.

RESULTSMost of the children had disease onset of leukemia at 1 to 5 years of age (85.7%), and acute myeloid leukemia accounted for 57.1% of these cases; 61.9% of the patients had increased lactate dehydrogenase level by 2 folds or more. Of the 13 cases undergoing echocardiaography, 10 (67.9%) showed abnormal findings, and complex congenital heart disease was common (38.5%). Six of the children received chemotherapy and complete remission was achieved in 4 cases; 2 patients died of infection, and the treatment-related mortality was 33.3%. The 2 patients receiving reduced intensive chemotherapy have so far had event-free survival for 21 and 43 months.

CONCLUSIONAcute myeloid leukemia is the most common subtype of DS-AL. Patients with DS-AL are sensitive to chemotherapy and the prognosis was favorable with reduced intensive chemotherapy.

Antineoplastic Combined Chemotherapy Protocols ; Child, Preschool ; Disease-Free Survival ; Down Syndrome ; complications ; Humans ; Infant ; Leukemia, Myeloid, Acute ; complications ; drug therapy ; Prognosis ; Remission Induction

We investigated the effect of tilianin upon inducible nitric oxide synthesis in the plasma of low-density lipoprotein receptor knock-out (Ldlr-/-) mice fed with high cholesterol diet and in primary peritoneal macrophages of Ldlr-/- mice. High cholesterol diet induced nitric oxide production in the plasma of Ldlr-/- mice. Tilianin reduced the level of nitric oxide (NO) in plasma from Ldlr-/- mice induced by the high cholesterol diet. Tilianin also inhibited the NO production from the primary culture of peritoneal macrophages treated with lipopolysaccharide. The inhibition of NO production was caused by the suppression of inducible nitric oxide synthase (iNOS) gene expression in peritoneal macrophages isolated from Ldlr-/- mice. Moreover, tilianin inhibited the transcriptional activation of iNOS promoter that has NF-kappa B binding element. Thus, these results provide the first evidence that tilianin inhibit iNOS expression and production of NO and may act as a potential anti-inflammatory agent.
Tyrosine/analogs & derivatives/metabolism ; Tissue Distribution ; Sinus of Valsalva/metabolism/pathology/ultrastructure ; Receptors, LDL/*genetics ; Promoter Regions (Genetics)/drug effects ; Nitric Oxide Synthase Type II/*metabolism ; Nitric Oxide/biosynthesis/blood ; NF-kappa B/metabolism ; Mice, Knockout ; Mice ; Male ; Inflammation/metabolism ; Glycosides/*pharmacology ; Flavonoids/*pharmacology ; Down-Regulation/drug effects ; Atherosclerosis/metabolism ; Animals