AIM: To investigate the chemical constituents of the plant drug Cornus officinalis Sieb et Zucc. which is widely used in Chinese traditional medicine, having actions of invigorating the liver and kidney, strengthening the body, and of an astringent, etc. METHODS: A sedoheptulose gallate was isolated from the water extracts of fruits of C.officinalis using various chromatographies. The structure determination was based on spectral (UV, IR, 1H & 13CNMR and MS) and chemical evidence. RESULTS: Its structure was characterized to be 7-O-galloyl-D-sedoheptulose (I). The proportion of its three major conformation forms of β-F, α-F and α-P in aqueous solution was estimated to be 57:24:19 according to their C-1 peak heights in the 13CNMR spectrum. CONCLUSION: I was found in natural world for the first time and its β-D-heptufuranosyl form was the predominant existing tautomer in its equilibrated aqueous solution according to NMR determination.

The effect of OQP, a pormula of traditional Chinese medieine belonging to the category "Precription of the Reinforeing Qi and Enriching Blood for keep the Original Qi", on cell strain of human hepatic carcinoma SMMC-7721 was observed. The results showed that OQP depressed the incorporation of both ~3H-TdR and ~(14)C-UR in cells of hepatic carcinoma. This indicated that OQP inhibited the synthesis of DNA and RNA, and the growth of the cancer cell. Such effect may result from the low ratio Cu/Zn and the high content of Se in OQP. In conclusion, in this study it has been demonstrated that OQP can not only improve the general condition of patients in advanced liver cancer and lcukopenia of patients in chemotherapy and radiotherapy, but also can inhibite the growth of the cell of hepatic carcinoma directly.

AIM: To determine the contents of corilagin in Erodium stephanianum Willd. from different collection month and to define the best collection time for this herb. METHODS: The contents of corilagin in each samples of E. stephanianum were determined by HPLC. RESULTS: The linear regression of corilagin was obtained in the range of 0.22～1.10?g. The average recovery was 98.6% with a RSD of 0.63%. CONCLUSION: The content of corilagin in E. stephanianum collected in August is the highest among the samples throughout the collection months.

OBJECTIVE: To explore the inhaled and food allergen distribution of patient with allergic rhinitis in north of Zhejiang and to analyze the difference of allergen distribution among different age groups. METHOD: One thousand and forty eight patients in north of Zhejiang diagnosed with allergic rhinitis in our outpatient department were tested with skin prick test (SPT). The positive rate of inhaled and food allergens were calculated. To analysis the difference of positive rate between children and adult. RESULT: Nine hundred and eighty-eight cases (94.3%) had the positive reaction. Dermato phagoides farinae and Dermatophagodies pteronyssinus had the highest positive ratio (72.1%, 71.8%) in inhalation group, followed cockroach (14.1%). In food group, Shrimp and peanuts had the highest positive ratio (18.3%,14.2%). Between children group and adults group, positive rate of food allergen was significantly different (P < 0.05), but of inhaled allergen was not significantly different. CONCLUSION The distribution of some allergens in children group and adults group was variable. The study shows that Dust mite was the mostly responsible common allergen in north of Zhejiang.
Adolescent ; Adult ; Aged ; Air Pollutants ; immunology ; Allergens ; immunology ; Child ; Child, Preschool ; Female ; Food ; Humans ; Male ; Middle Aged ; Rhinitis, Allergic ; immunology ; Skin Tests ; Young Adult

Objective To study the influence of sunscreens with different efficacy on delayed type hypersensitivity (DTH) and their immunoprotective effect in mice.Methods A cohort of mice were randomly divided into 5 groups with 10 mice in each group:group 1 as the positive control without irradiation,group 2 receiving solar-simulated radiation (SSR) only,group 3 receiving SSR and protected by sunscreen l with sun protection factor 15(SPF15)and persistent pigment darkening(PPD)12,group 4 receiving SSR and protected by sunscreen 2 with SPF 50 and PPD 28,and group 5 as the negative contml receiving SSR only.SSR was carried out on the back of mice with the UVA dose being 1.4 J/cm2 and UVB dose being 100 mJ/cm2 for 10 days.After a 5-day irradiation,the groups 1 to 4 were immunized by intraperitoneal injection with 100 μl(107 cells/ml) of Candida albicans suspension.On the 10th day both sides of the posterior foot pad were measured;then the foot pads were injected with additional 50 μl of the Candida albicans suspension.Twenty-four hours after the injection,the thickness of each foot pad was measured,and immunosuppression rate was calculated.Finally,the mice were sacrificed and skin samples were obtained from the back of these mice followed by the examination of CDla, CD80 and CD86 expression by Western blot.Resets The thickness of edema in foot pads was 0.41±0.38 mm,0.21±0.23 mm and 0.30 ± 0.25 mm in group 1,3 and 4,respectively,significantly higher than in group 5 and 2(0.04±0.03 mm,0.14±0.12 mm,respectively,all P<0.05),while no significant difference was observed between the group 3 and 4(P>0.05).Significant differences were observed in the immunosuppression rate between group 2,3 and 4(73.0%±11.3%,54.1%±6.4%,29.7%±7.5%,respectively,all P<0.01).Western blot revealed a significant increment in the expression of CDla protein in group 1 compared with group 2 as well as in the expression of CD86 protein in group 1 and group 3 compamd with group 2 and group 5(all P<0.05),but no statistical difference was observed between the other groups in the expression level of CDla,CD80 or CD86(P>0.05).Conclusions The exposure to sub-erythema dose of UV can induce DTH,and sunscreens have an immunoprotective effect in this process.Epidermal Langerhans cells are not essential for UV-induced immunosuppression.

Objective To summarize the phenotypic features of an unrecognized leukoencephalopathy in infants sharing same clinical features,and to better understand the disease and provide new evidence for identification of new leukoencephalopathy. Methods Clinical and follow-up data of 13 patients with unrecognized infantile leukoen-cephalopathy were collected from Peking University First Hospital from January, 2006 to December, 2014. Results (1) There were 7 male and 6 female. The average age of onset was 11 months (4-25 months). Thirty-eight percent (5/13 cases) of patients had incentives before the onset;all of the cases had acute onset and rapid motor function regression. Fifteen percent (2/13 cases) of the patients suffered from seizures in the course of the disease. Patients′condition became stable,and cognition and motor function improved gradually 1 month after onset. No patient died till the last follow-up. (2) Imaging features:magnetic resonance imaging (MRI) of the patients was characterized by im-plicating deep white matter,presenting T1 hypointense,T2 and fluid attenuated inversion recovery ( FLAIR) hyperin-tense in the periventricular area. All of MRI showed massive and symmetric lesions with heterogeneous signal and cystic degeneration. DWI showed patch or massive hyperintense in some of the lesions. The follow-up MRI showed the original lesions decreased in 88% ( 8/9 cases ) of patients, and white matters atrophied in 55% ( 5/9 cases ) of patients;the cystic degeneration still existed and even expanded;DWI showed regional linear or spot hyperintense in 88% (8/9 cases) of patients,which was smaller than before,and distributed around the original lesions. Conclusions The patients with leukoencephalopathy caused by unknown pathogenic gene were much likely to be mitochondrial leukoencephalopathy. This study provided evidence for further exploration of new pathogenic genes causing leu-koence-phalopathy.

Objective To analyze the clinical and MRI features of patients with type Ⅱ Alexander disease (AxD) in order to better understand and diagnose it earlier.Methods Four type Ⅱ AxD patients identified by glial fibrillary acidic protein gene mutations from Peking University First Hospital and 128 type Ⅱ AxD cases from published literatures were collected,and the clinical and MRI features were summarized.Results (1) In 4 type Ⅱ AxD patients,2 adult patients showed abnormal MRI features without clinical manifestation.The other 2 children patients both manifested motor dysfunction of lower limbs,pyramidal signs,paroxysmal deterioration,and seizures during the course of disease,while 1 of them had bulbar paralysis.The MRI of all the cases was abnormal,but only 1 case MRI corresponded with typical MRI features of type Ⅱ AxD.In the other 3 cases MRI showed thc atrophy in the medulla and upper spinal cord,or the brainstem lesions and abnormal signal in the periventricular white matter,and abnormal basal ganglia region.(2) In 128 reported type Ⅱ AxD cases,the age of onset was (32±19) years old.The initial syndromes mainly contained bulbar and/or pseudobulbar paralysis (32.48％,38/117 cases),motor dysfunction of the lower limbs (31.62％,37/117 cases) and autonomic nerve dysfunction (13.67％,16/117 cases).During the course of the disease,the clinical manifestation showed bulbar and/or pseudobulbar paralysis (73.50％,86/117 cases),pyramidal signs (60.68％,71/117 cases) and ataxia (51.28％,60/117 cases).The MRI of all cases was characterized by atrophy or abnormal signals in the brainstem,especially in medulla oblongata,and spinal cord.And abnormal signals in the cerebellar dentate nuclei,white matter,basal ganglia and thalamus were also commonly shown in the MRI.Conclusions The patients with type Ⅱ AxD are late-onsct.The clinical manifestation mainly contains bulbar and/or pseudobulbar paralysis,motor dysfunction of the lower limbs and pyramidal signs.The MRI is characterized by atrophy or abnormal signals in the brainstem (especially in medulla oblongata) and spinal cord.

Objective To analyze the clinical characteristics of 3 unrelated boys with paroxysmal nonkinesigenic dyskinesia and developmental delay caused by de novo mutation in KCNMA1,and to expand the knowledge of clinical phenotype of KCNMA1 mutation.Methods Clinical data of patients were collected,including gender,age,condition of the perinatal period,personal history,and family history.And the features of genotype data were collected including features of attack,developmental milestones,physical examinations,treatments,and responses to treatment.The data including blood biochemical results,results of metabolic screening and genetic testing and the pedigree validation were collected,while the relationship between phenotype and genotype was analyzed.Results (1)Phenotypic features:3 unrelated boys were diagnosed.The ages of disease onset were 20 days,7 months and 13 months,respectively.All the patients manifested paroxysmal nonkinesigenic dyskinesia and were characterized by the episodes that occurred during wakefulness,presented with sudden onset of asymmetric limb dystonic posture,sometimes with nystagmus and strabismus,or sudden decrease of voluntary movement of limbs with hypotonia and occasional esotropia and yawning.There was no loss of awareness during attack.No precipitating factors were observed before attacks.The developmental milestones were delayed.Three children had no response to anti-epilepsy drug before diagnosis.After diagnosis,2 cases used Clonazepam and 1 case showed less attack.There was not any epileptic seizure until the last follow-up at the ages of 3 years and 6 months old,7 years old,and 5 years and 8 months old,respectively.The frequency of attacks was decreased.The episodes were recorded during video-electroencephalogram(EEG) monitoring,which showed normal ictal and interictal EEG.(2)Genotypic features:all 3 children were detected to have KCNMA1 genetic heterozygous missense mutation,while c.2650G>A (p.Glu884Lys) mutation was identified in 1 patient,and c.3158A>G(p.Asn1053Ser)mutation in the other 2 patients,but no such mutation was found in their parents.Conclusion This finding expands the phenotype of KCNMA1mutation.KCNMA1 should be considered as one of the candidate genes for screening in patients with early onset of paroxysmal nonkinesigenic dyskinesia without triggers,or early-onset of developmental delay,with or without epilepsy.

Objective To investigate the effect of acitretin on the histopathology and ultrastructure of lesions from patients with bullous ichthyosiform erythrodermia (BIE), and to explore mechanisms underlying the modulation of keratinization process by acitretin. Methods Lesional tissue was obtained from the back of 4 patients with BIE before and after the treatment with acitretin. Light microscopy and transmission electron microscopy were performed to observe histopathological and ultrastructural changes in these lesions. Results After treatment, the improvement in clinical manifestations was more than 75% in all the 4 patients, and reached 90% in 1 of the 4 patients. As histopathology and ultrastructural study showed, there was an obvious improvement in hyperkeratosis and continuity of extra cellular lamellar membrane, and a decrease in keratin deposition in prickle and granular layer, but no remarkable changes were observed for the proliferation of prickle cells or acantholysis. Conclusions Acitretin shows a favorable efficacy in clinical treatment of BIE,with histopathological and ultrastructural improvement mainly located in the stratum corneum. The modulation of keratinization process in keratinocytes by acitretin appears more apparent in granular and corneum layers.

Obiective:To elucidate the phenotype and the genotype-phenotype correlations in Chinese patients with X-linked adrenoleukodystrophy(X-ALD).Methods:Clinical features of 40 Chinese patients with X-ALD were studied and mutation spectrums were investigated by polymerase chain reaction (PCR) and sequencing. Results:Among these patients, four were siblings from two unrelated families, the others were unrelated. There were 31 cases with childhood cerebral (CCALD), 8 cases with adolescent cerebral (ACALD) and 1 case with adrenomyeloneuropathy (AMN). Visual impairment, which presented in 12 cases (30%), was the most common initial symptom. Nine (69%) of 13 cases who had hydrocortisone and ACTH measured showed adrenal insufficiency. By follow-up date, 19 cases (47.5%) were dead. The interval from onset to death varied from 1 to 6 years and the average were 3.3 years. The mean age at death was 10.5 years. Eleven cases (27.5%) were in vegetable state. The mean interval from onset to apparently vegetable state was 2.8 years (range from 1 to 6 years). Four cases had progressive neurological disability. Four cases were lost follow-up. One case with CCALD and one case with ACALD progressed slowly. The courses of the disease of these two patients were 5 years and 15 years respectively. Thirty five mutations were identified in 40 cases. Most were located within exon 1-3 (40%, 16/40) and exon 6-8 (42%, 17/40). There is a distinct clustering of missense mutations in exon 6 (17%, 7/40). Five types of mutations were associated with CCALD, three with ACALD and a missense mutation was identified in the patients with AMN. The two patients with long disease courses had a missence mutation c.1559 T>A and a nonsense mutation c.1785 G>A respectively. The siblings with similar manifestations and onset age were observed in two families, whose mutations were c.887 A>G and c.1028 G>T. Conclusion:The phenotypes, disease severity and rate of neurodegeneration could not be predicted by the nature of mutations.