A case of the H syndrome was reported.An 18-year-old man presented with an 8-year history of progressive cutaneous sclerosis,skin hyperpigmentation,and hypertrichosis on the trunk and extremities.In addition,he suffered from short stature,hearing loss,cardiac anomaly,hepatosplenomegaly,and scrotal masses.Laboratory examination revealed hypogonadism.Cutaneous histopathologic examination showed hyperpigmentation of the basal layer with acanthosis,widespread fibrosis (moderate in dermis and severe in subcutis) and an infiltrate composed of histiocytes and plasma cells.A perivascular lymphocyte infiltrate was also observed throughout the dermis and subcutaneous fat tissue.These findings were consistent with the H syndrome.Differential diagnosis must be made with Winchester syndrome and polyneuropathy, organomegaly,endocrinopathy,M-protein and skin change (POEMS) syndrome.

Three cases of medallion-like dermal dendrocyte hamartoma are reported.The patients included two men and one woman,and were aged from 2 to 38 years.All the patients presented with medallion-shaped,welldefined,slightly atrophic and asymptomatic congenital patches with prominently dilated superficial veins that had grown proportionately with the body length.Histological examination revealed similar changes in all of these patients,including epidermal atrophy and dermal proliferation of spindle cells,which stained positive for CD34,factor ⅩⅢa and vimentin,but negative for S100,CD68,CDla,CD31 and smooth muscle actin (SMA).None of the patients received special therapy,and the lesions enlarged slightly with body growth within 3-4 years of follow-up after the diagnosis.

Objective To investigate the effects of dopaminergic medication on decision-making un-der ambiguity in patients with early Parkinson's disease( PD) . Methods Using Iowa Gambling Task ( IGT) for 24 early non-medication idiopathic PD patients( Hoehn and Yahr Scale≤Ⅱlevel) ,24 early idiopathic PD patients with regular dopaminergic medication and also for 24 healthy controls( HC) whose age,gender,and education match to PD patients to test their ability of decision-making under ambiguity. Results The results showed non-medication PD group showed impairments on digtal span and verbal fluency and decision-making task. There was significant difference in IGT task scores among the three groups(F=6.024, P=0.004) . The total net scores of advantageous choices in IGT were significantly lower in non-medication PD group( (-4.50 ±22.19) scores) than medication PD group((8.83±23.24)scores) and healthy group((15.92±15.77) scores) . The difference of net scores in block1 to block5 between non-medication PD group and medication PD group was gradually increased,and the difference of net scores in block5 was significant(P<0.05) . There was no significant difference in total net scores and net scores in block1 to block5 between medication PD group and healthy group(P>0.05) . As the game processing,medication PD group gradually shifted their se-lections toward the advantageous choices. But non-medication group did not exhibit this shift pattern and the performance was much poorer. Meanwhile, the study also indicated the total net scores of advantageous choices for non-medication PD group was positive correlation to the MoCA scores ( r=0.614, P=0.001). Conclusion The present study has shown that non-medication PD group has impairment in decision-making under ambiguity risk condition and prefer to choose risky options. when exogenous complement dopaminergic medication,the risk decision-making ability of medication PD group has been improved.

Animals ; Hyperbaric Oxygenation ; Lung ; enzymology ; Oxygen ; Protein Kinase C ; metabolism ; Rats

Objective To explore the clinical value of renal dynamic imaging and urinary N-acetyl-?-D-glucosaminidase(NAG),apoptosis DNA fragment(ADF) in evaluating the damage degree of hydronephrotic kidneys(HnK) in children with hydronephrosis.Methods Level of glomerular filtration rate(GFR) was detected in 41 children with congenital hydronephrosis by renal dynamic imaging,and urine NAG,ADF in pelvis in HnK and healthy kidneys (as controls) were detected by enzyme-linked immuno-sorbent assay(ELISA).Patholo-gic changes of HnK in 41 children were graded intoⅠ~Ⅴ according to Elder standard. And GFR,urinary NAG and ADF of HnK were divi-ded into subgroup according to pathologic changes ,at the same time statistical analysis was performed within each groups. And the correlations of pathologic grades with GFR,urinary NAG and ADF of HnK were analyzed.Results 1.Kindneys GFR in healthy kidneys and Hnk were (174.33?20.43)?10-3 L/min,(143.86?17.51)?10-3 L/min respectinely,and there was significant difference between healthy kidneys and Hnk (P0.05).3.There was significant negative correlation between GFR levels of HnK and pathologic grades(r=-0.814 P0.05).Conclusions For hydronephrotic kidneys,urinary NAG can eva-luate impaired nephric tubule whereas renal dynamic imaging may evaluate the damage level of glomeruli;urine ADF may not indicate the damage level of diseased kidneys in children with congenital hydronephrosis.

Two cases of frontal fibrosing alopecia (FFA) were reported.Case 1,a 44-year-old woman,presented with progressive recession of the frontotemporal hairline for 4 years and multiple skincolored facial papules for 2 years.Skin examination showed that the frontotemporal hairline was receded,the local skin was smooth and thin,and a few remaining fine hairs could be seen.Additionally,eyebrows,axillary and pubic hairs were partly shed,and plenty of millet-sized skin-colored papules were diffusedly distributed on the frontotemporal region and bilateral mandibular angles.Dermatoscopy showed reduced hair follicular openings,different hair shaft diameters,cicatricial white patches and perifollicular erythema.Case 2,a 55-year-old woman,presented with progressive frontotemporal hair loss for 2 years.Skin examination revealed the recession of bilateral frontotemporal hairline and partial loss of eyebrows,axillary and pubic hairs.Histopathological examinations of the 2 patients both revealed perifollicular infiltration of lymphocytes,liquefaction degeneration of basal cells and perifollicular lamellar fibrosis.Clinical manifestations and histopathological features of the 2 patients both confirmed the diagnosis of FFA.

OBJECTIVE:To explore the effects of inhaled budesonide on the efficacy and related indexes of patients with acute bronchitis. METHODS:102 patients with acute bronchitis were randomly divided into control group and observation group. Control group was given 100 mg/(kg·d) Cefotaxime sodium injection,adding into 150 ml 0.9% Sodium chloride injection intravenously by 2 times,as well as sedation,oxygen inhalation,rehydration,correcting acid-base balance and other conventional treatment;ob-servation group was additionally given 2 ml Inhaled budesonide suspension,twice a day. The treatment course for both groups was 7 d. Clinical efficacy,erythrocyte immune complex rosette(E-ICR),high-sensitivity C-reactive protein(hs-CRP),peak expiratory flow rate(PEF),forced vital capacity(FVC),1 second forced exhaled volume(FEV1),time of body temperature returned to nor-mal,cough disappearance time,rale disappearance time before and after treatment,and incidence of adverse reactions in 2 groups were observed. RESULTS:The total effective rate in observation group was significantly higher than control group,time of body temperature returned to normal, cough disappearance time and rale disappearance time were significantly shorter than control group,the differences were statistically significant(P<0.05). Before treatment,there were no significant differences in the E-ICR and hs-CRP levels,PEF,FVC and FEV1 between 2 groups(P>0.05). After treatment,E-ICR and hs-CRP levels were significant-ly lower than before,and observation group was lower than control group,PEF,FVC and FEV1 were significantly higher than be-fore,and observation group was higher than control group,the differences were statistically significant(P<0.05). And there were no adverse reactions during treatment. CONCLUSIONS:Based on conventional treatment,inhaled budesonide has obvious efficacy in the treatment of acute bronchitis,and it can reduce E-ICR and hs-CRP,improve pulmonary functions,with good safety.

Objective:To explore the application of real-time transrectal ultrasound (TRUS) during seminal vesiculoscopy in infertile men with azoospermia or oligoasthenospermia.Methods:We retrospectively analyzed the clinical data of 25 cases of azoospermia or oligoasthenospermia due to ejaculate ducts obstruction who were treated with real-time transrectal ultrasound-guided seminal vesiculoscopy between September 2011 and December 2015. Patients’ age was(29.4±4.5) years. All patients accepted semen analysis, serum sex hormone, MRI, TRUS and then diagnosed as obstructive azoospermia, and 13 cases had intractable obstructive azoospermia or oligoasthenospermia after the failure of simple seminal vesiculoscopy(the path to the ejaculatory duct and seminal vesicle couldn’t be found). All patients were treated with seminal vesiculoscopy under real-time guidance with TRUS. We assessed the success rate of surgery, surgical time and complications.Results:The scope was successfully inserted into the seminal vesicle in 21 of the 25 cases (success rate, 84%). The median operative time was 75(31, 148) min. None of the patients developed severe complications. Among 4 failure cases (4/25, 16%), 1 was due to abnormal congenital development. In 2 cases, a clear outlet of the dual ejaculatory duct could not be found after it was inserted into the prostatic utricle. One case was considered as a Müllerian tubular cyst, and the seminal vesicle scope was used to assess the cystic side wall. The 21 patients were followed up for 3 to 6 months, semen volume 2.0(0-5.2)ml, total sperm 28(0-832) ×10 6/ejaculate, sperm density 5.6(0-110.3)×10 6/ml, mobility rate of sperm 5.4%(0-63.6%), and the differences were significant as compared to that before the surgery [semen volume 0.4(0-2.8)ml, total sperm 0(0-342)×10 6/ejaculate, sperm density 0(0-90.7)×10 6/ml, mobility rate of sperm 0(0-24.1%), all P<0.05]. Among the 17 patients who underwent follow-up of 5 to 9 years, 3 patients was conceived naturally and 9 patients’ postoperative sperm quality has improved and pregnancy in vitro fertilization by extracting sperm from semen. Conclusions:Intraoperative real-time transrectal ultrasound guidance can improved the success rate of seminal vesiculoscopy and promoted operative safety.

The chemical investigation on Ganoderma philippii led to the isolation of sixteen compounds by silica gel and Sephadex LH-20 column chromatography. On the basis of spectroscopic data analyses, their structures were elucidated as 2, 5-dihydroxyacetophenone (1), methyl gentisate (2), (S) -dimethyl malate (3), muurola-4, 10 (14) -dien-11beta-ol (4), dihydroepicubenol (5), 5-hydroxymethylfuran carboxaldehyde (6), ergosta-7, 22E-dien-3beta-ol (7), ergosta-7, 22E-dien-3-one (8), ergosta-7, 22E-diene-2beta, 3alpha, 9alpha-triol (9), 6/beta-methoxyergo-sta-7, 22E-dien-3beta, 5alpha-diol (10), ergosta-4, 6, 8(14), 22E-tetraen-3-one (11), ergosta4, 6, 8-(14), 22E-etetraen-3beta-ol (12), 5alpha, 8alpha-epidioxy-ergosta-6, 22E-dien-3beta-ol (13), 7alpha-methoxy-5alpha, 6alpha-epoxyergosta-8-(14), 22E-dien-3beta-ol (14), ergosta-8, 22E-diene-3beta, 5alpha, 6beta, 7alpha-tetraol (15), and ergosta-5, 23-dien-3beta-ol, acetate (16). All the compounds were obtained from this fungus for the first time, and compounds 4 and 5 were isolated from the Ganoderma genus for the first time.
Ganoderma ; chemistry ; Medicine, Chinese Traditional ; Organic Chemicals ; analysis ; isolation & purification

OBJECTIVETo explore the effect of Jinwu Jiangu Recipe (JJR) on the expression of synovial cells' nuclear factor-kappaB (NF-kappaB) and serum interleukin 17 (IL-17) in collagen induced arthritis (CIA) rats.

METHODSTotally 60 Wistar rats were randomly divided into 6 groups, i.e., the blank control group, the model group, high, middle, and low dose JJR treatment groups, and the tripterygium control group, 10 in each group. Except rats in the blank control group, CIA model was established in rats of the rest 5 groups. Then they were treated from the 7th day of modeling. After 4 weeks of medication they were sacrificed, serum collected, and synovium of joints were isolated. The expression of serum IL-17 was detected in synovium of joints by enzyme linked immunosorbent assay (ELISA). And the expression of NF-kappaB/P65, Ikappabetaalpha and NF-KappaB/P50 were detected by Western blot.

RESULTSCompared with the blank control group, the serum IL-17 level increased in the model group (P <0. 01). Compared with the model group, the serum IL-17 level obviously decreased in high and middle dose JJR groups and the tripterygium control group (P < 0.01). Results of Western blot showed, when compared with the blank control group, protein activities of NF-kappaB/P65 and NF-kappaB/P50 were significantly enhanced in the model group (P < 0.01). Compared with the model group, protein activities of NF-kappaB/P65 and NF-kappaB/P50 significantly decreased in high and middle dose JJR groups and the tripterygium control group (P < 0.05, P < 0.01). All indices mentioned above were higher in the low dose JJR group than in the tripterygium control group (P < 0.05, P < 0.01).

CONCLUSIONJJR could lower the expression of serum IL-17 in CIA model rats, and inhibit protein activities of NF-kappaB/P65 and NF-kappaB/P50.

Animals ; Arthritis, Experimental ; drug therapy ; metabolism ; Drugs, Chinese Herbal ; chemistry ; Interleukin-17 ; blood ; NF-kappa B ; metabolism ; Random Allocation ; Rats ; Rats, Wistar ; Synovial Membrane ; drug effects ; metabolism ; Tripterygium ; chemistry