Objective To study clinical features, treatment and prognosis of nasal NK/T cell lymphoma associated Hemophagocytic Syndrome (HPS).Methods Retrospectively analysis method was used to analyze the clinical data of 3 patients with nasal NK/T cell lymphoma associated HPS. Results 3 patients with nasal NK/T cell lymphoma fulfilled the criteria of HPS. All patients had adverse prognostic factors of lymphoma.1 patient developed HPS as the main primary manifestations of underlying lymphoma,the other 2 patients developed HPS during lymphoma progression. In three cases, bone marrow was infiltrated with lymphoma cells.When HPS occurred,the disease progressed rapidly.The most obvious clinical features were fever,pancytopenia,hypofibrinogenemia,hyperferritinemia,and hemophagocytosis in bone marrow. After being treated according to the HLH-2004 combined with chemotherapy, all patients showed a clinical response,but with the progression of lymphoma,HPS quickly relapsed,and all patients died of severe hepatic dysfunction,coagulopathy,or DIC.Conclusion Nasal NK/T lymphoma associated HPS is an invariably fatal disease with poor prognosis,typically occurring at advanced stage or the terminal phase of the disease.HLH-2004 based protocol in combination with chemotherapy is helpful for nasal NK/T cell lymphoma associated HPS,which may delay disease progression and provid opportunities for the treatment of primary disease.

Objective To explore the inhibition effect of interleukin-10(IL-10)on pneumonic anaphylacticitis in mice with asthma and its effects on the level of serum IgE.Methods Thirty BALB/c male mice whose weight were(20?2)g were randomly divided into 3 groups:normal group(n=10),asthma model group(n=10)and IL-10 treatment group(n=10).Asthma model group and IL-10 group were sensitized with 1 g?L-1 ovalbumin,then asthma model was made with inhallating 10 g?L-1 VOA 20 mL.The normal group was not administered anything as a negative group.Before inhallating with 10 g?L-1 VOA,asthma model group was administered inhalation with 9 g?L-1 saline 0.1 mL,and IL-10 treatment group was administered inhalation with 0.1 mL IL-10 whose concentration was 0.2 ?g?L-1.The level of IgE was measured by ELISA reagent box,eviscerated lungs after collecting blood and then made them into pathological slices.Results There was an obvious distinction in the mice pathological change of lungs and bronchus between asthma model group and other groups,but no significant difference between IL-10 treatment group and normal group.There was no obvious infiltration of inflammatory cells in the airways of IL-10 treatment group and normal group.However,there were a great number of inflammatory cells in the interstitial and peribronchovascular regions of asthma model group.The levels of serum IgE were(5.219?0.427)?g?L-1,(6.633?0.145)?g?L-1 and(5.503?0.566)?g?L-1 in normal group,asthma model group and IL-10 treatment group,respectively.The level of serum IgE in asthma model group increased obviously compared with normal group and IL-10 treatment group,with significant differences(Pa0.05).Conclusions Inhalation of IL-10 can optimally control the pneumonic anaphylacticitis,improve the situation of airway inflammation in mice asthmatic model and reduce the IgE serum level.

Objective To investigate the clinical, electrophysiological and pathological features of critical illness polyneuropathy and myopathy (CIPNM). Methods The clinical outcomes, electromyogram Results as well as pathological features in nerves and muscles of 3 patients with CIPNM were investigated and analyzed. Results 3 patients were all provided with assisted respiration after tracheal intubation. 7～10 d after intubation, all the patients emerged muscle strength and tendon reflexes of extremities weakening; while 14 days after that, 2 patients appeared amyotrophy of extremities. Electromyogram showed that the conduction of many motor and sensory nerves for extremities decreased, while the amplitude of compound muscle action potential (CMAP) of part of motor nerves decreased. Biopsy for nerves revealed decreased medullated nerve fibers and regeneration phenomenon of auxiliary fibers; while that for muscles showed neuralgic damage and myopathy-like changes. Conclusion CIPNM can complicate after tracheal intubation. The electrophysiological and pathological examinations for nerves and muscles can be helpful for the diagnosis.

Objective To report clinical and pathological features in a Chinese family with desminopathy caused by a novel mutation in desmin gene. Methods There were 8 patients (7 male and 1 female) in a family manifesting desminopathy in an autosomal dominant inheritance pattern. The onset age ranged from 20--50 years old. Heart disease appeared in 7 cases, diarrhea in 1 case and limb weakness in 3 patients. Muscle biopsies were carried out in proband and his elder brother. The histological, enzymhistochemicai staining, ultrastructural examination and immunohistochemical staining with desmin antibody were performed. The sequence of desmin gene was analyzed in DNA from 4 patients, 5 asymptomatic members and 50 healthy controls. Results Muscle pathology in the proband revealed wide variation in size of fibers. Some fibers had eosinophilic changes with rimmed vacuoles. Muscle pathology in the brother revealed multiple small and round eosinophilic inclusions in the fibers. Desmin staining was positive in the inclusions and in the sarcolemma. Electron microscopy revealed deposits of abnormal granulofilamentous materials. A novel S12F mutation in desmin gene has been identified in 4 affected family individuals, but not in unaffected family members and controls. Conclusions The desminopathy caused by desmiu SI2F mutation dominantly affected male individuals. Most of victims had cardiac abnormalities. The myopatholgical features can vary in the different patients in one family.

OBJECTIVETo investigate the characteristics of father-to-son vertical transmission of Y chromosome microdeletions

METHODSWe detected the Y by detection of Y chromosome microdeletions in infertile men and analysis of some of their families. chromosome azoospermia factor (AZF) microdeletions in the peripheral blood of 1 052 infertile males, investigated the paternal relatives of 12 cases of AZFc, 1 case of AZFb and 1 case of AZFb + c microdeletions, and drew the family tree diagrams of the infertile paternal relatives according to the findings.

RESULTSAmong the 1 052 infertile patients, 89 (9.73%) were found with Y chromosomal microdeletions, including 56 with AZFc, 6 with AZFa, 5 with AZFb, 14 with AZFb + c, and 8 with AZFa + b + c deletion. The investigation of the 14 patients'families revealed 1 case of AZFb and 1 case of AZFb + c deletion de novo. Among the 12 cases of AZFc deletion, vertical heredity was found in 5 patients with severe oligozoospermia, but not in the other 7 with azoospermia.

CONCLUSIONAZFe deletion may be vertically inherited from the father in severe oligozoospermia patients, and it is different from the paternal phenotype, while in azoospermia patients, AZF deletion, whatever type it may be, is less likely to be associated with vertical paternal heredity.

Adult ; Chromosome Deletion ; Chromosomes, Human, Y ; genetics ; Humans ; Infertility, Male ; Male ; Mass Screening ; Pedigree ; Sex Chromosome Aberrations ; Sex Chromosome Disorders of Sex Development ; genetics ; Young Adult

Objective To investigate the relationship between body mass index (BMI) and homocysteine,pregnancy outcomes in patients with gestational diabetes mellitus.Methods From Jan,2013 to Oct,2015,120 patients with gestational diabetes mellitus were prospectively enrolled in this study.According to BMI level,all patients were divided into obese group (BMI≥25 kg/m2) and the control group (BMI ＜ 25kg/m2).The main outcome measures included homocysteine and pregnancy outcomes of the both groups.Results When compared with the control group,patients in the obese group got a significantly higher level of homocysteine (11.09 ± 1.91 vs 8.92 ± 1.57μmol/L,P =0.000);and BMI was positively associated with homocysteine (r =0.410,P =0.000).When compared with the control group,patients in the obesity group got a significantly higher rate of cesarean section (30.00％ vs 15 ％,P =0.049);a higher rate of macrosomia (20.00％ vs 5.00％,P =0.027);and a higher level of neonatal weight (3672.15 ± 475.45 vs 3220.93 ± 461.36g,P =0.000).There was no significantly difference between the two groups in weeks of gestation,postpartum hemorrhage,cephalopelvic said,abnormal fetal position,uterine atony,premature rupture of fetal membranes and neonatal asphyxia rate (P ＞ 0.05).Conclusion BMI in patients with gestational diabetes mellitus is positively correlated with homocysteine,cesarean section,macrosomia and neonatal weight.

Objective To evaluate the efficacy of radiosynovectomy with 32P colloid in haemophilic synovitis of adolescents. MethodsRadiosynovectomy with 32P colloid was primary performed on 26 male haemophilic patients(26 joints),whose average age was 16 years(11 to 21 years).The average dose of 32P colloid was 2.1 mCi(1.0 to 3.0 mCi). Results After 6-month interval,haemarthrosis was reduced by no less than 30% in 23 patients,with a total efficacy of 88.5%.The mean frequency of haemarthrosis was reduced from 1.9 per month of presynovectomy to 0.3 per month of postsynovectomy(P

OBJECTIVE To investigate the incidence and pathogens of infection in 1659 consecutive cases in single center hematological unit.METHODS The incidence,pathogen,and outcome of infection in 1659 hospitalized patients with hematological disorders from 1999 to 2006 were retrospectively analyzed.RESULTS The overall incidence of infection was 24.4% according to the person-times of hospitalization,which included 22.1% of nosocomial infection and 2.3% of community acquired infection.Most of the pathogenic bacteria of the nosocomial infection were Gram-negative.The most common bacteria in the sputum samples included Enterobacter cloacae(19.3%) and Pseudomonas aeruginosa(14.8%).The most common bacteria in the blood samples were coagulase negative Staphylococcus(CNS,39.3%),the next was Pseudomonas aeruginosa and Escherichia coli.There were 4.21% bacteria resistant to most of antibiotics in nosocomial infection.There were 114 fungi isolated.Candida albicans was accounted for 35.1%.The mortality due to nosocomial infection was 7.4%.CONCLUSIONS The patients in hematology ward are susceptible to infection.The pathogens of nosocomial infection are most likely G-bacteria.Some bacteria are resistant to almost all antibiotics.The incidence of fungal infection is increasing in the near 8 years.

Objective: To observe the clinical efficacy of deep electroacupuncture (EA) at Baliao points in treating stress urinary incontinence (SUI). Methods: A total of 60 female patients with SUI were divided into two groups according to the order of consultation, with 30 cases in each group. The control group was treated with pelvic floor muscle training. The treatment group was treated with deep EA at Baliao points [Shangliao (BL 31), Ciliao (BL 32), Zhongliao (BL 33) and Xialiao (BL 34)]. Results: The total effective rate was 93.3％ in the treatment group, versus 33.3％ in the control group, and the total effective rate of the treatment group was significantly higher than that of the control group (P<0.05). After treatment, the scores of international consultation on incontinence questionnaire-short form (ICIQ-SF) and the volume of urinary leakage in both groups were lower than those before treatment (all P<0.05), and the ICIQ-SF score and the volume of urinary leakage in the treatment group were lower than those in the control group (both P<0.05). Conclusion: Deep EA at Baliao points with long needles can improve the clinical symptoms in female patients with SUI, and it has a better curative effect than pelvic floor muscle training.

Biological characteristic of Salvia miltiorrhiza f. alba in field was studied. HPLC method was used to determine the lipophilic constituents (dihydrotanshinone, cryptotanshinone, tanshinone, tanshinone II (A) and miltione) and hydrophilic constituents (salvianolic acid, rosemarinic acid). The results showed that the fresh weight of S. miltiorrhiza f. alba which cropped for 2 years was decreased by 80.47%, while dry weight decreased by 79.42%. The normal diameter of the root was 0.3-0.5 cm, however, the diameter was 0.2-0.4 cm after 2 years, it was said that the decrease of the root diameter was the main reason for the decrease of the yield. The average contents of dihydrotanshinone, cryptotanshinone, tanshinone, tanshinone II (A), miltione, salvianolic acid and rosemarinic acid were decreased by 35.26%, 32.26%, 19.35%, 3.39%, 64.40%, 66.93% in plant which continuously cropped for 2 years, respectively. The yield and active constituents were mostly effected in the plant of S. miltiorrhiza f. alba, which continuously cropped for 2 years.
Drugs, Chinese Herbal ; analysis ; Linear Models ; Quality Control ; Reproducibility of Results ; Salvia miltiorrhiza ; chemistry ; growth & development ; Time Factors