Hemophagocytic lymphohistiocytosis (HLH) is characterized by hemophagocytosis and a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages,histiocytes) and CD8+ T cells.The majority of genetic causes identified to date affect the cytotoxic function of NK and T cells,crippling immunologic mechanisms that mediate natural immune contraction.The predominant clinical findings of HLH are fevers (often hectic and persistent),cytopenias,hepatitis and splenomegaly.Due to the life-threatening implications of the diagnosis of genetically determined HLH,antiinflammatory therapy,often consisting of steroids,etoposide or antithymocyte globulin,should be instituted promptly,followed by curative hematopoietic cell transplantation.Secondary HLH,associated with autoimmune disorders or viral infections in teens and adults,should control the primary inflammatory.

Objectives To study the impact of initial age of treatment on testicular morphology and function in male pa-tients with multiple pituitary hormone deficiency using human chorionic gonadotropin (hCG) and human menopausal gonadotro-pin (hMG). Methods Patients with multiple pituitary hormone deficiency were treated with hCG and hMG while keeping other hormone levels normal. Correlations among their testicular volume, follicle-stimulating hormone (FSH), luteinizing hormone (LH), and height, bone age were analyzed. Results After 6-month hCG and hMG treatment, the penis length of 54 male patients was significantly increased from (2.58 ± 0.69) cm to (4.19 ± 0.77) cm , and penis circumference was also increased from (3.71 ± 1.36) cm to (5.95 ± 1.26) cm;the testicular volume was increased from (1.76 ± 1.49) ml to (5.20±2.30) ml;height was increased from (147.01 ± 12.29) cm to (151.98 ± 11.52) cm and bone age was increased from (11.22 ± 2.71) years to (11.64 ± 2.72) years;the differences before and after treatment were statistically significant (all P<0.01). As the patients' age increased, their testicular volume and testosterone level increased slowly, and significant differences amongst each age group were found (P<0.05). The in-creased value of testicular volume, serum FSH and LH levels, and height growth tended to be negatively correlated to the initial age of therapy (r=-0.517~-0.334, P<0.05). Conclusions Early and proper treatment in male patients with multiple pituitary hormone deficiency using hCG and hMG improves testicular function and secondary sex characteristics.

BACKGROUND:A variety of cel growth factors are involved in skeletal muscle regeneration; moreover, these factors cooperate with each other to promote muscle repair and regeneration. OBJECTIVE:To explore the synergy mechanism of a variety of cel growth factors in promoting damage repair. METHODS:By using literature survey, Wanfang, CNKI and PubMed databases were searched for articles related to exercise-induced muscle damage and repair using the keywords of “cel growth factor; skeletal muscle damage;repair; fibroblast growth factor” in Chinese and English, respectively. Research achievements related to exercise-induced muscle damage, molecular biological characteristics of cel growth factors and skeletal muscle injury repair are discussed. RESULTS AND CONCLUSION: Basic fibroblast growth factor plays a basic biological role to promote cel proliferation and angiogenesis, which is the strongest cytokine known to promote cel growth and reflects a very important role in skeletal muscle repair. Epidermal growth factor is synthesized by monocytes and ectodermal cels, which is prominent to stimulate the division and proliferation of a variety of tissues and cels, enhance cel movement, division and synthesis of interstitial protein. Insulin-like growth factors are a family of insulin-like growth factor 1-related and insulin-like growth factor 2-related peptides, which can promote muscle protein synthesis, promote muscle cel proliferation and differentiation, and participate in skeletal muscle regeneration and repair, thereby accelerating wound healing of the muscles. Neurotrophic factor is a kind of trace soluble substances around sensory neurons and produced by neuron-targeted cels, which can specificaly promote neuronal growth and maintenance, and promote skeletal muscle repair. But studies on the synergy mechanism of a variety of cel growth factors in the repair of exercise-induced muscle damage are just at the initial stage, and further research is necessary.

Objective To find out whether NOTCH receptors can serve as direct downstream targets of transforming growth factor β(TGF-β)/Smad4 signaling in endothelial cells.Methods Real-time PCR and Western blotting were performed to verify whether the expression of notch1 and notch4 was regulated by TGF-β pathway.Luciferase reporter assay was employed to investigate how the promoter of notch1 and notch4 was regulated by TGF-β.Then, ChIP assay was used confirm whether the promoter of notch1 and notch4 physically interacted with SMAD protein.Results TGF-β1 and bone morphogenetic protein 4 (BMP4) treatment increased the expression of both notch1 and notch4 at the transcriptional level.In addition, SMAD4 was physically associated with the SMAD binding sites on the notch4 promoter, which was largely enhanced under the treatment of TGF-β1 and BMP4.Importantly, TGF-β1 and BMP4 failed to transactivate notch4 in the absence of endogenous SMAD4 or the SMAD binding regions on the notch4 promoter.Conclusion The expression of NOTCH receptor can be directly up-regulated by SMAD4-mediated TGF-β/BMP signaling in cerebrovascular endothelial cells.

Objective To study MRI features of pituitary stalk interruption syndrome(PSIS).Methods 10 cases in 42 with growth retardation patients diagnosed as PSIS by MRI,all of them were male,age ranged from 5 to 18 with average 13.6 years.Results The anterior-pituitary superior border was depressed,anterior-pituitary volume decreased(average height 2.1 mm,average width 7.8 mm),ectopic posterior-pituitary lobe located at infundibulum stem and pituitary stalk was absent in all cases.The blood hormorne test showed the growth hormone deficiency and multiple growth hormone deficienty.Conclusion PSIS often see in men,MRI findings of PSIS are characteristic.

Objective To further study the value of MR T2WI fat saturation sequence in acute Spinal Trauma. Methods 109 cases of acute Spinal Trauma underwent MR imaging were analysed retrospectively. The detection rate of lesions with bone marrow edema, Vertebral fracture and spinal cord injury was compared in T2WI and FS T2WI. Results FS T2WI could detect more lesions of obscure fracture and bone marrow edema than that in T2WI,and it could accurately show the extent and the feature of anatomic structure in acute injury of bone. Conclusion FS T2WI can improve the diagnostic sensition and accuracy in Spinal Trauma, and it should be used as a routine MR sequence for detecting Spinal Trauma.

Objective To study the imaging features of Scheuermann disease and compare the value of MRI, X-Ray and CT images in diagnosis of this disease. Methods The images of 14 Scheuermann disease patients were analyzed retrospectively. Results The pathological changed vertebraes have wedge-shaped changes in different degree. The edges of the vertebraes manifest roughness and have become step-shaped, or Schmorl′s nodes were demonstrated. Conclusion The multi-vertebraes wedge-shaped changes and the Schmorl′s nodes are the main imaging features of Scheuermann disease. The pathological change occurs in the different spine segments, and the imaging features are not completely same. X-Ray and MRI images are more important in diagnosis of this disease. MRI Multi-direction imaging displays clearly the Schmorl′s nodes, marrow dropsy, the fat accumulation and the intervertebral disc denaturation. What′s more important is that MRI has its own superiority in the differential diagnosis of this disease.

Objective To investigate the expression of 4-hydroxynonenal (4-HNE) in the kidney of diabetic rats and the effect of probucol.Methods The rats were being intraperitoneal injected with STZ (60 mg/kg) to establish diabetic models.Then diabetic rats were randomly divided into diabetic group (group D,n =24),probucol treated group (group P,n =24).Normal rats were taken as control group (group C,n =24).Rats in group P were treated by probucol (110 mg·kg-1·d-1); rats in group D and group C were given equal volume water instead.Scr,BUN,triglyceride (TG),total cholesterol (TC) and 24-hour urinary proteinin were measured at the 4th,8th and 12th week.PAS staining and HE staining were used to evaluate the pathological changes of the kidney.The immunohistochemistry and Western blotting were used to detect the expression of 4-HNE in renal tissue.Results Levels of Scr,BUN,TG,TC and 24-hour urinary protein in group D were higher than those in group C at the 4th,8th and 12th week(all P ＜ 0.05); Levels of Scr,BUN,TG,TC and 24-huor urinary protein in group P were lower than those in group D at 4th,8th and 12th week (all P ＜ 0.05).The pathological changes of the kidney in group D were more serious than that in group P.The expression of 4-HNE in group D were higher than group C at the 4th,8th and 12th week (all P ＜ 0.05);The expression of 4-HNE in the kidneys of group P decreased significantly compared to that of group D at the same time (P ＜ 0.05).Conclusions As an indicator of lipid peroxidation,the expression of 4-HNE significantly increases in the kidney of diabetic rat.Probucol may protect the diabetic kidney through decreasing the expression of 4-HNE and the level of lipid peroxidation.

Objective To observe the changes of adrenal cortical function in children with growth hormone deficiency before and after treatment with recombinant human growth hormone. Methods Seventy-two children diagnosed with GHD who received rhGH treatment for no less than 6 month were included, among whom there were 32 children who were accompanied with adrenal cortical hormone (ACTH) deficiency. The changes of fasting plasma cortisol (COR) and ACTH levels before and 3 and 6 months after treatment with rhGH were retrospectively analyzed. Results Thirty-two children with ACTH deficiency were treated with rhGH after COR level reached normal by the supplementing exogenous hydrocortisone. There was negative correlation between COR level before treatment and the HC dose that make the COR reach normal lowest limit level (r?=?-0.899, P??0.05). The level of COR was obviously decreased after treatment with rhGH, and there was statistical difference compared with that before treatment (P?

Objective To study the prevalence of toenail onychomycosis in the patients with diabetes mel-litus. Methods The incidence and predisposing factors of onychomycosis were studied in the in- and out-patients with diabetes mellitus(n = 456). The data were also compared with non-diabetic patients (control group, n = 350). Results The prevalence rates of toenail onychomycosis were 20.8% and 9.4% in the diabetics and control group, respectively, with statistical difference (P