Prader-Willi syndrome( PWS) is a type of genetic disease,which is associated with low mus-cle tone,growth and development,progressive fatal obesity and sleep related respiratory disorders(SRBD). Ob-structive sleep apnea hypopnea syndrome( OSAHS) is the most common type of SRBD. The cause,influencing factors and treatment of OSAHS in patients with PWS are described in this paper. To understand the degree of OSAHS in children with PWS,the polysomnography is recommended.

One hundred sixty cases were randomly divided into 2 groups, the treatment group and control group, and treated for 8 weeks by single blind administering method. Results revealed that the total effective rate (89. 5%) for clinical symptoms in the treatment group was far superior to that of control group (54. 2%). So was its effect on nervous functional defect. The total blood viscosity. plasmal comparative viscosity, platelet adhesion, thrombic elstic fingure. in vitro length of thrombus, were markedly improved (P

Platelet-derived growth factor (PDGF), a potent chemotactic and mitogenic factor, is involved in the regulation of hematopoiesis and platelet production. Our studies demonstrate the presence of functional PDGF receptors (PDGFR) on human megakaryocytes/platelets and CD34(+) cells, and their ability to mediate a mitogenic response. PDGF promotes the ex vivo expansion of human hematopoietic stem (CD34(+)) and progenitor (CD41(+)) cells. More significantly, PDGF enhances the engraftment of human CD45(+) cells and their myeloid subsets (CD33(+), CD14(+) cells) in NOD/SCID mice. PDGF also stimulates in vitro megakaryocytopoiesis via PDGFR and/or the indirect effect on bone marrow microenvironment to produce TPO and other cytokines. It also shows a direct stimulatory effect of PDGF on c-Fos, GATA-1 and NF-E2 expressions in megakaryocytes. We speculate that these transcription factors may be involved in the signal transduction of PDGF on the regulation of megakaryocytopoiesis. PDGF also enhances platelet recovery in mouse model with radiation-induced thrombocytopenia. This radioprotective effect is likely to be mediated via PDGFR with subsequent activation of the PI3K/Akt pathway. It provides a possible explanation that blockage of PDGFR may reduce thrombopoiesis and play a role in imatinib mesylate-induced thrombocytopenia.
Animals ; Hematopoietic Stem Cells ; cytology ; Humans ; Megakaryocytes ; cytology ; Mice ; Platelet-Derived Growth Factor ; metabolism ; Receptors, Platelet-Derived Growth Factor ; metabolism ; Thrombopoiesis

Objective: To investigate the biological functions of the Cyr61 gene in human lung carcinomas. Methods: The Cyr61 expression levels in the cancerous and peri-cancerous tissues of 60 lung cancer patients were measured by real-time polymerase chain reaction (PCR). Results: The expression of Cyr61 gene was down-regulated in 80% (48/60) primary lung cancer patients compared with the matched pericancerous tissues. The result was consistent with the immunohistochemical staining. Statistical analysis revealed that the difference between expression of cancerous tissues and the matched peri-cancerous tissues was significant [(2.742 ± 4.165) vs (4.933 ± 3.349), t = -5.112, P=0.000]. Furthermore, the clinical variables including tumor grade, tumor metastasis, tumor pathological classification, smoking, and family history influenced the level of Cyr61 expression (P<0.05). Conclusion: Cyr61 may play a key role in the progression of lung carcinoma and its protein might serve as an important target for the therapeutic intervention in clinic.

Animals ; Annexin A2 ; chemistry ; genetics ; metabolism ; physiology ; DNA Replication ; Endocytosis ; Exocytosis ; Humans ; Neoplasm Invasiveness ; Neoplasm Metastasis ; Neoplasms ; metabolism ; pathology ; Prognosis ; Tissue Plasminogen Activator ; metabolism ; physiology

Female ; Humans ; Infant ; Mastocytosis, Systemic ; diagnosis ; pathology ; therapy

Child, Preschool ; Epithelioid Cells ; Heart Atria ; pathology ; Heart Neoplasms ; Humans ; Perivascular Epithelioid Cell Neoplasms

Abdominal Pain ; Cough ; Humans

Objective To discuss the value of contrast-enhanced ultrasound and color Doppler in diagnosing esoph-ageal varices( EV) among patients suffering from portal hypertension. Methods The control group consisted of twenty nine patients without EV. Sixty patients with EV diagnosed by endoscopy were divided into two groups equal in number according to grade of EV. One being mild,the other was moderate to severe. All patients underwent color Doppler flow imaging and contrast-enhanced ultrasound, and the dynamic angiography data were collected. Time-in-tensity curves were drawn by software. The quantitative parameters including arrival time of hepatic artery ( HAAT) ,hepatic vein ( HVAT) and portal vein ( PVAT) ,damping index ( DI) of hepatic vein and portal vein ve-locity( PVV) were compared. Results The difference of PV-HV, PV-HA, PVV, DI showed statistically signifi-cant among the three groups(F=72.63,14.97,6.71,13.74,P<0.01). A comparison of the control and moderate to severe group among the above four parameters was statistically significant (P<0.01). PV-HV, PV-HA, DI in moderate to severe group showed statistically significant compared with mild group (P<0.01),however there exis-ted no significant difference grouping PVV between the two groups. Mild group contrasted to the control group,the differences of PV-HA were statistically significant (P<0.01), whereas PV-HV, DI, PVV displayed no significant difference. Conclusion Contrast-enhanced ultrasound and color Doppler are helpful in diagnosing EV,which is ex-pected to become a new noninvasive method.

Background and purpose:Breast cancer can be divided into several molecular subtypes according to its biomarkers. The pattern of distant metastasis has a great clinic significance but was rarely investigated. This study investigated the impact of molecular subtype of breast cancer on initial sites of metastasis..Methods:All the patients with operable invasive breast cancer diagnosed in Zhongshan People’s Hospital between 1998 and 2004 were recruited. Subtypes were defined as Luminal A, Luminal B, human epidermal growth factor receptor 2 (HER-2) enriched, and triple negative (TN) according to the expression of estrogen receptor (ER), progestogen receptor (PR) and HER-2 status. The first distant metastatic sites and the time of their appearances were recorded. Survival curves were constructed using the Ka-plan-Meier technique.Results:Among 390 eligible patients, there were 215(55.1%) with Luminal A, 43 (11.0%) with Lu-minal B, 52 (13.3%) with HER-2 enriched, and 80 (20.5%) with TN. The median follow-up time was 118 months (11-163 months). Seventy-two (18.5%) distant metastases occurred during follow-up: 37 metastases in Luminal A, 8 in Luminal B, 10 in HER-2, 17 in TN. Bone was the most common site of the first distant metastasis (39/72, 54.2%) followed by lung (25/72, 34.7%), liver (22/72, 30.6%), and brain (7/72, 9.7%). Among all the metastases, tumors of Luminal type (Luminal A 70.2%, Luminal B 50.0%) had a higher chance of bone involvement than that of HER-2 enriched (30.0%) and TN (35.3%,P=0.03). Both Luminal B (37.5%) and TN (17.6%) subtypes had a higher percentage of brain involvement than Luminal A and HER-2 enriched (P=0.01). The survival analysis showed no significant difference among the four subtypes in 9-year distant metastasis-free survival. However, distant metastasis appeared earlier in HER-2 enriched and TN breast cancer than in Luminal type.Conclusion:Organ-specific metastasis may depend on the molecular subtype of breast cancer. Bone metastasis occurs more in luminal type than in other types. Luminal B and TN types of tumors had more chance of brain metastasis than Luminal A and HER-2 enriched type of tumors.