Immune thrombocytopenia (ITP) is the most common hemorrhagic disease in childhood,and immunological abnormality is the most common cause of this disease.Humoral immune abnormalities are the major cause of ITP,involving platelet,platelet membrane surface glycoproteins associated antibodies,impaired Breg cells and upregulation of platelet Toll like receptors.Cellular immune abnormalities include the imbalance of Th1/Th2 cells results in increased inhibitory cytokines [interleukin (IL)-2,interferon-α,tumor necrosis factor-β],IL-17 and Th17 cells in patients with chronic ITP are higher than those in control group,and Treg decline is also common seen in ITP.Increased expression of apoptosis related genes Bak and Bax are also the cause of chronic ITP.Helicobacter pylori is an important cause of chronic ITP,which can be eased in some cases but not effective in new diagnosed cases.Vaccination can lead to ITP,repeated vaccines are applied usually in 3-6 months after the onset.

Epstein-Barr(EB)virus is the first carcinogenic virus discovered in 1964.EBV infection is common in pediatrics and most of the infected children are asymptomatic.When the immune function of children becomes low or deficient, EBV may cause a series of benign or malignant lymphoproliferative diseases, such as infectious mononucleosis, chronic active EBV infection, hemophagocytic lymphohistiocytosis, post-transplant lymphoproliferative disorders, X-linked lymphoproliferative syndrome and so on.Most of these diseases have complex pathogenesis, diverse clinical manifestations, poor prognosis and high mortality.The only effective treatment is allogeneic hematopoietic stem cell transplantation.

[Abstract ] Objective Nephroblastoma is one of the solid malignancies with fair prognosis in children .The lungs, liver, and bones are the common organs for distant metastasis of the tumor and important factors influencing the therapeutic effects as well .This study was to investigate the clinical characteristics and prognosis of nephroblastoma with distant metastasis in children . Methods We retrospectively analyzed the clinical characteristics , pathological features , and prognosis of 9 cases of nephroblastoma with distant metastasis treated in Nanjing Children Hospital from September 2011 to May 2015 . Results The patients presented with abdominal masses, distention and pain at preliminary diagnosis and confirmed with nephroblastoma , with pulmonary metastasis in 7 cases, liver metastasis in 1, and lung and liver metastases in the other .All were treated by surgery, radiotherapy, and chemotherapy and followed up for a median time of 34 months.By the end of the follow-up, 1 patient died of recurrence , 1 was undergoing chemotherapy , and the other 7 were being recovered, including complete disappearance of lung metastasis in 4, obvious reduction or calcification of lung metastasis in 2, evident disappearance of liver metastasis in 1, but relapse in none. Conclusion Lung and liver are common sites of nephroblastoma metastasis in children .As for the treatment of the disease , relatively good results can be achieved by radiotherapy , chemotherapy , and reoperation .

Objective:To study the pharmacokinetics of indometacin (IDM) enteric-coated dropping pills.Methods:Beagle dogs were used as the experimental animals and the marketed IDM enteric-coated tablets were applied as the control,the pharmacokinetics of IDM enteric-coated dropping pills was studied after a single oral administration.DAS software was used for the model fit and parameter calculation,and the bioequivalence was also evaluated.Results:Both IDM enteric-coated dropping pills and tablets were fitted a one-compartment model.Compared with those of IDM enteric-coated tablets,the Cmax,Tmax,AUC0-∞and Tlag of IDM enteric-coated dropping pills all showed notable differences (P<0.05),and the former two increased significantly,and the latter two were shortened significantly.The relative bioavailability of the dropping pills was (121.0±7.7)%.Conclusion:Compared with IDM enteric-coated tablets,IDM enteric-coated dropping pills are with significantly enhanced absorption and shortened lag time,which is worthy of further studies and development.

Objective: To study the status of indomethacin (IDM) in IDM enteric-coated dropping pills and its interaction with PEG 6000.Methods: IDM, PEG 6000, their physical mixture and IDM enteric-coated dropping pills were respectively studied by DSC, IR, X-ray diffraction and SEM.Results: In the map of DSC, the endothermic peak of IDM enteric-coated dropping pills disappeared at 164℃, and the endothermic peak of PEG 6000 at 57℃ shifted forward.In the map of IR, the carbonyl stretching vibration peak of IDM in IDM enteric-coated dropping pills disappeared, and a new peak appeared at 1 680 cm-1.In the map of X-ray diffraction, there was no diffraction peak in IDM enteric-coated dropping pills.SEM showed the morphology of IDM in the base was much smaller than that of IDM raw material.Conclusion: IDM in IDM enteric-coated dropping pills is dispersed in the matrix with an amorphous or molecular form, and there is hydrogen bond between IDM and PEG 6000.

Severe combined immunodeficiency(SCID)is a kind of severe primary immunodeficiency disease.Patients often develop symptoms after birth, which is characterized by recurrent and life-threatening infection, sometimes accompanied by varying degrees of dysplasia.According to the classification proposed by the PID expert Committee of the International Union of Immunological Societies, SCID is clinically classified by the number of lymphocytes.Early screening and intervention of SCID is significant to improve the prognosis.With the promotion of TREC and other early screening methods, the detection rate of SCID has significantly improved, but the accurate detection rate in different ethnic groups still needs further explored.Hematopoietic stem cell transplantation is a traditional therapy for radical cure of SCID.And the safety of gene therapy for SCID has been gradually guaranteed with continuous improvement during recent years.This article will review the classification, early screening methods and treatment progress of SCID.

Neuroblastoma(NB),the common extracranial solid tumor of childhood,is thought to derive from neural crest cells.In disialoganglioside (GD)2 is highly expressed on NB,whereas GD2 expression is weak and restricted to the central nervous system,peripheral pain fibers and skin melanocytes.Therefore,GD2 is an ideal antigen target for immunotherapy of NB.The research progress on GD2 as target for immunotherapy of NB was reviewed.

Objective Primitive neuroectodermal tumor ( PNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors, the purpose of the study was to investigate the clinical features, treatment and prognosis of Children PNET. Methods A retrospective study was performed on clinical data of 13 children with PNET hospitalized in our hospital from March 2010 to Octorber 2014.In order to analyze the clinical effects and prognostic results, statistical analysis was made on their clinical manifestations, CT and ultrasonic results, therapeutic schemes, postoperative pathology and immu-nohistochemical staining results, along with telephone and outpaitient follow-up of average 19 months. Results Of the 13 patients, 4 cases were central nervous system PNET(CNS-PNET) and 9 cases were peripheral PNET(pPNET).The clinical manifestations of the former were headache, vomiting, convolsion, movement disorder or decreased muscle strength, while the latter mainly showed mas-ses and abdominal distention.3 cases with metastasis to lung, lymph nodes and bones respectively showed masses on surface or in cavi-ty in imaging.Homer-Wright pseudorosette textures were found in 12 patients.Immunohistochemical results showed 11 cases with CD99(+), 9 cases with VIM(+) and 8 cases Syn( +).Among 11 patients underwent chemotherapy, 2 cases were lost to follow-up, 7 cases were in stable condition without occurrence and the other 2 cases recurred 4-6 months after chemotherapy.3 of 4 cases with CNS-PNET survived with the sequelae of decreased muscle strength, convolsion and movement disorder.1 case with CNS-PNET and 1 case with pPNET died 7 months and 16 months after diagnosis respectively. Conclusion PNET in children is a very highly malignant tumor with low long-term survival rate.Immunohistochemi-cal examination including CD99 and VIM is of important diagnostic value and CNS-PNET is prone to sequelae.

Objective To investigate the nursing of cancerous aching in patients during whole body hyperthermia. Methods Seventy patients with malignant tumors were carefully concerned before and after receiving whole body hyperthermia and their responses were recorded. Results The remission rate of aching was 88.55%, and the common side effects were burning of skin, bedsore, and nausea or vomiting. Conclusion The whole body hyperthermia therapy can relieve the cancerous pain, and good nursing can improve the efficacy.

Objective To analyze the imaging characteristics of one patient with primary progressive aphasia (PPA).MethodsThe patient was examined with MRI and functional MRI (fMRI) to perform the analyses of perfusion weighted imaging (PWI), magnetic resonance spectroscopy (MRS) and diffusion tensor imaging (DTI), and studied the changes of cerebral blood flow (CBF), metabolism, and association fibers of PPA.ResultsThere was frontal lobe and temporal lobe atrophy on general MRI, lower metabolism compared with contralateral on MRS; there were decreased association fibers between Broca domain and other domain, no association fibers between triangular area and Wernicke domain on DTI, mean fraction anisotropism (FA) and fibers of Broca and Wernicke domain decreased compared with healthy adult.ConclusionLeft temporal lobe and frontal lobe are the mainly diseased regions of PPA, and perfusion and metabolism are lower than the contral lobe, and the association fibers between Broca and Wernike domain are less than the control lobe.