ObjectiveTo explore the relationship between the changes of neuro-electrophysiology and prognosis in children with Guillain-Barr? syndrome(GBS).MethodsThirty-eight children with GBS were divided into group A(rapid recovery,n=16) and group B(slow recovery,n=22) according to the time required for podosoma motor function recovery,at the same time,they were divided into the better prognosis group(n=22) and the worse prognosis group(n=16),for analyzing the difference between group A and B in terms of age,preceding infections,maximal Hughes grades and neuro-electrophysiology including motor conduction velocity(MCV),distal complex muscle action potential(dCMAP) and F wave,and investigating the related factors with the prognosis of GBS.Results1.MCV of tibial nerve was(40.2?2.53) m/s and(33.4?2.46) m/s in group A and group B,respectively;MCV of peroneal nerve was(45.2?3.23) m/s and(38.3?2.16) m/s in group A and group B,respectively,and the difference between group A and group B was significant(Pa0.05);abnormal rate of F wave(68.42%) was higher than abnormal rate of MCV(42.11%) and dCMAP(42.11%)(Pa

0.05).3.Thirty-three point three percent of animals from parental strain group were found fibrillations potentials and the po-sitive sharp waves in gastrocnemius electromyogram,no obvious abnormal waves were found in animals from both waaF mutant and control group.Conclusions The ganglioside-like epitope in LOS of CJ is critical antigen in inducing GM1-IgG antibody and in inducing conduction block of peripheral nerve,therefore,provide a support for the molecular mimicry theory as a pathogenesis in the axonal GBS following CJ infection.

With patients' general situation, medication use, occurrence time of adverse drug reaction/event (ADR/ADE), clinical manifestations and prognosis as reference items, a retrospective study was made for 315 cases with ADR/ADE induced by Gastrodin in Chongqing from January 2008 to June 2014, in order to analyze the characteristics of ADR/ADE and provide reference for rational clinical medication. The results showed that among the 315 cases with ADR/ADE, 143 cases (45.4%) were males and 172 cases (54.6%) were females, most of them (74.9%) were aged above 45; 60 cases (19.0%) with ADE were caused by off-label indications and 66 cases (21.0%) with ADE were caused by over dosage; ADR/ADE cases induced by intravenous drip mainly happened within 30 min (85.5%), ADR/ADE cases induced by oral administration mainly happened within 2 h (74.4%), and all of ADR/ ADE cases induced by intramuscular injection happened within 10 min. Totally 593 ADR/ADE cases were reported, which were mainly damages in gastrointestinal system, skin and its adnexa; And 61.9% of ADR/ADE cases were newly reported. It is suggested that medical workers shall learn about the regularity and characteristics of ADR/ADE induced by gastrodin, apply it in clinic with standards, pay close attention to changes of patients' situations and attach importance to the monitoring of ADR/ADE, so as to enhance the safety of medication.
Adolescent ; Adult ; Aged ; Benzyl Alcohols ; administration & dosage ; adverse effects ; Child ; Child, Preschool ; China ; epidemiology ; Drug-Related Side Effects and Adverse Reactions ; epidemiology ; Drugs, Chinese Herbal ; administration & dosage ; adverse effects ; Female ; Gastrointestinal Tract ; drug effects ; Glucosides ; administration & dosage ; adverse effects ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Skin ; drug effects ; Young Adult

0.05),but the numbers of CD41+ cells and platelets were increased significantly(P

Objective To summarize the clinical characteristics and diagnosis of hemiconvulsion-hemiplegia-epilepsy(HHE) syndrome.Methods The clinical data of 5 children with HHE syndrome were analyzed.Cranial magnetic resonance imaging MRI and /or computerized tomography(CT) and electroencephalogram(EEG) were done in all patients.Results The age of onset was less than 4 years in 4 of 5 patients and initial seizures were associated with fever in 3 cases.The longest duration of convulsive seizures lasted more than 2 hours in all cases,followed by the appearance of hemiplegia involving the side of the body where the convulsions were localized.Subsequent epilepsy developed within 2 years of the hemiplegia in all 5 cases,with 4 cases simple partial seizure and 1 case psychomotor seizures.All cases had mental retardation.Cranial MRI showed left hippocampal sclerosis in 4 patients and CT brain scan showed right cerebral hemiatrophy in 1 case.The EEG showed abnormality in all patients.Epileptiform discharges were found in 4 cases,and the damage hemisphere showed slowing and low voltage of background activity in 1 case.All cases received mainly carbamazepine therapy,and the seizures were satisfactorily controlled or partly controlled.Conclusions HHE syndrome is recognized as hemiplegia-epilepsy syndrome after prolonged hemiconvulsion.The hippocampal sclerosis may be the consequence of repeated seizures,but not the cause.Better understanding of the syndrome,early and adequate treatment of prolonged seizures,especially of febrile convulsion,will reduce the incidence of HHE syndrome.

Objective To report the outcome of emergency repair traumatized limbs by vascularized free tissue transplantation.Methods From April 1988 to August 2004,86 patients,58 men and 28 women,had undergone emergency vascularized free tissue transplantation to have their injured limbs repaired in 54 cases and the missing thumbs reconstructed in 32.The patients aged from 5 to 55 (mean 27.9) years. The transplants included latissimus dorsi myocutaneons flap,anterolateral femoral skin flap,medial crural skin flaps,dorsal pedal flaps,medial plantar flap,composite tissue mass of the discarded limbs and big toe skin- nail flap.The operations were performed 1 to 5 days after injuries.Results Postoperative vascular crises occurred in 8 cases and were all followed by exploration with successes in 5 cases while failure in 3.The total survival rate of the transplants was 96.5% (83/86).In this series all the patients were followed up for 1 to 16 years with a mean of 7.5 years only to reveal satisfying functional recovery in all the repaired limbs and an ex- cellent and good rate of 87.5% in the reconstructed thumbs.Conclusion Emergency vascularized free tis- sue transplantation is an effective way to repair a traumatized limb and to reconstruct a traumatically missing thumb.

Fifty-six patients with Graves' ophthalmopathy(GO)were treated with antithyroid drug and oral prednisone for three months,TSH receptor antibody(TRAb)level was reduced,GO activity and severity of some patients were ameliorated but still positively associated with TRAb.It suggests that TRAb not only triggers off GO but also plays a possible role in the maintenance of the autoimmune process in GO.

OBJECTIVETo improve the accuracy of the diagnosis of the disease on the basis of the clinical features and genetic characteristics of patients with Silver Russell syndrome (SRS).

METHODPatients diagnosed with SRS by Price criteria in 2006 to 2011 were reviewed for their clinical manifestations, physical signs, laboratory examinations and treatments.

RESULTTwenty cases with SRS were 0.08-12.17 yr old. Fifteen were male and 5 were female. The clinical characteristics included more than 80% of cases had postnatal growth retardation 100% (20/20), craniofacial dysmorphism 100% (20/20), small for gestation age 95% (19/20), asymmetry and thinning of the face and/or limbs 90% (18/20), fifth finger clinodactyly 80% (16/20), BMI < -2 SDS 80% (16/20). Their height was obviously lagging behind in the bone age. HD SDS/average of bone retardation was 3.08. The two patients with the chief complaint of external genital abnormalities would have aggressive surgical treatment and they did not use the growth hormone (GH) treatment. Only six patients had used the GH treatment. GH treatment at a dose of 0.1 IU/(kg·d) used in 2 cases achieved a growth velocity (GV) 8 - 11 cm/yr but in another 2 cases < 5 cm/yr. In genetic study, 6 patients were found to have 11p15 low methylation, 1 had low and high methylation, 1 had duplication, no relation between clinical and methylation of 11p15 was found.

CONCLUSIONThere were great variations of clinical features in SRS characterized by small for gestation age and/or postnatal growth retardation, craniofacial dysmorphism, asymmetry of the face and/or limbs or ultrafine limbs, fifth finger clinodactyly. Severely low BMI was seen and height was obviously lagging behind in the bone age. The findings of laboratory tests and imaging of SRS were not specific. Some of SRS had 11p15 imprinting defects. The treatment of SRS is mainly symptomatic.

Abnormalities, Multiple ; diagnosis ; genetics ; Adolescent ; Body Height ; Bone Density ; Child ; Child, Preschool ; Chromosomes, Human, Pair 11 ; genetics ; DNA Methylation ; Female ; Genetic Association Studies ; Genomic Imprinting ; Growth Disorders ; diagnosis ; genetics ; Humans ; Infant ; Male ; Retrospective Studies ; Silver-Russell Syndrome ; diagnosis ; genetics

OBJECTIVETo study whether hematologic malignancy patients with anemia have a lower erythropoietin (EPO) response.

METHODSSerum EPO levels were detected by ELISA in patients with hematologic malignancies and with iron deficiency anemia (IDA). Eighty patients with hematologic malignancies, including 13 multiple myeloma (MM), 7 chronic lymphocytic leukemia (CLL) and 60 non-Hodgkin's lymphoma (NHL) were studied. Thirty of them had anemia(21 NHL,6 MM and 3 CLL). Twenty patients with IDA were studied as the control.

RESULTSHematologic malignancy patients with anemia had higher EPO levels [(97.8 +/- 183.9) IU/L] than those with normal Hb values [(27.8 +/- 85.4) IU/L; P <0.01]. In patients with IDA, serum EPO response was inversely correlated with Hb level (r= -0.5, P <0.05) , but no such inverse correlation was found in the hematologic malignancy patients with anemia (r = -0.14). After corrected for Hb level, the serum EPO levels were significantly lower in anemic patients with hematologic malignancies than in IDA patients (P = 0.032) , indicating a decreased EPO response in the former group.

CONCLUSIONAnemia associated with hematologic malignancy might result from an inappropriately low EPO response. EPO treatment for these patients may be beneficial.

Adolescent ; Adult ; Aged ; Anemia, Iron-Deficiency ; blood ; complications ; Enzyme-Linked Immunosorbent Assay ; Erythropoietin ; blood ; Female ; Hematologic Neoplasms ; blood ; complications ; Hemoglobins ; metabolism ; Humans ; Male ; Middle Aged ; Prospective Studies

OBJECTIVETo study the effect of culture supernatant of alveolar macrophage alveolar macrophages (AM) stimulated by SiO2 on the expression of matrix metalloproteinases (MMP-1), tissue inhibitor of metalloproteinase-1 (TIMP-1) and collagen of fibroblast human embryonic lung fibroblasts (HELF) in the development of silicosis fibrosis.

METHODSAMs were collected from a silicotic patient by bronchoalveolar lavage and exposed to SiO2, cultured human embryo lung fibroblast were allocated into a treated group, a control group, a positive group, and a blank group. HELF was incubated with the cultured supernatant of AMs for 6, 12, 18, 24, 36, 48 h. Immunocytochemical and Western blot technology were used to detect MMP-1 and TIMP-1 expressions in HELF and collagen expression in supernatant of HELF respectively.

RESULTSThe supernatant of AM exposed to SiO2 significantly decreased the expressions of MMP-1 (0.0605 +/- 0.0201, 0.0519 +/- 0.0117, 0.0412 +/- 0.0105 and 0.0213 +/- 0.0106 in the treated group at 18, 24, 36 and 48 h) compared with the control group and the blank group (P < 0.05, P < 0.01) but stimulated expressions of TIMP-1 and collagen (P < 0.05, P < 0.01). The ratio of TIMP-1 to MMP-1 increased. The ratio of TIMP-1 to MMP-1 was positively correlated with the expression of collagen III (r = 0.88, P < 0.01).

CONCLUSIONThrough AM mediation SiO2 can accelerate the expression of TIMP-1 and collagen, and inhibit the expression of MMP-1. The imbalance between the expression of TIMP-1 and that of MMP-1 is related with the abnormal increase in collagen III.

Cells, Cultured ; Collagen Type III ; metabolism ; Fibroblasts ; drug effects ; metabolism ; Humans ; Macrophages, Alveolar ; drug effects ; Male ; Matrix Metalloproteinase 1 ; metabolism ; Middle Aged ; Silicon Dioxide ; toxicity ; Silicosis ; pathology ; Tissue Inhibitor of Metalloproteinase-1 ; metabolism