Asia ; Congresses as Topic ; Humans ; Pediatrics

Insulin receptor substrate(IRS)-1 and IRS-2 expression levels of liver tissues and skeletal muscle in intrauterine growth retarded(IUGR)rats were investigated by RT-PCR and immunohistochemistry.An IUGR animal model was established by maternal nutrition restriction during pregnancy.IRS-2 expression level of liver tissue and IRS-1 expression level of skeletal muscle in IUGR rats at 0 and 3 weeks old were significantly lower than those in normal rats at the same age respectively,and insulin resistance was induced in IUGR,and these findings might be the molecular mechanisms susceptible to metabolic syndrome in IUGR rats.

Objective To observe the effect of angiotensin-converting enzyme inhibitors (ACEI) on anemia and erythropoietin (EPO) requirements in maintenance hemodialysis patients. Methods Ninety maintenance hemodialysis patients with hypertension and anemia were divided into 2 groups by random digits table, observation group (45 cases, using ACEI as antihypertensive treatment), control group [45 cases,using calcium channel blocker (CCB) as antihypertensive treatment]. The follow-up period after starting ACEI or CCB therapy was one year. The hemoglobin concentration, serum EPO, EPO requirements were compared after 0, 2, 4, 6, 8, 10, 12 months' treatment. Results In response to ACEI, the mean hemoglobin value in observation group decreased progressively, reaching statistical significance after 6 months, and it had significant difference compared with that in control group [6 months: (94.21±9.20) g/Lvs. (105.55±9.16) g/L,12 months: (95.90±6.75) g/L vs. (105.81±4.45) g/L,P <0.05]. The EPO requirements experienced a progressive increase in observation group and reached statistical significance after 8 months, compared with those in control group [8 months: ( 10 090.75±1918.35) U/week vs. (7010.32±1600.15) U/week, 12 months: (11 586.39±2009.76) U/week vs. (7068.48±1615.35) U/week,P<0.05].Serum erythropoietin concentration remained stable during the study in two groups. Conclusion ACEI can worsen anemia and reduce the efficacy of EPO in maintenance hemodialysis patients.

Child ; Humans ; Puberty, Precocious ; diagnosis ; therapy

Medicine, Chinese Traditional

Aged ; Aged, 80 and over ; Anthracosis ; blood ; Erythrocyte Indices ; Erythrocytes ; Hemoglobinometry ; Humans ; Middle Aged

Human cytomegalovirus (HCMV) late mRNA expression in megakaryoblast and in turn the pathogenesis of idiopathic thrombocytopenic purpura (ITP) patients with HCMV infection, and effectiveness of ganciclovir were investigated. Colony forming unit-megakaryocytes (CFU-MK) of 46 ITP patients with HCMV infection were incubated from patients' bone marrow mononuclear cells (MNC). Reverse transcriptase-polymerase chain reaction (RT-PCR) was subsequently used for CFU-MK for HCMV-late mRNA detection. Ganciclovir therapy was given to both HCMV-late mRNA positive and negative groups for comparison of therapeutic effectiveness. The results in 19 of 46 CFU-MK culture cells specimens with positive HCMV-DNA by PCR or positive CMV-IgM by enzyme linked immunosorbent assay (ELISA) in the correspondent serum of peripheral blood were positive for HCMV-late mRNA. Sixteen out of 19, patients with positive HCMV-late mRNA CFU-MK had a positive response to ganciclovir. Amongst 27 patients with negative HCMV-late mRNA CFU-MK, only 4 positive responders to ganciclovir therapy were observed. Curative effectiveness of ganciclovir in HCMV-late mRNA positive group was significantly higher than that in HCMV-late mRNA negative group (P<0.01). It was suggested that HCMV could directly infect CFU-MK, which might be one of the mechanisms responsible for HCMV related ITP. The ganciclovir is an effective therapy in resulting in the increases in thrombocyte in the ITP patients whose HCMV- late mRNA was positive in their CFU-MK.

To report a boy with reflex seizures induced by micturition.He suffered from seizures during micturition for four yesrs.The clinical symptoms consisted of head backwards,limbs dithering,sometimes accompanying tumble.During the episodes of the seizure,his consciousness did not lose.Few seizures showed only eyes up-staring for several seconds.The livelong process of micturition was often interrupted by seizures for several times.When he finished or stoped micturition,the seizures did not occur again.His seizures could not be induced by free of micturition or other stimulative factors.The interictal EEGs showed that background asymmetry manifested as the lower amplitude and irregular rhythm in the left hemisphere,sharp or sharp and waves in right hemisphere,especially mid and back temporal.There also had sharp,spike,and spike and waves in many regions of double spheres in interictal EEGs.The other assistant check-ups were normal.while this disease was infrenquncy,it would be worthy to regard.

Objective To investigate the role of electromyography(EMG) in diagnosis of nervous system diseases in children.(Met)-hods EMG tests were carried out in 354 patients with nervous system diseases,and the data and results of EMG tests were analyzed.Results One hundred and sixty-six patients′ results of EMG were abnormal.Among these abnormalities,36 cases were myogenic,47 cases were neurogenic,abnormalities of 69 cases were located in peripheral nerves,3 cases got positive in repetitive nerve stimulation(RNS),and 11 cases were on the borderline.In 36 myogenic patients,clinical diagnosis were as follow: progressive muscular dystrophy(PMD,18 cases),polymyositis(2 cases),mitochondrial encephalomyopathy(1 case), and the other 15 cases had no definite diagnosis.In 47 neurogenic patients,the diagnosis were spinal muscular atrophy(SMA,29 cases),sequela of poliomyelitis(2 cases),acute transverse myelitis(ATM,4 cases),and the other 12 patients had no definite diagnosis.In 69 cases of peripheral nerve abnormality,diagnosis were injury of brachial nerve(23 cases),hereditary motor sensory neuropathy(HMSN,2 cases),Guillain-Barre syndrome(GBS,9(ca)-ses),chronic inflammatory demyelinating polyradiculoneuropathy(CIDP,1 case),injury of facial nerve(4 cases),injury of common(pe)-roneal nerve(6 cases),metachromatic leukodystrophy(MLD,1 case),and the other 23 patients had no definite diagnosis.Three patients who got RNS positive were all diagnosed myasthenia gravis(MG),and ocular type(1 case),general type(2 cases).Eleven patients whose EMG results were borderline were all diagnosed indefinitely.One hundred and eighty-eight patients had normal results of EMG test.The diagnosis of these patients were included ocular MG(21 cases),cerebral palsy(CP,5 cases),ATM(2 cases),polymyositis((1 case)),and some other nervous system diseases(21 cases),and the other 138 were diagnosed indefinitely.Conclusions 1.EMG plays an important role in definite diagnosis of PMD,SMA,poliomyelitis and nerve injury;2.EMG can provide clue or basis in the differential(dia)gnosis of nervous system diseases which involved lower motor unit;3.EMG test has very low positive results in children with MG;(4.EMG) has little help in diagnosis of diseases involved upper motor unit only.

Objective To analyze the ABCD1 gene mutations in 5 cases of X-linked adrenoleukodystrophy(X-ALD) patients and 2 cases of their mothers.Methods Of 5 patients with X-ALD,10 exons and flanking intronic sequences of ABCD1 gene were amplified by polyme-rase chain reaction,and then sequenced directly.The outcomes were compared with normal ABCD1 sequencings to identify the mutation type and site.Thirty normal men were examined in the mean time as control for the confirmation of mutations and gene polymorphisms.Results Three patients showed ABCD1 gene mutations,1 had a point mutation in exon 6,Arg518Gly(CGG→GGG);2 patients showed the same novel mutation in exon 1 with 8 bases deletion(134del8).Four gene polymorphisms were identified in exon 7.They were Gly551X(GGC→GGT),Arg554His(CGT→CAT),Gln567Arg(CAA→CGA) and Val582Ile(GTC→ATC).ABCD1 gene mutation was not found in 2 mothers from 2 unrelated fa-milies with X-ALD.Conclusions Three cases of 5 were detected for ABCD1 gene mutations.Between them,the 134del8 mutation is a novel one.Four new gene polymorphisms were detected in exon 7 in normal Chinese people,which were Gly551X,Arg554His,Gln567Arg and Val582Ile.