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

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.

Objective To explore the influencing factors for the purity and viability of primary cultured cortical neurons of rat,and optimize the separating and cultivating conditions of the cortical neurons.Methods The primary cotical neurons were cultured in a serum-free culture system of B27-supplemented neurobasal medium.The differences in purity and viability of primary cultured neurons between embr-yonic rat group and newborn rat [postnatal 24 h and 5 d] group were evaluated by morphology,immunocytochemistry of neuron-specific enolase(NSE) and trypan blue staining.The changes of neurons purity and viability in different trypsin digestion time(0 min,5 min and 15 min) at different environment temperatures(20 ℃ and 30 ℃) were assessed by immunocytochemistry and trypan blue staining.Results The primary cultured neurons from fetal and newborn rats grew well.There was no significant difference in embryonic rat and postnatal 1 d newborn rat group[(91.30?1.03)%,(89.50?1.78)% respectively in purity;and(98.20?0.58)%,(97.10?0.98)% respectively in viability].The neurons from 5-days newborn rat were inferior to that from fetal and 1-day newborn rat in purity and viability[(82.00?1.25)% and(92.87?1.56)% respectively].Shortening operation time and adjusting digestion time according to the environment temperatures could improve neuronal viability:A digestion for 15min at the environment temperature of 20 ℃ or for 5 min at 30 ℃ could acquired cells with higher viability [(98.20?0.58)% and(96.70?0.64)% respectively].Conclusions It is an easy,practical choice to culture priamry cortical neurons from postnatal 1 d newborn rats.Optimizing the separating and cultivating condition(environment temperatures,digest time,et al.) will improve the neurons purity and viability.