1997年 · 第110卷第08期

OBJECTIVE To investigate the effects of NR1 subunit on the initiation and development of seizures and protection of cortical neurons from excitotoxicity by using antisense oligodeoxynucleotides (ODN) to NR1 in vivo and in vitro. METHODS Intracerebroventricular injection, temporal cortex slices discharge, cerebral cortical neuronal culture, induction of neurotoxicity and [3H]MK-801 binding were used in this study. RESULTS After an antisense ODN for NR1 was administered intracerebroventricularly (i.c.v. 100 μg in 10 μl) once daily, for three days in genetically epilepsy-prone rats (GEPR, P77PMC), the animals did not develop any clonic and tonic convulsions and their seizure scores were significantly lower compared to the control groups. The frequency and amplitude of early seizure-like events (SLEs) and late recurrent discharges (LRD), induced by lowering Mg2+, were reduced in entorhinal cortex (EC) of the temporal slice treated by antisense ODNs. Pretreatment with antisense ODN (2 μM) protected more than 52% of glutamate-sensitive neurons and reduced the [3H]MK-801 binding to 50% in cultured cerebral cortical neurons. CONCLUSIONS N-methy-D-aspartate-receptors (NMDAR), specifically the NR1 subunit, may participate and play important roles in the initiation and propagation of epilepsy in the P77PMC rat.

OBJECTIVE To detect the distribution of α5 (Ⅳ) chain of collagen on the glomerular basement membrane (GBM) and epidermal basement membrane (EBM) in the Chinese Alport syndrome (AS) kindreds and to develop a simple diagnostic alternative method to electronic microscopy for diagnosis of AS. METHODS Four male patients from 4 unrelated families manifested with hematuria, sensorineural hearing loss and had distinct family history. All patients had the characteristic AS pathologic changes by electron microscopy on their renal biopsy tissues. Three normal skin samples and 2 normal kidney samples were used as normal controls. Monoclonal antibody-based IF test was performed to examine the α5 (Ⅳ) NC1 domain in EBM of normal controls, X-linked AS patients and their parents, and in GBM of normal controls and AS patients. RESULTS In normal controls as well as the patients' fathers, all the monoclonal antibodies used in EBM and GBM staining showed positive reactions along basement membranes in a linear pattern. Characteristically, in AS patients there were negative reactions to monoclonal antibodies anti-α5 (Ⅳ) NC1 domain in EBM and anti-alpha 3-5 (Ⅳ) NC1 domains in GBM. In patients' mothers, α5 (Ⅳ) chain was distributed segmentally in EBM. CONCLUSIONS The staining of α5 (Ⅳ) NC1 domain in EBM by IF can be used to diagnose patients and screen defect-gene carriers of X-linked AS.

OBJECTIVE To review the paediatric upper gastrointestinal endoscopy service in a paediatric department in Hong Kong. METHODS Records of all endoscopies undertaken in this department from May 1995 to January 1996 were retrieved and analysed. RESULTS The commonest indication for upper endoscopy was dyspepsia with 88% positive histological findings. Helicobacter pylori (H. pylori) was found in 25% of this group of children. CONCLUSIONS Paediatric upper gastrointestinal endoscopy service is invaluable in a paediatric department by providing useful diagnostic information which would otherwise be missed.

OBJECTIVE To determine the effects of transforming growth factor-β3 (TGF-β3) on expressions of surfactant proteins (SPs). METHODS Northern blot hybridization and reverse transcriptase polymerase chain reaction (RT-PCR) were employed to determine the SP-A, SP-B and SP-C mRNA levels of cultured lung explants and type Ⅱ cells from fetal rat lungs. RESULTS TGF-β3 alone did not affect the expressions of SPs either in explants or in type Ⅱ cells. However, TGF-β 3 did inhibit the increase of SP-B, SP-C mRNA levels caused by dexamethasone (100 nmol/L), in a dose-dependent manner. Furthermore, TGF-β3 had no direct inhibition on the increase of SP-B, SP-C mRNA induced by dexamethasone in type Ⅱ cells, but had an indirect inhibitory effect mediated by fibroblasts. CONCLUSIONS TGF-β3 plays an important inhibitory role in the development of pulmonary surfactant proteins. The present study may provide a possible explanation for the unideal effect of glucocorticoids in the prevention of neonatal respiratory distress syndrome.

OBJECTIVE To determine antiganglioside antibodies (AGA) in cerebrospinal fluid (CSF) of children with neuropsychiatric lupus erythematosus (NPLE), and to investigate their association with clinical manifestations and other laboratory tests. METHODS An enzyme linked immunoabsorbent assay was used to detect AGA in 22 CSF samples obtained from 18 children with systemic lupus erythematosus (SLE). These patients were further classified into clinical active NPLE (9 patients) and SLE (9 patients) groups. Indirect immunofluorescent assay was used for determination of CSF antinucleus antibodies (ANA). RESULTS AGA could be found in CSF of all the 9 active NPLE children who had neuropsychiatric manifestations within 6 months before or after AGA was tested. A clear association was observed between AGA and brain CT scan. No detectable ANA was found in CSF samples even though high titer of ANA was present in corresponding serum samples of SLE patients. CONCLUSIONS Detection of AGAs in CSF, more sensitive and specific than routine CSF examination and EEG, and positively associated with brain CT scan, is a simple and practical test for early judgment of childhood NPLE.

OBJECTIVE To explore the relationship between heart failure and changes in intracellular calcium concentration of the myocardium. METHODS The intra-and extracellular concentration of ionized calcium and total calcium of myocardium in 11 cases of heart failure was measured using calcium fluorescence indicator Fura-2 and atom absorption spectrophotometry. The activity of the erythrocyte membrane pump was determined with hemolysate chemical method. RESULTS The concentration of ionized calcium in myocardial cells and the erythrocyte was significantly higher in the patients with heart failure (280.85±47.8 nmol/L, 1.76±0.04 F335/F385) than in those without heart failure (121.88±13.15 nmol/L, 1.47±0.08 F335/F385). Total calcium in the erythrocyte was also increased markedly in the patients with heart failure, but the activity of the erythrocyte membrane pump was lower than in those without heart failure. The intracellular calcium of the peripheral erythrocyte and the activity of membrane pump returned to normal after the heart failure was cured. CONCLUSIONS There is excessive calcium accumulation in the myocardium and erythrocyte and the latter may be cause of the disturbance of myocardial diastolic function during heart failure.

OBJECTIVE To investigate the actions of beta-blocker (atenolol) and ACE inhibitor (enalapril) for the secondary prevention of the main cardiac complications after acute myocardial infarction (AMI). METHODS 1106 cases of AMI from 7 hospitals in the Beijing area were collected and were divided randomly into three groups: control (group C), atenolol (group A), and enalapril (group E). Drugs for investigation were administered 2-4 weeks after the onset of AMI, and the subjects were followed up for a median period of 19 months. All patients were given aspirin 50 mg/day. The end points of observation were cardiac events and non-cardiac events. Cardiac events included sudden cardiac death (SCD), heart failure death, total cardiac deaths, and myocardial re-infarction. RESULTS The clinical conditions of the three groups were compatible. Sixty-six cardiac events (6.0%) occurred. Comparing with group C, the rate of SCD decreased significantly by 68% in group A after atenolol treatment for 28 months. Both atenolol and enalapril significantly increased left ventricular ejection fraction (LVEF), whereas in group C the LVEF did not change during the follow-up peroid. There was obvious decreasing tendency of the survival curve in group C, compared with the other two groups. Totally drugs decrease one cardiac death‰/month. But the rate of myocardial re-infarction was the same in the three observed. CONCLUSION Both atenolol and enalapril (domestic products) are beneficial to the secondary prevention of SCD and heart failure death after AMI, but not to re-infarction. Both drugs should be continued for a prolonged period to be effective. Drugs given 2-4 weeks after acute stage are also effective, with no serious side effects.

OBJECTIVE To analyse the relationship between the number of T-cell receptor (TCR) γ gene rearrangement and clonality of malignant cells in lymphocytic leukemia and lymphoma. METHODS The TCRγ gene characteristics of 73 cases of lymphocytic leukemia and lymphoma and other diseases that had presented 1 or 2 prominent bands of amplified TCRγ VI subgroups-J1/2 gene rearrangement (GR) were detected by polymerase chain reaction-restriction enzymes (PCR-RE), heteroduplex formation (HDF), DNA sequencing and single-strand conformational polymorphism (SSCP). RESULTS Thirty-four percent patients had 2 of TCRγ GR (biallelic rearrangement); twenty-six percent had 1 of TCRγ GR; fifty-four percent of acute lymphocytic leukemia's and nineteen percent of non-Hodgkin's lymphomas (NHL) had 2 of TCRγ GR. HDF could rapidly confirm more than 1 of alleles while more alleles were difficult to be recognized by restriction analysis. Sense and antisense strands of heteroduplex were composed of 2 of TCRγ GR respectively. By combining HDF method, oligoclonalities in three patients (2 acute lympholytic leukemia and 1 myelodysplastil syndrome and clone evolution in one NHL were found. CONCLUSIONS The fact that many patients had 2 of TCR GR means that complications of two neoplastic clones can be affirmed only if more than 2 of TCR GR are found. HDF can be used to detect the clonality, oligoclonality and polyclonality of lymphoid cells in lymphocytic leukemia and lymphoma by analysis of the difference of gene segments. HDF is a reliable method for the clone evolution research of lymphoid malignancies in progression.

OBJECTIVE To document the recent surgical results in the treatment of primary hyperparathyroidism (HPT) at Queen Mary Hospital. METHODS From 1995 to 1996, 30 patients underwent 31 operations for primary HPT. The data of 11 men and 19 women with ages ranging from 19 to 86 years (median: 57 years) were prospectively recorded with emphasis on the need of preoperative localization. RESULTS Symptoms of hypercalcemia were present in 20 (67%) and complications in 17 (57%) patients, respectively. Seventy-seven localization studies were performed in 28 patients (average: 2.6 tests/patient). Localization was accurate in 12 of 23 (52%) ultrasonographies, 11 of 26 (42%) CT scans and 16 of 27 (59%) scintigraphies. Twenty-six patients had a single adenoma excised while 3 patients with multiple endocrine neoplasia type I (MEN I) had subtotal parathyroidectomy for multiglandular hyperplasia during cervical exploration. Immediate normocalcemia was achieved in 29 patients. One patient had persistent hypercalcemia due to a supernumerary fifth gland in the superior mediastinum that was successfully excised in a second operation. One patient had a unilateral vocal cord paralysis and 4 patients needed calcium supplement on discharge. During a median follow-up of 5 months, all patients were normocalcemic with one requiring calcium supplements. CONCLUSIONS Surgical treatment for primary HPT is a safe procedure and is associated with a high success rate. In our experience routine preoperative localization study is not cost-effective.

OBJECTIVE To evaluate conservative management of patients with histological incomplete excision of CIN after large loop excision of transformation zone (LLETZ). METHODS Two hundred and seventeen patients with high grade CIN were treated with LLETZ from October 1, 1992 to December 31, 1994. Fifty-three patients (24.4%) had incomplete excision on histology. All patients were followed up cytologically every 3 to 6 months during the first 18 months and then yearly till December 1996. Those patients with positive endocervical margins were followed up with both cervical smear and endocervical smear. RESULTS The mean follow-up duration was 30.4 months. Ten patients were found to have persistent or recurrent disease. Five patients had second LLETZ and complete excision was achieved in 4 of them, one patient had cone biopsy and two had hysterectomy. One patient was found to have stage I a cervical cancer. CONCLUSIONS A report of incomplete excision of CIN after LLETZ calls for follow-up with cytology and colposcopy and not aggressive retreatment.

The second National Congress of Young Pediatricians was held in Yichang from the 21st to 22nd of October, 1996, organized by the National Pediatric Society, Chinese Medical Association. Young pediatricians from various municipalities and most of the provinces and autonomous regions submitted papers. During the first round of review, 259 papers were invited to Yichang to verbally present their papers. Finally authors of 8 papers were awarded the first prize, 16 papers received the second prize and 32 papers the third.