Objective To report a pedigree of generalized atrophic benign epidermolysis bullosa (GABEB),a special type of junctional epidermolysis bullosa(JEB). This is the first case report in China. Methods The clinical, histologic, electron microscopic, and immunofluorescence changes of the disease were evaluated. Results Besides the symptom of inherited epidermolysis bullosa, the patient had special clinical manifestations such as atrophic alopecia and dental dysplasia. The disease was inherited in an autosomal recessive mode in this pedigree. Electron－ microscopy found the fissure in lamina lucida, accompaning with dysplasia and sparse hemidesmosome. Immunofluorescence study did not show BPAg2 in the basement membrane zone, indicating that mutation in the gene encoding BPAg2 leading to the disease in this family. Conclusions The case is diagnosed as GABEB, a special type of JEB. Although GABEB has its characteristic, clinical manifestations, electron microscopy and immunofluorescence study are important for confirmative diagnosis and classification, and will guide the study of mutation detection.

Objective To study the clinical efficacy and adverse reactions of different blue-light and expo-sure patterns for the treatment of neonatal hyperbilirubinemia.Methods 127 neonates with hyperbilirubinemia were divided into cold-light illuminator blue-light irradiation group (group A, n=32), cold-light illuminator blue-light group intermittent irradiation group (group B, n=32), traditional blue-light irradiation group (group C, n=31) and traditional blue-light intermittent irradiation group (group D, n=32).The treatment course of the four groups was all 72 hours.The level of serum total bilirubin ( TBIL) , direct bilirubin ( DBIL) and auditory brainstem response before and after treatment in the four groups were respectively observed and compared.The adverse reactions associated with treatment were recorded and compared among the four groups.After 9 months of follow-up, the audi-tory brainstem response ( ABR) was respectively tested at 3 days, 3 months and 9 months after the treatment.Results The level of TBIL in the four groups after treatment were significantly decreased compared with that before treatment (p<0.01), and the level of TBIL in cold-light illuminator blue-light group was significantly decreased compared with that in traditional blue-light group ( p<0.05 ) .The total effectiveness in cold-light illuminator blue-light group was significantly higher than that in traditional blue-light group ( p<0.05) .The incidence of adverse reaction in cold-light illuminator blue-light group was significantly lower than that in traditional blue-light group ( p<0.01), and the incidence of adverse reaction in group C was significantly higher than that in group D (p<0.05).At day 3 after treatment, the abnormal rates of ABR in the cold-light illuminator blue-light group was significantly lower than that in the traditional blue-light group (p<0.05).After 3 months of treatment, the abnormal rates of ABR was insignificantly different among the four groups (p>0.05).The ABR in the four groups returned to the nor-mal after 9 months of treatment.Conclusion The therapy of the cold-light illuminator blue-light irradiation has significant clinical efficacy in the treatment of neonatal hyperbilirubinemia.It can effectively reduce the hearing im-pairment of neonates, and the incidence of adverse reaction and total effective rates are equivalent in therapeutic pat-terns of intermittent irradiation and continuous irradiation.

In order to study the relationship of keratin 5 gene and Weber-Cockayne epidermolysis bullosa simplex (WC-EBS), we use polymerase chain reaction (PCR) amplification combined with single strand conformation polymorphism (SSCP) methods and DNA sequenceing to detect the point mutation of keratin 5 in a family of WC-EBS. The results showed that there is a G→T substitution at the third base pair of codon 327, which caused methionine changed into isoleucine. This abnormality of keratin structure makes the normal assembly of tonofilaments impossible. Furthermore, the above change resulted in blister formation and epidermolysis clinically. This indicates that PCR-SSCP is useful for the gene and prenatal diagnosis of genodermatoses and also provides the basis of theroies for gene therapy.

Objective To investigate phases in normal rabbit liver contrast enhancement with the acoustic densitometry(AD).Methods Ten cases of normal rabbit were examined with second harmonic imaging(SHI).The AD curves and data of parenchyma were compared before and after the portal blood flow was blocked,when the contrast agents were administrated through ear veins.The AD curves and data of parenchyma were also investigated when the contrast agents were injected via ear veins and mesenteric veins.Results The curves of normal rabbit liver parenchyma were single-peak-shaped,looking like "a verticle triangle",when the contrast agent was administrated through ear veins.The curve was divided into 3 sections: ascending,summit,descending,with the value of peak intensity ( 18.4 ? 1.7 )dB.Ascending section indicated the arterial phase,and summit and descending sections the portal phase.The AD curves were quite similar before and after the portal blood flow was blocked,only with the peak value slightly decreased,and the values of peak intensity decreasing by 2 dB.Two peaks were seen in the "Hump-like" AD curves in the parenchyma,with the second peak higher than the first,as the contrast agents were administrated through mesenteric veins again 10-15 seconds after administration via ear veins.Conclusions The arterial phase is short and portal phase is long in normal rabbit liver contrast sonography.The enhancement of parenchyma is caused mostly by contrast from hepatic artery,and the contrast from portal veins only plays a minor role.

Objective: To investigate the possible pathogenesis and report the postoperative results of the late tricuspid regurgitation (TR) after left cardiac valve replacement. Methods: 56 patients developed severe TR after left cardiac valve replacement, including 10 patients with normal prosthesis valve function (group A) and 46 patients with prosthesis valve dysfunction (group B). Four patients underwent mitral valve replacement (MVR) and 6 patients underwent mitral and aortic valve replacement (DVR) in group A. In group B, 36 patients received MVR, 4 aortic valve replacement (AVR) and 6 DVR. Ten patients underwent tricuspid De Vega annuloplasty and 46 patients' tricuspid valves were normal during the initial operation. The surgical treatment of tricuspid valve included tricuspid valve replacement (TVR) in 9 and tricuspid valve plasty (TVP) in 47 at the second operation. Results: Two patients died postoperatively with hospital mortality of 3.6%. The 54 survivors were followed up from 6 to 132 months, mean 79.4 months. The heart function improved significantly in 8 after TVR and 40 after TVP. However, echocardiography showed moderate TR in 5 and severe TR in 1 patient after TVP and medical treatment was needed. Conclusion: The sustained pulmonary hypertension, irreversible right heart impairment, resumption of left ventricular function and sustained atrial fibrillation may be responsible for the development of late TR after left cardiac valve replacement. TVR may achieve a reliable result for severe functional TR and rheumatic tricuspid valve lesion. In some patients with TVP during the follow up, the TR might become more serious.

Objective To establish a new atomic fluorescence spectrometry for the determination of As,Hg and Se in human gastric and intestinal juice. Methods The KBH4 solution was used as a reducing agent to generate hydrides of As,Se and metallic vapour of Hg from samples. Results The lineal range of the method was 5.00-80.00 ng/ml for As, 0.10-4.00 ng/ml for Hg, 0.50-5.00 ng/ml for Se respectively. The RSD was less than 6.0%. For As, Hg and Se, the limits of detection were 0.20, 0.01, 0.33 ng/ml respectively, the recovery rates were 98.6%-104.4%, 92.0%-104.0%, 98.5%-100.5% respectively. Conclusion The method is simple, rapid and accurate with satisfactory results.

This article reports the results of the quantitative analysis for the DNA content of cell nucleus of the skin lesion in patients with endemic arsenism by using the technique of flow cytometry, the skin lesions include palmoplantar hyperkeratosis, abnormal pigmentation on trunk and skin cancers. Our results showed that the DNA indices of the different skin lesions of patients in endemic arsenism were significantly higher than those of controls(P

To study a method for determination of sulfamethoxazole, sulfadiazine and trimethoprim tablets, a compound tablet of sulfamethoxazole, sulfadiazine and trimethoprim. METHOD: A capillary zone electrophoresis (CZE) method was used to assay three components of this compound preparation. RESULTS: The complete separation of components was achieved with 0.05 molL-1 pH 6.0 running phosphate buffer and a constant voltage of 20 kV (current of 95 μA～105 μA). The retention times of individual components were between three and eight minutes and a good linearity was shown between concentration and peak area in the concentration range over 70 μgml-1～750 μgml-1. When acetanilide was used as internal standard, the relative standard deviation (RSD) of each component determined in a batch was less than 1% (n=9). The recovery of each component was not less than 96.45% with RSD less than 3%. The analytical results obtained from 6 samples of clinical use were inconsistent with those by standard method, however the quantity of each sulfa drug was obtained by CZE method. CONCLUSION: The results showed this method is accurate, simple, and rapid. When this method is used, the quantity of each three components is determined, but by the standard method, only the total quantity of the two sulfa drugs is obtained.

Objective To investigate the efficacy of modified electrical superficial surround acupuncture in treating postherpetic neuralgia. Method One hundred and eighty-six patients with postherpetic neuralgia were randomly allocated to electrical acupuncture, superficial acupuncture and electrical superficial acupuncture groups, 62 cases each. All the three groups were given acupuncture at ashi points around the painful region. The electrical acupuncture group received electroacupuncture alone. The superficial acupuncture group received sweeping-dispersing manipulation and following 24-hour retention of the soft tube. The electrical superficial acupuncture group received sweeping-dispersing manipulation based on modified superficial acupuncture and following electroacupuncture, without retention of the soft tube after the completion of treatment. The therapeutic effects were evaluated and compared after one course of treatment. Result There was a statistically significant pre-/post-treatment difference in the VAS score in the three groups (P<0.01). There was a statistically significant post-treatment difference in the VAS score between the electrical superficial acupuncture group and the electrical acupuncture or superficial acupuncture group (P<0.01) but no between the electrical acupuncture and superficial acupuncture groups (P>0.05). The evaluation of the therapeutic effects according to WHO pain rating showed that the marked efficacy rate and the total efficacy rate were 45.2% and 82.3%, respectively, in the electrical acupuncture group, 52.3% and 88.7%, respectively, in the superficial acupuncture group and 62.9% and 91.9%, respectively, in the electrical superficial acupuncture group. There was no statistically significant difference in the total efficacy rate between the three groups (P>0.05). There was a statistically significant difference in the marked efficacy rate between the electrical acupuncture and electrical superficial acupuncture groups (P<0.05)but no between the electrical acupuncture and superficial acupuncture groups (P>0.05) and between the superficial acupuncture and electrical superficial acupuncture groups(P>0.05). Conclusion The therapeutic effect of electrical superficial acupuncture is better than that of superficial acupuncture and electrical acupuncture. Electrical superficial acupuncture is characterized by selection of few points, small number of treatments and meanwhile, avoidance of potential medical risks.