Benign childhood epilepsy with centro-temporal spikes (BCECTS): early onset of seizures is associated with poorer response to initial treatment.

AIM: Benign childhood epilepsy with centro-temporal spikes (BCECTS) is the most common idiopathic partial epilepsy in children. Treatment attitudes remain a controversial issue. We examine features that could suggest refractoriness at onset. METHODS: We retrospectively reviewed the medical records of 144 children with BCECTS diagnosed at the Division of Pediatric Neurology, Asan Medical Center, from March 1, 1995, to April 30, 2002 and treated with AEDs. The patients were subdivided into two groups according to the number of antiepileptic drugs used for effective seizure control. RESULTS: Of the 144 patients, 75 were male and 69 were female, with a mean age at seizure-onset of 7.2 +/- 2.3 years (range, 2.1-14.3 years); 119 children were taking one antiepileptic drug (AED) (Group A), and 25 were taking more than one (Group B). There were no significant group differences in female-to-male ratio, prescribed AEDs, number of seizures before the start of treatment, interval between seizure-onset and start of treatment, presence of secondarily generalized seizures, or presence of bilateral EEG abnormalities. The groups differed however, in mean age at seizure onset (7.6 +/- 2.2 years versus 5.1 +/- 1.9 years, p < 0.05) and percentage of patients with seizure-onset before 3 years (p < 0.05). CONCLUSIONS: When treated with AEDs, children with BCECTS usually respond well. However, an earlier onset of seizures is associated with more frequent seizures and initial refractoriness to medical treatment.     

Lateral femoral condyle osteochondral fracture caused by a patella luxation: advantages and disadvantages of PLA fixation

A lateral patella luxation is occasionally complicated by an (osteo)chondral fracture. These fractures are usually located at the inferomedial part of the patella. In this case report we describe an osteochondral fracture of the lateral femoral condyle after a dislocation of the patella. The latter is a more severe injury because it involves an important weight bearing part of the knee joint. Refixation of the osteochondral fracture should be pursued. Various surgical techniques using nonresorbable materials have been described. We describe the advantages and disadvantages of refixation with biodegradable pin fixation for an osteochondral fracture caused by a patellaluxation.     

Nevus Lipomatosus Cutaneous Superficialis of the Clitoris

BACKGROUND: Nevus lipomatosus cutaneous superficialis (NLCS) is a relatively rare condition. Although NLCS can affect any region of the entire body, no case of NLCS in the clitoris has ever been reported. OBJECTIVE: To report a case of NLCS of the clitoris that presented manifestations that were similar to female pseudohermaphrodism. METHODS: This is a case report and review of the literature. RESULTS: A congenital, soft, skin-colored lesion of the clitoris in a 1-year-old girl resembled female pseudohermaphrodism. It was resected, and the histologic diagnosis was NLCS. Postoperatively, there was no subsequent change in the lesion during the 3-year follow-up period. CONCLUSION: We reported a case of congenital solitary NLCS of the clitoris. Although NLCS might be a relatively rare condition, we should consider it in the differential diagnosis of female pseudohermaphrodism.     

The epidemiology and surveillance of blood lead in Taiwan (R.O.C.): A report on the PRESS-BLL project

 To monitor the lead hazards in industries and to investigate the prevalence of elevated blood lead levels (BLLs) in lead-exposed workers, a lead surveillance system (PRESS-BLLs) has been established and operated in Taiwan, Republic of China, since July 1993. A cohort of lead-exposed workers who received a periodic annual health examination at 55 accredited hospital laboratories was constructed. A total of 9807 separate BLL measurements were reported to the system in 1994. The mean BLL was 15.8 μg/dl in male workers and 11.6 μg/dl in female workers. The mean BLL of lead-exposed workers was significantly (P<0.05, z-test) higher than that of the general Taiwanese population (8.6 μg/dl for males and 6.7 μg/dl for females). In addition, the BLLs of 983 (10.0%) workers exceeded the regulatory action level (40 μg/dl for males; 30 μg/dl for females). The workplaces and homes of 57% of the workers with elevated BLLs were thoroughly investigated to determine the sources of lead contamination. These actions identified the causes of elevated BLLs and set up strategies to reduce workers’ lead exposure. The establishment of this occupational lead surveillance system represents a method for monitoring of lead hazards from occupational and environmental settings to prevent lead poisoning. The information acquired from the system can help in the setting up of a priority of prevention and the development of control measures. It is also useful for further monitoring of changes in the BLLs of the lead-exposed-worker cohort. The Health Department of Taiwan can use this information to evaluate the effectiveness of current industrial hygiene practice. Subjects with elevated BLLs have been medically treated and placed on long-term follow-up for sequelae. Received: 2 September 1996/Accepted: 29 November 1996     

Purification and chemical modification of porcine bone morphogenetic protein

Implantation of porcine bone morphogenetic protein (pBMP) in the muscle induces differentiation of mesenchymal-type cells and results in endochondral bone formation. pBMP was isolated from porcine demineralized bone matrix and purified by hydroxyapatite chromatography, Sephadex G75 gel filtration, preparative sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE), preparative isoelectric focusing (IEF), and chromatofocusing fast protein liquid chromatography (FPLC). Porcine BMP has an MW of 26 K and a range of pI from 4.65 to 4.73 determined by SDS-PAGE and IEF, respectively. Reconstitution with the citrate buffer supernatant fraction enables as little as 50 micrograms of the soluble pBMP fractions to induce osteogenesis in an in vivo assay. Chemical modification studies indicate that the osteoinductive potential of the pBMP molecule depends on tyrosine, carboxyl groups, and disulfide bonds and can be increased by modification of sulfhydryl groups. Modification of arginine and tryptophan has no effect on bioactivity. By pepsin-limited proteolysis, fragments of pBMP with an MW of 6-14 K show definite, although reduced, BMP activity.     

Selective intra-arterial methylene blue injection: a novel method of localizing gastrinoma.

A 40-year-old woman had persistent Zollinger-Ellison syndrome despite excision of a 4-cm duodenal gastrinoma. Localizing studies including ultrasonography, computed tomography, magnetic resonance imaging, duodenal endoscopy, endoscopic ultrasonography, and intraoperative endoscopic transillumination of the duodenum failed to detect a tumor. Selective intra-arterial methylene blue injection was used to identify a 6-mm gastrinoma in the duodenum, which was locally excised. Postoperatively, the patient had a negative secretin provocative test result. This novel method uses selective arterial secretin injection with hepatic venous gastrin sampling to identify the vessel feeding the gastrinoma. An angiographic catheter is then positioned in this artery. At laparotomy, methylene blue is injected through this catheter to selectively stain the gastrinoma, facilitating its identification. Selective intra-arterial methylene blue injection can enhance intraoperative detection of small gastrinomas and may improve the rate of curative resection in the Zollinger-Ellison syndrome. Further evaluation of this novel localizing technique is warranted.     

Effects of posts on dentin stress distribution in pulpless teeth.

A finite element analysis was carried out to study the roles of posts in reducing dentin stress in pulpless teeth. Two-dimensional plane strain models of the midlabiolingual section of a human maxillary central incisor were first analyzed. The results showed that the gold alloy post reduced maximal dentin stress by as much as 30%. However, the integrity of the dentin was compromised and the effects of the post were likely to be exaggerated in such models. In an effort to correct for these problems, plane stress models with side plates and axisymmetric models were analyzed. Posts were found to reduce maximal dentin stress by only 3% to 8% when the teeth were subjected to masticatory and traumatic loadings in these latter models. Although posts reduced maximal dentin stress by as much as 20% when the teeth were loaded vertically, teeth such as incisors and canines normally are not subjected to vertical loadings. Thus the reinforcement effects of posts seem to be doubtful in these teeth.     

Restriction fragment length polymorphism analysis to study the genetic origin of complete hydatidiform mole.

To determine the genetic origin of the complete hydatidiform mole, 20 abnormal pregnancies were studied with restriction fragment length polymorphism with five genomic probes: EJ 6.6, beta-globin gene, 3'alpha-hypervariable region, J-Bir, and St14. In the 12 cases of molar pregnancy, pure paternal origin was proved in 11 cases, but both maternal and paternal inheritance were shown in only one case. In the cases with pure paternal origin, all of the restriction fragment length polymorphisms were homozygous, although those of the fathers were heterozygous at 15 loci. In the four cases that mimicked hydatidiform mole but were diagnosed as hydropic change of villi, both paternal and maternal inheritance were noted. In the four pregnancies with blighted ovum, both paternal and maternal inheritance were shown in three cases; and in one case with a balanced translocation between chromosomes 13 and 14, only paternal inheritance was noted. This study showed that most of the complete hydatidiform moles were caused by fertilization of an empty egg by a duplicated haploid sperm, but rare exceptions may exist.     

The value of ventilation scintigraphy after single lung transplantation.

BACKGROUND: A decrease in forced expiratory volume in 1 second (FEV(1)) as a diagnostic criterion for bronchiolitis obliterans syndrome (BOS) after single lung transplantation may be influenced significantly by the presence of the native lung. To quantify and to discriminate between the relative contribution of graft and native lung to the FEV(1), we retrospectively investigated the diagnostic value of combined FEV(1) measurements and ventilation scintigraphy in pulmonary dysfunction after single lung transplantation in 11 recipients with pulmonary vascular disease, 3 with obstructive lung disease, and 3 with restrictive lung disease. METHODS: We assessed function of the native lung and the graft, and subsequently calculated an adjusted grading of BOS by correcting routine FEV(1) measurements using linear interpolation of bi-annual lung ventilation scans. RESULTS: The contribution of the native lung to the total FEV(1) was slight (median, 9%) in recipients with obstructive disease compared with recipients with vascular (38%) or restrictive lung diseases (27%). Adjusted BOS grading was not useful in patients with obstructive disease. In the other patient groups, the onset of adjusted BOS Grade 1 and standard BOS Grade 1 was at a median of 220 days (range, 127-1146 days) and 836 days (184-3065 days), respectively. CONCLUSION: Ventilation scintigraphy is a useful adjunct in the (early) diagnosis of BOS in recipients of single lung transplants who have vascular and restrictive lung diseases.     

Status and trend of HIV-1 infection and AIDS in Taiwan, December, 1991.

From May 1, 1985 to December 31, 1991, a total of 4,962,707 serum samples from 8 population groups in Taiwan were tested for anti-human immunodeficiency virus type 1 (anti-HIV-1). In total, 256 samples were seropositive; of these individuals, 43 developed acquired immunodeficiency syndrome (AIDS): 29 were homosexuals; 5 were hemophiliacs; 8 were heterosexuals and 1 was of unknown risk. Although the prevalence of HIV-1 infection and AIDS remains low compared with other countries, since 1988 the increase has been rapid. Before 1977 the majority were homosexuals and hemophiliacs; thereafter the risk groups diversified, with a trend away from homosexuals and hemophiliacs towards heterosexuals and intravenous drug abusers (IVDAs). A few patients have caused serious social problems for the public, health care workers and families. Active community efforts are needed to achieve future success in the control of HIV-1 infection and AIDS in Taiwan.