Carbamazepine-exacerbated epilepsy with multifocal shifting independent epileptiform discharges on electroencephalogram: A case report

Abstract A 7 year old girl with epilepsy and spastic quadriplegia secondary to an episode of status epilepticus at 4 months of age is reported. At the age of 6 years, she began to experience increased generalized myoclonic and tonic seizures during treatment with carbamazepine (CBZ) 200 mg/day and clonazepam 1.5 mg/day. When the CBZ was increased to 400 mg/day, the seizures increased dramatically in frequency. Following discontinuation of CBZ, the seizure frequency decreased to a level less than that prior to starting CBZ. Serial electroencephalograms displayed multifocal independent epileptiform discharges (MIED) characterized by shifting localization, which could be one of the risk factors for exacerbation by CBZ. In this case MIED may indicate widespread rather than localized cerebral dysfunction.     

Developmental change in carotid artery blood flow waveform by component analysis in children

The carotid artery blood flow waveform (CABFW) is regarded as a summation of cardiac impulse responses. These impulse responses are divided into several components through a two-dimensional autoregressive modelling approach. Using this approach, we determined the developmental change in CABFW in 94 normal subjects from the neonatal period to adolescence. Our analysis demonstrated that: (i) the total power of impulse response increased significantly with increasing age. The component of impulse response was divided into six groups according to the damping frequency: group I (0 Hz), group II (1–5 Hz), group III (5–8 Hz), group IV (8–13 Hz), group V (13–17 Hz) and group VI (> 17 Hz); (ii) the power-density and the damping time of group I and II impulse response increased significantly with increasing age; (iii) the power-density and percent power of group III impulse response and power-density of group IV impulse response increased significantly with increasing age. Our results indicated that CABFW contained some regular impulses and that group I, II, III and IV, which were influenced by several factors, including cardiac contraction and the compliance and frictional forces of the carotid artery, appeared to be important to the developmental change of CABFW in children.     

SUCCESSFUL SURGICAL MANAGEMENT AND LONG-TERM FOLLOW-UP OF EPIDERMOLYSIS BULLOSA

Background. Esophageal stenosis and hand deformity are serious complications of recessive dystrophic epidermolysis bullosa that influence the prognosis of patients. To control such complications we have used surgical treatments with favorable results. Additional objectives were to summarize the results of long-term follow-up. Methods. Six patients with esophageal stenosis and nine patients (13 hands) with hand deformity (contracted fingers, mitten-like deformity) were treated surgically after anemia and malnutrition were corrected by intravenous iron infusion, high-energy diet, and blood transfusion. As intubation was contraindicated, topical anesthesia was used (eg., lidocaine spray to the nasopharyngeal mucosa for esophageal dilatation and brachial block, ketamine drip, and nitrous oxide inhalation for reconstruction of contracted fingers). We established a new method for esophageal dilatation using a microvasive rigiflex balloon catheter. This catheter was advanced to the stenotic area under radiography and then expanded by injecting contrast medium into the balloon. For reconstruction of hand deformities, the epidermal glove was initially peeled off, and then the combined digits separated carefully by hand, if the release of the contracture was not sufficient, a skin incision was made avoiding injury to nerves and blood vessels. Any skin defects that appeared after the release of the contracture were covered with skin grafts taken from the abdominal wall. K-wire fixation was used to maintain the extended position of the fingers. Three weeks after the operation, the K-wires were removed and rehabilitation was commenced. Results. The esophageal stenosis was successfully dilated with the balloon catheter; in all six cases the dysphagia was relieved immediately. There was no recurrence in any of the patients on long-term follow-up. After reconstruction of the hand, daily activity improved in 12 of the 13 hands. The remaining hand was difficult to reconstruct due to severe mutilation and bone deformity. During follow-up, 6 of the 12 hands maintained successful reconstruction, whereas the remaining 6 hands showed slight to moderate recontraction of the fingers. Conclusion. Esophageal dilatation with a balloon catheter is safer and has fewer side effects compared to other surgical procedures. This method can provide favorable results and can be carried out repeatedly in a short time. Daily and social activities of patients can be improved upon reconstruction of hand deformities.     

Prenatal Diagnosis and Outcome of Ebstein's Anomaly and Tricuspid Valve Dysplasia in Relation to Lung Hypoplasia

Although not all newborns with Ebstein's anomaly present with severe cardiomegaly in utero, some of them cannot live after birth because of the lung hypoplasia. To clarify the relationship between the intrauterine cardiomegaly and the outcome of the patients with Ebstein's anomaly or tricuspid valve dysplasia, we calculated the ratio of the area of the heart against the thorax (CTAR) in the transverse view of the thorax at the level of the cardiac four-chamber view in the fetal echocardiogram and compared it to the outcome. The study population consisted of four patients with Ebstein's anomaly and one patient with tricuspid valve dysplasia who were diagnosed in utero. The ranges obtained from 53 normal fetal cases were 20%± 8% (mean ± 2 SD) at below 20 week's gestation, 25%± 10% at the gestational age of 21 to 30 weeks, and 29%± 6.4% at the gestational age of 31 to 40 weeks. The CTARs of these five cases measured 81.6%, 51%, 55.2%, 47.5%, and 75.6%, respectively, and were abnormally higher than the normal value. Two fetuses died in utero with severe hydrops fetalis. Two fetuses whose cardiothoracic ratios by chest X ray were 100% died at twelve hours of life. One patient died at 110 days. The cross-sectional area of the thorax was smaller than the normal range in 3 (cases 1, 4, and 5) out of these 5 cases. Thus, we conclude that fetal Ebstein's anomaly and tricuspid valve dysplasia associated with massive tricuspid regurgitation with a large CTAR ratio (higher than 50%) and small thoracic cross-sectional area has a very poor prognosis bothprena-tally and neonatally. (ECHOCARDIOGRAPHY, Volume 11, May 1994)     

Subtyping of uropathogenic Escherichia coli according to the pathogenicity island encoding uropathogenic-specific protein: Comparison with phylogenetic groups

BACKGROUND: Phylogenetic analysis has been used widely to characterize extraintestinal pathogenic Escherichia coli in molecular epidemiological studies. We have recently reported a putative pathogenicity island (PAI), carrying uropathogenic-specific protein (usp) and a unique mosaic structure of small open reading frames following usp, providing four subtypes of PAIusp classified from their sequential patterns. METHODS: A total of 427 E. coli isolates from uncomplicated urinary tract infections (194 cystitis, 76 pyelonephritis, and 107 prostatitis) and 50 fecal isolates were examined for phylogenetic grouping and PAIusp subtyping as well as the prevalence of virulence factors (VF) and O serogroups. RESULTS: Both phylogenetic group B2 and usp-positive strains were equally predominant in cystitis, pyelonephritis and prostatitis (B2, 80.9%, 86.8%, and 86.9%; usp, 79.4%, 93.4%, and 88.8%, respectively). Furthermore, each PAIusp subtype was shown to be closely associated with several VF genes as well as several common O serogroups of uropathogenic E. coli. CONCLUSIONS: In molecular epidemiological studies, PAIusp subtyping will provide additional informative findings of E. coli strains belonging to phylogenetic group B2.     

Serum amino acids as indicators of cerebrospinal neuronal activity in patients with micturition disorders

OBJECTIVE: Our previous study showed that the spinal glycine level in rats was changed by spinal injury or bladder outlet obstruction, and this change was reflected by serum glycine levels. Therefore, we measured the serum glutamate and glycine levels in healthy volunteers and patients with cerebrospinal damage or benign prostatic hyperplasia (BPH) to confirm whether the change of serum amino acid levels was obtained from these patients as well as the animal experiment. METHODS: We measured the serum glutamate and glycine levels in 170 healthy controls, 57 patients with cerebrovascular disease (CVD), 68 patients with spinal cord injury (SCI), and 70 patients with BPH. Amino acid levels were compared between the controls and patients, according to gender, level of spinal injury and the type of bladder activity. RESULTS: In the healthy controls, glutamate levels were higher and glycine levels were lower in men than in women. On group comparison of each gender, there were no differences of glutamate levels. However, glycine levels were lower in male and female SCI patients and BPH patients than in controls. According to the level of spinal injury or the pattern of bladder activity and amino acid levels, there were no relationships among them. CONCLUSIONS: Serum glutamate and glycine levels were not related to the spinal injury level or bladder activity. However, serum glycine levels changed in patients with SCI or BPH patients, so it may be possible to use it as an indicator of spinal glycinergic neuronal activity.     

Concomitant adenosquamous carcinoma of the common bile duct and early adenocarcinoma of the gall-bladder

We report a 59 year old male with obstructive jaundice and a clinical diagnosis of carcinoma of the midportion of the common bile duct. Examination of the surgical specimen revealed a small tumour in the neck of the gall-bladder. Histologic examination revealed the tumour in the common bile duct was an adenosquamous carcinoma whereas that in the gall-bladder was an early papillary adenocarcinoma. Thus, this is a rare case of the simultaneous development of adenosquamous carcinoma and early papillary adenocarcinoma in the biliary tree.     

A Case of Melanosis Duodeni Alleviated by the Discontinuation of Ferrous Sulfite

Abstract: A case of melanosis duodeni is presented. The patient was an 80-year-old Japanese woman who had received oral ferrous sulfite for 19 months. Endoscopic examination of the duodenum showed marked pigmentation of the duodenal mucosa. Histological and electron microscopic examinations revealed that the pigment had histochemical features compatible with hemosiderin and was located mainly within macrophage lysosomes in the lamina propria. We discontinued the administration of ferrous sulfite and commenced follow-up endoscopy. The pigmentation disappeared within 7 months of discontinuing treatment. Thus, the ferrous sulfite was speculated to be the causative agent of the pigmentation in the present case. The patient had concomitant hypertension, rheumatoid arthritis, anemia and chronic renal failure. The possible associations of these complications with the development of duodenal pigmentation were also considered.     

Distal ureteral atresia: Recovery of renal function after relief of obstruction at ten months old

A large cystic mass that occupied more than half of the abdomen was identified by ultrasound in a 10-month-old boy. Intravenous pyelography failed to visualize the right kidney, so we created a loop ureterocutaneostomy followed by temporary nephrostomy to improve renal function. Exploratory surgery revealed complete atresia of the distal right ureter. A ureteral stricture developed after ureteroneocystostomy and undiversion of the loop, so a second reconstruction procedure was required (pelvi-ureteroplasty and reimplantation of the right ureter with a psoas hitch) to free the patient from dependence on catheters. Despite the occurrence of giant hydronephrosis secondary to complete ureteral obstruction at the age of 10 months, the function of the right kidney could be preserved. Accordingly, aggressive attempts to promote functional recovery may be justified even when patients have advanced hydronephrosis.     

Sensitization of human renal cell carcinoma cell lines to TRAIL-induced apoptosis by anthracyclines

BACKGROUND: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a new member of the tumor necrosis factor family. The present study investigated whether anthracyclines enhance TRAIL-induced apoptosis and cytotoxicity in renal cell carcinoma (RCC) cells. METHODS: Cytotoxicity was measured using the microtiter assay. Apoptosis was monitored using DNA ladder analysis. Caspase activity was determined using a quantitative colorimetric assay. RESULTS: Treatment of ACHN and Caki-1 human RCC lines with TRAIL, in combination with subtoxic concentrations of epirubicin (EPI) or pirarubicin (THP), enhanced induction of apoptosis and cytotoxicity. Sequential treatment with EPI followed by TRAIL induced significantly more cytotoxicity than the inverse treatment. The combined cytotoxicity of TRAIL and EPI was significantly inhibited by the TRAIL-neutralizing fusion protein DR5:Fc, although EPI did not affect the mRNA expression of DR4, DR5, DcR1 or DcR2. The combination treatment with TRAIL and EPI activated caspase-6 and -3, which were downstream molecules of the death receptor. Furthermore, the combined cytotoxicity of TRAIL and EPI was almost completely inhibited by Z-VAD-FMK, and partly inhibited by Ac-DMQD-CHO. CONCLUSION: These findings indicate that anthracyclines sensitize RCC cells to TRAIL-induced apoptosis and cytotoxicity through activation of caspases, suggesting that TRAIL, in combination with anthracyclines, has a therapeutic potential in the treatment of RCC.