Multiple agminated trichodiscomas in the earlobe

Trichodiscomas are hamartomas of the hair disk and appear as multiple, firm, well-circumscribed papules measuring a few millimeters. In most cases, trichodiscomas are distributed on the face and neck. Trichodiscomas may occur as isolated tumors or in association with other follicular tumors. In some cases, multiple trichodiscomas appear in association with fibrofolliculomas or acrochordons. We herein describe multiple agminated trichodiscomas in the earlobe.     

Standard versus fiberoptic pulmonary artery catheterization for cardiac surgery in the Department of Veterans Affairs: a prospective, observational, multicenter analysis

BACKGROUND: Controversy exists regarding the utility of continuous monitoring of mixed venous oxygen saturation (STvo2) during cardiac surgery. During a multicenter, prospective, observational study in the Department of Veterans Affairs (Cooperative Study #5), frequency of use of standard pulmonary artery catheterization (PAC) and STvo2-PAC was recorded. Here the authors relate these data to clinical outcomes. METHODS: Logistic and Cox regression models evaluating the association of PAC type with mortality, one or more postoperative complications, cardiac complications, time to extubation, and intensive care unit length of stay were constructed. The number of thermodilution cardiac outputs and arterial blood gas analyses performed in the first 24 h postoperatively were compared. RESULTS: Data from 3,265 patients undergoing myocardial revascularization (81.7%) or valve replacement-repair (18.3%) were considered. STvo2-PAC was used in 49% and PAC in 51% of patients. In the 14 hospitals, STvo2-PAC was used in all patients in four, in some patients in four, and never in six. No association of STvo2-PAC use with outcome were observed aside from unexplained hospital level effects. A small but statistically significant reduction in the number of arterial blood gas analyses (8 +/- 3 vs. 10 +/- 4, P < 0.0001, STvo2-PAC vs. PAC, respectively) and thermodilution cardiac outputs (14 +/- 8 vs. 15 +/- 9, P < 0.0001, STvo2-PAC vs. PAC, respectively) was observed with use of STvo2-PAC. CONCLUSIONS: Despite higher cost, STvo2-PAC was commonly used in this cohort. Our analysis failed to detect associations with improved outcomes aside from a small reduction in resource utilization. The precise role of STvo2-PAC remains uncertain.     

Multiple subpial transections: outcome and complications in 20 patients who did not undergo resection

OBJECT: The authors describe patient characteristics, surgical methods, complications, and outcome over time in a cohort of patients who underwent multiple subpial transection (MST) without concomitant cortical resection. METHODS: Twenty consecutive patients in whom drug-resistant epilepsy had been diagnosed a mean of 16 +/- 9 years earlier (mean +/- standard deviation [SD]) were treated with MST without cortical resection. The mean follow-up period was 49.3 +/- 18.3 months (mean +/- SD, median 58 months). At 12 months of follow up, two of the 20 patients were Engel Class I, one was Class II, six were Class III, and 11 were Class IV. At latest follow up, one patient was Engel Class I, one was Class II, seven were Class III, and 11 were Class IV. According to an alternative five-tiered classification system, two outcomes were excellent, seven were good, one was fair, nine were poor, and one was worse. Outcome was found to be better in patients with no lesions observed on magnetic resonance (MR) imaging, and worse in those with large MST areas. Outcome had a tendency to change (this occurred in 13 of 20 cases). Five patients (25%) improved and seven (35%) deteriorated in Engel outcome class, and in one (5%) both developments occurred over time. Most outcome class changes occurred before the end of the 2nd year (nine), and four were observed in the 5th year. There where seven transient neurological deficits and four surgical complications. There was no permanent significant morbidity, and there were no deaths. CONCLUSIONS: Forty-five percent of patients achieved a worthwhile improvement after pure MST, if Engel outcome Class III is deemed a worthwhile improvement. The alternative five-tiered classification resulted in 50% with worthwhile improvement (excellent, good, or fair outcome), 45% with poor, and 5% with worse outcome. Lesions that are detectable on MR imaging, and large MST areas are predictive of worse results. Significant intraoperative problems may arise, but this happens infrequently. There is a notable rate of transient morbidity but the rate of permanent morbidity is not significant.     

Status cataplecticus induced by abrupt withdrawal of clomipramine

Introduction: Cataplexy is one of the main narcoleptic symptoms and is characterized by sudden loss of muscle tone triggered by emotional stimuli while consciousness is mantained. Clomipramine is an effective treatment of cataplexy. Cataplexy that occurs repeatedly for hours or days is referred to as status cataplecticus.Patients: We report three adults with narcolepsy in whom cataplexy was chronically and effectively treated with clomipramine (75-150 mg/day). For diverse reasons, these three patients had an abrupt withdrawal of clomipramine, and after 2-9 days patients showed an invalidant status cataplecticus characterized by a marked increase of the frequency, duration and severity of their cataplectic attacks that were now elicited by mild emotional stimuli. After introduction of anticataplectic agents (clomipramine in two patients and fluoxetine in one patient), status cataplecticus was resolved in less than a week.Conclusion: In patients with narcolepsy, abrupt withdrawal of chronic treatment with clomipramine may be associated with status cataplecticus. This condition may be resolved with the reintroduction of anticataplectic agents.     

阿德福韦双酯治疗拉米夫定耐药的慢性乙肝患者时出现的耐药问题

背景:阿德福韦双酯(ADV) 是一种有效治疗野生型和耐拉米夫定乙肝病毒(HBV)的核苷酸类药物。在使用核苷酸类药物治疗慢性乙肝时,当治疗时间为 48、96、144周时,耐ADV变异体出现的累积发生率分别为0、 0．8-3％和0-5．9％。目的:研究67例对拉米夫定耐药且接受ADV治疗的慢性乙肝患者耐ADV病毒变异体的表型和基因型特点。方法:HBV DNA含量采用实时定量PCR技术。ADV变异体检测采用基质辅助激光解吸电离／飞行时间质谱为基础的基因分型     

Resistance of Copenhagen rats to chemical induction of glutathione S- transferase 7-7-positive liver foci

Copenhagen (Cop) rats are completely resistant to the chemical induction of mammary adenocarcinomas, but their susceptibility to hepatocarcinogenesis is virtually unknown. Rat liver is a well- characterized and easily manipulated tissue in which to study carcinogenesis. Therefore, if Cop rats are resistant to hepatocarcinogenesis, studies into resistance mechanisms may be feasible. Male Cop and F344 rats, 7-8 weeks old, were initiated using either N-nitrosodiethylamine (DEN) (200 mg/kg, i.p.) or a two-thirds partial hepatectomy (PH) followed by N-methyl-N-nitrosourea (MNU) (60 mg/kg, i.p.). The rats were then promoted using a modified resistant hepatocyte (RH) protocol (a combination of four doses of 2- acetylaminofluorene (2-AAF) and a single dose of CCl4 that provides a selective mitotic stimulus for initiated cells). Six weeks after initiation the rats were killed and liver sections were stained for glutathione S-transferase 7-7 (GST 7-7), a marker for putative preneoplastic hepatocytes. Cop rats were found to be highly resistant, having a approximately 9- and approximately 27-fold smaller percentage of liver area occupied by GST 7-7-positive foci than susceptible F344 rats following initiation by DEN and MNU respectively. Furthermore, gross liver nodules did not form in any of the Cop rats, whereas all F344 rat livers contained nodules. Hepatic necrosis caused by DEN during initiation, and CCl4 during promotion is necessary to stimulate compensatory hepatocyte division. We demonstrated that these agents do indeed increase serum transaminase levels and produce histologic evidence of necrosis in Cop rats. In order for liver foci to grow rapidly in the RH protocol, the surrounding normal hepatocytes must be mito-inhibited by 2-AAF. We found that the degree of mito-inhibition of normal hepatocytes by 2-AAF is the same in Cop and F344 rats. These results show that the Cop rat is highly resistant to the chemical induction of putative preneoplastic liver foci and nodules.      

Nasal inflammation and chronic ear disease in Australian Aboriginal children

Objective : Chronic middle ear disease is common in Aboriginal children, and may be linked to nasal inflammation and Eustachian tube dysfunction. The pattern of nasal inflammation is unknown. The study reported here was performed to define the role of allergy and infection in causing nasal inflammation in Aboriginal children with chronic middle ear disease.
Methodology : Thirty-one Aboriginal children aged between 3 and 7 years underwent clinical assessment, audiometry and allergy skin tests. Nasal swabs for bacterial culture and cytology were performed during the winter and again in spring to identify any seasonal variation. A randomized trial of nasal beclomethasone for 8 weeks was conducted in children with abnormal tympanometry to identify the effect of therapy upon nasal cytology.
Results : Twenty-six of the 31 children had abnormal tympanograms. Average hearing levels were reduced in nine children. Pathogenic organisms were isolated from most children: Streptococcus pneumoniae (82%), Haemophilus influenzae (79%), Moraxella catarrhalis (39%) and Staphylococcus aureus (29%). Eight of the 31 children (26%) were atopic. Nasal cytology disclosed a marked neutrophil infiltrate (80% of cells) during the winter, which fell significantly in spring to 52% of cells. Only two subjects had nasal eosinophilia of >10%. There was no effect of beclomethasone on nasal cytology.
Conclusions : Chronic ear disease in Aboriginal children is associated with nasal inflammation, neutrophil infiltration and the presence of bacteria. These features suggest respiratory infection as the main cause of chronic nasal inflammation in Aboriginal children with middle ear disease. There is a seasonal variation in the severity of the nasal infiltrate, consistent with increased infections during winter. Despite a high prevalence of atopy, allergic nasal disease was uncommon.     

Histidinaemia: a benign metabolic disorder

Histidinaemia is a relatively common inherited metabolic disorder with an incidence similar to phenylketonuria. This paper reports the long term outcome of patients diagnosed by newborn screening in the north west of England. Between 1966 and 1990, 108 infants were diagnosed as having histidinaemia by a regional neonatal screening programme (incidence 1:11,083). A further five children were detected following diagnosis in a sibling. Of the 113, nine were lost to follow up. Infants diagnosed before 1981 (n = 47) were placed on a low histidine diet (225 mg/kg/d) for an average period of 21 months (SD 4.5). All patients were reviewed regularly, Griffiths developmental quotients (DQ) were assessed at 2 and 4 years, and WISC-R intelligence quotients (IQ) at 8, 12, and 18 years. IQ data were converted to standard deviation scores (IQ SDS) to account for increasing IQ norms with time. Neither DQ nor IQ correlated with plasma histidine at diagnosis or with the mean plasma histidine throughout life. Growth was normal in all patients. There was no apparent benefit from a low histidine diet in early childhood. In contrast to other studies, there was no excess of clinical symptoms. On the basis of these findings, histidinaemia is a benign metabolic disorder that does not require treatment.