Glutaric aciduria type 1: proton magnetic resonance spectroscopy findings

Glutaric aciduria type 1 is an inborn error of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-coenzyme A dehydrogenase. The disease often appears in infancy with an encephalopathic episode that results in acute basal ganglia and white matter degeneration. The neuroimaging findings in glutaric aciduria type 1 have been well defined. However, the changes in magnetic resonance spectroscopy, a noninvasive tool for identifying the biochemical state of the brain, are scarce in glutaric aciduria type 1. This report presents the magnetic resonance spectroscopy findings in a 19-month-old male with glutaric aciduria type 1. Magnetic resonance spectroscopy of right frontal white matter and right lentiform nuclei revealed decreased N-acetylaspartate/creatine ratio, slightly increased choline/creatine ratio, and increased myoinositol/creatine ratio, compared with the age-matched control patients. We thought that these changes were in accordance with neuroaxonal damage, demyelination, and astrocytosis in these areas. In conclusion, proton magnetic resonance spectroscopy provides a tool for assessing metabolic disturbances and the extent of brain damage noninvasively in glutaric aciduria type 1.     

Comparative effects of high glucose on different adult sympathetic neurons in culture

In vivo, diabetes causes neuropathy in the sympathetic celiac/superior mesenteric ganglion (CG/SMG) but not the superior cervical ganglion (SCG). Therefore, our aim was to compare the effects of high glucose in vitro on adult rat SCG and CG/SMG neurons. High glucose decreased viability and neurite outgrowth and increased TUNEL staining in freshly dissociated neurons from both ganglia. In cultures established for 24 h before exposure to high glucose, a significant (p < 0.01) decrease in neurite-bearing neurons from CG/SMG but not SCG occurred. CG/SMG neurons took longer (p < 0.05) to initiate neurite outgrowth and had lower (p < 0.002) anti-oxidant defence enzyme activity. These properties may contribute to the selective development of neuropathy in CG/SMG in diabetes in vivo.     

Bcl-2 expression in preinvasive and invasive cervical lesions

PURPOSE: To determine the correlation between bcl-2 expression and clinicopathological findings in cervical intraepithelial neoplasias (CIN I, II, III) and invasive cervical lesions, and its effect on overall survival rate. METHODS: Fifty specimens derived from 22 preinvasive and invasive cervical cancer cases up to surgical stage IIb (21 squamous cervical carcinoma and 7 adenocarcinoma cases) were preserved in paraffin blocks from primary surgery and constituted the study group. Tissues were processed and stained by immunohistochemical methods to assess the degree of bcl-2 expression. RESULTS: Positive bcl-2 expression was detected in 54% (13/21) of invasive lesions, while negative in 46% of cases. In CIN's, overall bcl-2 positivity was detected in 68% of cases. Bcl-2 expression was highly relevant between low grade (CIN I) and high grade (CIN II-III) lesions (p < 0.05). As regards degree of tissue staining for bcl-2 in CIN III cases, a statistically relevant difference was detected in comparison with low-grade preinvasive lesions (p < 0.05). In invasive cervical cancer cases, patients with bcl-2 positivity had a longer survival rate. By using the Cox regression model, univariate analysis did not show any specific prognostic factor to be important for survival rate, whereas, on multivariate analysis, histopathologic subtypes (p = 0.0390) and stage of tumor (p = 0.0451) had a statistically significant impact on overall survival rate. CONCLUSIONS: Bcl-2 expression, especially in preinvasive lesions, may play a role in the apoptotic process and be regarded as a marker for disease progression. In invasive cervical carcinomas, bcl-2 expression has not been shown to be effective in overall survival rates.     

The prevalence and clinical features of tuberculous peritonitis in CAPD patients in Turkey, report of ten cases from multi-centers

OBJECTIVE: To determine the rate, risk factors and outcome of Tuberculous Peritonitis (TBP) in patients treated with continuous ambulatory peritoneal dialysis (CAPD) in our units. DESIGN: Retrospectively, we reviewed the medical data of all CAPD patients from 12 centers for TBP, covering the period between 1986 and December 2002. SETTING: All patients were from 12 renal clinics at tertiary-care university hospitals. RESULTS: Ten cases of TBP were identified among the CAPD patients in our centers. There were five male and five female patients with a mean age of 37.2 years. None of the patients had tuberculosis history, 6 patients had predominance of PNL. One patient had coincidental bacterial peritonitis. Two patients were successfully treated without the removal of the Tenckhoff catheter. CONCLUSION: TBP in CAPD patients is a very rare complication. In contrast to predominance of lymphocytes in nonuremic patients with tuberculous peritonitis, CAPD patients with tuberculous peritonitis may have predominance of PNL on examination of the peritoneal fluid. Since TBP has high morbidity and mortality, early diagnosis and treatment of disease are extremely important for improving outcome.     

Lipoma arborescens; successfully treated by yttrium-90 radiosynovectomy

Although radiosynovectomy (RS) applications have been carried out for many years, clinical indications of this non-invasive procedure is thought to be limited probably due to the lack of information of clinicians. Clinicians' preferential indication for RS is the treatment-resistant synovitis of individual joints, i.e. despite systemic pharmacotherapy and intra-articular steroid injections. We present here a case of "lipoma arborescens" treated by yttrium-90, which is a rare intra-articular lesion characterized by villous proliferation of the synovial membrane and hyperplasia of subsynovial fat. The results of clinical, biochemical and hematological examinations, magnetic resonance (MR) imaging, arthroscopy and histological analysis have shown that the etiology was lipoma arborescens in a female patient, aged 36 having swelling and sometimes associating pain at her right knee for 4 years. We have applied to our patient's right knee RS with 185 MBq yttrium-90 colloid together with 40 mg of methylprednisolone acetate, although in our literature survey we have not met any similar case being treated with such indication. Even a year after the application, the patient has absolutely benefited from the treatment clinically, and this was also confirmed by comparative MR images (pre- and post-treatment). Consequently, we consider that Y-90 treatment might be applicable in suitable cases with lipoma arborescens.     

Prognostic significance of microvessel density and vascular endothelial growth factor (VEGF) expression in non-Hodgkin's lymphoma

Angiogenesis has a major role in the pathogenesis of malignancies. Studies involving the role of angiogenesis have been most commonly performed in solid tumors. However, studies related to hemapoietic neoplasia and angiogenesis are relatively limited. We investigated the role of angiogenesis in non-Hodgkin's lymphomas (NHLs) and its relation with clinical and histopathologic prognostic indicators. In this respect, angiogenesis markers were evaluated in 71 patients with NHL and these were compared with other prognostic indicators including age, gender, histological grade, stage, extranodal involvement and survival. Microvessel density (MVD) using Factor VIII monoclonal antibody and vascular endothelial growth factor (VEGF) using monoclonal antibody for VEGF expression were studied in paraffin-embedded tissue samples. We did not find a significant relationship between MVD and patient characteristics including age, gender, stage, histological grade, nodal status, international prognostic index (IPI), and response to treatment. MVD was found to be greater in cases with B symptoms compared to those without B symptoms (14.6±5.7 and 11.4±5.3, respectively, p=0.002). No significant relationship was found between VEGF and age, gender, stage, histological grade, IPI, and overall survival. The complete and partial response rate to therapy was significantly higher in VEGF-negative patients than in the VEGF-positive patients (p=0.003). In conclusion, there appears to be a role for angiogenesis and angiogenic factors in NHLs. The combination of anti-angiogenic drugs with conventional anti-neoplastic treatment will probably be used in the future. Larger series of patients are needed to determine the prognostic value of angiogenesis in NHL.     

Serum carnitine levels during oxcarbazepine and carbamazepine monotherapies in children with epilepsy

Prolonged antiepilepsy drug treatment can result in secondary carnitine deficiency. The effect of oxcarbazepine on carnitine metabolism has not been reported previously. In this study, serum concentrations of total and free carnitine were measured in 20 children with epilepsy treated with oxcarbazepine monotherapy and were compared with 20 children with epilepsy who were taking carbamazepine as monotherapy. The assays were performed between 3 and 6 months of anticonvulsant treatment. The mean values of serum total and free carnitine levels in patients receiving carbamazepine monotherapy were 63.0 +/- 20.7 micromol/L and 49.1 +/- 16.7 micromol/L, respectively. The mean values of serum total and free carnitine levels in patients receiving oxcarbazepine monotherapy were 64.2 +/- 17.4 micromol/L and 50.3 +/- 13.7 micromol/L, respectively. The values were all between normal ranges. No significant difference was observed in the level of total and free carnitine levels between the two groups. Our results suggest that neither oxcarbazepine nor carbamazepine as monotherapy causes carnitine deficiency in otherwise healthy children with primary idiopathic epilepsy.     

Using Conjoint Analysis to Estimate Healthy-Year Equivalents for Acute Conditions: An Application to Vasomotor Symptoms

Objective:  Conventional standard gamble and time trade-off methods may be inappropriate for eliciting preferences for some health states because both require subjects to make trade-offs between a morbid health state and death. Thus, the objective of this study is to demonstrate the use of conjoint analysis to obtain time trade-off estimates of healthy-year equivalents (HYEs) for clinically relevant durations and severities of acute, self-limiting, or nonfatal conditions such as vasomotor symptoms.
Methods:  A self-administered, web-enabled, graded-pairs conjoint-analysis survey was developed to elicit women's preferences for reducing the frequency and severity of vasomotor symptoms (daytime hot flushes and night sweats). Observed trade-offs between symptom duration and symptom relief were used to calculate HYEs for different severities and durations of vasomotor symptoms.
Results:  A total of 523 women with a mean age of 52 years completed the survey. For these women, an improvement from severe to moderate vasomotor symptoms yields a gain of 4.44 HYEs, and an improvement from moderate to mild vasomotor symptoms over 1 year yields a gain of 4.62 HYEs over a period of 7 years. HYE gains for symptom relief are larger for younger women than for older women.
Conclusions:  Conjoint analysis is a feasible method for estimating HYEs for acute, self-limiting, or nonfatal conditions. This approach may provide an alternative utility-elicitation method when conventional standard gamble and time trade-off methods are inappropriate to the decision context.     

Antioxidant enzyme activities and lipid peroxides in the plasma of patients with benign prostatic hyperplasia or prostate cancer are not predictive

In this study, lipid peroxide and total sulfhydryl contents and activities of superoxide dismutase (SOD), glutathione peroxidase (GPx), and catalase (CAT) were investigated in the plasma of patients with benign prostatic hyperplasia (BPH) and prostate cancer. No significant change was found in lipid peroxidation or antioxidant systems in the plasma of patients with BPH and prostate cancer. The results indicate that evaluation of the prooxidant-antioxidant balance in the plasma of BPH and prostate cancer patients cannot be used as a marker to discriminate between these diseases. Received: 5 November 1998 / Accepted: 19 January 1999