Amino acid composition of whole cells of different yeasts.
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Biomedical subjects
Publications and source records attributed to A Martini.
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Wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described without renal involvement. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cutaneous lesions were associated with upper and lower respiratory tract involvement, low-grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagnosis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infiltrate and a late-stage small vessel vasculitis in the dermis. Histopathology of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patient's clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an "acneiform" presentation is a rare finding in WG.
A six-year-old boy with systemic lupus erythematosus developed insidiously a monolateral serous retinal detachment leading to severe visual loss. Serial determinations of anticardiolipin antibodies showed the presence of IgG anticardiolipin antibodies only at the time of ocular involvement. This case underlines the need for regular ophthalmologic evaluation in children with active systemic lupus erythematosus and supports the previous hypothesis of a relationship between antiphospholipid antibodies and retinal involvement in systemic lupus erythematosus.
The study aimed at the development of a clinically applicable methodology that could: (1) discriminate transient evoked otoacoustic emission (TEOAE) recordings from normal hearing or hearing impaired individuals; (2) classify the nature of the hearing loss as conductive or as cochlear, and (3) define clear-cut TEOAE clinical criteria. A classification algorithm based on a multivariate discriminant analysis of fast Fourier transform data from recordings evoked by click stimuli of 50 +/- 2, 62 +/- 2, 68 +/- 2 and 80 +/- 2 dB SPL was used to discriminate 302 normal subjects from 383 subjects suffering from mild to moderate hearing losses. The best discriminant model (QDF80) produced a sensitivity of 93.8% and a specificity of 79.4%. When extra correlation criteria were serially applied to the classification outcome, the specificity was increased to 85.3%, but the sensitivity was marginally decreased to 91.7%. The classification of the correctly identified hearing-impaired cases yielded 93.8% identification of conductive and 75.1% identification of cochlear cases. A sensitivity analysis of the misclassified hearing-impaired cases suggested that the TEOAE spectra are well correlated with the 2-kHz but poorly correlated with the 4-kHz octave frequency.
A standardized protocol was used for duplicate examination by two neurologists of the clinical history and neurological signs in 55 patients with reversible cerebral ischemia. One trained examiner from each of eight clinical centres involved in the Italian Cooperative Study on reversible cerebral ischemia participated in this research. Duplicate examination were compared in order to evaluate the percentage of agreement achieved in the responses. Several discrepancies relating to either historical data or neurological signs were detected. Possible causes of this disagreement are discussed. The inter-observer differences appear to be an important problem to be faced in cooperative studies on reversible ischemic attacks. The purpose of the study was to estimate these differences in the attempt to increase the degree of agreement by repeated training sessions and discussion between the different examiners. These efforts are needed to improve the quality of clinical investigations on reversible cerebral ischemia and to increase the validity of their results.
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PURPOSE: To warn the ophthalmologist to consider orbital dirofilariasis in the differential diagnosis of inflammatory masses of the orbit. PATIENTS: Two cases with non-specific swelling and pain of the orbit who had a initial diagnosis of "inflammatory pseudotumor of the orbit". RESULTS: These are two new cases of orbital dirofilariasis. CONCLUSION: In the ophthalmic literature, the highest incidence of ocular dirofilariasis is reported in Italy. Since both these patients had no history of travel in endemic areas of Dirofilaria, this parasitic infection is presumably not rare in Italy and is probably underdiagnosed.
This study evaluated the characteristics of transient evoked otoacoustic emission (TEOAE) time-frequency (TF) representations from normal and hearing-impaired ears. Linear and non-linear TEOAE recordings from normally-hearing subjects (40) and non-linear recordings from patients with sensorineural hearing loss (SNHL) (40) were analysed using the short-time-Fourier-transform spectrogram, the Gabor spectrogram, and the adaptive spectrogram. The TF representations of the TEOAE recordings indicated a considerable dispersion of energy across frequencies and TEOAE time segments >4.0 ms. The linear and non-linear recordings from the normal subjects showed common frequency peaks. The TF representations from the patients with SNHL indicated that the significantly reduced energy in the mid-to-high TEOAE frequencies did not correlate closely with the threshold elevation. As in the recordings from the normal subjects, a high percentage of the TEOAE cumulative energy was found within a short TEOAE segment (4-14 ms).
Linear and QuickScreen (non-linear) transient evoked otoacoustic emission (TEOAE) protocols were compared in terms of standardized clinical parameters in order to define the protocol producing recordings with the highest signal quality. Neonatal responses (520) were obtained from three different screening sites. The linear recordings were evoked by 69 and 75-dB p.e. SPL clicks. All responses were post-windowed by a 3.5- to 12.5-ms window, chosen by time-frequency analysis as the segment representing 97.35 per cent (linear) and 95.6 per cent (quick) of the total cumulative spectral energy. Evidence from hearing loss cases and the high similarity between the profile contours of the QuickScreen and the linear normal recordings have strongly suggested that a linear response evoked by a 75-dB p.e. SPL stimulus and post-processed by a 3.5 to 12.5 window is free of stimulus artefacts. The data indicate that the 75-dB linear protocol produces higher signal to noise ratios at 2.0, 3.0, and 4.0 kHz, higher wave reproducibility, and lower TEOAE noise values than the QuickScreen protocol.
The aim of this study is to outline the mechanisms leading cochlear cells to die. We utilized an immortalized cell line (OC-k3 cells) derived from the organ of Corti of transgenic mice in order to perform in-depth biochemical studies with no limitations on sample size and number. We probed these cells with cisplatin and gentamicin, two drugs which display in vivo undesired ototoxic side-effects. We investigated cell viability, reactive oxygen species (ROS) production and glutathione (GSH) levels and tested the effects of different concentrations of cisplatin and gentamicin from 0 to 48 h. Results show that cells undergo a dose- and treatment-time-dependent apoptosis characterized by nuclear fragmentation, integrity of the cell membrane and mitochondria, and absence of DNA endonuclease activity. During the early part of treatment, ROS production increases and intracellular GSH decreases, probably due to the activation of protein kinase C alpha. Use of antioxidants such as N-acetylcysteine, GSH and vitamin C rescues cells from apoptosis almost completely. Overall, these data indicate that ROS generation might play a central role in inducing inner ear cell apoptosis and may have an additive role in the ageing process.
Air and bone conduction brain stem responses were recorded in 20 adults and 20 infants (16-20 months postconceptional age) with normal hearing. The stimuli were administered using a shielded TDH-39 headphone and a standard B-70A vibrator. Our results show that adults and infants have similar air and bone conduction brain stem thresholds. The comparison of input latency functions obtained with air and bone conduction clicks indicates that the acoustic stimulus generated by the bone vibrator excites more apical regions than that stimulated by the air conduction transient. This is related to the spectrum of the bone conduction click which has an energy peak at 1-2 kHz. Furthermore we found that the difference in latency between adults and infants for air-conducted clicks decreases along with the stimulus intensity and the latencies tend to overlap near the threshold.
It was hypothesized that the relationship between transiently evoked otoacoustic emission (TEOAE) signals and the functional status of the outer hair cells provides an opportunity to design a clinical procedure that can evaluate the normality of cochlear function. To discriminate normal subjects from subjects suffering from mild to moderate hearing loss (HL), it was assumed that every subject population has unique and discrete TEOAE signal descriptors. The main classification algorithm was based on a discriminant analysis of raw fast Fourier transform data. When it was applied to a sample set of TEOAE recordings (from 56 normal and 68 HL subjects) elicited from 68-dB SPL click stimuli, it correctly identified 90.2% of the normal and 87.5% of the HL subjects. The same algorithm yielded an 85.5% discrimination between TEOAE recordings from conductive and cochlear HL cases.
Sixty-five families with non-syndromal sensorineural hearing loss (NS-SNHL) of genetic aetiology were subtyped according to Gorlin et al. Individual audiogram shapes were also classified in order to detect inter- and intra-familial variations. In 48 families with an Autosomal Dominant (AD) inherited form, 26 exhibited the features of (high-frequency) progressive NS-SNHL, 12 those of mid-frequency NS-SNHL, 5 were affected by congenital low-frequency NS-SNHL; 1 kindred showed a progressive low-frequency pattern and another 1 a unilateral NS-SNHL; only 3 kindreds were affected by severe congenital NS-SNHL. Autosomal Recessive (AR) inherited forms were composed of 9 kindreds with severe congenital NS-SNHL, and 7 with moderate congenital NS-SNHL. One X-linked form was identified. AD- and AR-inherited NS-SNHL differed significantly both in severity of hearing impairment and in audiogram shapes. With few exceptions, in each family classified according to Gorlin, most of the affected subjects shared the same audiogram profile. Intrinsic progression of the disease versus ageing was studied in the larger subtype of individuals with the high-frequency loss. Gorlin's classification still remains the best system to classify NS-SNHL, and can provide a broad base to separate a very heterogeneous group of disorders. Results obtained in gene mapping in single large human families or in homologous gene search could be tested in our families. For some of them, namely those with high frequency progressive and low-frequency NS-SNHL, testing should already be feasible.
Dehydroepiandrosterone (DHEA) exerts opposite effects on the growth of mammary carcinoma. A stimulatory effect is observed in absence of estrogens, due to interaction of DHEA metabolite(s) with the estrogen receptor (ER); by contrast, in presence of estrogens DHEA inhibits tumor growth. The mechanism underlying the latter DHEA effect, which might be related to activation of the androgen receptor (AR), is poorly understood. Focusing on this point, we measured over a 20 days period the areas of DMBA-induced mammary tumors in rats given DHEA and/or the anti-androgen flutamide (FLU). Results show that DHEA inhibitory effect on the growth of mammary carcinoma is no longer observed when the ARs are blocked by FLU. Data are consistent with an involvement of ARs in the inhibitory effect of DHEA.
OBJECTIVE: To investigate the efficacy of folinic acid in reducing the side effects associated with methotrexate (MTX) therapy in children with juvenile idiopathic arthritis (JIA) and to determine whether folate supplementation may reduce the benefit of MTX administration. METHODS: This was a retrospective, non-controlled study. Inclusion criteria were: 1) diagnosis of JIA according to the Durban 1997 criteria; 2) treatment with low to intermediate doses of MTX (10-20 mg/m2/week) as the sole second-line agent for at least 6 mos.; and 3) supplementation with folinic acid (2.5-7.5 mg) in a single weekly dose 24 hrs after MTX administration. All patients were started on folinic acid only after the development of a side effect. Exclusion criteria were: treatment with higher doses of MTX (> 20 mg/m2/week). The outcomes investigated were: hepatotoxicity (liver transaminase increase), gastrointestinal toxicity, disease flare, and clinical remission. The number of episodes per patient-year of MTX treatment of each outcome before and after folinic acid supplementation was compared by the Wilcoxon matched pairs test. RESULTS: A total of 43 children with JIA were included in the study. The mean duration of treatment before and after folinic acid supplementation was 1.1 years and 1.8 years, respectively. After the start of folinic acid supplementation, the mean number of episodes per patient-year of hepatotoxicity and gastrointestinal toxicity decreased from 2.30 to 0.32 (p < 0.001) and from 1.09 to 0.29 (p = 0.002), respectively. The mean number of disease flares and clinical remissions per patient-year did not change significantly. CONCLUSION: In our JIA patients, folinic acid supplementation resulted in a significant reduction in the most common side effects of MTX, without affecting the clinical efficacy of the drug.
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OBJECTIVE: To investigate the functionality of the Fas-induced apoptotic pathway in peripheral blood mononuclear cells (PBMC) from patients with systemic or pauciarticular juvenile idiopathic arthritis (JIA). METHODS: PBMC from 12 patients with systemic and 6 with pauciarticular JIA were activated with anti-CD3 and rhIL-2 and then incubated in the presence or absence of the anti-Fas MoAb CH11 inducing activation of the Fas apoptotic pathway. Apoptosis was evaluated by flow cytometry and fluorescence microscopy. RESULTS: The percentage of apoptotic cells following triggering of Fas did not differ between patients with systemic JIA (12.5 +/- 9.5%) or pauciarticular JIA (18.7 +/- 8.9%) and controls (16.1 +/- 6.8%). Evaluation of activation-induced cell death (AICD) in the absence of exogenous triggering of Fas showed that 44% (8/18) of the patients with JIA, compared to none of the controls (0/16), had a percentage of apoptotic cells higher than the mean + 2 SD of controls. The increased AICD was neutralized by the addition of an anti-TNF-alpha antibody. CONCLUSION: Patients with systemic or pauciarticular JIA do not show a defect in the Fas-dependent apoptotic pathway of T cells. The increased AICD present in some patients with JIA appears to be at least in part related to the inflammatory cytokine TNF-alpha.