Acropathia ulcero-mutilans acquisita.
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Biomedical subjects
Publications and source records attributed to G Moretti.
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BACKGROUND: Supraventricular tachyarrhythmias (SVTs) represent an intraoperative risk factor that should be always prevented/managed. The commonly used anti-arrhythmic drugs are accompanied by intrinsic hazards, such as pro-arrhythmic and toxic effects or unpredictable onset and duration of action. We underline the therapeutic use of transesophageal atrial pacing (TAP) for the interruption of particular re-entry SVTs occurred during surgical procedures in general anaesthesia. METHODS: Our study was carried out in 25 patients characterized by a personal clinical history of transient tachyarrhythmic episodes, subjected to general anaesthesia obtained by midazolam, propofol, N2O e O2, sevoflurane, fentanil and vecuronium bromide. We used TAP bursts of 3-5 sec, their minimal pacing rate being equivalent to the tachyarrhythmia cycle length, with an impulse intensity ranging from 18 to 25 mA. In such conditions, the re-entry was interrupted by the induction of refractoriness of the wave-front that sustained the underlying arrhythmogenic circuit. RESULTS: During the study, the following arrhythmias occurred in 7 out of all patients: 1 type I atrial flutter, 3 nodal tachycardias, 1 antidromic and 2 orthodromic atrioventricular tachycardias, respectively. TAP assured either atrial capture or prompt suppression of arrhythmias in all cases. Low intensity impulses did not ever allow ventricular capture. CONCLUSIONS: TAP can be considered as a valid therapeutic device for the management of re-entry SVTs occurred during general anaesthesia, resulting it effective, safe and easy-practicable.
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The purpose of this study was to evaluate the activity and the toxicity of the combination of gemcitabine with ifosfamide and cisplatin (GIP) in chemonaive patients with advanced non small cell lung cancer (NSCLC). Eighty chemonaive patients with Stage IIIB-IV NSCLC were treated with the combination of gemcitabine 1 g/m(2) on Days 1 and 8, ifosfamide 2 g/m(2) on Day 1 and cisplatin 80 mg/m(2) on Day 2. Cycles were administered on an outpatient basis every 3 weeks. Hematologic toxicity was the main side effect; Grade III-IV thrombocytopenia was observed in 54 (67%) patients and Grade III-IV leucopenia in 44 (55%) patients, with 4 episodes of febrile neutropenia and 1 toxic death. Thirteen patients received platelet transfusions and 38 were transfused with packed red cells. All patients were evaluable for response. The overall response rate was 54% (95% confidence interval 43 to 65%) with 1 complete response. In patients with Stage IIIB and IV disease, response rates were 58% and 52%, respectively. Median time to progression was 40 weeks (range 0-114) and median overall survival was 12 months (16.6 months for stage IIIB and 10.4 months for stage IV). Median and minimum follow-up were 19 and 12 months, respectively. The GIP combination shows a response rate and overall survival of clinical interest. Hematologic toxicity was the main toxic effect, especially in patients with low performance status. This regimen will be tested in a Phase III randomized trial.
We describe a case of dystrophic epidermolysis bullosa which occurred in a young boy who presented thickened and dystrophic nails both in hands and feet, atrophic scars on the elbows and knees, some large bullae and milia on the hands and ankles. The parents were clinically unaffected and the family medical history was negative for blistering disease. The immunofluorescence for type VII collagen was positive, yet low in intensity and the number of anchoring fibrils was reduced, as revealed by transmission electron microscopy. The diagnosis of a "sporadic" case of dominant dystrophic epidermolysis bullosa was suggested, although a mitis case of recessive dystrophic epidermolysis bullosa cannot be excluded on the basis of clinical, immunofluorescent and ultrastructural examination. However recent studies, carried out in a series of seemingly sporadic cases, have pointed out the possibility of inheritance of two mutant alleles from unaffected parents. This implies that 'mild' recessive dystrophic epidermolysis bullosa is commoner than once thought. This information is important for genetic counselling and determination of recurrence risk in the present and future generations.
OBJECTIVE: To detect early losses of contrast sensitivity (CS) in patients with pituitary adenomas, before the occurrence of visual acuity and visual field defects. METHODS: CS has been evaluated in both hemifields of 28 patients with different kinds of pituitary adenoma (mainly intrasellar) and normal visual acuity and visual field, as well as in 15 age-matched controls. Two different stimuli were used: a coarse (0.3 c/deg) dynamic (10 Hz) grating and a finer (2 c/deg) static grating. RESULTS: On average, CS and/or hemifield asymmetry were reduced in patients, whereas perimetric sensitivity was normal. CS losses were more frequent for 2 c/deg static-, as compared with 0.3 c/deg, 10 Hz stimuli. However selective losses for either stimuli were also found. CS losses did not correlate with anatomical measurements (size, chiasm involvement) of tumors as established by MRI scans. CONCLUSIONS: CS evaluation may provide a simple and effective tool for early detection and monitoring of visual dysfunction in patients with pituitary adenoma. The lack of correlation between CS losses and chiasm involvement suggests causes different from chiasmal compression for visual dysfunction.
With the aim to assess the prevalence and the main clinical correlations of skin lesions in diabetes mellitus, 457 diabetic subjects consecutively attending an outpatient clinic underwent a dermatological examination. Neurovascular foot lesions were excluded. Thirty-five of 64 IDDM patients (54%) had skin alterations mainly consisting of vitiligo (9% of all patients), psoriasis (9%) and eczema (8%). The most frequent skin lesions observed in 240/393 NIDDM subjects (61%) were represented by infections (20% of all patients) and diabetic dermopathy (12.5%), while other lesions were not common. NIDDM patients with skin infections had a worse metabolic control, and those with diabetic dermopathy had a greater prevalence of neuropathy and large vessel disease than patients without skin lesions. These data show that the prevalence of skin diseases in a large, unselected diabetic population is higher than expected and indicate that, in most cases, a careful dermatological examination and a better metabolic control are needed in order to improve quality of life in these patients.
The aim of this randomised trial was to compare the efficacy of bolus versus continuous infusion cisplatin combined with mitomycin C and vindesine (MVP) for chemotherapy-naive patients with stage IIIB-IV non-small cell lung cancer (NSCLC). 97 patients (49 given bolus cisplatin-arm A and 48 given continuous infusion cisplatin--arm B) were evaluable for response. In arm A, 2 patients achieved a complete response (CR), 21 achieved a partial response (PR), whilst in arm B, 14 patients achieved a PR (29%) (P = 0.07). Median survival was 8 months in both arms. Myelosuppression was the most frequent and severe toxicity, with a higher incidence of grade 3-4 leucopenia in arm A when compared with arm B (44% versus 25%). In conclusion, there is no advantage for a cisplatin 5 day infusion in the MVP regimen.
Seven of 112 hemophiliacs infected with human immunodeficiency virus type-1 (HIV-1) before 1986 through contaminated plasma products are currently healthy, with CD4 T-cell counts above 500 cells/microL, and have never received antiretroviral therapy (long-term nonprogressors [LTNPs]). Seven age and sex-matched hemophiliacs infected in the same period but who have progressive HIV disease (progressors) and one additional slow-progressing individual were also studied. One hundred-fold, 20-fold, and 10-fold lower levels of full-length HIV RNA in plasma, peripheral blood mononuclear cells (PBMCs), and proviral DNA in PBMCs, respectively, were found in LTNPs compared with progressors. Plasma and cell-associated HIV RNA and proviral DNA were lower in LTNPs who tested negative for viral isolation from PBMCs or who were positive only after removal of CD8+ cells. No substantial differences were observed in the in vitro production of chemokines including RANTES, MIP-1 alpha, MIP-1 beta, MCP-1, and interleukin-8 (IL-8) in supernatants of activated PBMCs or CD8-depleted PBMCs of LTNPs, even when HIV isolation was simultaneously accomplished exclusively after removal of CD8+ cells. Low levels of HIV load and replication in peripheral blood are the strongest correlates of nonprogression in this small number of infected hemophiliacs.
PURPOSE: To understand whether laser photocoagulation of the retina for the treatment of diabetic retinopathy (DR) alters directly or indirectly the function of the macular region. METHODS: Transient pattern electroretinograms (PERGs) to 30' checks have been recorded in DR patients before and 7-10 days after one treatment session (established protocol with Argon laser) of either the peripheral retina (panretinal treatment) or the central retina (paramacular focal treatment). RESULTS: Treatment of the peripheral retina causes, in most eyes, a small increase (about 15% on average) of the PERG amplitude. Focal paramacular treatment causes a marked decrement (about 40% on average) of the PERG amplitude. Simulated retinal amputation induced by focal treatment, obtained by means of adequate stimulus masking, resulted in a 15-20% PERG decrement only. CONCLUSIONS: The PERG increase after peripheral treatment may be due to rearrangement of both retinal and choroideal hemodynamics and does not necessarily result in useful long term changes. The PERG decrease after focal, paramacular treatment is far larger than that expected on the basis of the amputated area only. This may result from thermal and/or toxic damaging effects spreading outside the treated area. The results are relevant for both a better monitoring of central retinal function in the course of laser treatment and a better evaluation of the cost/benefit ratio after photocoagulation in DR.
Clostridium sordellii is an infrequent human pathogen. It has been demonstrated to be occasionally responsible for myonecrosis or gas gangrene. Interestingly, in the obstetric literature, some cases of postpartum maternal deaths have been associated with C sordellii infection causing a rapidly lethal toxin mediated syndrome. This is the first reported case of postpartum death in a 29 year old woman, in which a toxigenic C sordellii was isolated from the patient's blood antemortem during the fatal toxic shock, strongly indicating its role in this rare syndrome.
Recent experimental evidence has suggested that peripheral benzodiazepine receptors (PBR) may play a role in epilepsy and antiepileptic drug action. Since PBR are also present in circulating lymphocytes, and may interact with anticonvulsant drugs, this study was designed to look for possible modifications of these receptors and their endogenous ligand diazepam binding inhibitor (DBI) in the lymphocytes of epileptic patients treated with various drugs. PBR levels were 50% to 80% higher in patients treated with carbamazepine, phenobarbital and valproic acid than in controls and untreated epileptics. DBI levels were significantly increased in the lymphocytes of untreated patients, and showed only a slight further increase after anticonvulsant therapy. The possibility that PBR and DBI modifications in the lymphocytes of epileptic patients may be linked to the immunological alterations reported in these patients and/or may represent possible markers of neurochemical modifications in the central nervous system is discussed.
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It is currently assumed that steady-state pattern electroretinograms can be obtained only at high rates of pattern reversal. However, steady-state responses can also be obtained at very low temporal frequencies (less than 1 Hz), provided that the reversal is sinusoidal. In five healthy volunteers, we studied the frequency characteristics of the pattern-reversal electroretinogram in response to sinusoidal gratings of 0.4 and 4 c/deg, reversed sinusoidally in contrast at frequencies ranging from 1 to 27 Hz. Steady-state responses dominated by the second harmonic component were obtained at all temporal frequencies tested; the amplitude of the second harmonic changed with stimulus temporal frequency. In the low-temporal-frequency range, stimuli of high spatial frequency appear to elicit larger contribution of generators with sustained or tonic characteristics compared with stimuli of low spatial frequency.
Anticonvulsant drugs, such as carbamazepine, may exert some of their effects through peripheral benzodiazepine receptors (PBR), which are present in glial cells and regulate the synthesis of neurosteroids. PBR have also been demonstrated in human lymphocytes, where they might be used as peripheral markers of anticonvulsant drug effects. In the present paper we investigated the interaction of various antiepileptic drugs with PBR of human lymphocytes and evaluated possible effects of acute and chronic treatment with these drugs. At normal therapeutic concentrations, diazepam, carbamazepine and phenobarbital occupy respectively 70, 30 and 10% of PBR sites in human lymphocytes. Although no change of receptor density or affinity was observed after acute in vitro treatment, in epileptic patients chronically treated with carbamazepine, phenobarbital and valproic acid, PBR Bmax was increased with respect to controls and untreated epileptics. Since PBR of human lymphocytes may be affected by anticonvulsant drug treatment, we suggest that they might be involved in the immunological alterations reported in these patients and might be used as peripheral markers of drug effects on the central nervous system.
The purpose of this study was to test the efficacy and safety of thymostimulin (TS) administered in addition to conventional chemotherapy in patients with intermediate- and high-grade non-Hodgkin's lymphoma (IG, HG-NHL). A total of 150 patients with newly diagnosed IG- or HG-NHL were entered in a multicenter trial to compare the effectiveness of two different third-generation regimens (MACOP-B versus ProMACE-CytaBOM) and were randomized to receive chemotherapy (CT) alone or CT + TS. In both regimens doxorubicin was replaced by a 20% higher dose of epidoxorubicin. TS was administered i.m. at a dose of 1 mg/kg daily on days 22-28 of each drug course to patients treated with ProMACE-CytaBOM, and on days 22-29, 50-57, and 77-85 to patients treated with MACOP-B. There were 134 fully evaluable patients: 68 treated with CT alone and 66 treated with CT + TS. Patients treated with CT + TS had a higher complete remission (CR) rate compared to patients given CT alone (59.1% vs 42.4%; P = .05). CR were significantly higher for patients treated with CT + TS in the groups with IG-NHL (P = .01), in those aged less than 60 years (P = .05), with good performance status (P = .05), and normal hemoglobin levels (P = .05). Four-year survival rates are 64.5% for patients treated with CT + TS and 43.0% for those treated with CT alone (P = .30). No difference between the two treatment arms have been observed as regards drug-related toxicity and the number and severity of infectious episodes. The use of TS during the 7 days before chemotherapy has been associated with a significantly superior CR rate. The advantage of CT + TS was mostly obtained in patients with IG-NHL, and those with good performance status or normal hemoglobin levels. In these patients TS may have potentiated the host reactions against the tumor, leading to an increase in NK activity and the production of cytokines. This postulated increase in the effectiveness of chemotherapy after TS might also explain the absence of the expected myeloprotective action.
We report the second infant of nonconsanguineous parents with epidermolysis bullosa junctionalis associated with urinary bladder exstrophy, epispadias, anteriorized anus, and bilateral inguinal hernias. The family history also included the death of a maternal cousin due to epidermolysis bullosa. Our diagnosis was based on electron microscopy and immunofluorescence evidence. This patient is reported because of the rarity of this constellation of findings.