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PubMed2002

[Gaucher's disease ].

Gaucher disease is an uncommon autosomic recessive disorder. The disease is caused by a deficiency in the activity of the lysosomal enzyme glucocerebrosidase which is responsible for the degradation of glucosylceramide, résulting from the breakdown of red and white cell-membranes. In the absence of enzyme glucosylceramide accumulates in the lysosomes of macrophages. This accumulation leads to hepatomegaly, splenomegaly with subsequent haematologic abnormalities (leucopenia, anemia, thrombopenia) and bone manifestations. Three types of Gaucher disease are described: type 1 is the most common, type 2 and 3 are associated with neurologic symptoms. Macrophages are the likely cellular source of biochemical abnormalities: elevated blood level of ferritin, angiotensin converting enzyme, immunoglobulins and haemostasis abnormalities. Lysosomal perturbations lead to increased blood level of tartrate resistant acid phosphatase and chitotriosidase. Enzyme replacement therapy is available in France since 1991. In 2002, 136 patients are treated. The efficacy is overt on the asthenia, organomegaly and haematological manifestations. Bone pains disappear or decrease in intensity, however bone complications may be irreversible justifying treatment initiations before the appearance of lesions that may lead to serious functional impairment.

Biomarkers↗
PubMed2002

[Primary adrenal insufficiency due to autoimmune adrenalitis].

We report a 10 years old boy, admitted with a history of asthenia, anorexia and weight loss of 4 kg. Initial laboratory work up showed metabolic acidosis and hyponatremia. The patient had no circadian rhythm of serum cortisol and an adrenal stimulation test confirmed the presence of adrenal insufficiency. Anti-adrenal antibodies were positive. Treatment with cortisol and fluorocortisone resulted in a complete remission of symptoms.

Adrenal Cortex Diseases↗
PubMed2002

Methotrexate-associated liver toxicity in a patient with breast cancer: case report and literature review.

A patient with breast cancer developed severe asthenia, accompanied with progressively increasing transaminases, during adjuvant chemotherapy with CMF (cyclophosphamide, methotrexate and 5-fluorouracil). Additional blood tests and imaging were negative. A liver biopsy revealed a grade II toxic hepatitis. Because methotrexate was suspected to be the cause of the hepatotoxicity, the administration of this drug was stopped and mitoxantrone was given instead. A recovery of clinical symptoms and normalisation of the liver function tests was observed afterwards. In that sense, mitoxantrone appears to be a valuable alternative to methotrexate in cases of hepatotoxicity in patients with breast cancer. An overview of the literature regarding methotrexate hepatotoxicity is presented.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

[Effectiveness and safety of gabapentin in the preventive treatment of migraine].

INTRODUCTION: Migraine is a frequent and disabling pathological condition with important socioeconomic repercussions. Recent studies have explored the use of antiepileptic drugs in the prophylactic treatment of migraine. Preliminary studies have shown that gabapentin is a drug that is effective and well tolerated by patients. AIM. To evaluate the effectiveness and safety of gabapentin in the prophylactic treatment of migraine. PATIENTS AND METHODS: A prospective, open, multicentre, random clinical study, carried out according to IHS criteria, which compares the effectiveness and safety of gabapentin in 1,200 mg/day and 2,000 mg/day doses as a preventive treatment for migraine over a 16 week period. RESULTS: Significant differences were found in patients treated with gabapentin, as compared with their basal state, in the following: a lower number of attacks (reduction in weeks 4, 10 and 16: 2.4 2.8, 2.9 2.9 and 3.1 2.9 attacks/month on a basal rate of 5.3 3.5 attacks/month), lower intensity (on a scale of 0 3 of increasing pain intensity: basal rate: 2.7 0.4, week 4: 1.8 0.9, week 10: 1.7 0.9, week 16: 1.4 1.0) and how long the pain lasts (basal rate 390 hours/month, week 4: 180 hours/month, week 10: 180 hours/month, week 16: 120 hours/month). No statistically significant differences were found between doses of 1,200 or 2,000 mg/day. Mild adverse effects were seen in 62 patients (37.8%): drowsiness (22.6%), asthenia (7.9%), dizziness (4.9%), abdominal pain (3.7%) and dazedness (3.7%). No serious adverse events occurred. CONCLUSIONS: Gabapentin can be considered an effective and safe drug in the preventive treatment of migraine.

Acetates↗
PubMed2002

[Study of 1,595 brucellosis cases in the Almeria province (1972-1998) based on epidemiological data from disease reporting].

OBJECTIVE: To know clinical, diagnostic, and treatment issues of brucellosis in the Almeria province. METHODS: Descriptive, epidemiological study from 1,595 disease report cards in the Almeria province during the 1972-1998 period. Issues concerning complications and treatment were reported in 890 cards (57.8%), symptoms, signs and hemograms in 565 (35.4%), and other diagnostic findings in all cards. The chi2 test was used to compare independent qualitative parameters for a 95% CI. RESULTS: In the early phase the most common symptom was asthenia (95.2%); sweating in the chronic stage (95.8%). The most commonly recorded sign was spleen enlargement (37.5%) and the most commonly reported complication was bone and joint involvement (22.8%). No changes in symptoms occurred over years. The hemogram revealed anemia in 30.3% of cases. Blood culture was positive for 91.2%. Regarding serological diagnosis, rose Bengal was positive for 99.2% of cases; the most common agglutination titer to Brucella or B. mellitensis was 1/640 (13.5%). The most commonly used therapy consisted of the association tetracycline plus streptomycin (42.4%). For 42.6% of cards, patients were transferred to hospital, particularly since 1984. CONCLUSIONS: Generally speaking, clinical data recorded in the reporting card regarding brucellosis disease reveal the clinical features of the disease, after a long surveillance period. Rose Bengal should be used as screening test for the diagnosis of this disease.

Brucellosis↗
PubMedDate not supplied

[Subjective non-specific symptoms related with electromagnetic fields: description of 2 cases].

In Italy, as in other countries, an apparently increasing number of subjects is reporting a variety of subjective symptoms that the subjects themselves refer to the exposure to electric, magnetic or electromagnetic fields (EMF) from nearby electric appliances, cellular phones, antennas, etc. Terms like electricity hypersensitivity (EHS), EMF hypersensitivity, or other similar, are frequently adopted to describe such symptoms; nevertheless, up to now, these terms are not entered the medical terminology. No accepted diagnostic criteria or procedures for the diagnosis of EHS are currently available. Furthermore, apart from the subject's self-attribution of the symptoms to EMFs, no direct cause-effect relationship between EHS symptoms and electromagnetic fields has been proved; additionally, evidence of a possible pathogenetic mechanism is lacking. In this paper, two cases developing symptoms of EHS ascribed to overhead power line in the proximity of their house are discussed. Nervous system (asthenia, depression, paraesthesias etc.), cardiovascular system (cardiac palpitations) and the skin (tingling, itching, etc.), are mostly (but not exclusively) involved. Based on available scientific knowledge, the rationale for an approach to subjects claiming for EHS is discussed. The establishment of a National archive for the collection of cases is communicated.

Electromagnetic Fields↗
PubMed1975

[Reactional status of leprosy].

Reactional leprosy is studied according to its clinical forms A) Lepromatous a) Acute lepromatization: encroaching and invasive nature; the patient becomes more and more lepromatous ; bad prognosis. b) Erythema nodosum: "contusiform dermatitis"; variable prognosis not so bad as it is in the preceding case; allergic nature and its evolution is usually detained and therapeutics efficient. c) Erythema multiform. d) Lucio's phenomenon: vascular lesions and consequently necrosis as a complication of the "erythema necrotisans" (beautiful leprosy). B) Tuberculoid Reactional tuberculoid is the only one in this benign type, the Mitsuda's test must always be positive and prognosis consequently good. C) Dimorphous or "Borderline" whose Mitsuda's test is mostly negative, sometimes positive, but not stable. The lesions may stimulate the tuberculoid leprids but they invade mucous membranes, are impregnated by pigmentation, may present the Unna's band, and other characteristics of the Lepromatous type. Are associated (fever, asthenia and emaciation). Prognosis not very good, because of the possibility of lepromatization, according to its tendency. Evolution slower and frequent relapses. Besides there are nodular lesions. Pathogeny 1) Perifocal allergic reaction (Jadassohn). Similar to epituberculosis and Herxheimer reaction. 2) Septicemia. Sensitized tissues inside or outside the lesions, are invaded by the bacilli and so the allergic reaction takes place. Even without culture resources, Mycobacterium leprae has been found in the blood by direct examination. 3) Autoimmunization (Waldenstrom, Matthews and Trantman, 1965). Based upon the similarity between both humoral syndromes, in leprosy reactions and collagenous, diseases, as to: hypergammaglobulins, hypercryoproteins, antigammaglobulins, serological reactions (Wassermann, Kahn, Kline, VDRL) positives, Antistreptolysin O, protein C reactive, antinuclear factors, latex and Wadler-Rose test positives (rheumatoid tests) lowering of complement. If leprosy reaction is like this, it should be the less agressive of the autoimmune diseases. a) Its eruptions are cyclic not of long standing duration, as a general rule. b) Its prognosis has been recognized as good, except lately, because of the use of corticoid therapy which has been fatal, in many cases. After some years the leprosy reaction cures spontaneously. Treatment (see article)

Erythema Multiforme↗
PubMed1999

[Psychological issues in manned spaceflight].

As the duration of manned spaceflight becomes longer and as crews become more heterogeneous, psychological and interpersonal factors will be more important in affecting the safety of crew and flight mission. In space environment there are four types of stressors: physical, physiological, psychological and interpersonal. Psychological issues include "Asthenia", alteration in time sense, transcendent experiences, sleep problem, career motivation, psychosomatic symptoms and psychiatric issues. Interpersonal issues include interpersonal tension, interpersonal relationships decreased cohesiveness and deprivation, displacement [correction of dispiacement] of anger to outside personnel over time.

Adaptation, Psychological↗
PubMed2002

[The therapeutic use of D9-tetrahydrocannabinol (dronabinol) in refractory neuropathic pain].

INTRODUCTION: Despite the recent discovery of the potential mechanisms underlying the analgesic effects of cannabis, few clinical studies have so far assessed its analgesic effects, notably in the treatment of chronic non-malignant pain. All the studies used administration of cannabis alone. The aim of this open, pilot, study was to assess the efficacy and side effect profile of oral dronabinol (tetrahydrocannabinol - THC) in the treatment of refractory neuropathic pain. METHODS: Seven patients (3 women/4 men), aged 60 +/- 14 years, suffering from chronic refractory neuropathic pain, received oral THC titrated to the maximum dose of 25 mg/day (mean dose: 15 +/- 6 mg), during an average of 55,4 days (range: 13-128). Various components of pain (continuous, paroxysmal and brush-induced allodynia) were assessed using VAS scores. Health-related Quality of Life (HRQL) was evaluated using the Brief Pain Inventory, and the Hospital Anxiety and Depression scale was used to measure depression and anxiety. RESULTS: THC did not induce significant effect on the various pain, HRQL and anxiety and depression scores. Numerous side effects (notably sedation and asthenia) were observed in 5 patients out of 7, requiring premature discontinuation of the drug in 3 patients. CONCLUSION: The present study did not reveal any significant efficacy of THC in a small cohort of patients with chronic refractory neuropathic pain, but underlined the unfavorable side effect profile of the drug. These results may partly relate to the fact that oral dronabinol exhibits a poor therapeutic ratio (efficacy at the price of side effects). The development of new and better tolerated cannabinoids is warranted.

Aged↗
PubMedDate not supplied

Tolerability of Raltitrexed ('Tomudex') in elderly patients with colorectal cancer.

PURPOSE: Colorectal cancer (CRC) is one of the major health problems of the Western world and the proportion of elderly patients with CRC is growing. Raltitrexed ('Tomudex'), a specific thymidylate synthase inhibitor, has shown efficacy and manageable toxicity in elderly CRC patients. In this retrospective study, the tolerability of raltitrexed in patients with CRC was examined in relation to age. PATIENTS AND METHODS: Toxicity parameters, graded according to World Health Organization criteria, were assessed in two patient groups: < 70 and > or = 70 years old. In total, 56% (50 out of 90) of patients treated with raltitrexed (3 mg/m2 as a 15-minute intravenous infusion every 3 weeks) were aged > 70 years (M:F 28:22; Eastern Cooperative Oncology Group performance status 0-1:2 38:12). RESULTS: Overall, 437 cycles of chemotherapy were administered and grade 3-4 toxicity was reported in < 10% of patients. There were no clinically significant differences between the two age groups, apart from grade 3-4 asthenia, which was reported by 6% and 0% of patients aged > or = 70 and < 70 years, respectively. This was in spite of a significantly lower calculated mean creatinine clearance in patients aged > or = 70 years compared with those patients < 70 years of age. CONCLUSION: The raltitrexed toxicity profile does not appear to be significantly influenced by age; however caution is recommended in the management of elderly patients, particularly in the presence of impaired renal function.

Adenocarcinoma↗
PubMedDate not supplied

A new cisplatin/gemcitabine schedule in locally advanced (IIIB) and metastatic (IV) non-small cell lung cancer: relationship between dose-intensity and efficacy. A phase II study.

BACKGROUND: Cisplatin/gemcitabine are one of the "standard" chemotherapy schedules in locally advanced and metastatic NSCLC cancer. A number of trials documented that omission of gemcitabine on day 15 and reduction of cisplatin up to 70 mg/mq are equivalent in term of response rates to "classic" administrations on days 1, 8 and 15 with cisplatin 100 mg/mq. The aim of this study was to confirm this evidence and to demonstrate that a further reduction of gemcitabine dose-intensity may be performed with the same efficacy on response. PATIENTS AND METHODS: Fifty untreated patients with locally advanced and metastatic NSCLC entered the study: 24 stage IIIB and 26 stage IV. The median age was 65 years (range 32-76); 44 males and 6 females Genicitabine was administered 1000 mg/mq weekly on days 1 and 8 followed by a 2-week rest and cisplatin 80 mg/mq on day 2 of each 28-day-cycle. RESULTS: Forty-five patients were evaluable for response and all for toxicity. The overall response rates were 35.5% with 16 partial responses (95% Confidence Interval: 32%-61%). Most of the objective responses were seen in IIIB patients (56% of the stage IIIB and 44% of the stage IV patients responded). According to the intent-to-treat-principle, the response rates were 32% (16 out of 50 patients). The median dose-intensity of gemcitabine and cisplatin was respectively 477.6 mg/mq/week (481.4 for responders) and 19.5 mg/mq/week (19.9 mg/mq for responders). The median response duration was 5 months (range 1-18) and the median time to progression was 5 months (1-21); median survival was 9 months (range 2-31). The main toxicity was haematological: thrombocytopenia grade IV in 5 patients (10%) and grade III in 11 patients (22%); neutropenia grade III-IV in 4 patients (8%); grade III anemia in 3 (6%). Asthenia was the most significant non-haematological toxicity and was observed in 19 patients (38%). CONCLUSION: This trial confirmed the efficacy of a schedule with 2 administrations of gemcitabine (on days 1, 8) and a cisplatin dose on day 2 lower than 100 mg/mq. Moreover, the same efficacy was obtained with a median-dose intensity of cisplatin and gemcitabine lower than planned in a 21-day-schedule. For safety and low toxicity, we think that this schedule provides another chance to treat patients with non-small cell lung cancer, especially the elderly or patients with coexistent medical illnesses.

Adult↗
PubMed2002

Whipple's disease. Case report.

The paper reports the clinical case of a 58 -year-old male patient admitted for diarrhea (6-7 stools/day, diffuse abdominal pain, borborygma, weight loss (20 kgs in two years), asthenia and fatigue. Physical examination evidenced a poor nutritional state (body mass index 19 kg/m2). The abdomen was slightly distended. Biological tests evidenced moderate/severe anemia, hypoproteinemia and hypoalbuminemia. Endoscopic examination evidenced oedematous duodenal mucosa with white-yellowish deposits. Histology (HE stain) revealed the presence of foamy cells and the PAS-staining of the duodenal mucosa evidenced PAS-positive macrophages and numerous intracellular bacilli. Penicillin therapy 2 x 1 million U/day for 14 days, followed by tetracycline 4 x 250 mg/day improved the clinical picture, the patient had only one stool per day and gained weight. After 7 months of treatment the general condition was good and the patient had gained 17 kgs, the duodenal mucosa was normal. HE staining did not evidence foamy cells and no PAS-positive macrophages could be found.

Abdominal Pain↗
PubMed2003

Weekly docetaxel as neoadjuvant chemotherapy for stage II and III breast cancer: efficacy and correlation with biological markers in a phase II, multicenter study.

PURPOSE: This is one of the first reports of weekly docetaxel (Taxotere) in the neoadjuvant treatment of stage II and III breast cancer. We evaluated docetaxel's efficacy and safety and analyzed correlations between response and the expression of c-erbB2, ER status, and Ki-67 labeling index. EXPERIMENTAL DESIGN: Patients with previously untreated, stage II and III breast cancer were entered into the study. Docetaxel (40 mg/m(2)) was given i.v. once weekly for the first 6 weeks of an 8-week cycle for 2 cycles. RESULTS: A total of 56 patients were evaluated by intention-to-treat analysis for efficacy and safety. The overall clinical response rate was 68% (complete and partial response, 29 and 39%, respectively). Nine patients (16%) achieved a pathological complete response. There was no correlation between response to docetaxel and the expression of molecular markers, however, the majority of the pathological complete responses were observed in patients with c-erbB2-negative tumors. Nonhematological toxicity was more common than hematological toxicity, with alopecia and asthenia the most frequently reported adverse events (89 and 77% of patients, respectively). Severe hematological toxicity was rare. CONCLUSIONS: Weekly docetaxel appears to be very effective in the neoadjuvant setting. A high pathological response rate was achieved with tolerable toxicity.

Adult↗
PubMed2002

Giardia lamblia infection in a patient with myotonic dystrophy.

Myotonic dystrophy is an autosomal dominant muscle disorder characterized by muscle wasting and weakness and a number of other systemic abnormalities. Some patients have hypo-IgG that is asymptomatic in most of them. We report the case of a 42-year-old woman with myotonic dystrophy and hypo-IgG who experienced asthenia and weight loss secondary to Giardia lamblia bowel infection.

Adult↗
PubMed2003

[Acute renal failure following ingestion of Cortinarius orellanus in 12 patients. Initial presentation and progress over a period of 13 years].

INTRODUCTION: This study reports the largest series of acute renal failure following collective poisoning by Cortinarius orellanus since 1957. PATIENTS: Twelve men, in whom altered renal function appeared following ingestion of mushroom soup (Cortinarius orellanus) when they were 20 to 23 year-old, were followed up for 13 years. RESULTS: After a period of latency of between 2 to 5 days, the patients complained of asthenia, intense thirst and digestive and neurological disorders. On admission, 4 were anuretic and two exhibited polyuria. Leukocyturia was detected in all patients but without proteinuria. Renal biopsy was performed on day 14 in seven patients. It revealed severe tubulo-interstitial lesions with polymorphous cell infiltration, oedema, loose fibrosis and epithelial necrosis. Eight patients required haemodialysis. Nine patients received corticosteroids for less than 6 months. Over a follow-up period of 13 years, seven patients recovered normal renal function, four underwent transplantation and one was still under haemodialysis and died, victim of a car accident. CONCLUSION: The incidence of acute renal failure varies from 30 to 46%. It depends on individual sensitivity, pre-existing nephropathy and the cumulated dose of toxin ingested. Early and severe interstitial fibrosis, marked interstitial oedema and tubular epithelial necrosis are the most characteristics renal lesions. Renal failure regresses progressively over several months in 60% of cases. In the other patients, terminal renal failure appears immediately or after several years. The evolution is not influenced by corticosteroid therapy.

Acute Kidney Injury↗
PubMed2003

[Frequency of anaemia and indications for treatment with epoetin in chronic renal failure at the pre-dialysis stage].

OBJECTIVES: To determine the degree of anaemia corresponding to the decreasing levels of renal function in patients with chronic renal failure (CRF) and not yet on dialysis, and to assess the indications for treatment with recombinant erythropoeitin (epoetin). METHODS: We studied the relationship between haemoglobin (Hb) concentration and creatinine clearance (Ccr) in 403 consecutive patients with CRF regularly monitored in nephrology consultations between January 1 and June 30, 1999, and who received appropriate iron and vitamin supplementation. These patients were then followed-up until June 30, 2000 or until maintenance dialysis was initiated. RESULTS: There was a significant and close correlation between the degree of anaemia and renal dysfunction. An Hb value<11 g/dl (corresponding to the present threshold for the indication of epoetin) was observed in 62% of patients with creatinine levels>400 micromol/l and in 58% when Ccr was<20 ml/mn/1.73 m2. Whatever the level of CRF, the degree of anaemia was higher in the women than in the men. Among the 123 patients who had to start maintenance dialysis during the observation period, 85 (69%) were treated with epoetin before dialysis was started. CONCLUSION: In patients with CRF, clinically symptomatic anaemia is more frequent than imagined, and early treatment is required. Regular monitoring of Hb and iron levels is mandatory in order to allow patients to benefit from timely initiation of epoetin and thus prevent the development of disabling asthenia and other deleterious consequences of anaemia.

Adult↗
PubMed2002

[Simultaneous leiomyoma and transitional carcinoma of the bladder. Report of a case].

OBJECTIVES: To report this case of bladder leiomyoma, benign tumor of the bladder, due to its low frequency (0.43%) and to perform a bibliographic review. METHODS AND RESULTS: Case of a 46 year old female who refers a history of voiding symptoms, macroscopic haematuria with clots, asthenia, anorexia and right flank pain for 15 days prior to consultation. After blood tests, X ray tests, ultrasound and CT scan she underwent hysterectomy, anexectomy, radical cystectomy and ureteral reimplantation to a Mainz II sigmoid pouch. Currently, 44 months after surgery her outcome has been satisfactory. CONCLUSION: Bladder leiomyoma, a disease without specific symptoms, is occasionally found in pathological evaluation; biopsy is the confirmative diagnostic test. They are rarely found outside the uterus and gastrointestinal tract.

Carcinoma, Transitional Cell↗
PubMed1976

[Antigen therapy in chronic brucellosis].

The different forms of chronic brucellosis have been improved and well stabilized in the proportion of 75% by repeated injections of a vaccine consisting of suspensions of heat-killed B. melitensis. The vaccine should be used in a series of 8 subcutaneous injections, in increasing doses, at the rate of 2 injections a week. To obtain good results it is necessary to give an average of 3 to 4 series of injections, with a two months' interval between each series. After the 2nd series a distinct decrease of the patient's reactions to retarded hypersensitivity tests are generally observed. The most favourable results are obtained in cases of common subjective syndrome with physical and psychical asthenia.

Antigens, Bacterial↗