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[Celiac disease. Recent findings on its pathogenesis, diagnosis and clinical presentation].

Coeliac disease is a gluten-sensitive enteropathy which results in a permanent malabsorption of nutrients in that portion of the small intestine (the jejunum) that is damaged. A genetic, inheritable disease, it is directly related to ingestion of certain wheat proteins especially found in rye secalins, barley hordeins and, in a much lower amount, oat avenins. A fundamental role in the pathological response is played by grain prolamins (gliadins). The actual damage to intestinal mucosa is almost certainly mediated by the immune system but its mechanism has not been so far clarified. Coeliac disease incidence rate is ever increasing among children and adolescents and it is rather frequently reported as relapsing in the third and fourth decade. The most distressing problems of malabsorption syndrome are diarrhea, weight loss, meteorism, abdominal pain, vomiting and asthenia; nonetheless, not all patients report symptoms. Both diagnosis and differential diagnosis--intestinal lymphoma, refractory sprue--prove difficult: a diagnosis of gluten intolerance can be made through careful consideration of a series of laboratory findings which are being improved by researchers in order to avoid delays for patients with probable gluten-sensitive enteropathy with non-specific symptoms. Although there may be many clinical signs and laboratory tests indicating probable malabsorption, the likely gold standard of diagnosing coeliac disease remains to be the jejunal biopsy.

Autoimmune Diseases↗

[Hysteria and psychosomatic disorders in Portuguese immigrants].

Seventy patients presenting symptoms of hysteria (49 women and 21 men) were selected among patients observed at the Institute Minkowska during the year. This work is part of a research work on socio-cultural and environmental factors which can change mental status of immigrants. These are all portugese workers presenting for the first time atypical mental troubles called by the author: "bastard hysterical syndrome of the immigrant" and characterized partly or totally by the following symptoms: fatigue, anxiety, sense of suffocation, dyspnea, coughing, unilateral chills or generalized chil, abdominal or gastric pains, headaches and "diffused pains", paresthesia, aching back, tears and sorrow, fear of dying or having a cancer, asthenia, leg paresthesia and contractions, vomiting, diarrhea, cardiac pains, palpitations, dizziness and collapsing. These troubles appear sometimes without apparent motives but they are almost always due to a precipitating cause expressed by the patient: a delivery, a familial death, a homosexual proposition, a trauma without importance, a working conflict etc... But the most frequent cause invoked is "the french climate" without knowing precisely what the word "climate" means: atmospheric conditions, athmosphere or reception milieu? This latest interpretation seems more likely after months of psychotherapy. Most patients are not french speaking and cannot write; their origin is rural (familial villages well structured regarding their food and sexual economy), and people well "armed" by a system of defense mechanisms and well adopted conditioned reflexes. In this work, hysteria of the portugese immigrant is compared to childhood hysteria. As the hysterical burst of the child is aimed at calling attention, love of the mother, at finding a solution to a familial or social conflict, the hysterical burst of the immigrant is aimed at the absent family or at its substitutes, the bos, social security, the doctor. Furthermore, the attitude of the hosting Country--wanting and rejecting--is very ambivalent; "tenderness" at the time of reception, followed by indifference. Early attentions are followed by constant interdictions (threat of unemployment, false statements on sexual dangers of the immigrant etc;..). The immigrant, like the hysterical child, is periodically controlled (work and visit cards), supervised (supervisors), The narcistic satisfactions of being called a good worker can be followed by threats of firing in economic crisis. The society of the hosting country requires the immigrant to be identical to this society: language, physical appearance, food. The real paradoxical situation to which the immigrant is confronted and the real or hypothetical fears constitute conditions of experimental neurosis, to which portugese immigrants react very often by a bastard symptomatology of hysterical type, characteristic of displaced man. These preliminary studies are the frame for a future epidemiological survey in this specific population.

Adult↗

Antidepressive properties of I-glutamine. Preliminary report.

A brief review of the literature on this topic, and a comparison of the results obtained in children by administrating l-glutamine with those obtained by other antidepressive drugs, is primarily presented. Having been tested in a sampler of 43 adults, affected by different forms of depressive, neurasthenic and dissociative illnesses, 1-glutamine is indicated as owning clear anti-depressive properties, and the asthenia as target-symptom for its therapeutical use. Out of the endogenous depression with slowed-down motor activity, indeed good results were found in all cases, in which a "vital" level had been reached by the illness. Since 1-glutamine is noticed being a precursor of GABA, this fact itself could explain its antidepressive properties, but it is necessary to remember that they very complex metabolism of the drug is still mostly unknown.

Adult↗

Irinotecan versus infusional 5-fluorouracil: a phase III study in metastatic colorectal cancer following failure on first-line 5-fluorouracil. V302 Study Group.

In a multicenter phase III trial, 267 patients with nonbulky metastatic colorectal cancer who had failed first-line 5-fluorouracil (5-FU) therapy were randomized to receive second-line treatment with either the new topoisomerase agent, irinotecan (Rhône-Poulenc Rorer, Antony, France), or a high-dose infusional 5-FU regimen. Patients treated with irinotecan survived significantly longer than those treated with infusional 5-FU. The 1-year survival rate was 45% for patients receiving irinotecan compared with 32% for patients receiving 5-FU. There also was a significant difference in median progression-free survival (4.2 months v 2.9 months; P = .03) favoring irinotecan. Irinotecan administered at a dose of 350 mg/m2 every 3 weeks was associated with manageable toxicities (the main toxicities were neutropenia and diarrhea). The incidence of these adverse events and of vomiting was higher with irinotecan than with the comparator regimens of infusional 5-FU. However, the incidence of grade 3-4 asthenia was the same in the two treatment arms and mucositis and cutaneous adverse events were more common with 5-FU. Overall, mean global quality of life scores were similar in the two arms of the study throughout the period of treatment and follow-up, demonstrating that the more effective disease control achieved by irinotecan at least maintains quality of life. Indeed, deterioration in quality of life (defined as >50% decrease from baseline score) occurred significantly later in irinotecan-treated patients. In light of these data, irinotecan should be considered the reference treatment for patients with 5-FU-refractory advanced colorectal cancer.

Antimetabolites, Antineoplastic↗

Phase I trial of docetaxel with filgrastim support in pediatric patients with refractory solid tumors: a collaborative Pediatric Oncology Branch, National Cancer Institute and Children's Cancer Group trial.

Neutropenia is the dose-limiting toxicity of docetaxel in children. This Phase I trial was designed to determine the maximum tolerated dose, the dose-limiting toxicities, and the incidence and severity of other toxicities of docetaxel with filgrastim (G-CSF) support in children with refractory solid tumors. Docetaxel was administered as an i.v. infusion for 1 h every 21 days with a starting dose of 150 mg/m2 and an escalation to 185 mg/m2 and 235 mg/m2 in subsequent patient cohorts. G-CSF (5 microg/kg/day) was administered s.c., starting 48 h after docetaxel and continuing until the post-nadir neutrophil count reached 10,000/microl. Seventeen patients received 27 courses of docetaxel with G-CSF support. Generalized erythematous desquamating skin rash and myalgias were dose-limiting at 235 mg/m2. Localized and generalized rashes were seen at all of the three dose levels. Neutropenia (median nadir, 95/1microl) occurred at all of the dose levels but was brief in duration and not dose-limiting. Thrombocytopenia was minimal (median platelet count nadir, 139,000/microl), and the severity of neutropenia and thrombocytopenia did not seem to be related to the docetaxel dose. Other docetaxel-related toxicities included hemorrhage (associated with mucositis), sepsis, hypersensitivity reaction, transient elevation of liver enzymes, stomatitis, back pain, asthenia, and neuropathy. One minor response was observed in a patient with colon cancer. The maximum tolerated dose of docetaxel with G-CSF support in children is 185 mg/m2, which is 50% higher than the maximum tolerated dose of docetaxel alone in children and 85 % higher than the recommended adult dose.

Adolescent↗

[A case of peritoneal tuberculosis. Contribution of mini-endoscopy].

BACKGROUND: Tuberculosis in the genital and peritoneal region is increasing in last years. For this reason, the high value and efficacy of laparoscopy using a small caliber endoscope for the diagnosis of this disease, which often presents no specific and heterogeneous clinical features, is underlined. METHODS: A case of peritoneal tuberculosis in a young female presenting fever, asthenia and peritoneal effusion, is reported. Blood tests, X-ray and cytological examination of the peritoneal fluid all failed to point out the right diagnosis. Then multiple biopsies of the peritoneum and the external surface of uterus and ovaries were made using laparoscopy. RESULTS: Laparoscopy clearly showed the miliary nodules. The histology showed multiple granulomas composed by inner caseous necrosis and outer layer of epithelioid histiocytes and Langhans cells, leading thus to the diagnosis of peritoneal tuberculosis. The patient, treated with streptomycin and rifampicin, five months after diagnosis, did not show any feature of tubercular disease. CONCLUSIONS: Because of its safety, laparoscopy is a very useful and powerful diagnostic technique especially in those young women presenting with painful abdominal symptoms without any clear evident cause.

Adolescent↗

[Amebic liver abscesses with bacterial superinfection in a nonendemic area].

A 26-year-old man was admitted to hospital with asthenia, weight loss, right upper quadrant abdominal pain, diarrhea without blood, and fever. Abdominal ultrasonography showed multiple hypoechoic areas in the left hepatic lobe. On abdominal CT, multiple hypodense areas without contrast capture were consistent with hepatic abscesses. Cultures were obtained and the patient was placed empirically on metronidazole, gentamicin and ampicillin, without improvement in the first 72 hours, and a drain was placed in the largest lesion collecting a purulent material. The abscess culture was positive for group C beta-hemolytic Streptococcus. Entamoeba histolytica serology was positive and colon biopsies revealed trophozoites. Multiple left hepatic lobe abscesses secondary to E. histolytica, with bacterial superinfection, is an unusual presentation of amoebic infection, considering that Spain is not an endemic area.

Adult↗

[The psychopathology of tumors of the 3rd cerebral ventricle].

36 patients with tumors of diencephalic region of different histologic nature involving cavity of the third ventricle of the brain were observed. 27.7% of the patients had disorders of mental and physical development from the very first years of life. In presurgical period there was a wide spectrum of paroxysmal and permanent mental disorders from asthenia to rude disturbances of memory and consciousness which regressed or disappeared after the operation. The correlation was observed between such disorders, histologic structure and location of the tumor; individual characteristics of the patient (age, dextrality, sinistrality); scope and character of the operation (approach to the tumor, surgical interventions on the structures of the third ventricle).

Adolescent↗

UFT and oral calcium folinate as first-line chemotherapy for metastatic gastric cancer.

Locally advanced or metastatic adenocarcinoma of the stomach still carries a poor prognosis, with 5-year survival rates of < 15%. Palliative chemotherapeutic regimens for this disease are largely 5-FU-based. We have investigated the clinical activity of an oral combination of uracil and tegafur (UFT) with calcium folinate (Orzel), in patients with measurable metastatic disease. Thirty-six patients received a total of 94 courses of daily UFT 300 mg/m2 plus calcium folinate 90 mg for 28 consecutive days followed by a 7-day rest. Planned treatment doses were maintained in 83% of all evaluable courses. Main toxicities included diarrhea (21 patients) and nausea and vomiting (20 patients). Other side effects were asthenia, malaise, stomatitis, and myelosuppression. At present, 26 patients are evaluable for response. Of these, one achieved a complete response and three achieved partial remissions. In addition, six patients reached stable disease, yielding an overall response rate of 27%. We conclude that the combination of UFT and calcium folinate is a feasible outpatient regimen that warrants further clinical evaluation.

Adenocarcinoma↗

Accidental veterinary antibiotic injection into a farm worker.

A 29-year-old white male farm worker accidentally injected tilmicosin, a bovine antibiotic, into his finger. He developed temporary pulmonary, gastrointestinal, and neuromuscular symptomatology and a more persistent subjective asthenia.

Accidents, Occupational↗

[Giant benign gastric ulcer penetrating into the liver, pancreas and mesocolon].

Penetration of the liver, pancreas and transverse mesocolon by a giant benign gastric ulcer is relatively uncommon, and literature contains a few reports of this complication. The preoperative histological diagnosis may be difficult or impossible. A 63-year-old female patient with a history of seven months of lack of appetite, asthenia, epigastric pain, a remarkable weight decrease, presenting at physical examination a large, smooth margins, not pulsating, quite fixed abdominal mass, is reported. Echography confirmed the presence of a mass of approximately 14 x 19 cm, with solid and liquid content. Biopsy showed inflammatory elements and cellular detriti. Barium enema showed that the mass compressed the descendent colon, which appeared dislocated. Tumor markers (CEA, CA 19-9, alpha-fetoprotein) where in the normal range. Endoscopy showed a giant angular ulcer whose bottom was represented by necrotic material (after the definitive histological examination it proved to be hepatic tissue). At TC scan of the abdomen, a remarkable thickening of the gastric wall was present. At surgery the stomach appeared increased in volume, with remarkably thickened walls, tenaciously sticking to II and III hepatic segments, to the pancreas and transverse mesocolon. A total gastrectomy was performed because of the depth of the ulcer penetration and the extension of the alteration of the gastric wall, even if the giant gastric ulcer, in the literature, is more frequently benign than malignant.

Anastomosis, Roux-en-Y↗

[Benign hypertrophy of the prostate: which treatment, for whom?].

Approximately 25% of men over 40 or 50 suffer from lower urinary tract symptoms (LUTS) caused by benign prostatic hyperplasia (BPH). The bothersomeness of the symptoms varies considerably from one individual to the other and can fluctuate with time. Symptoms tend to gradually worsen as time goes on through. Surprisingly, there appears to be no particular relationship between symptoms and the overall prostate size and weight. Symptoms of BPH are divided in obstructive (voiding) and irritative (storage) symptoms, of which the irritative are the most bothersome. Until recently, surgery (open or transurethral resection) was the only treatment option. Nowadays, a range of less invasive treatments and pharmacological therapies are available to relieve BPH symptoms. Finasteride, for instance, reduces the prostate size by blocking 5-alpha-reductase, the enzyme which plays a role in the growth of the prostate. It takes however a long time before a clinically significant effect is noticed: +/- 6 to 12 months. Then, there are alpha 1-blockers. These agents result in relaxation of prostatic and bladder neck smooth muscle. alpha 1-blockers act relatively fast. Most alpha 1-blockers used in the treatment of symptomatic BPH were originally developed to treat hypertension. The adverse events most commonly associated with alpha 1-blockers, such as dizziness, headache, asthenia, tachycardia/palpitation, postural hypotension and syncope are possibly related to the blood pressure lowering effect. This stimulated the search for more selective alpha-blockers which act predominantly on the prostate and have less effect on the blood levels (afluzosin: Xatral and tamsulosin: Omic). Presently, alpha-blockers have become the first-line drugs in the medical treatment of symptomatic BPH. Surgery (open or TURP) is limited to patients with recurrent infections, large residue (> 200 ml), recurrent hematuria, bladder stones. New alternative and minimally invasive treatment such as TUNA generate necrotic lesions within the prostate through needle introduced endoscopically. This leads also to marked improvement in patients symptomatology.

5-alpha Reductase Inhibitors↗

[Primary non-Hodgkin's lymphoma of the stomach (a rare case of extensive spread to the entire organ)].

Primary gastric lymphoma is the most frequent extra nodal primary site for non-Hodgkin's lymphoma (NHL) and is itself uncommon. Moreover, a massive infiltration of all stomach (from cardias to antrum) simulating a linitis plastica, it's rare. We present a case report of this atypical presentation of primary gastric NHL in a 73 year old females. The patient came to our observation complaining of dyspepsia, epigastric pain and vomiting from 7 months associated with weight loss and asthenia. Physical examination revealed an epigastric palpable mass. Computed tomographic findings has been necessary to confirm that the massive infiltration of gastric wall (from cardias to pylorus) was ascribed to lymphoma. Dawson's criteria was respected to define primary gastric NHL and was performed a total gastrectomy with systematic lymphadenectomy. The histopathological evidences have confirmed clinical diagnosis of primary gastric NHL. Preoperative diagnosis to clarify the nature of lesions (primary or not) and accurate staging of neoplasm before the operation are indispensable for a correct therapeutic approach; in according to the Ann Arbor classification modified by Musshoff our cases was stage IIE and radical gastrectomy with systematic lymphadenectomy was performed. Surgical resection is generally considered to have a definitive role in the treatment of primary gastric lymphoma specially for the stage IE and IIE.

Aged↗

Docetaxel in the treatment of non-small cell lung cancer: review of single-agent trials.

Several phase II studies have evaluated docetaxel, administered as a 1-hour intravenous infusion at a dose of 100 mg/m2 every 3 weeks, for chemotherapy-naive patients with advanced non-small cell lung cancer. Results have been consistent across numerous trials, with an overall response rate in the range of 23% to 38% and a median survival of 9 months. Results of a multicenter phase III trial of docetaxel versus best supportive care for the first-line treatment of non-small cell lung cancer are pending. In the second-line setting, after failure of first-line platinum-based chemotherapy, four phase II studies of docetaxel 100 mg/m2 have achieved response rates ranging from 16% to 22%, with encouraging median survival times of 30 to 42 weeks. Preliminary results of a large, multicenter, randomized phase III trial also indicated an advantage for docetaxel over control with regard to response, time to progression, survival, and quality of life. Results of a multicenter phase III trial of docetaxel versus best supportive care as second-line treatment will be reported soon. Weekly docetaxel has been well tolerated in phase I studies, with dose-limiting toxicity being asthenia rather than myelosuppression. Phase II trials of a dosage of 36 mg/m2/wk in elderly patients with advanced non-small cell lung cancer are ongoing. Docetaxel is a potent radiosensitizer. The dose-limiting toxicity of docetaxel when administered weekly with concurrent chest radiation at 50 to 64 Gy is esophagitis. Phase II trials of weekly docetaxel plus concomitant chest radiotherapy are in progress at the recommended phase II dosage of 20 to 30 mg/m2/wk.

Antineoplastic Agents, Phytogenic↗

[Trichothiodystrophy: progresssive manifestations].

INTRODUCTION: Trichothiodystrophy is an autosomal recessive genodermatosis associating congenital dysplasia of the hair and neuroectodermal defects. Clinical expression is variable, although abnormalities are generally noted from birth. We report trichothiodystrophy in two brothers with the same phenotype who presented unusual progressive manifestations. OBSERVATIONS: Case 1: A six-year-old boy was seen for vesicular blisters due to photosensitization. Clinical examination showed dry, brittle, unmanageable hair, discrete koilonychia-type nail defects and an ichthyosiform state. The teeth were normal. In addition to psychomotor retardation, the patient presented a dysmorphic syndrome (poorly rimmed low-set ears; thick, triangular upper lip; scaphocephalic skull; short hands) and congenital bilateral cataract. The diagnosis of trichothiodystrophy was confirmed by a study of DNA repair after exposure to ultraviolet light. A repair defect was found similar to that in xeroderma pigmentosum group D. The patient experienced a worsening of psychomotor retardation and episodes of hair loss with edema and inflammation of the scalp resulting from infections. He also showed marked asthenia which resolved spontaneously within a few months. Case 2: The other brother, born as a collodion baby, presented the same clinical picture (cutaneous, exoskeletal, dysmorphic), including congenital bilateral cataract, photosensitivity and a parenchymatous blister-type pulmonary lesion probably secondary to bronchiectasis. The patient's cutaneous state progressively improved. He was seen at six years of age for an episode of inflammatory edema of the scalp with hair loss. Within six months, all of the hair redrew. The diagnosis of trichothiodystrophy was confirmed by a DNA repair defect after exposure to ultraviolet light. DISCUSSION: Trichothiodystrophy is clinically associated with photosensitivity (P), ichthyosis (I), dry, brittle hair (B), intellectual impairment (I), decreased fertility (D) and short stature (S), which accounts for the acronym PIBIDS or IBIDS syndrome, depending on whether photosensitivity is involved or not (actually in about 50 p. 100 of cases). Other possibly associated features include ungueal dysplasias, bilateral cataract, defective teeth, dysmorphic disorders predominant in the ears, neurologic disorders, pulmonary bronchiectasis and recurrent infections. The two cases presented here were thus very symptomatologically complete. The two problems of current concern are psychomotor retardation and temporary hair loss as a result of infections. The latter has only been described once in the literature. This case was similar to ours since photosensitivity was involved. Analysis of DNA repair also showed a defect after exposure to ultraviolet light similar to that found in xeroderma pigmentosum group D. Thus, episodic hair loss could be a symptom characteristic of forms of trichothiodystrophy with a DNA repair defect. However, the explanation for this hair loss is not known. Other ectodermal dysplasias can be complicated by hair loss with superinfection, such as AEC syndrome (ankyloblepharon, ectodermal dysplasia, cleft palate).

Abnormalities, Multiple↗

A review of the antiepileptic drug tiagabine.

Tiagabine (TGB), a recently approved antiepileptic drug (AED), has a specific mechanism of action that is unique among AEDs. A potent AED with linear, predictable pharmacokineties, it inhibits gamma-aminobutyric acid (GABA) reuptake into neurons and glia. Tiagabine does not have any clinically relevant effects on hepatic metabolism or on serum concentrations of other AEDs, nor does it interact with commonly used non-AEDs. The most common side effects of TGB in controlled studies are dizziness, asthenia, somnolence, accidental injury, infection, headache, nausea, and nervousness. These events are usually mild to moderate in severity and generally do not require medical intervention. At dosages of 30-56 mg daily, TGB is an effective add-on treatment for partial seizures. Although patients who have medically refractory epilepsy can be converted to TGB monotherapy, more controlled studies are necessary to confirm the efficacy of TGB as monotherapy and to determine the effective dosage range.

Animals↗

Docetaxel is effective in the treatment of metastatic endometrial cancer.

BACKGROUND: Systemic treatment of endometrial carcinoma with distant metastases is currently performed, inter alia, with anthracyclines, platinum, paclitaxel, if osfamid or progestins. This is the first report presenting experience in treatment of metastatic endometrial carcinoma with docetaxel. CASE REPORT: A 69-year-old women with adenocarcinoma of the endometrium was treated with primary combined radiotherapy. Two years later disseminated bilateral pulmonary metastases were detected and the patient was submitted to chemotherapy with epirubicin. After three cycles of chemotherapy with epirubicin examinations revealed metastatic progression. Thus, chemotherapy was changed to docetaxel. RESULTS: After three cycles of chemotherapy with docetaxel examinations revealed remission of the described pulmonary metastases more than 50%. A further three cycles of chemotherapy with docetaxel lead to continuing shrinkage of the detectable metastases to less than 25% of the original size. Because of various side effects, like increasing fatigue and asthenia, uncomfortable acral paresthesia and allergic skin reactions, the patient refused to continue chemotherapy. CONCLUSION: We conclude that docetaxel may be an active agent in patients with metastatic endometrial cancer, but care should be taken to minimize side-effects.

Adenocarcinoma↗

Telecardiology community: a new approach to take care of cardiac patients. "Boario Home-Care" Investigators.

BACKGROUND: Cardiovascular diseases are the leading cause of morbidity and mortality in Western countries and represent, in terms of diagnostic and treatment measures, a large amount of health care expenses. A telecardiology service may offer to general practitioners, in real time, a useful diagnostic tool and the possibility of an accurate screening of patients with suspected ischemic heart disease. METHODS: From February to July 1998, in the provinces of Bergamo and Brescia (Italy), 178 general practitioners received a portable Card Guard 7100 electrocardiographer transferring, by a mobile or fixed telephone, a 12 lead ECG to a receiving station, where a cardiologist was available for the reporting and interactive teleconsultation. RESULTS: During the first 6 months a total 2800 calls took place. Due to incompleteness of requested data only 2254 traces, corresponding to 2254 subjects (mean age 63 +/- 18 years) were entered in the study. In 27% of patients (n = 609) there was a history of systemic hypertension, in 12.5% (n = 283) there was a history of coronary artery disease, and in 38% (n = 834) there was no history of cardiovascular diseases. At the time of ECG recording, 42% of patients (n = 949) were symptomatic: more common symptoms were chest pain (39%), dyspnea (23%), palpitation (19%), dizziness or faint (10%), and asthenia (9%). No action was suggested by cardiologists in 74% of cases. Of the remaining patients, 16% (n = 92) were referred to the Emergency Department, 27% (n = 158) were offered further diagnostic tests as an out patient, and 56% (n = 328) were started on medication or had their treatment changed. CONCLUSIONS: A telecardiology system provides a useful support to general practitioners in the management, in real time, of patients with cardiovascular conditions, and possibly contributes to optimization of health care costs in terms of appropriateness of hospital admissions and diagnostic tests.

Aged↗