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Improvement of impaired counterregulatory hormone response and symptom perception by short-term avoidance of hypoglycemia in IDDM.

OBJECTIVE: To test the hypothesis that impaired counterregulatory hormone response and symptom perception, induced by recurrent hypoglycemic episodes over 2 days, may be improved by short-term (2-day) avoidance of hypoglycemia. RESEARCH AND DESIGN: We examined two groups of insulin-dependent diabetes mellitus (IDDM) patients (n = 16), none of whom exhibited signs of peripheral or autonomic neuropathy. Two sequential euglycemic-hypoglycemic clamp studies were performed applying stable glycemic plateaus of 5.6, 3.3, 2.2, and 1.7 mmol/l, at which the patients' awareness of and responses to hypoglycemia were evaluated. In the intervention group (n = 11), three short-term hypoglycemic ( < 2.2 mmol/l) episodes (days 1-3) preceded the first clamp study (day 4), whereas the second clamp study (day 6) followed a 2-day interval of strict avoidance of hypoglycemia. A control group (n = 5) was introduced to detect adaptation effects caused by the study procedure per se. RESULTS: This short-term avoidance of hypoglycemia caused improvement of the impaired counterregulatory hormone response during insulin-induced hypoglycemia involving adrenaline (P < 0.05), adrenocorticotrophic hormone (P < 0.03), and cortisol (P < 0.05). Improvement of hypoglycemia symptom awareness encompassed overall symptom perception (multiple analysis of variance, P < 0.04) and the automatic symptoms of heart pounding (P < 0.05) and sweating (P < 0.05). CONCLUSIONS: The previously reported compromised neuroendocrine counterregulation and symptom awareness, occurring as a consequence of repetitive hypoglycemic episodes over 2 days, may be improved by a single 2-day interval of strict avoidance of hypoglycemia

Adult↗

Hypohidrotic ectodermal dysplasia: dental features and carriers detection.

Ectodermal dysplasia is a heterogeneous condition characterized by affected ectodermal structures, among which the teeth and skin with its derivatives (hair sweat glands) are the most frequent. The aim of this work is to present the analysis of dental traits in five families (affected boys and their mothers) with hypohidrotic ectodermal dysplasia (HED), and to evaluate the importance of orofacial and dental findings in the determination of female HED gene carriers. Hypodontia (oligodontia) was found in all the patients. The mothers, gene carriers, had either hypodontia or a reduced size of teeth with a particular morphological, peg shape. In patients with hypohidrotic ectodermal dysplasia the deciduous second molar teeth were mostly affected by taurodontism. The characteristic dental finding in heterozygous females of the mandibular peg shaped incisors and canines, as well as of hypodontia or peg shaped upper lateral incisors can be used as a reliable criterion for the detection of HED gene carriers.

Adult↗

[The results of patients follow up at Gaziantep Number II Dispensary of Tuberculosis Control in the year 2004].

In this study, the aim was to investigate the performance of number-II Dispensary of Tuberculous Control (DTC) in Gaziantep in 2004. The patients diagnosed to have tuberculosis between 2004 January 1st and 2004 December 31st were included. The patients registries were evaluated retrospectively. The mean age of the patients was 28.92+/-14.43 years (ranged between 1-68), 70.5% of the cases were male, and 72.5% of the cases were between 15-44 years old. Forty-two (82.3%) of the cases had the diagnosis of lung tuberculosis. 47% of them did not have any health insurance. The symptoms detected in the patients were cough, night sweats, sputum and hemoptysis respectively. There was no symptom registry in ten patients (19.6%). Sputum analysis to detect acid-fast bacilli (AFB) was done in 18 patients (19.6%). AFB was perfomed in 3.9% of the patients following two-months of therapy, and in 1.9% of the patients at the end of the therapy. Diagnostic tuberculous culture was performed in only one patient (1.9%). There was no tuberculous culture performed neither at the second month nor at the end of the therapy course. AFB was found to be positive in direct or concentrated sputum samples in eight (44.4%) of the patients. All the patients were evaluated with radiographies. The eritrocyte sedimentation rate was measured in 33.3%, 9.8%, and 15.6% of the patients before the therapy, at the end of the two months of therapy, and at the end of the therapy, respectively. The diagnosis was established with radiology clinical findings in 34 patients (66.6%). Directly observed therapy was performed in one patient (1.9%). The cure rate among smear positive patients was 12.5%, and the 100% of the patients were completed the therapy. There exists many problems with tuberculosis "warfare" and we believe the solution lies in DOTS.

Adolescent↗

[Cystic fibrosis revealed by dehydration with hypochloronatremic alkalosis in 3 infants and a neonate].

BACKGROUND--Acute dehydration with hypochloronatremic metabolic alkalosis is a classical complication of cystic fibrosis of the pancreas. Its progressive development as a revealing manifestation of the disease is rare as is its appearance in newborns. Case n. 1.--A 13 month-old girl was admitted because of status epilepticus. She was severely dehydrated (20% weight loss) but had normal diuresis. Investigations showed metabolic alkalosis, hypochloronatremia and hypokalemia. All the manifestations disappeared within 5 days with treatment but three sweat tests were abnormal. Case n. 2.--A 7 month-old girl was admitted because she suffered from progressive loss of weight (10%); she was dehydrated and had metabolic alkalosis plus hypochloronatremia, but her diuresis was normal. Two sweat tests were abnormal. Case n. 3.--A 4.5 month-old boy was admitted because he suffered from severe (12%) weight loss. His diuresis was normal despite dehydration; metabolic alkalosis and hypochloronatremia were found. Two subsequent sweat tests were abnormal. Case n. 4.--A 3 day-old girl was admitted suffering from meconium ileus. Two initial mechanism analysis for protein were abnormal. She had a cardiorespiratory arrest on the 13th day of life, when she had lost 14% of her birth weight; investigations showed metabolic alkalosis, hyponatremia (83 mEq/l), hypochloremia (45 mEq/l); kalemia was 5.9 mEq/l. Peritoneal dialysis was needed to correct hydroelectrolytic changes. A sweat test performed on the 26th day of life was also abnormal. Investigations performed during the periods of dehydration, and repeated later, showed transient functional kidney failure. None of the four patients had any respiratory or gastrointestinal clinical manifestations of cystic fibrosis. CONCLUSION--Metabolic alkalosis with hypochloronatremia plus progressive, severe dehydration in infants whose diuresis is paradoxically normal must be followed by examination for cystic fibrosis.

Alkalosis↗

Variant cystic fibrosis phenotypes in the absence of CFTR mutations.

BACKGROUND: Cystic fibrosis is a life-limiting autosomal recessive disorder with a highly variable clinical presentation. The classic form involves characteristic findings in the respiratory tract, gastrointestinal tract, male reproductive tract, and sweat glands and is caused by loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR ) gene. Nonclassic forms of cystic fibrosis have been associated with mutations that reduce but do not eliminate the function of the CFTR protein. We assessed whether alteration in CFTR function is responsible for the entire spectrum of variant cystic fibrosis phenotypes. METHODS: Extensive genetic analysis of the CFTR gene was performed in 74 patients with nonclassic cystic fibrosis who had been referred by 34 medical centers. We evaluated two families that each included a proband without identified mutations and a sibling with nonclassic cystic fibrosis to determine whether there was linkage to the CFTR locus and to measure the extent of CFTR function in the sweat gland and nasal epithelium. RESULTS: Of the 74 patients studied, 29 had two mutations in the CFTR gene, 15 had one mutation, and 30 had no mutations. A final genotype of two mutations was more common among patients who had been referred after screening for common cystic fibrosis-causing mutations identified one mutation than among those who had been referred after screening had identified no such mutations (26 of 34 patients vs. 3 of 40 patients, P<0.001). Comparison of clinical features and sweat chloride concentrations revealed no significant differences among patients with two, one, or no CFTR mutations. Haplotype analysis in the two families revealed no linkage to CFTR. Although each of the affected siblings had elevated sweat chloride concentrations, measurements of cyclic AMP-mediated ion and fluid transport in the sweat gland and nasal epithelium demonstrated the presence of functional CFTR. CONCLUSIONS: Factors other than mutations in the CFTR gene can produce phenotypes clinically indistinguishable from nonclassic cystic fibrosis caused by CFTR dysfunction.

Adolescent↗

Comparisons of American-Chinese and Taiwanese patients' perceptions of dyspnea and helpful nursing actions during the intensive care unit transition from cardiac surgery.

OBJECTIVE: To compare perceptions by American-Chinese and Taiwanese patients who received ventilatory assistance of acute episodes of dyspnea and the related helpful nursing actions in the intensive care unit (ICU) after cardiac surgery. DESIGN: A descriptive qualitative design. SETTING: Two medical hospitals in northern California and Taiwan. PATIENTS: A purposive sample consisting of 30 adult Chinese patients who experienced acute episodes of dyspnea while receiving assistance from a volume-cycled ventilator following cardiac surgery in the ICU from one study site in the United States (n = 10) and one study site in Taiwan (n = 20). OUTCOME MEASURES: The perceptions, natures, helpful nursing actions, and conceptual definition of the acute episode of dyspnea from the patients' perspective. INTERVENTION: Data were gathered through semi-structured interviews and analyzed by qualitative content analysis. RESULTS: All American-Chinese and Taiwanese subjects experienced physical discomforts, including tightness and congestion in the chest, labored breathing, sweating, palpitations, pain, and loss of vitality. Seventy percent of American-Chinese subjects and 90% of Taiwanese subjects reported having a mortal fear of death during and after the acute episode of dyspnea. The nature of the patients' perceptions of dyspnea revealed in this study can be categorized as unpredictability, sequence, intercorrelation, and exhaustion. Data analysis shows that the acute dyspneic process includes the immediate, following, later, and final transitions. Helpful nursing actions were identified as comforting, companionship, allowing self-concentration, and providing information. CONCLUSION: Both American-Chinese and Taiwanese patients' perceptions of acute episodes of dyspnea and helpful nursing actions were identified and compared. Finally, the conceptual definition of patients' perceptions of acute dyspnea and a conceptual framework for this phenomenon were further tentatively developed to depict and delineate this phenomenon.

Asian↗

Classification of symptoms of hypoglycaemia in insulin-treated diabetic patients using factor analysis: relationship to hypoglycaemia unawareness.

The allocation of some symptoms of acute hypoglycaemia to autonomic and neuroglycopenic groups has proved problematical, with possible misinterpretation of studies which depend upon the use of diverse symptom questionnaires. Two hundred and ninety-five randomly selected insulin-treated diabetic patients were asked to report the symptoms which they usually experienced and believed to be caused by hypoglycaemia. Sweating, trembling, inability to concentrate, weakness, hunger and blurred vision were the most frequently reported symptoms. To classify symptoms of hypoglycaemia objectively, Factor Analysis was used to identify related symptoms which grouped together. This resulted in five groups or clusters of symptoms, four of which could be denominated as groups with a presumed common aetiology, and were labelled: 'neuroglycopenic', 'general malaise', 'autonomic', 'motor dysfunction', and 'sensory dysfunction'. The groups of symptoms derived by Factor Analysis were assessed in relation to partial or absent symptomatic awareness of hypoglycaemia based on historical evidence from the 295 insulin-treated diabetic patients. Neuroglycopenic symptoms were reported more commonly by the patients who had reported partial awareness of hypoglycaemia (number of symptoms 2.6 +/- 1.8 (mean +/- SD] than by the patients who had reported normal hypoglycaemia awareness (1.4 +/- 1.5 symptoms) (p less than 0.05). By contrast autonomic symptoms were reported less frequently by the patients who had reported absent hypoglycaemia awareness (1.3 +/- 1.4 symptoms) than by those with normal awareness (2.2 +/- 1.4 symptoms) (p less than 0.05), which was similar to the number of autonomic symptoms reported by the patients who had partial hypoglycaemia awareness (2.1 +/- 1.3 symptoms).

Analysis of Variance↗

Autonomic nervous system responses during sedative infusions of dexmedetomidine.

BACKGROUND: The purpose of this study was to determine the effects of dexmedetomidine on systemic and cardiac autonomic reflex responses during rest and during thermal stress. METHODS: Volunteers received either placebo or low- or high-dose dexmedetomidine (target plasma concentrations 0.3 or 0.6 ng/ml, respectively) infusions in a prospectively randomized, double-blinded crossover study design. After 1 h, baroreflex sensitivity was assessed, and then core body temperature was raised to the sweating threshold and then lowered to the shivering threshold. Plasma catecholamines and blood pressure were measured, and cardiac autonomic responses were assessed by analysis of heart rate variability. RESULTS: Compared with placebo, plasma norepinephrine concentrations, blood pressure, heart rate, and some heart rate variability measures were lower after 1-h infusion of dexmedetomidine, but baroreflex responses did not differ significantly. Dexmedetomidine blunted the systemic and cardiac sympathetic effects of sweating observed during placebo infusion but had no effect on parasympathetic measures. Increases in blood pressure, and systemic catecholamines due to shivering were observed during placebo and dexmedetomidine, but these responses were less with dexmedetomidine. During shivering, dexmedetomidine infusion was associated with higher low-frequency and high-frequency heart rate variability power but lower heart rate compared with the sweating threshold and with the control period, suggesting nonreciprocal cardiac autonomic responses. CONCLUSIONS: Infusion of dexmedetomidine results in compensated reductions in systemic sympathetic tone without changes in baroreflex sensitivity. Dexmedetomidine blunts heart rate and the systemic sympathetic activation due to sweating, but it is less effective in blunting cardiac sympathetic responses to shivering. During dexmedetomidine infusion, cardiac sympathetic and parasympathetic tone may have nonreciprocal changes during shivering.

Adult↗

Cutaneous microdialysis: is it worth the sweat?

Microdialysis is a minimally invasive technique for chronological study of metabolic, biochemical, and pharmacological events in living tissue. In the skin, probes are placed in the dermis or subcutis for research in two main areas, percutaneous penetration and various aspects of inflammation. Advances in technique, and the concept of data generation and analysis are leading to new areas of application.

Animals↗

Baseline water loss and cholinergic sweat stimulation in atopic dermatitis: a gravimetric measurement of local skin water loss.

The sweat gland function in atopic dermatitis (AD) and in respiratory atopy is a matter of controversy. We examined the baseline water loss and local sweating response in non-eczematous back skin of 146 young men: pure AD, AD with rhinitis/asthma, rhinitis/asthma alone, non-atopic dermatosis and non-atopic healthy. All AD subjects were further divided into the subgroups AD dry and AD normal skin. Following injections of saline and a high concentration of methacholine (5 x 10(4) mol/l) into separate sites the moisture losses were collected into closed pads over a period of 40 min. The baseline water loss was significantly increased (P less than 0.001) and median pure sweat loss was significantly decreased (P less than 0.01) in AD compared with nonatopic healthy individuals. These trends were accentuated in AD dry skin. Respiratory symptoms had no appreciable influence on results. A depressed sweating response occurred in 30% of AD subjects and 9% of non-AD subjects. An elevated baseline water loss value and a depressed sweat loss value coexisted in 22% of subjects with AD dry skin compared with 3% of the non-atopics.

Acetylcholine↗

Prognosis of follicular lymphoma: a predictive model based on a retrospective analysis of 987 cases. Intergruppo Italiano Linfomi.

Patients (n-987) with a histologically confirmed diagnosis of follicular lymphoma were studied with the aim of developing a prognostic model specifically devised for this type of lymphoma. We collected information on age, sex, Ann Arbor stage, number of extranodal disease sites, bone marrow (BM) involvement, bulky disease, B symptom criteria (fever, night sweats, and weight loss), performance status (PS), serum lactate dehydrogenase (LDH) level, serum albumin level, hemoglobin level, and erythrocyte sedimentation rate (ESR). In the training sample of 429 patients with complete data, multivariate analysis showed that age, sex, number of extranodal sites, B symptoms, serum LDH level, and ESR were factors predictive for overall survival. Using these 6 variables, a prognostic model was devised to identify 3 groups at different risk. The 5- and 10-year survival rate was 90% and 65% for patients at low risk, respectively; 75% and 54% for patients at intermediate risk; and 38% and 11% for those at high risk (log-rank test, 86.62; P <. 0001). The model was also predictive (P =.0001) in the validation sample of 265 patients with complete data only for the 6 variables used in the development of the model and even in the group of 210 patients from the validation sample uniformly treated with doxorubicin-containing regimens (P =.0001). The prognostic model appears to be very useful in identifying patients with follicular lymphoma at low, intermediate, or high risk.

Adult↗

Comparison of sweat loss estimates for women during prolonged high-intensity running.

PURPOSE: This study evaluated the error produced by four commonly used field estimates and two prediction equations of total body sweat loss. METHODS: Eight women distance runners were studied during a 30-km treadmill run (approximately 70% .VO(2max)) in a warm (30 degrees C T(db)) and a cool (14 degrees C T(db)) environment. Total sweat loss (TSL) was determined from changes in body mass corrected for fluid intake (FI), urine losses (UL), clothing (trapped sweat, TS), CO(2)-O(2) exchange (metabolic mass loss, MML), and respiratory water loss (RWL). TSL was compared with four estimates of sweat losses (often employed in the field) from body mass changes corrected for: a) FI only (F-1); b) FI and TS (F-2); c) FI and UL (F-3); or d) FI, TS, and UL (F-4). Two prediction equations were used also for comparison to TSL values. RESULTS: In the warm environment, F-1, F-3, and F-4 accurately estimated (0.2-6.9%; P > 0.05) TSL, whereas F-2 produced a large error (15.3%; P < 0.05). In the cool environment, all four estimates produced large errors (14-41%; P < 0.05). Both prediction equations markedly underestimated (20-22%) TSL in the warm environment and underestimated (41%) or overestimated (20%) TSL in the cool environment. CONCLUSION: TSL can be accurately estimated from changes in body mass using F-1, F-3, or F-4 methods in hot environments; however, none of the methods accurately estimated actual TSL values in a cool environment. Neither prediction equation provided accurate estimates of TSL in warm or cool conditions for women runners. These results illustrate the difficulty of accurately estimating and predicting sweat losses in the field.

Adult↗

Early increase precedes a depletion of VIP and PGP-9.5 in the skin of insulin-dependent diabetics--correlation between quantitative immunohistochemistry and clinical assessment of peripheral neuropathy.

Diabetic neuropathy affects both sensory and autonomic peripheral nerve fibres. Vasoactive intestinal polypeptide (VIP) is present in autonomic fibres which modulate sweat secretion, while calcitonin gene-related peptide (CGRP) is localized to cutaneous sensory fibres. In this study, immunohistochemistry and image analysis were used to assess changes of VIP and CGRP, and of the pan-neuronal marker protein gene-product (PGP)-9.5, in skin biopsies of 18 patients affected by type 1 diabetes (age range 18-46 years) and from seven aged-matched controls. Patients were divided into three groups: group 1 (n = 6), with diabetes for 6 months to 3 years; group 2 (n = 5), with the disease for 5-10 years; and group 3 (n = 7), with diabetes for more than 10 years. VIP immunoreactivity (IR) and PGP-9.5-IR were significantly reduced around sweat glands (P < 0.005) in groups 2 and 3. Epidermal CGRP-IR and PGP-9.5-IR were significantly reduced in group 3 (P < 0.05). Twenty-eight per cent (5/18) of all patients showed high VIP-IR around sweat glands (> 95 per cent confidence limits of controls) and all of these patients had diabetes for less than 3 years. Conversely, 55 per cent (10/18) of patients had low VIP-IR (< 5 per cent confidence limit of controls). The latter, compared with the former, showed a significantly longer duration of diabetes (Fisher exact test P = 0.002), presence of clinical autonomic neuropathy (Fisher exact test P = 0.04), and a reduced sural nerve conduction velocity (Fisher exact test P = 0.04). These results suggest that quantitative immunohistochemical analysis of peptide-containing cutaneous nerves allows an objective evaluation of nerve fibre alterations at early stages of diabetes than is currently possible with neurophysiological functional tests.

Adolescent↗

Prophylaxis against Frey's syndrome in parotid surgery.

In 1990 the authors reported their preliminary study of the prevention of Frey's syndrome in 55 patients utilizing a superficial musculoaponeurotic system (SMAS) flap in parotid gland surgery. During the past 10 years, numerous studies have supported their original thesis that interposition of living tissue between the resected gland bed and the skin could prevent the development of this complication. The authors have expanded their own patient population and now consider this a definitive study on the prevention of Frey's syndrome. A total of 160 patients are presented with a follow-up period of 5 to 22 years. All patients underwent subtotal or total parotidectomy performed by one of the authors. A history was acquired and testing for Frey's syndrome (Minor's starch iodine test) was performed. As a result of this approach, and in spite of the intensive search for it, no cases of Frey's syndrome were encountered. The hoped-for secondary benefit of preventing the postparotidectomy retromandibular depression was somewhat less satisfactory, although most patients remain satisfied with their appearance. The debilitating symptoms in Frey's syndrome, which is reported to have an incidence of 5% to 50% in the typical parotidectomy patient, can be avoided with thoughtful preoperative planning. The authors favor an aesthetic incision followed by the development of an SMAS flap. The parotidectomy is then performed using the surgeon's preferred technique. The SMAS flap is then placed into the bed of the resected parotid gland. This institutes a protective tissue barrier guarding against the aberrant anastomotic communication between the postganglionic secretomotor fibers intended for the parotid gland, and the now adjacent sweat glands. Their patient population is large enough to provide significant evidence that Frey's syndrome can be prevented, compared with a meta-analysis of parotid patients in multiple other studies in the literature. Assuming the patient's history and pathology does not preclude its use, the SMAS flap should be considered the standard of care for preventing Frey's syndrome in the postparotidectomy patient. If the SMAS flap is not available, a temporoparietal fascial flap has proved to be a good alternative.

Female↗

Inhaled bronchodilators for cystic fibrosis.

BACKGROUND: Recurrent wheeze and breathlessness are common in people with cystic fibrosis, and bronchodilators are commonly prescribed. Despite their wide-scale and often long-term use, there is limited objective evidence about their efficacy in cystic fibrosis. OBJECTIVES: To evaluate the effectiveness of inhaled bronchodilators in children and adults with cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic databases searches, and handsearches of relevant journals and abstract books of conference proceedings. Latest search of the Group's Trials Register: August 2005 SELECTION CRITERIA: Randomised or quasi-randomised trials comparing inhaled bronchodilators to placebo or another inhaled bronchodilator in people with CF, diagnosed clinically and by sweat or genetic testing and at all stages and severity of lung disease. DATA COLLECTION AND ANALYSIS: The authors independently extracted data and assessed trial quality. If data were missing, the primary author was contacted where possible. The data were subgrouped into classes of bronchodilator and for each class into short-term effects (less than one week) and long-term effects (greater or equal to one week). MAIN RESULTS: The search identified 43 references. Fourteen trials, with a total of 257 participants, were suitable for inclusion. The trials were all cross-over in design; in this case a meta-analysis was not possible. There were varied conclusions from the different trials, reflecting their heterogeneity. Compared to placebo, short-acting beta-2 agonists increased forced expiratory volume at one second (FEV(1)) in the short term in three out of five trials, and in the long-term increased peak expiratory flow rate in individuals who had been shown to have bronchial hyperreactivity or bronchodilator responsiveness or both. Compared to placebo, long-acting beta-2 agonists increased FEV(1) and forced expiratory flow between 25% and 75% of expiratory flow (FEF 25-75%) in the short term in participants known to have bronchodilator responsiveness, but produced inconsistent results in long-term trials. Short acting-anticholinergics had no consistent effect on lung function tests in either the short or the long term. We found no published trials of fenoterol, formoterol or tiotropium and the use of these agents in cystic fibrosis cannot be supported. AUTHORS' CONCLUSIONS: It was not possible to determine fully the effectiveness of inhaled bronchodilators in cystic fibrosis as a meta-analysis was not possible. However, both short and long-acting beta-2 agonists can be beneficial both in the short and long term in individuals with demonstrable bronchodilator responsiveness or bronchial hyperrresponsiveness.

Adrenergic beta-Agonists↗