Disease pattern in Danish patients with Peutz-Jeghers syndrome.

PURPOSE: In this paper, we aimed to collect genetic and medical information on all Danish patients with Peutz-Jeghers syndrome (PJS), in order to contribute to the knowledge of phenotype and genotype. Peutz-Jeghers syndrome is a hereditary syndrome characterized by multiple hamartomatous polyps in the GI tract, mucocutaneous pigmentations, and an increased risk of cancer in the GI tract and at extraintestinal sites. Over 90 % of patients harbour a pathogenic mutation in STK11. METHODS: Based on the Danish Pathology Data Bank, the Danish National Patient Register, as well as information from relevant departments at Danish hospitals, we identified patients and collected clinical and genetic information. RESULTS: We identified 43 patients of which 14 were deceased. The prevalence was estimated to be ∼1 in 195,000 individuals. The median age at first symptom was 27.5 with invagination of the small bowel as the most frequent presenting symptom. We noted 18 occurrences of cancer at various anatomical sites, including a case of thyroid cancer and penile cancer. Eight of the deceased patients had died of cancer. Eighteen different mutations in STK11 had been detected in 28 patients. CONCLUSION: This is the first comprehensive study of patients with Peutz-Jeghers syndrome in the Danish population identified from nationwide registers and databases. We have demonstrated that the expressivity of Peutz-Jeghers syndrome varies greatly among the patients, even within the same families, underlining the great phenotypic spectrum. Patients with PJS should be offered surveillance from childhood in order to prevent morbidity and reduce mortality.

Reduced effect of inhaled beta 2-adrenergic agonists on lung mucociliary clearance in patients with cystic fibrosis.

STUDY OBJECTIVE: It has been suggested that the defective cyclic AMP-dependent Cl- secretion and the increased Na+ absorption from airway epithelia in cystic fibrosis (CF) may lead to dehydrated secretions and impaired mucociliary clearance. Beta 2-adrenergic agonists are unable to stimulate the Cl- transport in the airways in CF while being able to do so in normal airways. Thus, we questioned whether the beta 2-adrenergic agonist, terbutaline, would improve mucociliary clearance in patients with CF as it does in healthy subjects. DESIGN: A double-blind, randomized cross-over trial. SETTING: Whole lung and regional mucociliary clearance of a radioaerosol was measured for 3 h by gamma camera. One minute of cough clearance was measured at 3 h. Pulmonary function was measured before and after the gamma camera study. PATIENTS: Ten young patients with CF were studied; three were heterozygous for deletion of the phenylalanine at position 508 (delta F508), and seven were delta F508 homozygous. INTERVENTIONS: Inhalation of 1 mg of terbutaline (4 puffs) and placebo via a metered-dose inhaler by spacer. MEASUREMENTS AND RESULTS: The largest differences in tracheobronchial retention (percent) in the whole lung and regional lung zones were generally observed within 1 h after terbutaline and placebo treatment: whole lung (mean +/- SD), 60 +/- 22 percent and 63 +/- 20 percent; central zone, 41 +/- 19 percent and 50 +/- 25 percent; midzones, 65 +/- 27 percent and 63 +/- 19 percent; and peripheral zones, 77 +/- 28 percent and 72 +/- 28 percent, respectively [p > 0.1]. Whereas none of these differences was statistically significant, subanalyses suggested an improvement in mucociliary clearance by terbutaline in the three delta F508 heterozygous patients but not in the seven delta F508 homozygous patients. One minute of voluntary coughing resulted in clearance of less than 8 percent of the initial radioactivity, with a trend of more peripheral zone clearance after terbutaline compared with placebo. A small significant decrease in airway resistance was detected 3 h after terbutaline. CONCLUSIONS: We conclude that 1 mg of terbutaline did not improve mucociliary clearance significantly in the patients with CF. Also, the effect of terbutaline was much less than demonstrated earlier in healthy subjects. This is consistent with earlier findings of a lack of beta-adrenergic modulation of Cl- transport in CF. Thus, no rehydration of the dry mucus in CF is expected, and a possible beta 2-agonist-induced increase in ciliary beating would have only a minor effect on overall mucociliary clearance.

[Pelvic inflammation after induced abortion]. / Underlivsinflammation efter abortus provocatus.

A total of 949 women referred for legal termination of pregnancy in the first trimester participated in an investigation to assess the risk of pelvic inflammation in connection with the intervention in women with and without previous pelvic inflammation in connection with previous pregnancies, the gestational age and the clinical experience of the surgeon. Women who had not borne children developed pelvic inflammation more frequently after legal termination of pregnancy (p < 0.01) than women who had previously borne children. Women with previous pelvic inflammation developed renewed pelvic inflammation, if they had not previously borne children (p < 0.01) or if they had previously undergone legal termination of pregnancy (p < 0.05). Women without previous pelvic inflammation developed pelvic inflammation significantly more frequently, if they had not previously been pregnant (p < 0.05). The occurrence of pelvic inflammation was independent of the gestational age and the clinical experience of the surgeon.

No effect of single dose ofloxacin on postoperative infection rate after first-trimester abortion. A clinical, controlled trial.

In a double-blind stratificated, placebo controlled study the efficacy and safety of a single oral dose of ofloxanin (Tarivid) (400 mg) in preventing postabortal pelvic inflammatory disease (P-PID) in women with induced first-trimester abortions were assessed. The tablets were given 1 1/2-2 h before the abortion, and the women were followed one month after the operation. Group I consisted of 308 with previous PID. Ofloxacin tablets were given to 149 women and 159 women received placebo tablets. In the ofloxacin group 20 women (13.4%) and in the placebo group 27 women (17%) developed P-PID, a non-significant difference (p = 0.39). Group II consisted of 765 women without a history of PID. Ofloxacin tablets were given to 376 women and 389 women received placebo tablets. Thirty-five women (9.3%) in the ofloxacin group and 46 women (11.9%) in the placebo group developed P-PID, a non-significant difference (p = 0.26). A single oral dose of ofloxacin (400 mg) did not reduce the incidence of P-PID either among women with previous PID or among women without previous PID.

Random blood glucose sampling as an early antenatal screening test for diabetes mellitus.

Random blood glucose was measured on 1,992 pregnant women during the initial attendance at our antenatal clinic. The visit usually took place during the first part of the second trimester. Women with a random blood glucose concentration in excess of 6.1 mmol/l within 2 hours of the last meal and 5.6 mmol/l more than 2 hours after the last meal were referred for a fasting blood glucose measurement. A 75 g oral glucose tolerance test was performed in those cases where the fasting blood glucose exceeded 4.0 mmol/l. The cut-off levels were exceeded in 22 cases, but only one of these women was found to be suffering from previously unsuspected diabetes mellitus, based on the criteria of WHO (1). Five other cases of gestational diabetes were not detected by the random blood glucose method. It is concluded that random blood glucose measurements cannot be recommended as a safe and reliable screening procedure during the early part of the second trimester.

Epidural clonidine enhances postoperative analgesia from a combined low-dose epidural bupivacaine and morphine regimen.

In a randomized, double-blind, placebo-controlled trial, the value of adding clonidine to a low-dose epidural regimen for postoperative pain treatment was assessed. Twenty-four patients scheduled for hysterectomy during combined thoracic epidural (bupivacaine and morphine) and general anesthesia were studied. Postoperative analgesia consisted of epidural bupivacaine (5 mg/h) and morphine (0.1 mg/h) for 12 h. In addition, the patients randomly received clonidine (75 micrograms), followed by an infusion of 18.75 micrograms/h or saline solution (placebo) epidurally. Pain was evaluated at rest, during cough, and during mobilization every hour. Sensory level of analgesia was evaluated by pinprick. We found no significant difference in pain scores at rest between the clonidine and placebo groups but an enhanced analgesic effect by clonidine during cough and mobilization (P less than 0.05). Arterial blood pressure decreased significantly during clonidine infusion and remained lower than in the control group throughout the study. We conclude that a continuous low-dose epidural clonidine infusion enhances analgesia from a combined low-dose epidural bupivacaine and morphine regimen after hysterectomy; however, the concomitant decrease in arterial blood pressure during epidural clonidine deserves further study before such a regimen can be recommended.

Single-dose versus six-day therapy with sulfamethizole for asymptomatic bacteriuria during pregnancy. A prospective randomised study.

Single-dose versus conventional six-day therapy with sulfamethizole for asymptomatic urinary tract infection in pregnant women was investigated in a prospective, randomised open study. During a nine-month period 4,274 pregnant women were screened for significant bacteriuria, which was found in 123 (2.9%), of whom only five had symptoms of urinary tract infection. Seventy-nine of the patients had a second urine culture performed, revealing significant counts of the same bacteria in only 41. These 41 patients were randomly allocated to one of the two treatment groups. Control urine cultures one week and 4-6 weeks after start of treatment revealed the same cure rates of approximately 50% in both groups. Single-dose treatment is not inferior to conventional therapy; future study is needed to find the best single-dose regimen.

A prospective randomized clinical trial comparing an individual dose of recombinant FSH based on predictive factors versus a 'standard' dose of 150 IU/day in 'standard' patients undergoing IVF/ICSI treatment.

BACKGROUND: The study aim was to compare the use of individual rFSH doses between 100 and 250 IU/day (calculated using the rFSH dose normogram) with a standard dose of rFSH of 150 IU/day. METHODS: This prospective randomized dual-centre clinical trial included 267 first IVF/ICSI cycles using the long agonist protocol in 'standard' patients. Following down-regulation, 262 patients were randomized using computer-generated lists using 'clusters of 10' into the individual dose (study) group (n = 131) or the standard dose (control) group (n = 131). RESULTS: In the study group, 101 patients (77.1%) had an appropriate response (defined as 5-14 oocytes), compared with 86 (65.6%) in the control group (P < 0.05). Fewer than five oocytes were retrieved in two patients (1.5%) in the study group, compared with 14 patients (10.7%) in the control group (P < 0.05). By comparison, >14 oocytes were retrieved from 27 patients (20.6%) in the study group and from 26 (19.8%) control patients (P = NS). Eighty-six per cent of the individual dose patients did not require any dose adjustment on day 8, compared with 45% of the standard dose patients (P < 0.01). The ongoing pregnancy rate per initiated cycle was 36.6% in the study group and 24.4% in the control group (P < 0.01). One patient (0.8%) in the study group, and four patients (3.1%) in the control group, were hospitalized due to ovarian hyperstimulation syndrome. CONCLUSIONS: An individual dose regimen in a well-defined 'standard' patient population increased the proportion of appropriate ovarian responses and decreased the need for dose adjustments during controlled ovarian stimulation. A higher ongoing pregnancy rate was observed in the individual dose group.

Does anaesthesia cause postoperative cognitive dysfunction? A randomised study of regional versus general anaesthesia in 438 elderly patients.

BACKGROUND: Postoperative cognitive dysfunction (POCD) is a common complication after cardiac and major non-cardiac surgery with general anaesthesia in the elderly. We hypothesized that the incidence of POCD would be less with regional anaesthesia rather than general. METHODS: We included patients aged over 60 years undergoing major non-cardiac surgery. After giving written informed consent, patients were randomly allocated to general or regional anaesthesia. Cognitive function was assessed using four neuropsychological tests undertaken preoperatively and at 7 days and 3 months postoperatively. POCD was defined as a combined Z score >1.96 or a Z score >1.96 in two or more test parameters. RESULTS: At 7 days, POCD was found in 37/188 patients (19.7%, [14.3-26.1%]) after general anaesthesia and in 22/176 (12.5%, [8.0-18.3%]) after regional anaesthesia, P = 0.06. After 3 months, POCD was present in 25/175 patients (14.3%, [9.5-20.4%]) after general anaesthesia vs. 23/165 (13.9%, [9.0-20.2%]) after regional anaesthesia, P = 0.93. The incidence of POCD after 1 week was significantly greater after general anaesthesia when we excluded patients who did not receive the allocated anaesthetic: 33/156 (21.2%[15.0-28.4%]) vs. 20/158 (12.7%[7.9-18.9%]) (P = 0.04). Mortality was significantly greater after general anaesthesia (4/217 vs. 0/211 (P < 0.05)). CONCLUSION: No significant difference was found in the incidence of cognitive dysfunction 3 months after either general or regional anaesthesia in elderly patients. Thus, there seems to be no causative relationship between general anaesthesia and long-term POCD. Regional anaesthesia may decrease mortality and the incidence of POCD early after surgery.

Cognitive dysfunction after minor surgery in the elderly.

BACKGROUND: Major surgery is frequently associated with postoperative cognitive dysfunction (POCD) in elderly patients. Type of surgery and hospitalization may be important prognostic factors. The aims of the study were to find the incidence and risk factors for POCD in elderly patients undergoing minor surgery. METHODS: We enrolled 372 patients aged greater than 60 years scheduled for minor surgery under general anesthesia. According to local practice, patients were allocated to either in- (199) or out-patient (173) care. Cognitive function was assessed using neuropsychological testing preoperatively and 7 days and 3 months postoperatively. Postoperative cognitive dysfunction was defined using Z-score analysis. RESULTS: At 7 days, the incidence (confidence interval) of POCD in patients undergoing minor surgery was 6.8% (4.3-10.1). At 3 months the incidence of POCD was 6.6% (4.1-10.0). Logistic regression analysis identified the following significant risk factors: age greater than 70 years (odds ratio [OR]: 3.8 [1.7-8.7], P = 0.01) and in- vs. out-patient surgery (OR: 2.8 [1.2-6.3], P = 0.04). CONCLUSIONS: Our finding of less cognitive dysfunction in the first postoperative week in elderly patients undergoing minor surgery on an out-patient basis supports a strategy of avoiding hospitalization of older patients when possible.