Hypermobility syndrome. When too much activity causes pain

Hypermobility of joints and spine is a consequence of abnormal laxity of ligaments, joint capsules and intervertebral discs. Hypermobility per se is a state, not a disease. However, it may lead to generalized arthralgia or localized symptoms (frequent ankle sprains, knee effusions, dislocations of the shoulders of recurrent episodes of back pain). These events may occur even after minor strains. Young women are preferentially affected. The term "hypermobility syndrome" is defined as a combination of hypermobility with arthralgia, low back pain, frequent dislocation or sprains. It is often the result of a hereditary disorder of connective tissue. Individuals with hypermobility are at increased risk for premature osteoarthrosis or polytopic disc degeneration of the spine. However, the vast majority of hyperlax individuals are free of symptoms. Those with symptoms who do not develop osteoarthrosis or disc degeneration usually experience spontaneous improvement with increasing age, thus losing their juvenile hyperlaxity. This usually occurs between the ages of thirty and forty. The present review article also provides guidelines for management and patient information.     

Emergency nurses'' perceptions of critical incidents and stress debriefing

The efficacy of examining antinuclear antibody (ANA) was investigated as a screening test detecting subclinical immune disorders in infertility and sterility. ANA was measured in 116 unexplained infertile or sterile patients. The ANA positive rate was 43.5% in group A (habitual abortion, n = 23), 38.1% in group B (consecutive miscarriages, n = 21), 30.0% in group C (one miscarriage, n = 10), 16.7% in group D (one or more deliveries n = 12) 22.0% in group E (primary sterility, n = 50), and 22.4% in the control group (n = 54). The positive rate for all the infertile patients (group A+B) was 40.9% and significantly higher than that in for the control group (p < 0.05). Ten patients with positive ANA had 12 deliveries and 20 patients with negative ANA had 23 deliveries. The frequency of preeclampsia in the patients with positive ANA was higher than in those with negative ANA (41.7% versus 4.3%, p < 0.05). A higher incidence of premature deliveries was recognized in the patients with positive ANA than in those with negative ANA (41.7% versus 8.7%, p < 0.1). Average birth weight (> 35 week) for patients with and without positive ANA was 2,976g (n = 8) and 3,122g (n = 19) respectively. The former tends to be smaller than the latter (p < 0.1).     

Gastrointestinal tract symptoms and self-reported abuse: a population-based study

BACKGROUND/AIMS: A link between abuse and irritable bowel syndrome (IBS) has been reported in outpatients but remains controversial. No population-based studies have investigated this issue. The aim of this study was to determine the prevalence of abuse and its association with symptoms in a representative community sample. METHODS: An age- and sex-stratified random sample of residents of Olmsted County, Minnesota ranging in age from 30 to 49 years was mailed a valid self-report symptom questionnaire. Abuse was assessed by standard published criteria. RESULTS: Of the 919 responders (74%), the age-adjusted prevalence of any abuse was 41% in women and 11% in men, resulting in an age- and sex-adjusted prevalence of 26%. Symptoms of IBS, dyspepsia, and frequent heartburn were reported by 14%, 23%, and 12%, respectively. There was a significant association between IBS and sexual abuse, emotional or verbal abuse, and abuse in childhood and adulthood. Similarly, dyspepsia and heartburn were both significantly associated with abuse. In the population, 31% had visited a physician for gastrointestinal symptoms; the odds of visiting a physician were highest in those reporting abuse in adulthood and childhood. CONCLUSIONS: Self-reported abuse is common in middle-aged subjects; those who report abuse are more likely to have symptoms consistent with IBS, dyspepsia, or heartburn and to visit a physician for bowel symptoms.     

Absorption of isomeric, palmitic acid-containing triacylglycerols resembling human milk fat in the adult rat

The effect of the positional distribution of palmitic acid (16:0) in triacylglycerols (TAG) on 16:0 apparent absorption in adult rats was investigated. The rats were fed two diets which contained 30 energy % as fat with identical total fatty acid compositions, both containing 30% 16:0. The Betapol diet contained TAG with 73% of total 16:0 in the sn-2 position, the control diet contained TAG with 6% of total 16:0 in the sn-2 position. After six weeks on these diets, the rats were killed two or six hours after the last meal, and the small intestine was removed, cut into 10-cm segments, and the fatty acid composition of the segment's contents was determined. At both time points the amount of 16:0 in the intestinal segments starting at 40 cm from the stomach was much lower in the animals fed Betapol than in the animals fed the control diet. Overall absorption of 16:0 and stearic acid was significantly greater in the Betapol group. Absorption of oleic and linoleic acid from the small intestine was similar in both groups, although the overall absorption was significantly greater in the animals fed Betapol. Total fat absorption was significantly higher in the Betapol-fed rats than in the control-fed rats. No effect on calcium and nitrogen absorption, on plasma total cholesterol and TAG levels, and on bodyweights (growth) was seen. The data demonstrate that the positional distribution of the fatty acids in the TAG molecule affects the site of absorption in the small intestine and particularly the net absorption of saturated fatty acids.     

Factors related to rehospitalization within thirty days of discharge after coronary artery bypass grafting

BACKGROUND: Early rehospitalization after coronary artery bypass grafting (CABG) is an expensive and frequently adverse outcome. Rehospitalization rates after various surgical procedures have been used as an indicator of quality of care. Determining the extent to which rehospitalization rates reflect patient case mix and severity of illness rather than quality of care requires detailed information regarding the patients, the care they received, and the reasons for their rehospitalization. METHODS: We conducted a nested case control study comparing 110 CABG patients who were rehospitalized within 30 days after discharge with 224 control patients. Control patients were randomly selected from patients undergoing CABG during the same time frame as the cases and were matched on age, gender, and priority of surgery. A detailed chart review provided information regarding treatment in the postsurgical period, in addition to the preoperative information collected on all CABG patients as part of an ongoing regional prospective study. RESULTS: The overall rehospitalization rate was 13.8%. The most common reasons for rehospitalization included: wound infection (19%), atrial fibrillation (13%), pleural effusion (11%), and thromboembolic event (10%). Preoperative severity of illness and comorbidity accounted for 24% of the total variance. After adjustment for these factors, discharge hematocrit less than 30% (OR = 2.01, p = 0.018) and several discharge medications including: antiarrhythmics (OR = 3.26, p = 0.047), diuretics (OR = 2.18, p = 0.055), beta blockers (OR = 0.44, p = 0.036), and long length of stay (more than 7 days; OR = 2.09, p = 0.029) were the most important predictors of rehospitalization risk. CONCLUSIONS: Although the reasons for rehospitalization after CABG are heterogeneous and related to patient severity of illness as well as comorbid status, several of the most common are potentially preventable and related to quality of care. Rehospitalization was not related to early discharge.     

Daily subcutaneous injection of low-dose interleukin 2 expands natural killer cells in vivo without significant toxicity

We aimed to determine the toxicity and immunological effects of daily s.c. administered low-dose interleukin (IL) 2. Adult cancer patients received a single daily s.c. injection of IL-2 as outpatients for 90 consecutive days. Cohorts of four to nine patients were treated at escalating IL-2 dose levels until the maximum tolerated dose (MTD) was defined. Peripheral blood mononuclear cell phenotyping, IL-2 serum levels, and the presence of anti-IL-2 antibodies were investigated. Thirty-eight patients were treated at seven IL-2 dose levels ranging from 0.4 to 1.75 million International Units (mIU)/m2 daily. The MTD was 1.25 mIU/m2, with constitutional side effects, vomiting, and hyperglycemia dose limiting. Severe toxicity did not occur at or below the MTD, although mild local skin reaction and mild constitutional side effects were common. Objective tumor regressions were not observed during this Phase I trial. Low-dose IL-2 resulted in natural killer (NK) cell (CD3(-) CD56(+)) expansion at all dose levels. This effect was dose dependent (P < 0.01), ranging from a 154 to 530% increase over baseline. Peak NK levels were achieved at 6-8 weeks and sustained through 12 weeks of therapy. As predicted by in vitro studies of IL-2 receptor structure-activity relationships, the subset of NK cells that constitutively express high-affinity IL-2 receptors (CD3(-)CD56(bright+)) showed more profound dose-dependent expansion, with increases ranging from 368 to 2763% (P = 0.015). NK expansion occurred at peak IL-2 levels <10 pM (2.3 IU/ml). Three patients developed nonneutralizing anti-IL-2 antibodies. Thus, we concluded that selective expansion of NK cells may be achieved in vivo with daily s.c. injections of low-dose IL-2 with minimal toxicity.     

Effect of allowing for ethnic group in prenatal screening for Down's syndrome

We conducted a study to investigate ethnic group differences in levels of serum markers used in screening for Down's syndrome [serum alpha-fetoprotein (AFP), unconjugated oestriol (uE3), total human chorionic gonadotrophin (hCG), free alpha- and free beta-hCG, and dimeric inhibin-A], to estimate the extent to which maternal weight differences between ethnic groups explain these differences, and to estimate the effect of adjusting for ethnic group and maternal weight on screening performance. Serum measurements were taken from women who were screened prenatally for Down's syndrome. AFP, uE3, and hCG concentrations were available from 9462 white, 4215 black, and 4392 South Asian women with singleton pregnancies without Down's syndrome or neural tube defects between 15 and 22 weeks' gestational age. Frozen serum samples were available from a subset of 922 white, 449 black, and 135 South Asian women and were used for measurement of free alpha-hCG, free beta-hCG, and inhibin. Values were expressed as multiples of the median (MOM) for women of the same gestational age. There were statistically significant differences in the serum marker levels between ethnic groups that were not explained by differences in maternal weight. The main differences were found in black women compared with white women; black women had serum AFP levels 22 per cent higher (95 per cent confidence interval 20-24 per cent), total hCG levels 19 per cent higher (16-22 per cent), and free beta-hCG levels 12 per cent (3-21 per cent) higher. The other differences were less than 10 per cent. Adjusting for ethnic group only had a small estimated effect on screening performance: a maximum of about 0.5 per cent extra detection at a 5 per cent false-positive rate. At a fixed risk cut-off level, the false-positive rate will not be materially different between different ethnic groups. Adjusting serum markers for ethnic groups improves Down's syndrome screening performance to a very small extent. It is worthwhile because of its established value in AFP screening for open neural tube defects.     

Approach to the patient with pulmonary disease

The approach to the HIV-infected patient with pulmonary disease is summarized by the algorithms in Figures 3 and 4. These are not intended to be followed in a rigid step-wise fashion. Rather, the practitioner's knowledge of the patient with his or her accompanying medical risks influences the path taken, including the depth and the speed of the evaluation. For example, the patient with cough who is afebrile and breathing at 18 breaths a minute, with a normal chest radiograph and a CD4 count of 350 cells/mm3, is reasonably treated with a macrolide or cephalosporin for bacterial bronchitis and clinical follow-up while awaiting cultures (see Fig. 4). A febrile patient with a cough productive of thin mucus, but known to have a CD4 count of 60 cells/mm3 should be started on anti-PCP therapy while being evaluated for PCP with an induced sputum and if nondiagnostic, a bronchoscope despite a normal chest radiograph. Screening can be as simple as placing an oximeter on the patient's finger in the clinic. If the oxygen saturation of a patient with a normal chest radiograph is low, then the patient should be hospitalized and begun on treatment for PCP while diagnostic evaluation is initiated. If the oxygen saturation is normal, the patient can be exercised to elicit desaturation. If there is no desaturation, PCP is unlikely. If the results are equivocal (i.e., a decrease in saturation, but less than 3%), rest and exercise arterial blood gases can be performed, along with a Dlco-Gallium scanning can be done in patients known to have abnormal Dlco or those who cannot exercise. Patients with focal infiltrates who have acute onset of symptoms (see Fig. 4) commonly have bacterial infections, but the possibility of PCP or TB should not be dismissed. Induced sputum should be examined if TB or PCP is suspected. Patients who are severely ill might go quickly to bronchoscopy without awaiting improvement on empiric therapy. The patient with diffuse infiltrates (see Fig. 4) needs no screening because the presence of disease is apparent from the radiograph. The diagnostic part quickly leads to bronchoscopy for these patients and the initiation of therapy for PCP when suspected. In patients with known pulmonary KS, gallium scanning can be helpful to rule out acute infection, but bronchoscopy is warranted if the patient is severely ill, or at high risk for PCP. This approach should avoid unnecessary procedures in patients with simple bacterial infections, without missing opportunistic infections and tumors.     

Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition

BACKGROUND: The molecular basis of protein-losing enteropathy is unknown. However it has been shown that sulphated glycosaminoglycans may be important in regulating vascular and renal albumin loss. METHODS: We describe three baby boys who presented within the first weeks of life with massive enteric protein loss, secretory diarrhoea, and intolerance of enteral feeds. All required total parenteral nutrition and repeated albumin infusions. No cause could be found in any case despite extensive investigations, including small intestinal biopsy sampling, which were repeatedly normal. FINDINGS: By specific histochemistry, we detected gross abnormality in the distribution of small intestinal glycosaminoglycans in all three infants, with complete absence of enterocyte heparan sulphate. The distribution of vascular and lamina propria glycosaminoglycans was, however, normal. INTERPRETATION: The presentation of these infants suggests that enterocyte heparan sulphate is important in normal small intestinal function.