Hemoptysis and a cardiac murmur: is it primary or secondary antiphospholipid syndrome?

Endocarditis is most frequently infective in origin, and thus, when a patient presents with a clinical picture suggestive of endocarditis, an extensive work up aimed at finding the infectious agent is warranted. Among systemic lupus erythematosus (SLE) patients, cardiovascular disease is prevalent in more than 50% of patients including valvular disease and non-infective endocarditis, known as Libman-Sacks (LS) endocarditis. The prevalence of LS syndrome among SLE patients with secondary antiphospholipid syndrome (APS) is higher than in SLE without APS. Here, we present a case of a patient diagnosed with primary APS who presented with hemoptysis and a cardiac murmur. The diagnosis of SLE was established following the findings of non-infective verrucous vegetations together with diffuse alveolar hemorrhage (DAH). Treatment with high-dose corticosteroids and intravenous immunoglobulins yielded substantial resolution of the vegetations and regression of the DAH. Hence, aortic valve replacement was successfully performed as an elective procedure and without any postoperative complications. The patient is in remission after a 6-month follow-up. The clinical findings of DAH and double valve non-infectious endocarditis prompted the diagnosis of SLE with secondary APS.

Lobar distribution in non-cystic fibrosis bronchiectasis predicts bacteriologic pathogen treatment.

Non-cystic fibrosis bronchiectasis (NCFBr) is a major cause of morbidity due to frequent infectious exacerbations. We analyzed the influence of patient age and bronchiectasis location on the bacterial profile of patients with NCFBr. This retrospective cohort study included 339 subjects diagnosed with an infectious exacerbation of NCFBr during the 9-year period between January 2006 and December 2014. Bronchoalveolar lavage (BAL) cultures and high-resolution computed tomography scans (HRCT) were utilized to characterize the location of the bronchiectasis and bacteriologic pathogenic profile. In univariate logistic regression, the frequency of Haemophilus influenzae was higher in patients aged ≤64 years (OR = 0.969, p < 0.0001, 95 % CI 0.954-0.983), whereas the frequency of Pseudomonas aeruginosa (OR = 1.027, p = 0.008, 95 % CI 1.007-1.048) and Enterobacteriaceae (OR = 1.039, p = 0.01, 95 % CI 1.009-1.069) were significantly higher in patients aged >64 years. The lobar distribution of bronchiectasis in the subjects was 25.9 % in the right middle lobe (RML), 20.7 % in the right lower lobe (RLL), 20.4 % in the left lower lobe (LLL), 13.8 % in the lingula, 13 % in the right upper lobe (RUL), and 6.2 % in the left upper lobe (LUL). In the lower lobes, H. influenzae was the dominant species isolated, whereas in the RUL it was P. aeruginosa and in the LUL it was non- tuberculous mycobacterium (NTM). H. influenzae was more prevalent in younger patients, whereas P. aeruginosa, Enterobacteriaceae and NTM predominated in older patients. Different pathogens were associated with different lobar distributions. The RML, RLL and LLL showed a greater tendency to develop bronchiectasis than other lobes.

Bronchial anthracofibrosis: treatment using airway stents.

Bronchial anthracofibrosis is a rare disease characterised by chronic bronchial inflammation, with bronchoscopic findings showing dark anthracotic pigmentation on the bronchial mucosa in conjunction with bronchial narrowing or obliteration which causes stenosis of the airways. We describe our experience in two patients with severe bronchial anthracofibrosis and discuss a novel treatment of the stenotic bronchi using endobronchial stents.

Impact of PCR-based diagnosis of invasive pulmonary aspergillosis on clinical outcome.

The mortality rate of 60-90% in invasive pulmonary aspergillosis (IPA) is partly explained by diagnostic delay due to the limitation of current diagnostic tests. We assessed the influence of Aspergillus species (ASP) DNA detection by PCR from bronchoalveolar lavage (BAL) fluid, a new tool for diagnosing IPA, on the outcome of this disease in immune-compromised patients. The study population comprised 107 consecutive patients with hematological malignancies from a single medical center with IPA diagnosed between 1998 and 2005. Clinical variables and mortality rates were compared between two groups diagnosed according to traditional criteria without and with PCR-based ASP DNA detection in BAL fluid. The overall mortality rate during the study period was 38.3%. The addition of PCR to the diagnostic criteria shifted 31 patients from possible to probable IPA. Patients diagnosed with probable IPA according to traditional microbiological methods had significantly higher mortality rates compared to their counterparts who had in addition a PCR-based diagnosis (80 vs 35.6%, P=0.003). This study demonstrates that PCR-based ASP DNA detection for a diagnosis of IPA from BAL fluid has a significant effect on the outcome of patients with IPA, probably related to earlier diagnosis.

Somatostatin therapy ameliorates chronic and refractory gastrointestinal bleeding caused by diffuse angiodysplasia in a patient on anticoagulation therapy.

We present a patient receiving chronic anticoagulant treatment with recurrent and intractable gastrointestinal bleeding due to diffuse angiodysplasia. Following failure of previous medical and surgical treatment, and in light of the patient's need for chronic anticoagulation due to mechanical heart valve, she was treated with somatostatin analogue, octreotide s.c. 100 microg on alternate days for 28 months. Treatment significantly decreased the occurrence of bleeding episodes, the need for hospitalization and blood transfusion requirements despite continued anticoagulant therapy. Octreotide treatment should be considered in patients with refractory gastrointestinal bleeding due to angiodysplasia in particular in those who need anticoagulant treatment.

A repetitive DNA sequence that characterizes human papillomavirus integration site into the human genome is present in vulvar vestibulitis.

OBJECTIVE: To determine the DNA sequence of polymerase chain reaction (PCR) products obtained from surgical specimens of patients with severe vulvar vestibulitis, in order to identify and type the human papillomavirus (HPV)-DNA associated with vulvar vestibulitis. STUDY DESIGN: Fifty three women, referred for dyspareunia and diagnosed as having severe vestibulitis, underwent perineoplasty operation consisting of surgical removal of the sensitive vestibule. PCR analysis using L1 HPV primer was performed, and DNA sequencing of the samples that were found to contain HPV-DNA was undertaken, using the dideoxy chain termination method. RESULTS: Using PCR, HPV-DNA was detected in 31 of 53 tissue specimens (58%). DNA sequencing of 12 HPV-positive PCR products revealed extensive homology to human Alu consensus sequence, albeit not to any known HPV sequence. CONCLUSIONS: The presence of interspersed, repetitive-DNA sequence Alu, which is known to be the preferred site for HPV integration into human genome, in the PCR product reinforces previous observations, suggesting that HPV may have a role in the pathogenesis of vulvar vestibulitis. It further implies a possible integration of the HPV into human DNA in these cases.

Uterine curettage following second trimester abortion by extraovular instillation of prostaglandin E2. A prospective-randomized trial.

OBJECTIVE: To evaluate the benefits associated with routine uterine curettage following complete second trimester termination of pregnancy by extraovular prostaglandin E2. STUDY DESIGN: Fifty-five patients between 15 and 24 weeks' gestation who had undergone complete termination of pregnancy by continuous extraovular instillation of prostaglandin E2 (PGE2), were randomly assigned into either no further intervention (n=25), or uterine curettage under general anesthesia (n=30). The need for late uterine curettage, clinical and ultrasonographic parameters at 1 and 42 days follow-up, as well as the incidence of the minor and major complications, were compared between groups. RESULTS: Baseline and post-abortion clinical and ultrasonographic characteristics were similar in both groups. Mean (+/- Standard error of the mean) number of post-abortion bleeding days in the curettage group was 8.9+/-1.8 versus 10.1+/-2.6 days in the non-curettage group (P=NS). No patient in the former group, compared to three patients in the latter group, needed late uterine curettage, (P=NS). Major and minor complications rates in the curettage and in the no-curettage groups were not significantly different. Considerably more patients in the curettage group needed analgesic agents following the abortion compared to the no-curettage group (60% vs. 3.3%, respectively; P<0.001). CONCLUSIONS: Routine uterine curettage in patients undergoing complete second trimester termination of pregnancy by extraovular instillation of PGE2, exerts no benefit.

Cessation of gonadotropin-releasing hormone analogue (GnRH-a) upon down-regulation versus conventional long GnRH-a protocol in poor responders undergoing in vitro fertilization.

OBJECTIVE: To determine whether a controlled ovarian hyperstimulation (COH) regimen that involves GnRH agonist (GnRH-a) discontinuation before administration of gonadotropins would benefit poor responders. DESIGN: A prospective, randomized controlled trial. SETTING: Hospital-based IVF Unit. PATIENT(S): Sixty-three patients with previous poor response to COH and/or high basal FSH level (> or =9 mIU/mL) undergoing 78 IVF-ET cycles. INTERVENTION(S): In both groups, administration of GnRH-a was started in the midluteal phase. Whereas in the study group (40 cycles), it ended before administration of gonadotropins, in controls (38 cycles) GnRH-a treatment was continued throughout the follicular phase. MAIN OUTCOME MEASURE(S): Ovarian stimulation patterns and IVF outcome. RESULT(S): A significantly higher cancellation rate was noted in the study group than in the controls (22.5% versus 5%, respectively). The new and control regimens resulted in similar stimulation characteristics and clinical pregnancy rates (11% versus 10.3%, respectively). In 13 patients with a basal FSH level that was not persistently high, the new regimen resulted in a significantly higher number of retrieved oocytes compared with the standard protocol (7.6+/-1.03 versus 4.0+/-0.68, respectively). CONCLUSION(S): Whereas for most low responders, the new COH regimen offers no further advantage, future prospective studies may demonstrate whether it can confer a benefit for a subset of patients with a basal FSH level that is not persistently high.

Polymorphism for mutation of cytosine to thymine at location 677 in the methylenetetrahydrofolate reductase gene is associated with recurrent early fetal loss.

OBJECTIVE: This study was undertaken to determine whether a cytosine to thymine mutation at nucleotide 677 in the gene encoding for methylenetetrahydrofolate reductase is associated with particular subtypes of recurrent unexplained spontaneous abortion. STUDY DESIGN: The prevalences of cytosine to thymine mutation at nucleotide 677 in the gene encoding for methylenetetrahydrofolate reductase among 41 patients with recurrent unexplained spontaneous abortions and among 18 healthy control subjects were determined with polymerase chain reaction. RESULTS: Homozygosity and heterozygosity for the cytosine to thymine mutation at nucleotide 677 in the gene encoding for methylenetetrahydrofolate reductase were observed at nonsignificantly different rates among patients and control subjects (9% and 48% versus 22% and 38%, respectively, P <.95). Among patients with recurrent unexplained spontaneous abortions both homozygosity and heterozygosity were associated with significantly increased prevalence of recurrent early fetal loss rather than with repeated anembryonic gestations (P <.0001). CONCLUSION: The observation that polymorphism for the cytosine to thymine mutation at nucleotide 677 in the gene encoding for methylenetetrahydrofolate reductase is associated with repeated early fetal losses rather than with anembryonic gestations strengthens the argument for the role of hypercoagulability and abnormal uteroplacental vasculature in recurrent spontaneous abortion.

Immediate versus delayed treatment of perimenopausal bleeding due to benign causes by balloon thermal ablation.

STUDY OBJECTIVE: To compare the effectiveness and safety of thermal balloon ablation without pretreatment with endometrium-thinning agents compared with delayed ablation with pretreatment for women with perimenopausal menorrhagia. DESIGN: Prospective, randomized, controlled trial (Canadian Task Force classification I). SETTING: Hospital-based ambulatory medical center. PATIENTS: Thirty women age 46 to 51 years with severe enough perimenopausal menorrhagia to make them candidates for either hysterectomy or endometrial ablation. Two patients with submucosal myomas and six who had undergone cesarean section were included. INTERVENTIONS: Thirteen patients were randomly assigned to be treated within 30 days and received a single intramuscular administration of gonadotropin releasing hormone (GnRH) analog; 17 women were allocated to be treated within 3 days of enrollment without uterine preparation. A thermal balloon was inserted transcervically under general anesthesia, and after inflation in the endometrial cavity with 5% dextrose in water, was heated to 87 degrees C for 8 minutes. MEASUREMENTS AND MAIN RESULTS: Immediate and long-term major and minor complications and success rates were analyzed. Bleeding patterns and mean duration of menstrual flow were compared between groups at 6-month follow-up. No major intraoperative or postoperative complications occurred in either group, including the women who had recently undergone hysteroscopic myomectomy or had a history of cesarean section. Minor side effects were similar in both groups, and did not exceed 5%. Overall, at 6-month follow-up, 7 women were amenorrheic, 20 hypomenorrheic, and 3 eumenorrheic. No significant differences were noted between women treated with immediate or delayed ablation in either the distribution of bleeding patterns or days of flow per cycle (mean +/- SEM 1.8 +/- 0.42 vs 2.1 +/- 0.75 days, respectively). CONCLUSION: This pilot study suggests that prompt treatment of perimenopausal menorrhagia with thermal balloon endometrial ablation is as effective and safe as deferred therapy combined with GnRH analog as an endometrium-thinning agent. In light of our results, the theory that previous cesarean section and presence of small submucosal myomas constitute relative contraindications for the procedure merits further consideration. (J Am Assoc Gynecol Laparosc 6(2):145-150, 1999)

Second-trimester membrane rupture. Abortion induced with prostaglandin E2 after oxytocin failure.

OBJECTIVE: To test an effective method of terminating second-trimester pregnancy with ruptured membranes in women who fail to abort from an oxytocin infusion. STUDY DESIGN: Five patients with rupture of membranes during the second trimester of pregnancy and failed to abort using the traditional method of intravenous oxytocin infusion were treated with intrauterine instillation of prostaglandin E2 (PGE2) solution through a double-balloon device. RESULTS: All five patients aborted within 8.8 +/- 4.5 hours from the beginning of PGE2 instillation. No major complications occurred. The only side effect was short-duration pyrexia (less than 48 hours). CONCLUSION: Use of the double-balloon device and intrauterine instillation of PGE2 was effective for termination of pregnancy in patients with rupture of membranes who do not respond to oxytocin.