Combined life-threatening thromboses and hemorrhages in a patient with afibrinogenemia and antithrombin deficiency.

BACKGROUND: Patients with congenital afibrinogenemia suffer from spontaneous recurrent severe bleeding. While fibrinogen concentrates are known to effectively treat bleeding episodes, thrombotic complications often occur upon replacement therapy, rendering clinical management highly challenging. CASE PRESENTATION: We hereby report a case of combined afibrinogenemia and congenital antithrombin deficiency manifested by recurrent life-threatening bleeding, as well as spontaneous severe arterial occlusion, such as acute coronary syndrome and stroke, and venous thromboses like pulmonary embolism.Secondary fibrinogen prophylaxis is recommended following any initial life-threatening bleeding episode in patients with afibrinogenemia, yet the high associated risk of thrombosis illustrates the complexity of choosing the most effective prophylaxis strategy combining fibrinogen concentrate with antithrombotic agent for optimal protection against the risk of both severe bleeding and thrombosis. For our patient, the thrombin generation assay objectively confirmed her prothrombotic tendency. CONCLUSION: This case may help us better understand the pathophysiology of arterial thrombosis in afibrinogenemia, while highlighting the difficulty of managing such complications.

Chemotherapy-induced differentiation to cure botryoid rhabdomyosarcoma: a case report and review of the literature.

Rhabdomyosarcoma (RMS) occurs rarely in adults and constitutes 2-6% of all uterine neoplasms. The authors report the case of a 26-year-old woman diagnosed with botryoid RMS that presented discordant progression results on follow up imaging and cytodifferentiation on pathologic control. This case showed that radiological evaluation could be misleading as the tumor demonstrated chemotherapy-induced differentiation without volume reduction. This case illustrates the limitations of using the imaging anatomical dimensions of sarcomas for treatment planning and highlights the potential role of functional imaging to assess the response to treatment.

[Ultrasound comparison of adjustable single-incision (Ajust) and transobturator tapes to assess post-operative fixation and function and clinical efficacy]. / Ultrazvuková studie porovnání kvality fixace pásky a lécebného efektu pásky zavádené z jedné incize (Ajust) a transobturatorní pásky TVT-O.

OBJECTIVE: The aim of this study was to compare fixation and mobility of adjustable single-incision (Ajust) and standard transobturator midurethral sling (TVT-O) tapes based on postoperative ultrasound monitoring of the position of the tape at rest and at maximal Valsava (tape descent). The hypothesis was that fixation of SIMS Ajust is comparable to that achieved by the standard transobturator midurethral sling. SETTING: Gynecology and Obstetrics Department, GUH and 1st Charles University Prague; Gynecology and Obstetrics Department Masarykova nemocnice Ústí nad Labem. DESIGN: Randomized trial, secondary analysis of ultrasound follow-up. METHODS: Between May 2010 and May 2012 100 women with proven urodynamic stress urinary incontinence were included in this randomized trial.All patients underwent a complete urogynecological investigation before the procedure (clinical examination, urodynamics, ultrasound examination), and they filled in the ICIQ and iQol questionnaires; after surgery, to evaluate their satisfaction with the procedure, VAS and Likert scales were added.Ultrasound examination as part of each post-operative check-up was performed the first day after surgery, two weeks, three months and one year after surgery. Ultrasound measurements were taken in a supine position at rest and during maximal Valsalva. Position of the bladder neck was assessed, and the position of the tape with respect to upper and lower tape margins. RESULTS: At one-year follow up no differences in subjective cure rates and objective cure rates were observed. In all checks after surgery there was no difference between the position of the tape at rest and at maximal Valsalva between the Ajust and TVT-O groups.The length of the upper and lower tape descent was similar. In TVT-O patients the mean length of upper tape margin descent increased from 8.8 mm on the first day after surgery to 10.0 mm three months after surgery; afterwards it remained stable. For Ajust patients there was virtually the same development - from 9.1 to 12 mm - and there was no further increase in the tape descent following the three-month measurement. CONCLUSIONS: The quality of the tape fixation after the Ajust procedure is as good as after standard transobturator midurethral sling. We did not observe any statistically significant differences in tape position and descent, indicating that the function is similar to obturator tape, which in turn suggests the same clinical efficacy. Ultrasound monitoring should be the part of post-operative monitoring whenever novel surgical techniques are introduced.

[Different outcome in two case reports of births showing symptoms of amniotic fluid embolism in the years 2002 and 2012 in the district hospital of Decin]. / Dve kazuistiky porodu s príznaky embolismu plodovou vodou v letech 2002-2012 s ruznými výsledky v Krajské zdravotní a. s., Nemocnice Decín, o.z.

This paper presents two case reports of amniotic fluid embolism quite identical in the onset of symptoms, 1,20 hr, respectively 1,40 hr after extraction of the fetus during delivery by caesarean section, both births were induced by prostaglandins. Both newborns were male. One patient died with autopsy providing evidence of massive pulmonary embolism, laboratory findings showed hemolysis. The second patient survived with neurological disorders, laboratory findings temporarily showed nonspecific antibodies. Both patients were subdued to hysterectomy, no trace of amniotic fluid components were found in the uterine vessels in either one of them.

Revealing a cancer diagnosis to patients: attitudes of patients, families, friends, nurses, and physicians in Lebanon-results of a cross-sectional study.

BACKGROUND: Disclosure of a cancer diagnosis to patients is a major problem for physicians in Lebanon. Our survey aimed to identify the attitudes of patients, families and friends, nurses, and physicians regarding disclosure of a cancer diagnosis. METHODS: Study participants included 343 physicians, nurses, cancer patients, families, and friends from clinics in two major hospitals in Lebanon. All completed a 29-item questionnaire that assessed, by demographic group, the information provided about cancer, opinions about the disclosure of the diagnosis to cancer patients, perceived consequences to patients, and the roles of family, friends, and religion. RESULTS: Overall, 7.8% of the patients were convinced that cancer is incurable. Nearly 82% preferred to be informed about their diagnosis. Similarly, 83% of physicians were in favour of disclosing a cancer diagnosis to their patients. However, only 14% of the physicians said that they revealed the truth to the patients themselves, with only 9% doing so immediately after confirmation of the diagnosis. Disclosure of a cancer diagnosis was preferred before the start of the treatment by 59% of the patients and immediately after confirmation of the diagnosis by 72% of the physicians. Overall, 86% of physicians, 51% of nurses, and 69% of patients and their families believed that religion helped with the acceptance of a cancer diagnosis. A role for family in accepting the diagnosis was reported by 74% of the patients, 56% of the nurses, and 88% of the physicians. All participants considered that fear was the most difficult feeling (63%) experienced by cancer patients, followed by pain (29%), pity (8%), and death (1%), with no statistically significant difference between the answers given by the participant groups. CONCLUSIONS: The social background in Lebanese society is the main obstacle to revealing the truth to cancer patients. Lebanese patients seem to prefer direct communication of the truth, but families take the opposite approach. Physicians also prefer to communicate the reality of the disease at the time of diagnosis, but in actuality, they instead disclose it progressively during treatment. Faith is helpful for acceptance of the diagnosis, and families play a key role in the support of the patients. An open discussion involving all members of society is necessary to attain a better understanding of this issue and to promote timely disclosure of a cancer diagnosis.

Overview on the role of preoperative therapy in the management of kidney cancer.

The advent of molecular therapy through targeted kinase inhibitors (TKI) has revolutionized the management of renal cell carcinoma. Although surgical resection remains the cornerstone of any therapeutic plan, an increased risk of morbidity and mortality can be of concern in large and complex bulky tumors. Preoperative therapy with TKIs is hypothesized to facilitate resectability, reduce surgical morbidity and allow nephron-sparing surgery. Many concerns on the safety, efficacy and tolerability of these agents before surgery have halted the progress in this setting. In this paper, we will review the indications and safety of preoperative TKIs in RCC as well as the future approaches.

Transcatheter aortic valve implantation: a safe and efficient procedure to treat an aortic valve stenosis before lung cancer resection.

A 75 year old man presented with left upper lobe squamous cell carcinoma. Severe aortic stenosis has been discovered during preoperative assessment. In this situation, two therapeutic strategies are described: concomitant or two-staged procedures. The debate has not yet led to the conclusion on whether preferably performing one or the other. Furthermore, in an oncologic context, using cardiopulmonary bypass is still controversial. So, thoracic and cardiac surgeons jointly proposed to the patient a transcatheter aortic valve implantation followed, 72 h later, by lung cancer resection. This case report describes a way to treat both diseases avoiding cardiopulmonary bypass adverse effects and without delaying lung cancer treatment.

A case of synchronous relapse of breast cancer and uterine müllerian adenosarcoma post tamoxifen in a premenopausal woman.

PURPOSE & METHODS: We report a case of a 42-year-old multigravida, premenopausal woman with breast carcinoma, who presented after four years of use of adjuvant tamoxifen with synchronous liver, bone, and lung metastasis of breast cancer with müllerian adenosarcoma. RESULTS: Immunohistochemical stains on the uterine tumor for estrogen and progesterone receptors showed positivity for both epithelial and stromal cells, actin, and desmin while the proliferative index (MIB-1) showed positivity for stromal cells only. The patient underwent a hysterectomy followed by palliative chemotherapy. She died 14 months after her relapse because of progressive disease (cerebral, bone, liver and lung metastases). CONCLUSION: Our case is the only one reported in the literature with synchronous relapse of breast adenocarcinoma and a Müllerian adenosarcoma. Moreover, it is one of the rare cases occurring in a premenopausal woman since all except two cases were postmenopausal.

Water deprivation decreases strength in fast twitch muscle in contrast to slow twitch muscle in rat.

AIM: The effects of dehydration on muscle performance in human are still contradictory, notably regarding muscle force. The effect of water deprivation (WD) on mechanical properties of skeletal muscle, and more precisely its impact on slow and fast muscles, remains largely unknown. The aim of this study was to determine for the first time whether WD leads to changes in contractile properties of skeletal muscle and whether these changes were muscle-type-specific. METHODS: Sixteen-week-old male rats were assigned to either a control group (C) with water or a 96-hour WD group. At the end of the period, twitch and tetanus properties, as well as biochemical and structural analysis, were performed on soleus (SOL) and extensor digitorum longus (EDL) muscles. RESULTS: Absolute twitch (Pt) and tetanic (P0 ) tension were, respectively, 17% and 14% lower in EDL of WD rats as compared with C rats, whereas unexpected increases of 43% and 25% were observed in SOL. Tensions normalized with respect to muscle mass were not affected by WD in EDL, whereas they were increased by more than 40% in SOL. A 96-hour WD period leads to a decrease in fibre cross-sectional area and absolute myofibrillar content only in EDL. CONCLUSION: It is hypothesized that differences in the results between slow and fast muscles may come from (i) a muscle-type-specific effect of WD on protein balance, EDL showing a greater myofibrillar protein breakdown and (ii) a greater sensitivity to osmolality changes induced by WD in EDL than in SOL.

Endovascular repair of the descending thoracic aorta: mid-term results and evaluation of magnetic resonance angiography.

AIM: The endoluminal stent-grafting represents an alternative to surgery in the treatment of lesions of the descending thoracic aorta. The purpose of the present study was to evaluate the mid-term results of the Talent stent-graft in the different indications of aortic disease and the use of magnetic resonance angiography (MRA) in the diagnosis of complications. METHODS: Over a 3-year period, 23 patients with a high surgical risk and presenting a localized lesion of the descending thoracic aorta had an implantation of a Talent stent-graft. Indications were degenerative aneurysm (n=13), false aneurysm (n=7) and penetrating atherosclerotic ulcer (n=3). The feasibility of the endovascular treatment and sizing of the aorta and stent-grafts were determined pre-operatively by MRA and intraoperative angiography. Immediate and mid-term technical and clinical success were assessed by clinical and MRA follow-up. RESULTS: Endovascular treatment was completed successfully in all patients with no conversion to open repair. There was no intraoperative mortality. The mean operative time was 38+/-7 min. Primary success rate was 100%. We didn't have perioperative mortality. The mean follow-up period was 15+/-5 months. The survival rate was 97% (n=22). Regression of the aneurysmal size was observed in 70% (n=16). MRA diagnosed 3 over 4 postoperative endoleaks that were not diagnosed with the CT-scan, and did not interfere with the nitinol structure of the stent-graft. CONCLUSIONS: Endovascular treatment of the various localized diseases of the descending thoracic aorta is a promising, feasible, alternative technique to open surgery in well selected patients. MRA is well adapted to diagnose postoperative endoleaks.

Metachronous single lung transplantation after contralateral pneumonectomy. A ''big'' challenge?

Single lung transplantation for cystic fibrosis is an uncommon therapy. Contralateral pneumonectomy is, in these cases, rarely done before transplantation. Herein, we report the case of a single lung transplantation in a patient who had previously a contralateral pneumonectomy.

[Results of video-assisted mitral surgery in a non-selected population]. / Résultats de la chirurgie mitrale vidéo-assistée dans une population non sélectionnée.

A prospective 'analysis of operative risk and results in video-assisted mitral valve surgery performed in a non selected population is reported. Seventy two consecutive patients (1997-2004) with mean age 60 +/- 12 years underwent a video-assisted mitral valve procedure using a femoral CPB. A transthoracic direct aortic clamping was done in 28 patients (TT) and an endo-aortic occlusion balloon was used in 44 patients (Endo). The surgical approach was a right lateral minithoracotomy in all cases; 16 patients had a previous cardiac surgery. The expected mitral operation (39 repairs, 33 replacements) was done in all cases, without conversion. There were 4 early deaths (1 st month), all in Endo group: 1 aortic dissection, 1 heart failure and 2 sudden deaths. Postoperative complication occurred in 17 patients with 5 reoperations for hemostasis of the thoracic wall. Cumulative rate of mortality and morbidity was 29% in Endo and 28% in TT (ns). Hospital stay was 8 +/- 2 days. At discharge, 4 patients had a residual grade 2 echocardiographic mitral regurgitation after valve repair. In January 2005, with a 1.8 years follow-up, there were 4 late deaths, 3 patients underwent a valve reoperation, 2 patients were still in NYHA class 3 and 5 patients had a residual grade 1 or 2 mitral regurgitation. The 3-year actuarial survival was 86 +/- 10% and the 3-year probability to be free of reoperation was 95 +/- 6%. In mitral valve surgery, video-assisted approach is reliable, the operative risk is controlled and midterm results are not compromised. Video-assisted mitral valve surgery is a new less invasive standard; it is the procedure of choice in valve replacement, in reoperation and in non complex valve repair with good cosmetic results.

[Air embolism during lumbar discal hernia repair. Retroperioneal vessels lesions have to be suspected]. / Symptomatologie d'embolie gazeuse au cours d'une chirurgie de hernie discale lombaire: suspecter une plaie vasculaire rétropéritonéale.

Occurrence of deep PETCO(2) drop during surgical lumbar disk repair is rare but dramatic. This case report leads to the diagnosis of retroperitoneal vessels lesions. We review the different diagnosis related to the drop of the PETCO(2) during surgery in the genupectoral position. We recommend that the diagnosis of retroperitoneal vessels lesion have to be suspected early if air embolism occurs during lumbar disk surgery.

[Comparison of four formulas of adjusting QT interval for the heart rate in young elite athletes]. / Intervalle QT chez les athlètes : comparaison de quatre formules de correction dans une population de jeunes sportifs de haut niveau.

PURPOSE: Sudden cardiac death in young athlete is always tragic. Some international guidelines recommend the realization of an electrocardiogram before practicing competitive sports to carry out the risk of sudden cardiac death due to genetic cardiopathy like QT long syndrome. Unfortunately, the diagnosis can be difficult because intensive sport can increase the QT interval over normal recognized values for sedentary people. Using a QT correction formula free of heart rate appears essential. PATIENTS AND METHODS: Four hundred and forty-six young athletes (aged 10 to 18) had an electrocardiogram. QT intervals were measured and four methods were used to correct the QT interval for heart rate. RESULTS: The Bazett formula performed the worst in terms of rate adjustment success. Hodges and Fridericia formulas are the best both in males and females, independently of age. Female had longer QTc intervals than males. CONCLUSION: The most widely used Bazett formula should be surrendered whereas Hodges and Fridericia formulas should be preferred, particularly in young athletes.

Management of an aortic wall ulcer after an aortic dissecting hematoma.

We report the case of a 40-year-old woman who presented with an ulcer of the descending aorta 9 months after ascending aortic surgery for intramural aortic hematoma, which was treated surgically with a supra-coronary aortic replacement. Treatment of the second aortic lesion and the choice between endovascular or surgical approaches are discussed.

[Preliminary clinical experience with the Ventrica automatic distal anastomosis system in coronary surgery]. / Expérience clinique préliminaire avec le système d'anastomose distale automatique Ventrica en chirurgie coronaire.

The surgical treatment of coronary lesions is based on bypassing the anatomical lesions with autologous vascular grafts. This procedure has traditionally been "invasive", requiring a micro-surgery technique, institution of extra-corporeal circulation, as well as temporary cardiac arrest with a cardioplegic solution. Recently, an automatic distal anastomosis procedure has been developed (Ventrica, Medtronic Inc.), based on a magnetic coupling with two implanted intravascular magnets, allowing easy connection between the graft and the coronary artery. The immediately obvious advantages are the time saved, ease of use, reproducibility and reliability. The learning curve is fast. Furthermore, the use of this automatic process does not compromise a manual anastomosis in case of implantation failure. The immediate post-operative results, as well as angiography immediately and at 6 months are all satisfactory. This technique is applicable for multiple revascularisations, all types of autologous grafts, terminal or sequential bypasses, as well as nearly all types of coronaries. The contribution to beating heart, closed thorax coronary surgery seems equally promising.

[Fatal surgical extraction of cardiac resynchronisation therapy defibrillator leads in a context of endocarditis due to methicillin-resistant Staphylococcus epidermidis. A case report and review of literature]. / Extraction chirurgicale fatale de sondes de défibrillateur cardiaque triple chambre dans un contexte d'endocardite à Staphylococcus epidermidis méthicilline-résistant. À propos d'un cas et revue de la littérature.

A 82-year-old man equipped with a cardiac resynchronisation therapy defibrillator for dilated cardiomyopathy with normal coronary arteries, in complete atrioventricular block, develops six months after a change of the generator-pocket a severe endocarditis due to a methicillin-resistant Staphylococcus epidermidis with a large lead vegetation. After 4 days of adapted antimicrobial therapy, a surgical device removal is realised with unfortunately a fatal end during extraction. This observation points out the severity of cardiovascular device infections in old and weak population, as well as the difficulty of treatment choices because of both infectious and rhythmic constraints. The lead extraction is a strong recommendation but the modality and timing of extraction are not consensual, especially in cardioverter defibrillator-dependent patients. Surgical removal remains an alternative to percutaneous lead extraction but with a higher operative risk.

Acute brain death abolishes the cardioprotective effects of ischemic preconditioning in the rabbit.

BACKGROUND: Myocardial preconditioning with brief coronary artery occlusions before a sustained ischemic period is reported to reduce infarct size. We wished to evaluate whether ischemic preconditioning (IP) is efficient in an experimental brain death (BD) model in the rabbit. METHODS: Rabbits were randomized into four experimental groups of eight animals each. In the control group (CTRL), anaesthetized rabbits were subjected to 30 min of left coronary marginal branch occlusion and 90 min of reperfusion without any pretreatment. In the CTRL+IP group, anaesthetized rabbits were preconditioned with a 3-min ischemia and 3-min reperfusion sequence before coronary occlusion. In the BD group, rabbits were subjected to 90 min of BD before 30 min of coronary occlusion and 90 min of reperfusion. In the BD+IP group, BD rabbits were preconditioned as in the CTRL+IP group before coronary occlusion. BD was induced by rapid inflation of an intracranial balloon and was validated by clinical and electroencephalographic examinations. At the termination of the experiment, left ventricular volume (LVV), myocardial volume at risk (VAR) and infarct volume (IV) were determined with methylene blue and tetrazolium staining and were measured using planimetry. RESULTS: LW was not significantly different among the four experimental groups (CTRL, 6.54+/-0.90 cm3; CTRL+IP, 5.92+/-0.60 cm3; BD, 5.87+/-0.81 cm3; BD+IP, 6.16+/-0.95 cm3; P=ns). Furthermore, myocardial VAR, expressed as a percentage of LVV, was not significantly different between groups (CTRL, 20.0+/-4.2%; CTRL+IP, 22.32+/-2.25%; BD, 21.38+/-3.36%; BD+IP, 21.64+/-3.39%; P=NS). IV, expressed as a percentage of VAR, was significantly reduced in the CTRL+IP group compared with the CTRL group (15.76+/-8.47% vs. 49.95+/-1.51%; P<0.0001). In contrast, there was no significant difference in IV, expressed as a percentage of VAR, between the BD and the BD+IP groups (50.0+/-1.52% vs. 49.72+/-1.58%; P=NS). CONCLUSION: The data indicate that the infarct-limiting effect of IP is lost in BD rabbits. Thus, the clinical potential of IP in the context of organ transplantation seems to be severely compromised.

Aging effects on monoamines in rat medial vestibular and cochlear nuclei.

Noradrenaline (NA), dopamine (DA); serotonin (5-HT) and their metabolites-3-methoxy, 4-hydroxyphenylglycol (MHPG), 3,4-dihydroxyphenylacetic acid (DOPAC) and 5-hydroxyindoleacetic acid (5-HIAA)-were determined using HPLC in medial vestibular nucleus (MVN), anteroventral cochlear nucleus (AVCN), dorsal+posteroventral cochlear nucleus (DCN+PVCN), locus coeruleus (LC) and raphe dorsalis of Dark Agouti-Hanovre (DA-HAN) rats aged 4, 21 and 24 months. In older rats, the main noradrenergic changes were a decrease of NA content with an increase of the MHPG/NA ratio in MVN and a selective NA increase in AVCN. 5-HT and 5-HIAA levels were increased in all the brainstem nuclei except raphe dorsalis. DA and DOPAC remained unchanged. These data show that noradrenergic neurons in sensory nuclei are differently affected by aging whereas serotonergic activation occurs in most of them possibly as a compensatory response to dysfunction of sensory input and processing. The increase of NA stores in the AVCN of aged rats is in line with the elevated auditory brainstem threshold reported in old rats and could improve the signal-to-noise ratio. Noradrenergic neurons in the MVN seem to be more sensitive to age effect than cochlear nuclei; however, even if neuronal loss occurs, NA synthesis and/or metabolism increase to ensure normal or increased noradrenergic activity.