Predicting the risk of recurrent venous thromboembolism: Impact and therapeutic consequences of inherited thrombophilia.

BACKGROUND: Over the past decades, thrombophilia testing in patients with venous thrombo-embolism has increased tremendously. However, the role of inherited thrombophilie in prediction the risk of recurrence remains controversial. Consequently, it is still unclear whether thrombophilia testing influences decisions regarding duration of anticoagulation in clinical practices. The aim of this study was to evaluate the impact if inherited thrombophilia on venous thrombosis treatment decisions and on predicting the risk of recurrence. METHODS: A retrospective longitudinal study (January 2011-Decembre 2016) including 131 patients with confirmed venous thrombo-embolism referred to the hematology laboratory from the internal medicine department for inherited thrombophilia screening was carried out. RESULTS: The mean age patients was 39.4 years and the sex ratio (M/F) was 0.61. Inherited thrombophilia was confirmed in 27.5% of patients. A long term anticoagulation was decided in 46.9% of patients with thrombophilia. There was no significant difference in the duration of anticoagulation between patients with or without thrombophilia. Thrombosis recurrence was recorded in 16 (17%) patients. The 24 years cumulative incidence of recurrence was 19% in patients with thrombophilia and 17% in those without (plog Rank= 0.6). Inherited thrombophilia was not associated with increased risk of recurrence after treatment withdrawal (Hazard ratio=1.31 IC (0.47-3.63); P=0.6). CONCLUSION: In clinical practice, inherited thrombophilia did not influence anticoagulation duration and was not associated with a higher venous thrombosis risk of recurrence. It seems to be less relevant for decision making than presumed.

Addition of magnesium sulfate to bupivacaine improves analgesic efficacy after tonsillectomy: A randomized trial and a CONSORT analysis.

OBJECTIVES: The main objective of this study was to compare the wound infiltration (peritonsillar fossa) of magnesium sulphate combined with bupivacaine, bupivacaine alone and saline solution on post-tonsillectomy pain in children. The accessory objectives were to evaluate the effect of magnesium sulphate infiltration on prevention of laryngospasm and occurrence of nausea/vomiting. METHODS: This study is a prospective; double blinded and randomized clinical trial. Seventy-five children undergoing tonsillectomy were enrolled. Patients were randomized into three groups using closed envelop technique. Group 1 (N=24) received saline solution (NaCl), group 2 (N=25) received 0.25% bupivacaine (1mg/kg) and group 3 received magnesium sulphate (5mg/kg) and 0.25% bupivacaine (1mg/kg) after tonsillectomy using three-point technique. Pain was evaluated using mCHEOPS scale. The occurrence of laryngospasm, nausea and vomiting was monitored. RESULTS: The mCHEOPS scores of the group 3 were significantly lower than those of the group 2 and 1 (P<0.001). Time to first analgesic administration was longer for the group 3 than for the groups 2 and 1 (P<0.001). The mean consumption of additional analgesic drugs was lower for the group 3 than the other groups (P<0.001). There were no episodes of laryngospasm in the group 3 in comparison with the other groups. The difference of the incidence of nausea and vomiting was not statistically significant (P=0.628). CONCLUSION: The adjunction of magnesium sulphate to bupivacaine proved to provide more efficient pain control than bupivacaine alone. However, the small number of participants and the absence of sampling at the P level of 0.005 do not allow to conclude with absolute certainty.

The international normalized ratio (INR): What reagent, what instrument? The assessment of the agreement between INR values according to different reagent/instrument combinations.

WHAT IS KNOWN AND OBJECTIVE: The international normalized ratio (INR) is widely used to monitor patients on vitamin K antagonists. This study aimed to assess the agreement of INR values obtained with different thromboplastin/instrument combinations. MATERIAL AND METHODS: International normalized ratio was determined on plasmas from 330 patients undergoing antivitamin K treatment (with acenocoumarol), using two calibration methods and four reagent/instrument combinations: Both Neoplastine CI and Neoplastine CI Plus on STA-R instrument from Diagnostica STAGO, Asnières, France; and both Thromborel S and Innovin on SYSMEX 2100i instrument from Siemens Health Care Diagnostics, Marbung, Germany. The agreement analysis was done using the Bland-Altman plot and the Cohen Kappa coefficient. RESULTS: The mean of the differences between the INR values and the limits of agreement were -0.07 [-0.51 to 0.38] for the Neoplastine CI plus and Neoplastine CI reagents, -0.08 [-1.18 to 1.03] for the Thromborel S and Innovin reagents when the INR was calculated, -0.1 [-1.15 to 0.95] for the Thromborel S and Innovin reagents when the INR was directly calibrated and -0.1 [-0.7 to 0.5] for the Neoplastine CI plus and Thromborel S. Cohen's kappa coefficients were 0.94, 0.76, 0.85 and 0.82, respectively. NEW FINDINGS AND CONCLUSION: The agreement between the four reagent/instrument combinations was high enough to classify patients as inefficaciously or efficaciously anticoagulated. The data interpretation should always be related to the clinical purpose.

The evaluation of the relevance of thrombin generation and procoagulant activity in thrombotic risk assessment in BCR-ABL-negative myeloproliferative neoplasm patients.

INTRODUCTION: It has been recently suggested that microparticles (MP) play a role in the pathogenesis of thrombotic complications. This study aimed to assess the contribution of procoagulant activity expressed by circulating MP in thrombotic events in MPN patients. METHODS: Seventy-four MPN patients were enrolled in a trans-sectional study. The MP procoagulant activity was measured using two assays: (i) the thrombin generation (TG) assay used in different conditions with the addition of both tissue factor (TF) and phospholipids (PL) and with the addition of TF or PL alone and (ii) the PROCOAG-PPL assay. RESULTS: The mean age was 62 (26 men and 48 women). The prevalence of thrombotic events was 28%. When comparing patients with thrombosis to those without, age, sex, MPN type, cardiovascular risk factors, and history of thrombosis were not significantly associated with thrombosis. The JAK2 V617F mutation was significantly associated with thrombotic events (90% vs 67%; P=.04). Results from the TG assay and the PROCOAG-PPL assays did not demonstrate a significant association between the MP procoagulant activity and thrombotic events. CONCLUSION: The MP procoagulant activity did not predict thrombosis in MPN patients. The contribution of TG assay in the assessment of the thrombotic risk is still in debate.

[Parathyromatosis: An uncommon cause of recurrent hyperparathyroidism]. / Parathyromatose : une cause rare d'hyperparathyroïdie récidivante.

INTRODUCTION: Parathyromatosis is a rare cause of recurrent hyperparathyroidism. It results from hyperfunctioning parathyroid tissue scattered throughout the thyroid bed region. CASE REPORT: A 51-year-old man with a history of parathyroidectomy, presented 18 years later with recurrent primary hyperparathyroidism. Surgical exploration identified a single parathyroid gland. The act was completed by a central compartment dissection and ipsilateral lobectomy. The patient was free of recurrence after a one-year follow-up. CONCLUSION: Parathyromatosis a rare cause of recurrent hyperparathyroidism. Its management is challenging. Extensive surgery is required with clearance of the central neck compartment and homolateral lobectomy. Medical therapy could be used to decrease parathormone level in recurrent parathyromatosis.

[The syndrome of increased upper airways resistance: What are the clinical features and diagnostic procedures?]. / Syndrome de haute résistance des voies aériennes supérieures: quelles approches cliniques et quelles procédures diagnostiques?

The upper airway resistance syndrome "UARS" is a poorly defined entity, often described as a moderate variant of the obstructive sleep apnea syndrome. It is associated with respiratory effort-related arousal, absence of obstructive sleep apnea, and absence of significant desaturation. It is a relatively common condition that predominantly affects non-obese young adults, with no predominance in either sex. The degree of upper airway collapsibility during sleep of patients with UARS is intermediate between that of normal subjects and that of patients with mild-to-moderate sleep apnea syndrome. Craniofacial and palatal abnormalities are often noted. Patients frequently complain of a functional somatic syndrome, especially daytime sleepiness and chronic fatigue. Polysomnography with esophageal pressure measurements remains the gold standard diagnostic test. The absence of any neurological abnormality gives UARS a good prognosis and it is potentially reversible if treated early. However, some studies suggest that untreated UARS has an increased risk of arterial hypertension. It can also evolve into obstructive sleep apnea.

[Surgery of pleomorphic adenoma of the parotid gland]. / Chirurgie de l'adénome pléomorphe de la parotide.

INTRODUCTION: Pleomorphic adenoma is the most common benign tumor of the parotid gland. It has a tendency of recurrence and malignant transformation. The surgical excision of this lesion continues to be the subject of a major debate. In this study, we discuss optimal surgical options for pleomorphic adenoma. MATERIALS AND METHODS: We reviewed clinical records of 82 patients who underwent surgery. RESULTS: The tumor was localized in the superficial lobe in 81.7% of cases. Tumors of the deep lobe were removed by total parotidectomy. Those of the superficial lobe underwent partial exofacial parotidectomy (7 cases), exofacial parotidectomy (44 cases), or total parotidectomy (16 cases). Transitory facial paralysis was higher after total parotidectomy. Only one patient developed a recurrence, his tumor showed capsule infiltration. DISCUSSION: Conventional or partial superficial parotidectomy may be a good compromise with fewer complications and low incidence of recurrence.

[Idiopathic facial paralysis in children]. / Paralysie faciale idiopathique de l'enfant.

BACKGROUND: Idiopathic facial palsy is the most common cause of facial nerve palsy in children. Controversy exists regarding treatment options. The objectives of this study were to review the epidemiological and clinical characteristics as well as the outcome of idiopathic facial palsy in children to suggest appropriate treatment. PATIENTS AND METHODS: A retrospective study was conducted on children with a diagnosis of idiopathic facial palsy from 2007 to 2012. RESULTS: A total of 37 cases (13 males, 24 females) with a mean age of 13.9 years were included in this analysis. The mean duration between onset of Bell's palsy and consultation was 3 days. Of these patients, 78.3% had moderately severe (grade IV) or severe paralysis (grade V on the House and Brackmann grading). Twenty-seven patients were treated in an outpatient context, three patients were hospitalized, and seven patients were treated as outpatients and subsequently hospitalized. All patients received corticosteroids. Eight of them also received antiviral treatment. The complete recovery rate was 94.6% (35/37). The duration of complete recovery was 7.4 weeks. DISCUSSION: Children with idiopathic facial palsy have a very good prognosis. The complete recovery rate exceeds 90%. However, controversy exists regarding treatment options. High-quality studies have been conducted on adult populations. Medical treatment based on corticosteroids alone or combined with antiviral treatment is certainly effective in improving facial function outcomes in adults. In children, the recommendation for prescription of steroids and antiviral drugs based on adult treatment appears to be justified. CONCLUSION: Randomized controlled trials in the pediatric population are recommended to define a strategy for management of idiopathic facial paralysis.

[Low-grade adenocarcinoma of nasal cavities: a case report]. / Adénocarcinome de bas grade des fosses nasales : à propos d'un cas.

Low-grade sinonasal adenocarcinomas are uncommon and recently described entities. Its histologic diagnosis is challenging. This tumour is characterized by a tendency to local invasion, and rare distant metastases. Well treated, the prognosis is excellent. We describe a case of low-grade nasal cavity adenocarcinoma and discuss the anatomoclinical, therapeutic and evolutionary characteristics of this malignant tumour. A 54-year-old female patient presented with a 10 years history of right-sided nasal obstruction and recurrent epistaxis. On examination the patient had a large, firm mass in the right nasal cavity. Endoscopic sinonasal surgery was performed. The lesion was found to originate from the posteriolateral wall of the right nasal cavity. Histopathology analysis identified a low-grade sinonasal adenocarcinoma. Upon follow-up 4 years after surgery, the patient exhibited no clinical evidence of recurrence. Low-grade sinonasal adenocarcinomas are poorly defined neoplasms, accounting for 4 to 20% of all sinonasal malignancies. The nasal cavity is the most frequently involved site. Low-grade sinonasal adenocarcinomas pose a diagnostic challenge for the pathologist because they must be distinguished from benign tumours, especially adenomas. The primary treatment of sinonasal adenocarcinoma is complete surgical excision.

Primary mucinous carcinoma of the thyroid gland: case report with review of the literature.

Primary mucinous thyroid carcinoma (PMTC) are extremely rare lesions that are histologically indistinguishable from mucinous carcinoma of other sites. We describe the clinicopathological, histological and immunohistochemical features of this rare tumour with a review of the literature. We describe a case of thyroid tumour, in 56-year-old Tunisian man, composed of small nests and sheets of malignant epithelial cells associated with extensive extracellular mucin that entrapped the follicular parenchyma of thyroid. Thyroglobulin and thyroid-specific-transcription factor 1 (TTFl) were focally positive. Follow-up did not reveal another neoplasm at other sites. Based on these features, we classified this tumour as PMTC. Mucinous carcinoma of the thyroid gland can be a cause of pitfall in differential diagnosis. For correct diagnosis, complete clinical history, restricted histological criteria and immunohistochemical panel are necessary.

[Bronchoscopy for foreign body inhalation in the pediatric population: lessons learned from 223 cases]. / Bronchoscopie pour inhalation de corps étrangers chez l'enfant : à propos de 223cas.

INTRODUCTION: Inhalation of foreign body in children is a serious accident that may compromise the vital prognosis of the child. The diagnostic was difficult in the absence of a recognizable penetration syndrome. Bronchoscopy is still recommended as the appropriate diagnostic and treatment of foreign bodies. The purpose of this study was to analyze the diagnostic and the treatment result of bronchoscopy and discuss its indications. MATERIAL AND METHODS: [corrected] A retrospective study analyzing data related to 223 children undergoing bronchoscopy due to suspicion of foreign body aspiration over a period of 10 years (2000-2009). The average age of the children was 29 months (range: one month-13 years). Approximately, two thirds of these patients were boys. The penetration syndrome was reported in 79.8% of cases. RESULTS: During bronchoscopy, the foreign body was confirmed only in 57.4%. Foreign bodies were found in the bronchus in 79.7% of cases. Among the foreign bodies, 78.1% were of vegetal origin. The average time of stay of the foreign body was of 16.1 days. Penetration syndrome and abnormal physical exam were the most sensitive parameters (79.7% and 82.8%, respectively) but with low specificity (24.2% and 35.8%, respectively). The combination of clinical and radiological signs suggestive of foreign body was the most specific sign (74.7%). Similarly, we found a statistically significant correlation between positive bronchoscopy and simultaneous suggestive clinical and radiological signs (P=0.03). The multivariate study showed that predictors factors of positivity of the bronchoscopy were: abnormal physical exam (P=0.016), abnormal radiological exam (P=0.003) and type of indication (P=0.005). DISCUSSION: The diagnosis of laryngotracheobronchial foreign body recures an array of arguments. It is suspected on the clinical interview specially penetration syndrome and on the clinical and radiological presentation. Any suspicion should lead to a bronchoscopy.

Primary sinonasal meningioma.

INTRODUCTION: Primary meningiomas of the sinonasal tract are rare tumors. Their positive diagnosis is difficult to establish. From one case observation, we report the clinical features, the diagnosis difficulties and the therapeutic modalities of primary meningioma of the sinonasal tract. CASE REPORT: A seventeen-year-old girl consulted for a left unilateral nasal obstruction with progressive evolution without episodes of epistaxis, smell disorder or headaches over a year. Physical examination revealed a grayish polypoid tumor in the left nasal fossa. CT scan evidenced an isodense lesion of the left nasal fossa slightly enhanced pushing back the lateral nasal wall without invasion or intracranial connection. Biopsy was in favour of an inverted papilloma. The tumor was resected via endoscopic approach. Pathological examination established the diagnosis of meningothelial menigioma. The prognosis was favourable without recurrence after a six-month follow-up. CONCLUSION: The positive diagnosis of primary sinonasal meningioma is difficult to establish because of their infrequent occurrence in this ectopic site and of their non-specific clinical appearance. The final diagnosis rests on the histological examination. Immunohistochemical studies are helpful to establish the accurate diagnosis. Imaging confirms the primitive nature of these tumors. Prognosis is excellent after complete surgical extirpation without the necessity of adjuvant therapy.

Internal jugular vein thrombosis by protein C activated resistance.

INTRODUCTION: Jugular vein thrombosis is mainly due to infectious, neoplastic and iatrogenic causes. Activated protein C (APC) resistance is an exceptional cause of jugular vein thrombosis. CASE REPORT: A 40-year-old woman consulted for left lateral neck swelling present for two weeks. Neck ultrasound revealed left internal jugular vein thrombosis, which was confirmed by contrast CT. The rest of the examination, including routine clotting assessment and inflammatory work-up, was normal. Further investigations demonstrated APC resistance with Factor V Leiden mutation. Treatment consisted of oral anticoagulants with a good outcome. DISCUSSION: APC resistance is a recently identified and relatively frequent cause of thrombophilia, mostly due to Factor V Leiden mutation. APC resistance is responsible for 20% to 50% of all thrombotic events. The laboratory diagnosis is based on two tests: a phenotypic test based on APTT with and without APC and a genotypic test based on detection of a Factor V Leiden mutation. CONCLUSION: Screening for APC resistance and Factor V Leiden mutation is now part of the aetiological work-up of thromboses, particularly in subjects younger than 50. Treatment is based on oral anticoagulants.

[Post-radiation mucocele in two patients treated for nasopharyngeal cancer]. / Mucocèle après radiothérapie chez deux patients traités pour cancer du nasopharynx.

A 30-year-old woman, with a history of nasopharyngeal carcinoma, which was treated by radiotherapy nine years previously, presented with occasional diplopia and recent headaches. A nasopharyngeal biopsy showed no recurrence. The imaging revealed a sphenoidal sinus mucocele. Endoscopic marsupialization of the mucocele allowed clinical improvement. A 56-year-old woman presented, five years after radiotherapy for nasopharyngeal carcinoma, with a fronto-orbital mass. CT-scan revealed a fronto-ethmoidal mucocele. Nasopharyngeal biopsy showed tumour recurrence. Marsupialization of mucocele was performed. Recurrence of the carcinoma was treated by radiotherapy and chemotherapy. Sphenoidal sinus mucocele developing after radiotherapy for nasopharyngeal carcinoma has rarely been reported. CT scan and MRI are useful tools in making the diagnosis. Biopsy is required to diagnose recurrence or associated radio-induced tumor. Endoscopic approach gives good results.

Unusual form of cutaneous leishmaniasis: erysipeloid form.

UNLABELLED: We report the epidemiological and clinical characteristics of the erysipeloid form of cutaneous leishmaniasis as well as its diagnostic and therapeutic challenges. CASE REPORT: A 63-year-old woman, with no medical history, presented with a one-month history of erythematous nasal swelling. The lesion appeared after an accidental trauma. Erythematous infiltrative plaque was noted on the center of the face. There were also crust formations on the traumatic region. Despite local treatment and oral antibiotherapy, there was no improvement. The diagnosis of cutaneous leishmaniasis was confirmed by positive skin smears. Histopathological examinations of a skin biopsy showed no malignancy. The patient was treated intramuscularly with 10mg/kg per day systemic meglumine antimoniate with partial regression of symptoms. CONCLUSION: The erysipeloid type is a rare and unusual presentation of cutaneous leishmaniasis that often causes late diagnosis. Diagnosis is confirmed by the demonstration of the parasite by skin smear, histopathological examination and polymerase chain reaction. There are various therapeutic options. The evolution is generally favourable.

Paraneoplastic dermatomyositis accompanying nasopharyngeal carcinoma: diagnosis, treatment and prognosis.

Dermatomyositis (DM) is a multisystem inflammatory disorder primarily affecting the skin and muscles. Its pathophysiology is still very poorly understood, but humoral and cellular immune dysregulation is apparent. Diagnosis of DM is based on five criteria: proximal limb muscle weakness, serum muscle enzyme elevation, histopathologic muscle abnormalities on muscle biopsy, electromyographic abnormalities, and clinical inflammatory dermatological manifestations (heliotrope rash, poikiloderma, and inflammatory lesions on the hands and facing joints). DM is frequently associated with certain cancers, and may appear before, concurrent with, or after diagnosis of cancer. DM has been reported to be associated with approximately one per 1000 cases of nasopharyngeal carcinoma. Treatment is based on long-course nonselective immunosuppression, particularly corticosteroids, by general route, even when malignancy is present, but new-targeted therapies may modify the treatment strategy in the near future. Despite iatrogenic immunosuppression, the prognosis of nasopharyngeal cancer is not worse in patients with paraneoplastic DM. We report one case as an illustration of this paraneoplastic course (evolving in parallel with the cancer), and to make an update on the state of knowledge on paraneoplastic DM in such cancers.

Cholesteatoma of the frontal sinus.

PURPOSE OF STUDY: Cholesteatoma of the paranasal sinuses is a rare pathology. A review of the literature reported less than 30 cases. These lesions mainly involve the frontal sinus. CASE REPORT: A 25-year-old man presented with a right painful fronto-orbital mass associated with an ipsilateral eyelid oedema and a fever as high as 40°C. He experienced a general epileptic seizure requiring his admission in an intensive care unit. CT-Scan with iodine injection evidenced the opacification of the right frontal sinus with bone lysis of the posterior wall. Complete surgical resection of a cystic structure containing keratin material was performed via eyebrow incision. The pathological examination confirmed the diagnosis of sinus cholesteatoma. Neurological signs entirely disappeared after surgery. Craniofacial MRI realized 2 months later showed no sign of recurrence. Obliteration of the right frontal sinus was performed 4 months later. CONCLUSION: Although benign, cholesteatoma can spread to the surrounding structures leading to several complications including infections that can be life-threatening for the patient. CT-scan and MRI are useful examinations for diagnosis and follow-up. Complete surgical resection is required in order to avoid recurrence.

Occult lymph node metastasis in laryngeal squamous cell carcinoma: therapeutic and prognostic impact.

OBJECTIVES: To assess the incidence and impact on treatment and prognosis of occult lymph node metastasis in laryngeal cancer. PATIENTS AND METHODS: A retrospective study was performed on 164 patients treated for laryngeal cancer, initially classified as N0, with cervical lymph node dissection. RESULTS: Occult metastases were found in 41 neck specimens (12.5%) from 32 patients (19.5%). Involvement per neck level was: 7% level IIa, 2.4% IIb, 4.2% III and 2.7% IV. Lymph node involvement was significantly increased in case of T3T4 tumor or invasion of the pre-epiglottic space or cartilage. Survival was significantly influenced by pN status (pN- = 12 years, vs pN+ = 9 years; P = 0.006). CONCLUSION: Level IIb or IV involvement is rare. Superselective neck dissection (IIa, III) seems to be indicated in T1T2 N0 tumor. In case of advanced tumor or pre-epiglottic space or cartilage invasion, functional neck dissection is mandatory.

B-cell lymphoma of the mandible.

INTRODUCTION: Primary malignant lymphoma of the mandible is rare. It is frequently mistaken for a dental lesion, delaying diagnosis. We here report a case of mandibular lymphoma and present the clinical and radiological characteristics and means of treatment of this pathology. CASE REPORT: A 17-year-old woman consulted for right facial tumefaction with 1 year's evolution. Clinical examination found a mass facing the horizontal branch of the mandible, extending to the right parotid region without cutaneous involvement. CT and MRI showed a large expansive process of mandibular origin with parotid, temporal and intracranial extension. Biopsy indicated large B-cell lymphoma. The patient showed good evolution 2 years after chemotherapy. CONCLUSION: Lymphoma is the second most frequent form of head and neck tumor, after epidermoid carcinoma. Only 0.6% of locations are mandibular, almost always consisting of B-cell lymphoma. They are often initially misdiagnosed as a dental pathology. Complete remission after chemotherapy ranges from 60 to 80% at 1 year.