Evaluation and management of thromboprophylaxis in Moroccan hospitals at national level: the Avail-MoNa study.

Venous thromboembolism (VTE) is a common clinical problem that is associated with substantial morbidity and mortality. The aim of this study was to describe the clinical practices in VTE prophylaxis in university and peripheral hospitals in Morocco. This is a national, cross-sectional, multicenter, observational study assessing the management of the VTE risk in selected Moroccan hospitals (four university and three peripheral). The thromboembolic risk of the selected patients was assessed according to the American College of Chest Physicians (ACCP) guidelines (2008). We hypothesized that interventions for VTE guideline implementation in those hospitals may improve prophylaxis use for hospitalized patients. A total of 1318 patients were analyzed: 467 (35.5%) medical and 851 (64.5%) surgical. The mean age of patients was 52.6 ± 16.5 years, and 52.7% were female. A total of 51.1% patients were considered to be at risk of VTE according to ACCP guidelines and were eligible for thromboprophylaxis (TP). Medical patients were more likely to present risk factors than surgical patients (53.6 vs. 50.7%, respectively). TP was prescribed for 53.1% of these patients, 57.4% in at-risk surgical patients and 50.3% in at-risk medical patients. TP was also prescribed for 42.9% of non-at-risk patients. The concordance between the recommended and the prescribed prophylaxis was poor for the total population (kappa = 0.110). TP did not improve sufficiently in our hospitals, even after implementation of the guidelines. New strategies are required to appropriately address TP in hospitalized patients.

[Lymphohistiocytic activation syndrome (LHAS)]. / Le syndrome d'activation lympho-histiocytaire (SALH).

Lymphohistiocytic activation syndrome (LHAS) is related to inappropriate stimulation of macrophage cells in bone marrow and lymphoid system. LHAS combines the non-specific clinical signs (fever, poor general condition, hepatosplenomegaly, lymphadenopathy) and suggestive biological elements (bi-or pancytopenia, abnormal liver function, coagulopathy, increased LDH, ferritin and triglycerides). The diagnosis of SALH remains an emergency every clinician should discuss before any febrile cytopenia. The etiology of LHAS is still obscure, but recent advances in the genetic study of familial forms provide some essential elements in understanding.

[Acquired ichthyosis and haematological malignancies: five cases]. / Ichtyose acquise et hémopathie maligne : cinq observations.

BACKGROUND: Acquired ichthyosis is a rare condition that can reveal an unsuspected haematological malignancy, thus allowing early diagnosis and management. If ichthyosis regresses under treatment for the haematological disorder, its recurrence reflects a turning point in the course of the disease and implies worsening of the prognosis. PATIENTS AND METHODS: The patients were examined at a joint dermatology/haematology consultation. The diagnosis of ichthyosis was based on clinical examination alone with no patients undergoing skin biopsy. RESULTS: Our series included three men and two women aged 38 to 65 years consulting for a variety of reasons including asthenia, anaemia and adenopathy. Ichthyosis occurred 2 to 9 months after the initial symptoms of the blood disease. Lesions consisted of diffuse brown scales. The disease was associated with lymphadenopathy and biological inflammatory syndrome. Two patients were presenting non-Hodgkin lymphoma, one had Hodgkin's disease, one had chronic myeloid leukaemia in progression and one had an undifferentiated lymphomatous process. Treatment was based on chemotherapy and emollients. The ichthyosis progressed in step with the underlying malignancy in all cases, with regression being complete in three cases, partial in one case and absent in one case. DISCUSSION: In rare cases, acquired ichthyosis reveals systemic disease, and may be of infectious, endocrine or drug origin; it may also be idiopathic. However, it is most often a paraneoplastic syndrome with cutaneous expression encountered during haematological malignancies. Because of the variety of causative blood dyscrasias, ichthyosis cannot be used to guide their diagnosis, although it remains a reliable monitoring tool. CONCLUSION: Acquired ichthyosis should prompt the clinician to search for a neoplastic condition, primarily a haematological disorder, guided by other associated signs, given that in our study, skin lesions generally appear to precede signs of the blood disease.

[Intracardiac thrombosis in Behçet disease: clinical presentation and outcome of three cases]. / Thrombose intracardiaque au cours de la maladie de Behçet : étude de trois observations.

BACKGROUND: Behçet's disease is recognized as a multisystem disorder that affects mainly young adults in Mediterranean, Middle Eastern, and Far Eastern countries. The diagnosis is very difficult because there is no laboratory test. Clinical features - such as orogenital aphtae, ocular and skin lesions, arthritis, and neurologic, gastrointestinal, vascular, and pulmonary symptoms - are helpful for diagnosis. Various cardiovascular manifestations, such as pancarditis, acute myocardial infarction, conduction system disturbances, and valvular diseases, have been reported but are rare. Intracardiac thrombus formation, as seen in our patients, is exceptional even among cardiovascular cases of Behçet's. OBSERVATIONS: We report three cases of intracardiac thrombosis among 204 patients followed for Behçet's disease within our unit over a period of 7 years. We report outcomes after corticosteroid, cyclophosphamide, and oral anticoagulant therapy. DISCUSSION: Cardiovascular involvement has been reported in 7 % to 29 % of patients with Behçet's syndrome. Intracardiac thrombosis is extremely rare and the right heart is the most common site of involvement. The first symptoms and signs of the disease frequently precede systemic organ manifestations. CONCLUSION: Diagnosis of Behçet's disease might be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical features of the condition. This is particularly applicable if the patient is a young male from the Mediterranean basin or the Middle East. We suggest that the treatment could include colchicine, anticoagulant therapy, and corticosteroids and discuss immunosuppressive therapy.

[Thrombosis of the inferior vena cava revealing primary antiphospholipid syndrome: a case report]. / Thrombose de la veine cave inférieure sur syndrome primaire des antiphospholipides : à propos d'un cas.

Thrombosis of the inferior vena cava is a rare clinical expression of primary antiphospholipid syndrome. This case clearly illustrates the clinical manifestations, work-up findings and management principles of thrombosis of the inferior vena cava in primary antiphospholipid syndrome before the stage of complication. The patient was a 24-year-old female with a history of recurrent deep venous thrombosis. She was admitted to the department of internal medicine for dyspnea and pain of the right hypochondria. Physical examination disclosed an edematous ascitic syndrome. The scanner coupled with Doppler ultrasonography showed thrombosis of the inferior vena cava. On these radiological findings, an immunological work-up was requested, which showed the presence of antiphospholipid antibodies.

[Wilson's disease. Clinical presentation, treatment and evolution in 21 cases]. / La maladie de Wilson. Etude clinique, thérapeutique et évolutive de 21 cas.

PURPOSE: Wilson's disease is characterized by neuropsychiatric symptoms with frequent extrapyramidal and intellectual presentations. They have an insidious evolution that leads to a late diagnosis and less therapeutic effectiveness in the advanced forms. METHODS: We report 21 cases of Wilson's disease with neurological complications, emphasizing clinical semiology, diagnostic means and problems of the therapeutics in our country. RESULTS: The average age at the beginning of the disease was 17.6 years, with a female prevalence (8/13). The signs at first were mostly all neurological (71.4%), then psychiatric (19%) or hepatic (19%). The most common neurological signs were dystonia of members (81%), dysarthria (76%), tremors (76%) or disorders of motoricity (71.4%). Sometimes there were sialorrhea or disorders of the handwriting. The Kayser-Fleischer ring was present in 19 patients. Eighteen patients had clinical and/or biological hepatic involvement. The diagnosis was confirmed by biochemical examinations, which found a low rate of copper in blood, a sinking rate of ceruloplasmin and a very high rate of urinary copper. The cerebral computer tomography shows a cortical and/or subcortical atrophy (37%), and/or a low density of the central grey cores (35%). The treatment was based on D-penicillamine and/or zinc sulfate, according to the availability of the drugs. The evolution was favourable among 18 patients (85%) and not good in 42.8% of the cases. Six of the first patients had poor evolution after many years of follow-up. Finally, only 12 patients (57%) had a very good outcome. The family investigation made among 17 patients revealed 13 family cases. The only predictive factor of a poor evolution was the therapeutic noncompliance (P = 0.006). CONCLUSIONS: The neurological presentations are traditional during the Wilson's disease, but are often ignored. We must suspect the disease in children when faced with disorders of handwriting or school failures and in the adult, when faced with neurological symptoms in a patient having a hepatic disease. We must not hesitate to consider it even given purely psychiatric signs, and we had better know to seek the neurological ones.