New analysis of the toxic compounds from the Androctonus mauretanicus mauretanicus scorpion venom

Scorpion venoms are very complex mixtures of molecules, most of which are peptides displaying different kinds of biological activity. Indeed, these peptides specifically bind to a variety of pharmacological targets, in particular ionic channels located in prey tissues, resulting in neurotoxic effects. Toxins modulating Na+, K+, Ca2+ and Cl(-) currents have been described in scorpion venoms. In this work, we have used several specific antibodies raised against the most lethal scorpion toxins already described to screen the Moroccan scorpion Androctonus mauretanicus mauretanicus venom in order to characterize new compounds. This immunological screening was also implemented by toxicity tests in mice and with mass spectrometry study, providing new informations on the molecular composition of this venom. In fine, we were able to determine the molecular masses of 70-80 different compounds. According to the immunological data obtained, many toxins cross-react with three sera raised against the most lethal alpha-toxins found in North African scorpion venoms, but not at all with those raised against the main beta-toxins from South and North American venoms. Some of the previously described toxins from Androctonus mauretanicus mauretanicus venom could thus be detected by combining immunological tests, toxicity in mice and molecular masses. Among these toxins, one of them, which showed a mild cross-reaction with the serum raised against AaH I (a highly potent toxin from the venom of Androctonus australis), was identified as Amm III and fully sequenced.     

Gyrate atrophy of the choroid and retina: a case report

BACKGROUND: Gyrate atrophy of the retina and choroid is a rare disease, with recessive autosomal transmission, characterized by progressive chorioretinal atrophy causing blindness. It results from a congenital deficit in aminotransferase ornithine. Case report:The authors present the case of a young patient aged 15 years old consulting for a progressive fall of visual acuity with hemeralopia. Eye funduscopy showed regions of confluent rounded chorioretinal atrophy. The visual field, the electroretinogram and the retinal angiography were all alterated. Gyrate atrophy of the retina and choroid was evocated. DISCUSSION: It is a systemic and rare metabolic disease where ocular features are dominating. Differencial diagnosis are pigmentary retinopathies. Cataract and/or myopia are often joined to the retinal lesions. General signs could consist in muscular weakness, thin and rare hairs and mental retardation. More than; visual fields, electroretinogram, retinal angiography that are alterated; the plasmatic dosage of the ornithine is often high. The treatment is based on the dietetics with uncertain results. The genic therapy would be the treatment of future.     

Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus: case report

BACKGROUND: Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION: A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.     

Aortic cusp extension valvuloplasty for rheumatic aortic valve disease: midterm results

BACKGROUND: The introduction of composite graft repair of aortic root aneurysm by Hugh Bentall in 1968 promised Marfan patients the choice for a normal life expectancy. We performed our first Bentall composite graft procedure in 1976 and herein report our 24-year experience with 271 Marfan patients. METHODS: Between September 1976 and August 2000, 232 Marfan patients had a composite graft replacement of the aortic root, 15 patients received a homograft, and 24 had a valve-sparing procedure. RESULTS: Two hundred thirty-five Marfan patients underwent elective aortic root replacement with no 30-day mortality. Two early deaths occurred among 36 patients who underwent urgent or emergent operation. Eighty-three percent of patients in this series are currently alive. The actuarial freedom from thromboembolism, endocarditis, and reoperation on the residual aorta 20 years postoperatively was 93%, 90%, and 74%. Twenty-four patients have undergone valve-sparing procedures with encouraging results. CONCLUSIONS: Elective aortic root replacement for Marfan patients can be performed with low operative risk. Elective repair before the aortic root reaches 6 cm in diameter is recommended to minimize risk of dissection and rupture.     

Myocardial infarction by complete thrombosis of the common coronary trunk. Two case reports

Complete thrombosis of the left main coronary artery is a rare angiographic finding. It carries a very high mortality rate related to cardiogenic shock; malignant arrhythmia or sudden death. We report two case of a 37 and 65 years old women, admitted to our hospital with complete occlusion of the left main coronary responsible of anteroseptal myocardial infarct. The revascularisation consisted in surgical treatment in one case and percutaneous angioplasty in the second patient. The aim of our study is to discuss the different therapeutic approaches and the prognosis of this affection.     

Mitral valve prolapse and sudden death: a case report

Mitral valve prolapse (MVP) is a common disorder that, in general, has a good prognosis. Rare occasions of sudden death have been reported in patients with MVP and it is presumed that the basis of sudden death is arrhythmias. We report a case of a 47 years old men affected by MVP complicated by ventricular arrhythmias and sinoatrial block; who died suddenly from ventricular tachycardia. The pathophysiology and risk factors of sudden cardiac death in MVP are discussed.     

Splenic arteriovenous fistula and portal hypertension. A case report

The authors report a rare case of portal hypertension following to an arteriovenous fistula. The embolisation of the fistula permitted to treat portal hypertension and liver histologic alterations.     

Pacemaker infections

Infectious complications following pacemaker implantation are not common but can be particularly severe. The reported incidence varies from 0.5 to 5% in the literature. The duration of the procedure and repeat procedures are considered to be predisposing factors. The main cause of these infections is thought to be local contamination during the implantation. The commonest causal organism is staphylococcus. Because the presentation may be atypical, the diagnosis is often confirmed by transesophageal echocardiography which is the investigation of choice for imaging a vegetation on an endocavitary pacing lead in cases of infectious endocarditis. The seriousness of this infection requires early diagnosis and adapted treatment including double bactericidal antibiotherapy and complete ablation of the material. Systematic preoperative prophylactic antibiotic therapy is recommended.     

Treatment by splenectomy of a portal vein aneurysm in hepatosplenic schistosomiasis

Portal vein aneurysm is a rare medical entity that can be caused by chronic hepatic diseases with portal hypertension. We describe a 45-year-old man with variceal bleeding from hepatosplenic schistosomiasis and an incidentally found intrahepatic aneurysm. Diagnosis was confirmed with non-invasive imaging exams, arteriography and liver biopsy. Following splenectomy, the aneurysm diameter decreased substantially.