Acute pericarditis due to pegylated interferon alpha therapy for chronic HCV hepatitis - Case report

Background  

Cardio toxicity due to interferon therapy was reported only in small case series or case reports. The most frequent cardiac adverse effects related to interferon are arrhythmias and ischemic manifestations. The cardiomyopathy and pericarditis are rare but can be life threatening. The predisposing factors for interferon cardio toxicity were described only for ischemic manifestations and arrhythmias.     

Actinomyces graevenitzii pulmonary abscess mimicking tuberculosis in a healthy young man

Pulmonary actinomycosis is a rare disease that is often misdiag-nosed as tuberculosis or lung cancer. Actinomyces graevenitzii is a relatively new recognized Actinomyces species isolated from various clinical samples. The authors report a case of pulmonary actinomycosis caused by A graevenitzii. A computed tomography examination revealed an excavated consolidation in the middle right lobe of a previously healthy young man who presented with a long history of moderate cough. Cultures of the bronchoalveolar lavage fluid confirmed the diagnosis of pulmonary abscess caused by A gravenitzii. At the three-month follow-up consultation and, after six weeks of high-dose amoxicillin, the pulmonary lesion had completely disappeared.     

Arachnoid cyst associated with pyloric stenosis in a young boy

An unusual association between an arachnoid cyst and a decompensated pyloric stenosis in a three years-old boy is presented. The little patient was admitted into hospital with haematemesis, melena, influenced generally condition and acute posthemorrhagic anaemia following aspirin intake for hypertermia. Specific intensive care was successful and the little patient was discharged but without an upper digestive endoscopy(parents refusal, technical reasons). After one week he returned with progressive worsening vomitings and an intracranial hypertension was suspected. CT documented an arachnoid cyst in the right middle cranial fossa and the patient is directed to the Neurosurgical Clinic where a cyst fenestration was done. Subsequent to operation the vomitings reinstaled with severe dehydration and an upper GI series showed a decompensated pyloric stenosis. He was operated on underwenting an antrectomy. Finally the child recovered with good short and long-term evolution. The coincidental presence of an intracranial congenital mass and a complicated aspirin-induced peptic ulcer in this young patient, misleaded us and in the lack of an early endoscopy an intempestive neurosurgical operation was initially done.     

Dopamine dysregulation syndrome in a patient with restless legs syndrome

A middle-aged architect, suffering from familial, idiopathic, levodopa-responsive restless legs syndrome (RLS) progressively abused levodopa for 10 years, to the point of taking 20 tablets/day (4000 mg/day) for 6 months. Such abuse (possibly linked to the rewarding effect of dopamine) has been recently monitored in the context of Parkinson’s disease (PD) (the “dopamine dysregulation syndrome”). Physicians who now routinely treat RLS patients with dopaminergic agents should be aware of this abuse potential. This case also constitutes an experimental model. As levodopa abuse has only been described in patients with PD, it was suspected to be promoted by central dopamine depletion (with consequent sensitization of dopamine receptors). This idea should be revised because functional imaging showed that this RLS patient had no dopamine depletion. Later, he had no impulse disorders (no gambling, hypersexuality, excessive shopping), which occur with dopamine agonists, suggesting that levodopa abuse and impulse disorders can result through different mechanisms.     

Distribution of actin filament lengths and their orientation measured by gel electrophoresis in capillaries

Summary F-actin was electrophoresed in capillary tubes filled with agarose gel. The use of capillary imparted high resistance on the gel allowing the use of high enough concentration of salts to keep F-actin polymerized, and allowed the application of high electric fields without liberating considerable amount of heat. The intensity profile of the electrophoretic band of F-actin showed a peak, which in 1% agarose in the electric field of 17.8 V cm^–1 at 0° C, migrated at 3.4 cm hr^–1. Microscopic observation of actin filaments extracted from different positions along the gel showed that during electrophoresis filaments distributed themselves in such a manner that the longest polymers migrated slowest and the shortest migrated fastest. Using this observation we calculated the weight and number distributions of filament lengths from corresponding experimental intensity profiles. Phalloidin-labelled F-actin oriented in the gel upon application of an electric field. F-actin showed unusual orientational response: it oriented rapidly when the field was applied, but relaxed very slowly when the field was removed. Orientation of F-actin varied within an electrophoretic band, longest polymers showing the best orientation and short oligomers and monomers not orienting at all. The degree of orientation increased with the size of the electric field. When F-actin was labelled with phalloidin before electrophoresis, it was no longer able to migrate in the gel, but the electric field oriented it in the same way as when it was labelled after the electrophoresis. These results show that the electrophoresis of F-actin in agarose fractionates it according to its length, that by using electrophoresis it is possible to rapidly obtain distribution of filament lengths, and that F-actin migrates in agarose by the process of reptation.     

Acquired haemophilia complicated with gastrointestinal bleeding and spontaneous iliopsoas muscle haematoma in a woman with chronic C hepatitis under treatment with pegylated IFN alpha 2a and ribavirin

Acquired haemophilia A is a very rare (1-2 cases per million people) but often life-threatening haemorrhagic disorder characterized by antibodies directed against coagulation factor VIII. We report the case of a 55-year old woman under treatment with Pegylated alpha 2a interferon (IFN) and Ribavirin for chronic viral C hepatitis, who developed a progressive severe haemorrhagic syndrome diagnosed as acquired haemophilia based on supplementary laboratory data (prolonged activated partial thromboplastin time, extremely low factor VIII level - 1%, high titre of factor VIII inhibitor - 30 Bethesda U/ml).The onset was insidious, about three months before presenting to our unit. Antiviral therapy had been stopped three weeks before current admission. Emergency intensive treatment included: haemostatic agents - rFVII (Novoseven), FEIBA (Factor VIII Inhibitor Bypassing Activity), vitamin K, adrenostazin, cryoprecipitate, fresh frozen plasma, as well as immunosuppressive therapy (high dose corticotherapy and cyclophoshamide), immunoglobulins (Humaglobin), prophylactic PPI and antibiotics. The evolution was slowly favourable with the remission of the haemorrhagic syndrome and regression of the iliopsoas muscle haematoma. Clinicians should be aware that acquired forms of haemophilia do exist, representing a rare diagnosis and a therapeutic challenge. To our knowledge, this is the first reported case of acquired haemophilia in Romania, in a patient with chronic viral C hepatitis under antiviral treatment.     

Pierre Marie-Sainton cleidocranial dysplasia

The paper presents a family with hereditary transmitted Marie-Sainton dysplasia affecting the father and his both children. This is a rare syndrome presenting an autosomal pattern of inheritance, characterized by a generalized defect in both membranous and endochondral bone formation resulting in clavicular aplasia, delayed ossification of the fontanelles and the sutures of the skull and prolonged retention of deciduous dentition with delayed eruption of the permanent teeth. The diagnosis is suggested by more or less complete clinical picture and confirmed by multiple radiological explorations (skull, thorax, spinal column, pelvis) and genetical examination. The genetic mutation for cleidocranial dysplasia (CCD) is found on chromosome six and is called CBFA1 (short for core biding factor al or RUNX2) and is the only gene known to be associated with CCD. The normal version of CBFA1 acts to induce osteoblasts which are the type of cells that lay down bone. Although associated psychosocial disorders can occur, the prognosis and life expectancy of this condition are favorable being conditioned however by the complexity of orthodontic procedures which are determinant for these patients life quality.