Bullous pemphigoid in children. Report of three cases

Bullous pemphigo?d (BP) is an acquired immunobullous disease that usually affects adults and rarely children. About 60 cases of infant PB have been reported. Diagnosis is based on immunofluorescence investigations. Clinical, histological and immunopathological findings in childhood PB appear to be not different from the adult. Nevertheless, oral mucosal and palm and sole lesions seem to occur more frequently. We report 3 cases of children BP in one girl and 2 boys, aged respectively of 7.2 and 3 years. Palm and sole were affected in 1 case, and no oral mucosal lesion was noted. Recovery was reached in all cases under dapsone. Characteristics of BP in children are discussed with comparison to literature data.     

Congenital diaphragmatic hernias in the adult: report of six cases

The anterior hernia or the fissure of Larrey and Bochdalek hernia are exceptional in adult. We present 6 cases of congenital diaphragmatic hernia revealed in adult. Three hernia of the fissure of Larrey and three Bochdalek hernia. We discuss the different clinical, radiological, and therapeutic aspects of these hernia. We insist on the gravity of strangulated hernia and their surgical treatment.     

Dubin-Johnson syndrome: molecular basis and pathogenesis

The Dubin-Johnson syndrome (DJS) is an autosomal recessive liver disorder characterized by a chronic conjugated hyperbilirubinemia a dark greenish appearance of liver tissue, a double peaked sulfobromophthalein clearance curve, and a characteristic lysosomal accumulation of black pigment "melanine-like" in the hepatocytes. Laboratory datas indicated an increased urinary excretion of coproporphrin isomer I and leukotriene metabolites. In an effort to understand the morphological pattern and the pathogenesis of this disease we reviewed four cases of DJS.     

Malignant hyperthermia

Malignant hyperthermia is a potentially fatal pharmacogenetic disease triggered by volatile anesthetics and/or succinylcholine. Dysregulation of intracellular calcium homeostasis is the trigger of the acute crisis. Malignant hyperthermia crisis correspond to an hypermetabolic state, which occurred acutely and interesting skeletal muscular cell. Early manifestations grouped tachycardia, tachypnea, masseter spasm, mixed acidosis and raise of the end expiratory CO2 pressure. Hyperthermia is a late sign, rhabdomyolysis is a sign of the severity of the malignant hyperthermia. The successful treatment is based on an early diagnosis, immediately interruption of triggering agents, intravenous administration of Dantrolene in sufficient dosage and starting of adequate symptomatic treatment. Prevention of this complication is based on asking the patient about genetic predisposition to malignant hyperthermia. Confirmation of the susceptibility to malignant hyperthermia can be provided by in vitro contracture test with halothane or caffeine after muscle biopsy.     

Hypertrophic cardiomyopathy: a rare cause of vascular dementia. A case report

Herein, we report a case of a 51 year old man who experienced three ischemic cerebral infarcts in a time of few months. The patient consulted after the third accident. Neurological presentation included pseudobulbar syndrome with a mild cognitive deficit, aphasia, left hemiparesia, hemiasomatognosia and homonymous lateral hemianopsia. Cerebral tomodensitometry and magnetic resonance imaging evidenced large infarcts images involving right middle cerebral artery territory and bilateral borderline zones in the junction of the territories of the middle and posterior cerebral arteries. Ambulatory 24 hours ECG recording (Holter) revealed two hits of non-sustained ventricular tachycardia. Transoesophageal echocardiography conveyed to the diagnosis of hypertrophic cardiomyopathy and displayed the presence of a left auricular thrombus. Anticoagulant therapy and rehabilitation allowed a substantial recovering of the patient's cognitive functions and wasting of the intracardiac thrombus. The clinical features observed in our patient meet the recommended DSM IV diagnosis criteria of vascular dementia, an exceptional complication of HCM. The clinical findings, neuroimagery investigation results, and the chronological link between cerebral attacks and cognitive function deterioration argue for a demential syndrome of vascular origin resulting from multiple embolic infarcts involving medium sized arteries (multi-infarct dementia). The authors emphasize the rarity of such observation. HCM must be considered as a potential cause of embolic stroke and likewise a multi-infarct dementia.     

Tunisian health profile: present state and trends

We will try to value the Tunisian Health Profile, its present state and its tendencies. Data have been collected by various sources, in particular international and national organism. The total fertility rate passed from 8 in 1966 to 2.01 in 1999. Besides a light ageing of the population is started (9% of the population are aged of more than 60 years in 1999). Death rates recorded a decrease, mainly of the infantile mortality (140%@1000 in 1966 against 24.7%@1000 in 1999) and the maternal mortality (68.9 per 100,000 living births in 1994 against 220 in 1980-1984). The life expectancy at birth passed from 51 years in 1966 to 72.4 years in 1999. According the morbidity we attend the receding of the majority of transmitted diseases. Resources injected in the system of cares also evolved: The medical density passed from 1 per 6700 inhabitants in 1966 to 1 per 1300 in 1999. Tunisia recorded the most elevated scores for the preventive activities in particular at the vaccinal coverage. The global evolution of the Tunisian health profile is marked by the epidemiological transition buy which pass the country currently. This phase is delicate because it's generating of a growth of care expenses. Decision-makers must be careful to consider advantage priorities and the profitability of the investments.     

Interpretation of hypertransaminasemia

Raised serum level of transaminases aspartate (aminotransferase and alanine aminotransferase) is a frequent situation in medical practice. It is considered as moderate when the level is under 10 times normal and as chronic when it lasts for more than 6 months. The most common etiologies for chronically elevated transaminases are alcohol use, viral hepatitis, liver steatosis, diabetes, obesity and medications. Many non invasive tests, including history, physical examination, blood tests (markers for hepatitis A and B, muscular enzymes), and imaging procedures (abdominal ultrasonography) are usually done and lead to a correct diagnosis in 80% of patients. When the diagnosis cannot be determined non invasively a liver biopsy is recommended in order to make diagnosis, to evaluate the prognosis and to start an adapted treatment.     

Clinical evaluation of acetate-free biofiltration at 84 0/00 in patients with chronic renal insufficiency

We report in this study the outcome of chronically hemodialysed patients with a new technique: Acetate free biofiltration 84@1000. This study is divided into two steps: during the first period we studied the hemodynamic stability of the technique in patients hemodynamically unstable under bicarbonate hemodialysis. During the second period we compared the hemodynamic effects and the quality of dialysis in these patients with other patients under bicarbonate hemodialysis. We obtained less hypotensive episodes, a better correction of acidosis, a better tolerance of the technique and comfort of the patient with the new technique, the quality of dialysis was the same with both techniques. We recommend the use of acetate free biofiltration 84@1000 whenever the patients are hemodynamicallyunstable with bicarbonate hemodialysis.     

Quality monitoring of composting processes of wastewater sludge in Alexandria

There are two wastewater primary treatment plants in Alexandria (west and east). The produced primary sludge is mechanically dewatered and transported to sludge disposal site 9N where composting is carried out. However, prior to 1970, composting played a very minor role in sludge or solid wastes treatment because of greatly unfavorable balance between its economics and those of the principal competing option, namely landfill disposal. This study aims at monitoring and evaluating the composting process of demitted sludge produced from Alexandria wastewater treatment plants. Ten batches of sludge were composted. During the composting process the batches were been investigated and followed up to 3 months. Representative samples (10 for each batch) were taken from these batches at the start of windrowing and after each turning (4-15 days) and were analyzed for physical; chemical; bacteriological; and parasitological characteristics, heavy metals, and plant nutrients. Results revealed that C/N ratio of the final compost product comply with the decree of the Minister of Agriculture No. 100 11967, while moisture, C%, and TKN did not. Heavy metals, faecal coliforms, and helminthes complied with the decree No. 222/2002 for the Minister of Housing, Utilities, and Urban Communities. Moisture had positive correlation with both C and VS and negative correlation with pH. Temperature had negative correlation with TKN. Both total and faecal coliforms had negative correlation with temperature and positive correlation with C, N, and VS. It is recommended to optimize the quality of the produced sludge compost by use bulking agent rich in carbon and nitrogen as Hay or Rice straw, instead of the matured sludge compost.