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.     

Value of reagent strips in screening urinary infection in children

The aim of this study is to specify the role of rapid tests in the screening of childhood urinary tract infection. During the period between july to december 1998, 572 urinary samples were collected from pediatric out-patient in H?pital d'Enfants de Tunis and aged from 1 month to 15 years. Only 75 samples (12.5%) were culture positive. The predictive value of leucocytes or nitrites test was 97.2%. These results allowed the use of rapid test in the screening of urinary tract infection in children. However, if clinical symptoms are present, the culture of urine must be associated to the rapid test.     

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.     

Bronchopulmonary aspergillosis: study of 17 cases

Aspergillosis is a fungic infection depending on the local or general physiologic and immunologic state of the host. We report the result of retrospective five year study (1995-1999) about 17 cases in the laboratory of Parasitology-Mycology of Rabta hospital in Tunis. Six aspergillomas were observed, they occurred after a pulmonary tuberculosis, two cases of allergic broncho-pulmonary aspergillosis described in two asthmatic patients, nine cases of invasive pulmonary aspergillosis complicating two cancers, one leukaemia, six chronic granulomatous disease. Aspergillus fumigatus is the most frequent species (67%). The clinical and biological characteristic of those will be studied, and compared with those of the literature.     

Cerebral venous thrombosis and Behcet's disease

Behcet's disease is a chronic relapsing multisystem disorder of unknown etiology. Neurological complications are frequent, occurring in 10 to 49% of cases. We report 4 cases with Behcet's disease (3 females and 1 male) who had symptomatic intracranial hypertension due to cerebral venous sinus thrombosis within a mean delay of 2 years. The mean age at onset was 31 years and the mean age on referral was 39.5 years. The predominant manifestation in our series were headache, papilledema, seizures and pyramidal syndromes. CT Scan showed non specific abnormalities in all of them and the sinus venous thrombosis was confirmed by MRI in 3 cases. The authors emphasize on the importance of MRI with angio MRI for the diagnosis, the outcome and the evaluation of the cerebral venous thrombosis after 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.     

Radiologically guided lumbar injections

Lumbar injections of corticosteroids are an established part of sciatica nonsurgical treatment; that's to their anti-inflammatory properties. Fluoroscopically monitored injections are more likely to place medication at the exact target site and with higher concentration; then they maximize therapeutic results. Lumbar steroid injections are efficient at short and middle term, and they precipitate relief.     

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.     

Abdomino-pelvic actinomycosis: a case report

The actinomycosis is a chronic suppurative granulomatosis disease. It is owed to a bacillus gram positive; actinomycès israelli. The cervical and thoracic localizations are most frequent. The digestive localization represents 20% of cases. It interest very rarely the pelvis and the genital tracts. We bring back the observation of a patient old of 30 years admitted for mass abdominal. To the exam, the patient had a sensibility of the left hypochondriac area and we found a mass of 6 cm of diameter. To the rectal touch, we found a mass in the bag of Douglas. The echography and the computed tomography revealed a collection under the spleen and a pelvic collection. A rectotomy is performed. The bacteriological study isolates actinomycès israelli. The collection under the spleen is drained under radiological control. Actinomycès israelli is also recovered in the pus brought back by the puncture. The patient is treated by Penicillin. The patient had a favourable evolution. No etiology is found at this patient. For this observation, the collection was accessible to a drainage permitting the diagnosis and the treatment of the actinomycosis while avoiding a mutilated surgery.