Tuberculous meningitis in Bacille Calmette-Guerin-vaccinated children: clinical spectrum and outcome.

The Bacille Calmette-Guérin vaccination (BCG) contributed widely to reduce tuberculosis incidence in developing countries. The aim of this report was to assess the clinical "spectrum" and outcome of tuberculous meningitis in 16 Bacille Calmette-Guérin-vaccinated Tunisian children. They were 9 boys and 7 girls aged 2 to 168 months (median 72 months ± 65.88). Patients presented mainly with nonspecific symptoms. Neurologic severity was classified as grade I (n = 6) and grade II or III (n = 10). At short-term course, the majority of patients developed serious complications: hydrocephalus (n = 12), seizures (n = 8), tuberculoma (n = 6), and acute respiratory failure (n = 2). Three patients died. Among survivors, 4 patients showed a complete recovery while 9 developed permanent sequelae which were mild (n = 6) to severe (n = 3). Despite the Bacille Calmette-Guérin vaccination, tuberculous meningitis remains a life-threatening condition; vaccinated children have shown common presentation of tuberculous meningitis in terms of severity and poor outcome.

Bronchogenic cyst: an uncommon cause of congenital lobar emphysema.

We report a case of a 1-month-old boy who has developed respiratory distress. Chest X-ray and CT scan showed over distension of the left upper lobe and a mediastinal shift in favour of congenital lobar emphysema (CLE) of the left upper lobe. One month after uneventful lobectomy, he was readmitted at hospital for another episode of respiratory distress. Chest radiography revealed relapse of compressive emphysema in the remaining left lobe. Gastro oesophageal transit and MRI were performed, which have shown a mediastinal cystic mass. Accordingly, the patient underwent thoracotomy. Surgical examination found a subcarinal bronchogenic cyst which compressed the main left bronchus, causing the CLE of both upper and lower left lobes. Histological examination of removed cyst confirmed these data. Authors discuss causes of diagnostic delay.

Neonatal heart failure revealing a cardiac rhabdomyoma with tuberous sclerosis.

BACKGROUND: Cardiac rhabdomyoma is the most common cardiac tumour in childhood and is often associated to tuberous sclerosis (TS). However, a neonatal cardiac failure was uncommonly reported as the first manifestation of tuberous sclerosis. AIM: We report a case of a cardiac tumour revealed by a heart failure in a newborn who developed later clinical manifestations of TS. CASES REPORT: We report a case of a seven-day-old girl, admitted for cardiac failure. Echocardiography discovered a voluminous single mass in the right ventricle that affected partially heart output consistent with rhabdomyoma. Clinical course improved with symptomatic treatment. Seven months later, she developed generalized seizures associated to depigmental macules and facial neuro-fibroms. Cerebral magnetic resonance imaging showed cerebral hamartoms. Subsequent eight years follow-up revealed a spontaneous regression of cardiac rhabdomyoma and no further cardiac manifestations in contrast to a slight debility and a partial control of epilepsy. CONCLUSION: A cardiac rhabdomyoma discovered in neonates is a good indicator of TS suggesting careful followup and management

Congenital lobar emphysema. Report of 17 cases.

UNLABELLED: The aim of this report is to determine frequency and clinical characteristics of Congenital lobar emphysema (CLE) at Children's Hospital of Tunis. METHODS: Cases of CLE managed between January the 1st 1994 until December the 31st 2004 were reviewed. RESULTS: Amongst 31 cases of cystic pulmonary malformations we report 17 CLE. They were 12 males and 5 females. The mean age at diagnosis was 41/2 months (20 days, 22 months). Symptoms were: progressive respiratory distress (n=11) recurrent attacks of dyspnea (n=5); pulmonary infection (n=1). Chest X ray and CT scans showed hyper aeration of the affected lobes. Three patients had two affected lobes. CLE was associated to bronchogenic cyst (n=2) and to congenital cardiac anomalies (n=3). All patients underwent lobectomy. Post operative course was uneventful in 16 children. CONCLUSION: CLE is an uncommon cause of respiratory distress in neonates and infants. CLE is the most common cystic pulmonary malformation in our institution.

[Clinical characteristics and outcome of cystic fibrosis: report of 16 cases]. / Particularités cliniques et évolutives de la mucoviscidose. A propos de 16 observations.

AIM: The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis (CF). METHODS: Cases of CF managed at Infantile Medicine A Department in Children's Hospital of Tunis during 13 years (1994-2006) were reviewed. RESULTS: 16 children had CF. They were 8 males and 8 females. 13 patients were consanguineous and four had similar familial cases. The mean age at diagnosis was 19 months (10 days, 13 years). 3/4 of patients were symptomatic within the first trimester of life. Revealing symptoms were: obstructive bronchopathy associated to chronic diarrhea (n=6), edema-anemia-hypotrophy-hypoproteinemia syndrome (n=3), meconium ileus (n=4), bronchiectasis (n=2) and chronic diarrhea (n=1). The diagnosis was confirmed by sweat test and genotypic data. The F508 del was the most frequent mutation (54%). Clinical outcome was characterized by the occurrence of respiratory and nutritional complications: acute respiratory failure (n=6), chronic respiratory failure (n=3), chronic pseudomonas aeruginosa infection (n=6) at a medium age of 3.8 years, recurrent haemoptysis (n=2), pleural effusion (n=2), a malnutrition (n =10) and diabetes associated to puberty delay in one patient. Seven patients died at mean age of 4.4 years (6 months, 17.3 years). Among surviving patients, six had no compromised nutritional status or lung function. Prenatal diagnosis was performed in three families. CONCLUSION: CF is characterized by earliest onset and severity of symptoms. Therapeutic insufficiency is the main cause of precocious complications and poor prognosis in our series.

[Infancy asthma: diagnostic and therapeutic approach]. / L'asthme du nourrisson: approche diagnostique et therapeutique.

BACKGROUND: Asthma in infants is characterized by recurrence of three episodes of dyspnea and wheezing within two first years of age. Asthma in infancy is a heterogeneous condition with different clinical phenotypes and outcome. So, the diagnostic and therapeutic approach are often difficult. THE AIM of this systematic review was to provide answers to the following questions in the management of recurrent wheeze in infants: is it asthma?, should we treat wheezy infants?, what are the modalities of prescriptions? what is the long term outcome of wheezy infants? METHODS: Electronic literature search was performed in Medline. Key-words used for the final search were "infant", "wheezing", "atopy", "asthma", "inhaled corticosteroids". We considered for analysis meta analysis, randomized controlled trials, systematic reviews, cohort studies and consensus statement reports. RESULTS: The diagnosis of asthma in infants is more difficult than in older children or adults since many causes of eezing" may simulate an asthma associated to difficulties to evaluate bronchial hyper responsiveness in this age. There are three groups of wheezy infants: transient early wheezers, persistent non atopic wheezers and persistent atopic wheezers or asthmatic. Since recent data have proved early bronchial inflammation in wheezy infants, inhaled corticosteroids have become the main treatment. However, management of asthmatic infants is more difficult according to anatomic and functional respiratory peculiarities. There is no reliable clinical or biological indicators of future asthma. The evidence of atopy in infants with recurrent wheezing seems to be the main risk factor of the development of persistent asthman.