[Tuberculosis in childhood: clinical features and problems in diagnosis. report of 30 cases]

The clinical polymorphism and the low yield bacteriological tests make the diagnosis of tuberculosis [TBC] in children often difficult. The aim of this report is to specify hospital incidence of childhood TBC and to discuss problems in diagnosis. We reviewed retrospectively cases of TBC enrolled at Medicine A Department in Children's Hospital of Tunis during the last ten years [1998 - 2007]. Diagnosis of TB was supported according to bacteriological or histological confirmation or regarding the association of epidemiological data [TB contagium], clinical and radiological findings and favourable outcome with anti tuberculous drugs. Thirty children had TBC. They were 18 girls and 12 boys. The main age at diagnosis was 8. 6 years [3 months-14 years]. All children were vaccinated with BCG. Thirteen patients had definite familial history of TBC contact. Tuberculin-skin test was positive in 15 patients. The diagnosis was supported within a mean period of 44 days [8, 240 days]. Pulmonary TBC occurred in five patients and extra-pulmonary TBC in 25. Four patients had more than two TBC localizations. Miliary and TBC meningitis occurred in seven patients. The rate of diagnosis confirmation was 40%. Clinical outcome improved in 29 children with anti tuberculosis therapy while one infant died with miliary TBC. Five patients developed pleural, neurological or bone sequelaes and another patient presented autoimmune bicytopenia, diffuse bronchectasis and pulmonary aspergillosis. TBC occurs in 0, 91/ year/1000 hospitalized children in our institution. Low diagnosis confirmation rate was observed with infants and in pleural and primary TBC. Although all patients received BCG vaccine, 23. 3% of them developed a life-threatening form of TBC

Clinical characteristics and outcome of cystic fibrosis: report of 16 cases

The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis [CF]. Cases of CF managed at Infantile Medecine A Department in Children's Hospital of Tunis during 13 years [1994 -2006] were reviewed. 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], oedema-anemia-hypotrophy-hypoprotidemia syndrome [n = 3], meconium ileus [n = 4], bronchiectasis [n = 2] and chronic diarrhea [n = l]. 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. 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

[Congenital lobar emphysema: report of 17 cases]

The aim of this report is to determine frequency and clinical characteristics of Congenital lobar emphysema [CLE] at Children's Hospital of Tunis Cases of CLE managed between January the 1st 1994 until December the 31 St 2004 were reviewed. 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. CLE is an uncommon cause of respiratory distress in neonates and infants. CLE is the most common cystic pulmonary malformation in our institution

11-Beta Hydroxylase Deficiency: a clinical study about seven cases

11 beta-hydroxylase deficiency is a rare recessive autosomal disorder. The aim of this report was to describe among a retrospective study of seven cases, different clinical pictures, problems in diagnosis and management. The frequency of 11 beta-hydroxylase deficiency was 17.5% of congenital adrenal hypererplasia etiology in our study. Consanguinity was found in all cases. The sex ratio was 5 boys/2 girls. Median age on diagnosis was 4.3 years. Five cases were revealed with precocious puberty associated with hypertension. One patient had sexual ambiguity, Prader IV stage, hypertension appears later. One patient developed heterosexual precocious puberty and hypertension at five years of age. One patient had bilateral testicular adrenal rests. Hypertension was diagnosed early in 4 cases and secondarly in the other 3 cases. Hypertension was severe complicated by convulsions, facial paralysis and epistaxis. Hypo kaliemia was identified in six cases. Hormonal investigations confirmed diagnosis in all cases. The secondary sexual characteristics were controled by glucocorticoid substitution. Antihypertensive treatment was necessary initially and prolonged only in three cases Prognosis of final height of patients with late diagnosis was particularly compromised

[Sensibilization to pollen in asthmatic child. A study of 86 cases]

This retrospective study was designed to determine the place of pollen sensibilization in asthmatic child and to assess its epidemiological and clinical characteristics. 86 from 638 asthmatic child were allergic to pollen and included in the study. The mean age was 4 years [extremes: 1-12 years]. The mean age of first respiratory manifestations was 1 and half years. The male/ female ratio was 1.46. Asthmatic attacks was noted in 77% of cases, chronic cough was the only allergic manifestation in 16% of cases. Associated rhinitis or conjunctivitis were found in 55% of cases , atopic dermatitis was found in 4 patients [4,6% of cases]. Atopic asthma was per annual in 78% of cases. Pollinic asthma was diagnosed in 51 child. Other sensibilization were found in 60% of cases. 26 child [32%] were allergic to pollen only and 18 child to sample pollen. The gramineae pollen sensibilization is the most commun allergy. followed by olive tree and cypress tree and herbaceous pollens

[Prevalence and severity of asthma, allergic rhinoconjunctivitis and atopic eczema in "Grand Tunis" schoolchildren. ISAAC]

The aim of this study was to assess the prevalence and severity of asthma, allergic rhinoconjunctivitis and atopic eczema in 13 - 14 year old children living in "Grand Tunis", Using the international study of asthma and allergies in childhood [ISAAC] questionnaire, 3350 schoolchildren aged 13-14 years, from the Grand Tunis [Ariana, Ben Arous, Manouba, Tunis] were studied. Our results showed that in the past year 13.2% of children had wheezed, 1.4% had more than 12 attacks and 4.3% had experienced a speech limiting attack. 29.7% had symptoms of allergic rhinoconjunctivitis and 8.3% atopic eczema. The classic preponderance of asthma in boys has not been retrieved in our study. The highest level of wheeze was found in an agricultural area. According the published data, asthma prevalence in Tunisian schoolchildren is intermediate and allergic diseases are perhaps a common childhood diseases in Tunisia

Aspartylglucosaminuria in a Tunisian family

We report on the first 4 cases of aspartylglucosaminuria [AGU] diagnosed in Tunisia. Four siblings with the clinical and laboratory findings of AGU were the products of a first cousins' mating. The index case was a 20 month old male who presented with heart failure and coarse features. He had a slow psychomotor development and skeletal changes consistent with numerous changes in small bones. Enzymatic essays in cultured skin fibroblasts showed aspartylglucosaminidase deficiency. His 2 sisters and his brother were 11, 3 and 8 1/2 years of age, respectively and also presented a slow psychomotor development and dysmorphia