Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study

Background: Extracranial Germ cell tumors [GCT] are a rare and a heterogeneous group of pediatric cancers but highly curable

Aim: We aimed to review management, outcome and prognostic factors that influence overall survival [OS] in a pediatric Tunisian oncologic unit

Methods: We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit

Results: Patients had a mean age of 57 months [ranges: 1 day-13 years]. There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months [ranges: 0-96 months], OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage [p=0.04], the completeness of surgery [p<0.001] and the relapse [p = 0, 0001]. A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors [p=0,021] for 5-year OS

Conclusion: Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments

[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

[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

[Neonatal heart failure revealing a cardiac rhabdomyoma with tuberous sclerosis]

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. We report a case of a cardiac tumour revealed by a heart failure in a newborn who developed later clinical manifestations of TS. 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. A cardiac rhabdomyoma discovered in neonates is a good indicator of TS suggesting careful follow-up and management

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

[Infancy asthma: diagnostic and therapeutic approach]

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? 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. The diagnosis of asthma in infants is more difficult than in older children or adults since many causes of "wheezing" 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

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

[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