[Pediatric Crohn's disease in Tunisia]

Crohn's disease occur mainly in adults. However, pediatric onset forms are not rare and have many characteristics.To study clinical, diagnostic, therapeutic and evolutive characteristics of crohn's disease in tunisian children. Retrospective multicenter study conducted in 10 pediatric departments on a period of 10 years [2000-2008]. 43 children were included. The sex-ratio was 1.68. The mean age at the onset of the symptoms was 11 +/- 2.3 years [5-16 years]. The age of onset was inferior to 10 years in 25% of the children. The delay before management was superior to 1 year in 25% of cases. The initial symptoms were dominated by diarrhea [95%]. Perineal manifestations were present at diagnosis in 30% of children and extra-digestive manifestations in 53%. Ileocolonic localization was the most frequent [46%]. The initial disease flare was moderate in 83% of cases. The treatment was medical in 77% of cases, nutritional and medical in 18.5%. Maintenance therapy was instituted in 86% of cases and consisted essentiallay in azathioprin [62%]. The mean follow-up was 3 years and 4 months. 60% of the children had at least one acute flare. During evolution, 7% of children had anoperineal surgery and 11% an intestinal resection. Crohn's disease seems rare in Tunisia. The time of diagnosis is often delayed. The management is based on immunosuppressive therapy and nutritional support

[Mediastinal tuberculosis mass in a three-month-old boy]

Mediastinal mass of tuberculous origin is exceedingly rare in infant. To report an exceedingly rare case of mediastinal mall of tuberculous origin. We report a three-month-old boy who presented a one month history of wheezing a persistent pneumopathy. Radiological investigations showed a large posterior mediastinal mass which infiltrates lungs. Thoracoscopic biopsy showed caseous necrosis with granuloma suggestive of tuberculosis. The outcome was favourable with antituberculous chemotherapy. Mediatinal mass of tuberculous origin should be considered in differential diagnosis of mediastinal masses in children; be suggested in mediastinal mass in children

[Congenital cutaneous candidiasis associated with respiratory distress in a full-term newborn]

Congenital candidiasis is rare occurring in most cases in premature and low birth weight new born. It can produce a spectrum of disease ranging from a diffuse skin eruption to a severe spectrum of disease with or without skin involvement. Amphotericin B is the first-line agent for the treatment of systemic disease. To describe a congenital candidiasis in a full-term new born. At birth, the newborn had a generalized, erythematous, papulovesicular eruption. He was treated by topic antifungal therapy. However, on the third day, he developed a systemic disease with respiratory distress and fever. Ear, skin swab and gastric aspirate grew to Candida albicans. The new born was given fluconazole for 10 days with favourable outcome. Early topic therapy did not prevent systemic spread of congenital cutaneous candidiasis in our case. Treatment with fluconazole, as the first- line agent, seems effective and safety

Utility of lumbar puncture for febrile seizures among infants under one year old.

Febrile seizure is a frequent cause of hospitalization. Its management remains problematic According to the American Academy of Pediatrics, lumbar puncture, which is not devoid of risk, is strongly recommended in infants under 1 year. To define the utility of lumbar puncture in the management of febrile seizures in infants under 12 months and to identify risk factors of meningitis and to respond to this question: " lumbar puncure should be done in which infant with febrile seizure?" This retrospective study was conducted in the department of pediatrics B in the Children Hospital of Tunis on eight-year period [2000-2008]. In all statistical tests, significance level was set at 0.05. One hundred and six cases were collected during the period of study. Seizures related to bacterial meningitis were seen in 11 cases [10%]. We have separated two groups: GI infants presented bacterial meningitis, and G2 infants with febrile seizure. The comparison between the two groups G1 and G2 showed the following predictors factors of meningitis: age 5mm [p<0.001], recurrence of seizure in the same day[p=0,006], neurological abnormalities p<0.001], CRP>20 mg/1[p=0.03], hyponatremia

Marfan syndrome in a Triplo-X girl: a new association?

Triple X is a sex chromosomal abnormality that involves the presence of three sex chromosomes resulting in 47, XXX karyotype. Most patients suffering from this syndrome are usually mentally normal or subnormal with no gross malformation. To report an unusual association between Triple X and Marfan disease in a girl. A case of a triple X girl with craniofacial dysmorphy and skeletal anomalies, who did feat Marfan criteria by age, is presented. To the best of our knowledge this association has never been reported. Some clinical features are common between Triplo X and Marfan disease so a careful follow-up is needed and investigations should be performed in these patients because Marfan syndrome may be incomplete in early age

Unusual case of thiamine responsive megaloblastic anemia

Thiamine responsive megaloblastic anemia syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and neurosensoriel deafness, responding in varying degrees to thiamine treatment. Report an unusual case of this rare disorder. We report the case of a four-year-old boy who presented unusual features of thiamine- responsive megaloblastic anemia. In addition to the typical triad of the syndrome, he presented leuconeutropenia, hepatosplenomegalia, cardiac abnormalities including absent P waves, mitral and tricuspid insufficiency, retinitis pigmentosa, nystagmus, developmental delay and a brain Magnetic resonance imaging ischemic lesion. Lactate levels in serum and the lactate/ pyruvate ratio were increased. The mitochondrial mutation m.3243A > G located in MTTL1 gene encoding for transfer RNA leucine [tRNALeu[UUR]] was not found. Treatment with thiamine resulted in normalisation of the haemoglobin level, white cell count, and glucose and lactate levels. On three years follow up, the patient did not need insulinotherapy. These data sign the crucial role that thiamine plays for many cells and tissues and its importance in the activity of the respiratory chain

[ randomized, controlled trial of nebulized terbutaline in the first acute bronchiolitis in infant less than 12 months old]

Despite the common clinical practice, the available evidence on the efficacy of bronchodilators therapy for bronchiolitis is conflicting. The aim of this study is to evaluate the efficacy of nebulized terbutaline in bronchiolitis as measured by improvement in clinical score, oxygen saturation or reduction in duration of hospitalization. This prospective, double blind, placebo controlled, randomized clinical trial was performed at Children's Hospital of Tunis from December 2004 to April 2006. A total of 35 patients less than 12 months of age with diagnosis of moderately severe bronchiolitis were enrolled and assigned to receive nebulized terbutaline or normal saline placebo at admission [TO], at 30 minutes after admission [T30] and every four hours during a study period. Outcome measurements included: Respiratory Distress Assessment Instrument [RDAI] score, respiratory rate, oxygen saturation, heart rate and the duration of hospitalization. There were no significant difference between terbutaline and placebo at baseline, T30 min, T60 min, and T120 min after start study in RDAI score, oxygen saturation in room air, rate respiratory and heart rate. There was no difference in duration of hospitalization. Nebulized terbutaline therapy does not appear effective in treating moderately ill infants with the first acute bronchiolitis

Extra pulmonary tuberculosis in children: a study of 41 cases

Extrapulmonary tuberculosis accounts for up o one third of all cases of tuberculosis and children show a higher predisposition to the development of extra-pulmonary tuberculosis. To review the clinical features of the extrapulmonary tuberculosis in children. Forty one children with extrapulmonary tuberculosis followed in the Children Hospital of Tunis between January 1995 and December 2007 were reviewed. Extrapulmonary tuberculosis constitutes 57.9%of all cases of tuberculosis. Male to female ratio was 0.7 and the mean age was 75 years. The most commonly involved sites were the peripheral lymphadenitis [14 cases] followed by abdominal [11 cases], central nervous system [7 cases], osteoarticular [5 cases] and multifocal [4 cases]. A positive family history of active tuberculosis was detected in 22.5%of the cases. Diagnosis delay was 4.7 months. Sequelae observed during the follow up were: neurosensory in 5 cases, and vertebral deformation in 1 case. Extrapulmonary tuberculosis represents an important fraction of tuberculosis in our study. The most common form is lymph nodes localization followed by abdominal and central system nervous forms. Neurosensory sequelae were frequent in central system nervous tuberculosis

[Infectious diarrhoea in children under five years]

To determine the prevalence of rotavirus in acute diarrhea among children under 5 years and to describe the epidemiology, clinical trials of rotavirus diarrhea and compare them with those of other gastroenteritis. A Prospective study during 1 year [1 January 2007-31 December 2007] was conducted in the department of Pediatrics B of the Children's Hospital of Tunis. 117 children aged under than 5 years and admitted for acute community gastroenteritis were included in the study. A search of rotavirus in the stool, collected within 48 hours of admission, was performed by a rapid detection of viral antigens: proteins specific RV group A [VP6 protein of the capsid] using enzyme immunoassay technique: sandwich ELISA [IDEIA Rotavirus Dako] Rotavirus diarrhea accounted for 25%of acute gastroenteritis. The average age of rotavirus gastroenteritis was ii months +/- 1 .78. We have noted a male predominance with a sex ratio of 1.2. The seasonal distribution of rotavirus gastroenteritis showed three peaks of incidence [January, May and July]. The respiratory signs were more frequently found in rotavirus gastroenteritis group. Rotavirus gastroenteritis had a higher severity score compared with other gastroenteritis [8.44 vs 6.75 P=0.01]. The duration of hospitalization was longer in rotavirus group with a difference close to significance p=0.07. The seasonal distribution of rotavirus gastroenteritis has three peaks of incidence. The gastroenteritis Rotavirus affects very young infants and has more severe symptoms than other gastroenteritis and is significantly associated with respiratory signs

[Community acquired pneumonia in children]

Community acquired pneumonia is responsible for a high morbidity in children. The etiological diagnosis is not always easy and treatment remains probabilistic. To analysis clinical patterns and the outcome of community acquired pneumonia and to find arguments in favour of its origin. A prospective analysis of the files of 39 children [20 boys, 19 girls] hospitalized between [1 December 2004-30 June 2005] for community acquired pneumonia was performed. Included children whad between 6 months ad 15 years-old. The mean age was of 3 years and three months. Germ was identified in 41%of cases: Mycoplasma pneumoniae was the most important germ found in9 cases, pneumococcus was found in 4 cases and hemophilus in four cases. Coinfection pneumococcus and mycoplasma were found in two cases. None virus was identified. We have separated two groups: bacterial pneumonia and pneomoniae without definite etiology. Fat cough was associated to bacterial pneumonia mycoplasma pneomoniae was significantly associated with high fever and dry cough. We haven't found any others associations between clinical, biological or radiological patterns and the two groups of pneumonia. C-reactive protein more than 66 mg/I has sensitivity of 77%, specificity of 73.3%, positive predictive value of 46.7 99 of and negative predictive value of 91.7%. The outcome was favourable in all cases. Theses results showed the necessity to improve our microbiological methods to identify infectious agents of pneumonia. Mycoplasma pneumonia seems to be a frequent germ in preschool children

Infantile onest of cockayne syndrome without photosensitivity in a Tunisian Girl

Cockayne syndrome is a rare autosomal recessive disorder with dwarfism, mental retardation, and otherwise clinically heterogeneous features. Classically, the onset of Cockayne syndrome starts in the second year of life. The failure of RNA synthesis to recover to normal rates after UV-C irradiation provides a useful diagnostic test and the clinical feature that correlates most strongly with defective RNA synthesis is photosensitivity. To report an unusual case of Cockayne Syndrome. A case of a five-year-old girl with Cockayne with an onset in early infancy the girl and without photosensitivity is presented. The diagnosis was confirmed by the failure of RNA synthesis to recover to normal rate after UV-C irradiation. The patient died at the age of 6 of pneumonia. Although rare, Cockayne syndrome may be presented without photosensitivity and had an early onset

[Community-acquired pneumonia due to panton-valentine producing staphylococcus aureus: first description in Tunisia]

Community-acquired pneumonia clue to Panton-Valentine producing S. aureus is a serious infection recently described. Many cases have been reported worldwide. We report here the first case in Tunisia. Our patient is a previously healthy fourteen-year-old girl hospitalized for bilateral hypoxemic pneumonia. The clinical course had violently deteriorated two hours later, marked by massive hemoptysis that lead to rapid degradation of her hemodynamic state and death. Toxicologic research and blood cultures were negatives. A post- mortem pleural specimen culture yielded a meticillin-resistant Staphylococcus aureus strain that carried the Panton-Valentine leucocidin genes. Community-acquired pneumonia due to Panton-Valentine producing Staphylococcus aureus is a serious affection unrecognized in our country. Thus, this pathogen must imperatively be included in the spectrum of those responsibles for pulmonary infections in children and young adults

[Fanconi anemia complicated by neutropenic enterocolitis]

Neutropenic enterocolitis [NEC] also known as typhlitis is an acute, life-threatening inflammation of the small and large bowel often seen in patients with leukaemia undergoing cytotoxic chemotherapy; occasionally this syndrome could present in other immunocompromised patients. To report an unusual case of fanconi anemia complicated by neutropenic enterocolitis. We report a case of a 13-year-old boy affected by Fanconi anemia with severe neutropenia and who presented Neutropenic enterocolitis. Abdominal ultrasonography showed thickening of cecum and ascending colon of 1.18cm. The outcome was favourable with medical management. Neutropenic enterocolitis in Fanconi anemia is a rare and a severe illness; however the outcome is improved with early management

[Neonatal meningitis by Neisseria meningitidis B]

Neisseria meningitidis is one of the major causes of meningitis in children and adolescents, but it is uncommonly found in neonatal meningitis. To report a rare case of meningitis by Neisseria meningitides B. We report the case of neonatal meningitis in a 20 day-old girl without shock or purpura. The symptoms were fever and seizures. The culture of cerebrospinal fluid showed to be positive for Neisseria meningitidis B. culture blood was negative. Antibiotic therapy was started at admission and maintained for 3 weeks. The outcome was favourable without neurological sequelae. Early diagnosis and treatment are mandatory for life saving

[Cholestasis in infant: a study of the children's hospital of Tunis]

Cholestasis in infant constitutes an heterogeneous group of disease; diagnosis and management are often difficult. The Aim of the study is to describe clinical, paraclinical characteristics and outcome of infants hospitalized for cholestasis in children's Hospital of Tunis. A retrospective study of 94 infants with cholestasis was conducted. Patients were hospitalized in four departments of paediatrics of our hospital between January 1995 and December 2005. Cholestasis complicating severe sepsis and visceral leishmaniasis were excluded. Incidence of cholestasis was 8.5 cases/year which represented 0.72% of the hospitalizations. Sex ratio was 1.08 and mean age at diagnosis was 105 days [extremes: 1 day- 24 months]. Biliary atresia was the most common cause of extra hepatic cholestasis [13.8%]. Normal A GT cholestasis [11.7%], benign neonatal cholestasis [11.7%] and bile duct hypoplasia [9.5%] represented the most common aetiologies of intra hepatic cholestasis. Aetiology remained unknown in 12.7% of cases. Only three infants with biliary atresia had Kasai operation. After a mean follow-up of 6 years, 18% of patients had portal hypertension, 14.8% had hepatic failure and mortality rate was 14.8%. Cholestasis of unknown aetiologies are frequent in our hospital. Poor prognosis, in our study, is due to delay to diagnosis and difficulties in medical and surgical management

[Congenital hepatic fibrosis in children: about 9 cases and review of the literature]

Congenital hepatic fibrosis is a recessive autosomic disease with two major risks: gastrointestinal haemorrhage caused by portal hypertension and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. The aim of our study is to define epidemiological features, the presenting symptoms, the diagnosis, the evolution and the management of this disease. Between January 1990 and December 2000, we reported the cases of nine children with this disease at children hospital of Tunis.Three were male and six female. The mild age was three years and six months. Consanguinity was present in five cases and similar cases were found in six cases. The FHC was revealed by portal hypertension in five cases, angiocholitis in one case and by portal hypertension and angiocholitis in three cases. Liver biopsy was done in seven children. Ultrasound examination of the liver and kidney revealed caroli syndrome in five cases and polykystose renal in two cases The intravenous pyelography was performed in four cases showing precalicial canalicular ectasia in four cases. Eosophageal endoscopy had shown oesophageal varices in six patients. The follow up had shown that three patients had gastrointestinal bleeding, three had angiocholitis. One patient died with multivisceral failure. The treatment of acute bleeding has needed blood transfusion in four cases. Primary prevention of bleeding was done by endoscopic sclerosis alone in one case and associated to betablokers in two cases. Secondary prevention of varices bleeding was done by sclerotherapic in two cases, by beta blokers alone in one case and by betablokers associated to elastic ligation of oesophageal varices in one case

kyste hydatique du poumon chez l'enfant a propos de 54 cas

The purpose of this retrospective study is to clarify the clinical, radiological and evolutionary aspects of the hydatid cyst of the lung. Over a period of 9 years [January, 1983 - December, 2001], we brought together in the pediatric service B of Children's hospital of Tunis, 54 cases of hydatid cyst of the lung. The average age is of 7 years 3 months [extremes 2 and 14 years], and the sex- ratio of 1, 16. 61% of the children are of rural origin. Bronchpulmonary infection is the most frequent circumstance of discovery [72% of cases], hemoptysis is revealing in 37% of cases. Discovery is fortuitous in 7% of cases. The radiography of the chest is of a big diagnostic contribution. The radiological aspects obtained are: a homogeneous opaqueness [61%], a diverse opaqueness [26%], an image of lung abscess [22%], an aspect of floating membrane [4%], a pleural effusion [9%], and an opaqueness with growing gas [4%]. Chest echography was contributory in 77% of cases. 74% of the children were operated without complications. The others were lost

Premiere crise convulsive de l'enfant. Strategie d'exploration

We have curried out a retrospective study on 51 children aged between 2 years 3 month and 13 years in order to determine the findings and to define the indications of neuroimaging modalities in children with a first unprovoked seizure. Children who had neuroimaging studies were divided into two groups based on the results of neuroimaging normal or abnormal. We have compared the two groups according to each clinical and electroencephalographic parameters studied. Neuroimaging abnormalitie were found in 47% of cases. According to our results and literature review, we concluded that the imaging should be done if we have at least one of the criteria: an abnormal neurological examination after the seizure and focal slowing waves on the electroencephalogram