[ anomalies des arcs aortiques etude de 7 cas]

Anomalies of the aortic arch and the great vessels emerging from the heart are rare. We report here seven cases: double aortic arch [three cases], compressive arterial brachiocephalic trunk [three cases] and retroesophageal right subclavian artery [one case]. The anomalies of the aortic arch were associated to cleft palate in one case and to Klippel Feil syndrome in an other case; this association was never been reported to the best of our knowledge. Surgical treatment was indicated in five cases and was performed in two cases with favourable outcome. One patient died

[Pseudohypoaldosteronism type I: systemic form]

Pseudohypoaldosteronism type 1 [PHA1] is a rare condition characterized by renal insensitivity to the action of mineralocorticoids. Patients manifest neonatal salt wasting, hyperkalemia, and metabolic acidosis despite elevated aldosterone levels. Autosomal recessive and dominant or renal forms of the disease have been described. In the recessive form, patients have salt wasting from the kidney, colon, sweat, and salivary glands; because of dramatic volume depletion, patients require massive sodium supplementation throughout life. We report a case of a one month and three weeks old who presented a systemic type 1 pseudohypoaldosteronism

[Subdural empyemas complicating listeria monocytoenes meningitis in an immunocompetent infant]

Empyemas complicating Listeria monocytogenes meningitis is a rare event in immunocompetent children, beyond the neonatal period. We report a case of Listeria monocytogenes meningitis in a 15-month-old-girl. Magnetic resonance imaging showed subdural empyemas. Specific treatment was established with ampicillin for six weeks. The outcome was favourable

[Pneumococcal meningitis: clinical features and prognostic factors]

Despite advances in antibiotic therapy strategies and pediatric intensive care, prognosis of Streptococcus pneumoniae meningitis remains very poor. To determine epidemiological and clinical features of pneumococcal meningitis and the factors associated with hospital mortality and neurologic sequelae. We conducted a retrospective study of 21 cases of childhood pneumococcal meningitis admitted in the department B of the Children hospital of Tunis during a 9-year-period. Twenty one children were included in this study. The mean of age was 22 months. 10 children had presented seizures before admission and one was admitted in coma. The direct examination of the cerebrospinal fluid was positive in 85 per cent soluble antigens were positive in 66 per cent and the culture was positive in 100 per cent. Immediate complication were notified in 9 cases and neurosensorial sequelae in 6 cases. Pneumococcal meningitis is a serious disease with a high rate morbidity and mortality then, we suggest introducing pneumococcal vaccine

[Neonatal infection and streptococcus pneumonae meningitis]

Streprococccus pneumoniae is a major cause of morbidity and mortality in childhood infectious disease; however, it is unusual in the neonatal infection. We report the case of a newborn, admitted in our department to manage respiratory distress. One the clinical examination, he presented hypothermia, polypnea and hypotonia. The blood and cerebrospinal fluid cultures were positive for Streptococcus pneumoniae. Brain imaging showed a hydrocephalus. He received cefotaxim and vancomycin for 56 days

[Laryngotracheobronchial foreign body in children: predictive factors of respiratory sequelae]

Inhalation of laryngotracheobronchial foreign body in children is a serious accident that may compromise the prognosis of the child and the respiratory function in the long term. Aim: identify the predictive factors of respiratory sequelae of laryngotracheobronchial foreign body inhalation. This retrospective study was conducted in the children hospital of Tunis during a period of nine years [2000 - 2008]. In all statistical tests, the significance level was set at 0.05. 60 children were included in the study. The average age was 24.9 +/- 3.4 months. 2 / 3 of the children were boys. The foreign body was plant in 80% of cases. The penetration syndrome was reported in 83.7% of cases. The average time of stay of foreign body was 14 days. The chest radiograph was abnormal in 77.4% of cases. Endoscopic extraction was performed in 59 cases and a pneumectomy was conducted in one child. 30 children were followed for an average of 23 months. 18 children had not respiratory sequelae [clinical, radiological and scintigraphic]. 10 children had respiratory sequelae [clinical and radiological and/or scintigraphic] at the last follow-up and four patients developed bronchial dilatation. Respiratory sequelae were correlated with the stay period of the foreign body exceeding 84H. Neither age, nor sex, nor the nature of foreign body or its location, nor the presence of radiological opacity at the initial radiograph, were predictive factors of respiratory sequelae. The inhalation of foreign body is a serious accident affecting essentially male infants. Clinical, radiological and scintigraphic follow up is mandatory

[Pleural effusion with pneumonia caused by Neisseria meningitidis C: a case report]

Pleural effusion with pneumonia is an uncommon manifestation of Neisseria meningitidis infection. We report the case of a 3-year-old child who presented a one week fever and abdominal pain. Chest X ray showed pleural effusion with lung condensation. The outcome was favourable with antibiotics

Neonatal cholestasis as the presenting feature of cystic fibrosis

Neonatal cholestasis as the presenting feature of cystic fibrosis is rare. We reported a 2 and half months infant who presented neonatal cholestasis since 27 days of life. The punch biopsy of the liver showed signs of cholestasis. Serological tests revealed CMV infection; sweat test showed elevated chloride and confirm the diagnosis of cystic fibrosis. This patient developed a pulmonary pseudomonas infection. The outcome was fatal. Several etiologies may be involved in neonatal cholestasis and the presence of one does not preclude the other

A severe illness of a congenital cytomegalovirus infection

Congenital cytomegalovirus [CMV] infection occurs in about 1 per cent of newborns and 10 per cent of them exhibit symptomatic infection. Nervous system damage influences prognosis and newborns management. We report the case of a newborn with a s6vere and progressive congenital CMV infection. He was admitted because of petechiae and thrombocytopenia. Investigations of the newborn showed a bilateral optic atrophy, severe nervous system damage with hydrocephalus, enormous periventricular pseudocysts and cerebellum hypoplasia. No brain calcifications were found. Diagnosis relied on blood CMV viral load measurement in both the newborn and his mother. The newborn received Ganciclovir. Hydrocephalus progressed and required ventriculoperitoneal shunt placement. This case illustrates that pseudocysts and progressive hydrocephalus must suggest CMV congenital infection even not associated to brain calcifications. Nervous system damage is serious in congenital CMV infection and requires a systematic screening during pregnancy

[Bardet - biedl syndrome - a rare cause of end - stage renal failure]

Bardet - Biedl syndrome is an autosomal recessive condition caracterised by retinitis pigmentosa, polydactylye, obesity, mental retardation, hypogonadism, an renal dysfonction. We report an observation of 14 year old boy wich present an end - stage renal disease

[Kawasaki disease. About 9 cases]

To analyze epidemiologic, clinical and evolutive profile of Kawasaki disease, we studied retrospectively 9 cases of children with Kawasaki disease fulfilled during ten years [1994 - 2003]. It is about 8 boys and 1 girl, aged from 4 months to 9 years [mean age at 3 years].5 patients fulfilled at least 5 major criterias of The illness and the four others presented an incomplete form We noted cardiac manifestations in 3 patients, neuromeningeal, renal and digestive symptoms in 5 cases for each one, articular in 2 cases and pulmonary in 1 case. Biologic inflammatory syndrome is present in all cases and high rate of platelets has been found in 6 cases/9. Aspirin with anti inflammatory dose has been instituted in 8 cases / 9. Intravenous immunoglobulin has been administrated in 6 cases /9. Cardiac manifestations have disappeared completely in the 3 cases. The outcome has been favorable for all cases. In conclusion, Kawasaki disease is unknown rather rare, incomplete or atypic forms are frequents and source of diagnostic delay. Early treatment permit to improve the prognosis of this disease