[Listeriosis in Tunis: seven cases reports]. / Les infections à Listeria monocytogenes à Tunis: à propos de sept cas.

Listeria monocytogenesis a Gram positive facultative intracellular bacterium that can be responsible for severe infections, affecting essentially pregnant women, immunocompromised patients at the early and later stages of life. In Tunisia, invasive L. monocytogenes infections are thought to be exceptional and limited data are available about listeriosis. We reported seven cases (five newborn children and two infants) of human listeriosis that occurred in Tunis from 2000 to 2008. The newborn children were hospitalized for suspicion of maternofoetal infections. The two infants were hospitalized for fever associated with digestive signs in one case and neurological signs in the other. L. monocytogenes-was isolated from culture of cerebrospinal fluid in four cases, peripheral samples in two cases and from blood culture in one case. Isolates identification was based on conventional methods. Antimicrobial susceptibility was realized according to the recommendation of the "Comité de l'antibiogramme de la Société française de microbiologie". All L. monocytogenes isolates were sensitive to amoxicillin and aminoside but resistant to 3rd generation cephalosporins. Investigations of the immune system were realized for the two infants including phenotypic analysis of peripheral blood cells by flow cytometry, lymphocyte proliferation assays, phagocytic cell functions and measurement of immunoglobulins as well as complement. All these explorations were normal for both infants. The outcome was fatal in only one case (a newborn child), and all the other patients recovered after adapted antibiotic treatment. In conclusion, our study shows that listeriosis is not exceptional in Tunis. Thus, it is necessary to know how to evoke this diagnosis, at any age, in order to establish an early and adapted antibiotic treatment and to avoid fatal outcome.

The Antley-Bixler syndrome. A new case without radiohumeral synostosis.

The author report a new case of the syndrome described by Antley and Bixler. It concerns a female new-born who presents a craniosynostosis with brachycephaly, midface hypoplasia, unilateral choanal atresia, multiple joint contractures and arachnodactyly. There was no bowing of the femora and only the radius and the ulna were bowed. The absence of radiohumeral synostosis reported in all observations of the literature represents the main particularity of this observation.

[Wolman disease]. / La maladie de Wolman.

BACKGROUND: Wolman disease is a severe disease associated with hepatosplenomegaly and adrenal calcifications; it is nearly always fatal in the first year of life. CASE REPORT: A boy was born to consanguineous parents. His weight was 3,500 g, height 53 cm. Hepatomegaly was observed at the age of 26 days; he also had vomiting and watery stools with failure to thrive. Diagnosis of Wolman disease was suspected due to family history. Two sisters had died at the age of 3 months without precise diagnosis; both had abdominal distension, hepatosplenomegaly, anemia and inanition; CT scan showed calcifications of adrenal glands in one of them that had been attributed to adrenal hemorrhage. Investigations in our patient showed no adrenal calcification, hepatomegaly without splenomegaly, anemia (Hb: 8 g/100 ml). Liver biopsy showed enlarged and vacuolated parenchymal and Kupffer cells but the marrow did not contain foam cells. Acid lipase deficiency was demonstrated in cultured skin fibroblasts, permitting prenatal diagnosis in a further sib. CONCLUSION: This case of Wolman disease was the first seen in Tunisia; it was inherited as an autosomal recessive disorder; this patient, as two of his sisters, died during the first 6 months of life.

[Bronchopulmonary dysplasia and corticosteroid therapy]. / Dysplasie broncho-pulmonaire et corticothérapie.

Inflammation plays a major role in the pathogenesis of bronchopulmonary dysplasia of preterm infants. Having a strong anti-inflammatory effect, corticosteroids have been proposed in the management of this disease. Antenatal steroids protect the newborn against its development. Postnatal systemic administration of steroids reduces the duration of mechanical ventilation and oxygen therapy, but is associated with short term and long term adverse effects. Early administration of dexamethasone (before 7 days of life) reduces the incidence and the severity of chronic lung disease at 28 days of life and 36 weeks of post-conceptional age. Inhaled steroids are associated with less adverse effects than dexamethasone administration, but they are also less effective.

[Pharmacologic study of monohydrated caffeine in the treatment of apnoea of premature infant]. / Etude pharmacologique de la caféine monohydratée dans le traitement des apnées du prématuré

UNLABELLED: Monohydrated caffeine was the only respiratory xanthine available in our country to treat apnea of premature infant. The aim of this study was to evaluate plasma levels of this molecule at dosages of 20 mg/kg (equal to 18 mg/kg of caffeine base) as a loading dose and 5 mg(-1) kg(-1) (equal to 4.5 mg(-1) kg(-1) of caffeine base) as a maintenance dose. PATIENTS AND METHODS: The study was prospective including premature infants less than 34 weeks of gestational age born between the 1st of july 2001 and 15th december 2001 and receiving monohydrated caffeine to prevent apnea. Each premature infant has received orally a loading dose of 20 mg/kg in the first hours of life followed, 24 h after, by a maintenance dose orally once a day of 5 mg/kg until 35 weeks of post-conceptional age. Caffeinemia plasma levels were measured by high performance liquid chromatography immediately before the second dose to determine the loading residual rate and immediately before the sixth dose to determine the maintenance residual rate. RESULTS: Twenty-one premature infants were included. Their medium term was 31.4 weeks (27.4-33.3 weeks), birth weight was 1684 g (1000-2800 g) and sex-ratio M/F was 1.3. Fifteen infants (71.4%) have presented apnea with an average of 4.1 episodes per infant and per day. Tolerance of the medicament was good in all cases. The medium loading residual rate was 3.26 microg/ml (1.75-7.80) and the medium maintenance residual rate was 4.26 microg/ml (2.13-7.64). CONCLUSION: Prescribed at a dosage close to twice the recommendations of the literature, monohydrated caffeine does not provide efficient plasma rates. This is probably due to a difference in its oral bio-availability compared with caffeine citrate and further study with greater dosages is needed to appreciate its efficacy.

[Diastematomyelia: antenatal diagnosis with successful outcome, two cases]. / Diastématomyélie: à propos de deux observations anténatales et revue de la littérature.

To discuss, through a review of the literature, clinical signification and antenatal and postnatal management of diastematomyelia. We present two cases highlighting the clinical presentation and discuss management options. The first case was diagnosed in a 32-year-old primagravida female physician. The conssanguinous couple had a history of primary hypofertility. Antenatal magnetic resonance imaging revealed the presence of a sagittal spinal spur separating two asymmetrical hemi cords. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Ultrasonography performed at 22 weeks gestation provided the diagnosis in the second case. The mother was a 30-year-old primagravida anesthesist who had been addressed for suspected spina bifida. The consanguinous couple had a history of male hypoferility. The ultrasound scan revealed an echogenic spinal spur and integrity of the skin. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Diastematomyelia is a rare malformation which can be diagnosed antenatally. Careful ultrasonography can distinguish diastematomyelia from myelomeningocele. Antenatal assessment is essential to identify forms with good prognosis because the neonatal outcome of isolated diastematomyelia is generally good, even if surgical repair is required.

Efficacy and safety of bromocriptine in the treatment of macroprolactinomas.

To assess the effectiveness of bromocriptine (BRC) as primary therapy in reducing the size of PRL-secreting macroadenomas with extra-sellar extension, we conducted a multicenter study in 29 patients without prior radiotherapy. Clinical presentation, response to medical treatment and long term follow-up of 29 patients with macroprolactinoma (pituitary mass more than 10 mm in diameter) were analysed. There were 19 women for 10 men. Mean age was 33 years. An hypogonadism was present in 94% of women vs 57% for men. Headaches and or visual abnormalities were present in 68% of women vs 90% of men. The mean basal serum prolactin level before treatment was 1 501 ng/ml: 202 ng/ml for women (range: 70-478 ng/ml) and 3 870 ng/ml for men (range: 100-20 476 ng/ml) and was correlated to tumoral size. 25 patients were treated with BRC as primary therapy with a mean dose of 9,7 mg/day (13 mg/day in men and 8 mg/day in women). BRC no malized serum PRL levels in all men and in 17/19 women over a mean period of 6 months, ranging from 3 to 72 months in both sexes. Tumor size was reduced by more than 50% in 18/29 patients (62%) with secondary empty sella in 5 patients and by less than 50% in 11 patients. Visual field improved in most of the patients in whom it was initially abnormal. Reduction in size was quite fast in most of patients but slower in some of them. The cumulative radiological response to BRC increased with time. Therefore, it appears that prolonged medical therapy is effective and safe in macroprolactinomas.

[Hereditary nature of nephroblastoma. Apropos of a case of 3 siblings]. / L'hérédité du néphroblastome. A propos d'une fratrie de trois membres atteints.

Three nephroblastomas, including one bilateral tumour, have been detected in three children belonging to the same family and born from a consanguinous marriage. The clinical and diagnostic particularities compared to non familial "sporadic" nephroblastomas are studied. The pathogenesis with the incidence of chromosomal anomalies, the diagnosis and the frequency of bilateral diseases along with the associated deformities and a non invasive therapeutic attitude are discussed.

[Bilateral neuroblastoma. Study of 5 cases and review of the literature]. / Néphroblastomes bilatéraux. Etude de cinq observations et revue de la littérature.

Five cases of bilateral nephroblastoma out of total of 99 cases have been observed during a period of 18 years at the Children's Hospital of Tunis (5%). The frequency of associated anomalies, the familial incidence, the young age of the patients are underlined. The main therapeutic modalities and prognosis are envisaged.

[Tracheal atresia. A case report]. / Atresie tracheale. A propos d'un cas.

The tracheal agenesis is a rare malformation of the respiratory tract. It must be suspected in any new born with respiratory distress, absence of crying, and difficulty or impossibility of intubation. Since the initial case report by Payne in 1900, 87 cases have been reported in the literature. The authors report one case of tracheal agenesis out of 2500 autopsy realised in the laboratory.

[Fetal pathology study of 88 cases of letal spina bifida]. / Etude foeto-pathologique de 88 cas de spina bifida létaux.

Lethal spina bifida continue to be frequent in Tunisia; we report 88 cases of letal spina bifida: 1.05 per thousand births. This pathology was more frequent with women. The up letal spina bifida situated is predominant with female and the dow spina bifida situated is frequent with male. We have noted an association with anencephalia (46 cases) and hydrocephaly (21 cases). Prevention is based on obstetric health care and hygiene dietetic advices to avoid alimentary deficit.

[Epidemiology of maternal-fetal group B streptococcal infections]. / Épidémiologie des infections materno-fœtales à streptocoque du groupe B.

GOAL: The aim of this study was to determine the incidence, risk factors, and outcome of maternal-fetal infection due to group B streptococcus. PATIENTS AND METHODS: We identified all cases of maternal-fetal group B streptococcus infection between January 2003 and December 2007, from neonatal unit reports at the Charles Nicolle Hospital. RESULTS: Ninety cases were identified out of 17,922 live births, incidence 5 ‰ of which 2.3 ‰ of bacteremia. Twenty percent of all newborns were premature and 22.2% had a low birth weight. Peripartum maternal fever was recorded in 52.2% of cases and membrane rupture more than 12 hours before delivery occurred in 74.4%. Among the newborns, 45.6% were symptomatic at birth. Forty percent of group B streptococci were resistant to erythromycin and 3.3% with intermediate resistance to ampicillin. The global neonatal mortality after group B streptococcus infection was 3.3%. CONCLUSION: Maternal-fetal infection due to group B streptococcus is still frequent and continues to be a major cause of morbidity and mortality.

[Usefulness of serum procalcitonin in the early diagnosis of maternal-fetal bacterial infection. A prospective study]. / Apport de la procalcitonine dans le diagnostic précoce de l'infection materno-fœtale bactérienne. Étude prospective.

UNLABELLED: Early diagnosis avoiding unnecessary treatment of maternal-fetal bacterial infection remains one of the greatest challenges for obstetricians and pediatricians. To meet these objectives, many inflammatory mediators were used, including procalcitonin (PCT). The aim of our study was to determine the usefulness of PCT in early diagnosis and management of neonatal infection. SUBJECTS AND METHODS: Over a period of 8 months, all living newborns with highly suspected maternal-fetal bacterial infection who were to receive antibiotic treatment according to our neonatal unit protocol were included in this prospective study. Serum PCT concentrations were determined at birth and after 12h of life using a specific immunoluminometric assay. Two distinct populations were defined based on clinical, biological, and bacteriological criteria: group 1: infected neonates, and group 2: noninfected neonates. STATISTICAL METHODS: We compared PCT means in different groups and determined the cut-off value correlated with maternal-fetal bacterial infection by analyzing the receiver operating characteristics curve (ROC). RESULTS: A total of 130 neonates were included in the study: 38 (29%) were classified in group 1 with 29 possible infections and 9 defined infections, including 5 cases of septicemia. The average PCT at birth in group 1 was significantly higher than in group 2 (3.52 ± 8.19 ng/ml vs 0.43 ± 0.73 ng/ml; P<0.001). The PCT threshold value at birth found by the ROC curve with the highest sensitivity (71.1%) and highest specificity (62%) was 0.215 ng/ml. The negative predictive value (NPV) was 83.8%, making it possible to avoid unnecessary treatment in the majority of the cases. The PCT threshold value within 12h of birth was 3.78 ng/ml, for a sensitivity of 89.5% and 1 NPV of 94.4%. CONCLUSION: PCT is a valuable biological examination because it can be administered early, it is sensitive, and it has a NPV. These characteristics make PCT a biological argument that can be used in the initial decision on whether to administer antibiotics. Another study will be conducted to establish the cut-off value.