Acute bronchiolitis management in Tunisia: Impact of the national guidelines.

BACKGROUND: Acute bronchiolitis management involves all pediatricians and primary care physicians. The national guidelines for bronchiolitis diagnosis and treatment were published in Tunisia to reduce excessive use of diagnostic tests and unify bronchiolitis management. OBJECTIVES: We aimed to assess the real impact of the national guidelines on acute bronchiolitis management in Tunisia. METHODS: We conducted an evaluative cross-sectional study. We randomly distributed anonymous questionnaires to physicians managing acute bronchiolitis during the period from 1st March 2014 to 30 November 2015. RESULTS: We analyzed 140 questionnaires. Ninety-three interviewed physicians (66.4%) were advised of the latest national guidelines, half of them (33.6%) declared they didn't follow these guidelines. Real and complete guidelines adherence was observed in only 1.4% of interviewed physicians. According to bronchiolitis diagnosis, appropriate Chest X-rays and blood tests were requested respectively by 57.8% and 59.3% of interviewed doctors. Regarding bronchiolitis therapeutic management, bronchodilators and epinephrine nebulization weren't prescribed by respectively 45.7% and 38.6% of them. Antibiotics were prescribed by 92.9% of interviewed doctors and chest physiotherapy was well indicated by 47.8% of them. CONCLUSIONS: There is a disconnect between the bronchiolitis guidelines and clinical practice. National strategies have to be developed to reduce excessive use of diagnostic tests and unrecommanded therapies.

ABO hemolytic disease of newborn : Does newborn's blood group a risk factor?

BACKGROUND: Due to the marked decline of maternal-fetal rhesus incompatibility, ABO alloimmunization has become the leading cause of the newborn hemolytic disease. It is estimated that 15-25 % of all pregnancies are concerned by ABO incompatibility. AIM: Neonatal blood group B seems to be more predisposing to acute hemolysis and severe hyperbilirubinemia. We propose to find if the newborn's blood group B represents a risk factor for severe hemolysis and/or severe hyperbilirubinemia. METHODS: We conducted a comparative study in the pediatrics department "B" of the Children Hospital of Tunis. We collected retrospectively the medical files of the newborn hospitalized for ABO alloimmunization (January 2011 - March 2014), then we compared two groups, OA group with OA alloimmunization and OB group with OB alloimmunization. A significant threshold was fixed to 0.05. RESULTS: We collected 98 cases of newborn ABO hemolytic disease. Both groups, OA and OB, were similar for the onset of jaundice, age of hospitalization, initial hemoglobin and indirect bilirubin levels. There were no statistically significant difference in the severity of hyperbilirubinemia and the use of exchange transfusion for the two groups. However, transfusion was statistically more frequent in the OB group compared to OA group (81.6‰ vs 10.2‰, p = 0,039, OR=2.9, 95% IC (1.1 - 7.8)). CONCLUSION: OB alloimmunization seems to induce more active hemolysis than OA one, with no difference for severe hyperbilirubinemia in both groups.

A Novel Mutation c.153 C>A in a Tunisian Girl With Wolman Disease and Unusual Presentation: Hemophagocytic Lymphohistiocytosis.

Wolman disease is an ultrarare lysosomal storage disease caused by a mutation in the LIPA gene. The clinical features of Wolman disease include early onset of vomiting, diarrhea, failure to thrive, hepatosplenomegaly, and bilateral adrenal calcification. We report the case of a 3-month-old infant who presented clinical features of hemophagocytic lymphohistiocytosis. Genetic sequence analysis of the LIPA gene revealed homozygous mutation c.153 C>A (p.Tyr51*). The parents were heterozygous for this mutation. Prenatal diagnosis has been carried out in the next pregnancy. To our knowledge, this mutation has never been reported before, and this is an unusual case of secondary hemophagocytic lymphohistiocytosis complicating Wolman disease.

Pseudo-Bartter syndrome as the sole manifestation of cystic fibrosis in a child with 711+G>T/IVS8-5T mutation: a new face of an old disease.

Pseudo-Bartter syndrome (PBS) describes an uncommon complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. PBS as the sole manifestation of cystic fibrosis in children is extremely rare and has never been described in patients carrying 5T variant. We report a clinical, biochemical and genetic study of a four year-old boy presenting a pseudo-Bartter syndrome as the sole manifestation of cystic fibrosis. All 27 exons and the flanking intron regions of the CFTR gene were analysed by PCR and direct sequencing. Direct sequencing was also used to analyse TGmTn and M470V polymorphisms in the patient and his parents. Two sweat tests were abnormal with elevated chloride levels at 78 and 88 mmol/L. DNA sequencing revealed a heterozygous mutation 711+1 G>T and an IVS8-T5 allele. The mutation 711+1 G>T is in trans with the IVS8-T5-TG11 allele and the child carried M470/V470 genotype. To the best of our knowledge, the genotype 711+1 G>T /IVS8-5T found in our patient is described for the first time. The role of TG11-5T-V470 allele in cases of cystic fibrosis with PB syndrome remains to be determined.

Community-acquired pleuropneumonia in children: Bacteriological and therapeutic challenges.

Background Community-acquired pleuropneumonia (CPP) is a common complication of pneumonia in children. It is serious given its high morbidity and significant mortality. Aim To study clinical and paraclinical features of CPP in children and to establish a common therapeutic strategy. Methods Our retrospective study included patients who were hospitalized for CPP between 2004 and 2012. All data were collected from patients' medical files. Statistical analysis was made by Epi-Info 6. Results One hundred and sixty four patients were registered. The mean age was 32 months (15 days - 14.5 years). The hospital incidence of CPP doubled between 2004 and 2012. The symptomatology was dominated by fever (93.9%), cough (56.7%) and dyspnea (48.1%). The pleural effusion was frequently moderately abundant and loculated. Pleural sample, performed in 53.6% of cases, was the most beneficial bacteriological examination (p=10-6 ). The bacteriological confirmation was attained in 44.5% of cases with the predominance of Staphylococcus aureus (59%) followed by Streptococcus pneumoniae (26%). The S. aureus occurred basically in most young infants (p=0.04) and was responsible for the most severe cases (p=0.01). The CPP management included heterogeneous intravenous antibiotics associated with a pleural drainage in 40% of cases. The quarter of our patients were transferred to an intensive care unit. Six patients died. Conclusion The bacteriological confirmation is difficult. Pleural aspiration is the key tool. S. aureus is the first microorganism followed by S. pneumoniae. A therapeutic strategy is proposed based on large spectrum intravenous antibiotics. The pleural drainage indication is limited.