Rabies encephalitis in children: a resurgence of a fatal anthropozoonosis

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Flexible bronchoscopy contribution in the approach of diagnosis and treatment of children’s respiratory diseases: the experience of a unique pediatric unit in Tunisia

Objective: Our study aimed at assessing the role of flexible bronchoscopy (FB) in improving diagnosis and management of children’s respiratory conditions in the pediatric unit of FB, newly created and unique in Tunisia.Methods: Retrospective study including all the FB achieved in our pediatric unit from 2009 to 2014.Results: We performed 365 FB in 333 patients aged 46 months on average (1 month - 15 years), often under conscious anesthesia (81.6%). FB was performed for diagnostic purposes in 341 cases and for therapeutic purposes in 24 cases. Eight anatomical abnormalities were revealed in 22 patients. An intraluminal bronchial obstruction was found in 71 FB, mainly due to a foreign body (n=36). A vascular anomaly was responsible for nine cases out of 17 extraluminal obstructions. Airways malacia was observed in 60 FB. Bronchoalveolar lavage was performed in 196 cases. It was determinant in 43.9% of the cases. FB was of great diagnostic value in 74.8% of the cases. It influenced the management of the patients in 58% of the cases. The FB for therapeutic purposes was beneficial in all cases. Few complications occurred (5.5%).Conclusion: FB is a safe tool providing precious diagnostic and/or therapeutic help for the clinician.Keywords: Flexible bronchoscopy, child, bronchoalveolar lavage, foreign body aspiration, pulmonary alveolar proteinosi

Cystic fibrosis in Tunisian children: a review of 32 children

Background: Cystic fibrosis is rare in Tunisia.Its diagnosis requires experienced specialists. Its prognosis is poor in developing countries.Objectives: To study the epidemiologic, clinical, genetic features and the therapeutic challenges of cystic fibrosis in Tunisian children.Methods: Covering a period of 21 years, this retrospective study included all patients with a definite diagnosis of cystic fibrosis from the Pediatrics Department B of The Children’s Hospital of Tunis.Results: Data from 32 children (14 boys and 18 girls) were collected. The diagnosis was made during the first year of life in 28 cases. Meconium ileus was found in 5 cases, respiratory manifestations in 22 cases, chronic diarrhea in 19 cases, faltering growth in 17 cases and a pseudo Barter syndrome in 2 cases. The sweat chloride test was positive in all cases. The most frequent mutation was F508del (56% of cases). Respiratory complications marked the outcome. Among our 32 patients, 15 patients (50%) died at an average age of 5 years and 3 months, mainly due to respiratory failure. The mean age of the surviving patients was 5 years.Conclusion: Cystic fibrosis prognosis is poor in our series compared to developed countries due to the longer diagnostic delay and the limited therapeutic options.Keywords: Cystic fibrosis, children, Tunisia

Rabies encephalitis in children: a resurgence of a fatal anthropozoonosis

Rabies is a ubiquitous fatal disease and its large wild life reservoir may precipitate a flare-up whenever stringent control is relaxed. It remains a serious public health challenge in the developing world. It is estimated that up to 40\u201360,000 cases of human rabies occur annually, mostly in rural areas of Africa and Asia, and with a particularly high incidence in young children (under the age of 15 years). Although Tunisia has implemented a national program against rabies since 1982, we are still recording one or two cases every year; in these cases, the victims generally did not consult after a dog bite and did not receive prophylaxis. We report in this article the case of an eleven year old child, who received post exposure prophylaxis and unfortunately developed fatal rabies

Flexible bronchoscopy contribution in the approach of diagnosis and treatment of children\u2019s respiratory diseases: the experience of a unique pediatric unit in Tunisia.

Objective: Our study aimed at assessing the role of flexible bronchoscopy (FB) in improving diagnosis and management of children\u2019s respiratory conditions in the pediatric unit of FB, newly created and unique in Tunisia. Methods: Retrospective study including all the FB achieved in our pediatric unit from 2009 to 2014. Results: We performed 365 FB in 333 patients aged 46 months on average (1 month - 15 years), often under conscious anesthesia (81.6%). FB was performed for diagnostic purposes in 341 cases and for therapeutic purposes in 24 cases. Eight anatomical abnormalities were revealed in 22 patients. An intraluminal bronchial obstruction was found in 71 FB, mainly due to a foreign body (n=36). A vascular anomaly was responsible for nine cases out of 17 extraluminal obstructions. Airways malacia was observed in 60 FB. Bronchoalveolar lavage was performed in 196 cases. It was determinant in 43.9% of the cases. FB was of great diagnostic value in 74.8% of the cases. It influenced the management of the patients in 58% of the cases. The FB for therapeutic purposes was beneficial in all cases. Few complications occurred (5.5%). Conclusion: FB is a safe tool providing precious diagnostic and/or therapeutic help for the clinician. Keywords

Acute Pesticide Poisoning in Children: A Review of 50 Cases

Background: Pesticide poisoning is very common in Tunisia. Various factors are involved in the analysis of the clinical presentations and the severity of this condition. Major factors are the chemical nature of the pesticides and the quantity entered the body. Methods: This is a retrospective study, reporting the pediatric cases that presented to us with signs and symptoms of pesticide poisoning. Fifty cases pesticide poisoning were admitted to the hospital between January 2013 and October 2016. Results: A total of 50 pediatric cases were included in this study with the mean age of 3 years and 4months. The poisoning was accidental in 49 cases and self-inflicted in one, with the mode being oral (N=45), respiratory (N=2) and cutaneous (N=3). The average duration of hospital care for these patients was 2 hours and 30 minutes (range: 30 min-24 hr). The clinical manifestations of poisoning noted were due to muscarinic and nicotinic receptors inhibition. Upon clinical examination, 29 patients had no pesticide in the gastric lavage fluid and urine and demonstrated no abnormal cholinesterase activity. The therapeutic management was mainly symptomatic with antidote medications prescribed (atropine and oxime). All patients had favorable outcomes and no death occurred. Conclusion: This study demonstrated the frequency of pesticide poisoning in a pediatric setting and the importance of early management. Optimal therapeutic approaches were evaluated, demonstrating that prevention still remains the best solution in such cases

Long-term outcome of Tunisian children with primary ciliary dyskinesia confirmed by transmission electron microscopy.

Background: Primary ciliary dyskinesia (PCD) is rare. Its diagnosis requires experienced specialists and expensive infrastructure. Its prognosis is variable. Objective: To study the long-term outcome of PCD in Tunisian children with ciliary ultra-structure defects detected by electron microscope. Methods: Covering a period of 20 years (1996-2015), this retrospective study included all patients with definite PCD (outer dynein arms (DA) defects and/or situs inversus) and presumed PCD (other ciliary ultra-structure defects). The clinical data and the investigations made were registered at diagnosis and during the follow-up. Results: Patients with a definite PCD (G1, n=7) were diagnosed earlier compared to those with a presumed PCD (G2, n=13) (2.5 vs. 9.3 years on average). At diagnosis, bronchiectasis was more frequent in G1 (3/7 vs. 4/13). The inner DA loss was constant in G1 and predominant in G2. The treatment adhesion was more often irregular in G2 (2/7 vs. 8/13). During a mean follow-up of 11 years, G1 showed less severe outcome (clubbing (0 vs. 3), bronchiectasis (3 vs. 11; more expanded in G2), proximal and distal airway obstruction (0/3 vs. 5/7), lobectomy (0 vs. 2), and death (0 vs. 2)). Conclusion: Precocious diagnosis and regular treatment may enhance the PCD prognosis

Cystic fibrosis in Tunisian children: a review of 32 children

Background: Cystic fibrosis is rare in Tunisia.Its diagnosis requires experienced specialists. Its prognosis is poor in developing countries. Objectives: To study the epidemiologic, clinical, genetic features and the therapeutic challenges of cystic fibrosis in Tunisian children. Methods: Covering a period of 21 years, this retrospective study included all patients with a definite diagnosis of cystic fibrosis from the Pediatrics Department B of The Children\u2019s Hospital of Tunis. Results: Data from 32 children (14 boys and 18 girls) were collected. The diagnosis was made during the first year of life in 28 cases. Meconium ileus was found in 5 cases, respiratory manifestations in 22 cases, chronic diarrhea in 19 cases, faltering growth in 17 cases and a pseudo Barter syndrome in 2 cases. The sweat chloride test was positive in all cases. The most frequent mutation was F508del (56% of cases). Respiratory complications marked the outcome. Among our 32 patients, 15 patients (50%) died at an average age of 5 years and 3 months, mainly due to respiratory failure. The mean age of the surviving patients was 5 years. Conclusion: Cystic fibrosis prognosis is poor in our series compared to developed countries due to the longer diagnostic delay and the limited therapeutic options

A gain-of-function mutation in zinc cluster transcription factor Rob1 drives Candida albicans adaptive growth in the cystic fibrosis lung environment

International audienceCandida albicans chronically colonizes the respiratory tract of patients with Cystic Fibrosis (CF). It competes with CF-associated pathogens ( e . g . Pseudomonas aeruginosa ) and contributes to disease severity. We hypothesize that C . albicans undergoes specific adaptation mechanisms that explain its persistence in the CF lung environment. To identify the underlying genetic and phenotypic determinants, we serially recovered 146 C . albicans clinical isolates over a period of 30 months from the sputum of 25 antifungal-naive CF patients. Multilocus sequence typing analyses revealed that most patients were individually colonized with genetically close strains, facilitating comparative analyses between serial isolates. We strikingly observed differential ability to filament and form monospecies and dual-species biofilms with P . aeruginosa among 18 serial isolates sharing the same diploid sequence type, recovered within one year from a pediatric patient. Whole genome sequencing revealed that their genomes were highly heterozygous and similar to each other, displaying a highly clonal subpopulation structure. Data mining identified 34 non-synonymous heterozygous SNPs in 19 open reading frames differentiating the hyperfilamentous and strong biofilm-former strains from the remaining isolates. Among these, we detected a glycine-to-glutamate substitution at position 299 (G299E) in the deduced amino acid sequence of the zinc cluster transcription factor ROB1 ( ROB1 G299E ), encoding a major regulator of filamentous growth and biofilm formation. Introduction of the G299E heterozygous mutation in a co-isolated weak biofilm-former CF strain was sufficient to confer hyperfilamentous growth, increased expression of hyphal-specific genes, increased monospecies biofilm formation and increased survival in dual-species biofilms formed with P . aeruginosa , indicating that ROB1 G299E is a gain-of-function mutation. Disruption of ROB1 in a hyperfilamentous isolate carrying the ROB1 G299E allele abolished hyperfilamentation and biofilm formation. Our study links a single heterozygous mutation to the ability of C . albicans to better survive during the interaction with other CF-associated microbes and illuminates how adaptive traits emerge in microbial pathogens to persistently colonize and/or infect the CF-patient airways