[A furoncular ankle cellulitis in a teenager with a difference: Dermatobia hominis]. / Un passager clandestin à ne pas méconnaître: Dermatobia hominis, à propos d'un cas.

We report a case of botfly myiasis presenting as an ankle cellulitis in a teenager returning from Guyana. Main clinical features and therapeutic approaches are described.

[Hurler syndrome. Early diagnosis and successful enzyme replacement therapy: a new therapeutic approach. Case report]. / Mucopolysaccharidose de type I: intérêt d'un diagnostic et d'une enzymothérapie substitutive précoces. A propos d'un cas.

Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder due to alpha-L-iduronidase deficiency. Its severe prognosis has been significantly improved by enzyme replacement therapy using recombinant human alpha-L-iduronidase (laronidase). We report the case of a boy who was diagnosed at 19 months of age with Hurler's disease, the most severe form of MPS I, and received thereafter a treatment by laronidase, resulting in clinical and biological improvement. The aim of this case report is to draw physicians' attention on the presenting signs of Hurler's disease, in order to enable an earlier diagnosis, increasing the treatment's benefits.

[Macrolides, Pseudomonas aeruginosa and cystic fibrosis]. / Macrolides, Pseudomonas aeruginosa et mucoviscidose: concepts actuels.

Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic.

[Varicella, acute postinfectious arteriopathy and cerebral arterial thrombosis in childhood: a unique clinical and etiologic framework to be fully acknowledged]. / Varicelle, artériopathie aiguë post-infectieuse et infarctus cérébral artériel de l'enfant: un cadre clinique et étiologique à ne pas méconnaître.

Acute arteriopathies are the etiological factor of 25% of all the cerebral arterial thrombosis cases in childhood; they give way to ischemic strokes which have specific clinical and radiological features; varicella is the etiologic factor in 60% of the cases; favorable outcome is the rule; physiopathology and treatment remain under discussion.

[Hereditary angioneurotic edema: a case report in a 3-year-old child]. / Oedème angioneurotique héréditaire: à propos d'une observation chez un enfant de trois ans.

Hereditary angioneurotic edema is a dominant autosomal disease (incidence 1/150,000), whose diagnosis is crucial as this condition can lead to fatal asphyxia within minutes. We report the case of a three-year-old girl, misdiagnosed as allergic asthma. Recognition of the syndrome led to adapted care restoring normal family and school life. Pathogenic cascades involved and therapeutic principles of this disease are reviewed.

[Aspergillosis in cystic fibrosis patients]. / Aspergillose et mucoviscidose.

Allergic bronchopulmonary aspergillosis (ABPA) should be suspected in any CF patient whose pulmonary disease deteriorates precipitously, suddenly or unexpectedly, and has to be correlated to fungal hypersensitivity biological tests. The standard therapeutic approach is based upon systemic corticosteroids. Invasive pulmonary aspergillosis (IPA) may occur mainly but not exclusively in immunosuppressed patients undergoing lung transplantation. New antifungal compounds (Triazoles, Itraconazole, Voriconazole), Echinochandins (Caspofungin) are effective, synergistic, well tolerated, should their contraindications and side effects be carefully respected and monitored.

[Home environment and acute propylene glycol intoxication in a two-year old. An unusual case report]. / Environnement domestique et intoxication aiguë au propylène glycol chez un nourrisson de deux ans. A propos d'une observation inhabituelle.

BACKGROUND: Acute propylene glycol intoxication in a two-year-old toddler underlines the potentially serious toxicity in children of this chemical agent present as a diluent in many drugs and environmental products such as cosmetics, diapers, cleansing towels, despite a common consideration of safety and lack of toxicity. CASE REPORT: A two-years-old boy previously healthy was found in the morning by his parents in his cradle, lethargic, responsive only to sharp pain. On admission, vital signs were: temperature 38.5 degrees C, lethargy, polypnea; propylene glycol intoxication through disposable cleansing towels chewing was ascertained by anamnesis and blood urine analyses which revealed metabolic acidosis and serum propylene glycol peak. CONCLUSION: Environmental acute propylene glycol intoxication must be considered and searched for in front of a metabolic acidosis case of unknown origin in children.

[Haemophilus influenzae bacterial meningitis: residual risk; case report]. / Méningite bactérienne à Haemophilus influenzae: le risque résiduel; à propos d'un cas.

UNLABELLED: Bacterial meningitis due to Haemophilus influenzae has become a rare, albeit not exceptional occurrence since generalized vaccination against that pathogen was instated, concerning as well incapsulated b and non-b Haemophilus influenzae strains, as non-incapsulated strains. CASE REPORT: A 19-month-old fully immunized infant was referred to our hospital for bacterial meningitis. CSF analysis elicited biotype III, non-incapsulated Haemophilus influenzae. CONCLUSION: Generalizing Haemophilus influenzae preventive inoculation has revolutionized the epidemiology of bacterial meningitis; however, a residual risk exists, which deserves to be taken into account.

[Pseudotumoral presentation of cat scratch disease]. / Maladie des griffes du chat de présentation pseudotumorale.

BACKGROUND: A frequent cause of chronic benign lymphadenopathy, cat-scratch disease (CSD) occurs mainly in children and young adults. Bartonella henselae is the agent responsible for CSD. The most common symptoms of the disease are regional lymphadenopathy and fever. Atypical forms occur in about 10% of patients; among them, CSD may initially present as a tumor. CASE REPORT: A 4-year-old child developed a 'tumor' of the arm with fever. The values of white blood cell count and CRP were normal. Ultrasonography, MRI and arteriography did not contribute to the diagnosis, which was established on histologic examination and serologic test for infection with B. henselae. The outcome was favorable with antibiotic treatment. CONCLUSION: In case of tumor of the limbs, cat-scratch disease should be searched for.

[Osteogenesis imperfecta: a new, early therapeutic approach with biphosphonates. A case report]. / Ostéogenèse imparfaite: une nouvelle approche thérapeutique précoce par les biphosphonates. A propos d'une observation.

UNLABELLED: Management of type III osteogenesis imperfecta (O.I.) (brittle bone disease) is primarily supportive; early introduction of cyclic intravenous pamidronate administration in children younger than 2 years of age is an innovative and promising therapeutic approach. CASE REPORT: We present the case of a 6-month-old infant, whose preliminary data have already been partly published, with severe type III O.I. referred because of aching and crumbling from multiple fractures. Cyclic intravenous disodic pamidronate administration improved the clinical status (fracture incidence, pain, growth curve) and biological status (bone density, osseous alkaline phosphatases, urinary desoxypiridoline excretion), allowing a remarkable recovery. CONCLUSION: Biphosphonates are a new and innovative therapeutic agent in O.I. Clinical safety, easy administration, and overall efficacy are likely to extend their use in severe type III O.I. from the very first months of life, the time of best efficacy.

[Secondary kidney amyloidosis and mucoviscidosis]. / Amylose rénale secondaire et mucoviscidose.

BACKGROUND: Due to the improvement in cystic fibrosis management, life expectancy has risen; on the other hand, longer survival has led to new complications, including secondary renal amyloidosis, which has been so far very uncommonly reported. CASE REPORTS: Secondary nephropathic amyloidosis was seen in two 25-year and 22-year-old adults with cystic fibrosis. Both had developed recurrent pulmonary infections due to Pseudomonas aeruginosa over several years. One patient died after 2 years of progressive kidney failure. CONCLUSION: Severe renal insufficiency due to secondary amyloidosis may complicate the course of cystic fibrosis and become the main prognosis factor in adults.