[General practitioners' management of childhood asthma in Sfax, Tunisia]. / Prise en charge de l'asthme de l'enfant par le médecin de première ligne au Gouvernorat de Sfax, Tunisie.

AIMS: To study primary care physicians' attitudes toward childhood asthma management and their adherence to international guidelines. METHODS: Cross-sectional, descriptive and analytical survey conducted among 400 primary care physicians practicing in the governorate of Sfax. Data collection was done through a self-administered questionnaire with 36 questions. RESULTS: the participation rate was 53.75%. The average age was 49.72years and the sex ratio=1.52. 56.3% reported that they assisted in childhood asthma medical education between 2019 and 2020. Poor knowledge was found in 53.3% of practitioners. It concerns in 60.5% of cases the long-term asthma treatment. We found that 49.8% of doctors did not use the GINA guidelines in their daily practice. These guidelines were considered too complex by 45.8%. Oral salbutamol was prescribed by 10.2% of physicians in childhood asthma exacerbation and 64.2% antibiotics as therapy for childhood febrile asthma exacerbation. The practice of prescribing antihistamines as long-term therapy is still present in 28.8% of physicians. In front of exercise-induced asthma, sports exemptions were given by 33% of participants. Adherence to asthma guidelines was found in 34,41%. Physicians who are aged between 35 and 45years and who used GINA guidelines had better childhood asthma management score than other physicians. CONCLUSION: Despite guidelines, childhood asthma is still underdiagnosis and undertreated. Our study revealed difficulties faced by primary care physicians in the management of childhood asthma.

A new combined predicting model using a non-invasive score for the assessment of liver fibrosis in patients presenting with chronic hepatitis B virus infection.

OBJECTIVES: Several non-invasive markers have recently been proposed to predict liver fibrosis without percutaneous liver biopsy (PLB). We aimed to evaluate the performance of non-invasive scores and to highlight the value of a new combined score in the prediction of liver fibrosis in chronic hepatitis B (CHB) patients. PATIENTS AND METHODS: We performed a retrospective study of patients presenting with CHB who underwent PLB between 2008 and 2016. We calculated ASAT/Platelet Ratio Index (APRI), Fibrosis-4 Score (FIB4), GGT-to-platelet ratio (GPR), and ASAT/ALAT Ratio (AAR). Then, we combined APRI and FIB-4 scores into a new combined score. We assessed their performance in predicting liver fibrosis according to the Metavir score. RESULTS: A total of 179 patients presenting with CHB were included. Multivariate analysis showed that the APRI score was the only independent factor of significant fibrosis (OR=3.78; P=0.02), whereas the FIB-4 score was the only independent factor for severe fibrosis (OR=2.85; P<0.001) and cirrhosis (OR=2.5; P=0.001). At a threshold of severe fibrosis, APRI had the best specificity (75%) and FIB-4 had the greatest sensitivity (74%). Using the combined score, we improved the diagnostic performance of APRI and FIB-4 scores at the three thresholds of liver fibrosis. With this combined score, maximum 25.1% of patients presenting with CHB would undergo PLB. CONCLUSION: APRI, FIB-4, and GPR scores were well performing to predict liver fibrosis during CHB. The new combined score using APRI and FIB-4 was more accurate at the three-fibrosis thresholds.

Fulminant mulch pneumonitis in a previously healthy child.

Chronic granulomatous disease (CGD) is associated with multiple and recurrent infections. In patients with CGD, invasive pulmonary infection with aspergillus species remains the greatest cause of mortality. Acute fulminant presentations of fungal pneumonia are catastrophic. It is a medical emergency, and currently the treatment is based on association of corticosteroids and antifungal therapy. We describe the case of an 11-year-old boy, with late initial presentation of CGD, which was revealed by fulminant aspergillus pneumonia. He was successfully treated with an association of high doses of steroids and voriconazole.

[Ocular tuberculosis : A case series]. / La tuberculose oculaire : une série de 14 cas.

OBJECTIVES: Ocular tuberculosis is a rare form of extra pulmonary tuberculosis. It represents 1-2% of all clinical forms. The aim of this work was to focus on diagnostic and therapeutic characteristics of ocular tuberculosis. METHODS: We report a case series of 14 patients with ocular tuberculosis seen in an infectious diseases department between 2006 and 2015. The diagnosis was retained on clinical data and a positive tuberculin skin test or interferon-gamma release assay. RESULTS: The patient's mean age was 40.7±9years. The most common clinical presentation was uveitis (11 patients and 16 eyes). An extra ocular involvement was associated in three patients. The mean duration of antitubercular therapy was 10±2.5 months. Corticosteroid therapy was associated in 11 cases. The outcome was favorable in all cases. Two patients had maintained visual sequelae. CONCLUSION: Ocular tuberculosis is a rare disease but still remains a diagnostic problem. It should be considered in case of any chronic ocular symptoms, especially in endemic countries. Early management can improve the visual prognosis.

[Management of patients with major beta thalassemia in a paediatric department in the south of Tunisia: About 26 cases]. / Prise en charge thérapeutique des patients atteints de bêta-thalassémie majeure dans un service de pédiatrie du sud tunisien : à propos de 26 cas.

AIM: Our objectives were to assess the management of patients with major thalassemia and identify the various complications and monitoring means. PATIENTS AND METHODS: A retrospective study was conducted on 26 ß-thalassemic patients in the department of paediatrics, Hédi Chaker hospital, Sfax, Tunisia during a period of 25 years (from 1 January 1990 to 31 December 2014). RESULTS: The mean age of the beginning of transfusion was 11.5 months. That was with phenotyped red blood cells but not leukodepleted blood. Twenty-three patients received chelation. Before 2001, all patients received deferoxamine, poor adherence to this treatment was observed in 66% of cases. It was replaced by deferiprone since 2006 and deferasirox since 2009. A combination of 2 or 3 chelators was indicated for four patients. A total splenectomy was performed in 10 cases patients; it was due to hypersplenism. The bone marrow transplant was performed for one patient at the age of 9 year but it was rejected. Many complications were detected: endocrine complications (19 cases), immune complications (9 cases), gallbladder stones (5 cases), cardiac complications (4 cases), osteoporosis (3 cases), infectious complications (3 cases) and thromboembolic complications (2 cases). We noted some side effects related to chelation therapy in twelve cases. Four patients were dead. CONCLUSION: Improving the medical care of homozygous ß-thalassemic children requires adherence to transfusion regimen and chelation therapy. Bone marrow transplantation remains the only possible curative therapy, which must be promoted in our country.

[Symmetrical peripheral gangrene: 4 cases]. / La gangrène périphérique symétrique : à propos de 4 cas.

BACKGROUND: Symmetric peripheral gangrene (SPG) is a symmetrical distal ischemic lesion on at least 2 or more extremities in the absence of proximal arterial obstruction and vasculitis. It is a rare and severe clinical entity. The aim of this study was to describe clinical symptoms, etiological agents and the management of SPG through a series of 4 cases. PATIENTS AND METHODS: We included all cases of SPG hospitalized between 2000 and 2014. The inclusion criterion was the presence of distal ischemic damage at two or more sites in the absence of large vessel obstruction. RESULTS: Four patients (2 men and 2 women) were included. The mean age was 43.2±12 years. Two patients had a history of splenectomy. All patients had blackening of the tips of the fingers and toes. Three patients presented with septic shock. The etiology was bacteremia involving Streptococcus pneumoniae in two cases and a malignant form of Mediterranean spotted fever (MSF). In addition to specific antibiotics, we used a potent vasodilator (iloprost) in two cases and curative heparin therapy in two cases. The outcome was favorable in 3 cases, with regression of necrotic lesions. One case required the amputation of non-perfused necrotic fingers and toes. CONCLUSION: SPG can complicate MSF in some rare cases. Thorough and repeated skin examinations are essential to ensure timely diagnosis and treatment of GPS in order to improve the prognosis.

Macrophagic activation syndrome related to an infection by Rickettsia conorii in a child.

Since the Arab Spring, a resurgence of zoonotic diseases such as rickettsiosis, endemic in the Mediterranean basin, has been observed. It preferentially infects microvascular endothelial cells of mammalian hosts inducing vasculitis with endothelial injury. Rickettsioses are considered benign infectious diseases. Severe systemic manifestations have been reported and are often explained by a delay in diagnosis. We present a case of hemophagocytic syndrome occurring in a 4-year-old Libyan girl as a complication of Mediterranean spotted fever. Rickettsial infection was confirmed by serology and the patient was treated with clarithromycin, with a favorable outcome.

Occipital dermoid cyst associated with dermal sinus complicated with meningitis: A case report.

BACKGROUND: Occipital dermal sinus, usually associated with dermoid cyst, is a rare entity; it results from the persistence of an abnormal embryonal communication between the skin and the intradural space. Its main complication is intracranial infection. CASE DESCRIPTION: This 2-year-old girl was hospitalized for meningitis. Neuroradiological studies revealed a cystic mass of the posterior fossa communicating with the skin and hydrocephalus. The diagnosis of dermoid cyst associated with dermal sinus was established at surgery. The patient was treated with radical excision of both the occipital cyst and the dermal sinus associated with systemic antibiotic therapy. She had a good outcome. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with chronic occipital skin lesion, especially a dermal sinus. We emphasize the importance of early neurosurgical treatment of dermoid cysts to prevent the development of severe complications.

Chediak-Higashi syndrome presenting in accelerated phase: A case report and literature review.

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive lysosomal disorder characterized by frequent infections, oculocutaneous albinism, bleeding diathesis, and progressive neurologic deterioration. In 85% of cases, CHS patients develop the accelerated phase characterized by pancytopenia, high fever, and lymphohistiocytic infiltration of liver, spleen, and lymph nodes. Treatment of accelerated-phase CHS is difficult and the prognosis is poor. Here, we report a case of CHS in a 2-year-old boy who presented in the accelerated phase of the disease. CHS diagnosis was made on the basis of clinical characteristics, hair analysis, and identification of pathognomonic giant azurophilic granules in peripheral blood and bone marrow.

Ecthyma gangrenosum: A manifestation of community-acquired Pseudomonas aeruginosa septicemia in three infants.

OBJECTIVES: Pseudomonas aeruginosa sepsis usually carries a high mortality rate in immunocompromised children. Ecthyma gangrenosum is a known cutaneous manifestation due mainly to Pseudomonas infection with or without septicemia. We describe clinical, biological, and therapeutic data. PATIENTS AND METHODS: Of those children admitted to the pediatric intensive care unit, three pediatric cases of community-acquired P. aeruginosa septicemia associated with ecthyma gangrenosum were retrospectively reviewed. RESULTS: The three patients were aged 5 months, 9 months, and 1 year. Underlying hypogammaglobulinemia was detected in the oldest patient. Pseudomonas aeruginosa was isolated in all patients in blood and once in cultures of skin lesions and endotracheal aspirate. Two deaths occurred due to septic shock and multisystem organ failure despite numerous aggressive resuscitation attempts. CONCLUSIONS: P. aeruginosa sepsis should be treated as early as possible. Recognition of ecthyma gangrenosum allows early diagnosis and prescription of adequate antibiotic therapy without awaiting blood culture reports.

[Hereditary hemorrhagic telangiectasia. Report of a pediatric case]. / La maladie de Rendu-Osler-Weber : à propos d'une nouvelle observation pédiatrique.

Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is an autosomal dominant multiorgan disorder. This multisystemic vascular dysplasia is determined by a mutation of one of two main genes, endoglin (ENG) or HHT1, or ACVRL1 or HHT2. These mutations induce vascular disorders that cause recurrent epistaxis and eventually multiple telangiectasia and arteriovenous visceral malformations. We report the case of a 7-year-old girl who developed severe hypoxemia due to multiple pulmonary arteriovenous malformations.

[Megalencephaly with dystonia revealing Canavan disease]. / Macrocéphalie avec dystonie révélant une maladie de Canavan.

Canavan disease, or N-acetyl aspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of the brain. The disease results from the accumulation of N-acetyl aspartic acid in the brain, due to aspartoacylase deficiency. We report the case of a 6-month-old girl who presented with megalencephaly, peripheral hypertonia, and a developmental delay noticeable after 4 months of age. Magnetic resonance imaging of the brain with spectroscopy was suggestive of Canavan disease, which was confirmed by chromatography of urinary organic acids.

[Posterior reversible encephalopathy syndrome induced by acute postinfectious glomerulonephritis]. / Syndrome d'encéphalopathie postérieure réversible induit par une glomérulonéphrite aiguë postinfectieuse.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric edema on neuroimaging. It has been described in adults more than in children. Many pathological conditions and treatments have been associated with this syndrome. CASE REPORT: We report the case of an 11-year-old boy who presented with acute postinfectious glomerulonephritis, renal failure, and arterial hypertension. Two days after admission he developed generalized tonic clonic seizures. His blood pressure was 145/90 mmHg. Computed tomography showed hypodense lesions in the occipital and frontal areas. Based on the clinical manifestations and the typical pattern of brain imaging, the diagnosis of PRES was retained. Clinical manifestations and radiological abnormalities resolved after antihypertensive therapy. Axial FLAIR images and diffusion-weighted magnetic resonance images showed a slight hyperintensity signal in the left occipital area. CONCLUSION: Acute postinfectious glomerulonephritis in children should be considered a risk factor for developing PRES.

Murine typhus in Tunisia: a neglected cause of fever as a single symptom.

OBJECTIVES: We had for aim to describe the epidemiologic and clinic characteristics of murine typhus in a series of 43 serologically confirmed cases, in our region. PATIENTS AND METHODS: Serologic screening for IgG and IgM against Rickettsia typhi was performed in 1024 patients during three years (2006-2008). The characteristics of patients with a positive serology were examined retrospectively. One hundred and seventy sera obtained from blood donors were tested to detect IgG against R. typhi to determine the seroprevalence of the infection. RESULTS: There was evidence of recent R. typhi infection in 43 patients (4.2%) during the study period, and 3.7% of blood donors had IgG against R. typhi. The mean age of patients was 43.1 years and the sex-ratio was 1.04. Among the patients, 58.1% were from rural areas. No patient reported any exposure to rats or rat-fleas. There were more cases during the summer and fall. The most frequent complaint was fever as a single symptom (67.5%). A cutaneous rash was reported in 44.1% and headache in 60.5% of patients. Among the patients, 44.1% presented with thrombopenia and 47.2% with elevated liver enzymes. CONCLUSION: Murine typhus seems to be frequent in Tunisia. This infection could be a threat for travellers. Serology should be performed systematically in patients with fever as a single symptom since its clinical presentation is non-specific.