[Primary Sjögren syndrome in a child]. / Syndrome de Goujerot-Sjögren primitif de l'enfant : à propos d'un cas.

Sjögren syndrome is uncommon in children and occurs most often in association with autoimmune diseases (secondary Sjögren syndrome). We describe the clinical and biological features of a 7-year-old girl with primary Sjögren syndrome revealed by recurrent parotiditis. CASE REPORT: A 7-year-old girl was referred for investigation of multiple episodes of parotid swelling since age 4 years, without systemic symptoms. The examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations and labial salivary gland biopsy revealed Sjögren syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotiditis in children is an uncommon condition. The onset of parotid swelling at 5 years or over deserves screening for disimmune disorders, sarcoidosis, or Sjögren syndrome. Diagnosis of Sjögren syndrome is based on diagnostic criteria.

[Management of patients treated for acute ST-elevation myocardial infarction in Tunisia: Preliminary results of FAST-MI Tunisia Registry from Tunisian Society of Cardiology and Cardiovascular Surgery]. / Prise en charge de l'infarctus du myocarde en Tunisie : résultats préliminaires du registre FAST-MI Tunisie de la Société tunisienne de cardiologie et de chirurgie cardiovasculaire.

UNLABELLED: FAST-MI Tunisian registry was initiated by the Tunisian Society of Cardiology and Cardio-vascular Surgery to assess characteristics, management, and hospital outcomes in patients with ST-elevation myocardial infarction (STEMI). METHODS: We prospectively collected data from 203 consecutive patients (mean age 60.3 years, 79.8 % male) with STEMI who were treated in 15 public hospitals (representing 68.2 % of Tunisian public centres treating STEMI patients) during a 3-month period at the end of 2014. The most common risk factor was tobacco (64.9 %), hypertension (38.6 %), diabetes (36.9 %) and dyslipidemia (24.6 %). RESULTS: Among these patients, 66 % received reperfusion therapy, 35 % with primary percutaneous coronary interventions (PAMI), 31 % with thrombolysis (28.6 % of them by pre-hospital thrombolysis). The median time from symptom onset to thrombolysis was 185 and 358 min for PAMI, respectively. The in-hospital mortality was 7.0 %. Patients enrolled in interventional centers (n=156) were more likely to receive any reperfusion therapy (19.8 % vs 44.6 %; p<0.001) than at the regional system of care with less thrombolysis (26.9 % vs 44.6 %; p=0.008) and more PAMI (52.8 % vs 8.5 %; p<0.0001). Also the in-hospital mortality was lower (6.4 % vs 9.3 %) but not significant. CONCLUSIONS: Preliminary results from FAST-MI in Tunisia show that the pharmaco- invasive strategy should be promoted in non-interventional centers.

[Serratia marcescens osteomyelitis as the first manifestation of chronic granulomatous disease]. / Granulomatose septique chronique révélée par une ostéomyélite à Serratia marcescens.

Chronic granulomatous disease is a rare, primary immunodeficiency disorder characterized by a defect in oxidative metabolism in phagocytes and recurrent bacterial and fungal infections. We report a case in a 2-month-old infant admitted with metacarpic osteomyelitis due to Serratia marcescens. Chronic granulomatous disease is rare but must be considered in cases of uncommon or atypical infection.

[Septic cavernous sinus thrombosis following ethmoiditis: a case report]. / Thrombose du sinus caverneux compliquant une ethmoïdite aiguë

Acute ethmoiditis is a rare infection of ethmoidal cells. The pathognomonic sign is an edema of the internal corner of the eye. Imaging may be necessary to verify the absence of orbital or endocranial complications. Thrombophlebitis of the cavernous sinus is a serious complication of this infectious process. We report the case of an 11-year-old boy who presented with ethmoiditis complicated with thrombophlebitis of the cavernous sinus, with right hemiplegia and left Bell palsy sequelae. Early diagnosis of this disorder and urgent therapy are essential. Treatment is based on the antibiotic therapy.

[Atypical hemolytic and uremic syndrome associated with von Willebrand factor-cleaving protease (ADAMTS 13) deficiency in children]. / Syndrome hémolytique et urémique atypique et déficit en ADAMTS 13.

Hemolytic and uremic syndrome (HUS) is a classical form of thrombotic microangiopathies characterized by the association of hemolytic anemia with schizocytes, thrombocytopenia, and acute renal failure. Two forms of HUS have been described: the typical form that occurs after ingestion of a strain of bacteria, usually Escherichia coli types, which expresses verotoxin (also called shiga-like toxin), typically followed by bloody diarrhea, and atypical HUS, which is rare during childhood and can also be revealed by bloody diarrhea. We report a case of a 25-month-old infant who presented with hematuria and pallor after an episode of diarrhea. Biological tests revealed anemia, thrombocytopenia, and renal failure. The diagnosis of typical HUS was made, but the causal microorganism was not identified. Progression was favorable within 5 days of plasma transfusions. Two months later, the patient presented with the same symptoms and neurological impairment without any diarrhea. Von Willebrand factor-cleaving protease activity (ADAMTS 13) was low. Therefore, the diagnosis of atypical HUS by severe deficiency of ADAMTS 13 was suggested. The treatment was based on plasma transfusions resulting in remission. Atypical HUS associated with severe ADAMTS 13 deficiency rarely occurs in childhood. The prognosis, usually threatening, has been completely transformed thanks to a better understanding of the pathogenesis and to therapeutic progress.

[Mucopolysaccharidoses in children. Experience of a general pediatric service. 11 cases]. / Les mucopolysaccharidoses de l'enfant. Expérience d'un service de pédiatrie générale. A propos de 11 cas.

The mucopolysaccharidosis are hereditary diseases. The neurological attack constitutes the principal factor of gravity of these affections. We conducted a retrospective study over a period of 12 years (1988-1999) in the pediatric department of Sfax University Hospital. This study allowed us to observe 11 cases of mucopolysaccharidosis confirmed by an enzymatic proportioning, with 3 cases of Hurler disease (IH), 3 cases of the disease of sanfilippo, (two II A and one III B), 3 cases of the disease of Morquio A (type IVA) and 2 cases of the disease of Maroteaux Lamy (type VI). A sex ratio of 1.75. The parents were cousins in 90% of the cases. The age of revelation ranged between 6 months to 4 years. The clinical examination has found a staturo-pondral delay in 81.8% of the cases, a craniofacial dysmorphy in 100%, deformations of the rachis in 63.6% of the cases, a psychomotor regression in 54.5% of the cases, a medullary compression in 18% of the cases, hepatosplenomegaly in 36.4%, and corneal opacities in 45.4% of the cases. The therapeutic treatment was limited to the symptomatic measures with genetic consulting and antenatal diagnosis.