[Sickle cell retinopathy in children: Report of 42 cases]. / La rétinopathie drépanocytaire chez l'enfant : à propos de 42 cas.

We aimed to describe the epidemiological, etiological and clinical features, treatment and clinical course of sickle cell retinopathy in children and to determine the risk factors for serious involvement. METHODS: This was a retrospective study including all children diagnosed with sickle cell retinopathy. Epidemiological, clinical and therapeutic characteristics, as well as clinical course, were analysed retrospectively by chart review. Two groups were defined: Group 1 (Goldberg stage 1 and 2); Group 2 (Goldberg stage 3, 4 and 5). In order to identify factors independently associated with severe sickle cell retinopathy, we conducted a logistic regression analysis in descending order. RESULTS: The frequency of sickle cell retinopathy was 14.48%. Forty-two patients (84 eyes) were included; among them 23 boys and 19 girls, aged 10 to 17 with a mean age of 14±1.98 years. Twenty patients were of genotype SS, 11 patients of genotype SC, 8 Sß and 3 SO Arab. The three patients in group 2 were all of SS genotype. The majority of patients (32) had an HbF level of less than 15%. All our patients had sickle cell retinopathy distributed as follows: 62% at stage 1; 31% at stage 2; 5% at stage 3 and 2% at stage 4. Multivariate analysis revealed a single risk factor independently linked to severe involvement - an HbF level<15%. CONCLUSION: Retinopathy is a frequent complication of sickle cell disease which may lead to blindness. The HbF level is negatively correlated with severe involvement.

[Orbital cellulitis in children: Report of 60 cases]. / Les cellulites orbitaires chez l'enfant : à propos de 60 cas.

The goal of this study was to describe the epidemiological, etiological, clinical and therapeutic features and clinical course of orbital cellulitis in children, and to assess the risk factors for retroseptal involvement. METHODS: This was a retrospective study including 60 children (67 eyes) diagnosed with orbital cellulitis. Two groups were defined according to the clinical form: pre- or retroseptal. RESULTS: We studied 29 cases (34 eyes) of preseptal cellulitis and 31 cases (33 eyes) of retroseptal cellulitis. The mean age was 4 years. The male: female ratio was 1.3. The prescription of anti-inflammatory drugs and antibiotics prior to hospitalization was noted in respectively four and 10 patients. Eyelid edema was the principal sign (100% of cases); exophthalmia was noted in 19 eyes, ptosis in 27 eyes and chemosis in 10 eyes. Oculomotor disorders were present in 4 eyes. The mean C-reactive protein level was 53.15±27mg/l in preseptal cellulitis and 92.09±21mg/l in the retro-septal cases. Orbital computed tomography was performed in 31 patients and MRI in 5 patients. The pathway of entry of the orbital infection was primarily from the sinuses (23 cases). All of our patients had received broad spectrum intravenous antibiotic therapy. Three children had a cavernous sinus thrombosis and had been treated with anticoagulant therapy. Surgical drainage was performed in five patients. The course was favorable and without sequelae for all the patients. Two independent risk factors for retroseptal involvement were identified: the prescription of anti-inflammatory drugs prior to hospitalization, and sinus involvement. CONCLUSION: Orbital cellulitis in children is a serious infection and requires close collaboration between the ophthalmologist, otolaryngologist, and pediatrician in order to be diagnosed and treated early so as to improve the prognosis for vision and life.

[Primitive lung abscess: an unusual situation in children]. / Abcès pulmonaire primitif à staphylocoque: situation rare chez l'enfant.

INTRODUCTION: Lung abscess is a localized area of non tuberculosis suppurative necrosis of the parenchyma lung, resulting in formation of a cavity containing purulent material. This pathology is uncommon in childhood. CASE REPORT: A 3-year-6 month-old boy was admitted with prolonged fever and dyspnea. Chest X-ray showed a non systemized, well limited, thick walled, hydric, and excavated opacity containing an air-fluid level. Chest ultrasound examination showed a collection of 6. 8 cm of diameter in the right pulmonary field with an air-fluid level. Hemoculture showed Staphylococcus aureus. The patient received large spectrum antibiotherapy. Three days after, he presented a septic shock and surgical drainage was indicated. Histological examination confirmed the diagnosis of lung abscess. Any underlying condition such as inoculation site, local cause or immune deficiency, was noted and diagnosis of primary abscess was made. The patient demonstrated complete recovery. He is asymptomatic with normal chest X-ray and pulmonary function after 3 years of evolution. CONCLUSION: Lung abscess represent a rare cause of prolonged fever in childhood. An underlying condition must be excluded to eliminate secondary abscess.

[Pediatric Crohn's disease in Tunisia]. / La maladie de Crohn de l'enfant en Tunisie.

UNLABELLED: Crohn's disease occur mainly in adults. However, pediatric onset forms are not rare and have many characteristics. AIM: to study clinical, diagnostic, therapeutic and evolutive characteristics of crohn's disease in tunisian children. METHODS: Retrospective multicenter study conducted in 10 pediatric departments on a period of 10 years (2000-2008) RESULTS : 43 children were included. The sex-ratio was 1.68. The mean age at the onset of the symptoms was 11+/-2.3 years (5-16 years). The age of onset was inferior to 10 years in 25 % of the children. The delay before management was superior to 1 year in 25% of cases. The initial symptoms were dominated by diarrhea (95%). Perineal manifestations were present at diagnosis in 30% of children and extra-digestive manifestations in 53%. Ileocolonic localization was the most frequent (46%). The initial disease flare was moderate in 83% of cases. The treatment was medical in 77 % of cases, nutritional and medical in 18.5%. Maintenance therapy was instituted in 86% of cases and consisted essentially in azathioprin (62%). The mean follow-up was 3 years and 4 months. 60% of the children had at least one acute flare. During evolution, 7% of children had anoperineal surgery and 11% an intestinal resection. CONCLUSION: Crohn's disease seems rare in Tunisia. The time of diagnosis is often delayed. The management is based on immunosuppressive therapy and nutritional support.

[The re-emergence of pertussis in Tunisia]. / La réémergence de la coqueluche en Tunisie.

OBJECTIVE: The authors had for aim to analyze pertussis epidemiology in Tunisia by studying nasopharyngeal specimens of infants hospitalized in Tunis. METHODS: Between march 2007 and march 2008, clinical nasopharyngeal samples were collected from infants with a suspected diagnosis of whooping cough, pertussoid cough, or pertussis-like syndrome, admitted at the Tunis children's hospital. The laboratory diagnostic criteria were culture isolation of Bordetella species on Bordet-Gengou medium and real-time PCR. RESULTS: Fifty-nine percent of the 74 investigated children with suspected pertussis were less than two months of age. The diagnosis of pertussis was proved positive by real-time PCR for 41%. Culture was negative in all cases. CONCLUSIONS: Whooping cough is still prevalent in Tunisia despite an important vaccination coverage. Real-time PCR is an invaluable tool for the rapid diagnosis of pertussis, however culture must also be associated.

[Iatrogenic meningitis after diagnosis lumbar puncture: 3 cases reports in the paediatric Children's Hospital of Tunis]. / Méningite bactérienne iatrogène après ponction lombaire de diagnostic: à propos de trois cas observés à l'hôpital d'enfants de Tunis.

We have collected cases of iatrogenic meningitis managed in the Children's Hospital of Tunis, between January 1998 and December 2006. Clinical information about each patient were collected, all bacterial samples were investigated in the microbiology laboratory of the hospital. Bacterial isolates were identified according to conventional criteria. In the interval under study, we recorded three cases of iatrogenic meningitis after lumbar puncture. Two cases occurred in newborn admitted for suspicion of neonatal infection and one in a 2-month-old infant admitted for exploration of hyperpyretic convulsion. In all patients, the initial cerebrospinal fluid was normal. All patients developed symptoms of acute meningitis within 72 hours after lumbar puncture; the second cerebrospinal fluid was, then, typical for purulent meningitis. The causal agents isolated in the three cases were Klebsiella pneumoniae, Enterobacter cloacae, and Serratia marcescens, all resistant to beta-lactams by extended spectrum beta-lactamase production. The use of quinolones was required in all cases. Different complications were recorded: hydrocephalus and brain abscess in one case, respiratory and hemodynamic failure managed in the intensive care unit in the second, and brain hygroma in the third case. This study shows high morbidity of iatrogenic meningitis. Simple aseptic precautions undertaken before the procedure of lumbar puncture can prevent such cases. The urgent need for increasing the awareness among medical personnel in hospitals of developing countries cannot be overemphasized.

[Neisseria meningitidis invasive infections in children in Tunis: about 79 cases]. / Les infections invasives a Neisseria meningitidis chez l'enfant a Tunis : a propos de 79 cas.

We conducted a retrospective study of meningococcal invasive diseases (MID) contracted in children in Tunis between January 1997 and January 2006. The purpose of this study is to specify the clinical, epidemiological, therapeutic and evolutionary features of these infections and to determine antimicrobial susceptibility and the antigenic formula of N. meningitidis isolates. During the study period, we have collected 79 cases of MID arising in children aged 3 days to 11 years. The majority of children's were less than of 4 years (57.3%). We note a frequency of the MID in winter and in spring. The most frequent clinical shape was meningitis (53%). Twenty one patients (26.6%) had a fulminant meningococcal disease. In our series, the rate of lethality was equal to 17.7%. Among the 46 meningococcal isolates, the most frequent serogroup was the B (73%) followed by C and A. A high heterogeneousness of the antigenic formulae was observed The most frequent phenotype was NT: NST for the group B isolates and 4:P1.13 for the group C ones. N meningitidis with reduced susceptibility to penicillin and to amoxicillin account for 54% and 10% of all isolates respectively. The cefotaxim and the rifampin were uniformly active.

Gastric atrophy and Helicobacter pylori infection in children.

AIM: To assess the prevalence of gastric atrophy (GA) in Tunisia (a high prevalence region for Helicobacter pylori), and describe its histological, clinical and endoscopic features in children. METHODS: 345 children, 151 male and 194 female, mean age 8.6 +/- 3.7 years, underwent upper gastrointestinal (UGI) endoscopy with gastric biopsies for recurrent abdominal pain (n=232, 67.2%), vomiting (n=72, 20%) associated with or without upper gastrointestinal bleeding (n=59, 17.1%) and miscellaneous causes (n=53, 15.4 %). Biopsies performed both in the gastric antrum (n=2) and corpus (n=2) were analysed for histological assessment according to the updated Sydney classification system and bacterial culture. A positive result was recorded where histology and/or culture were positive, confirming the presence of H. pylori infection (H. pylori +ve). A negative result was recorded when both tests were concomitantly negative (H. pylori -ve). RESULTS: 9.3% (32/345) of the total population, and 14.5% (32/221) of chronic gastritis patients exhibited GA, M/F: 16/16, mean age (SD) 9.4 (3.4) years. Amongst the 32 children with GA, 30 (93.7%) were H. pylori +ve and 2 (6.3%) were H. pylori -ve. GA was localised in the antrum (n=26, 81.2%), the fundus (n=2, 6.3%) and was also seen in both (n=4, 12.5%). GA was categorised as mild, grade 1 (n=18, 56.3%); moderate, grade 2 (n=13, 46.6%); and severe, grade 3 (n=1, 3.1%). GA was associated with mild active gastritis in 18 cases (56.3%). The prevalence of moderate or severe antral GA was detected in 9/26 (34.6%) of H. pylori +ve vs. any of H. pylori -ve (p=0.4), whereas GA in the corpus was detected in 1/2 (50%) vs. none, respectively. None exhibited intestinal metaplasia. There were no clinical features specific to this pathology. UGI endoscopy in GA patients showed nodular gastritis (n=17, 53.1%), congestive gastritis (n=9, 28.1%), and normal tissue (n=6, 18.8%). GA was significantly associated with H. pylori infection (p<0.0001) and nodular gastritis (p<0.005). CONCLUSION: GA was found in 9.3% of Tunisian children undergoing UGI endoscopy and was significantly associated with H. pylori infection and nodular gastritis.

Localization of the Schwartz-Jampel syndrome (SJS) locus to chromosome 1p34-p36.1 by homozygosity mapping.

Schwartz-Jampel syndrome (SJS, MIM 255800), also known as chondrodystrophic myotonia, is a rare autosomal recessive disorder characterized by generalized myotonia, skeletal abnormalities and facial dysmorphism. Using homozygosity mapping, we localized the SJS locus to chromosome 1p34-p36.1 in a 8 cM interval flanked by markers D1S199 and D1S234. Families of different ethnic backgrounds (Tunisia and South Africa) showed genetic linkage to the same locus. Moreover, one Algerian family also demonstrated evidence of genetic linkage to 1p34-p36.1. Taken altogether, our results suggest genetic homogeneity, at least in the group of families analyzed.

[Pulmonary sequestration in an infant]. / Séquestration pulmonaire chez un nourrisson.

A case of intralobar pulmonary sequestration in an infant is reported. The lesion presented as recurrent bronchopulmonary infections. The chest film showed a density in the right lower lobe, in which air-filled images and air-fluid levels developed rapidly. Aortography demonstrated systemic blood supply to the lesion from three arteries stemming from the thoracic aorta. The pathological study of the operative specimen confirmed the diagnosis. Postoperative outcome was satisfactory.