[Aetiologies and outcome of bronchiectasis in children: a study of 41 patients]

Bronchiectasis remains an important cause of chronic suppurative lung disease in the developing world. This study is to describe the epidemiological characteristics, clinical features, underlying aetiologies and Outcome of bronchiectasis in the paediatric hospital of Tunis. A retrospective study of 41 children with bronchiectasis was conducted between January 1994 and December 2006. Diagnosis was made in patients with clinical suspicion of bronchiectasis associated with abnormalities on chest X ray [n=37] and/or on high resolution computed tomography [HRCT] [n=36]. Mean age at diagnosis was 5 years 9 months; [range: 6 months-14 years]. Persistent cough and bronchorhea were the most common symptoms. Fourteen patients [34%] had dyspnoea on first presentation, 11 of them [26.8%] had chest deformation and/or finger clubbing. Haemoptysis was noted in only two cases. Mean time to diagnosis from symptom onset was 2.7 years [range: 2 months- 4 years]. The underlying aetiologies were identified in 52% of patients. Cystic fibrosis [17%], previous pneumonic illness [9.7%], primary ciliary dyskinesia [9.7%] and immunodeficiency [9.7%] were the most common causes. After a mean follow-up of 6.6 years, the annual lower respiratory infection rate decreased from 7.2 +/- 3 to 3.1 +/- 2.6 [p<0.05], Twenty one point nine per cent of patients had chronic respiratory failure and five patients required surgery. Delays diagnosis of bronchiectasis remains important in our country. Congenital and indeterminate aetiologies are the most common forms. Prognosis is poor with a high prevalence of chronic respiratory failure

[Systemic juvenile idiopathic arthriris: diagnosis and management]

Systemic juvenile idiopathic arthritis is a relatively rare disease in childhood. Sometimes, difficulties are encountered because it is a diagnosis of exclusion. The authors describe the clinical, biologic, therapeutic and evolutive characteristics of this affection. It is a retrospective study during a period of 16 years from 1990 to 2005. Inclusion criteria respond to Durban criteria, after exclusion of other etiologies of febrile eruptions. The authors analyse epidemiologic and clinical characteristics of the disease, biologic exams, treatment and evolution. Nine eases are reported during study period. Patients are between 22 month and II year old. Biologic analysis shows an important inflammatory syndrome with a sedimentary rate superior to 80 mm at first hour in average, a white blood cell count superior to ll.000/mm3. the evolution under treatment is characterized by resistance to steroids in one case and dependence to steroids in 2 eases. The complications include a macrophage activation syndrome in 2 cases and complications secondary to steroids in one ease. Systemic juvenile idiopathic arthritis remains a relatively rare disease in childhood. It's management is improved by the emergency of new treatments bnt its evolution is unpredictable

[ adnexal torsion in children]

Adnexal torsion is the most frequent gynaecological emergency in children. It requires an early diagnosis and an urgent surgical treatment. To study the clinical, paraclinical and therapeutic aspect of adnexal torsion in paediatric population this is a retrospective review of nine girls with the diagnosis of ovarian torsion observed over a 7 years period [January 1999 to December 2005]. The average age is 9 years [extreme 6 to 13 years]. This pathology was located in 5 cases on the right side and in 3 cases on the left side; a case of bilateral torsion of poly-cystic ovary was encountered in a girl with Down's syndrome. Clinical presentation is made in all the cases by abdominal pains and vomiting. The disorders of the transit and the urinary signs are associated in 3 and 2 cases respectively, the clinical examination objectified a pelvic defense in all the cases and an abdominal mass in 2 cases. Pelvic ultra-sonography was made in 6 observations and give the diagnosis of torsion of the ovary in 4 cases, whereas it was doubtful in the 2 remaining cases when an ovarian mass was observed. In the 3 remaining cases, this examination was not performed since one the diagnosis of acute appendicitis was retained and the patient operated in emergency. All the children of our series were operated; in 1/3 of the cases we found a necrosis of the ovary. 4 cases out of 9 present a torsion on pathologic ovary [cyst, dysplasia], whereas in the 5 remaining cases. We noted a torsion on healthy ovary 4 young girls have undergoes a annexectomy, of which one was bilateral. The evolution was favorable in all the cases. Adnexal torsion is a surgical emergency that need an early diagnosis and management to preserve ovarian function in girls and Doppler sonography every must be done every time there is a pelvic pain without fever in girls

[Epidemiologic profile and management pediatric medical emergencies consultants of Tunisian child's hospital]

Background: the pediatric medical emergencie's of Tunisian child's hospital service manages all children fifteen years old. It received 45 000 children in year in average

The aims: establish an epidemiologic profile of consultants and study the encountred morbidity

Methods: we made a transversal describing study of the pediatric emergencie's activity during three successive months in 2004 [February, March, April]. We described the characteristics of the population consulting this service, study the severity and immediate behaviours in emergencies

Results: The number of all consultants is 10560 children during these three months. 54.9% of cases are between one month old and two years old, 54.7% of them are boys

The recruitment mode is direct in 92.9% of cases. The maximum of consultants is in the week-ends with an average of 129 patients per day

In the other days of the week, the average number is 115 +/- 27 patients per day ant the extrems are 75 and 225 patients per day. In the morning, a third of the daily activity is made the morbidity is dominated by the pulmonary pathologies in 39.9% of cases, otorhinolarygologic affections in 31.8% of cases, gastroenterologic pathologies in 6.72% of cases and brain system's pathology in 3.43% of cases. The neonatal affection represents 2.74% of total consultants. At the end of the consultations, 38.7% of patients go back home with medical prescriptions. 45.6% of them receive nebulisations and 21.3% are hospitalized. The bulk is the first dysfunction of the emergencies service. The second dysfunction is the inappropriate use of this service which works like a first structure. Some affections such as febrile seizures, pyelonephritis are excessively hospitalized in 97.5% and 100% respectively

Conclusion: The dysfunction of pediatric medical emergencies's service can be improved by sanitary education of the parents about the role of emergencie's service and the formation of the doctors in the management of patients suffering from usual emergencies

11-Beta Hydroxylase Deficiency: a clinical study about seven cases

11 beta-hydroxylase deficiency is a rare recessive autosomal disorder. The aim of this report was to describe among a retrospective study of seven cases, different clinical pictures, problems in diagnosis and management. The frequency of 11 beta-hydroxylase deficiency was 17.5% of congenital adrenal hypererplasia etiology in our study. Consanguinity was found in all cases. The sex ratio was 5 boys/2 girls. Median age on diagnosis was 4.3 years. Five cases were revealed with precocious puberty associated with hypertension. One patient had sexual ambiguity, Prader IV stage, hypertension appears later. One patient developed heterosexual precocious puberty and hypertension at five years of age. One patient had bilateral testicular adrenal rests. Hypertension was diagnosed early in 4 cases and secondarly in the other 3 cases. Hypertension was severe complicated by convulsions, facial paralysis and epistaxis. Hypo kaliemia was identified in six cases. Hormonal investigations confirmed diagnosis in all cases. The secondary sexual characteristics were controled by glucocorticoid substitution. Antihypertensive treatment was necessary initially and prolonged only in three cases Prognosis of final height of patients with late diagnosis was particularly compromised

[Hyperlipidaemia associated to inaugurated diabetic ketoacidosis]

Severe hyperlipidaemia in presenting diabetic ketoacidosis was rarely reported in child.We report the case of a three-year-old girl with presenting diabetic ketoacidosis. Family history was negative for hyperlipidaemia. Serum was creamy pink in gross appearance. The child has a hyperlipidaemia with serum triglycerides 18.5 mmo1/1 and serum cholesterol 13.8 mmol/l. The hyperlipidaemia disappeared with insulin therapy within seven days. We study the clinical, laboratory, pathogenic and evolution features of this uncommon trouble in diabetic children

[Chronic septic granulomatosis in children revealed by invasive pulmonary aspergillosis]

Chronic granulomatosis disease [GCD] is a hereditary abnormality of phagocytic cells, frequently revealed by invasive pulmonary Aspergillosis. The authors report the case of 8 year old girl investigates for hypotrophia, recurrent lung disease and hemoptysia. She was treated for pulmonary tuberculosis because of biological inflammatory syndrome, right alveolar, opacity and positive tuberculin skin test. The course was marked by the development of a thoracic wall mass. Anti- aspergillus serology was positive; culture of the thoracic mass puncture and bronchoalveolar lavage isolated fumigatus Aspergillus. GCD was suggested and confirmed by the NBT test