Unusual onset of Graves' disease associated with thymic hyperplasia in a 5-year-old girl with congenital bilateral clinical anophthalmia: diagnostic and therapeutic challenges.

OBJECTIVES: Graves' disease (GD) is a rare auto-immune disorder in pediatric population. The association between GD and thymic hyperplasia was rarely reported in children. Diagnosis and management of GD are challenging in children. CASE PRESENTATION: This report presents the case of a 5-year-old girl with a personal history of asthma and congenital bilateral isolated clinical anophthalmia who presented with acute congestive heart failure, sinus tachycardia and atypical signs of orbitopathy with edema and erythema of the lower right eyelid and excessive tearing. The diagnosis of GD was based on detecting a suppression of serum TSH level and the presence of high titers of TRAbs. Relapse occurred after 10 months of antithyroid drugs with chief complaints of palpitations, dyspnea and dysphagia. Computed tomography showed heterogeneous anterior mediastinal mass with no invasion into the surrounding tissue. The marked shrinkage of the mass after radioiodine therapy supported the diagnosis of thymic hyperplasia associated with GD. CONCLUSIONS: The presence of clinical anophthalmia may be a confusing factor for the diagnosis of Graves' ophthalmopathy. Recognition of the association between GD and thymic hyperplasia would avoid invasive diagnostic procedures and unnecessary surgical resection. Radioiodine therapy may be used in young children with repeated relapses of GD.

Influenza A/H1N1 pdm 09 in children's Hospital Bechir Hamza of Tunis.

BACKGROUND: Influenza A (H1N1) is a contagious acute respiratory infection caused a pandemic in 2009. The outcome was variable among populations. AIM: To describe a clinical spectrum and the outcome of Tunisian children with pandemic H1N1/09 influenza virus. METHODS: This is a retrospective, descriptive study of children with pandemic H1N1/09 influenza virus hospitalized in the children's hospital of tunis, between November 2009 and February 2010. The diagnosis was made on positive rapid test or PCR. RESULTS: thirty two children were included. The median age was 12 months. The most frequently symptoms were: fever (87,5%), digestive disorders ( 59,4%) and dyspnea (15,6%). The mean length of stay was 3,8 days. The outcome was complicated by a bacterial infection (56,3%), and one death. CONCLUSION: Mild form of H1N1/influenza virus is the most common presentation; however severe forms can be observed especially in infants.

Neurological variant of Lemierre's Syndrome with purulent meningitis: a case report and literature review.

We report an unusual variant of Lemierre's Syndrome (LS) in a 10-year-old-girl admitted to the intensive care unit for septic shock with meningitis. The primary infection was otitis media. A gram negative bacillus was identified in the direct exam of the purulent ear discharge and the cerebrospinal fluid but cultures were negative. Computerized tomography of the neck revealed a thrombus in the internal jugular vein. Septic shock improved rapidly under supportive treatment. The patient recovered without sequellae after a prolonged duration of parenteral antibiotherapy and hospital stay. Neurologic variants of LS with meningitis, previously reported in the literature, are reviewed.

Prevalence and risk factors of overweight and obesity in elementary schoolchildren in the metropolitan region of Tunis, Tunisia.

BACKGROUND: Local data about prevalence of obesity in emerging countries are rather scarce. Risk factors for obesity, well known in most industrialized countries, are poorly understood in Tunisia. AIMS: To assess prevalence of overweight and obesity and to investigate associations with possible risk factors in a group of 6-12 year- old schoolchildren in Tunis, Tunisia. METHODS: A descriptive transversal study including a sample of 1335 schoolchildren (6-12 years; mean: 9.7 ± 1.5 years) was conducted in Tunis. Personal and parental data were collected by questionnaires completed by parents. Height and weight were measured and body mass index was calculated. Prevalence of overweight and obesity was defined based on international agreed cut-off points. RESULTS: Prevalence of overweight and obesity was 19.7% and 5.7%, respectively. Risk factors associated with overweight were: high degree- educated mother and father: 17.3% vs 11.7% (p=.01) (OR (95%CI): 1.58; 1.09-2.29) and 26% vs 17.4% (p=.002) (OR: 1.66; 1.21-2.29), respectively; mother, father high in occupational hierarchy: 7.2% vs 3.6% (p=0.009) (OR: 2.1; 1.2-3.7) and 14% vs 9% (p=.014) (OR: 1.6; 1.1-2.48), respectively. Overweight children had a significantly higher consumption of bread (p=.044), of snack intake (p=0.046) and of soft drink consumption (p=.035). CONCLUSIONS: Prevalence of overweight and obesity in this cohort are 19.7% and 5.7%, respectively. Substantial differences in food choices in families with the highest socio-economic status are among risk factors contributing to obesity development.

Nephrotic syndrome and Guillan-Barré Syndrome: a rare association in child.

Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS) have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro-spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he developed severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He remained free of proteinuria during his 20 months of follow-up.

[Study of 116 mothers of the knowledge, behavior, and practices of vitamin D supplementation]. / Enquête cap (connaissances, attitudes, pratiques) auprès de 116 mères concernant la supplèmentation en vitamine D.

BACKGROUND: In our country, the prevention of rickets is based on daily vitamin D intake from birth to eighteen months. This vitamin D intake has decreased according to our hospital practice. AIM: To assess vitamin D supplementation prevalence and to study mother knowledge, attitude and practices about vitamin D and factors that influenced them and to define target population who needs further education. METHODS: A predefined questionnaire was submitted to 116 mother's child who was aged from two months to three years and were hospitalised for acute disease in Tunis children hospital. The questionnaire was filled by the paediatrician. He included as well questions on vitamin D supplementation and questions on sociodemographics characteristics. RESULTS: 68% of the infants had received vitamin D.The various significant factors who influenced the knowledge about vitamin D were father and mother out of work, low education level, and the living in a deserited area. The factors that influenced significantly the practices were the one parity and the living far away, up to five kilometre, from a primary health care. CONCLUSION: Given that the supplementation of vitamin D is recommended for all infants the study show that supplementation prevalence is unsatisfactorily low. Various risks factors were identified. In view of the new evidence emerging on additional preventive properties of vitamin D and the resurgence of rickets, its promotion must be discussed anew.

[Thrombocytopenic idiopathic purpura: predictive factors for chronic disease]. / Facteurs predictifs de passage a la chronicite du purpura thrombopenique idiopathique.

UNLABELLED: THE AIM of this study is to determine factors predicting development of chronic thrombocytopenic idiopathic purpura. METHODS: It was a retrospective study, regarding the cases of PTI diagnosed in "service de medicine infantile C Hôpital d'Enfants de Tunis" during 11 years. A comparison was done between two groups: the first including acute PTI and the second including chronic and recurrent PTI. Factors predicting development of chronic disease were searched by the Fisher test which was significant when p < 0.05. RESULTS: 33 cases of PTI were diagnosed. The ratio sex was 1.06. The mean age was five years and a half. The disease onset was insidious in three cases; all of them have had a chronic course. An haemorrhagic personnel background was present in three cases. Counselling cause was always petechies and ecchymoses. The mean platelets rate was 14555.5/mm3. Therapeutics abstinence was carried out in five cases. 20 infants received an initial corticotherapy. The immunoglobulins were prescribed in nine cases, witch six associated to Corticotherapy. Three modes of outcome were discerned: acute PTI (n = 23), recurrent PTI (n = 4) and chronic PTI (n = 6). Factors predicting development of chronic disease were: haemorrhagic personnel background, an insidious disease onset and the failure of an initial therapeutic abstinence. CONCLUSION: Acute PTI is the most common. The disease had a chronic or recurrent outcome in 1/3 of cases. The search after factors predicting chronic disease allows an early prognosis. Thus, a best management of the disease can be achieved.

[Value of procalcitonin measurement in maternal fetal infection]. / Apport de la procalcitonine dans le diagnostic des infections materno foetales.

BACKGROUND: Materno foetal infection (MFI) remains one of the major causes of neonatal morbidity and mortality. Early detection of neonatal sepsis can be difficult, because the first signs of the disease may be unspecific and similar to symptoms of other non-infectious processes. AIM: We aimed to investigate the role of procalcitonin (PCT) in the diagnosis of fetal infection (MFI), and to compare it with those of the C-reactive protein (CRP). METHODS: We have conducted a prospective study during 20 months: which concerned 25 newborns suspected of MFI and admitted before 12 hours of life. All newborn had anamnestic and/or physical signs of possible infection. MFI was confirmed in newborns with positive bacterial analysis. CRP and PCT were determined in the sera at H12, H24, H36 and H48. Newborns were divided into: patients with recognized MFI (group 1), patients with possible MFI (group2) and non infected newborns (group 3): RESULTS: The specificity of PCT was 80% versus 27% for the CRP. Negative predictive value of PCT was 85% versus 66% for the CRP. The mean values, at H12, H24, H36 and H48, of PCT for newborn who had MFI were statistically grater than those for no infused group (p<0.05). No statistical difference was observed concerning CRP values. CONCLUSIONS: PCT may a useful tool in early diagnosing of MFI; it has better specificity and negative predictive value than CRP.

Feminizing adrenocortical adenoma in a 5 year-old girl.

Adrenocortical adenoma is a rare etiology of isosexual precocious puberty in girls. We report the case of a 5 year-old girl with isosexual precocious puberty. Abdominal ultrasound and magnetic resonance imaging revealed a well-defined mass of 32 x 28 mm situated in the left suprarenal region, but no signs of calcification or metastasis. Estradiol and testosterone levels were elevated, cortisol plasma level was normal, and bolus intravenous injection of GnRH showed unresponsiveness of LH and FSH. Pathological examination after complete surgical resection showed an adrenocortical adenoma. Five years later, there had been no recurrence and abdominal ultrasound examination was normal. The diagnosis of feminizing adrenocortical adenoma was confirmed.

[Cholestasis in infants: a study of the Children's Hospital of Tunisia]. / Les cholestases du nouveau né et du nourrisson: experience de l'Hôpital d'Enfants de Tunis.

BACKGROUND: Cholestasis in infant constitutes an heterogeneous group of disease; diagnosis and management are often difficult. THE AIM of the study is to describe clinical, paraclinical characteristics and outcome of infants hospitalized for cholestasis in children's Hospital of Tunis. METHODS: A retrospective study of 94 infants with cholestasis was conducted. Patients were hospitalized in four departments of paediatrics of our hospital between January 1995 and December 2005. Cholestasis complicating severe sepsis and visceral leishmaniasis were excluded. RESULTS: Incidence of cholestasis was 8.5 cases/year which represented 0.72% of the hospitalizations. Sex ratio was 1.08 and mean age at diagnosis was 105 days (extremes: 1 day- 24 months). Biliary atresia was the most common cause of extra hepatic cholestasis (13.8%). Normal A GT cholestasis (11.7 %), benign neonatal cholestasis (11.7%) and bile duct hypoplasia (9.5%) represented the most common aetiologies of intra hepatic cholestasis. Aetiology remained unknown in 12.7% of cases. Only three infants with biliary atresia had Kasaï operation. After a mean follow-up of 6 years, 18% of patients had portal hypertension, 14.8% had hepatic failure and mortality rate was 14.8%. CONCLUSION: Cholestasis of unknown aetiologies are frequent in our hospital. Poor prognosis, in our study, is due to delay to diagnosis and difficulties in medical and surgical management.

[Diagnosis of hyper transaminasemia in childhood]. / Conduite a tenir devant une elévation des transaminases chez l'enfant.

Elevated serum levels of transaminases must always be considered as an abnormal finding in children. Drugs and toxins must be eliminated first as possible hepatotoxic agents or co-factors. Antiviral hepatitis A immunoglobulin M serology is the first test to perform. However, others viruses with spontaneous benign courses are the most frequent cause. Only if initial presentation is severe or if liver tests remain abnormal after several weeks, other rare diseases can be sought. Aetiologies of persistent cytolysis associated to cholestasis are different in infancy and childhood. Metabolic, auto immune, genetic, muscular, endocrine disorders and obesity may cause isolated persistent hyper transaminasemia. Early diagnosis and management is essential. Isolated elevation of serum amino transferases in healthy looking children with negative investigations is mostly a benign condition that usually resolves within a year. Liver biopsy is does not contribute much to diagnosis and is probably unnecessary.

Etiology and outcome of bronchiectasis in children: a study of 41 patients.

BACKGROUND: Bronchiectasis remains an important cause of chronic suppurative lung disease in the developing world. The aim of this study is to describe the epidemiological characteristics, clinical features, underlying aetiologies and outcome of bronchiectasis in the paediatric hospital of Tunis. METHODS: 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). RESULTS: Mean age at diagnosis was 5 years 9 months; (range: 6 months-14 years). Persistent cough and bronchorrhea 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. CONCLUSIONS: 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.

[Helicobacter pylori infection in childhood revealed by hematemesis: endoscopic and pathologic patterns]. / L'infection a Helicobacter pylori chez l'enfant revelee par une hemorragie digestive: aspects endoscopiques et histologiques la Tunisie.

BACKGROUND: Helicobacter pylori infection frequency in hematemesis was scarcely studied. AIM: to asses the frequency of this infection in children with upper gastrointestinal bleeding and to study the endoscopic and histological features. METHODS: It is a retrospective study including 180 children who underwent an endoscopy for upper gastrointestinal bleeding. Our population was divided in two groups. The group 1 (n=95) has performed gastric biopsy. The group 2 (n=95) has'nt performed gastric biopsy. For each group, we studied the personal and familial history of gastroenterologic disease, the hospital where they come from, the importance of bleeding, the drug intake effecting the gastric mucosa, the endoscopic and histological features. RESULTS: The helicobacter pylori infection was present in 48% of the children. The mean age of these children was 99.8 +/- 42.1 months versus 95.7 +/- 44 months (p=0.13) The comparison of the two groups according to Hp infection, and the others parameters don't found any differences. All the infected children have chronic gastritis 40/40 versus 13/44 in the non infected children (p>10 -6). CONCLUSION: The frequency of Hp infection was high in this group of patients with upper gastrointestinal bleeding. It was probably underestimated because the investigation was not complete. We emphasize that Hp infection has to be investigated and systematically eradicated whenever there were severe symptoms like hematemesis indicating therefore organic disease.