[Community-acquired pleuropneumonia in children: bacteriological and therapeutic challenges]

Background: Community-acquired pleuropneumonia [CPP] is a common complication of pneumonia in children. It is serious given its high morbidity and significant mortality

Aim: To study clinical and paraclinical features of CPP in children and to establish a common therapeutic strategy

Methods: Our retrospective study included patients who were hospitalized for CPP between 2004 and 2012. All data were collected from patients' medical files. Statistical analysis was made by Epi-Info 6

Results: One hundred and sixty four patients were registered. The mean age was 32 months [15 days - 14.5 years]. The hospital incidence of CPP doubled between 2004 and 2012. The symptomatology was dominated by fever [93.9%], cough [56.7%] and dyspnea [48.1%]. The pleural effusion was frequently moderately abundant and loculated. Pleural sample, performed in 53.6% of cases, was the most beneficial bacteriological examination [p=10-6 ]. The bacteriological confirmation was attained in 44.5% of cases with the predominance of Staphylococcus aureus [59%] followed by Streptococcus pneumoniae [26%]. The S. aureus occurred basically in most young infants [p=0.04] and was responsible for the most severe cases [p=0.01]. The CPP management included heterogeneous intravenous antibiotics associated with a pleural drainage in 40% of cases. The quarter of our patients were transferred to an intensive care unit. Six patients died

Conclusion: The bacteriological confirmation is difficult. Pleural aspiration is the key tool. S. aureus is the first microorganism followed by S. pneumoniae. A therapeutic strategy is proposed based on large spectrum intravenous antibiotics. The pleural drainage indication is limited

Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study

Background: Extracranial Germ cell tumors [GCT] are a rare and a heterogeneous group of pediatric cancers but highly curable

Aim: We aimed to review management, outcome and prognostic factors that influence overall survival [OS] in a pediatric Tunisian oncologic unit

Methods: We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit

Results: Patients had a mean age of 57 months [ranges: 1 day-13 years]. There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months [ranges: 0-96 months], OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage [p=0.04], the completeness of surgery [p<0.001] and the relapse [p = 0, 0001]. A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors [p=0,021] for 5-year OS

Conclusion: Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments

[Cystic fibrosis: a report of 33 pediatric Tunisian cases]

Background: The frequency of cystic fibrosis is unknown in Tunisia, regarding the limited number of reported surveys and patients

Aim: to determine the clinical characteristics, outcome and genetic data of cystic fibrosis in Tunisian pediatric patients

Methods: Cases of cystic fibrosis managed at pediatric departments of Tunis, during 15 years [1997-2012], were reviewed

Results:33 children [23 males and 10 females] were enrolled. The Onset was within the first year of life in 26 patients. Revealing symptoms were the following: recurrent bronchopneumonia [28 cases], chronic diarrhea [17 cases], hepatomegaly [6 cases], malnutrition [15 cases], pseudo Bartter syndrome [3 cases], edemaanemia- hypoprotidemia [4 cases] and meconium ileus [4 cases]. The diagnosis was confirmed by sweat test and genotypic data, the F508 del was the most frequent mutation [17 cases]. Several complications had occurred during follow-up: chronic pseudomonas aeruginosa infection [15 cases], chronic respiratory failure [14 cases], recurrent hemoptysis [2 cases], pleural effusion [3 cases] and cirrhosis [2 cases]. Ten patients died at a mean age of 7 years. One patient had pulmonary transplantation. Prenatal diagnosis was performed in 9 families

Conclusion: In Tunisia, cystic fibrosis is not exceptional, but its diagnosis is delayed. Our survey is characterized by more severe earliest forms, difficult and insufficient therapeutic management. A Better medical awareness and a national action plan are needed

[Granulocytic sarcoma of the maxillary sinus: a pediatric case report]

Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvment of the head and neck region is rare, generally concerning the orbit. To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. We report a case of maxillary sinus granulocytic sarcoma in a 13-month old boy revealed by facial nerve palsy, ptosis and jugal swelling, without any evidence of haematological disorders. The patient underwent computed tomography and magnetic resonance imaging exam which demonstrated a non specific maxillary sinus mass with destruction of the orbit floor and the alveolar bone. Diagnosis was obtained after bone biopsy allowing histological and immunohistochemical studies. Granulocytic sarcoma is a serious condition because of the associated hematologic disorders. In a sinus or orbit location, imaging features are non specific. Unless hematologic history is present, diagnosis is difficult and an immuno histo chemical study is required

[Tuberculosis in childhood: clinical features and problems in diagnosis. report of 30 cases]

The clinical polymorphism and the low yield bacteriological tests make the diagnosis of tuberculosis [TBC] in children often difficult. The aim of this report is to specify hospital incidence of childhood TBC and to discuss problems in diagnosis. We reviewed retrospectively cases of TBC enrolled at Medicine A Department in Children's Hospital of Tunis during the last ten years [1998 - 2007]. Diagnosis of TB was supported according to bacteriological or histological confirmation or regarding the association of epidemiological data [TB contagium], clinical and radiological findings and favourable outcome with anti tuberculous drugs. Thirty children had TBC. They were 18 girls and 12 boys. The main age at diagnosis was 8. 6 years [3 months-14 years]. All children were vaccinated with BCG. Thirteen patients had definite familial history of TBC contact. Tuberculin-skin test was positive in 15 patients. The diagnosis was supported within a mean period of 44 days [8, 240 days]. Pulmonary TBC occurred in five patients and extra-pulmonary TBC in 25. Four patients had more than two TBC localizations. Miliary and TBC meningitis occurred in seven patients. The rate of diagnosis confirmation was 40%. Clinical outcome improved in 29 children with anti tuberculosis therapy while one infant died with miliary TBC. Five patients developed pleural, neurological or bone sequelaes and another patient presented autoimmune bicytopenia, diffuse bronchectasis and pulmonary aspergillosis. TBC occurs in 0, 91/ year/1000 hospitalized children in our institution. Low diagnosis confirmation rate was observed with infants and in pleural and primary TBC. Although all patients received BCG vaccine, 23. 3% of them developed a life-threatening form of TBC

[Study [knowledge, behaviour practices] of 116 mothers on vitamin D supplementation]

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. 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. 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 socio- demographics characteristics. 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 kilometer, from a primary health care. 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

[Neonatal heart failure revealing a cardiac rhabdomyoma with tuberous sclerosis]

Cardiac rhabdomyoma is the most common cardiac tumour in childhood and is often associated to tuberous sclerosis [TS]. However, a neonatal cardiac failure was uncommonly reported as the first manifestation of tuberous sclerosis. We report a case of a cardiac tumour revealed by a heart failure in a newborn who developed later clinical manifestations of TS. We report a case of a seven-day-old girl, admitted for cardiac failure. Echocardiography discovered a voluminous single mass in the right ventricle that affected partially heart output consistent with rhabdomyoma. Clinical course improved with symptomatic treatment. Seven months later, she developed generalized seizures associated to depigmental macules and facial neuro-fibroms. Cerebral magnetic resonance imaging showed cerebral hamartoms. Subsequent eight years follow-up revealed a spontaneous regression of cardiac rhabdomyoma and no further cardiac manifestations in contrast to a slight debility and a partial control of epilepsy. A cardiac rhabdomyoma discovered in neonates is a good indicator of TS suggesting careful follow-up and management

[Cholestasis in infant: a study of the children's hospital of Tunis]

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. 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. 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 Kasai 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%. 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

Metaiodobenzylguanidine scintigraphy in neu-roblastoma

The aim of this study is to evaluate meta-iodo-benzyl-guanidine scintigraphy in the diagnosis and the management of neuroblas-toma. betwen September 1999 and jun 2004, 16 children aging from 4 months to 6 years, with neuroblastoma, underwent mlBG scintigraphy in military hospital nuclear medicine department. 23 mlBG scans were done. The exam was indicated for the initial staging in 7 cases, to evaluate chemotherapy or surgery in 12 cases and to seek for reccurences in 3 cases. The sensitivity of mlBG was 57% in the detection of the primary tumor, 100% for liver metastases and 67% for medullar involvement. mlBG scintigraphy should be a standard in the initial staging of neuroblastoma and in evaluating therapy response

[Upper gastrointestinal endoscopy in Neonates -experience of pediatric gastroenterology unit]

The aim of our study was to assess the frequency of the dif-ferent lesions occurring as well as to precise indications of upper gastrointestinal endoscopy in neonates

Method: We have achieved a retrospective study about 128 neonatal gastrointestinal endoscopies. Three groups were constituted according to macroscopic findings:- Group I: normal aspect [n=1]- Group II: isolated esophagitis [n=19]- Group III: esogastritis or gastroduodenitis or esogastroduo-denitis [n=92

Results: The neonates undergoing endoscopy for malaise were more frequent in group I than in group II and III, respectively 36.5% versus 15.8% and 9.8% [P = 0.04]. Digestive hemorrhage [hematemesis and/or melena] was more frequent in group III than in group II and I, respectively 90.2% versus 78.9% and 63.6% [P = 0.03], Digestive hemorrhage was in our study the main indication of upper gastrointestinal endoscopy in neonates [85.9%] which showed a macroscopic lesion in 93.5% of cases

Conclusion: Hematemesis and suspicion of esophagitis are good indications for upper gastrointestinal endoscopy in neonates

[Congenital hepatic fibrosis in children: about 9 cases and review of the literature]

Congenital hepatic fibrosis is a recessive autosomic disease with two major risks: gastrointestinal haemorrhage caused by portal hypertension and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. The aim of our study is to define epidemiological features, the presenting symptoms, the diagnosis, the evolution and the management of this disease. Between January 1990 and December 2000, we reported the cases of nine children with this disease at children hospital of Tunis.Three were male and six female. The mild age was three years and six months. Consanguinity was present in five cases and similar cases were found in six cases. The FHC was revealed by portal hypertension in five cases, angiocholitis in one case and by portal hypertension and angiocholitis in three cases. Liver biopsy was done in seven children. Ultrasound examination of the liver and kidney revealed caroli syndrome in five cases and polykystose renal in two cases The intravenous pyelography was performed in four cases showing precalicial canalicular ectasia in four cases. Eosophageal endoscopy had shown oesophageal varices in six patients. The follow up had shown that three patients had gastrointestinal bleeding, three had angiocholitis. One patient died with multivisceral failure. The treatment of acute bleeding has needed blood transfusion in four cases. Primary prevention of bleeding was done by endoscopic sclerosis alone in one case and associated to betablokers in two cases. Secondary prevention of varices bleeding was done by sclerotherapic in two cases, by beta blokers alone in one case and by betablokers associated to elastic ligation of oesophageal varices in one case

Analyse des modalites therapeutiques et evolutives des oesophagites caustiques severes chez l'enfant traites par les bolus de methyl prednisolone experience d'une unite d'endoscopie digestive

We reviewed the case histories of 28 children seen at children hospital from 31 December 1991 to 31 December 2001. These children has second and 'third degree oesophageal burns and they were treated by systemic Methylprednisolone [1000mg/1,73/m2SC]. We divided the 26 children in four groups according to the time we began the steroids [before or, beyond the 24th hours of the accident and according the number of steroids's bolus [less or more than 21 bolus].we analysed the number and the treatment of stricture in each group. High doses of methyl prednisolone seem to decrease the risk of oesophageal stricture. We found no difference between the children treated before the 24th hours and those treated after the 24 hours and those treated with less than 21 bolus and those with more than 21 bolus

Osteoarthropathie hypertrophiante revelant un thymome malin chez un enfant. Apport de l'imagerie

Association of hypertrophic osteoarthropathy and malignant thymoma is very rare in children. Authors report a case of a 14 year old girl followed for rheumatic fever since she was 8 years old. She was explored for arthralgia. The exam showed Hippocratic fingers, soft tissues hypertrophy of lower limbs and dyspnoea. Plain radiography had demonstrated symmetrical ossifying periostisis in tubular bones of lower limbs evocating hypertrophic osteoarthropathy diagnosis. Chest radiography has demonstrated bilobate anterior mediastinal mass. Ultrasonography and MRI revealed a voluminous anterior mediastinal mass with cystic and fleshy components. Thymic adenocarcinoma diagnosis was made by surgical biopsy