Correction to: Full title: peripheral venous catheter complications in children: predisposing factors in a multicenter prospective cohort study.

Following publication of the original article [1], one of the authors flagged that the title of the article was submitted (incorrectly) with "Full title:" at the beginning.

Peripheral venous catheter complications in children: predisposing factors in a multicenter prospective cohort study.

BACKGROUND: Peripheral venous catheterization (PVC) is frequently used in children. This procedure is not free from potential complications. Our purpose was to identify the types and incidences of PVC complications in children and their predisposing factors in a developing country. METHODS: We conducted a prospective observational multicenter study in five pediatric and pediatric surgery departments over a period of 2 months. Two hundred fifteen PVC procedures were conducted in 98 children. The times of insertion and removal and the reasons for termination were noted, and the lifespan was calculated. Descriptive data were expressed as percentages, means, standard deviations, medians and interquartile ranges. The Chi2 test or the Fisher test, with hazard ratios and 95% confidence intervals (CI95%), as well as Student's t test or the Mann-Whitney U test were used to compare categorical and quantitative variables, respectively, in groups with and without complications. The Spearman test was used to determine correlations between the lifespan and the quantitative variables. The Kruskal Wallis test was used to test for differences in the median lifespan within 3 or more subgroups of a variable. Linear regression and logistic binary regression were used for multivariate analysis. A p-value <0.05 was considered significant. RESULTS: The mean lifespan was 68.82 ± 35.71 h. A local complication occurred in 111 PIVC (51.9%) cases. The risk factors identified were a small catheter gauge (24-gauge) (p = 0.023), the use of a volume-controlled burette (p = 0.036), a longer duration of intravenous therapy (p < 0.001), a medical diagnosis of respiratory or infectious disease (p = 0.047), the use of antibiotics (p = 0.005), including cefotaxime (p = 0.024) and vancomycin (p = 0.031), and the use of proton pump inhibitors (p = 0.004).The lifespan of the catheters was reduced with the occurrence of a complication (p < 0.001), including the use of 24-gauge catheters (p = 0.001), the use of an electronic pump or syringe(p = 0.036) and a higher rank of the intravenous device in each patient (p = 0.010). CONCLUSIONS: PVC complications were frequent in our pediatric departments and are often associated with misuse of the device. These results could engender awareness among both doctors and nurses regarding the need for rationalization of the use of PVC and better adherence to the recommendations for the use of each drug and each administration method.

A Founder Effect of c.257 + 2T > C Mutation in NCF2 Gene Underlies Severe Chronic Granulomatous Disease in Eleven Patients.

Chronic granulomatous disease (CGD) is the prototypic functional neutrophil disorder caused by genetic defects in one of the five genes encoding the superoxide-generating nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase subunits of phagocytes. Mutations causing the most prevalent form of CGD in western populations are located in the X-linked-CYBB gene. The four remaining autosomal recessive (AR) forms collectively account for one-third of CGD cases. We investigated the clinical and molecular features of eleven patients with CGD from 6 consanguineous families, originating from contiguous regions in the west of Tunisia. The patients' clinical phenotype is characterized by a high incidence of mycobacterial infections. Five out of the eleven patients died despite treatment arguing in favor of a severe clinical form of CGD. These findings correlated with the absence of functional p67phox protein as well as the absence of residual reactive oxygen intermediates (ROI) production. Genetic analysis showed the presence, in all patients, of a unique mutation (c.257 + 2T > C) in NCF2 gene predicted to affect RNA splicing. Segregating analysis using nine polymorphic markers overlapping the NCF2 gene revealed a common haplotype spanning 4.1 Mb. The founder event responsible for this mutation was estimated to have arisen approximately 175 years ago. These findings will facilitate the implementation of preventive approaches through genetic counseling in affected consanguineous families.

Soft Tissue Myoepithelial Carcinoma in a Child: Case Report.

BACKGROUND: Myoepithelial carcinoma was only recently recognized to occur primarily in soft tissue. Only a small number has been reported in children. OBSERVATION: We report a rare case of myoepithelial carcinoma of the leg in a 4-month-old boy with a good response to chemotherapy initially. However, he presented secondarily during chemotherapy a local and metastatic progression. CONCLUSIONS: The rarity of the tumor and unusual age of discovery have prompted us to report this case. Our case suggests that this disease can have an aggressive behavior. This is why we advise a rapid and correct diagnosis followed by an aggressive treatment.

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.

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.

[Cystic fibrosis: A report of 33 pediatric Tunisian cases]. / La mucoviscidose chez L'Enfant Tunisien: a porpos de 33 observations.

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.

Childhood acute lymphoblastic leukemia in the Middle East and neighboring countries: a prospective multi-institutional international collaborative study (CALLME1) by the Middle East Childhood Cancer Alliance (MECCA).

BACKGROUND: Little is known about childhood ALL in the Middle East. This study was undertaken by MECCA as initial efforts in collaborative data collection to provide clinical and demographic information on children with ALL in the Middle East. PROCEDURE: Clinical and laboratory data for patients with ALL between January 2008 and April 2012 were prospectively collected from institutions in 14 Middle East countries and entered into a custom-built-database during induction phase. All laboratory studies including cytogenetics were done at local institutions. RESULTS: The 1,171 voluntarily enrolled patients had a mean age of 6.1 ± 3.9 years and 59.2% were boys. T-ALL represented 14.8% and 84.2% had B-precursor ALL. At diagnosis, 5.6% had CNS disease. The distribution of common genetic abnormalities reflected a similar percentage of hyperdiploidy (25.6%), but a lower percentage of ETV6-RUNX1 translocation (14.7%) compared to large series reported from Western populations. By clinical criteria, 47.1% were low/standard risk, 16.9% were intermediate risk, and 36% were high risk. Most patients received all their care at the same unit (96.9%). Patients had excellent induction response to chemotherapy with an overall complete remission rate of 96%. Induction toxicities were acceptable. CONCLUSIONS: This first collaborative study has established a process for prospective data collection and future multinational collaborative research in the Middle East. Despite the limitations of an incomplete population-based study, it provides the first comprehensive baseline data on clinical characteristics, laboratory evaluation, induction outcome, and toxicity. Further work is planned to uncover possible biologic differences of ALL in the region and to improve diagnosis and management.

Treatment of nephroblastoma in Africa: results of the first French African pediatric oncology group (GFAOP) study.

BACKGROUND: The multidisciplinary management of nephroblastoma has been defined through multicentric prospective studies and an average 90% of patients cured expected. In Africa, such studies are uncommon and results are fragmentary or unknown in most of the countries. We report the results of the GFAOPNEPHRO 01 study using SIOP 2001 protocol approach. PROCEDURE: From April 1, 2001 to March 31, 2004, 8 African Pilot Units were selected to participate in a nonrandomized prospective study. All patients referred with a clinical and radiological diagnosis of nephroblastoma were registered, those aged over 6 months and less than 18 years with a unilateral tumor not previously treated were included in this study and received preoperative chemotherapy. Patients with unfavorable histology or with a tumor other than Wilms tumor, and those with stage IV tumor and persistent disease after surgery were secondarily excluded. RESULTS: Of the 229 patients initially registered, 166 were included and finally 133 retained in the study, after surgery. Tumor rupture occurred in 7.5% of the patients. Thirty-five percent were stage I, 22% stage II, 23% stage III, and 18% stage IV. Two-year disease-free survival and 5-year survival are, respectively: 77.9% and 76.7% for localized tumors, 72.7% and 71.6% for all study patients. CONCLUSIONS: It is possible to conduct African multicentric therapeutic studies within the framework of GFAOP. Results in terms of event-free survival and survival are satisfactory. Improvements with respect to procedure, data collection, and outcome are expected in a new study.

Pseudotumoral cutaneous aspergillosis in chronic granulomatous disease, report of a pediatric case.

Invasive aspergillosis is a life-threatening condition in patients with chronic granulomatous disease (CGD). Skin invasion by Aspergillus occurs most commonly by contiguity to a neighboring cavity. We describe an unusual case of invasive cutaneous aspergillosis presented as a large burgeoning tumor in a 4-year-old girl with CGD who underwent surgical treatment for bifocal osteomyelitis of the left leg. The skin invasion occurred 4 months after a "successful" treatment of invasive pulmonary aspergillosis. Atypical presentation and diagnostic difficulties are discussed. Invasive cutaneous aspergillosis may be polymorphic. The diagnosis should be considered early in the etiological investigation of any suspicious skin lesions in CGD even in uncommon aspects such as burgeoning tumors.

[Granulocytic sarcoma of the maxillary sinus: a pediatric case report]. / Sarcome granulocytaire du sinus maxillaire. À propos d'un cas pédiatrique.

BACKGROUND: Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvement of the head and neck region is rare, generally concerning the orbit. AIM: To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. CASE: 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. CONCLUSION: 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.

Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs.

AIM: To establish a preliminary national report on clinical and genetic features of cystic fibrosis (CF) in Tunisian children as a first measure for a better health care organization. METHODS: All children with CF diagnosed by positive sweat tests between 1996 and 2015 in children's departments of Tunisian university hospitals were included. Data was recorded at diagnosis and during the follow-up from patients' medical records. RESULTS: In 12 departments, 123 CF children were collected. The median age at diagnosis was 5 months with a median diagnosis delay of 3 months. CF was revealed mostly by recurrent respiratory tract infections (69.9%), denutrition (55.2%), and/or chronic diarrhea (41.4%). The mean sweat chloride concentration was 110.9mmol/L. At least one mutation was found in 95 cases (77.2%). The most frequent mutations were Phe508del (n=58) and E1104X (n=15). Fifty-five patients had a Pseudomonas Aeruginosa chronic colonization at a median age of 30 months. Cirrhosis and diabetes appeared at a mean age of 5.5 and 12.5 years respectively in 4 patients each. Sixty-two patients died at a median age of 8 months. Phe508del mutation and hypotrophy were associated with death (p=0.002 and p<0.001, respectively). CONCLUSION: CF is life-shortening in Tunisia. Setting-up appropriate management is urgent.

[Tuberculosis in childhood: clinical features and problems in diagnosis. Report of 30 cases]. / La tuberculose de l'enfant: aspects cliniques et problèmes diagnostiques, a propos de 30 observations.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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 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.

Treatment of B-cell lymphoma with LMB modified protocols in Africa--report of the French-African Pediatric Oncology Group (GFAOP).

BACKGROUND: The French African Paediatric Oncology Group (GFAOP) was set up in October 2000 to improve the quality of care of children with cancer in Africa. Eight pediatric oncology units from Algeria, Cameroon, Madagascar, Morocco, Tunisia, and Senegal have been involved. METHODS: Patients less than 18 years with cytology or histology proven B-cell non-Hodgkin lymphoma were included. Two LMB89 modified regimens were proposed (MAT and GFA). RESULTS: From April 2001 to April 2004, 343 cases were registered. Thirty seven patients were excluded. Thirteen patients were stage I, 26 stage II, 209 stage III and 50 stage IV including 8 L3 acute lymphoblastic leukemia (ALL3) cases. Three year OS of the whole population of patients is 61%. In GFA group 36 months OS is 63.6% in stages I/II, 51.6% in stage III and 35.8% in stage IV. In MAT group, the OS is 84.4% in stages I/II, 76.2% in stage III and 55.6% in stage IV. Seventy one patients died during treatment, 32 at pre-induction phase, 27 at induction and 12 at consolidation. Treatment related mortality decreased during the 3-year inclusion period (first year: 25.7%, second year: 19.1%, third year: 11.6%). The improvement of supportive care translated into an increase of the overall survival rates from 54% in the first year to 73% in the third year. CONCLUSION: These data demonstrate the feasibility of prospective multicentric studies in Africa. An improvement of quality of care has been noticed during the 3 first years.

[Clear-cell sarcoma of the kidney. Two pediatric cases]. / Sarcome à cellules claires du rein. A propos de deux cas pédiatriques.

Clear-cell sarcoma of the kidney is a rare pediatric renal sarcoma with poor prognosis and propensity to metastasize to bone. It is a distinctive renal malignancy regarded as a morphologic feature of Wilms' tumor. We report two cases occurring in four-year and 12-month-old boys, explored for abdominal masses. They were treated by surgery and chemotherapy. The histological diagnosis was clear cell sarcoma. Patients were doing well at 10 and seven months respectively.

[Cholestasis caused by a choledochal botryoid rhabdomyosarcoma in a 22-month-old boy]. / Une choléstase causée par un rhabdomyosarcome botryoïde du cholédoque chez un nourrisson de 22 mois.

Botrioid rhabdomyosarcoma of the extrahepatic bile ducts is a rare cause of jaundice in children. It has a very poor prognosis and is rarely diagnosed preoperatively. We report a choledochal botrioid rhabdomyosarcoma in a 22-month-old boy who developed an obstructive jaundice. Radiographic explorations suggested cystic lymphangioma. The gallbladder, the cystic duct, the common bile duct and the pancreatic head were resected. The diagnosis was made on pathological examination; adjuvant chemotherapy followed. The patient was disease free 20 months following treatment.

[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.

[Treatment of upper gastrointestinal bleeding in portal hypertension in children]. / Traitement des hémorragies digestives dans l'hypertension portale de l'enfant.

Variceal bleeding is the most common cause of severe gastrointestinal bleeding in chilhood. Biliary atresia and portal venous obstruction are the most common causes. The patients with acute bleeding, pharmacotherapy with octreotide should be used immediately. All of these patients should undergo therapeutic endoscopy with band ligation. All children surviving variceal hemorrage should undergo secondary prophylaxis with band ligation. Transjugular intrahepatic portosystemic shunt creation in children is as safe in children than in adults. The primary prophylaxis in children with oesophageal varices is controversed.