Pediatric Noncerebral Thromboembolism: Etiological Assessment and Outcome.

OBJECTIVE: To report clinical presentation and etiologic investigation findings during pediatric noncerebral thromboembolism. METHODS: Retrospective study of cases of vascular non cerebral thromboses admitted in Medicine infantile A Department of the Children's Hospital of Tunis over 08 years. RESULTS: We confirmed 14 cases of non cerebral vascular thromboses. So that these accidents constitute 0,26 ‰ of the overall etiologies of hospitalizations in the Department. The mean age of our patients was 56±41 months [25 days-12 1/2 years]. The sex ratio was 1.8. The vascular incident was venous in 2/3 of cases. The clinical presentation was mainly painful swelling in four cases, abrupt dyspnea and hematemesis in three cases each and the incident was locally asymptomatic in four cases. Thromboses locations included deep vein thrombosis of limbs (n=6), vena cava thrombosis (n=1), portal thrombosis (n=4) and pulmonary embolism (n=3). The promoting factors identified were: tumors in seven cases, thrombophilias and catheterization in four cases each, trauma, surgery and Behçet disease in one case each. Eleven patients received anticoagulant treatment including unfractioned heparin in three cases and low molecular weight heparin in the other cases. No one died while four patients developed sequelae. CONCLUSION: Vascular thromboses are rare in children. They are mostly venous and diagnosed in ill children especially those having central venous catheters. Outcome of pediatric thromboembolism depends on efficient anticoagulation therapy which is well tolerated by children.

Molecular identification of Gd A- and Gd B- G6PD deficient variants by ARMS-PCR in a Tunisian population.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymopathy. More than 200 mutations in the G6PD gene have been described. In Tunisia, the A-African and the B-Mediterranean mutations predominate the mutational spectrum. The purpose of this study was to apply the amplification refractory mutation system (ARMS-PCR) to the identification of Gd A+, Gd A- and Gd B- variants in a cohort of deficient individuals and to establish a phenotype/genotype association. 90 subjects were screened for enzymatic deficiency by spectrophotometric assay. The molecular analyses were performed in a group of 50 unrelated patients. Of the 54 altered chromosomes examined, 60% had the Gd A- mutation, 18% showed the Gd B- mutation and in 20% of cases, no mutations have been identified. The ARMS-PCR showed complete concordance with the endonuclease cleavage reference method and agreed perfectly with previous Tunisian studies where Gd A- and Gd B- were the most encountered. Also, similarities in spectrum mutations with North African and Mediterranean countries suggest gene migration from Africa to Europe through Spain. In conclusion, ARMS has been introduced in this study for common G6PD alleles identification in Tunisia. It gives some advantages compared to the traditional endonuclease digestion method since it is more convenient and timesaving and also offers the possibility to be applied in mass screening surveys.

[Management of purpura fulminans apropos of 21 cases: diagnostic delay and therapeutic problems]. / Prise en charge du purpura fulminans a propos de 21 cas: retard diagnostique et problemes therapeutiques.

AIM: To evaluate the diagnostic delay and therapeutic insufficiencies delay before the transfer in ICU of the children admitted in the ICU of the children's hospital of Tunis with a purpura fulminans (PF). METHODS: A retrospective, descriptive study, of children with PF referred between January 2000 and January 2006 to a the paediatric intensive care unit (PICU) of the children's hospital of Tunis. The PF diagnosis was retained in any child presenting a feverish purpura and circulatory insufficiency signs. The optimal diagnostic and therapeutic charge taking was defined in three levels: parental, the first line doctors, and the hospital doctors. The symptoms' duration and the various treatments which were lavished to the patients were taken from the medical observations of the patients transferred in our PICU. RESULTS: Twenty one observations were collected. Twelve patients (57.1%) were addressed by a doctor exerting in a dispensary or by a free practicing doctor, 5 patients (23.8%) were transferred from a regional hospital and 4 children (19.1%) directly consulted the children hospital of Tunis urgencies delayed parental recognition occured in 11 children. The PF diagnosis was not evoked by the first line doctor in 62%. Eleven (52.4%) of the children with meningococcal disease were seen but not admitted by a doctor in the 48 hours before admission. Apart from 2 patients (9.5%) who were hospitalized in reanimation directly of the urgencies, all the other patients forwarded by a general pediatry service. In general pediatry, the PF diagnosis was not evoked in 3 cases (15.8%), 31.6% of patients had unnecessary a lumbar punctures and shock was not recognised or treated in 26.3%. Twelve patients (52.2%) died. The duration of hospitalization in general pediatry is significantly higher among deceased patients (5.5 +/- 6.6 hours) than among the surviving patients (2.6 +/- 1.5 hours); p < 0.05. CONCLUSION: Suboptimal treatement in PF is due to failure of parents, general practioners and hospital doctors to recognise specific features of the illness. Improvement in outcome could be achieved by public education and better training of clinicians in recognition, resuscitation, and stabilisation of seriously ill children.

[Acute respiratory distress syndrome in infants at term and near term about 23 cases]. / Le syndrome de détresse respiratoire aigue du nouveau-né à terme et proche du terme: à propos de 23 observations.

AIM: to precise the incidence of acute respiratory distress syndrome in infants at term and near term and the clinical characteristics of this disease not yet well recognised. METHODS: Retrospective study of the medical records of infants admitted in the paediatric intensive care unit for ARDS along a period of 4 years. Diagnosis of ARDS was based on the following criterias: (1) Gestational age > or =35 weeks of gestation; (2) Severe and immediate respiratory distress requiring mechanical ventilation with PEEP > or =4 cmH2O and FiO2 > or = 0.5 during at least 6 hours; (3) Dependence on oxygen > or =48 hours ; (4) Diffuse alveolar damage in the chest radiograph; (5) PaO2 < or =60 mmHg under FiO2 > or = 0.5. RESULTS: During the period of the study, 23 infants (gestational age = 36 +/- 1.1 weeks of gestation; birth weight = 2756 +/- 453 gr) were included in the study. Their mean age at admission was 16.5 +/- 14.6 hours. The majority of infants (91.3%) were born by caesarean section before the onset of labour in 78.3% cases. All infants had a severe acute hypoxemic respiratory failure (D(A -a)O2 = 468 +/- 165; OI = 19 +/- 8.4). Five infants (21.7 %) improved their oxygenation parameters under conventional mechanical ventilation (CMV) (p < 0.001; p = 0.002 et p = 0.003 respectively for D(A - a)O2, OI and PaO2/FiO2). Eighteen infants (78.2%) required high frequency oscillatory ventilation (HFOV)with a rapid and persistant improvement of oxygenation parameters. Five patients (21.7%) developed pulmonary air leak. One infant died. CONCLUSION: ARDS represents 6.8% of etiology of respiratory distress in infants at term and near term. Caesarean section before the onset of labour seems to be a triggering factor. The outcome seems to be favourable (rate of survival = 95.7%) if the management is suitable.

High-frequency oscillatory ventilation in paediatric patients with acute respiratory distress syndrome--early rescue use.

UNLABELLED: In order to determine the response to high-frequency oscillatory ventilation (HFOV), used as an "early rescue" therapy, in a cohort of paediatric patients with acute respiratory distress syndrome (ARDS), a prospective clinical study was performed in a tertiary care paediatric intensive care unit. Ten consecutive patients, aged 12 days to 5 years with ARDS and hypoxaemic respiratory failure on conventional ventilation (CV), using a lung protective strategy, were managed with HFOV early in the course of the disease process (median length of CV 4 h). Arterial blood gases, oxygenation index (OI), alveolar-arterial oxygen difference (P(A-a)O2) and PaO2/FIO2 ratio were prospectively recorded prior to HFOV (0 h) and at predetermined intervals throughout the course of the HFOV protocol. There was a significant improvement in PaCO2 4 h after institution of HFOV (P = 0.012). A significant and sustained increase (P < 0.001) in PaO2/FIO2 ratio and a significant and sustained decrease (P < 0.001) in OI and P(A-a)O2 were demonstrated during the HFOV trial. These improvements were achieved 4 h after initiating HFOV (P < 0.05). Eight patients survived. There were no deaths from respiratory failure. CONCLUSION: In paediatric patients with acute respiratory distress syndrome and hypoxaemic respiratory failure on conventional ventilation, using a lung protective strategy, high-frequency oscillatory ventilation used as an "early rescue" therapy, improves gas exchange in a rapid and sustained fashion and provides a good outcome. Use of this therapy should probably be considered early in the course of the disease process.