[Erythrapheresis in children with sickle cell disease, experience of a paediatric apheresis unit]. / Érythraphérèses chez l'enfant drépanocytaire, expérience d'une unité d'aphérèse pédiatrique.

Erythrocyte exchanges on cell separators can be used in children with sickle cell disease and are effective in lowering the level of haemoglobin S. Of the 938 aphereses performed in 2020 in our unit, we observed a low rate of failure of procedures and few complications. Ninety-six percent of erythraphereses were performed in the context of chronic exchange programs, in more than 80% of cases for cerebral vasculopathy or after the occurrence of ischemic strokes. Less than 4% of the procedures were performed for specific indications (preparation for cholecystectomy most often). The vascular access is rarely an obstacle to the realisation of the apheresis. In case of insufficient venous capital, installing an arteriovenous fistula may be considered. Depending on the child's weight, haemoglobin level, and the severity of the sickle cell anaemia, precautions may be necessary when priming the procedure. Nurses experienced in paediatric apheresis and a good medical knowledge of sickle cell disease allowed us to use this technique from the age of 3years and the weight of 15kg.

[Orbital bone infarction in a child with homozygous sickle cell disease]. / Crise vaso-occlusive orbitaire chez un enfant drépanocytaire.

Vaso-occlusive crises are the most common complication of sickle cell disease. Orbital bone infarction is an unusual manifestation of sickling disorders. It is suspected in patients with acute painful periorbital swelling. Orbital compression syndrome with possible optic nerve injury is a rare but serious complication; therefore, this diagnosis should be considered. Orbital infarction can be difficult to distinguish from osteomyelitis or skin infections. Imaging can be helpful in differentiating infection from infarction. We report a case of orbital bone infarction in a 14-year-old boy with sickle cell disease. Under medical treatment, the clinical course resolved with no sequelae.

[Vestibular neuronitis in a teenager with sickle cell disease. Treatment is urgent]. / Syndrome vestibulaire chez une adolescente drépanocytaire SC. Le traitement est urgent.

Vestibular syndrome is not frequently described in patients with sickle cell disease. We report the case of a teenager with sickle cell disease who had a vestibular syndrome with vertigo that successfully responded to exchange transfusion. We discuss guidelines and review the literature in view of this case report. Sensorineural disorders should be considered as stroke syndromes. They require urgent treatment consisting of exchange transfusion or maintaining optimal hydration associated with blood withdrawal. Treatment of vestibular syndrome in sickle cell disease is urgent.

[Impact of CRP rapid test in management of febrile children in paediatric emergency units of Ile-de-France]. / Impact de la protéine C-réactive (CRP) en microméthode sur la prise en charge des enfants fébriles aux urgences pédiatriques en Ile-de-France.

OBJECTIVE: Fever is a common cause of children visits to emergency units. Clinical evaluation does not always eliminate a bacterial infection. Among blood markers, several publications showed the interest of CRP. This study was undertaken to evaluate correlation between two techniques of CRP, one by usual technique at the laboratory and the other by a rapid test, and to evaluate the impact of this rapid test for febrile children at the emergency room, when a hospitalization was not immediately decided. MATERIAL AND METHODS: The study was undertaken in 2004-2005 in eight emergency paediatric units in Ile-de-France concerning febrile children during two periods. In period A, children had at the same time a CRP dosage through two methods, whereas in period B, only a rapid CRP test was first managed. The test used was NycoCard CRP Single test (Progen Biotechnique). RESULTS: Between September 2004 and June 2005, 572 children were included, 268 in period A and 304 in period B. Comparison of CRP results by the two methods showed for 247 children (93%) a fairly good linear correlation (r: 0.929). Blood cell count was the most often prescribed test (99.4 vs 10.5%). Conversely to chest radiography, blood culture, fibrinogen and urinary test were significantly most frequent in period A. The average cost of the additional examinations was 2.6 times more important during the first period. Duration of children management in the units was approximately two times shorter when rapid CRP test was used (199.7+/-92.8 vs 103.5+/-98.6 min). CONCLUSION: This study shows the interest of rapid CRP test for febrile children in the emergency units, and has to be confirmed in ambulatory paediatric practice.

Minimally invasive endoscopic approach to the cervicothoracic junction for vertebral metastases: report of two cases.

The anterior cervicothoracic junction is difficult to expose and many techniques have previously been described. Most of them require an extensile exposure, which can lead to significant morbidity. The aim of this study is to present a less invasive approach, allowing the same exposure on the spine as a larger one. The approach begins with the same incision as the Smith-Robinson technique: a blunt dissection of the posterior face of the manubrium is performed with the finger. An endoscope is inserted through 10-mm trocars, one above the manubrium and the second through the second rib space. The upper mediastinal space is exposed; the dissection is performed on the left side, between the esophagus and trachea medially, between the innominate vein and brachio-cephalic artery distally, and between the left common carotid and internal jugular vein laterally. The recurrent nerve must be protected. Two patients with spine metastases underwent this new approach. A strut graft was fixed anteriorly after decompression of the spinal cord. Levels T1-T3 can be well exposed through this approach, allowing complete vertebral body removal at level T1 or T2. After body removal, the posterior longitudinal ligament is well exposed, allowing complete release of the spinal cord. The use of the endoscope is the key to providing a good view of the spine without an extensile exposure. This new approach is technically feasible. The exposure is sufficient for vertebral body resection and reconstruction by strut graft. The procedure is less aggressive and painful than sternotomy.

Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation.

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.

[Lumbar Chance fracture in an child associated with an intra-abdominal lesion]. / Fracture de Chance lombaire chez l'enfant associée a une lésion intra-abdominale.

We report the case of a teenager who was involved in a road traffic accident. She presented a flexion-distraction type of vertebral injury, (Chance fracture). This fracture was associated with an intra-abdominal injury. The child was a passenger in a rear seat using a shoulder seatbelt restraint.

Characterization of inflammatory reaction in upper airways of cystic fibrosis patients.

Inflammatory cell populations have not been yet precisely evaluated in cystic fibrosis (CF) airways. We intended to characterize morphological modifications, inflammatory cell infiltration and cell proliferation in nasal tissues obtained from 15 CF patients and from 6 non-CF patients with nasal polyposis. Morphological analysis showed an intense inflammatory infiltration in CF and non-CF tissues with only few modifications in the epithelium from CF tissues. Inflammatory cell populations characterized by specific immunolabeling were quantified, showing a predominance of macrophages and T- and B-lymphocytes and only moderate numbers of neutrophils in CF tissues; in non-CF polyps, lymphocytes and eosinophils were abundant. Proliferating cell percentages quantified after proliferating cell nuclear antigen immunolabeling were 5.3+/-4.1% (mean +/- SD) in CF polyps and 3.1+/-1.2% in non-CF polyps in epithelium but were very low in lamina propria. Intense inflammation in nasal tissues from CF patients is therefore dominated by macrophages and lymphocytes rather than by neutrophils. While morphology is preserved, proliferation is high in epithelium from CF polyps. These findings should be regarded in the future for a better understanding of inflammation in CF airway disease.

Expression of the transforming growth factor beta isoforms in inflammatory cells of nasal polyps.

OBJECTIVE: To determine the expression and the potential role of transforming growth factor beta (TGF-beta) in nasal polyposis. DESIGN: Comparison of TGF-beta expression between normal and inflammatory nasal mucosa and polyps; in inflammatory nasal polyps, characterization of the TGF-beta isoforms expression and their potential location in macrophages and eosinophils. SETTING: Patients and samples were selected at the Hôpital Intercommunal, Créteil, France, and immunohistochemistry and immunoblots were performed at the Institut National de la Sante et de la Recherche Medicale U296 (Universite Paris XII, France). SUBJECTS: Nasal polyps and nasal mucosa were sampled in 21 patients during ethmoidectomy, and muscosa was sampled in 6 healthy patients during rhinoplasty. METHODS: Immunohistochemistry and Western blot analysis were performed using specific antibodies to TGF-beta1-3, TGF-beta1, TGF-beta2, and TGF-beta3 isoforms. Double labeling was also performed using anti-TGF-beta1 antibody together with macrophages or eosinophil-specific antibodies. RESULTS: The expression of TGF-beta(1-3) was significantly higher in inflammatory nasal polyps than in inflammatory nasal mucosa and higher in inflammatory nasal mucosa than in nasal mucosa from healthy patients. Transforming growth factor beta1 was the main isoform detected in inflammatory nasal polyps, and it was present in numerous macrophages and in some eosinophils. CONCLUSIONS: Transforming growth factor beta, mainly TGF-beta1, is strongly expressed in inflammatory nasal mucosa, where it could be produced by macrophages and eosinophils. Transforming growth factor beta could induce epithelium and connective tissue modifications and therefore be involved in the pathogenesis of nasal polyposis.

[Interbody arthrodesis of the lumbar vertebrae using retroperitoneal videoendoscopy. A preliminary study of 38 cases]. / Arthrodèse intersomatique du rachis lombaire par vidéoendoscopie rétropéritonéale. Etude préliminaire de 38 cas.

AIM OF THE STUDY: To report a series of 38 patients presenting retroperitoneoscopic inter-body fusion of the lumbar spine from L2 to L5, performed between 1995 and 1998. PATIENTS AND METHODS: This series included 25 women and 13 men aged from 16 to 74 years (mean age: 48.5 years). Surgery was performed in 32 patients for primary degenerative or post-operative instability of the lumbar spine, in five patients for painful sequels of burst fracture, and in one patient for sequels of disc infection. The main complaint was lumbar pain but a real sciatic pain was present in nine patients and was not a contraindication for this surgery. Standard X-rays were performed for each patient, and MRI performed in 30 patients confirmed the diagnosis and was also useful in determining vascular abnormalities. A cast immobilisation of the lumbar spine was done as a preoperative test in every patient. RESULTS: Forty-two levels were fused: 31 with a cage filled with cancellus bone and screwed between the end plates, and 11 with cancellus bone alone or in association with bone substitute, such as beta TCP. Post-operative complications included only a transient paresthesia of the thigh in two cases and a chyloretroperitoneum spontaneously resolutive. After 2.3 months of plaster immobilisation with a follow-up of 11.4 months, patient satisfaction rate was 84.5%, with 68.5% reporting no further back pain. The improvement was estimated by Prolo score. Fusion was considered effective by X-ray examination in all patients with an increase in the intervertebral space of 35% and a recovery of the local lordosis of 15%. CONCLUSION: Retroperitoneoscopic surgery is an elegant and secure method for lumbar interbody fusion of L2 to L5 with very few postoperative complications.

Tri-calcium phosphate ceramics and allografts as bone substitutes for spinal fusion in idiopathic scoliosis as bone substitutes for spinal fusion in idiopathic scoliosis: comparative clinical results at four years.

The authors present the results of a comparative study of two series of posterolateral arthrodeses for scoliosis performed using COTREL DUBOUSSET instrumentation. Fifty-four consecutive patients underwent surgery for idiopathic scoliosis using the same technique. Thirty received a graft consisting of a mixture of corticocancellous autologous and allogenic bone frozen at -80 degrees, and 24 patients were grafted with a mixture of cortico-cancellous autologous bone and sticks of tricalcium phosphate (TCP, Biosorb, SBM, Lourdes, France). All patients were seen at three, six and twelve months, then once a year for at least four years with clinical and radiological evaluation at each visit. At the final follow up visit, no radiologic signs of pseudoarthrosis were found in either group with a minimum follow-up of 4 years. The appearance of bone callus was considered satisfactory at 6 months in all cases; moreover callus seemed to be more important in the TCP series, although this assessment was subjective. TCP resorption was total after 2 years, while allograft fragments were visible on x-rays after 2 years. Minor mechanical complications occurred but did not influence the results. Loss of correction was 8% of that initially obtained in the allograft group and 2% in the TCP group. Loss of correction did not progress after 6 months in the TCP group and after 2 years in the allograft group. Based upon this experience, the use of synthetic bone substitutes such as TCP would appear to be a valuable alternative to allografts in posterolateral spinal arthrodesis for idiopathic scoliosis, and it would eliminate the risk of viral contamination inherent to allograft implantation. To our knowledge, there have been no previous comparative studies concerning the use of tricalcium phosphate versus allograft in the literature.

Anterior evacuation of a spontaneous cervical epidural hematoma.

The case of a spontaneous cervical epidural hematoma treated by anterior corporectomy and arthrodesis is reported. An anterior approach is preferable when an epidural hematoma is anterior to the dural sac and when MRI shows an aspect of old clotted blood that can not be easily evacuated by a posterior laminectomy.

[Palpebral edema with fever and Streptococcus sanguis septicemia]. / Oedème palpébral fébrile et septicémie à Streptococcus sanguis.

A case of facial cellulitis complicated by regional thrombophlebitis and septicemia is reported in a 6 year-old girl. Streptococcus sanguis, a bacterial agent, unusually responsible for cellulitis, was isolated from 5 blood cultures. This child had no immunosuppression or endocarditis or dental infection. She had been previously given a non-steroidal anti-inflammatory agent, which was potentially responsible for the diffusion of infection.