[Psychomotor regression due to vitamin B12 deficiency]. / Régression psychomotrice par carence en vitamine B12.

The vitamin B12 reserves of newborns are dependent on transplacental transfer and secondarily on food intake. This vitamin is involved in hematopoiesis and in neurological development. We report the case of a severe vitamin B12 deficiency in a 7-month-old infant. A neurological evaluation performed at 5 months of age for psychomotor regression found nonspecific cortical atrophy. The infant was hospitalized for impaired general condition and worsening of neurological symptoms. Examinations revealed bicytopenia with normocytic anemia (Hb: 7.4g/dl, MCV : 84µm3). The combination of psychomotor regression, malnutrition, and anemia suggested there was a deficiency, specifically of vitamin B12 (blood values of < 50pg/mL, normal range=200-1000); bone marrow aspiration showed characteristics of megaloblastic dystrophy. Psychomotor regression associated with hematological disorders and a failure to thrive can be related to vitamin B12 deficiency. Replacement therapy must be established as early as possible to avoid potentially irreversible neurological damage.

[Life-threatening hypercalcemia as the initial presentation of childhood acute lymphoblastic leukemia]. / Hypercalcémie menaçante révélatrice d'une leucémie aiguë lymphoblastique de l'enfant.

Hypercalcemia in childhood acute lymphoblastic leukaemia (ALL) is a well-known but uncommon complication. Here, we report a case of B-ALL in which the first signs were life-threatening hypercalcemia associated with diffuse osteolytic lesions with no hematologic abnormalities. We draw attention to the difficulties formally establishing the ALL diagnosis. Bone marrow examinations must be repeated if necessary. Furthermore, biological, cytogenetic, and molecular aspects need to be investigated. Measurement of intact PTH can exclude hyperparathyroidism. PTHrP is possibly involved in hypercalcemia processes induced by tumor cells. The t(17;19) translocation and its E2A-HLF transcript fusion, which have been thought to be a poor prognostic factor, must be determined. Regarding severe hypercalcemia control, treatment is based on both underlying disease management and serum calcium level reduction with aggressive hydration and if necessary bisphosphonates.

[Life-threatening Panton-Valentine leukocidin-associated staphylococcal infections in children. A broad spectrum of clinical presentations]. / Infections sévères à Staphylococcus aureus sécréteurs de la leucocidine de Panton-Valentine chez l'enfant : un large spectre de presentations cliniques.

Panton-Valentine leukocidin (PVL) is a major toxic virulence factor secreted by community-acquired methicillin-sensitive or methicillin-resistant Staphylococcus aureus (SA). SA-PVL can be responsible for life-threatening infections in healthy children with a wide spectrum of clinical presentations involving lung, skin, and soft tissues or bones and joints. PVL production should always be considered in severe SA infections. The pediatric medicine community remains poorly informed regarding the therapeutic management of this infection, which should be early and aggressive. Intravenous empiric antibiotics against SA and its toxins must be given with early and sometimes iterative surgical procedures to drain abscesses and to stop bacterial proliferation and necrosis in the tissues. Here, we report the cases of three patients admitted for SA-PVL infections to the pediatric intensive care unit. Initial clinical presentation in the three patients was multifocal osteomyelitis associated with necrotizing pneumonia; severe skin infection with septic shock; and non-necrotic pneumonia with pleural and pericardial effusion. Appropriate treatments resulted in a good outcome in all cases. Following these illustrations, we describe a number of practical key points in the optimal medical and surgical management of severe SA-PVL infections, with a review of the literature.

Gastroschisis and omphalocele: retrospective study of initial postoperative management in the ICU.

AIM OF THE STUDY: The purpose of this study is to describe the management of infants with gastroschisis (G) and omphalocele (O) during the first 7 days after surgery. METHODS: A retrospective review of all cases of O or G managed at the ICU of the Robert Debré Teaching Hospital between January 1993 and July 2000 was carried out. PATIENTS: 29 infants with G, 15 with O (12 unruptured O [UO] and 3 ruptured O [RO]). RESULTS: Ventilatory support consisted of conventional mechanical ventilation (46 %) and/or in high-frequency oscillatory ventilation (61 %). After day 4, ventilatory requirements evaluated by mean airway pressure (MAP) differed significantly between G (n = 10/29) and O (n = 7/15; group vs. day of life, p = 0.04). The average of MAP measured on days 5, 6, and 7 was significantly higher in O than in G (14.7 +/- 3.0 versus 10.9 +/- 2.8, p < 0.01, respectively). Volume expansion was required at least once in 90 % of patients. Mean fluid requirements were significantly lower in UO than in G and in RO (41 +/- 31 ml/kg, 91 +/- 73 ml/kg, and 137 +/- 25 ml/kg, respectively; p = 0.02 for each comparison). Patients with G were significantly more likely to receive norepinephrine (59 % vs. 20 %, p = 0.027) than patients with O. Twenty-six infants with G (90 %) and 11 with O (73 %) were discharged alive from ICU. CONCLUSIONS: Haemodynamic instability can be expected in patients with G or RO, and ventilatory requirements were higher in infants with O than in infants with G during the first week after surgery.

Body composition in children with bronchopulmonary dysplasia predicted from bioelectric impedance and anthropometric variables: comparison with a reference dual X-ray absorptiometry.

UNLABELLED: Since children with bronchopulmonary dysplasia often suffer from malnutrition and growth failure, evaluation of body composition is a very important tool to nutritional support. The aim of this study was to compare assessment of fat-mass (FM) and fat-free mass (FFM), evaluated by bio-impedancemetry and anthropometry compared to dual-X-ray-absorptiometry (DXA) in children with bronchopulmonary dysplasia. PATIENTS: Seventy-one children, aged 4-8 years, with bronchopulmonary dysplasia were enrolled. METHODS: FM and FFM measured using anthropometry and bio-impedancemetry were compared to FM and FFM obtained by DXA using the Bland-Altman method. RESULTS: Both bio-impedancemetry and anthropometry gave good agreement with DXA to evaluate FM and FFM. Anthropometry method, in general, slightly under-estimated FM (mean difference: -0.02 kg, standard deviation: 0.99) and FFM (mean difference: -0.70 kg+/-1.72). Bio-impedancemetry method overestimated FM (mean difference: 0.34 kg+/-2.06) and underestimated FFM (mean difference: -1.24 kg+/-3.32). CONCLUSION: In children with bronchopulmonary dysplasia aged, 4-8 years, both anthropometry and bio-impedancemetry cannot be used to precisely evaluate body composition.

[Severe acute White-Spirit poisoning with pulmonary hypertension]. / Un cas d'intoxication aiguë sévère au White-Spirit avec hypertension artérielle pulmonaire.

UNLABELLED: The Authors report a case of acute White-Spirit poisoning with pulmonary hypertension associated to respiratory distress syndrome. CASE REPORT: A 14-month-old infant drank an unknown quantity of White-Spirit while his parents were painting. After he spontaneously vomited, he presented a seizure at the emergency department. After a 36 h stay in Pediatric Intensive Care Unit (PICU), acute lung injury required mechanical ventilation and vasoactive support. Cardiac ultrasounds showed pulmonary hypertension, which rapidly resolved with inhaled nitric oxide. The child was discharged of PICU after five days. Respiratory follow-up two months after poisoning was normal. CONCLUSION: Pulmonary hypertension should be checked for in case of White-Spirit ingestion complicated with severe acute lung injury.

[A case of atrial flutter after umbilical venous catheterization]. / Une observation de flutter auriculaire après pose de cathéter veineux ombilical.

UNLABELLED: Isolated atrial flutter is an extremely rare form of supraventricular tachycardia in the neonatal period. It may be initiated by central venous catheterization. CASE REPORT: A male infant was born at 35 weeks by cesarean section for placenta praevia. He was eutrophic. Apgar score was 10 at 1 and 5 minutes. He secondary developed a respiratory distress syndrome. He was then ventilated by nasal CPAP. Immediately after an umbilical venous catheterization, a tachycardia appeared without preexistent cardiac dysfunction. An intravenous dose of adenosine (Striadyne) showed a characteristic sawtooth pattern of P waves on inferior leads. The cardiac-US examination was normal. This atrial flutter was converted to normal sinus rhythm by transoesophageal pacing, without adjunction of antiarrhythmic drugs. The newborn was weaned from mechanical ventilation 48 hours later and discharged from hospital at seven days post natal age. His development and clinical examination were normal two months later. CONCLUSION: The isolated atrial flutter is rare in the neonate. It may be triggered by a venous catheterization. Transoesophageal atrial pacing is safe and effective for conversion.