[Thrombopenia and the nature of the microorganism in infected critically ill children]. / Thrombopénie et nature des germes infectants en réanimation pédiatrique.

BACKGROUND: Thrombopenia is correlated with sepsis and mortality in pediatric intensive care units. The purpose of this study was to find an association between thrombopenia and the microorganism type to guide the choice of empiric antibiotic therapy in infected critically ill children. MATERIALS AND METHODS: We conducted a prospective descriptive study, including all newborns, infants, and children admitted to a pediatric surgical intensive care unit from 1st January to 31st December 2009. We identified patients who developed an infection and/or thrombopenia (platelet count less than 100,000/mm(3)) during hospitalization. RESULTS: One hundred ninety-seven patients were included (57 newborns, 41 infants, 99 children). Ninety patients developed 100 infectious episodes during the study period. Of the 57 newborns enrolled in the study, 31 (54%) developed 37 infections. Seventy-six microorganisms (55 Gram-negative bacilli [GNB], 17 Gram-positive cocci, two Gram-negative cocci, two fungal pathogens) were identified during 65 infectious episodes in 55 patients. Thirty-four episodes of thrombopenia were observed in 30 patients. Thrombopenia was observed only in infected patients (P<0.001). Thrombopenia was associated with infections caused by GNB (26/28 vs 20/37, P=0.001) and by Klebsiella (16/28 vs 6/37, P=0.001) and may be associated with infections caused by GNB producing extended-spectrum beta-lactamases (P=0.07). Gram-positive cocci infections were correlated to the non-occurrence of thrombopenia (P=0.02). Postoperative peritonitis was also significantly associated with thrombopenia (P=0.03). The mortality rate in our patients was 12.7% (22.8% in neonates). There was an association between thrombopenia and death in univariate analysis (11/25 vs 19/172, P<0.001). Multivariate logistic regression analysis did not confirm thrombopenia as an independent predictive factor of mortality in children. CONCLUSION: Because of the relatively high proportion of resistant GNB, an empiric antibiotic therapy combining a carbapenem and an aminoglycoside may be indicated in infected critically ill children developing thrombopenia.

[Growth hormone deficiency and pituitary stalk interruption in Fanconi anemia]. / Déficit en GH par interruption de la tige pituitaire dans l'anémie de Fanconi.

Fanconi anemia is a rare disorder inherited by recessive autosomic transmission belonging to the group of chromosomal instability syndromes. It is characterized by progressively developing medullary aplasia, various congenital malformations and especially a high risk of cancer, particularly acute myeloblastic leukemia and certain solid tumors. The association is quite common in patients with endocrine disease which constitutes an additional factor of morbidity and must be diagnosed and treated. We report a case of Fanconi anemia revealed by severe delay in statural growth and primary amenorrhea with a 21-year-old girl. The diagnosis was suggested by asymptomatic pancytopenia caused by a medullary hypoplasia and confirmed by a cytogenetic investigation using cross-linking agents that showed a large number of chromosomal breaks. Hormonal exploration revealed hypopituitarism with complete growth hormone (GH) deficiency and hypogonadotrophic hypogonadism caused by interruption of the pituitary stalk. The aim of this case report is to illustrate the importance of early exploration of retarded growth which, in some patients, can reveal potentially serious, and treatable, disease.