P. Boutté

Fluconazole therapy for Candida albicans urinary tract infections in infants

Abstract. Candiduria is rare in newborns and infants, occurring most often in patients with risk factors. When associated with a candidal bezoar in the urinary tract, candiduria is usually treated by systemic amphotericin B and flucytosine plus local irrigation with amphotericin B. We describe the successful treatment of five newborns with a urinary tract infection, on major urological malformations, due to Candida albicans (including three with a candidal bezoar) by fluconazole alone. No adverse effects or recurrences were observed. Fluconazole therapy permits early discharge from the hospital and seems suitable for infants and newborns with a C. albicans urinary tract infection.

Nephrocalcinosis and Prematurity: Importance of Urate and Oxalate Excretion

Nephrocalcinosis was described in preterm infants by several authors who tried to determine its association with hypercalciuria and furosemide therapy. We evaluated these potential mechanisms along with other lithogenic factors not previously studied in 10 premature babies. Hypercalciuria was an inconsistent finding like in other reports; elevated uric acid excretion and hyperoxaluria were observed in 5 and 6 cases, respectively. The aminocid excretion was normal in all infants. Our data suggest that in addition to hypercalciuria, other lithogenic factors may play a role in the pathophysiology of nephrocalcinosis of premature infants.

Immunoglobulines polyvalentes intraveineuses et ictère néonatal par allo-immunisation érythrocytaire

Neonatal jaundice resulting from immunological hemolysis is not uncommon. While it is possible to prevent a large number of Rh-isoimmune hemolytic diseases by administration of specific anti-D immunoglobulins to the mother, the prevention of incompatibility in the ABO groups is not feasible. In spite of advances made in the use of phototherapy, and in order to avoid kernicterus, the treatment of these jaundices can require one or several exchange transfusions (ET), a therapy which is not devoid of risk. For some time now, the data concerning the efficiency of high-dose intravenous immunoglobulin therapy (HDIIT) in the treatment of these jaundices have been increasing. A review of the literature shows that, if used as soon as possible in newborn infants over 32 weeks of gestation age, afflicted with Rh or ABO hemolytic disease, the HDIIT brings about, with no undesirable side effects, a significant decrease in the ET number as well as a significant reduction in the length of phototherapy and hospitalization. The data suggesting that HDIIT could increase the risk of late transfusion is open to controversy.

Noninvasive Procedures to Evaluate Liver Involvement in HIV-1 Vertically Infected Children

Objectives: Progressive liver injury is a concern in HIV-infected children exposed to long-term antiretroviral drugs and to the cytopathic effect of HIV. Yet liver biopsy is usually considered too invasive to be repeated in these patients. The aims of this study are to evaluate the feasibility of noninvasive hepatic investigations in HIV-1-infected children, assess the prevalence of signs of liver affection, and analyse the influence of the HIV disease severity and the exposure to antiretroviral therapy. Materials and Methods: A cross-sectional study conducted in 26 HIV-1 vertically infected children ages 8 to 18 years old. Liver function was assessed with standard serum biochemical markers, FibroTest, ActiTest, SteatoTest, Forns index, aspartate aminotransferase to platelet ratio index, ultrasound, and Fibroscan. Results: Nineteen (>60%) children had signs of liver affection on at least 1 of the test results: 13 (50%) had elevated liver enzymes, 15 (63%), 8 (33%), 5 (21%), and 5 (21%) had abnormal FibroTest, ActiTest, Forns index, and aspartate aminotransferase to platelet ratio index results, respectively. Four children (17%) had mild liver steatosis on ultrasound. Fibroscan measures were significantly higher in patients than in age-matched healthy children. Patients with elevated Fibroscan measures also had significantly higher FibroTest results. Age, HIV stage N in the Centers for Disease Control and Prevention classification and exposure duration to nucleoside reverse transcriptase inhibitor and non-nucleoside reverse transcriptase inhibitor drugs were the main risk factors for hepatotoxicity. Conclusions: More than half of our population of HIV-infected children had biological and/or radiological signs of liver affection. Regular follow-up of liver function is necessary in these patients, which is now possible with noninvasive procedures.

Mise au pointImmunoglobulines polyvalentes intraveineuses et ictère néonatal par allo-immunisation érythrocytaireHigh-dose intravenous immunoglobulin therapy and neonatal jaundice due to red blood cell alloimmunization

Neonatal jaundice resulting from immunological hemolysis is not uncommon. While it is possible to prevent a large number of Rh-isoimmune hemolytic diseases by administration of specific anti-D immunoglobulins to the mother, the prevention of incompatibility in the ABO groups is not feasible. In spite of advances made in the use of phototherapy, and in order to avoid kernicterus, the treatment of these jaundices can require one or several exchange transfusions (ET), a therapy which is not devoid of risk. For some time now, the data concerning the efficiency of high-dose intravenous immunoglobulin therapy (HDIIT) in the treatment of these jaundices have been increasing. A review of the literature shows that, if used as soon as possible in newborn infants over 32 weeks of gestation age, afflicted with Rh or ABO hemolytic disease, the HDIIT brings about, with no undesirable side effects, a significant decrease in the ET number as well as a significant reduction in the length of phototherapy and hospitalization. The data suggesting that HDIIT could increase the risk of late transfusion is open to controversy.

Successful use of recombinant factor VIIa for management of severe menorrhagia in an adolescent with an acquired inhibitor of human thrombin

Antibodies directed against human thrombin are exceedingly rare, having only been reported in adult patients with underlying diseases. Consensus on the most appropriate management has not yet been reached. A 12‐y‐old girl presented with intractable menorrhagia several days after an acute infectious episode. Laboratory tests revealed disturbed clotting tests: prothrombin index 17%, activated partial thromboplastin time >150 s, thrombin time >120 s, and failure to achieve correction with a normal pooled plasma. Further studies demonstrated the presence of an antibody directed against human thrombin. Viral serology revealed a 1/128 titre for adenovirus. Massive haemorrhage was unresponsive to standard treatments, but intravenous administration of recombinant factor VIIa resulted in a successful outcome.

Kawasaki disease with exceptional cutaneous manifestations.

The cutaneous eruption is a key symptom of Kawasaki disease but atypical skin presentation with papules or keratotic lesions should not lead to exclude the possibility of Kawasaki disease, especially in children under 1 year, and must not delay diagnosis and treatment. Kawasaki disease (KD) is an acute febrile illness whose morbidity is dominated by the risk for coronary aneurysm formation. Mucocutaneous findings are often characteristic and comprise four of the six diagnostic criteria. Incomplete or atypical cases may delay or obscure the diagnosis. We report a very unusual case of KD in a 5-month-old boy, whose course was remarkable for the severity of the illness, and for the intensity, the type and the course of the cutaneous manifestations. A previously healthy 5-month-old boy developed an abrupt fever (39 C) with asthenia and a scarlatiniform rash, which started on the hands and feet. Generalised oedema, bilateral conjunctival injection, and a cervical lymphadenopathy of 2 cm diameter were also noted. Laboratory data showed an erythrocyte sedimentation rate of 91 mm/h, a C-reactive protein level of 94 mg/l, a white blood cell count of 14.6·10/ml (58% polymorphonuclear cells) and thrombocytosis of 450·10/ml. Bacterial cultures and serology for viruses (EBV, CMV, parvovirus B19, HIV, and hepatitis A, B, and C), as well as titres of anti-streptolysin O, were negative. The diagnosis of KD was made based on the presence of the five diagnostic criteria. Five days after the first symptom (day 5) a treatment regimen of high-dose acetylsalicylic acid (30 mg/kg per day) and intravenous immunoglobulin (IVIG) (1 g/kg per day for 2 consecutive days) was initiated. A second course of IVIG was administrated at day 8 due to persistent fever and fatigue and to the development of red, fissuring cheilitis. Despite this treatment, the fever persisted and the inflammatory syndrome worsened. On day 14 he was transferred to the paediatric ward of our University Hospital. There was an extensive red-orange papulosquamous plaque-like eruption, most prominent on the extremities, with milder involvement of the face, groin, and chest. The back was spared. The hands and feet displayed numerous confluent keratotic 1 to 2 mm papules (Fig. 1). Generalised oedema with prominent acral involvement was present. The lips were red, dry, and fissured, and both conjunctiva were intensely injected. A skin biopsy revealed parakeratosis alternating with a few orthokeratotic areas, as well as marked lymphocytic exocytosis without spongiosis and many necrotic keratinocytes. A scant inflammatory infiltrate was present in the dermis. Direct immunofluorescence was negative. On day 15, 2-dimensional echocardiography demonstrated a 4 mm left coronary aneurysm. In spite of a third course of IVIG (day 15), the fever persisted, the general condition deteriorated, the platelet count increased to more than 750·10/ml, and the aneurysm increased to 7 mm (day 21). A single pulse of intravenous prednisolone, 1000 mg/m was administered with improvement of symptoms. A relapse of fever to 39 C 2 days later led to the administration of a second pulse injection and prednisolone was continued orally at 1 mg/kg per day. This resulted in a marked and lasting improvement, but the cutaneous exanthem did not improve. Marked hyperkeratosis developed in the previously scaling areas, in some areas exhibiting a very verrucous character (Fig. 2). The cutaneous eruption exhibited delayed improvement, with reduction in erythema and hyperkeratosis by day 30. A progressive desquamation of keratotic moulds conforming to the contours of the fingers and toes and including the nails occurred and lasted 15 days Eur J Pediatr (2002) 161: 228–230 DOI 10.1007/s00431-001-0905-5

Accidents d’exposition au VIH chez l’enfant. Expérience du service d’admission d’urgence du CHU de Nice

Resume L’accident d’exposition au virus de l’immunodeficience humaine (VIH), soit par piqure accidentelle avec une seringue usagee, soit par exposition sexuelle dans le cadre de sevices, est un probleme frequent en pediatrie. Patients et methodes – Sur une periode de sept ans, 28 enfants (dont 14 garcons) ont ete suivis pour accident d’exposition. Trois consultations (j0, j30 et j90 apres exposition) comportant examen clinique, transaminases, antigenemie P24, PCR-RNA VIH (depuis 1998), serologies VIH, hepatites B et C ont ete proposees ainsi qu’un traitement antiretroviral prophylactique en cas d’exposition consideree a haut risque de contamination par le VIH. Des immunoglobulines anti-hepatite B etaient administrees en l’absence de couverture vaccinale. Resultats – L’âge median etait de 9,3 ans (1,5 a 16,7 ans). Quatre-vingt-treize pour cent des enfants ont consulte dans les 48 heures suivant l’accident. Le sujet source n’etait connu que dans cinq cas. Neuf patients ont recu un traitement anti-retroviral pendant quatre semaines (sept bitherapies, deux tritherapies) sans effets secondaires majeurs. Il s’agissait de cinq agressions sexuelles avec penetration, de deux expositions muqueuses et de deux piqures accidentelles profondes. Seuls respectivement 18 et 11 enfants ont ete revus aux 2 e et 3 e consultations. Aucune infection VIH ou VHC n’a ete observee. Conclusions – Aucun cas d’infection liee au VIH n’a ete retrouve dans notre serie d’enfants examines apres piqure accidentelle par une seringue usagee. Les circonstances favorisantes rapportees a l’occasion de contaminations accidentelles du personnel de sante sont rarement conjuguees lors des accidents survenant chez les enfants sur la voie publique. Bien que n’ayant constate aucune contamination par voie sexuelle dans notre population, les risques de transmission apres ce type d’exposition sont reels. Il nous semble logique de proposer une prophylaxie pour chaque cas de sevices avec penetration sexuelle.

Le syndrome de Pearson : à propos de 2 observations à révélation néonatale

Among the etiologies of anemia in the newborn, those related to mitochondrial cytopathies are rare. Pearson syndrome is mostly diagnosed during infancy and characterized by refractory sideroblastic anemia with vacuolization of marrow progenitor cells and exocrine pancreatic dysfunction. We describe two diagnosed cases of Pearson syndrome in the early neonatal period caused by severe macrocytic aregenerative anemia. Bone marrow aspiration revealed sideroblastic anemia and vacuolization of erythroblastic precursors. The diagnosis was confirmed by genetic analysis revealing a deletion in the mitochondrial DNA. These two newborns received monthly transfusions. Five other newborns suffering from Pearson syndrome with various clinical symptoms were found in literature. Pearson syndrome, rarely diagnosed in newborns, should be suspected in the presence of macrocytic aregenerative anemia and requires a bone marrow aspirate followed by a genetic analysis from a blood sample.

Secondes grossesses chez les femmes VIH-1 séropositives. Étude prospective monocentrique

OBJECTIVES To evaluate the influence of successive pregnancies on the materno-foetal prognosis in a population of HIV-infected women. Clinical, biological data and treatment strategies were compared during iterative pregnancies. PATIENTS AND METHODS We conducted a monocentric prospective study between August 1995 and January 2007 in a French university hospital (Nice). RESULTS Twenty-six HIV-infected women had two consecutive pregnancies during our study. We noticed an increase in CD4 cell count between the two pregnancies. Viral load variations were non significant. The maternals prophylaxis changed. Percentage of HAART increased from 26 to 54%. Modes of delivery, HIV or treatments side-effects remained the same between the successive pregnancies. DISCUSSION AND CONCLUSION Successive pregnancies do not seem to influence the materno-foetal prognosis related to HIV infection.