[The mother-infant encounter at birth must be protected]. / Il faut protéger la rencontre de la mère et de son nouveau-né autour de la naissance.

The medical decision to separate a newborn baby from his or her mother is frequent. This medical practice must be evaluated. The scientific literature regarding humans and other mammals was reviewed in order to describe the physiology of the interactions between mother and offspring and the consequences of disrupting their interactions around birth. Mother-infant bonding is common to all mammals. Attachment is the result of three mechanisms: behavioral programing, secretion of neuroendocrine substrates, and activation of sensory cues. Breastfeeding is a fourth component that plays a crucial role. Experimental research provides evidence that early-life maternal separation can alter biological responses to stress, disturb learning behaviors, and impair social skills. Recent advances in epigenetic research may partly explain how neonatal maternal deprivation at birth can lead to biological and behavioral disorders in adulthood. Therefore, the decision to separate a newborn infant from his or her mother is not harmless and must be carefully considered. In order to better take into account these data, the organization of perinatal care in France should be revised.

[Neonatal Pearson syndrome. two case studies]. / 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.

[High-dose intravenous immunoglobulin therapy and neonatal jaundice due to red blood cell alloimmunization]. / 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.

[A case of acquired encephalopathy in a child. A cause that we thought had disappeared]. / Un cas d'encéphalopathie acquise de l'enfant. A propos d'une cause que l'on croyait disparue.

Subacute central nervous system infection must be considered in any infant presenting with progressive encephalopathy. We present the case of an 18-month-old child with normal neuromotor development until the age of 14 months admitted for spastic hypertonia of the legs and arms associated with axial hypotonia. The mother reported that she recently had been found to be HIV-seropositive. HIV antibodies were negative during the first trimester of pregnancy. On the child's blood sample, the HIV test was positive associated with a major decrease in CD4 cell count. Viral load (ARN-PCR) was 720 copies par millilitre. On brain MRI, hypersignals were found in the white matter. HIV related encephalopathy caused by maternal fetal transmission was diagnosed. After 2 months of antiretroviral treatment (azidothymidine, lamivudine, and boosted lopinavir), the child's neurological condition improved. HIV infection must be suspected in all infants with progressive encephalopathy. The HIV test in pregnant women must be proposed at the beginning of pregnancy and repeated during the last trimester.

[Lemierre syndrome: a complication of an oropharyngeal infection]. / Syndrome de Lemierre : une infection oropharyngée compliquée.

We report a case of Lemierre syndrome in a healthy infant, initially presenting with otitis media and angina. Lemierre syndrome is a disease that every pediatrician must know. Early diagnosis and treatment with antibiotics are necessary to decrease mortality. A review of the history and the complications of Lemierre syndrome is presented.

[Second pregnancies in HIV-1-infected women. A prospective monocentric study]. / Secondes grossesses chez les femmes VIH-1 séropositives. Etude 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 maternal's 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.

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

UNLABELLED: 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. CONCLUSION: This is the first report of an anti-human thrombin antibody associated with severe bleeding in a child. Recombinant factor VIIa could represent a novel therapeutic approach for such patients.

[Juvenile dermatomyositis in the Nice area: a retrospective study 1991-2001]. / Dermatomyosite juvénile dans la région niçoise.

INTRODUCTION: Juvenile dermatomyositis is a rare disease. We conducted a retrospective chart review on patients with juvenile dermatomyositis diagnosed in the Nice area from 1991 to 2001. Our purpose was to review diagnosis criteria and treatment strategies. PATIENTS AND METHODS: The cases of juvenile dermatomyositis were identified by phone investigation of physicians of the departments of paediatrics, dermatology, rheumatology and internal medicine. RESULTS: Seven cases of juvenile dermatomyositis (sex ratio M/W: 0.75, medium age: 7.7 years) were identified. Myalgia and/or weakness were the main reasons for initial consultation. Cutaneous lesions were present in all patients on initial presentation. Muscular enzymes were abnormal in 4 cases out of 7. Muscular biopsy was conducted in 6 patients. In one case, MRI revealed an inflammatory involvement although no clinical or biological sign existed. Systemic corticosteroids was the initial treatment, associated with monthly intravenous immunoglobulins in 5 cases, allowing initial control of the disease in all cases. The other therapies were: methotrexate (3 cases), ciclosporin A (1 case), and chloroquine (2 cases). The evolution was monocyclic in three cases, polycyclic in four cases. With a median follow-up of two years, all the patients are alive: five under treatment, three still in first flare. COMMENT: The diagnosis strategy seems relevant because at least 3 criteria of Peter and Bohan were found in 6/7 patients. Muscular biopsy appears fundamental in the diagnosis strategy. MRI was useful when it was conducted but its use has to be assessed. Therapies were those of previous published studies, except for the use of intravenous immunoglobulins as first-line treatment, associated with corticosteroids. This strategy may be justified for corticosteroid-sparing purposes. CONCLUSION: Working on a real, well-codified strategy of diagnosis and treatment would enhance the uniform management of these patients.

[HIV exposure accidents in children. Experience at the emergency admission services of the CHU of Nice]. / Accidents d'exposition au VIH chez l'enfant. Expérience du service d'admission d'urgence du CHU de Nice.

UNLABELLED: Accidental exposure to human immunodeficiency virus (HIV), either by injury with a discarded syringe or after sexual abuse, increases among the children population. PATIENTS AND METHODS: The entire period of the study was seven-year. Twenty-eight children (14 males, 14 females) underwent three visits after an accidental exposure at Day 0, Day 30th and Day 90th. During each visit, a physical examination, liver enzymes, Ag P24, PCR-RNA HIV, B, C hepatitis serologies were performed. An antiretroviral prophylaxis was proposed when a high risk of HIV transmission was recognized. Anti hepatitis B globulins were administrated when no previous immunization had been made. RESULTS: The median age was 9.3 years (range: 1.5 to 16.7 years). 93% of the children consulted within 48 hours after exposure. Five of them consulted after a sexual aggression with penetration, two after a mucosus exposition and two after deep needle injuries. The source of the contamination was known in five cases. Nine patients were given an antiretroviral treatment for four weeks (seven bitherapy, two tritherapy), with no major side effects. Eighteen and 11 children were seen at the second and third visits, respectively. No case of HIV or HCV infection was observed. CONCLUSION: No case of infection after injury with a discarded syringe was found in our study. This situation differs from what is observed in health care workers, where the higher risk factor is observed. Even if no contamination was found after sexual aggression in our population, the risk of HIV is actual. This is why we propose an antiretroviral prophylaxis against HIV in only one indication, corresponding to sexual penetration.

[Primary Gougerot-Sjögren syndrome in a 13-year-old girl]. / Syndrome de Gougerot-Sjögren primitif chez une fille de 13 ans.

UNLABELLED: Sjögren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sjögren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sjögren's syndrome, revealed by recurrent parotitis. CASE REPORT: This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sjögren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sjögren's syndrome. Diagnosis of Sjögren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.

[Mammary staphylococcal cellulitis of the newborn]. / Dermohypodermite mammaire staphylococcique néo-natale.

BACKGROUND: Neonatal cellulitis is usually caused by staphylococcal infection of the mammary gland which has generally reached the stage of abscess formation at the time of diagnosis. The circumstances of onset and possible complications remain poorly known. We report a recent case. CASE REPORT: A female neonate developed major bilateral mammary hypertrophy. Her mother had tried to express the breasts, fearing "congestion". Three days later, the right breast showed signs of inflammation with a fluctuant central zone suggestive of a cellulitic infectious abscess confirmed at ultrasound. Recovery was achieved with incision and antistaphylococci antibiotic therapy. DISCUSSION: Mammary cellulitis of the newborn appears to generally occur following an attempt to manipulate a pre-existing physiological hypertrophy of the breast. Staphylococcus aureus is the most commonly found agent. Early surgical care is generally required as there is a real risk of progression to necrotizing faciitis.

Partial splenectomy in a child with human immunodeficiency virus-related immune thrombocytopenia.

Immune thrombocytopenia (IT) is a frequently occurring disease in childhood and a well known complication of HIV infection. Splenectomy is a part of the treatment strategy for severe chronic IT. However, overwhelming infections after splenectomy have limited its use, especially in young children. A 7-year-old child with maternal-fetal HIV-1 infection and related thrombocytopenia underwent splenectomy after previous treatment failed to improve her platelet count. Approximately 75% of the spleen was removed. The postoperative period was uncomplicated, and the platelet count increased significantly to greater than 500,000/mm3. Ultrasonographic examination performed 3 months later showed a stable volume of the spleen stump (40 x 40 x 20 mm) with effective "vascularization." The platelet count 12 months after surgery showed a sustained increase greater than 150,000 cell/mm3. Subtotal splenectomy may be a safe and effective alternative for patients with HIV and immune thrombocytopenia.

[Antiseptic treatment of the umbilical cord in newborns: survey and recommendations]. / Antisepsie du cordon ombilical du nouveau-né: enquête et recommandations.

AIM: To determine whether umbilical cord care of the neonate is in accordance with the guidelines of antiseptic treatment at this age of life. MATERIAL AND METHODS: A survey was conducted during the 3rd trimester of 1996 in 57 maternity units and departments of neonatalogy in the region of Provence-Alpes-Côte d'Azur (south of France). A questionnaire was sent to the head of each unit asking the modalities of disinfection of the umbilical cord. RESULTS: Fifty units answered the questionnaire. Six different groups of antiseptic products were used, corresponding to 17 distinct commercial preparations. The simultaneous association of several products (two or three) was done in 70% of cases. Eosin was the most frequently used (60%), in association with 25 units. Alcohol was used in 28 centers (56%). It was associated 22 times. Chlorhexidine was used in 16 units (32%), twice alone, and with another topic 14 times. The commercial association chlorhexidine-benzalkonium chloride (Biseptine) was reported seven times (six times in association with another topical treatment). Ektogan (a powder of Zn and Mg peroxide and Zn oxide) was used in ten centers, always in association. Hexamidine was used in four units, once in association. Silver nitrate, Milian solution, iodinated alcohol, and povidone iodine were respectively used once. CONCLUSION: This survey shows that a great variety of umbilical cord care modalities is used in this region, and that the recommendations for antiseptic treatment in young babies, are not always respected. According to these, eosin, ethanol, Ektogan and iodine should not be used for this purpose. Although chlorhexidine has been proven to be the most suitable disinfectant, it comes only in third place, used in association in 95% of the cases. Several studies in neonates have shown that it is well tolerated and efficient even if it delays cord separation. This study should lead to interdisciplinary consensual guidelines for umbilical cord care.

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.

[Pharmacokinetics of netilmicin in the first use in newborn infants with gestational ages greater than 34 weeks]. / Pharmacocinétique de la nétilmicine lors de la première utilisation chez le nouveau-né de plus de 34 semaines de gestation.

BACKGROUND: Neonatal bacterial infections are potentially lethal. The infant must be started on an antibiotic regimen to cover the organisms most frequently implicated. Since the introduction of gentamicin therapy for neonatal infections, attention has focused on aminoglycoside pharmacokinetics in these very young patients. PATIENTS AND METHODS: The pharmacokinetics parameters of netilmicin during its first administration were analysed in 22 newborn infants with a gestational age over 34 weeks, aged 1 to 3 days, in whom a maternofetal infection was suspected. Netilmicin was given intravenously at a dose of 6 mg/kg/day in two daily injections for 35 minutes. Blood concentrations of netilmicin were measured from samples taken 5, 15, 30, 60 minutes and 2 1/2, 5 1/5 and 11 1/2 hours after injection. The patients were also given cefotaxime plus ampicillin. RESULTS: The kinetics were bicompartimental: prematurity, proven infections and other perinatal factors influenced the pharmacologic parameters and it was not possible to define a predictive formula for antibiotic administration. CONCLUSION: The blood levels of netilmicin must be monitored even in infants who were not born prematurely. Because of the large distribution volume and the long half-life, we propose a dose of 6-7.5 mg/kg given once daily.