Aplasia cutis congenita associated with Goltz syndrome in a male neonate.

Here we report the association of giant aplasia cutis congenita in a newborn black male with Goltz syndrome. The cutis aplasia defect is extensive and circonscript at the vertex. The cerebral structures are visible through the lesions. In addition, the patient has a low birth weight, syndactyly, adactyly, cutaneous atrophy, and areas of hyperpigmentation on the legs and hypoplastic maxillary. We think that these signs are probably due to mosaic mutations in PORCN. We reviewed 18 cases of Goltz syndrome in 18 male neonates but none has aplasia cutis congenita. Such a combination of severe aplasia cutis congenita was not reported previously in Goltz syndrome.

Le noma de l'enfant en milieu hospitalier de Bobo-Dioulasso (Burkina-Faso): aspects épidemiologiques et diagnostiques

Introduction: Le noma est une affection peuconnue au Burkina Faso. Cette étude décrit les aspectsépidémiologiques et diagnostiques de 59 cas de nomaà Bobo-Dioulasso (Burkina Faso).Méthodologie C'est une étude rétrospective sur10 ans incluant tous les enfants de moins de 15 ans,hospitalisés pour noma.Résultats : L' incidence du noma est de sept cas/an. La tranche de 1-5 ans a été la plus touchée. Dans46 cas (78%), le noma est survenu sur un terrain demalnutrition, associé à une mauvaise hygiène bucco-dentaire dans 45 cas (76,3%). Le tableau clinique estcelui d'un noma évolutif dans 51 cas (51/59) et d'unnoma séquellaire dans huit cas (8/59).Discussion-conclusion : Notre incidence de 7 cas/an est plus élevée que dans certaines études. D'autrestravaux trouvent comme nous que la tranche de 1-5ans a été la plus touchée. Le noma survient sur unterrain malnutri (78%) et d'hygiène orale défec-tueuse. Il devrait être mieux connu pour un traite-ment préventif adapté

[Genital bacterial carriage during the last trimester of pregnancy and early-onset neonatal sepsis]. / Portage génital bactérien au dernier trimestre de la grossesse et infection néonatale précoce.

UNLABELLED: Bacterial infections remain a major cause of morbidity and mortality in newborn infants. OBJECTIVE: To determine the bacterial ecology and pathological status of the genital organs during the last trimester of pregnancy and the germs of the following early-onset neonatal sepsis, in order to evaluate the risk of materno-foetal infections and to find out a drug prophylaxis. METHOD: Vaginal and endocervical samples, usually taken during the first trimester of pregnancy were delayed and taken during the last trimester of pregnancy. A macroscopic examination described the aspect of the vagina, the cervix uteri, leukorrhea and of possible inflammatory lesions or ulcerations. A microscopic examination searched for parasites, epithelial cells, clue cells and leukocytes. The appropriate bacteriological cultures were performed after reading the Gram stain and scoring the vaginal flora. The clinical and cytobacteriological aspects were used to identify the bacterial ecology and the pathological genital states. An exploration was carried out in every newborn suspected of infection. RESULTS: Genital samples were collected from 306 pregnant women. Among them, 118 were at 29-32 weeks of gestation, 104 at 33-36, and 84 at 37-40. The most frequent germs were C. albicans (33,5%), Enterbacteriaceae (20.3%) including E. coli (10.9%), S. aureus (15.4%), Gardnerella (13.6%), and Trichomonas (10.6%), in monomicrobian (79.2%) and polymicrobian carriage (20.8%). Lower genital tract pathological states such as vaginitis (29.4%), bacterial vaginosis (21.5%) or endocervicitis (10.4%), asymptomatic bacterial carriage (23.5%) and normal genital flora (15%) were identified. These pregnancies led to 334 live births with 27 cases of early-onset neonatal sepsis to which endocervicitis (25%) and vaginosis (19,7%) were most often linked. CONCLUSION: Genital samples at the last trimester of pregnancy could evaluate the risk of maternofoetal infections and allow to adapt a drug prophylaxis of Enterobacteriaceae, the most frequent germ of neonatal infections, as it has been done for Streptococcus agalactiae. But larger studies are required to evaluate the risk of maternofoetal infections and to state the drug prophylaxis.

Burkitt's lymphomain Burkina Faso hospital center : therapeutic and course.

Summary A retrospective study of 41 cases of children sent to hospital and treated for the Burkitt's lymphoma for a period of 10 years has been carried out in the hospital of Bobo-Dioulasso in order to study the progressing and therapeutic features of the disease. The therapeutic means were the ablation surgery of voluminous tumors (19.5%) and the monochimiotherapy with cyclophosphamid (83%) according to Burkitt's procedure (64.7%) and N'gu procedure (35.3%). The mortality under chimiotherapy was 8.8%. The immediate evolution has shown 58.8% of complete remission, 26.5% of partial remission, 11.8% of resistance to the treatment.

The child's noma in an hospital center in Burkina Faso : Clinical features and course.

In order to study the therapeutic and developing aspect the child's noma in Burkina Faso, we have undertaken a retrospective study on a ten-years-period, with the files of 59 children hospitalized in the hospital of Bobo-Dioulasso, completed with a visit to the children's families after their leaving the hospital. The medical treatment has allowed to reach a recovery rate of 79.6% at the cost of aesthetic and functional after-effects which aren't negligible. The death rate in hospital was 13.7%. 31 children have profited from the restoring surgery of lesions. The care out of hospital has showed that the surgical results were mitigated.

Le lymphome de Burkitt en milieu hospitalier du Burkina Faso: Aspects thérapeutiques et évolutifs

Une étude rétrospective portant sur 41 dossiers d'enfants hospitalisés et traités pourlymphome de Burkitt, sur une période de 10 ans, a été effectuée dans les services de pédiatrie et de chirurgie maxillo-faciale de l'hôpital de Bobo-Dioulasso aux fins d'étudier les aspects thérapeutiques et évolutifs de la maladie. Les moyens thérapeutiques ont été la chirurgie d'ablation des volumineuses tumeurs (19,5%) et la monochimiothérapie au cyclophosphamide (83%) selon les procédés de Burkitt(64,7%) et de N'gu (35,3%). La létalité sous chimiothérapie était de 8,8%. L'évolution immédiate a montré 58,8% de rémission complète, 26,5% de rémission partielle, 11,8% de résistance au traitement

[Tuberculosis in the newborn: recrudescence in areas with high endemic HIV infection]. / Tuberculose du nouveau-né: résurgence en milieu de forte endémie de VIH.

Despite the rising prevalence of tuberculosis due in part to the HIV pandemic in Africa, there have been few reports describing neonatal or congenital tuberculosis and its association with maternal HIV infection has been rare. The purpose of this study was to evaluate the clinical and epidemiological features of tuberculosis in newborns from areas with high endemic rates of both tuberculosis and HIV infection. During the 2-year study period all neonates admitted to the Campus Teaching Hospital in Lomé, Togo for differential diagnosis of symptoms compatible with tuberculosis were investigated. The clinical profile of tuberculosis in the newborn was correlated with that of the mother with or without HIV infection. Perinatal tuberculosis was diagnosed in 13 of the 79 newborns investigated including 8 whose mothers were co-infected by HIV and tuberculosis. Seven cases were classified as congenital tuberculosis. The predominant clinical features were respiratory distress (10/13), fever (9/13), hepatomegaly (9/13), intra-uterine growth retardation (8/13), stagnation or loss of weight (6/13), cough (4/13) and splenomegaly (4/13). Diagnosis of maternal HIV and tuberculosis infection was never made prior to newborn admission to our department. Four newborns and two mothers died within 3 months after childbirth. This study on perinatal tuberculosis in children born to mothers with or without HIV infection demonstrates the need for early diagnostic methods, consensual therapeutic protocols, and further study in larger geographical area to specify epidemiologic features and reduce high mortality.

[Neonatal septicaemia: bacteriological aspects and outcome in the university hospital center of Lome]. / Septicémie néonatale: aspects bactériologiques et évolutifs au centre hospitalier universitaire de Lomé, Togo.

UNLABELLED: Despite the progress in neonatal intensive care and antibiotics, the neonatal septicaemia remains very frequent and lethal in tropical areas. OBJECTIVE: To study the bacteriology and outcome of the neonatal septicaemia through an analysis of 50 confirmed cases. METHODS: Every child aged of less than 29 days, suspected of infection and with at least one positive blood culture has been included. His perinatal antecedents, clinical features, bacteriological findings, treatments and clinical outcome have been collected. The patients have been divided into 3 groups in regard to the postnatal age at the first positive blood culture: before 48 hours, between 3 and 7 days and after 7 days of life. RESULTS: The neonatal septicaemia occurred in 50% of the cases before 48 hours, 24% from the 2nd to the 7th day and 26% from the 8th to the 28th day of life. Enterobacteriaceae (54%) with E. coli predominant (30%) and S. aureus (28%) were the most frequent germs and particularly during the first week of life: 20/27 and 11/14 respectively. The resistance to the association of ampicillin or amoxicillin and gentamicin was 70% for Enterobacteriaceae and 71% for Staphylococci. The lethality was 36% with an excess of lethality for S. aureus (43%). CONCLUSION: The high rates of frequency and lethality of the neonatal septicaemia in tropical areas require a perinatal care improvement to reduce its morbidity and mortality.

[Comparative study of neonatal bacterial meningitis in Lomé, Bobo-Dioulasso, Casablanca and Lyon]. / Analyse comparative des méningites bactériennes néonatales à Lomé, Bobo-Dioulasso, Casablanca et Lyon.

UNLABELLED: Bacterial meningitis in new-borns remains a serious event because of its high mortality and morbidity rates in Africa. OBJECTIVE: To identify the clinical and bacteriological epidemiology and the outcome of neonatal bacterial meningitis in three African cities. METHOD: We have analysed and compared three hospital studies done in humid tropical, Sahelian, and desert Africa with a European study. RESULTS: Compared with the European study this African study is characterized by a high mean frequency of neonatal meningitis (6 cases per year against 1.4), more important risk factors linked to pregnancy and childbirth (50% against 33%), high rates of death (61 to 68% against 5%) and sequelae (25 to 40% against 30%), rarity of Streptococcus agalactiae (7 to 15% against 38%) and absence of Listeria. Enterobacteriaceae were predominant both in African (50 to 68%) and European (43%) studies. E. coli appeared as the most frequent germ in both European and African studies and Salmonella as more frequent in Sub-Saharan Africa than in occidental countries. CONCLUSION: The epidemiological, bacteriological and evolutional aspects of the neonatal meningitis were identical in the three African cities. The African studies were different from the European only by their high incidence, the rarity of S. agalactiae and Listeria and the difficulties of bacterial diagnosis and management, all might explain the high rates of death and sequelae. An epidemiological survey and adequate antimicrobial therapy according to antibiotic susceptibility may improve the outcome.

Tuberculose du nouveau-né: Résurgence en milieu de forte épidémie de VIH

Malgré l'augmentation de la fréquence de la tuberculose en Afrique,due en partie à la pandémie duVIH,les publications sur la tuberculose néonatale ou congénitale restent rares et peu en rapport avec l'infectionVIH de la mère. Evaluer les aspects épidémiologiques et cliniques dela tuberculose du nouveau-né dans une loca-lité de forte endémie de VIH et de tuberculose,tel a été le but assigné à cette étude. Durant deux ans,tout nou-veau-né admis au Centre hospitalier universitaire-Campus de Lomé pour des signes faisant discuter le diagnos-tic de tuberculoseabénéficié d'une ex p l o ration.Le pro filclinique de latuberculose du nouveau-né et ses rap p o rt savec celle de la mère infectée ou non par le VIH ont été déterminés. Des 79 nouveau-nés ainsi explorés,13 avaientune tuberculose néonatale. Huit d'entre eux sont nés de mère infectée par le VIH et la tuberculose. Sept étaientatteints de tuberculose congénitale. Les signes cliniques prédominants étaient :la détresse respiratoire (10/13),lafi è v re (9/13),l ' h é p at o m é galie(9/13),le re t a rd de croissanceintra - u t é rine (8/13),la stag n ation ou la perte depoids( 6 / 1 3 ) ,latoux(4/13) et lasplénomégalie (4/13). Chez aucune desmèresle diagnostic de VIH et de tuberculose n'étaitétabli avant l'admission de leurs nouveau-nés. Quatre nouveau-nés et deux mères sont décédés au cours des troismois suivant l'accouchement. Cette étude de la tuberculose du nouveau-né de mère infectée ou non par le VIHdemande des méthodes de diagnostic précoce,des protocoles thérapeutiques consensuels et d'autres études surchamp géographique étendu pour en préciser l'épidémiologie et réduire la forte létalité

[Bacterial flora in the genital tract the last trimester of pregnancy]. / Flores bactériennes génitales au dernier trimestre de la grossesse.

UNLABELLED: Very widespread in our clinical setting, early-onset sepsis is due to organisms that commonly colonize or infect the maternal genital tract; identifying such organisms would help improve prevention and treatment. OBJECTIVE: To determine the bacterial ecology and the pathological status of the genital organs during the last trimester of pregnancy, in order to evaluate the risk of materno-fetal infections and to improve the present prophylactic measures based on monitoring bacterial carriage during the first trimester. METHOD: Vaginal and endocervical samples, usually taken during the first trimester of pregnancy were delayed and taken during the last trimester of pregnancy, in patients with no signs of sepsis and not taking antibiotics. A macroscopic examination described the aspect of the vagina, the cervix uteri, leukorrhea and possible inflammatory lesions or ulcerations. A microscopic examination searched for parasites, epithelial cells, Clue cells and leukocytes. The appropriate bacteriological cultures were performed after reading the Gram stain and scoring the vaginal flora. The clinical and cytobacteriological aspects were used to identify the bacterial ecology and the pathological genital states. RESULTS: Genital samples were collected from 306 pregnant women. Among them 118 were at 29-32 weeks of gestation, 104 at 33-36 and 84 at 37-40. The most frequent germs were C. albicans (33.3%), Enterobacteriaceae (20.3%) including E. coli (10.9%), S. aureus (15.4%), Gardnerella (13.6%), and Trichomonas (10.6%), in monomicrobian (79.2%) or polymicrobian carriage (20.8%). Lower genital tract pathological states such as vaginitis (29.4%), bacterial vaginosis (21.5%) or cervicitis (10.4%) and asymptomatic bacterial carriage (23.5%) and normal genital flora (15%) were identified. CONCLUSION: This is the first report of genital bacterial carriage in African women during the last trimester of pregnancy. Larger studies are required to evaluate the risk of maternofetal infections and to improve current prophylaxis measures.

[Post-hemolytic renal failure in children with glucose-6-phosphate dehydrogenase deficiency at the University Hospital Center in Lome]. / Insuffisance rénale post-hémolytique chez l'enfant déficient en glucose-6-phosphate déshydrogénase au Centre Hospitalier Universitaire de Lomé.

The purpose of the study was to identify predisposing factors for acute hemolysis and post-hemolytic renal failure in children with glucose-6-phosphate dehydrogenase deficiency (G6PD). Any child presenting hemoglobinuria during the study period was prospectively evaluated. Evaluation included detection of the presence of hemolytic agents, laboratory tests to measure hemolysis, G6PD activity, infection and renal failure, and assessment of outcome and management of hemolysis and renal failure. G6PD deficiency was documented in 32.1% of the 230 children admitted with hemoglobinuria. Anuric renal failure occurred during the hemolysis episode in 35.1% of patients with G6PD deficiency (21 boys and 5 girls between 30 months to 13 years old). Acute hemolysis associated with infection occurred before any treatment in 53.8% of cases and after beginning treatment in 46.1%. In 84.6% of cases, occurrence of acute hemolysis involved association of drugs considered as nonhemolytic either with themselves or with other drugs. Anuric renal failure occurred after beginning treatment in all cases and was most severe in patients with of multiple-germ infection (30.7%) and drug association (84.6%). Renal failure was reversible in 80.7% and fatal in 19.2%. Multiple-germ infection and drug association appeared as the main predisposing factors for post hemolytic anuric renal failure in patients with G6PD deficiency. The high frequency of these factors in tropical areas suggests implication of local endemic infections.