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La Covid-19 s’est abattue sur l’Alsace en quelques jours, mettant à mal les hôpitaux de Mulhouse et de Colmar. Bien que proches, des différences notables existent au sein de ces deux structures en termes architecturaux et de ressources humaines. Comment cette vague a-t-elle modifié les organisations de chacun ? Quelles alternatives ont pu être mises en place ? Après la présentation de chaque site sont analysées les adaptations nécessaires afin d’absorber les flux de patients et leur proposer une prise en charge décente malgré des conditions parfois très dégradées. Nouvelles filières, modifications organisationnelles, renforts de personnel en interne puis en externe, augmentation capacitaire nette, recherches de solutions alternatives aux voies classiques d’approvisionnement des matériels… ont été une partie de la solution. Malgré les alertes du terrain, l’action nationale est restée en décalage de compréhension de la gravité de la situation locale, avec des recommandations et des actions soit inapplicables, soit trop tardives. Les établissements et professionnels de santé ont eu un sentiment d’isolement, renforcé par l’absence de représentant des tutelles et notamment de l’Agence régionale de santé sur le terrain. Deux éléments majeurs peuvent être mis en avant : une cohésion majeure de l’ensemble du centre hospitalier avec travail d’équipe et de terrain entre soignants et direction, et une implication de l’ensemble des acteurs de la médecine d’urgence (public, privé, libéraux, service départemental d’incendie et de secours) ; ils ont été les déterminants dans notre capacité à faire face à cet événement inédit.

We aim to assess physicians’ level of resilience and define factors that improve or decrease the resilience level during the COVID-19 pandemic. Physicians from hospitals located in areas with different COVID-19 caseload levels, were invited to participate in a national e-survey between April and May 2020. Study participants were mainly emergency physicians, and anaesthesiologists, infectious disease consultants, and intensive care. The survey assessed participant’s characteristics, factors potentially associated with resilience, and resilience using the Connor-Davidson Resilience Scale (RISC-25), with higher scores indicative of greater resilience. Factors associated with the resilience score were assessed using a multivariable linear regression. Of 451 responding physicians involved in the care of COVID-19 patients, 442 were included (98%). Age was 36.1 ± 10.3 years and 51.8% were male; 63% worked in the emergency department (n = 282), 10.4% in anesthesiology (n = 46), 9.9% in infectious disease department (n = 44), 4.8% in intensive care unit (n = 21) or other specialties (n = 49). The median RISC-25 score was at 69 (IQR 62–75). Factors associated with higher RISC scores were anesthesia as a specialty, parenthood, no previous history of anxiety or depression and nor increased anxiety. To conclude, this study is the first to characterize levels of resilience among physicians involved in COVID-19 unit. Our data points to certain protective characteristics and some detrimental factors, such as anxiety or depression, that could be amenable to remediating or preventing strategies to promote resilience and support caregivers in a pandemic.

Objectives Patients hospitalized for influenza should receive early treatment with a neuraminidase inhibitor. Patients and methods We conducted a retrospective study of the prescription of oseltamivir during the 2016–2017 influenza epidemic among patients hospitalized for influenza confirmed by RT-PCR in the infectious disease department. Results Treatment with oseltamivir was initiated as recommended in 96% of hospitalized patients presenting with influenza. However, a delay in prescription was observed with only 18% of prescriptions made on the first day. The prescriptions were exclusively initiated in the infectious disease department. Conclusion To improve the early prescription of oseltamivir during the influenza season, two recommendations are essential: oseltamivir availability in the emergency department pharmacy, awareness of physicians of the need to prescribe to any patient hospitalized for a lower respiratory tract infection treatment with a neuraminidase inhibitor upon admission to the emergency department.

Introduction Chaque annee la grippe saisonniere est responsable de nombreux passages aux urgences, hospitalisations et deces. Sa prise en charge repose sur l’introduction rapide d’un traitement par inhibiteur de neuraminidase (IN) chez les patients a risque ou hospitalises. En pratique, de nombreux defauts ou retards de prise en charge sont notes. L’arrivee de tests moleculaires de diagnostics rapides plus sensibles que les TROD classiques peut aider a la prise en charge des grippes notamment en SAU. Durant la saison 2017-18 le test moleculaire rapide Alere-I® a ete introduit au service d’accueil d’urgence (SAU) adulte de notre etablissement mais suite a de nombreuses ruptures de stock certains patients ont continue a beneficier durant cette saison d’un depistage classique par RT–PCR technique en laboratoire. Une etude retrospective a ete menee pour comparer l’apport du test rapide sur la prise en charge des patients grippes. Materiels et methodes Etude retrospective avec inclusion de tout patient de 18 ans et plus avec resultat positif de grippe par test moleculaire Alere-I® realise au SAU ou RT–PCR classique realise au laboratoire. Les parametres etudies comprenaient le nombre de traitements par IN, leur delai d’introduction, le nombre d’antibiotherapies, d’hospitalisations et les isolements respiratoires mis en place. Resultats 451 tests rapides ont ete realises dont 119 tests positifs (26 %) versus 365 tests RT–PCR dont 128 tests positifs (35,1 %) ; 72 et 106 dossiers ont pu etre exploites. Les 2 groupes ne se differenciant pas en terme d’âge de sexe-ratio et de repartition des souches virales A/B. Au total,57 % de traitements par IN ont ete debutes dans le groupe test rapide contre 62 % dans le groupe RT–PCR (NS), mais avec un delai de mise en route plus court (9h32 vs 23h41 ; p Conclusion Les tests moleculaires rapides de grippe au SAU sont un moyen efficace d’ameliorer la prise en charge des patients grippes en periode epidemique.

Resume Les anomalies de la differenciation du sexe relevent de multiples etiologies et sont susceptibles d’un retentissement physique et psychologique important ; certaines provoquent une anomalie des organes genitaux (internes et externes) des individus au caryotype 46 XY. Notre travail concerne ceux qui seront eleves en fille. Il porte sur l’analyse du fonctionnement psychique (identite sexuee, image de soi, image du corps, angoisse, depression, etc.) et cognitif, vise l’etude des comportements sexuels et apprecie la qualite de vie. Le protocole de recherche propose aux 15 femmes rencontrees consiste en un entretien semi-directif, une echelle d’intelligence (Wechsler Adult Intelligence Scale [WAIS] III), deux epreuves projectives (Rorschach et TAT), une echelle de qualite de vie (WHOQOL 26), un questionnaire des comportements sexuels. Les donnees analysees au niveau qualitatif et quantitatif montrent des capacites cognitives heterogenes et dans la limite inferieure de la normale ; les huit femmes qui ont change d’assignation sexuee pendant l’enfance (de garcon vers fille) ont des resultats plus faibles que les sept autres. La qualite de vie est significativement faible du point de vue psychologique et dans le domaine des relations sociales. La vie amoureuse et sexuelle de ces femmes est marquee par une forte inhibition ; l’acte heterosexuel est evite alors que la sexualite genitale homosexuelle est significativement plus elevee que dans la population generale. Le doute, quant a leur identite de femme, est patent et s’associe souvent a des difficultes dans les relations a la mere. Les secrets familiaux impregnent leurs recits de vie. L’isolement social et familial est marque et la souffrance psychique manifeste. Enfin, l’ensemble des femmes se plaignent de l’absence d’une « parole parlante » de leurs parents, notamment de leur mere, sur leur histoire personnelle.

Resume Les thrombopenies secondaires aux chimiotherapies anticancereuses peuvent provoquer des hemorragies genitales severes au cours de l’adolescence. Un traitement œstroprogestatif entrainant une amenorrhee therapeutique est possible, mais peut augmenter les risques thromboemboliques et hepatiques de certaines chimiotherapies. Objectifs de l’etude. – Evaluer retrospectivement le risque d’hemorragie genitale pour des adolescentes presentant une thrombopenie severe apres chimiotherapie. Patientes et methodes. – Parmi les 140 jeunes filles âgees de 12 a 18 ans traitees entre 1991 et 1998 par chimiotherapie, 24 jeunes filles puberes ayant presente au moins un episode de thrombopenie severe (taux de plaquettes Resultats. – Six adolescentes sur 24 ont recu un traitement preventif œstroprogestatif. Un seul episode d’hemorragie genitale, sans risque vital associe, a ete observe, chez une adolescente ne recevant pas de traitement hormonal preventif. Trente-trois pour cent des patientes presentaient une amenorrhee spontanee avant la chimiotherapie et 17 % une insuffisance ovarienne secondaire definitive. Conclusion. – Le risque hemorragique est faible chez les adolescentes soumises a une chimiotherapie entrainant une thrombopenie. L’amenorrhee observee frequemment au debut du traitement, qu’elle soit fonctionnelle en rapport avec l’amaigrissement ou secondaire a la toxicite de la chimiotherapie, rend discutable la necessite d’un traitement hormonal preventif.

Introduction Les donnees des patients pris en charge pour exacerbation d’asthme au service des urgences des hopitaux civils de Colmar ont ete analysees sur une periode de plus d’un an, afin d’en connaitre les caracteristiques. Methodes Notre etude prospective s’est deroulee du 1er juin 2015 au 15 juillet 2016, aux urgences des hopitaux civils de Colmar et incluait des patients de plus de 17 ans presentant une exacerbation d’asthme. Un questionnaire a ete mis en place dans le service des urgences et complete par les medecins urgentistes, dans le but de recueillir les donnees concernant les caracteristiques principales de ces patients. Nous avons egalement recueilli les donnees des patients qui se sont rendus a la consultation specialisee post urgence, mise en place en pneumologie pour l’etude, afin d’evaluer leur devenir. Resultats Les donnees de 46 patients pris en charge aux urgences pour asthme ont ete analysees : il s’agissait de sujets en majorite jeunes de moins de 45 ans (moyenne d’âge de 39,5 ans), sans suivi pneumologique specialise (58,7 % des patients) et ne beneficiant d’aucun traitement de fond pour la moitie d’entre eux. Quinze pour cent des patients avaient deja ete hospitalises aux urgences au moins une fois pour exacerbation de leur asthme dans l’annee precedente. D’apres le traitement de fond dont ils beneficiaient au moment de leur exacerbation, il s’agissait d’asthme persistant modere a severe dans un peu plus de 40 % des cas (traitement de palier 3 ou 4–5, [1] ). Le facteur declenchant de l’exacerbation etait principalement infectieux (75 % des cas) ou lie a une exposition allergenique (1/4 des cas). Il s’agissait d’exacerbations legeres a moderees dans 60 % des cas, severes dans 40 % des cas, d’apres les criteres GINA [1] . Apres leur passage aux urgences, les patients ont regagne leur domicile dans 80 % des cas et plus de 60 % d’entre eux ont ete vus en consultation post-urgence. Parmi ces patients revus au decours immediat de l’exacerbation, le traitement de fond de leur asthme a ete considere comme insuffisant dans plus de la moitie des cas. Aucun de ces patients n’a finalement ete candidat a une biotherapie. Conclusion Dans le contexte de la prise en charge de l’asthme, une collaboration etroite entre medecins urgentistes et pneumologues parait donc utile, dans le cadre d’une filiere asthme et urgence, dont le but est l’amelioration du controle de l’asthme et la prevention des exacerbations, dans une population de patients asthmatiques majoritairement sans suivi specialise.

Long cycles and excessive uterine bleeding are the most common menstrual disorders in adolescents. Long cycles usually result from a benign transitory functional disorder which spontaneously resolves within two to three years after menarche; however, this diagnosis can only be retained in the absence of hirsutism or galactorrhea as revealing symptoms of functional ovarian hyperandrogenism, polycystic ovary syndrome, late form of congenital adrenal hyperplasia or hyperprolactinemia; ovarian failure will be considered in case of isolated long cycles persisting more than two years after menarche. Excessive uterine bleeding usually occurs early after menarche. Although the main cause is functional, resulting from anovulation and the related absence of progesterone, coagulation disorders must always be excluded. Management of these disorders are described.

To determine the clinical characteristics of pubertal metrorrhagia and its treatment, depending on severity; to evaluate the frequency of etiologies and the influence of hemostatic abnormalities; and to describe severe pubertal metrorrhagia. Design, setting and participants. This retrospective study examined all the files (n = 105) of adolescents presenting for pediatric gynecology consultation at a children's hospital between January 1979 and June 1993.The mean age of patients at the first consultation was 13 years. Metrorrhagia began in the year after the first menstrual period in 85% of cases. The causes were functional (83 cases), primary hemostatic disorder (14 cases), hemostatic disorder secondary to renal or hepatic disease (7 cases), or tumor (1 case). The cases were assigned to one of three groups, according to the severity of anemia; group I, mild anemia, hemoglobin Hb11 g%, 64 cases; group II, moderate anemia, Hb 8-11 g%, 23 cases; and group III, severe anemia, Hb8 g%, 18 cases. Of the 14 patients suffering from primary hemostatic disorders, 7 had been diagnosed before the onset of metrorrhagia; the disorder was revealed by the metrorrhagia in the remaining 7. Only 1 of these patients was severely anemic (known factor X deficiency). Four patients suffering from moderate von Willebrand's disease were discovered after specific tests; they were mildly or moderately anemic. The severe anemias (group III) all occurred during the first three periods. This group had functional disorders in 15 of 18 cases. Treatment continued to be required in 10 of 18 cases followed for more than 3 years. Treatment was progestin for group I and II patients or an estroprogestin, followed by a progestin, for group III. Curettage was never required.The most common cause of pubertal metrorrhagia is a functional disorder (80% of cases). Hemostatic disorders likely to cause severe menstrual hemorrhage were known before the age of menarche; these disorders must be controlled by hormone treatment begun before or at the time of the first menstrual period. Severe forms that arise during the initial three menstrual cycles are functional in most cases. These should be given a course of treatment lasting several years. There is a high risk of recurrence. Treatment is medical and hormonal in all cases.

Resume La coalescence des petites levres est une affection frequente, benigne et asymptomatique de la petite fille. Que le decollement soit fait avec ou sans pommade anesthesiante, avec ou sans preparation par les estrogenes ou sous anesthesie generale, il est toujours inutile, souvent traumatisant et dans la majorite des cas suivi de recidive. L’accolement regresse toujours spontanement au cours de l’enfance ou au plus tard lors des premieres modifications vulvaires qui accompagnent le demarrage pubertaire.

The human testis-determining factor resides within a 35-kilobase (kb) region of the Y chromosome immediately adjacent to the pseudoautosomal boundary. A candidate gene for human sex determination (SRY) was isolated in this region. Here, we describe a study of 25 cases of XY females with pure gonadal dysgenesis for mutations on the Y chromosome short arm, including SRY. Southern blotting revealed a sex-reversed female harboring a deletion extending from approximately 8 kb from the pseudoautosomal boundary of the Y chromosome to at least 33 kb and no more than 60 kb upstream, toward the centromere. The deletion begins no more than 1.8 kb upstream from the first ATG of the SRY open reading frame present in the clone pY53.3. To our knowledge, no mutation has been described previously outside the SRY "HMG box" on the short arm of the Y chromosome, which is associated with sex reversal. Since the 5' extent of the SRY transcriptional unit has not been defined, the deletion may remove upstream exons of SRY and/or transcriptional regulatory motifs, either situation resulting in lack of testicular development. It cannot be formally excluded that the mutation removes a second locus, independent of SRY, that is critical for sex determination. Denaturant gradient gel electrophoresis analysis of the SRY open reading frame in the remaining 24 cases revealed de novo single base-pair transitions in the SRY conserved domain in 4 cases.

Lichen planus is less common in children about in adults and mostly affects the skin.A 9-year-old girl was referred for a purplish blue and violaceous, unilateral and asymptomatic vulvar lesion noted 4 months earlier and stable. Histopathologic examination showed typical features of lichen planus. She responded well and rapidly with topical steroids.We report the first case of vulvar lichen planus in a little girl. Mucosal involvement is uncommon in children with lichen planus and genital localisation is extremely rare. Vulvar lichen sclerosis is the principal differential diagnosis. The risk of vulvar synechia and development of vulvar carcinomas warrants regular long-term follow-up.

Parmi les consequences endocriniennes des hydrocephalies, l'amenorrhee n'est pas exceptionnelle ; le retard pubertaire et l'obesite en sont les symptomes les plus frequents. Apres la derivation, la fonction hypothalamique gonadotrope se normalise en quelques mois dans la majorite des cas. Observation. - Une adolescente de 16 ans porteuse d'une maladie de Recklinghausen se plaignait d'une amenorrhee 2 ans apres le debut de sa puberte. La radiographie du crâne montrait une selle turcique agrandie qui va conduire au diagnostic d'hydrocephalie par stenose de l'aqueduc de Sylvius. L'hydrocephalie s'etait exprimee par une avance du perimetre cranien a + 3 DS des le premier mois de vie. Il s'agissait donc d'une stenose congenitale de l'aqueduc de Sylvius qui dans le cadre de la maladie de Recklinghausen est rare, de l'ordre de 2 %. Conclusion. - Toute avance de perimetre crânien qui atteint la 3 e DS superieure doit etre exploree par une echographie transfontanellaire. Le traitement chirurgical, indique dans toutes les formes symptomatiques de stenose de l'aqueduc de Sylvius est base sur la ventriculocistemostomie par voie endoscopique ; c'est dire l'importance d'un diagnostic precoce.

Fibroadenomas and breast growth disorders are the most common breast diseases in adolescent women. Assessment of breast disorders in this age group generally involves clinical evaluation through history and physical examination and when is needed ultrasonography. Due to the absence of breast cancer in adolescent women, it is easy to reassure women at the first consultation. Breast growth disorders can lead great psychological and physical embarrassment. Treatment consists of surgical procedures when the cosmetic defect is severe. According to the ANDI classification, small fibroadenomas are normal, clinical fibroadenomas are a mild aberration of the normal processes, and giant or multiple fibroadenomas are placed to the disease end of the spectrum. Fibroadenomas can be treated conservatively provided diagnosis is confident. Giant fibroadenomas are treated by surgical enucleation. Breast abscess is mainly due to the duct ectasia. In adolescence, ectasia has been described as an exaggeration of sinus duct development and can be considered as a variant of normality. Diseases of the adolescent breast are usually benign and their management are simple using medical strategy and more rarely surgical therapy.

Severe genital bleeding during adolescence can occur in the event of thrombocytopenia related to chemotherapy. Preventive hormonal treatment to induce therapeutic amenorrhea is recommended by some clinicians. Nevertheless, the adverse effects of oestroprogestative treatment, such as thromboembolic risk or hepatic toxicity, could potentialize the adverse effects of some chemotherapies.To assess retrospectively the risk of genital bleeding associated with thrombocytopenia secondary to chemotherapy in a population of adolescent girls for whom therapeutic amenorrhea was induced or not.Among 140 girls, 12 to 18 years old, who were subjected to chemotherapy between 1991 and 1998, 24 girls presented at least one thrombocytopenic event (platelet level20 x 10(9) l(-1)) and were included.Six out of 24 adolescent girls received hormonal therapy to induce amenorrhea. Only one bleeding event was observed, in a girl who had not received preventive oestroprogestative treatment. Vital prognosis was preserved. Furthermore, spontaneous amenorrhea occurring before chemotherapy was observed in 33% of the patients and permanent secondary ovarian insufficiency in 17% of the patients.The risk of genital bleeding among adolescent population at risk of chemotherapy-induced thrombocytopenia is low. The high frequency of amenorrhea, secondary to weight loss or to chemotherapy toxicity, should raise questions as to the usefulness of preventive oestroprogestative treatment.

Objective: To report the first case of a 46XX female infant with Smith-Lemli-Opitz syndrome (SLOS), adrenal insufficiency and abnormal genitalia. Methods: The patient was assessed for hormonal status on day 4 and 6 months later and was followed-up from the study time (2.5 years of age). Results: The patient had a persistent urogenital sinus, posterior labial fusion without clitoromegaly. She presented with a salt-wasting syndrome on day 4. Adrenal insufficiency was confirmed. Adrenal androgen levels, including 17-hydroxyprogesterone and 11-deoxycortisol were moderately elevated. Conclusion: Children with SLOS should be assessed for adrenal insufficiency. In female infants, abnormal external genitalia can be observed even if the precise mechanism behind these abnormalities is yet to be determined.

Long cycles and excessive uterine bleeding are the most common menstrual disorders in adolescents. Long cycles usually result from a benign transitory functional disorder which spontaneously resolves within two to three years after menarche; however, this diagnosis can only be retained in the absence of hirsutism or galactorrhea as revealing symptoms of functional ovarian hyperandrogenism, polycystic ovary syndrome, late form of congenital adrenal hyperplasia or hyperprolactinemia; ovarian failure will be considered in case of isolated long cycles persisting more than two years after menarche. Excessive uterine bleeding usually occurs early after menarche. Although the main cause is functional, resulting from anovulation and the related absence of progesterone, coagulation disorders must always be excluded. Management of these disorders are described.

The authors wish to thank Nicole Souleyreau for excellent technical assistance and Drs. Marguerite Prieur, Catherine Turleau, Geraldine Viot, and Michel Vekemans for helpful discussions. R.A.V. has been supported by the Fondation pour La Recherche Medicale.

Resume Introduction Le lichen plan de l’enfant est plus rare que celui de l’adulte ; sa localisation est essentiellement cutanee. Observation Une enfant de 9 ans etait adressee pour une lesion situee a la face interne de la grande levre gauche, bleu violine, asymptomatique decouverte depuis 4 mois et stable. L’examen anatomopathologique revelait un lichen plan vulvaire. L’evolution etait rapidement favorable sous dermocorticoides de classe tres forte. Discussion Nous rapportons le premier cas de lichen plan vulvaire isole chez une petite fille. Au cours du lichen plan de l’enfant, l’atteinte muqueuse est rare, et la localisation genitale exceptionnelle. Le principal diagnostic differentiel est le lichen sclereux vulvaire. Les risques de synechies vaginales et de transformation maligne, connus chez l’adulte, justifient une surveillance au long cours.

We report a child with Down's syndrome in whom metrorrhagia and precocious puberty revealed primary autoimmune hypothyroidism. The patient had a decreased growth velocity, exaggerated weight gain, bone age delay, and bilaterally enlarged multicystic ovaries. Delays in the diagnosis and treatment of hypothyroidism can lead to this peculiar presentation.

During mammalian embryogenesis, the presence of the SRY gene determines the bipotential gonad to develop as a testis. 46,XY sex reversal has been described in man. It is associated with an essentially female phenotype and a streak gonad. In a collaborative study, we analysed 36 patients with a 46,XY sex reversal. The testis determining region of the Y chromosome was analysed by Southern blotting and by DGGE analysis of the SRY open reading frame (orf). We found a total of 7 mutations in the testis determining region including the SRY gene. This brings to 19 the total number of mutations in SRY associated with sex reversal. No relationship was found between the SRY status and the presence or absence of gonadoblastoma. However, a correlation was observed between the SRY genotype and the histology of the gonad. A mutant in SRY is associated with a completely dysgenetic gonad. The presence of immature testicular tubules is usually observed when SRY is normal. These latter results suggest the existence of as yet unidentified testis determining genes.

Ein 9jahriges Madchen wurde in die senologische Sprechstunde uberwiesen wegen einer seit 3 Monaten bestehenden Schwellung und lividen Verfarbung der linken Mamille, welche trotz lokaler antibiotischer, antimykotischer und Steroid-Therapie persistierte. Ipsilateral in der Axilla fanden sich einige vergroserte dolente Lymphknoten. Die genaue Anamneseerhebung ergab, das das Machen 4 Wochen vor Auftreten der Mamillenveranderung an der linken Mamma von einer Zecke gebissen wurde. Die Borrelien-Serologie ergab folgende Werte: IF-Abs-Test 5120 (normal bis 40), IF abs IgM 80, P39 EIA grenzwertig. Der Western-Blod auf Borrelia burgdorferi fiel im IgG- und IgM-Bereich positiv aus, wodurch eine Borreliose serologisch gesichert war. Histologisch konnte bei einer aus der linken Mamille entnommenen Probebiopsie die klinische Diagnose einer Lymphadenosis cutis benigna Bafverstedt nach Zeckenbis bestatigt werden. Unter systemischer antibotischer Therapie mit Fenoxypen 3x1 Mio/die wahrend 14 Tagen bildete sich die lokale Symptomatik deutlich zuruck.

Treatments given for childhood malignancies can alter gonadal function by several mechanisms: (1) cranial irradiation may cause either gonadotrophin deficiency or premature puberty. (2) Irradiation of the testes can induce germinal epithelium dysfunction even if the dose delivered is very low; Leydig cell failure occurs beyond 5-6 Gy and ovarian insufficiency beyond 6-7 Gy. (3) Chemotherapy is considerably more toxic for the germinal epithelium of the testes than for the ovaries; alkylating agents are especially toxic. Analysis of the consequences of preparation for bone marrow transplantation by chemotherapy or total body irradiation will be required when longer follow-up are available.

The GnRH analogue Buserelin was given for one year to six girls with central precocious puberty in a daily subcutaneous dose of 20 micrograms/kg/day. A decrease of plasma estradiol and vaginal maturation index to prepubertal values was obtained in 5 out of 6 cases. Bone maturation decreased and final predicted adult height improved significantly. This analogue of GnRH appears to be an effective medication for gonadotropin dependent precocious puberty in girls.

In a continued surgical and medical evaluation of the surgical management of female congenital adrenal hyperplasia, 48 girls were studied. Clitoroplasty (relocation and recession) was considered satisfactory in 22 out of 26 children of pubertal age. Vaginoplasty results were rated as excellent in 8 cases, and fair in 19 cases. Six patients indicated to have normal sexual intercourse. However a second surgical procedure will be necessary in 16 cases (30%) during or at the end of their puberty. These cases are among the more severely masculinized with a vaginal opening located at the upper part of the urethra. A prolonged follow-up is necessary in order to perform an adequate evaluation of these procedures, some of which have to be repeated at the age of adolescence.

Dexamethasone has been proposed as therapy of congenital adrenal hyperplasia in adolescents with menstrual abnormalities. Seven patients previously treated with hydrocortisone (mean dosage of 26 mg/m2/day) or prednisone (6 mg/m2/day), received dexamethasone doses between 0.3 and 0.73 mg/m2/day during at least 4 months. Regular menses and satisfactory adrenal suppression were obtained in most cases, but the occurrence of severe striae, sometimes with excessive weight gain, led to the cessation of this treatment. Analysis of data indicate that glucocorticoid activity of dexamethasone is 50-90 times more potent than that of hydrocortisone. Its use, after cessation of growth, should be carefully monitored, starting with much lower doses when necessary.

True precocious puberty occurred in 16 children (15 girls and 1 boy) with non tumoral shunted hydrocephalus at a mean age of 6.8 years. They had mild clinical manifestations of precocious puberty, and the other pituitary functions were found to be normal. Except for one child, precocious puberty did not correlate with raised intracranial pressure or lack of cerebral drainage by the shunt. Growth was the main concern in this group as the predicted height fell at a mean value of 1.7 SD below the parental target height, and even more in children with myelomeningocele. This growth retardation is due to an early progression of bone age observed even prior to the appearance of breast or pubic hair. Therefore we suggest that these children might benefit from early treatment by an LHRH analogue as soon as precocious puberty occurs.

Although information on contraception is readily available and a 1974 French law allows adolescents to receive contraceptives in family planning centers at no cost and without parental consent, over 1/2 of French adolescents have their 1st sexual intercourse with contraceptive protection and 13,000 adolescents under 17 become pregnant each year. Factors in the imperfect access of adolescents to contraception include conflicts with adult sources of contraception information, faulty perception of the risk of pregnancy, and presentation of contraceptive information in rational and technical terms to the exclusion of affective and relational aspects. Practical difficulties in finding a physician and paying for the consultation and fear of the examination itself are obstacles for some adolescents. The ambivalence of family planning providers faced with very young clients can be a significant barrier to access. At the 1st consultation, the adolescent should be seen alone. A complete medical consultation should be carried out including discussion of the adolescent's activities, habits, and affective life. Information on sexually transmitted diseases should be provided in a straightforward way, with possible symptoms included. The consultation usually ends in prescription of a combined oral contraceptive (OC), but for undecided adolescents or those with episodic sex lives several prescriptions may be given to provide a choice between OCs, condoms and spermicides, or the morning-after pill. Follow-up usually entails evaluation of weight and blood pressure, a gynecological examination with annual Pap smear, and management of secondary effects. A breast examination is necessary because of the sensitivity of breasts to estrogen at this age. OCs with a progestin dominance should be chosen for adolescents. In case of menstrual headaches the OC should be modified or terminated. Minor side effects such as acne are often the cause of termination and should not be ignored. Follow-up should be frequent and regular in the 1st year. Very young adolescents should be given standard-dose OCs. In case of absolute contraindications to combined OCs, a low-dose progestin pill may be selected. A low-dose pill and dietary counselling may be provided in case of weight gain, and an estrogen-dominant pill may help in case of moderate acne. OCs should be stopped in the event of hepatitis or expected surgery. Combined OCs are usually without medical contraindications and well tolerated by adolescents.

The present review is based on the retrospective study of 124 children with precocious puberty, 92 girls and 32 boys. In girls, the analysis of the clinical initial presentation has shown that premature pubarche (n = 18), premature adrenarche (n = 2) or isolated menstruations (n = 3) must be ruled out, as these symptoms can remain isolated for more than a year. However, in most cases the presence of growth acceleration and vaginal estrogenisation was of major diagnostic value. Bone maturation, although generally accelerated, can be normal in recently developed puberty. Precocious puberty may proceed by steps, with complete disappearance of physical signs in the intervals. Organic causes were found in 31% of the girls, and 44% of the boys with some characteristic features as rapid progression, and elevated LH response to LRF stimulation. Main causes were glioma of the optic chiasma (n = 11), 3rd ventricule invasive tumors, hamartoma (n = 8). The latter should be looked for by a non invasive procedure as the CT scan. In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. As the effect of precocious puberty on the final adult height is variable, the evaluation of therapeutic results remains uncertain. Medroxyprogesterone as well cyproterone acetate have not been fully efficient in controlling bone maturation. More recently, and still controversial, the treatment with long acting LRF analogues might provide a more satisfactory statural prognosis.

The use of pelvic ultrasonography was evaluated as a diagnostic and follow-up tool in girls with precocious puberty. Before treatment 23 of 33 patients with central precocious puberty presented an increased size of the uterus. In 10 cases with prepubertal size of the uterus, the precocious puberty was only beginning or of mild severity. During treatment with a LHRH analogue, changes in uterine size were slow in spite of a satisfactory and rapid control of estrogen secretion. At onset of treatment, transient ovarian cysts were seen in 2 patients. In our experience, pelvic ultrasonography did not provide significant information on the control of the disease by LHRH analogue therapy. Of 16 girls with presumed premature thelarche, 3 presented signs of estrogenic stimulation of the uterus. It remains a useful technique to rule out the presence of ovarian cysts or tumors at time of diagnosis.

Ovarian function was investigated in 17 patients aged 13 5/12 to 30 years who had received various types of combined chemotherapy without any irradiation. Ovarian insufficiency was found in 6 cases with amenorrhea (n = 5) or irregular menstruations (n = 1). There is a high risk of sterility in these cases although as described in one case, a normal pregnancy occurred in spite of evidence of ovarian failure. Cyclophosphamide seemed to be less harmful when given before puberty. Great variations in individual susceptibility for relatively low doses were observed with this drug. The combination with other drugs in some protocols might play a role in these cases. At variance with results reported in adults, the MOPP chemotherapy used in children with Hodgkin's disease did not induce ovarian dysfunction.