SMW Established in 1871 Swiss Medical Weekly Formerly: Schweizerische Medizinische Wochenschrift An open access, online journal • www.smw.ch Supplementum 222 Joint annual meeting Swiss Society of Paediatrics and ad Swiss Med Wkly the Swiss Society for Allergology and Immunology 2017;147 May 29, 2017 Jointly organized with the 4th Lymphoid Tissue Meeting “Focus on Stromal Cells” St. Gallen (Switzerland), June 1–3, 2017 Abstracts TABLE OF CONTENTS 1 S Oral Communications 2 S SGPO 1–SGPO 24 Free communications SSP 9 S SPN 1–SPN 11 Free communications SwissPedNet 13 S SGPP 1–SGPP 128 Posters SSP 48 S SSAIO 1–SSAIO 12 Short oral communications SSAI 51 S SSAIP 1–SSAIP 54 Posters SSAI 65 S LTMO 1–LTMO 12 Short oral communications LTM4 69 S LTMP 1–LTMP 59 Posters LTM4 Index of first authors 84 S Impressum Editorial board: © EMH Swiss Medical Publishers Ltd. All communications to: Listed in: Prof. Adriano Aguzzi, Zurich (ed. in chief) (EMH), 2016. 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FREE COMMUNICATIONS SSP 2 S SGPO 1 Background and aims: Recently, the definition of sepsis in adults has Androgen excess in an adolescent girl due to been refined as life-threatening organ dysfunction caused by a an ovarian tumor: diagnostic and therapeutic challenges dysregulated host response to infection. Objectives: We analysed the relationship of number of organ Haamberg T.1, Marti N.1, Malikova J.1, Escher G.2, Flück C.E.1 1 dysfunctions with case fatality rate in a prospectively collected dataset Department of Pediatrics, Division of Pediatric Endocrinology and on sepsis in children. Diabetology, Inselspital, Bern University Hospital, University of Bern, 2 Methods: Prospective observational cohort study of newborns and Switzerland; Department of Nephrology and Hypertension, Inselspital, children <17 years with blood culture-proven sepsis admitted to ten Bern University Hospital, University of Bern, Switzerland paediatric hospitals in Switzerland between 9/2011 and 12/2015. Background: Virilization in pubertal girls raises suspicion for late Sepsis and organ dysfunctions were defined according to the 2005 onset congenital adrenal hyperplasia (CAH), androgen producing pediatric consensus definition. tumor of adrenals or ovaries or polycystic ovary syndrome. Signs of Results: Of 1204 blood culture-proven sepsis episodes, organ virilization are hirsutism, acne, clitoromegaly and amenorrhea. dysfunction was present in 474 (39%). In 590 (49%) episodes patients Adrenocortical tumors in children are rare (0.3–0.4 per million). Recent were admitted to the intensive care unit, and in 323 (55%) of those work revealed that androgen production occurs through several episodes patients required mechanical ventilatory support. In 90 of biochemical pathways, e.g. the classic and backdoor pathway, but the 1204 (7.5%) episodes the outcome was fatal in the first 30 days after role of novel pathways in tumors is unknown. sepsis onset. The odds ratio of death increased by 2.9 (95%CI Objective: To assess the role of backdoor androgen production in a 2.5–3.5, p <0.001) for every additional organ dysfunction; from a case rare ovarian, androgen producing tumor. fatality rate of 0.7% (95%CI 0.3–1.7) in 730 episodes with no organ Case report, results and follow-up: We report on a 14-year-old girl dysfunction to 46% (95%CI 34.4–58.7) in 69 episodes with 4 or more with secondary amenorrhea, hirsutism and weight gain. On clinical organ dysfunctions. examination hirsutism, normal blood pressure, Tanner B5 P6 were Conclusion: Only a minority of children presenting with blood noted but no palpable abdominal mass. Laboratory work-up showed culture-proven sepsis as per 2005 pediatric consensus definition had high androgen levels in serum and urine, AFP was normal. Ultrasound an organ dysfunction. Presence and number of organ dysfunctions revealed a solid mass arising from the left ovary, MRI-scan confirmed were strongly associated with mortality, and should be considered for an ovarian tumor. Laparoscopic left salpingo-oophorectomy was future sepsis definitions to discriminate children with infection from performed. Histopathological assessment showed a very rare children with life-threatening dysregulated host response to infection. steroid-cell tumor NOS (“not otherwise specified”). Specific immunohistochemical studies of steroid enzymes showed upregulated enzymes of the classic and backdoor pathway such as RoDH (retinol SGPO 3 dehydrogenase), CYP17A1 (17α-hydroxylase) and AKR1C3 (aldo- Impact of introducing cerebrospinal fluid enterovirus ketoreductase family 1 C3). Postoperatively, androgen levels polymerase chain reaction testing on duration of normalized and menstrual bleedings became regular. But 12 months antimicrobial treatment and hospital stay in infants later, severe acne and amenorrhea relapsed, and androgen levels <90 days of age presenting with fever were found elevated again. To preserve fertility and prevent Barbey F.1, Paioni P.1, Berger C.1 ovarectomy, we tested whether tumoral hormone production is still 1 responsive to regulation. Dexamethasone was found to suppress University Children’s Hospital Zurich, Division of Infectious Diseases androgen levels significantly, while LH-RH did not inhibit hormonal and Hospital Epidemiology activity. Because the tumor had upregulated enzymes involved in the Background: Enteroviruses are the main cause of aseptic meningitis classic and backdoor pathway, we started the patient on Androcur® in infants presenting with febrile illness at the emergency room. Infants (an inhibitor of the androgen receptor). <90 days of age presenting with fever undergo a septical workup Conclusions: Our patient report shows that androgen production by including lumbar puncture and receive empirical antimicrobial the ovarian tumor occurs through the classic and the backdoor treatment not to miss serious bacterial infection. Introducing enteroviral pathway. Drugs Abiraterone and Finasterid would inhibit CYP17 or polymerase chain reaction testing in cerebrospinal fluid (CSF EV-PCR) 5α-reductase activities, respectively; both enzymes are part of classic may impact duration of antimicrobial treatment and hospital stay of and backdoor androgen production. Thus it is possible that those these infants, if the test result is readily available. We investigated this drugs that are in use for prostate cancer or CAH could be used to treat potential impact of introducing CSF EV-PCR in infants <90 days of age our patient’s tumor. However, these drugs are not in routine use in presenting with fever. pediatrics. Methods: Infants <90 days of age presenting with fever at the University Children’s Hospital Zurich between June 2015 and October 2016 and for whom CSF EV-PCR testing was performed were SGPO 2 investigated by chart review. Infants with bacterial meningitis were excluded. Duration of empirical antimicrobial treatment and hospital Frequency of organ dysfunction and impact on stay were compared between children with positive and negative CSF mortality in children with blood culture-proven sepsis – EV-PCR results as well as to a corresponding control group of infants results from The Swiss Pediatric Sepsis Study before the introduction of CSF EV-PCR. Agyeman P.1, Posfay-Barbe K.M.2, Giannoni E.3,4, Stocker M.5, Results: A total of 74 children were enrolled in the study, 47 in CSF Kuehni C.6, Heininger U.7, Bernhard-Stirnemann S.8, Hasters P.9, EV-PCR group and 27 in the control group, respectively. The median Niederer-Loher A.10, Kahlert C.10, Baer W.11, Relly C.12, Aebi C.1, age was 37 days in both groups (range 3 to 85 days). CSF EV-PCR Berger C.12, Schlapbach L.J.1,13 tests were positive in 25 of 47 patients (53%). CSF EV-PCR positive 1Department of Pediatrics, Inselspital, University Hospital Bern, patients showed a significant reduction in the mean duration of University of Bern, Switzerland; 2Department of Pediatrics, Children’s antimicrobial treatment compared to EV-PCR negative patients and to Hospital of Geneva, University Hospitals of Geneva and University of controls (23.1, 45.1 and 61.4 hours respectively, p