日本新生児成育医学会雑誌　第30巻　第2号　101～107頁（2018年）

日本新生児成育医学会雑誌　30(2)：349-355；2018　

受付日：平成30．01．12

受理日：平成30．03．09

Late preterm Wilson-Mikity 症候群の1 女児例：各種バイオマーカーの経日的測定による病態の検討

A Case of a Late Preterm Female Infant with Wilson-Mikity Syndrome： Serial Investigations of Several Kinds of Biomarkers for Clarification of the Pathophysiology of This Disease

＊1武蔵野赤十字病院　新生児科，＊2東京大学医学部附属病院　小児科，＊3名古屋市立西部医療センター　小児科

＊1Department of Neonatology, Japanese Red Cross Musashino Hospital，＊2Department of Pediatrics, the University of Tokyo Hospital，＊3Department of Pediatrics, the Nagoya City West center

中村利彦＊1・畠中大輔＊1・草苅倫子＊1・高橋秀弘＊1・蒲原　孝＊1・鹿嶋晃平＊2・高橋尚人＊2・後藤玄夫＊3

Toshihiko NAKAMURA＊1，Daisuke HATANAKA＊1，Michiko KUSAKARI＊1，Hidehiro TAKAHASHI＊1， Takashi KAMOHARA＊1，Kohei KASHIMA＊2，Naoto TAKAHASHI＊2，Haruo GOTO＊3

Key Words：late preterm，Wilson-Mikity syndrome，biomarkers，acute phase reactants score（APR-score）， cytokine profiles

　Late preterm のWilson-Mikity syndrome（MWS）の検討は少ない。症例は，在胎36 週6 日，出生体重2,654g の女児。出生1 週間前に不正性器出血を契機に腹緊増強し，経腟分娩で出生した。絨毛膜羊膜炎はⅠ°，臍帯炎なし。児は，軽度の呼吸障害が遷延した。臍帯血IgM 49.6mg/dL，WBC 22,300/μL，CRP 0.08mg/dL であった。胸部レントゲンは，索状陰影の増強と透過性低下を認めた。日齢10 の胸部CT は，肥厚した間質性病変に過膨張した気腫状病変が混在していた。APR-score は，日齢0 でHp・AGP 陽性の2 点で日齢2 まで持続し以後漸減した。臍帯血 IL-8 は，2,123.7pg/mL と著高しており，その後急激に低下した。その他IL-1β，IL-6 も上昇していた。KL-6 は日齢 8 に868U/mL の頂値を呈して以後漸減した。以上より，胎児炎症反応症候群を発症し，その炎症の亜急性期に出生後が該当するものと判断した。preterm WMS の多くは，中等度以上のCAM と臍帯炎が存在するが，APR-score が 0 ないし1 点であることが多く，胎内において炎症が既に消退しかけた状態と報告したが，この時相の相違が興味深い。

　We present a case of a late preterm female infant at 36 weeks of gestation with Wilson-Mikity syndrome（ WMS）. The mother developed an intrauterine infection 1 week before delivery and experienced a natural labor attack resulting in the infant being born by vaginal delivery. After birth, the respiratory disorder was not particularly severe and, after admission to the NICU, the patient was manageable with solely low concentration of oxygen（ maximum FiO2 0.25） in the incubator. Mild tachypnea and retraction eventually lasted until 2 months after birth, but eventually resolved. The patient’s growth and development is now within normal limits at the age of 2 years.
　We preserved residual serum from day to day with umbilical cord blood from Day 0 after birth and analyzed 17 types of cytokine profiles, including acute phase reactant proteins（ CRP, haptoglobin, and α1-acidglycoprotein）, pulmonary biomarkers（ KL-6, SP-A and SP-D）, and IgM. IL-1beta, IL-6 and IL-8 were markedly elevated and chorioamnionitis on placental histopathology was mild. However, the patient still presented with fetal inflammatory response syndrome. We determined that the transition of the inflammatory response after birth was not in the acute phase but in the subacute phase. Chest CT findings taken at 10 days of age were also consistent with WMS findings as in the previous report. Compared with the gestational age of less than 32 weeks prevalent for WMS, inflammation was observed in the lungs of this patient after the alveolar period, suggesting that the postnatal respiratory disorder did not become too severe.