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Placental pathology predicts infantile neurodevelopment
No full text浜松医科大学Hamamatsu University School of Medicine博士（医学）The aim of present study was to investigate the association of placental pathological findings with infantile neurodevelopment during the early 40 months of life. 258 singleton infants were enrolled in the Hamamatsu Birth Cohort for Mothers and Children (HBC Study) whose placentas were saved in our pathological division. To assess the infantile neurodevelopment, we used Mullen Scales of Early Learning (gross motor, visual reception, fine motor, receptive language, expressive language) at 10, 14, 18, 24, 32, and 40 months. For obtaining placental blocks, we carried out random sampling and assessed eleven pathological findings using mixed modeling identified ‘Accelerated villous maturation’, ‘Maternal vascular malperfusion’, and ‘Delayed villous maturation’ as significant predictors of the relatively lower MSEL composite scores in the neurodevelopmental milestones by Mullen Scales of Early Learning. On the other hand, ‘Avascular villi’, ‘Thrombosis or Intramural fibrin deposition’, ‘Fetal vascular malperfusion’, and ‘Fetal inflammatory response’ were significant predictors of the relatively higher MSEL composite scores in the neurodevelopmental milestones by Mullen Scales of Early Learning. In conclusion, the present study is the first to report that some placental pathological findings are bidirectionally associated with the progression of infantile neurodevelopment during 10–40 months of age
A silicone disc for liver retraction in laparoscopic gastrectomy reduces the postoperative increase in the liver enzyme level
Get PDF浜松医科大学Hamamatsu University School of Medicine博士（医学）doctoral医学系研究科thesi
Early Diagnosis and Prompt Waiting List Registration Leading to Deceased-Donor Liver Transplantation in a 4-Month-Old Patient with Ornithine Transcarbamylase Deficiency
Get PDFオルニチントランスカルバミラーゼ欠損症（ornithine transcarbamylase deficiency：OTCD）は，X染色体連鎖性遺伝形式により生じる尿素サイクル異常症であり，オルニチントランスカルバミラーゼの先天的欠乏によりアンモニアが体内に蓄積する疾患である．本症では高アンモニア血症により重篤な神経障害きたすため，一部の症例では肝移植が必要となる．症例は母方家系にOTCD家族歴を有する男児．出生直後よりOTCDの精査が進められた．日齢0に高アンモニア血症を呈し，生化学的検査，遺伝子検査によりOTCDと診断された．両親の既往歴等により生体肝移植が困難であったため，新生児期に脳死肝移植待機リストに登録された．生後3か月時の高アンモニア血症を契機に脳死肝移植待機リスト順位が上昇し，生後4か月時に脳死肝移植に至った．本症例は，生体肝移植が困難なOTCD患者において，出生後の早期診断と迅速な脳死肝移植待機リスト登録により，肝移植までの期間を短縮できたことを示す重要な症例である．Ornithine transcarbamylase deficiency (OTCD), a urea cycle disorder, is an X-linked metabolic disease characterized by a congenital deficiency of ornithine transcarbamylase, an enzyme predominantly expressed in the liver. This deficiency leads to the accumulation of toxic ammonia in the body due to impaired conversion of ammonia to urea, resulting in hyperammonemia, which can cause neurological damage and life-threatening conditions. We report a case of a male infant with a maternal family history of OTCD. Early investigations were initiated immediately after birth, as the patient was at high risk of the disease. On day 0, the patient developed hyperammonemia and was diagnosed with OTCD based on biochemical and genetic testing. Due to parental medical conditions, living-donor liver transplantation was not feasible, and the patient was registered on the deceased-donor liver transplantation waiting list. At 3 months of age, a second episode of hyperammonemia occurred, which prioritized his position on the waiting list. At 4 months of age, a brain-dead donor liver transplantation was successfully performed. This case represents an important example of how early postnatal diagnosis and prompt registration on the liver transplantation waiting list can shorten the time to transplantation in OTCD patients for whom living-donor liver transplantation is not feasible.journal articl
Two cases of malignant peritoneum mesothelioma diagnosed by diagnostic laparoscopic surgery
Get PDF悪性中皮腫は主に胸膜、腹膜に発生する予後不良な疾患である。アスベスト曝露との関連性がよく知られているが、明らかではない症例もみられる。そして悪性腹膜中皮腫は稀な疾患であり、臨床的に特異的な所見もないため鑑別に苦慮する。今回我々は癌性腹膜炎を指摘され、診断的腹腔鏡手術により悪性中皮腫と診断できた2例を経験した。症例1: 37歳 発熱、食欲不振を主訴に近医を受診し癌性腹膜炎のため紹介されたが、腹水細胞診、セルブロック法による確定診断は困難であった。症例2: 65歳 咳嗽を主訴に近医を受診し、癌性腹膜炎のため当科を紹介されたが腹水貯留は限定的であり、腹腔穿刺を実施できなかった。2症例とも診断的腹腔鏡手術を行い、症例1は悪性腹膜中皮腫 肉腫型もしくは二相型、症例2は悪性腹膜中皮腫もしくは胸膜中皮腫 上皮型と診断された。癌性腹膜炎の原因疾患は多岐にわたり、その確定診断はしばしば困難なこともある。その中でも稀な悪性腹膜中皮腫の2症例の確定診断に診断的腹腔鏡手術が有用であった。Malignant mesothelioma is a disease with a poor prognosis that occurs primarily in the pleura and peritoneum. Although the association with asbestos exposure is well known, it is unclear in some cases. Malignant peritoneal mesothelioma is a rare condition characterized by the absence of specific findings, making it difficult to distinguish. In this report, we describe two cases of malignant mesothelioma diagnosed through diagnostic laparoscopic surgery in patients with peritonitis carcinomatosa. Case 1: A 37-year-old woman was referred for peritonitis carcinomatosa due to fever and anorexia. However, the diagnosis was difficult to make by cytology and cell block of ascites. Case 2: A 65-year-old woman was referred for peritonitis carcinomatosa with a chief complaint of cough. Nevertheless, the ascites was limited in some area and abdominal paracentesis could not be performed. Consequently, diagnostic laparoscopic surgery was performed in both cases. We found that the diagnosis of Case1 was malignant peritoneal mesothelioma sarcomatoid type or biphasic and that of Case2 was malignant peritoneal mesothelioma or pleural mesothelioma epithelioid type. The diagnosis of peritonitis carcinomatosa is complicated and malignant peritoneal mesothelioma should be considered as a differential diagnosis, and diagnostic laparoscopic surgery proved to be a valuable option in achieving a precise diagnosis.journal articl
A case of tuberculous endometritis discovered during examination of repeated implantation failure
Get PDF性器結核は自覚症状がなく経過するため診断が難しいが, 不妊症の検査を契機に発見されることがある．今回我々は子宮腔内癒着疑いに対し精査の結果, 結核性子宮内膜炎と診断した1例を経験したので報告する．症例は40歳, フィリピン出身, 0妊．不妊を主訴に前医を受診した．3回胚移植するも妊娠成立せず, 反復着床不全の精査目的に子宮鏡検査を行なったところ, 子宮腔内の癒着を疑われ当院に紹介された．子宮内膜組織を病理検査に提出したところ, リンパ球の浸潤, 多核巨細胞と共に類上皮細胞肉芽種, Ziehl-Neelsen染色陽性桿菌を認めた. インターフェロンγ遊離試験陽性, 子宮内膜を用いた抗酸菌培養及び, 結核菌核酸増幅法は陽性であった．現在肺結核に準じた治療を行なっている．結核性子宮内膜炎は肺外結核の中でも稀な疾患であり遭遇する機会は少ないが, 治療には長期間を有するため, 不妊を伴う子宮内膜炎が疑われた場合には鑑別に挙げるべきである．Genital tuberculosis is difficult to diagnose because it progresses asymptomatically, but it is sometimes discovered during treatment of infertility. We report a case of tuberculous endometritis diagnosed after careful examination of suspected intrauterine adhesions. The patient is a 40-year-old woman gravida 0 from the Philippines. She visited previous doctor with the chief complaint of infertility. Despite three times embryo transfers, no pregnancy was achieved, and when a hysteroscopy was performed to investigate repeated implantation failures, adhesions within the uterine cavity were suspected, and the patient was referred to our hospital. The endometrial tissue was examined pathologically. Upon submission, we found infiltration of lymphocytes, epithelioid cell granulomas with multinucleated giant cells, and bacilli with positive Ziehl-Neelsen staining. Moreover, positive interferon γ release test, positive endometrial acid-fast bacterium culture, and positive tuberculosis PCR test were observed. The patient is treated according to pulmonary tuberculosis. Tuberculous endometritis is a rare disease among extrapulmonary tuberculosis, and the chances of encountering are low. If associated endometritis is suspected, it should be included in the differential diagnosis.journal articl
Placental pathology predicts infantile neurodevelopment
Get PDF浜松医科大学Hamamatsu University School of Medicine博士（医学）doctoral医学系研究科thesi
