Background: We investigated the incidence and clinical characteristics of eyes showing retinal detachment (RD) after anti-vascular endothelial growth factor (VEGF) for retinopathy of prematurity (ROP). Methods: A retrospective chart review of 76 consecutive eyes of 45 patients (18 girls and 27 boys) with stage 3 ROP who received anti-VEGF therapy between January 2012 and August 2020 with a minimum follow-up of 6 months was conducted. Eyes were divided into two groups: the vitrectomy (V) group that required vitrectomy for RD after anti-VEGF therapy and the non-vitrectomy (non-V) group that did not require vitrectomy. Data were collected from patient charts, including sex, postmenstrual age (PMA) at birth, birth weight, PMA at anti-VEGF therapy, comorbidities, reactivation, examination interval, and subsequent vitrectomies. Results: The median PMA at birth was 24.7 (range, 22.1-29.3) weeks. Twenty-seven eyes (35.1%) exhibited ROP reactivation at 6.4 ± 3.1 weeks after anti-VEGF therapy. The V group included six eyes of five patients, all of whom exhibited reactivation and developed RD 10.1 ± 6.5 weeks after anti-VEGF therapy. The types of RD were conventional (classic) in two eyes and circumferential (unique to RD after anti-VEGF) in four eyes. Three eyes required repeated vitrectomy. All eyes, except one eye in the V group, achieved retinal attachment at the last examination. The non-V group included 70 eyes of 40 patients, of which 21 exhibited reactivation and were treated successfully with laser (17 eyes) or second anti-VEGF (4 eyes). The proportion of eyes with plus disease was significantly higher in the V group (50.0%) than in the non-V group (10.0%) (P = 0.035). V group included 3 of 22 eyes (13.6%) in which the interval between the last examination and the diagnosis of reactivation was <1 week and 3 of 5 eyes (60.0%) in which the interval was more than 1 week (P = 0.024). The two groups showed no significant differences in the other factors. Conclusion: Approximately 8% of eyes developed RD about 10 weeks after anti-VEGF therapy for ROP. Eyes with history of plus disease should be carefully monitored at appropriate intervals after anti-VEGF therapy for ROP.

PURPOSE: To investigate the efficacy and risk factors of intravitreal anti-vascular endothelial growth factor injection (anti-VEGF therapy) for retinopathy of prematurity (ROP). METHODS: We retrospectively reviewed 80 consecutive eyes of 43 patients with type 1 ROP or worse who received anti-VEGF therapy during January 2012-February 2018. Patients were divided into those who were injected with 0.25 mg bevacizumab (IVB group, 37 eyes) and 0.25 mg ranibizumab (IVR group, 43 eyes). Serum VEGF concentrations of 18 patients were measured before and after IVR. RESULTS: Anti-VEGF therapy reduced ROP activity in all eyes; however, 14 eyes (17.5%) exhibited reactivation. The reactivation rates of the IVB and IVR groups were 13.5% and 20.9%, respectively (p = 0.556). Multivariate logistic regression analysis showed that postmenstrual age (PMA) ≤35 weeks at anti-VEGF therapy (p = 0.014) and aggressive posterior ROP (p = 0.044) were significantly associated with reactivation. Serum VEGF was significantly suppressed at days 1 (p < 0.001) and 7 (p = 0.012) after IVR and returned to preinjection level by day 14 (p = 0.210). CONCLUSIONS: Both IVR and IVB seemed effective in reducing ROP activity. Reactivation after anti-VEGF therapy may be associated with younger PMA at anti-VEGF therapy and aggressive posterior ROP.

[Abstract] A female infant born at 30 weeks of gestation and weighing 1,276g developed a suspected intracranial cavernous hemangioma invading the sella turcica at the age of one month. She received high-dose steroid treatment with 5mg/ kg/ d of prednisolone, resulting in a reduction in the size of the hemangioma, and follow-up care began after discharge. At the age of six, it was noted that the girl's growth in height was insufficient. Head magnetic resonance imaging (MRI) showed pituitary atrophy, and the results of a loading test led to a diagnosis of growth hormone deficiency resulting in a short stature. However, the administration of growth hormone brought her height up to the average by the age of 11.The patient's intracranial lesion during the newborn period, which was suspected to be a cavernous hemangioma, may have contributed to her pituitary atrophy. The findings of this case suggest that infants who develop a space-occupying lesion in the sella turcica must be kept under long-term observation in view of the possible occurrence of a defective pituitary function.

Background Patients with minimal change nephrotic syndrome (MCNS) often also have allergic diseases. Abnormalities of Th2-derived cytokines and T-cell functions contribute to development of these diseases. On the other hand, imbalances between reactive oxygen species (ROS) and antioxidants have been implicated in MCNS and progression of atopic dermatitis. ROS, produced mainly within mitochondria, subject cells to oxidative stress, while prohibitin 2 protects mitochondria by increasing tolerance to ROS. Additionally, podocin, a member of the slit diaphragm protein complex, contains PHB-like domain that serves as a signaling platform regulating podocyte function through associated transmembrane proteins.Patients and method Then, we performed exome sequencing analysis in five patients with frequently relapsing their MCNS associated with allergic disease and serum IgE concentrations of 2000 IU/L or higher.Results We detected a heterozygous prohibitin 2 polymorphism, c. 873-3_ 873-2 delCA (rs111523336), in 1 patient. This mutation in exon 9 caused frameshifts in regions connected to splicing sites, where they could disrupt transcription of prohibitin 2. Frequency of this polymorphism in exon 9 is 7.3% among Japanese. Increase in peripheral blood ROS even MCNS remission state suggests the heterozygous prohibitin 2 variant may contribute to give more susceptibility towards the recurrence of MCNS as well as atopic skin disease. This increase may have progression of atopic dermatitis, which sometimes heralded.Conclusion The prohibitin-2 polymorphism may reduce ROS tolerance in glomerular epithelium and led to high local exposure to ROS, increasing permeability of the glomerular basement membrane to result in proteinuria. Imbalance between ROS and antioxidants together with failure of signal transduction in the glomerular slit membrane caused by prohibitin 2 abnormality could have contributed to nephrotic syndrome in our patients. Prohibitin 2 analysis is needed in additional MCNS patients with concomitant allergic disease.

生後10時間男児。呼吸困難を主訴とした。胎児期や周産期に特別な異常は指摘されておらず、在胎40週1日、体重3310g、自然分娩で出生し、出生直後より咽頭内腔を占拠する隆起性病変を認めたが、呼吸状態に異常はなかった。しかし、生後10時間より喘鳴が出現し、陥没呼吸の増悪とSpO2低下を認めたため、経口気管内挿管を行い、MRI T2強調画像で咽頭上部に内部不均一、一部高信号を呈する境界明瞭な腫瘍を認めた。生後9日に術野と気道の確保を目的とした気管切開術を行い、生後20日に腫瘍摘出術を実施したところ、腫瘍径は2.5cm大で一期的切除が可能であり、出血量も少量であった。病理所見より成熟型の咽頭奇形腫(病期分類I期)と診断し、術後は呼吸障害や哺乳障害はなく、術後1年時点で再発は認めていない。

Development of both Crohn disease and Guillain-Barre syndrome likely involves autoimmunity associated with excessive inflammatory cytokines. We treated a girl who developed Guillain-Barre syndrome during the course of Crohn disease. Although high-dose -globulin therapy administered initially for Guillain-Barre syndrome was ineffective, plasmapheresis ameliorated her acute neuropathic symptoms. Crohn disease was managed with Salazopyrin administration and enteral feeding. Chronic inflammation of the intestinal mucosa caused by Crohn disease can allow presentation of microbial intestinal antigens normally hidden from the immune system. Such presentation could incite an extraintestinal immune response on the basis of molecular mimicry, leading to activation of systemic autoimmunity against the nervous system. Accordingly, concurrence of Guillain-Barre syndrome and Crohn disease in our patient appeared to result from shared autoimmune mechanisms and systemic and local increases in cytokine concentrations. The patient also developed erythema nodosum and gall stones, relatively common complications of Crohn disease. However, Guillain-Barre syndrome is rare.

Background: Patients with X-linked agammaglobulinemia (XLA) develop immune-complex induced diseases such as nephropathy only rarely, presumably because their immunoglobulin (Ig) G concentration is low. We encountered a patient with XLA who developed tubulointerstitial nephritis during treatment with intravenous immunoglobulin (IVIG).Case presentation: A 20-year-old man was diagnosed with XLA 3 months after birth and subsequently received periodic gamma-globulin replacement therapy. Renal dysfunction developed at 19 years of age in association with high urinary gamma 2-microglobulin (MG) concentrations. A renal biopsy specimen showed dense CD3-positive lymphocytic infiltration in the tubulointerstitium and tubular atrophy, while no IgG4-bearing cell infiltration was found. Fibrosclerosis and crescent formation were evident in some glomeruli. Fluorescent antibody staining demonstrated deposition of IgG and complement component C3 in tubular basement membranes. After pulse steroid therapy was initiated, urinary beta 2-MG and serum creatinine concentrations improved.Conclusion: Neither drug reactions nor collagen disease were likely causes of tubular interstitial disorder in this patient. Although BK virus was ruled out, IgG in the gamma-globulin preparation might have reacted with a pathogen present in the patient to form low-molecular-weight immune complexes that were deposited in the tubular basement membrane.

Alport syndrome (AS) is a renal disorder caused by a genetic abnormality of type IV collagen alpha 3 and alpha 4, or alpha 5 genes and shows a poor prognosis. Since the defect of type IV collagen synthesis disturbs the maturation process of the glomerular capillary loop, residual immature glomeruli persist after birth. The therapeutic efficacy of cyclosporin A (CyA) for AS patients seems to be controversial. We recently noted that renal specimens obtained from a child with AS who was treated with CyA and then developed CyA nephropathy included an increased number of undifferentiated embryonic-type glomeruli. We analyzed renal histologic and immunohistologic findings in children with AS who did (n = 3) or did not (n = 2) develop CyA-induced nephropathy despite appropriately low serum CyA concentrations (< 100 ng/mL) being maintained over a period of 2 years. To discriminate embryonic-type from mature glomeruli, staining for type IV collagen alpha 1, laminin beta 1, and laminin beta 2 accompanied by light microscopic observation were employed. Staining patterns were used to semiquantitatively assess glomerular immaturity (glomerular immaturity index, or GII). In initial biopsy specimens, residual embryonic-type glomeruli were observed in each patient. Patients with early-onset CyA nephropathy had a high GII (median value 2.91 vs 1.23 +/- A 0.62 normal kidney tissues). In the follow-up biopsy after CyA treatment, surviving embryonic-type, collapsing embryonic-type, and sclerotic glomeruli that had failed to differentiate were observed. Taken together, the number of these glomeruli essentially equaled the total number of embryonic-type glomeruli in specimens obtained before CyA treatment. Our findings indicate a need for caution in CyA therapy for patients with AS, even for a relatively short course of administration, because some patients may have an unexpected number of embryonic-type glomeruli that predispose to CyA nephropathy.

[Abstract] We reported the case of a newborn manifesting heart failure caused by a chorioangioma. A male infant was admitted to Kinki UniversityHospital Neonatal Intensive Care Unit (NICU) 4 hours after birth because of cyanosis. He had respiratory distress, and ultrasound echocardiographyshowed serious mitral regurgitation. An improvement of the mitral regurgitation 27 hours after birth resulted from olprinone hydrochloride administration, and mechanical ventilation was discontinued 2 days later. Pathologic examination of the placenta revealed a chorioangioma ; a 290g giant angioma adhering to a 250 g placenta, it therefore seemed likely that the cause of the respiratory distress was hemorrhagic pulmonary edema associated with theheart failure resulting from the blood-flow short circuit in the chorioangioma. Thus, in order to diagnose the cause of heart failure in a newborninfant, doctors should be aware of chorioangioma, and pathologic examination of the placenta could be important when an unusual course is observed in newborns.

腹腔内出血により死亡し、病理解剖にて肝海綿状血管腫の破裂と診断された低出生体重児の1例を経験した。症例は在胎31週4日、体重1500gで出生した品胎第3子の男児で、日齢4に腹腔内出血による循環血漿量減少性ショックで発症し、集中治療にも関わらず日齢14に死亡した。病理解剖にて肝臓に裂傷と暗赤色の腫瘍を認め、腫瘍は海綿状血管腫と診断された。腹腔内出血の原因は、肝左葉海綿状血管腫の破裂であった。新生児、特に低出生体重児では、一旦出血して全身状態が不良となれば治療方法は限定され救命は困難になりやすいため、早期発見・早期治療が極めて重要である。

Introduction: We analyzed renal histologic and immunohistologic findings in children with nephrotic syndrome (NS) who did (n = 5) or did not (n = 17) develop cyclosporine A (CyA) nephropathy despite appropriately low serum CyA concentrations being maintained over 2 years. Methods: To discriminate embryonic-type from mature glomeruli, we performed staining for type IV collagen alpha 1, laminin beta 1 and laminin beta 2. Staining patterns were used to semiquantitatively assess glomerular immaturity (glomerular immaturity index, or GII). Results: In follow-up biopsy specimens, residual embryonic-type, collapsed embryonic-type and sclerotic glomeruli that had failed to differentiate were observed. Patients with early-onset CyA nephropathy had a high GII. In patients with a high GII, arteriopathy developed early in CyA treatment. Arteriopathy was observed mostly near embryonic-type glomeruli. Taken together, these glomeruli (surviving embryonic-type, collapsing embryonic-type, and sclerotic glomeruli) essentially equaled the total number of embryonic-type glomeruli in specimens obtained before CyA treatment. Conclusion: Our findings indicate a need for caution in CyA therapy for patients with NS, even for a relatively short course of administration, because some patients may have embryonic-type glomeruli or immature arterioles that predispose them to CyA nephropathy.

（目的）出生体重1500g未満の新生児における未熟児網膜症の臨床経過について検討すること．
（対象および方法）対象は1999年1月～2010年4月までに近畿大学眼科で診療した出生体重1500g未満の新生児142例（男児65例、女児77例）であった．出生体重、在胎週数、未熟児網膜症の発症と進行，および治療内容について検討した．
（結果）平均体重は1109±250g，平均在胎週数は29.3±2.4週数であった．未熟児網膜症に対するレーザー網膜光凝固術は（以下；レーザー治療）は19.7%に対して施行した．レーザー治療は在胎31週未満の新生児の一部に必要で，修正30週を超えて治療が必要となった．冷凍凝固術，輪状締結術，硝子体手術，抗VEGF療法などの治療が必要であった症例は4.9%であった．
（結論）未熟児網膜症に対する治療は，在胎31週未満の新生児に頻度が高かった．

Focal segmental glomerular sclerosis (FSGS) is a leading cause of the nephrotic syndrome and characterized by the sclerosing lesions that affect one or more segments of some glomeruli. We encountered a female patient with a partial deletion of chromosome 6p, who presented proteinuria at age 3 years. Detailed chromosomal analysis disclosed an interstitial deletion of 6p: del(6)(p22.1p22.3). No abnormality such as hydronephrosis or renal agenesis was disclosed by imaging, but FSGS was present in a renal biopsy specimen. The patient is currently 11 years old and shows mental retardation with mild deterioration in the renal function. To address the defective genes in the present patient, we carried out comparative genomic hybridization (CGH), showing that E2F3 on chromosome 6p is absent in this patient. E2F3, a member of the E2F family transcription factors, inhibits expression of vascular endothelial growth factor (VEGF) and induces apoptosis during vascular development. The deletion of E2F3 was also detected by employing a PCR method, suggesting that glomerular architecture had been compromised in this patient. Serum VEGF concentrations were elevated to 177 +/- 21.4 pg/mL (upper limit of 33.3 pg/mL), when she was 6 years old, associated with the enhanced expression of VEGF in glomeruli. These findings suggest that the dysregulation of VEGF synthesis caused by the deletion of E2F3 may be associated with development of FSGS. In conclusion, among patients with idiopathic FSGS, an abnormality of E2F3 may exist on chromosome 6p. Therefore, one might consider chromosomal analyses in children with FSGS who have mental retardation.

We evaluated and treated a girl with Henoch-Schönlein purpura (HSP), who initially developed redness, swelling, and pain in all 4 limbs accompanied by Raynaud syndrome and then had convulsions and disturbance of consciousness. HSP was diagnosed based on later findings of purpura in both legs and a decrease in factor XIII activity not accompanied by thrombocytopenia. She was normotensive. A skin biopsy specimen showed small-vessel vasculitis accompanied by immunoglobulin A deposition. The cause of erythema and limb pain, convulsions, and disturbed consciousness presumably was vasculitis. The possibility of HSP should be considered in patients with limb pain despite initial absence of purpura and in patients with central nervous system symptoms such as convulsions.

日本小児科学会による病院小児科・医師現状調査は多くの小規模な病院小児科で不十分な体制のまま不本意な医療を小児科医が個人の生活や健康を犠牲ににしておこなっている現状を示した。今後、質の高い小児医療を継続的に提供していくために病院小児科医の労働環境改善が必要であると考えられた。

病院小児科の時間外診療と医師の時間外労働の現状についてのアンケート調査結果を解析した。多数の小規模な病院小児科がそれぞれに当直、時間外診療を行っている現状、若手小児科医師の月の時間外労働時間が平均130時間を越えていることなどが明らかになった。病院小児科の統合がそれらの問題点の解決策のひとつとなり得ると考えられた。

大阪の病院小児科にアンケートを送付して得られた回答を解析した結果、病院小児科のworkforceを確保し質の高い小児医療の提供を続けるためには、病院小児科の統合が必要と思われた。

Prader-Willi syndrome に眼皮膚白子症を合併した１例を報告した。症例は０歳の女児で、Fish法による染色体検査によりPrader-Willi syndromeと診断された。皮膚は白く、虹彩の一部に色素の脱失があり、眼底は白子眼底を呈していた。

Background : Locally produced growth factors may mediate cell differentiation during regeneration following acute renal injury. Thus, we examined the participation of two different forms of heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF), a new member of EGF family of growth factor, in regeneration of renal tubules after acute injury. Methods : To evaluate preventive and therapeutic effects of soluble (s) HB-EGF, recombinant (r) human (h) s-HB-EGF (250 μg/kg) was administered to BALB/c mice prior to or after onset of cisplatin-induced acute tubular renal failure (ATRF). Results : Administration after onset of ATRF suppressed increases in blood urea nitrogen (BUN) and serum creatinine (Cr). Pretreatment with rh s-HB-EGF showed a similar effect. Histologically evident tubular injuries, characterized by epithelial cell necrosis and detachment of cells from the tubular basement membrane were much milder in rh s-HB-EGF-treated mice than in untreated controls. Additionally, rh s-HB-EGF stimulated DNA synthesis in damaged renal epithelial cells according to 5-bromo-2'-deoxyuridine (BrdUrd) incorporation. To determine involvement of a precursor form of HB-EGF, proHB-EGF, in recovery from ATRF, we constructed a line of NRK 52E cells stably expressing proHB-EGF (NRK^) and cultured cells in three-dimensional type I collagen gels. NRK^ formed tubule-like structures mimicking renal tubules, unlike NRK 52E cells transfected with empty vector alone (NRK^), which formed only round colonies. Conclusions : HB-EGF may function importantly in resistance to and recovery from ATRF, and may have therapeutic potential.

子どものあざについて解説した。

周産期専門医を志望する医師を対象としたテキストで、未熟児網膜症について解説した。

周産期の疾患の項において、染色体異常症について解説した。

新生児呼の診療における酸素の投与とその効果療につき、患者家族用と医療者用の別に解説した。

新生児一過性多呼吸の病態と診断、治療につき、患者家族用と医療者用の別に解説した。

システマティックレビューにもとづいて新生児慢性肺疾患の診療におけるパルスオキシメータの活用法について述べた。

新生児慢性肺疾患重症化に関与が疑われている silent aspiration の診断方法としての気管吸引液デンプンテストについて解説した。

低出生体重児に対する酸素療法の実施方法を具体的に解説した。

低出生体重児に見られやすい疾患について解説した。

軽症から中等症の胎便吸引症候群の新生児に対する酸素療法と管理上の注意点について解説した。

【目的】近年、海外では抗 VEGF 治療が未熟児網膜症の第一選択であるという論調がみられる。当院では2011 年 11 月より抗 VEGF 療法の治験が開始された。今回、我々は、抗 VEGF 治療が新生児の全身状態に及ぼす影響について網膜光凝固と比較し検討したので報告する。 【対象・方法】網膜光凝固を受けた 5 例と抗 VEGF 治療を受けた 5 例の術中鎮静方法、術中術後の呼吸管理方法、術後酸素投与日数、術後炎症反応、手術時間を比較した。 【結果】網膜光凝固群では、在胎週数の平均は、25週（23～26 週)、出生体重は819g（680～949g）、全例気管挿管、人工呼吸管理を施行し、術中鎮静はミダゾラム、ペンタゾシンで行った。術後1日で抜管し、絶食期間は1日であった。また、抜管後は、2 例でCPAPを施行し、その他 3 例では酸素投与は施行していない。手術時間は平均97分、術後1日目の炎症所見の上昇は認めていない。一方、抗VEGF治療群では、在胎週数の平均は、27週（23～29週）、出生体重は

[はじめに]FIPとは①組織学的および臨床上NECを認めない限局性消化管穿孔②発症後早期に血液検査で炎症所見認めず、肉眼的および組織学的に穿孔部周辺に炎症細胞浸潤を認めない。と定義されている。今回、我々は3回FIPを繰り返した症例を経験した。文献的考察とともに報告する。 [症例]在胎24週2日、体重579g、Apgar 2 / 5点であった。出生後、RDSのため、サーファクタント投与後HFOにて人工呼吸器管理となった。日齢3に母乳開始するも通過は不良であった。PDAに対して日齢9 にインドメタシン １ クール施行するも閉鎖せず、日齢 12 に外科的に閉鎖術を施行した。術後経過は良好であり、排便も認めたため、日齢 20 より母乳による経腸栄養を再開した。しかし、日齢22にレントゲン上 free airを認め、消化管穿孔を疑い腹腔内ドレナージを施行した。その後、炎症所見は改善したが、日齢 27 に炎症所見の再上昇、レントゲン上腸管ガス像の拡張を認めた。再穿孔を疑い、腹腔内洗浄を行っ

小児科医の労働環境についてのワークショプで、EU加盟国における医師の労働規制の実施状況について報告した。

著明な肺動脈の拡張を指摘された動脈管早期収縮例の胎児期および出生後の心エコー所見を報告した．症例は在胎38週6日の胎児．妊婦健診の胎児心エコーで著明な肺動脈の拡張を指摘され緊急入院．肺動脈径は，主幹部13.4 mm，左右肺動脈径は5.9/6.0 mm．三尖弁弁輪径は12.2 mmで右室壁肥厚を認めた．三尖弁逆流は中等度，右室右房収縮期圧較差は67 mmHg，動脈管狭窄遠位部で収縮期2.4 m/sec，拡張末期1.2 m/secの血流加速を認め,動脈管早期収縮と診断．一次中隔は左房側へ偏位し，卵円孔の左房側での血流速は0.8 m/secであった．静脈管血流で逆流(-)，臍帯動脈のRIは0.46．妊娠中のNSAIDの使用(-)．CTGで，variabilityに乏しく，accelerationが消失したため，同日緊急帝王切開で出生．Apgar Scoreは，8/9点，出生時体重は2956g．出生後狭小な動脈管での左右短絡を認め，心房間では左右短絡を認めた．生後2日に肺動脈弁輪径は6.4 mm,左右肺動脈径は4.6/4.4 mmとなった．補液および酸素投与を施行．生後8日に退院．著

マルチディクターCT（以下、MDCT）は近年、成人領域の大動脈病変や冠動脈疾患の診断に繁用され、その有用性については論を持たない。今回我々はNICUに収容した新生児の中で心臓超音波検査によって大血管病変や肺静脈病変の存在が診断された（あるいは疑われた）14例に対しMDCTを撮影し、その有用性と問題点を検討した。 【対象】大血管病変あるいは肺静脈病変の存在が診断された（あるいは疑われた）NICU収容新生児14例で撮影回数は合計20回。初回撮影時の日齢は1～38、体重は1.3Kg～3.5Kgであった。疾患別内訳は総肺静脈還流異常が3例、大動脈離断が2例、肺動脈閉鎖あるいは狭窄が4例、心室中隔欠損、左心低形成、僧帽弁逆流、ファロー４徴、総動脈幹がそれぞれ1例。 【方法】①使用機器はGE社製right speed VCT、②鎮静薬としてトリクロホスナトリウム80mg/Kgを服用させた、③24G留置針で静脈を確保し、非イオン化造影剤を2倍に希釈して1.5ml/Kgを0.6ml/秒の速さで注入した。対象疾

NICUにおける呼吸モニターの方法として、カプノメーターの利用、パルスオキシメーターの利用方法について解説した。また、パルスオキシメーターの頭部センサーの有用性について報告した。

CTLA-4遺伝子の異常を認めた日本人SLEの2家系について報告した。CTLA-4遺伝子異常に基づくT-cell activationの制御の異常により、家族性SLEの発症に関与している可能性があると考えられた。

妊婦の抗HLA抗体、抗HPA抗体、抗赤血球抗体の検出頻度について検討した。新生児血小板減少症(NAIT)の症例を呈示し、出生前の抗体スクリーニングの有用性を述べた。

EEC SYNDROME と思われる母体から出生した児について、その診断、経過、治療につき症例報告した。

［はじめに］重症肺動脈弁狭窄では，右室サイズがbiventricular repairの可否を決定する．また，三尖弁弁輪径(TVD)は，右室サイズを反映する．出生後にTVDが増大したため，治療方針が，胎児心エコ－後・出生直後の説明と異なることになった例を経験した．胎児期からの心エコ－所見の変化を報告する． ［症例］妊娠38週2日，IUGRで，AFI4cmと減少，心胸郭比増大を認め入院．胎児心エコ－では，肺動脈狭窄（右室肺動脈圧較差70mmHg），右室が小さく，三尖弁は低形成で可動性に乏しかった．漏斗部狭窄の所見と考えた．右室サイズの増大は見込めないと判断し，将来の右室バイパス術を説明した．11日後分娩誘導時にfetal distressを認め，帝王切開で出生した（体重2,040g）．心エコ－で重症肺動脈弁狭窄・三尖弁低形成・心房中隔欠損・動脈管開存と診断した．直ちにリポPGE1を使用したが，SpO2が低下し，生後2日にPGE1-CDに変更した．体重増加を待って，肺動脈弁切開兼短絡手術の方針とした．し