Management of pregnancy and delivery in women with Kawasaki disease and residual coronary artery lesion with detailed analysis of labor analgesia: combined experience of 13 cases in two institutions.

INTRODUCTION: Approximately half of Kawasaki disease patients are expected to have transitioned to adulthood, and an increasing number of patients with cardiovascular sequelae have gotten pregnant. Management of women with Kawasaki disease who have residual coronary artery disease is poorly established. Thus, we conducted detailed analysis of these cases. METHODS: We reviewed 19 pregnancies in 13 such women in two tertiary perinatal facilities, Saitama Medical Center and National Cardiovascular Center. The medical records were reviewed in all women with Kawasaki disease and coronary artery lesion between 1998 and 2015, with regard to age of diagnosis, types of coronary artery lesion, location, previous treatment, pregnancy course and medical management for coronary lesion, cardiac function, and planned mode of delivery. RESULTS: Fourteen parturients attempted vaginal delivery, and all but one received neuraxial analgesia, providing stable hemodynamics. Four elective and two emergency cesarean deliveries were performed due to obstetric indications, while one woman required cesarean delivery at preterm due to maternal cardiac indication. Among 14 attempted vaginal deliveries, instrumental vaginal delivery was performed in 50%. Cardiac events were noted in four women, all in post-partum period, such as non-sustained ventricular tachycardia or chest discomfort without ECG changes. Antithrombotic medication was exclusively low dose aspirin in 11 of 19 pregnancies (58%), and none received anticoagulation during pregnancy or delivery. CONCLUSION: Our case series support the practice of preferred vaginal delivery, with neuraxial labor analgesia in indicated patients, while highlighting the need for vigilance in the post-partum period.

Outcomes of labor epidural analgesia among women aged over 40: A single-institution retrospective study.

AIM: The purpose of this study was to investigate the effects of labor epidural analgesia (LEA) on maternal and neonatal outcomes among parturients aged 40 years or older. METHODS: We retrospectively reviewed medical records of all laboring, singleton and cephalic deliveries at ≥36 weeks' gestation at the Saitama Medical Center from April 2003 to September 2012. Women aged ≥40 years who received LEA (≥40 with LEA group) were compared with women aged ≥40 years who delivered without LEA (≥40 without LEA group) and women <40 years who received LEA (<40 with LEA group). Extracted outcomes included mode of delivery, oxytocin augmentation, duration of labor, amount of estimated blood loss, umbilical artery pH, Apgar scores, and neonatal intensive care unit admission. RESULTS: This study included 4441 women. There were 74 women in the ≥40 with LEA group, 369 in the ≥40 without LEA group, and 601 in the <40 with LEA group. The maternal outcomes of emergency cesarean delivery rate (9.5%, 12.5%, 9.0%), instrumental delivery rate (33.8%, 10.3%, 28.3%), duration of labor (521 min, 321 min, 565 min), and estimated blood loss (524 g, 351 g, 412 g) were reported for the ≥40 with LEA, ≥40 without LEA, and <40 with LEA groups, respectively. Neonatal outcomes were not different between these groups. LEA use was not associated with emergency cesarean delivery in the multivariable analysis. CONCLUSION: Our study showed that parturients aged ≥40 with LEA can expect similar LEA-associated labor outcomes to younger parturients with LEA.

Success rate and challenges of fetal anesthesia for ultrasound guided fetal intervention by maternal opioid and benzodiazepine administration.

OBJECTIVE: The safe and effective methods of fetal anesthesia/analgesia during ultrasound guided direct fetal procedure are yet to be determined. The authors investigated whether maternal diazepam/fentanyl administration meets this purpose. METHODS: The medical/anesthesia records were retrospectively reviewed in cases between 2001 and 2010 at a tertiary perinatal center. Success rate as well as maternal and fetal complications were analysed. RESULTS: Among the 150 procedures in 118 fetuses, diazepam 10 mg and fentanyl 200 µg sufficiently prevented fetal movement upon the procedure in 56% of the procedures. Supplemental anesthetic agents such as nitrous oxide and propofol were needed in other cases. No serious maternal complication was noted, while fetal cardiac arrest/severe bradycardia was noted in three fetuses, one of which was successfully resuscitated by intracardiac adrenalin injection. CONCLUSIONS: Maternal diiazepam/fentanyl administration offered adequate fetal condition without significant maternal complications. Since these procedures are performed to treat severe fetal conditions, preparation for fetal resuscitation is also important.

Facial morphology and occlusion of a patient with fibrodysplasia ossificans progressiva (FOP): a case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare disease characterized by postnatal heterotopic ossification (HO). When HO affects the masticatory muscles, mouth opening becomes restricted. This paper presents the changes in facial morphology and occlusion of a patient with FOP who was followed from the age of 8 to age 21. At the initial examination, he had a severely protruded maxilla and Angle Class II Division 1 malocclusion. His mouth opening was restricted (5.0 mm). He had a large overjet and this enabled him to clean his teeth and to eat. Orthodontic correction was not planned, and his facial growth was closely followed with attention to his oral hygiene. The maxillary protrusion and a low mandibular plane angle became more prominent as the patient aged. His mandible rotated in a counterclockwise direction. His molars had delayed eruption or were impacted and seven were extracted. His mouth opening increased slightly and his oral hygiene improved to excellent.

Tumor-induced osteomalacia associated with a maxillofacial tumor producing fibroblast growth factor 23: report of a case and review of the literature.

Tumor-induced osteomalacia (TIO) is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia. Recently, it was reported that tumors associated with TIO produce fibroblast growth factor (FGF) 23, identified as the last member of the FGF family and of which excessive action causes several hypophosphatemic diseases whereas deficient FGF23 activity results in hyperphosphatemic tumoral calcinosis. In this case, although it was difficult to locate the associated tumor, an abnormal mass in the left maxilla was detected by imaging. The tumor was removed by partial resection of the left maxillary alveolar region. Thereafter, serum level of FGF23 rapidly decreased, hypophosphatemia improved, and the clinical symptoms greatly improved. Histopathologic diagnosis of the tumor was phosphaturic mesenchymal tumor, mixed connective tissue variant. Immunohistochemical findings confirmed that the removed tumor produced FGF23. These results indicate that development of osteomalacia in this patient was related to the maxillary tumor, which overexpressed FGF23.

Helicity induction and memory of the macromolecular helicity in a polyacetylene bearing a biphenyl pendant.

A novel, cis-transoidal poly-(phenylacetylene) bearing a carboxybiphenyl group as the pendant (poly-1) was prepared by polymerization of (4'-ethoxycarbonyl-4-biphenylyl)acetylene with a rhodium catalyst followed by hydrolysis of the ester groups. Upon complexation with various chiral amines and amino alcohols in dimethyl sulfoxide (DMSO), the polymer exhibited characteristic induced circular dichroism (ICD) in the UV/Vis region due to the predominantly one-handed helix formation of the polymer backbone as well as an excess of a single-handed, axially twisted conformation of the pendant biphenyl group. Poly-1 complexed with (R)-2-amino-1-propanol showed unique time-dependent inversion of the macromolecular helicity. Furthermore, the preferred-handed helical conformation of poly-1 induced by a chiral amine was further "memorized" after the chiral amine was replaced with achiral 2-aminoethanol or n-butylamine in DMSO. In sharp contrast to the previously reported memory in poly((4-carboxyphenyl)acetylene), the present helicity memory of poly-1 was accompanied by memory of the twisted biphenyl chirality in the pendants. Unprecedentedly, the helicity memory of poly-1 with achiral 2-aminoethanol was found to occur simultaneously with inversion of the axial chirality of the biphenyl groups followed by memory of the inverted biphenyl chirality, thus showing a significant change in the CD spectral pattern.