Pregnancies with factor V deficiency: a case report and review of the literature.

CASE REPORT: A 30-year-old Japanese nulliparous woman visited for pregnancy at 33 weeks with a massive ovarian tumor located in the pouch of Douglas. By preoperative screening, her prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged, and her FV activity was significantly decreased to 4.8%. After prophylactic FFP 20 ml/kg was administered and her FV factor was 19.3%, cesarean delivery was performed, and her perioperative course was uneventful. One year later, she underwent a dilatation and evacuation because of a missed abortion, although prophylactic FFP was not administered. During a third pregnancy, after prophylactic FFP 20 ml/kg was administered and FV activity increased to 21.1%, elective cesarean delivery was performed, and her postoperative course was uneventful. CONCLUSION: For surgical therapy or delivery, the goal of therapy is to maintain FV activity above 20%. It is particularly useful to administer prophylactic FFP.

Amniocentesis can be useful during the third trimester of pregnancy for antenatal diagnosis of Pallister-Killian syndrome: a case report.

Pallister-Killian syndrome (PKS) is an extremely rare genetic disease characterized cytogenetically by tetrasomy 12p mosaicism. We recently encountered a case of maternal hydramnios associated with congenital diaphragm hernia according to the prenatal diagnosis. Prenatal diagnosis revealed a non-mosaic 47, XY, i(12)(p10) karyotype at amniocentesis of G-band and M-FISH analysis. We performed chromosomal analysis in both interphase and metaphase cells from a cord blood lymphocyte specimen. Mosaic tetrasomy of chromosome 12p was supported by G-banding or FISH analysis. When fetal observations are performed in detail using 2D/3D US, PKS may be diagnosed. In addition, it is effective to perform amniocentesis during the third trimester of pregnancy.

Vascular endothelial growth factor in monochorionic twins with twin-twin transfusion syndrome.

OBJECTIVE: The purpose of this study was to determine vascular endothelial growth factor (VEGF) concentrations in the donor and the recipient in monochorionic twin pregnancies with twin-twin transfusion syndrome (TTTS) and single pregnancies in order to investigate the involvement of VEGF in the pathophysiology of TTTS. METHODS: Six twin pregnancies in 11 monochorionic twin pregnancies complicated with TTTS and 11 single control pregnancies were compared. Gestational age-matched fetal blood and placental samples were obtained at birth. Serum VEGF concentration in the umbilical vein was measured by an enzyme-linked immunoabsorbant assay. Tissue protein expression of VEGF was determined by using immunohistochemistry. Western blot analysis and scanning densitometry were used to quantify and compare the VEGF expression in the terminal villi. RESULTS: Serum VEGF concentrations in the umbilical vein in both donors and recipients tended to be higher than those in the controls. Immunolocalization of VEGF in terminal villous placenta samples in both donors and recipients was mainly observed in the syncytiotrophoblastic layer and vascular endothelial cells with less intense staining in stromal cells. The expression of VEGF in the donor placenta increased significantly (p=0.006) compared to that in the control placenta, but the expression of VEGF in the recipients tended to be higher than in the controls. CONCLUSION: Intrauterine circulatory imbalance may induce changes in VEGF expression and these alterations may be involved in both donor and recipient in the pathogenesis of TTTS, due to the maintenance of hemodynamic stability between the circulation of the twins.

[Cataract epidemiology survey in the three climatically different areas in Japan--prevalence of cataracts and types of lens opacification].

A cataract epidemiology study of 1,615 subjects from three climatically different places in Japan, village S in Hokkaido, town M in Noto and village Y in Okinawa, was conducted by one study group. Cataract diagnosis and grading of cataracts were objectively done with a photo-documentation system. The percentages of cataracts of over grade I classified by the Japanese Cooperative Cataract Epidemiology Study Group were 46.6%, 64.6% and 38.0% in village S, town M, and village Y, respectively. Those in their 50s, 60s, and 70s in the above places were 24.3%, 51.1% and 71.4% (village S), 38.4%, 65.3% and 84.6% (town M), and 25.2%, 42.9% and 65.4% (village Y), respectively. Cortical cataract was the most common, followed by nuclear and subcapsular types. Although the highest percentage of nuclear cataracts was seen in the subjects of village Y, subcapsular cataract was seen almost equally in the three areas. Lens transparency changes were evaluated by the light scattering intensities in the lens layers through photographed images. Intensity increased with ageing either linearly or exponentially.

Additional novel Cryptosporidium genotypes in ornamental fishes.

Current knowledge on the prevalence and genotypes of Cryptosporidium in fishes is still limited. This study investigated the prevalence of Cryptosporidium species in 171 ornamental fishes, belonging to 33 species, collected from 8 commercial aquariums around Perth, Western Australia. All samples were screened by nested PCR targeting the 18S rRNA locus. A total of 6 positives were identified by PCR at the 18S locus from 4 different species of fishes (red eye tetra, Moenkhausia sanctaefilomenae; gold gourami, Trichogaster trichopterus; neon tetra, Paracheirodon innesi; goldfish, Carassius auratus auratus), giving an overall prevalence of 3.5% (6/171). Four different genotypes were identified, only one of which has been previously reported in fish; piscine genotype 4 in a neon tetra isolate, a rat genotype III-like isolate in a goldfish, a novel genotype in three isolates from red eye (piscine genotype 7) which exhibited a 3.5% genetic distance from piscine genotype 1 and a piscine genotype 6-like from a gold gourami (1% genetic distance). Further biological and genetic characterisation is required to determine the relationship of these genotypes to established species and strains of Cryptosporidium.

Recent refinements of glissonean pedicle approach for liver resection / Шинэ санаа Шинэ нээлт

Background: The glissonean pedicle approach was introduced by Couinaud and Takasaki in the early 1980s. The key of the glissonean pedicle approach is clamping the pedicle first, secondly confirming the territory, and finally dissecting the liver parenchyma. In this presentation, we introduced our recent refinements of glissonean pedicle approach for liver resection. “Approach to the glissonean pedicles at the hepatic hilus” Couinaud described three approaches to the hepatic hilus. 1) Intra-fascial access (Control method): The conventional dissection at the hilus or within the sheath is referred to as intrafascial access However, dissection performed under the hilar plate is dangerous and surgeons have to consider any variations of the hepatic artery and bile ducts. 2) Extra-fascial access (Glissonean pedicle approach): The glissonean pedicle is dissected from the liver parenchyma at the hepatic hilus before dissecting the liver parenchyma. This procedure prevents intrahepatic metastasis of HCC, which spreads along the portal vein and improves the overall survival after surgery. 3) Extra-fascial and transfissural access: If the main portal fissure or the left suprahepatic fissure is opened after dissecting the liver parenchyma, the surgeon can confirm the pedicles that arise from the hilar plate or the umbilical plate. “Operative techniques” 1) Preoperative 3D simulation of the precise anatomy of portal vein, hepatic artery and bile duct at hepatic hilus should be performed. 2) Right glissonean pedicle: The hilar plate is detached from the quadrate lobe. The assistant pulls the liver parenchyma cranially and the operator conversely pulls the hepatoduodenal ligament caudally. Mayo scissors are inserted along the liver parenchyma between the liver parenchyma and glissonean capsule (Fig.1). Then forceps are inserted in the same way and the right main pedicle is taped (Fig.2). The right anterior and posterior glissonean pedicles are taped as well. 3) Left glissonean pedicle: The hilar plate is detached from the liver parenchyma. Then, the Arantius duct is confirmed and the left pedicle is dissected along the left pedicle at the ventral side of the Arantius duct. “Pitfall of glissonean pedicle approach” The right pedicle should be dissected in the liver side as much as possible to prevent the injury of left hepatic duct. If possible, the right pedicle is recommended to be dissected at the level of the second branches separately (Fig.3). The right posterior hepatic duct sometimes branches from the left hepatic duct and the Arantius duct is confirmed and the left pedicle should be dissected along the left pedicle at the ventral side of the Arantius duct because the right posterior hepatic duct branches from the left hepatic duct at the dorsal side of Arantius’ duct. In addition, the intraoperative cholangiogram should be used in the case with the abnormal anatomy of bile duct. Conclusions: Any anatomical hepatectomy can be performed using “glissonean pedicle approach” which allows simple, safe and easy liver resection.

Antenatal diagnosis and treatment of a case of fetal goitrous hypothyroidism associated with high-output cardiac failure.

A case of fetal goitrous hypothyroidism associated with high-output cardiac failure is presented. At 32 weeks of gestation, the antenatal diagnosis of goiter was made based on ultrasound examination, and the fetal thyroid function was examined by amniocentesis and cordocentesis. Color and pulsed Doppler examinations demonstrated a high vascular flow pattern in the goiter and marked elevation of the maximum velocity in the common carotid artery at the level of the neck. It was suspected that arteriovenous shunting through the large goiter resulted in high-output cardiac failure with cardiomegaly and pleural effusion. The fetus was treated by injection of levothyroxine sodium into the amniotic fluid at 33 weeks of gestation and the goiter thereafter decreased in size, with subsequent improvement of the high-output cardiac failure. The maximum velocity in the common carotid artery fell rapidly before the shrinkage of the fetal goiter and in parallel with the fetal level of thyroid stimulating hormone.

Transient hydrops fetalis of the donor fetus in twin-twin transfusion syndrome after therapeutic amnioreduction.

We present a case of twin-twin transfusion syndrome associated with transient hydrops fetalis observed in the donor after therapeutic amnioreduction at 22 weeks of gestation. After the amnioreduction, the bladder of the donor could be visualized and the donor subsequently began to make amniotic fluid, with spontaneous regression of hydrops fetalis. It is suspected that after therapeutic amnioreduction, intrauterine hemodynamic changes occurred and the donor developed transient hydrops fetalis due to volume overload.