Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure

A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. Brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.     

Experimental study of the effects of deletion variant of hepatocyte growth factor on hepatic ischaemia-reperfusion injury

BACKGROUND: Hepatic ischaemia-reperfusion (IR) injury is still a serious complication following liver surgery. The effect of the deletion variant of hepatocyte growth factor (dHGF) on hepatic IR injury was examined in rats. METHODS: Male Wistar rats were divided into two groups after 90 min of partial liver ischaemia: the dHGF group which was given dHGF 0.5 mg/kg intravenously immediately after reperfusion, followed by 0.5 mg/kg every 12 h, and the control group, which received vehicle buffer only. Serum chemistry, histopathological findings and liver weights were compared between the groups. RESULTS: In the dHGF group, the increase in serum alanine transaminase and hyaluronic acid levels was significantly reduced, and the serum albumin level increased after reperfusion. The extent of hepatic necrosis 24 h after reperfusion was decreased in the dHGF group. Moreover, the proportion of terminal deoxynucleotidyl transferase-mediated 2'-deoxyuridine 5'-triphosphate nick end labelling-positive hepatocytes 6 h after reperfusion was reduced in the dHGF group. The non-ischaemic-, ischaemic- and whole-liver weight : body-weight ratio significantly increased in the dHGF group after reperfusion. The proportion of proliferating cell nuclear antigen-positive hepatocytes in the dHGF group markedly increased after 6 h after reperfusion in the non-ischaemic lobes, while in the ischaemic lobes it increased 24 h after reperfusion. CONCLUSION: These data suggest that dHGF not only improves recovery from IR injury, but also accelerates recovery from these injuries. dHGF may be an effective pharmacological agent for prevention and treatment of hepatic IR injury.     

Synthesis and photophysical properties of photostable 1,8-naphthalimide dyes incorporating benzotriazole-based UV absorbers

We developed a series of blue-emitting 1,8-naphthalimide dyes covalently attached to 2-(2-hydroxyphenyl)-2H-benzotriazoles that retard photodegradation of the fluorophore. The dyes displayed weaker fluorescence emissions than the parent 1.8-naphthalimide. Quantum chemical calculations suggested that the decreased fluorescence was caused by the nonradiative deactivation promoted through the excited state intramolecular proton transfer (ESIPT) in benzotriazole components. The dyes'' phosphorescences in a degassed solution at 77 K were more efficient than that of the parent 1.8-naphthalimide, indicating a possible deactivation pathway through intersystem crossing. PMMA films doped with these dyes showed higher resistance against photoaging than the film doped with an equimolar mixture of constituent 1.8-naphthalimide and the benzotriazole derivatives. Thus, the covalently linked benzotriazole units slow fluorophore degradation not only by preferential absorption of harmful UV light, which is found in the film with a simple mixture of two components, but also by the nonradiative deactivation involved in benzotriazole units.

Highly photostable blue fluorescence dyes were developed by hybridization of 1,8-naphthalimides with benzotriazole-based UV absorbers enabling a non-radiative energy dissipation process of excited-state intramolecular proton transfer (ESIPT).     

Late recurrence after resection of mass-forming intrahepatic cholangiocarcinoma: report of a case

The outcome after surgical resection for intrahepatic cholangiocarcinoma has not been satisfactorily evaluated due to its malignant behavior. Surgical resection, however, has the potential to improve the prognosis and may allow surgeons to experience rare cases with long survival. This report presents the case of a patient who developed recurrence 9?years after resection of intrahepatic cholangiocarcinoma. A 76-year-old female was diagnosed to have intrahepatic cholangiocarcinoma and underwent an extended right posterior subsegmentectomy. The gross appearance showed a mass-forming type tumor. The histopathological examination revealed well to moderately differentiated adenocarcinoma associated with portal vein invasion. Subcutaneous metastasis in the head as the first sign of relapse was diagnosed 9?years after hepatectomy. The histopathological findings of the subcutaneous tumor were similar to those of the intrahepatic cholangiocarcinoma, thus suggesting metastasis from intrahepatic cholangiocarcinoma. Positron emission tomography with 2-[fluorine-18]-fluoro-2-deoxy-d-glucose was useful for detecting multiple metastases. Long-term follow-up for more than 5?years is recommended because the present case shows that late recurrence of intrahepatic cholangiocarcinoma occurs even 5?years after resection.     

Mechanical analysis of the lumbar vertebrae in a three-dimensional finite element method model in which intradiscal pressure in the nucleus pulposus was used to establish the model

We established a three-dimensional finite element method (FEM) model of the 4th and 5th vertebrae, using computed tomography (CT) images (2-mm slice thickness) of a healthy 29-year-old man. Because of the lack of specific data regarding the material characteristics of the nucleus pulposus of intervertebral discs, we used intradiscal pressure in the nucleus pulposus to establish the model. We referred to data from Nachemson and from Sato et al. regarding intradiscal pressure and to the methods of Shirazi-Adl for data for other material characteristics (see text for these references). The mid-position model bears a load of 294 N in the vertical direction, while the models of the flexed and extended positions bear loads of 15 N-m. In addition, a degenerative disc model without intradiscal pressure was created for the standing model. The use of these models allowed the investigation of von Mises stress on the vertebral endplates and the annulus fibrosus. We also examined von Mises stress on the facet joint in normal and degenerative disc models. There was increased von Mises stress on the vertebral endplate in the anterior, center portions. von Mises stress on the annulus fibrosus increased in the posterior portion, the entrance to the neural foramen, and the exit of the neural foramen. von Mises stress was greater during flexion in the posterior portion; in particular, increasing to about 1.6 times the level seen with other postures. No changes were observed in von Mises stress on the vertebral endplates or annulus fibrosus in the degenerative disc model, but von Mises stress on the facet joints was about 2.5 times that seen in the normal disc model. Received: June 28, 2001 / Accepted: October 27, 2001     

Pathogenic implications of remnant vitelline structures in gastroschisis

Purpose

The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis.

Methods

Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed.

Results

Paraumbilical bands, possible remnants of vitelline structures, were observed in 4 cases (9.8%). All 4 bands were attached to the skin edge of the abdominal defect without incorporation into the umbilical cord. The band ended at the mesentery in 3 cases and at the antimesenteric site of the ileum in the remaining case. Histologic findings showed fibrous tissues in all cases. One was possibly associated with the development of colonic atresia. Another was noticed after silo reduction when herniated bowels became strangulated by the band. The other 2 cases were uncomplicated.

Conclusions

Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally.     

Choroid plexus ependymal cells host neural progenitor cells in the rat

We previously demonstrated that choroid plexus epithelial (modified ependymal) cells (CPECs) differentiated into astrocytes after grafting into the spinal cord. In the present study, we examined whether CPECs from rats at postnatal 1 day (P1), 7 day (P7), and 8 weeks (P8W) can function as neural progenitor cells that give rise to neurons and glial cells. Cell spheres were produced in cultures of whole tissue of the choroid plexus from the fourth ventricle of rats at each postnatal period. beta-tubulin class III (Tuj-1), glial fibrillary acid protein (GFAP)-, and O4-positive cells differentiated from cell spheres in the differentiation medium. We produced a monoclonal antibody 3E6 specifically labeling microvilli of CPECs. Using this monoclonal antibody, CPECs were isolated from the choroid plexus of P8W rats by cell sorter (FACS). Immunocytochemistry confirmed that there was no contamination from fibroblasts, endothelial cells, macrophages, or Schwann cells in the FACS-isolated 3E6-labeled cells. Cell spheres formed in the cultures of these 3E6-labeled CPECs. After expansion, these cell spheres gave rise to Tuj-1- (5%), GFAP- (45%), and O4-positive cells (0.16%). The remaining cells (45%) were unlabeled neural or glial markers. Some CPECs of the P8W rat were immunohistochemically stained with lineage-associated markers of Musashi-1 and epidermal growth factor-receptor (EGF-R). In addition, infusion of EGF or fibroblast growth factor-2 (FGF2) into the ventricle increased the number of bromodeoxyuridine (BrdU)-positive cells among CPECs from 0.03% (untreated) to 1.14% (38-fold, EGF) and 1.03% (35-fold, FGF2), respectively. These findings indicate that neural progenitor cells exist among CPECs in the rat.     

Living donor liver transplantation for end-stage liver disease with severe hepatopulmonary syndrome: report of a case

Hepatopulmonary syndrome (HPS) is a serious complication of terminal liver disease, which manifests as severe hypoxia without any pulmonary anatomic or functional causes. The precise indications for liver transplantation in patients with severe HPS also remain unclear. A 49-year old woman was referred to our department for investigation and management of liver cirrhosis with severe hypoxia (PaO₂, 38 mmHg). A pulmonary perfusion scintigram showed an intrapulmonary shunt ratio of 40%, confirming a diagnosis of severe HPS. She underwent living donor liver transplantation using a right lobe graft donated by her 27-yearold daughter. Her post-transplant graft function was excellent, and her oxygenation recovered slowly but steadily. She was discharged from hospital on posttransplant day 39 with an SpO₂ of 94% under 3 l/min of O₂, delivered nasally. Despite the severity of the HPS, her postoperative recovery was smooth after the liver transplant.     

Long-term culture of Japanese human embryonic stem cells in feeder-free conditions

Human pluripotent embryonic stem cells (hESCs) have great promise for research into human developmental biology, development of cell therapies for the treatment of diseases, toxicology, and drug discovery. Traditionally, undifferentiated hESCs are maintained on mouse embryonic fibroblasts (MEFs), which impede the clinical applications of hESCs. Here we have examined the long-term stability of the Japanese hESC line (KhES-1) in feeder-free culture. KhES-1 cells were cultured with MEF conditioned medium (CM) and different doses of basic fibroblast growth factor (bFGF) in six-well-plates of which the surface was coated with Matrigel. KhES-1 cells were maintained for at least 40 passages. In this culture system, the cells maintained stable proliferation rates and steadily expressed Oct-4, Nanog, and alkaline phosphatase. In addition, KhES-1 cells maintained without direct feeder contact formed embryonic bodies with expression of markers from the three germ layers. Here we demonstrated that Japanese human embryonic stem cells KhES-1 were cultured long term in a feeder-free method, while retaining pluripotency in vitro.     

Locally advanced non-small cell lung cancer completely resected after induction therapy

We analyzed 8 patients with unresectable locally advanced non-small cell lung cancer who responded to chemotherapy or chemoradiotherapy and underwent complete resection between June 2003 and June 2005. The patients were all male with a mean age of 61 years (range, 42 to 72 years). Histological subtypes included adenocarcinoma in 4 patients and squamous cell carcinoma in 4 patients. Clinical staging included T2N2M0 in 3 patients, T2N3M0 in 2 patients, and 1 patient each for T3N2M0, T4N2M0, and T4N3M0. Preoperative treatment included chemotherapy in 5 patients and chemoradiotherapy in 3 patients, all of whom had a partial response. Surgical procedures included lobectomy in 6 patients and pneumonectomy in 2 patients. In addition, all of the patients underwent mediastinal lymph node dissection (ND2a). Pathological effect included Ef. 0 in 1 patient, Ef. 1 in 2 patients, Ef. 2 in 2 patients, Ef. 3 in 3 patients. The median survival time from initial treatment (or surgery) was 16 (14) months in all cases, 22 (19) for ycN0, 12 (8) for ycN2, 31 (27) for Ef. 3, 13 (9) for Ef. 0-2, 27 (23) for pN0, 13 (9) for pN1-3, 31 (27) for chemoradiotherapy, 16 (13) for chemotherapy, 24 (21) for adenocarcinoma, and 15 (11) for squamous cell carcinoma. Multimodality treatment, including surgery, is beneficial for patients with unresectable locally advanced non-small cell lung cancer who respond to chemotherapy or chemoradiotherapy, especially those patients with ycN0 or pN0.