Living‐Related Liver Transplantation for Siblings with Progressive Familial Intrahepatic Cholestasis 2, with Novel Genetic Findings

Progressive familial intrahepatic cholestasis is a syndrome of severe cholestasis progressing to biliary cirrhosis and liver failure that develops in childhood. This report describes two siblings with PFIC‐2 who underwent living‐related liver transplantation from their genetically proven heterozygous parents. Both patients had normal gamma‐glutamyl transpeptidase levels, but showed severe pruritus with sleep disturbance, cholestasis, jaundice and growth failure. Genetic testing of each patient revealed two missense mutations of the bile salt export pump, S901R and C1083Y, which have not previously been associated with PFIC‐2. Usual medical treatment failed to improve their clinical symptoms, and the two siblings underwent living‐related liver transplantation from their heterozygous parents. The transplants improved their clinical symptoms significantly, and the patients have since shown age‐appropriate growth. Electron microscopic findings of the explanted liver of the younger sister revealed dense and amorphous bile, which is characteristic of PFIC‐2. In the cases presented here, living‐related liver transplantation from a heterozygous donor was associated with better quality of life and improvement of growth, and thus appears to be a feasible option for PFIC‐2 patients. Mutation analysis is a useful tool to help decide the course of treatment of PFIC.     

Conus perimedullary arteriovenous fistula with multiple shunt points including the cauda equina: a case report

Perimedullary arteriovenous fistula (AVF) is usually located on the surface of the spinal cord and is fed by the anterior spinal artery and/or the posterior spinal artery. We report a rare case of Conus perimedullary AVF with multiple shunt points including the cauda equina. A 68-year-old man presented with leg pain when walking long distances. Magnetic resonance imaging at the thoracic and lumbar level revealed multiple signal voids with marked cord signal change. Angiography showed the perimedullary AVF fed by the anterior spinal artery from the right T9 intercostal artery at the level of the conus medullaris and the fistula fed by the left lateral sacral artery from the left internal iliac artery at the level of the cauda equina. In the first surgery, we performed surgical interruption of feeding arteries from the filum terminale and coagulated AV shunt of the conus medullaris. However residual perimedullary AVF was found at the conus medullaris in the postoperative angiography. Secondary surgery was carried out to treat residual AVF. Follow-up angiography showed complete disappearance of all AVFs. Postoperatively, the patient`s symptoms were improved. Because the Conus perimedullary AVF has the characteristics of multiple feeding arteies, multiple shunt points, and complex venous drainage, it must be kept in mind that other fistula could exist in the cauda equina or filum terminale.     

Polarized osteoclasts put marks of tartrate-resistant acid phosphatase on dentin slices--a simple method for identifying polarized osteoclasts

Osteoclasts form ruffled borders and sealing zones toward bone surfaces to resorb bone. Sealing zones are defined as ringed structures of F-actin dots (actin rings). Polarized osteoclasts secrete protons to bone surfaces via vacuolar proton ATPase through ruffled borders. Catabolic enzymes such as tartrate-resistant acid phosphatase (TRAP) and cathepsin K are also secreted to bone surfaces. Here we show a simple method of identifying functional vestiges of polarized osteoclasts. Osteoclasts obtained from cocultures of mouse osteoblasts and bone marrow cells were cultured for 48 h on dentin slices. Cultures were then fixed and stained for TRAP to identify osteoclasts on the slices. Cells were removed from the slices with cotton swabs, and the slices subjected to TRAP and Mayer's hematoxylin staining. Small TRAP-positive spots (TRAP-marks) were detected in the resorption pits stained with Mayer's hematoxylin. Pitted areas were not always located in the places of osteoclasts, but osteoclasts existed on all TRAP-marks. A time course experiment showed that the number of TRAP-marks was maintained, while the number of resorption pits increased with the culture period. The position of actin rings formed in osteoclasts corresponded to that of TRAP-marks on dentin slices. Immunostaining of dentin slices showed that both cathepsin K and vacuolar proton ATPase were colocalized with the TRAP-marks. Treatment of osteoclast cultures with alendronate, a bisphosphonate, suppressed the formation of TRAP-marks and resorption pits without affecting the cell viability. Calcitonin induced the disappearance of both actin rings and TRAP-marks in osteoclast cultures. These results suggest that TRAP-marks are vestiges of proteins secreted by polarized osteoclasts.     

Cardiac anesthesia induction by low target plasma concentration setting of propofol using target-controlled infusion

BACKGROUND: Propofol-anesthesia administerd using target-controlled infusion (TCI) has been proposed for cardiac surgery. But, moderate target concentration of propofol during induction using TCI has not been studied in detail. METHODS: Thirty patients scheduled for cardiac surgery under cardiopulmonary bypass (CPB) and TCI propofol anesthesia were randomly divided into two groups to receive a computer-controlled infusion of propofol with target concentrations of 1.5 or 2.0 micro/g x ml(-1) [1.5 microg x ml(-1) group (n=15) and 2.0 microg x ml(-1) group (n=15)]. Mean arterial pressure (MAP), heart rate (HR) and bispectral index scale (BIS) values were recorded at 5 time points during induction of anesthesia. RESULTS: MAP was significantly lower in 2.0 microg x ml(-1) group compared with 1.5 microg x ml(-1) group. In both groups, a rise of BIS value did not occur during tracheal intubation. CONCLUSIONS: We have demonstrated that propofol TCI at a target concentration of 1.5 microg x ml(-1) is effective for hemodynamic stability during induction of anesthesia in patients for cardiac surgery under CPB.     

Curious complication after oroesophageal reconstruction with free jejunal flap: intussusception-like deformity of transferred jejunum

Regions of oropharyngeal cancer, hypopharyngeal cancer, and cervical esophageal cancer in a 70-year-old man were surgically resected and reconstructed with a free jejunal flap. Postoperatively the patient showed severe dysphagia and a chimneylike protrusion in the oral cavity. In a second operation, a dorsal, longitudinal incision for phimosis was made to release the stenosis; however, the dysphagia persisted, and peristalis seemed to interfere with deglutition. Here, we discuss the cause of the dysphagia, a complication of total reconstruction of oroesophageal defects with a single free jejunal flap, and the procedures of reconstruction.     

Differentiation of transplanted bone marrow cells in the adult mouse brain.

BACKGROUND: Bone marrow transplantation is reportedly effective in preventing the progression of neurological deterioration in lysosomal storage disorders, although the mechanism underlying the therapeutic effects remains to be elucidated. Recent research on stem cell biology suggests that bone marrow cells contain nonhematopoietic stem cells, including brain precursor cells. To evaluate the contribution of bone marrow cells as carriers for cell and gene therapy of neurological disorders, we studied the fate of transplanted bone marrow cells in the adult mouse brain. METHODS: Bone marrow cells were genetically marked with a retroviral vector containing the green fluorescence protein gene and then transplanted into irradiated mice by either systemic infusion or direct injection. To identify cell types, brain sections were stained with specific antibodies against neuronal cell markers-neuron specific enolase for neurons, glial fibrillary acidic protein (GFAP) for astrocytes, carbonic anhydrase II (CAII) for oligodendrocytes, and ionized calcium binding adaptor molecule 1 (Iba1) for microglia-and then examined under a confocal microscope. RESULTS: Twenty-four weeks after systemic infusion, transplanted cells expressed Iba1 but none of the other brain cell markers. Conversely, 12 weeks after direct injection, transplanted cells were stained with antibodies against GFAP, CAII, and Iba1. CONCLUSIONS: Bone marrow contains cells capable of differentiating into oligodendrocytes, astrocytes, and microglia when exposed to the brain microenvironment. Autologous bone marrow cells may be useful as carriers for ex vivo gene therapy for lysosomal disorders with neurological symptoms.     

Preoperative chemotherapy increases cytokine production after lung cancer surgery.

OBJECTIVE: Many phase II trials have shown that preoperative chemotherapy for lung cancer is feasible but associated with postoperative morbidity and mortality. However, little is known about the effect of preoperative chemotherapy on surgical stress and postoperative complications associated with surgical intervention. We evaluated the effect of preoperative chemotherapy on perioperative inflammatory cytokine production as a surgical stress marker. METHODS: The study group comprised 38 patients undergoing anatomical lung resection and mediastinal nodal dissection for clinical stage IB/II non-small cell lung cancer during the period October 2001-December 2003. Nineteen patients received a single cycle of cisplatin (80 mg/m(2)) and docetaxel (60 mg/m(2)) chemotherapy prior to surgery (neoadjuvant group), and 19 patients underwent surgery without any previous chemotherapy (control group). White blood cell and neutrophil counts and serum concentrations of C-reactive protein (CRP), interleukin-6 (IL-6), and granulocyte colony-stimulating factor (GCSF) were determined before surgery and on postoperative days 1 and 3. Postoperative complications were reviewed. Differences were assessed by repeated analysis of variance. RESULTS: Serum concentrations of IL-6 and GCSF rose significantly on postoperative days 1 and 3 in the neoadjuvant group in comparison to concentrations in the control group, but white blood cell count, neutrophil count, and CRP did not differ between the groups. No major complication occurred in either group. CONCLUSIONS: A single cycle of cisplatin and docetaxel chemotherapy followed by surgery can exacerbate overproduction of inflammatory cytokines during the perioperative period in lung cancer patients.     

Spinal cord ischemia after endovascular aortic repair versus open surgical repair for descending thoracic and thoracoabdominal aortic aneurism

Purpose

Thoracic endovascular aortic repair (TEVAR) an emerging less invasive alternative to surgery, is now being increasingly employed, but spinal cord ischemia (SCI) is still a threat with this procedure. Delayed paraplegia has been frequently observed after TEVAR, suggesting there may be different courses of SCI between TEVAR and the conventional open surgical repair (OSR) of thoracic and thoracoabdominal aneurysms. Therefore, we conducted a study to investigate the risk factors for and the course of SCI after TEVAR and OSR.

Methods

We studied a series of 414 OSR and 94 TEVAR patients prospectively. Postoperative motor function, sensory disturbance, and bladder disturbance were assessed daily to evaluate the course of SCI. Previously reported risk factors for SCI were investigated.

Results

Spinal cord ischemia occurred in 6 patients (6.4 %) in the TEVAR group, and in 18 patients (4.3 %) in the OSR group, resulting in no significant difference (p = 0.401). A greater percentage of patients (n = 4, 66.7 %) with SCI in the TEVAR group had a delayed onset, compared with 16.7 % (n = 3) in the OSR group (p = 0.038). The rate of recovery of walking function after SCI and the incidence of sensory disturbance and bladder dysfunction was similar in the two groups. Multivariate analysis demonstrated that, in the TEVAR group, the stent length of aortic coverage was a significant risk factor for SCI.

Conclusion

The incidence of SCI was similar in the OSR and TEVAR groups, but delayed SCI occurred more frequently in the TEVAR group. Except for the delayed onset of SCI, SCI showed a similar course of recovery in the two groups.     

Hemangiopericytoma on the intradural thoracic spinal cord: a case report

Hemangiopericytoma develops from many organs. In the central nervous system, most tumors arise in the intracranial portion, and tumors originating from the spinal cord are rare. Its clinical course and neurological characteristics have not been disclosed. We present a case of a 51-year-old woman with gradually progressing paraparesis. Magnetic resonance (MR) images of the thoracic spine demonstrated an intradural tumor at the 6 and 7 thoracic vertebral body level. The patient underwent total excision of the tumor. The histological diagnosis was hemangiopericytoma. MR images after the operation showed no residual tumor and the patient was followed up without adjuvant therapy. However, 5 years later, the patient complained of back pain and gait disturbance again, and MR images showed a recurrence of the tumor. We resected the tumor under motor evoked potential (MEP) monitoring and removed the extradural part of the tumor, but the part of the tumor which had infiltrated the spinal cord was left due to the lowering of MEP amplitude. The operation resulted in partial resection. Spinal intradural hemangiopericytoma is very rare, and only 15 cases including the present case have been reported. This paper will discuss the clinical characteristics and treatment for this tumor.     

A case of thoracic disc herniation with sudden onset paraplegia on toilet straining: case report

Thoracic disc herniation is less common rather than cervical or lumbar herniation. Cases of sudden onset without trauma are especially rare. Generally, the neurological onset of disc herniation is caused by mechanical cord compression due to a protruded disc, and its onset is usually gradual. Ischemia is also considered as a factor of neurological onset. We report a case of a 78-year-old male with sudden paraplegia while straining at the toilet. T2 weighted MR image on admission showed mild disc protrusion at the level of Th8-9 and intramedullary high signal intensity below the Th8-9 level. We speculate that Valsalva-like maneuver had led to the congestion of vertebral venous plexus or compression of the anterior spinal artery, and spinal ischemia occurred.