Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatis

BackgroundAlagille syndrome (ALGS) is an autosomal dominant disorder, characterized by a paucity of intrahepatic bile ducts, resulting in significant cholestasis, and peculiar extrahepatic features. Some ALGS patients show a considerable overlap with biliary atresia (BA), and they can undergo Kasai procedure. The purpose of this study is to show the manifestations of BA overlapped ALGS cases in our institution, and to compare the outcomes of ALGS patients following liver transplantation (LT) between those who previously underwent Kasai surgery (ALGS-Kasai group) and those who did not (ALGS-non-Kasai group).MethodsMedical records of ALGS patients who underwent LT in Kyoto University Hospital, Japan from January 1992 to March 2018 were analyzed. ALGS diagnosis was determined according to physical, radiologic, and histopathological findings.ResultsThirty-one patients were ascertained (ALGS-Kasai: 4 males and 5 females vs. ALGS-non-Kasai: 14 males and 8 females, p = 0.43). Of 31 ALGS patients, 96.8% of children had pulmonary artery stenosis, 54.8% showed facial features, 29% revealed skeletal anomalies and 9.7% demonstrated ocular anomalies. The age at LT was significantly younger in ALGS-Kasai than ALGS-non-Kasai group (1.47 [interquartile range (IQR), 0.75–1.92] vs. 5.1 [IQR, 1.4–9.29] years; p = 0.038). Overall patient survival did not significantly differ between ALGS-Kasai (88.9%) and ALGS-non-Kasai patients (86.4%) (p = 0.84). Furthermore, the 1-year, 5-year, and 10-year patient survival rates for ALGS-Kasai group were 100%, 88.9%, and 88.9%, respectively, whereas those for ALGS-non-Kasai group were 90.9%, 90.9%, and 86.4%, respectively, with p-values of 0.36, 0.90, and 0.84, respectively.ConclusionsBA overlapped ALGS cases had neonatal progressive cholestasis which prompted Kasai procedure, and early liver dysfunction after Kasai led to performing LT. The ALGS-Kasai patients undergo LT at earlier ages than the ALGS-non-Kasai patients, however, overall patients' survival rates are similar between groups. Overall ALGS patients' survival rate after LT is considered high.Levels of EvidenceLevel III; Case–control study or Retrospective comparative study.     

Reversible posterior leukoencephalopathy in a patient with Wegener granulomatosis

A 14-year-old girl with rapidly progressive glomerulonephritis was transferred to our hospital because of acute renal failure. A diagnosis of Wegener granulomatosis was made according to the symptom triad of a renal biopsy demonstrating crescentic glomerulonephritis, severe sinusitis, and serological findings of raised proteinase 3 anti-neutrophil cytoplasmic antibody level. In spite of combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange, her renal function gradually deteriorated. Thereafter, she suffered a severe headache and generalized seizures. Brain computed tomography (CT) scan revealed bilateral low-density areas in the parieto-occipital lobes. Magnetic resonance imaging (MRI) disclosed a high-intensity area on T2-weighted images and a low-signal intensity area on T1-weighted images in the same lesion. Follow-up brain CT scan 3 weeks and MRI 2 months after the first studies showed complete resolution of the abnormal lesions, which indicated reversible posterior leukoencephalopathy syndrome. In addition to renal failure, hypertension, and cyclophoshamide, the primary disease may have played a role in the development of this uncommon syndrome in our patient.     

Surgical statistics of the Department of Urology, Isesaki Municipal Hospital during a five-year period (June 1998-May 2003)

We reviewed the surgical statistics of the Department of Urology, Isesaki Municipal Hospital between June 1998 and May 2003. A total of 1940 surgeries were performed. These consisted of 399 surgeries on the kidney, adrenal, and pelvis, 212 on the ureter, 433 on the bladder, 256 on the prostate, 149 on the urethra and the penis, 192 on the scrotum, 255 on the arteriovenous fistula and CAPD. In September 2003, a screening program for prostate cancer was started, and the number of prostate biopsies and total prostatectomies increased. As the adaptation of many procedures to laparoscopy expanded, the number of laparoscopic surgeries increased.     

Cervical myelopathy due to OPLL: clinical evaluation by MRI and intraoperative spinal sonography

BACKGROUND: Concerning the relationship between morphology and clinical outcome, there have been many reports using computed tomography/myelography but not so many using axial magnetic resonance imaging (MRI) of the spinal cord. This is the first report to correlate axial cord image, intensity changes in MRI, and cord expansion pattern using intraoperative ultrasonography. OBJECTIVE: The objectives were to correlate MRI studies, axial cord images/expansion, and changes in MRI intensity to see if there is a direct prognostic significance to these changes and to determine whether preoperative axial MRI images of the spinal cord predict recovery from compressive myelopathy. METHODS: Posterior cervical decompressions with laminoplasty were performed in 44 patients with cervical myelopathy due to ossification of the posterior longitudinal ligament. On T2-weighted MR images, the cross-sectional shape of the cord at the level of maximal compression was categorized as boomerang, teardrop, or triangle. Additionally, with use of intraoperative ultrasonography, the expansion pattern of the cord that occurred intraoperatively was contrasted with that seen on postoperative MR images. RESULTS: Clinical recovery rates were the worst for those with triangular, intermediate for those with boomerang, and the best for those with teardrop shape. Preoperative low T1 and high T2 signals were found in most cases with triangular cord configurations. Triangular cord configurations showed the least expansion among the three categorized spinal cords. CONCLUSION: Patients with triangular deformity of the cord have atrophy as confirmed on MR studies where there is a low T1 and high T2 signal in the cord. Poor postoperative clinical recovery correlates with the lack of postoperative cord expansion on either MR or ultrasound evaluations. Those with either teardrop or boomerang deformities demonstrate a relatively good recovery rate.     

Motion‐induced disturbance of auditory–motor synchronization and its modulation by transcranial direct current stimulation

The timing of personal movement with respect to external events has previously been investigated using a synchronized finger‐tapping task with a sequence of auditory or visual stimuli. While visuomotor synchronization is more accurate with moving stimuli than with stationary stimuli, it remains unclear whether the same principle holds true in the auditory domain. Although the right inferior–superior parietal lobe (IPL/SPL), a center of auditory motion processing, is expected to be involved in auditory–motor synchronization with moving sounds, its functional relevance has not yet been investigated. The aim of the present study was thus to clarify whether horizontal auditory motion affects the accuracy of finger‐tapping synchronized with sounds, as well as whether the application of transcranial direct current stimulation (tDCS) to the right IPL/SPL affects this. Nineteen healthy right‐handed participants performed a task in which tapping was synchronized with both stationary sounds and sounds that created apparent horizontal motion. This task was performed before and during anodal, cathodal and sham tDCS application to the right IPL/SPL in separate sessions. The time difference between the onset of the sounds and tapping was larger with apparently moving sounds than with stationary sounds. Cathodal tDCS decreased this difference, anodal tDCS increased the variance of the difference and sham stimulation had no effect. These results supported the hypothesis that auditory motion disturbs efficient auditory–motor synchronization and that the right IPL/SPL plays an important role in tapping in synchrony with moving sounds via auditory motion processing.     

Involvement of estrogen receptors in prostatic diseases

Accumulating evidence shows that estrogens participate in the pathogenesis and development of benign prostatic hyperplasia and prostate cancer by activating estrogen receptor α. In contrast, estrogen receptor β is involved in the differentiation and maturation of prostatic epithelial cells, and thus possesses antitumor effects in prostate cancer. However, the natural ligands of estrogen receptor β are not fully understood, and its mode of action according to its ligands and the binding sites located in the promoter regions of downstream genes remains to be elucidated. Here, we review recent experimental investigations of estrogen receptors and their urological relevance. Estrogen receptor‐mediated signaling in the prostate is essential together with the androgen receptor‐mediated pathway, providing a new therapeutic target for prostatic diseases.     

Neutrophil elastase activity in pulmonary venous blood during lung resection

OBJECTIVES Neutrophil elastase has been reported to play an important role in acute lung injury, which is a major cause of postoperative mortality after pulmonary resection. Neutrophil elastase released in the lungs reaches the peripheral circulation via the pulmonary veins. This study was performed to compare neutrophil elastase activity in pulmonary venous blood (collected during lobectomy) with that in the peripheral blood, and to determine the perioperative changes of neutrophil elastase activity. METHODS In 34 patients undergoing lobectomy with mediastinal lymph node dissection, the leucocyte count, neutrophil count, neutrophil elastase activity and levels of tumour necrosis factor-α, interleukin-6 and interleukin-8 were measured in the pulmonary venous blood and peripheral arterial blood before and after surgery. Then, these parameters were compared between before and after surgery with peripheral and pulmonary blood. RESULTS Neutrophil elastase activity was found to be significantly higher in pulmonary venous blood at the completion of surgery than at the start (during thoracotomy), while the neutrophil elastase activity of peripheral arterial blood showed no significant change between the start and completion of surgery. CONCLUSIONS In conclusion, measurement of neutrophil elastase activity in pulmonary venous blood revealed changes associated with lobectomy.     

Cardiopulmonary function in elderly patients with cervical spondylotic myelopathy

Background  

Most elderly patients have cardiopulmonary diseases anamnesis and a perceived risk of perioperative complications. The responsible lesion may be located more cranially in elderly patients with cervical spondylotic myelopathy (CSM) compared with that in younger patients. The study aimed at evaluating cardiopulmonary dysfunction of CSM and effects of surgery on cardiopulmonary function and perioperative complications.     

Surgery combined with radiosurgery of large acoustic neuromas

The treatment of acoustic neuromas has been improved by advancements in microsurgical techniques and in radiosurgery. To further elucidate the degree of clinical improvement, we evaluated the treatment results of a combination of surgery and radiosurgery for large acoustic neuromas. METHODS: From January 1994 through December 2000, we treated 14 patients with large acoustic neuromas using a combination of surgery and radiosurgery. Of these, 8 were male and 6 were female patients, with an average age of 47 years (range, 18-64). The average maximum diameter of the tumor was 42 mm (range, 30-58 mm). All patients underwent operations using the retrosigmoid approach, and one patient was retreated using the transpetrosal transtentorial approach. The tumors were removed subtotally in thirteen patients and partially in one who had a very large hypervascular acoustic neuroma. There were no mortality and no surgical complications, such as hemorrhage or CSF leakage. Postoperative facial palsy was avoided in 10 patients (71%). Radiosurgery was performed 1 to 6 months (mean, 2.9 months) after surgery. At the time of radiosurgery, the treatment size (mean diameter) became 19.2 mm (range, 9.8-36.1 mm). The average tumor marginal dose was 12.1Gy (range, from 10-14 Gy). The mean follow-up period was 32 months after radiosurgery. RESULTS: The tumor size decreased in 6 patients, unchanged in 5 patients, and increased in 3 patients. Only 1 patient (7%) with extra large tumor needed surgical resection 1 year after radiosurgery. Excellent facial nerve function (House & Brackmann Grade I or II) was preserved in 12 patients (85.7%) in the final follow-up. CONCLUSIONS: In the case of large acoustic neuromas, subtotal removal and subsequent radiosurgery is one option for maintaining cranial nerve function and long-term tumor growth control.     

Tissue factor antisense oligonucleotides prevent renal ischemia-reperfusion injury

BACKGROUND: Tissue factor (TF) expression is induced on macrophages and endothelial cells during the immune response. We designed an antisense (AS) phosphorothioate oligodeoxynucleotide (ODN) to specifically inhibit the expression of rat TF to study the effects of the AS ODN on renal ischemia-reperfusion injury in the rat. METHOD: AS-1 ODN for TF was delivered intravenously to inhibit the expression of TF in endothelial cells. After 8 hr, the right kidney was harvested and the left renal artery and vein were clamped. The kidney was reperfused after 90 min of ischemia, and rats were killed at 0, 1.5, 5, 12, and 24 hr after reperfusion. TF expression was analyzed by immunohistochemical staining using monoclonal antibody. RESULTS: In the untreated ischemic group, 0 of 20 rats survived beyond day 3. However, treatment with AS-1/TF led to 12 of 20 rats surviving beyond day 4. TF was detected on distal tubular epithelial cells, endothelial cells, and blood vessels but not on necrotic and proximal tubular epithelial cells. The necrotic area extended and encompassed nearly all of the ischemic kidney within 12 hr after reperfusion. The necrotic area and the grade of TF staining were more significantly reduced in the AS-1/TF-treated group than in the control group. Furthermore, fluorescein isothiocyanate-labeled AS-1/TF was significantly intense in tubular epithelial cells 8 hr after intravenous administration. CONCLUSIONS: The results indicate that AS-1/TF inhibited the ischemia-reperfusion injury of the kidney. Microcirculatory incompetence resulting from microthrombus may cause the formation and development of necrosis.