Hemiarch replacement during pregnancy (19 weeks) utilizing normothermic selective cerebral perfusion

A 20-year-old woman with Marfan syndrome in the 19 weeks of pregnancy was admitted for a dissecting aneurysm of the ascending aorta to the proximal arch. She wanted to continue with pregnancy. A cardiotocography and a trans-esophageal echo probe which allowed visualization of the fetal heart contraction were attached on the abdomen. A cardio-pulmonary bypass was established via the right axillar and femoral arteries with bicaval drainage. The aortic isthmus and arch branches were clamped. After opening the ascending aorta, selective cerebral perfusion was initiated by quick cannulation into the left carotid and left subclavian arteries. Peripheral pressure was maintained above 80 mmHg with the flow of the selective cerebral perfusion at 1.0 L/min and flow from the femoral artery at 3.5 L/min. Hemi-arch replacement was performed using a Dacron graft. The body temperature passively descended to 35 °C. She normally delivered a healthy female baby after 37 weeks’ of gestation.     

Disruption of the gene encoding the beta1-subunit of transducin in the Rd4/+ mouse

PURPOSE: The Rd4/+ mouse inherits an autosomal dominant retinal degeneration that cosegregates with a large inversion spanning nearly all of mouse chromosome 4 (Chr 4). This inversion is homozygous lethal. The hypothesis for the study was that disruption of a gene at one of the two breakpoints in the Rd4 chromosome is responsible for the retinal degeneration. The purpose was to identify the disrupted gene. METHODS: Genotyping was performed by PCR and gel electrophoresis. The Rd4/+ phenotype was confirmed by ERG. Fluorescence in situ hybridization (FISH) analysis was performed with bacterial artificial chromosome (BAC) probes. Northern and quantitative PCR procedures were used to evaluate Gnb1 mRNA expression. Protein expression was measured by Western blot. RESULTS: To identify the Rd4 gene defect, the breakpoints were first localized with a testcross and the locus refined by using FISH. Genetic testcross data revealed that the inversion breakpoints are located within a few centimorgans of both the telomeric and centromeric ends of Chr 4. Initial FISH analysis showed the proximal breakpoint of the inversion to be in the centromere itself. Therefore, we focused on the distal breakpoint and found that it lies in the second intron of the gene Gnb1, coding for the transducin beta1-subunit (Tbeta1) protein that is directly involved in the response to light of rod photoreceptors. Before the beginning of retinal degeneration in Rd4/+ retina, the levels of Gnb1 mRNA and Tbeta1 protein are 50% of those in wild-type retina. CONCLUSIONS: The results suggest that disruption of the Gnb1 gene is responsible for Rd4 retinal disease.     

Interleukin‐8‐producing primary cardiac undifferentiated sarcoma in a child with sustained fever

We report the case of a 12‐year‐old boy with primary undifferentiated sarcoma of the left atrium. He had sustained fever during the clinical course and multiple lung and brain metastases. Chemotherapy and irradiation were ineffective; he died 41 days after hospitalization. On retrospective analysis, interleukin‐8 (IL‐8) was elevated; this was supported by immunohistochemistry and gene expression analysis of tumor samples. IL‐8 continued to increase with tumor progression accompanied by elevated neutrophil count and C‐reactive protein. IL‐8 is involved in malignant tumor proliferation, migration, and angiogenesis and may have been related to the clinical condition and prognosis in the present case.     

Examination of soluble Fas (sFas) and soluble Fas ligand (sFasL) in patients with burns

The FasL–Fas system is one of the recognized apoptosis-inducing systems, and has been determined to have important functions in relation to homeostasis and biological defense mechanisms. In this study, we investigated the serum levels of soluble Fas (sFas), soluble FasL (sFasL) and tumor necrosis factor (TNF-) in patients with burns. The sFas levels were found to be significantly higher in the patients who eventually died as compared to those in the patients who survived (3.9±1.8 ng/ml versus 2.6±1.0 ng/ml). On the other hand, the sFasL levels were significantly higher in the patients who survived (61.5±29.9 ng/ml versus 37.2±14.4 ng/ml) than in those who eventually died. A positive correlation was noted between the TNF- level and the sFas level, and a negative correlation was observed between the TNF- level and the sFasL level. These findings suggest that worsening of the condition of a burns patient may be related to changes in the Fas–FasL system.     

7-year survey of anesthesia for cesarean section--comparison of tetracaine and bupivacaine as intrathecal anesthetic agents

BACKGROUND: In our institution, spinal anesthesia is the first choice for cesarean section. After the introduction of bupivacaine in 2000 in Japan, the intrathecal anesthetic agent shifted from tetracaine to bupivacaine. We analyzed the anesthesia for cesarean section in recent 7 years and compared the anesthetic quality of tetracaine with that of bupivacaine. METHODS: The anesthetic records were reviewed in the patients who had received cesarean section between January 1998 and December 2004 at our institution. RESULTS:There were 10456 deliveries during the study period with a cesarean section rate of 28.2% (2947 cases). Ninety-one percent of cesarean section was performed under spinal anesthesia. Spinal anesthetic agent shifted from tetracaine to bupivacaine in 2000-2001, both of which was prepared as a hyperbaric solution and supplemented with 0.1 mg of morphine hydrocloride. Of the 2711 patients in whom a cesarean section was started under spinal anesthesia, 20 (0.7%) required conversion to general anesthesia. Three hundred eighteen patients (11.7%) required some analgesic supplementation. The incidence of intra-operative analgesic supplementation was greater in the patients anesthetized with hyperbaric tetracaine and morphine than in those anesthetized with hyperbaric bupivacaine and morphine (22.96% vs 4.20% ; P<0.01). The conversion rate from spinal to general anesthesia for cesarean section was 0.7%. CONCLUSIONS: Comparing these two intrathecal anesthetic agents, the rate of analgesic supplementation in those anesthetized with bupivacaine was lower than in those anesthetized with tetracaine. This suggests that bupivacaine provides the more profound blockade of the visceral pain than tetracaine, and is superior as a local anesthetic.     

Inhibition of plasminogen activator inhibitor-1 by angiotensin II receptor blockers on cyclosporine-treated renal allograft recipients

INTRODUCTION: We previously showed that proteinuria from a renal graft was significantly decreased by administration of losartan potassium, an angiotensin II receptor blockers (ARB). To further evaluate the mechanism, we performed another clinical study focusing on the change in plasma plasminogen activator inhibitor-1 (PAI-1) levels among cyclosporine (CyA)-treated renal allograft recipients. METHODS: Among 12 hypertensive CyA-treated kidney transplant patients, four received 25 to 50 mg/day of losartan; four, 4 to 8 mg/day of candesartan cilexetil; and another four, 20 to 40 mg/day of nifedipine. Four CyA-treated kidney-transplanted patients without hypertension were selected as a control group. Informed consent was obtained from all participants. PAI-1 and serum creatinine (S-Cr) levels were monitored every 3 months for 1 year. RESULTS: Considering the pretreatment of PAI-1 as 100%, the mean percent of PAI-1 at 1 year after the onset of study for losartan, candesartan, nifedipine, and control groups were 78.6 +/- 6.7%, 81.4 +/- 8.0%, 96.7 +/- 7.6%, and 110.4 +/- 9.2%, respectively. The ARB groups demonstrated significant differences from the control group (P < .01), while the nifedipine group did not. S-Cr levels among ARB-administered groups were increased slightly but temporarily. As for S-Cr levels, no significant differences were seen among the four groups. CONCLUSIONS: Control of hypertension itself is important for all renal graft recipients; however, PAI-1 reduction by ARBs was thought to be a key for renal preservation. We expect that ARBs will contribute to prolonged renal allograft survival.     

Chronic kidney disease and clinical outcomes in patients with COVID-19 in Japan

Clinical and Experimental Nephrology - Identifying predictive factors for coronavirus disease 2019 (COVID-19) is crucial for risk stratification and intervention. Kidney dysfunction contributes to...     

Surgical outcomes of nephron-sparing surgery for renal tumors

PURPOSE: We retrospectively assessed the surgical outcomes of nephron-sparing surgery (NSS) for patients with renal tumors. PATIENTS AND METHODS: From 1985 to March 2001, a total of 99 NSSs were performed on 94 patients with renal tumors. The patients were divided into three groups. Group I comprised of 22 patients who underwent imperative surgeries for renal cell carcinoma (RCC). The tumors were found in 18 patients bilaterally (including 8 patients with von Hippel-Lindau disease), in 3 with solitary kidney, and in 1 with chronic renal failure. The mean +/- standard deviation of patient age and tumor diameter was 46 +/- 23 years and 36 +/- 23 mm, respectively. Twenty-three in situ NSSs were performed on 18 patients in Group I, and the remaining 4 patients were treated with 3 simultaneous operations for bilateral renal tumors with or without 2 ex vivo surgeries. Group II consisted of 49 patients who had small RCCs with the normal contralateral kidney and underwent NSSs (elective indication). The mean age and tumor diameter was 54 +/- 10 years and 28 +/- 11 mm, respectively. Group III consisted of 23 patients with non-RCC tumor (10 angiomyolipomas, 8 cystic tumors, 2 adenomas, 2 metastatic tumors, and 1 degenerative lesion), all of whom were treated with NSS. The mean age and tumor diameter was 47 +/- 14 years and 41 +/- 29 mm, respectively. RESULTS: In Group I, 3 patients died of cancer including 2 patients who had had multiple lung metastases preoperatively. The five-year tumor specific survival rate was 87.3% with a postoperative follow-up of 49 +/- 36 months. In Group II, there were few peri-operative complications or no local recurrence at follow-up of 52 +/- 38 months. A patient developed lung metastasis, which was removed surgically with no evidence of recurrence at 159 months after NSS. Postoperative renal scintigraphy on 35 patients showed well-preserved renal function of the operated kidney. Improvement in surgical techniques resulted in less-invasive surgery in 22 operations during the last 4 years. The patients of Group III were also operated uneventfully, although 1 experienced postoperative bleeding. In 12 patients with solitary kidney (11 in Group I and 1 in Group III) serum creatinine level increased transiently, decreased to 1.3 times of preoperative values within 3 months, and almost recovered at 1-year follow-up. CONCLUSION: Excellent outcomes in cancer control and preservation of renal function support the validity of nephron-sparing surgery to treat renal tumors. The candidate patients may include those with bilateral kidney tumors, tumor occuring in the solitary kidney or small renal cell carcinomas with the normal contralateral kidney. Earlier detection of small lesions and less invasive surgical techniques will facilitate a wider indication of NSS.