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Objective: In subjects with chronic kidney disease (CKD), the effect of low-protein diet (LPD) is expected to alleviate uremic symptoms. However, whether LPD is effective in preventing loss of kidney function is controversial. The aim of this study was to evaluate the association between LPD and renal outcomes., Methods: We conducted a multicenter cohort study of 325 patients who suffered CKD stage 4 and 5 with eGFR ≥10 mL/min/1.73 m, 2 between January 2008 and December 2014. The primary diseases of the patients were chronic glomerulonephritis (47.7%), nephrosclerosis (16.9%), diabetic nephropathy (26.2%), and others (9.2%). The patients were divided into four groups, based on the mean protein intake (PI)/day, group 1 (n = 76): PI < 0.5 g/kg ideal body weight/day, group 2 (n = 56): 0.5 ≤ PI < 0.6 g/kg/day, group 3 (n = 110): 0.6 ≤ PI < 0.8 g/kg/day, group 4 (n = 83): PI ≥ 0.8 g/kg/day. Dietary supplementation with essential amino acids and ketoanalogues was not used. The outcome measure was occurrence of renal replacement therapy (RRT) (hemodialysis, peritoneal dialysis, renal transplantation (excluding preemptive transplantation)) and all-cause mortality until December 2018. Cox regression models were used to examine whether LPD was associated with the risk of outcomes., Results: During a mean follow-up of 4.1 ± 2.2 years. Thirty-three patients (10.2%) died of all causes, 163 patients (50.2%) needed to start RRT, and 6 patients (1.8%) received a renal transplant. LPD therapy of 0.5 g/kg/day or less was significantly related to a lower risk of RRT and all-cause mortality [Hazard ratio = 0.656; 95% confidence interval, 0.438 to 0.984, P = .042]., Conclusions: These results suggest that non-supplemented LPD therapy of 0.5 g/kg/day or less may prolong the initiation of RRT in stage 4 and 5 CKD patients., (Copyright © 2023 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

A new cholestane-type steroidal glycoside, solamyriaside A ( 1 ), was isolated from the fruits of Solanum myriacanthum Dunal (Solanaceae), along with two known steroidal glycosides, namely, solaviaside A ( 2 ) and aculeatiside A ( 3 ), and three known steroidal alkaloid glycosides, namely, solamargine ( 4 ), khasianine ( 5 ) and solasonine ( 6 ), which were isolated for the first time from this plant. Based on spectroscopic data as well as chemical evidence, 1 was determined to be 3- O -α-L-rhamnopyranosyl-(1→2)- O -[α-L-rhamnopyranosyl-(1→4)]-β-D-glucopyranosyl-22 R ,25 R -cholest-5-ene-3β,16α,22,26-tetraol 26- O -β-D-glucopyranoside. The cytotoxic activity of 1 - 6 against HL-60 human promyelocytic leukaemia cells was examined. Compounds 4 - 6 showed cytotoxic activity. Among them, 4 exhibited the strongest activity with an IC 50 value of 4.64 ± 0.17 μM, similar to the activity of cisplatin, a positive control.

Two new oleanane-type triterpenoids with an epoxy bridge between C-3 and C-25, named lantacamaric acids A and B, were isolated from the leaves and stems of Lantana camara L. (Verbenaceae). In addition, seven known triterpenoids, two known iridoid glycosides, four known phenylethanoid glycosides, one known flavonoid glycoside, and one known cyanogenic glycoside were isolated. Their structures were determined based on the spectroscopic data. Furthermore, the cytotoxic activities of the isolated triterpenoids toward HL-60 cells were examined. The IC 50 values of all compounds were obtained and ranged from 1.16 to 68.4 μM, with three compounds exhibiting stronger activities than the positive control, cisplatin. In addition, the structure-activity relationship was investigated.

We describe a rare case of giant cauda equina tumor in the upper lumbar spine with dural ectasia in the lumbosacral region. This patient had a long history of acromegaly due to pituitary adenoma, for which bromocriptine was administered. The cauda equina tumor was surgically resected en bloc and proved to be a benign schwannoma. Postoperatively, with the exception of the left L5 dermatome, sensory disturbance decreased markedly and motor function slowly improved. Although resection of a giant schwannoma of the cauda equina often sacrifices other cauda equina spinal nerves involved by the tumor, neurological deterioration was not observed in the case presented.

Study Design: In 38 patients with adolescent idiopathic scoliosis (AIS), the correlation between left and right differences in premotor time (D-PMT) of back muscle and clinical findings were analyzed., Objective: To investigate the clinical relevance of back muscle D-PMT in AIS., Summary of Background Data: There have been numerous studies investigating back muscle asymmetry of AIS by EMG, but to date, no studies have measured D-PMT in back muscles., Materials and Methods: D-PMT in the back muscles measured in a similar manner as that in the extremity muscles was assessed in 10 nonscoliotic teenaged girls and 38 AIS patients. The correlation between back muscle D-PMT and four factors (age, Risser sign, Cobb angle, and progression of deformity) was investigated., Results: The D-PMT values of back muscle at all levels in the control group were within +/-5 ms, but those in 20 of the 38 AIS patients were more than 5 ms. D-PMT at the lower-end vertebra level was strongly correlated with progression of deformity, but not with age, Risser sign, and Cobb angle. All five patients with D-PMT of more than 10 ms at the lower-end vertebra level had progressive deformity., Conclusions: D-PMT in back muscle at the lower end vertebra level in AIS correlated closely to the progression of scoliotic deformity, thus suggesting that this phenomenon is associated with the progression in AIS.

Study Design: Seven patients with severe angular kyphotic deformity of the spine were treated by circumferential spinal wedge osteotomy using a single posterior approach., Objective: To evaluate the surgical outcomes for seven patients with severe angular kyphosis or kyphoscoliosis treated by spinal wedge osteotomy., Summary of Background Data: Excellent surgical outcomes have been reported for procedures such as hemivertebra excision, vertebral body resection, and spinal osteotomy for angular kyphosis or kyphoscoliosis. However, the safety and efficacy of these procedures for severe and rigid deformities have not been established., Methods: The surgical procedure involves circumferential exposure of the apex vertebra to the anterior aspect using a single posterior approach. Sparing only the spinal cord, the surgeon performs circumferential wedge bone resection, closure, correction, and stabilization by instrumentation while monitoring the spinal cord. Seven patients (3 kyphotics and 4 kyphoscoliotics) treated by this procedure were evaluated for a minimum of 2 years. Underlying conditions comprised five cases of congenital deformity with hemivertebrae and two cases of skeletal dysplasias. The mean age at surgery was 16.5 years, and the mean follow-up period was 6.9 years., Results: Before surgery, the mean kyphotic curve was 105.4 degrees (range, 68-150 degrees ), and the mean scoliotic curve was 85.3 degrees (range, 60-132 degrees ). After surgery, the curves averaged 48.9 degrees and 40 degrees, respectively, yielding corrections of 52.9% and 54.9%. The mean posterior trunk shift in global sagittal balance was 21 mm before surgery, becoming 3 mm after surgery. Progressive spinal cord dysfunction had developed in three patients before surgery. In all three, postoperative neurologic improvement was observed. Postoperative complications consisted of transient, unilateral leg paresis in two patients. No incidents of infection or pseudarthrosis were observed., Conclusions: Spinal wedge osteotomy by the single posterior approach is a reliable and safe surgical technique for correcting severe rigid angular kyphosis or kyphoscoliosis.

Chondrodysplasia punctata (CDP) is a heterogeneous group of skeletal dysplasias characterized by stippled epiphyses. A subtype of CDP, X-linked dominant chondrodysplasia punctata (CDPX2), known also as Conradi-Hünermann-Happle syndrome, is a rare skeletal dysplasia characterized by short stature, craniofacial defects, cataracts, ichthyosis, coarse hair, and alopecia. The cause of CDPX2 was unknown until recent identification of mutations in the gene encoding Delta(8),Delta(7) sterol isomerase emopamil-binding protein (EBP). Twelve different EBP mutations have been reported in 14 patients with CDPX2 or unclassified CDP, but with no evidence of correlation between phenotype and nature of the mutation. To characterize additional mutations and investigate possible phenotype-genotype correlation, we sequenced the entire EBP gene in 8 Japanese individuals with CDP; 5 of them presented with a CDPX2 phenotypes. We found EBP mutations in all 5 CDPX2 individuals, but none in non-CDPX2 individuals. Three of these CDPX2 individuals carried novel nonsense mutations in EBPand the other two, separate missense mutations that had been reported also in different ethnic groups. Our results, combined with previous information, suggest all EBP mutations that produce truncated proteins result in typical CDPX2, whereas the phenotypes resulted from missense mutations are not always typical for CDPX2. Patients with nonsense mutations showed abnormal sterol profiles consistent with a defect in Delta(8), Delta(7) sterol isomerase. X-inactivation patterns of the patients showed no skewing, an observation that supports the assumption that inactivation of the EBP gene occurs at random in affected individuals., (Copyright 2000 Wiley-Liss, Inc.)

Spondylo-epi-metaphyseal dysplasia is a rare skeletal dysplasia showing radiographic abnormalities in the epiphyses and metaphyses of the long and short tubular bones, and in the spine. There are very few reports of a case followed throughout the entire growth period. Here we report the case of a Japanese girl followed from 1 month old to skeletal maturity at 16 years old. She developed progressive kyphoscoliosis at 7 years old, and underwent a surgical correction of the spine at 14 years old. The diagnosis of spondylo-epi-metaphyseal dysplasia was made from examining all the follow-up radiographs showing abnormalities in the spine, long bones and hands involving epiphyses and metaphyses. She had normal stature--a rare finding in this condition.

The oxygen consumption per kilogram of body weight was measured during walking at speeds of 3.0-6.0 km/hr on a tread-mill in twelve patients with spinal deformities, before and approximately two years after either an anterior surgical correction or a posterior surgical correction. The mean age was seventeen years. There were five male and seven female patients. There was no significant change in the pre-operative and the post-operative oxygen consumption per kilogram of body weight and respiratory rates. A significant increase was noted in the post-operative oxygen removal (VO2/VE) in both the anterior and posterior procedures groups. The post-operative improvement of pulmonary function was discussed.

When female analbuminemic rats were injected with 0.8 mg uric acid every 3 h, their survival time at 5 degrees C increased from 14 h to 28 h, but uric acid had no effect on analbuminemic male rats. When female rats were oophorectomized 1 week before cold exposure, the injection of uric acid had no effect on their survival. Furthermore, uric acid did not increase the survival of the female rats that were administered a pellet containing 5 mg testosterone 1 week before the cold exposure. When the male rats were castrated 1 week before cold exposure, their survival time decreased from 20 h to 14 h, and administrations of 5 mg estradiol pellet at the time of castration and 0.8 mg uric acid every 3 h during cold exposure increased their survival time to 23 h.

Analbuminemic rats died within 18 h after a rapid decrease of body temperature whereas control Charles River, Wistar, and Sprague Dawley rats survived for 40 h, when the animals were kept at 5 degrees C without food. Five low molecular weight fractions obtained from Sprague Dawley rat sera were administered to analbuminemic rats kept under these conditions. The duration of survival was extended by the administration of two of the fractions. Several characteristics of one of these fractions coincided with those of uric acid, and body temperature of analbuminemic and Sprague Dawley rats increased within 5 min after uric acid administration.