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C L Jones

Publications and source records attributed to C L Jones.

At least 55 records · Page 3Linked to original sources

Acid phosphatase positional correlations in solid surface fungal cultivation: a fractal interpretation of biochemical differentiation.

Colour image analysis was used to measure the positional correlation between acid phosphatase intracellular concentration and hyphal cellular differentiation which leads to concentric circular zonal activity patterns. Acid phosphatase is strongly implicated in the biochemical control of hyphal branching, and exo-enzyme secretion, such as laccase in fungi, occurs predominately via the hyphal tips. Different concentrations of an organic dye were used to effect substrate induction of the enzyme response, which was shown to be statistically correlated according to a fractal power law (H approximately 0.39). A self-organized critical state for the molecular responsivity of dissipative enzyme expression is hypothesized as an efficient mechanism for hyphal adaptation, also accounting for the underlying biophysics of the observed pattern formations.

Acid Phosphatase↗

Familial hypomagnesaemia--hypercalciuria leading to end-stage renal failure.

Several disorders of hypomagnesaemia of hetary renal origin are now recognised. The cases of two sisters from a consanguineous marriage with the syndrome of renal magnesium wasting, hypercalciuria and nephrocalcinosis are presented. Pathological examination of the heterozygous parental kidneys revealed mild focal interstitial fibrosis. This condition is a previously unreported cause of end-stage renal failure in childhood, and this report suggests that transplantation from heterozygous parental donors can be successfully undertaken without recurrence of the syndrome.

Calcium↗

Comparison of height and weight in homosexual versus nonhomosexual male gender dysphorics.

The authors' clinical impression that homosexual gender-dysphoric males are physically smaller than nonhomosexual gender-dysphoric males was tested. Subjects were 176 homosexual and 246 nonhomosexual male outpatients, ages 16 to 65, who complained of discontent with their biological sex. Compared with the nonhomosexual male gender dysphorics, the homosexual gender dysphorics were shorter, lighter, and lighter in proportion to their height. The homosexual gender dysphorics were also shorter than men in the general population, whereas the nonhomosexual gender dysphorics were not. The smaller physiques of homosexual gender-dysphoric men may partly explain the clinical observation that these patients are somewhat more successful in passing as women.

Adolescent↗

Renal transplantation in very young children.

Results of renal transplantation in very young children with end-stage chronic renal failure have been poor compared with those in older children and adults. Consequently small children either may not be treated or may be placed on chronic dialysis programmes. Between 1988 and 1992, six children under the age of 5 years received seven renal transplants at the Royal Children's Hospital, Parkville, Victoria, Australia; five from live donors and two from cadaver donors. All children were treated with peritoneal dialysis before transplantation, and immunosuppressed with a standardized regimen of cyclosporine, azathioprine and prednisolone. An extraperitoneal incision was used, and the donor renal vessels were anastomosed to the lower abdominal aorta and inferior vena cava or the common iliac vein. All children received intensive monitoring and fluid replacement during the peri-operative period. Patient survival was 100%. One cadaver graft failed 1 week after transplantation because of irreversible acute rejection. This child subsequently received a successful second transplant. Two children developed postoperative urinary fistulae which were treated successfully by further operation. Current renal function in all children is excellent. The success of this programme has led us to review our attitude towards renal transplantation in this age group and to advocate live donor renal transplantation as the treatment of choice in very young children with end-stage chronic renal failure whenever possible.

Cadaver↗

Renal transplantation in young children.

AIMS: To review the outcome of renal transplantation in small children treated with triple immunosuppression at a single Australian centre. METHODS: The medical records of all children under the age of five years undergoing renal transplantation from 1988 were reviewed. The duration of follow-up was 30 months (range 18-36). RESULTS: Six children received seven renal allografts (five living-related [LR] and two cadaveric [CD]). They had a median age of 3.75 years (range 1.5-4.9) and weight of 11.6 kg (9.1-14.5) at the time of transplantation. All patients received an immunosuppressive regime involving cyclosporin A, azathioprine and prednisolone. There were no deaths. The only graft lost was a CD graft (severe acute rejection within one week of transplantation). Hypertension occurred in all recipients and usually required more than one antihypertensive drug for treatment. Renal function measured by serum median creatinine concentration (range) was 0.05 mmol/L (0.03-0.11) at three months (n = 6) and 0.10 mmol/L (0.07-0.22) at 30 months (n = 4). Growth estimated from median (range) height standard deviation scores was -1.97 (-1.36-(-4.04)) at three months (n = 6) and -1.90 (-1.74-2.50) at 30 months (n = 4). No patient was entirely weaned from prednisolone. Cyclosporin A side effects included hirsutism (five patients), gingival hyperplasia (six patients) and nephrotoxicity (three patients). CONCLUSIONS: Satisfactory patient and graft survival can be accomplished in this recipient age group. The results compare with other international experience and accumulating Australian experience. Hypertension and poor skeletal growth were consistent observations. The long-term outcome of renal function using triple immunosuppression remains to be determined.

Azathioprine↗

Who treats feet?

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Foot Diseases↗

Properties of classic protein kinase C in human small cell lung carcinoma NCI-H345 cells.

Bombesin-like peptides (BLPs) activate protein kinase C (PKC), which leads to proliferation in nonmalignant Swiss 3T3 cells. The purpose of this study was to determine if PKC expression in the classic human small cell lung carcinoma NCI-H345 cell line, which has an autocrine growth loop involving BLPs, exhibited unique properties that could result in malignant behavior. PKC activity and phorbol dibutyrate binding in NCI-H345 cells had properties similar to other reports. PKC activity in the cytosolic fraction increased to 100% as cells proliferated through lag, log, and plateau growth states. However, during the first 3 days after plating (lag growth state), 40-50% of the PKC activity was membrane associated, indicating a substantial portion in an activated form, possibly a result of BLP autocrine stimulation. NCI-H345 cells expressed the PKC isoenzymes alpha, beta, delta, sigma, and eta, but not gamma or epsilon, a pattern different from Swiss 3T3 cells or normal brain. further characterization of the Ca2+/phospholipid-dependent (classic) PKC isoenzymes, alpha and beta, showed that PKC beta was predominantly cytosolic (80%) as expected, but PKC alpha was primarily membrane associated (80-90%). Exposure of NCI-H345 cells to 200 nm phorbol 12-myristyl 13-acetate rapidly (within 2 min) decreased cytosolic PKC activity, with no change in the particulate activity, but did not alter [3H]-thymidine incorporation or subcellular distribution of PKC alpha or beta by Western blot. These results suggest altered PKC regulation in human small cell lung carcinoma NCI-H345 cells, which could contribute to their malignant behavior.

3T3 Cells↗

Triple immunosuppression with subsequent prednisolone withdrawal: 6 years' experience in paediatric renal allograft recipients.

Thirty-four children (< or = 15 years of age) with end-stage renal failure received 39 renal allografts between 1985 and 1991 and were treated with cyclosporin A (CyA), azathioprine and low-dose prednisolone (PNL). We aimed to withdraw PNL by 6 months after transplantation. Median duration of follow-up was 2 years 4 months (range 0.1 month to 6 years 4 months). There were no deaths. Crude graft survival for living-related grafts (n = 9) was 100%, although only 1 patient has been followed for > 2 years. For cadaveric grafts (n = 30), 1- and 5-year actuarial graft survivals were 90% and 79% respectively. At 12 months posttransplant, the median (range) glomerular filtration rate for all patients was 63 (19-109) ml/min per 1.73 m2 (n = 25) and at 5 years was 48 (17-64) ml/min per 1.73 m2 (n = 9). Complications observed included rejection episodes which occurred after discontinuation of PNL. Long-term (after 12 months), 28% of patients remain on PNL. Hypertension was present in more than 50% of patients. Severe CyA nephrotoxicity was not seen. Catch-up growth as determined by the change (delta) in mean height standard deviation score (Ht-SDS) was noted at 1 year [delta SDS/year = +0.60; P < 0.001 (n = 18)] and at 2 years [delta SDS/year = +0.27; P < 0.01 (n = 16)] in pre-pubertal patients. The median Ht-SDS at 2 years for pre-pubertal children was -0.71 SD and growth velocity did not improve thereafter.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗

Tamm-Horsfall protein: are serum levels a marker for urinary tract obstruction?

Tamm-Horsfall protein (THP) has been found in the renal interstitium in patients with obstructive uropathy. The aim of this study was to investigate whether serum concentrations of THP could serve as a screening test for urinary tract obstruction. The presence of THP in normal human serum was confirmed by sodium dodecyl sulphate polyacrylamide gel electrophoresis and Western blotting. A specific enzyme immunoassay was then used to measure the serum concentration of THP. Serum THP concentrations were estimated in a cross-sectional study of a group of 23 patients who had technetium-99m-diethylene-triaminepenta-acetic acid (DTPA) nuclear urinary excretion studies to define urinary tract obstruction, and in longitudinal studies in 2 patients who developed acute bilateral ureteric obstruction following operations for ureteric reimplantation. The subjects with DTPA-proven urinary tract obstruction had higher concentrations of serum THP (n = 10, median = 43.9 ng/ml, range 10.4-152.1 ng/ml) than those who did not have obstruction (n = 13, median = 9.6 ng/ml, range 1.26-61.9 ng/ml). While this difference was significant (P < 0.01, Mann-Whitney U test), 6 of the 10 patients with obstruction had serum THP concentrations within the range of those patients without obstruction. The patients who developed acute bilateral ureteric obstruction both had increases in serum THP concentrations with obstruction and decreases in serum THP concentrations following relief of obstruction. These changes paralleled those in serum creatinine. The studies indicate that urinary tract obstruction results in increases in serum THP concentrations but these changes are not sufficient in magnitude to allow screening of children for urinary tract obstruction.

Adolescent↗

The effect of growth hormone on growth and blood urea levels in children with chronic renal failure.

It has been claimed that low protein diets slow deterioration of chronic renal failure (CRF) by reducing renal solute load. The anabolic effect of recombinant human growth hormone (rhGH) also has potential to reduce renal solute load and thereby slow progression of renal failure. The aim of this study was to determine the effect of rhGH on growth, renal solute load and renal function in children with CRF. Seven prepubertal children, aged 2-14 years, with moderately severe CRF (creatinine clearance 7.7-23.4 mL/min per 1.73m2) were treated with daily subcutaneous rhGH, 1 U/kg per week for 10-12 months. As expected, mean height velocity standard deviation scores (SDS) increased, from -2.87 before treatment to +3.39 on rhGH, and mean height increased from -3.1 to -2.4 SDS. Serum urea concentrations decreased in most patients during the first month of growth hormone treatment from a mean of 20.0 +/- 7.7 mmol/L to 14.8 +/- 5.8 mmol/L (P = 0.006). The serum urea then returned to pretreatment levels over the next few months. In the 12 months before treatment with growth hormone, mean creatinine clearance decreased from 19.3 mL/min per 1.73 m2 to 16.7 mL/min per 1.73 m2. In the next 12 months on rhGH mean creatinine clearance decreased further to 13.5 mL/min per 1.73 m2. Therefore the rate of deterioration of renal function was unaffected during treatment with growth hormone. Initial treatment with rhGH is associated with decrease in serum urea concentrations in children with CRF, probably mediated by stimulation of anabolic incorporation of dietary nitrogen into body protein.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Developmentally timed expression of an embryonic growth phenotype in vascular smooth muscle cells.

Little is known about the phenotypic changes that occur in vascular smooth muscle cells (SMCs) as the developing aorta undergoes the transition from a loosely organized, highly replicative tissue to a morphologically mature, quiescent tissue. In the present study, we have characterized the in vivo pattern of SMC replication during intrauterine and neonatal aortic development in the rat and have cultured and assessed the in vitro growth properties of embryonic, fetal, and neonatal vascular SMCs. Embryonic SMCs, which exhibited a very high in vivo replication rate (75% to 80% per day), demonstrated a significant potential for self-driven replication, as assessed by the ability to proliferate under serum-deprived conditions. Several lines of evidence suggest that the autonomous growth of SMCs in the "embryonic growth phenotype" may be driven by a unique mechanism independent of known adult SMC mitogens: embryonic SMC replication was not associated with the detectable secretion of mitogenic activity capable of stimulating adult SMCs, and embryonic SMCs were mitogenically unresponsive to a variety of known adult SMC growth factors. The capacity for self-driven growth was lost by embryonic day 20, suggesting that important changes in gene expression and phenotype occur in developing SMCs between embryonic days 18 and 20. Taken together, the data describe a unique embryonic growth phenotype of vascular SMCs and suggest that the replication of aortic SMCs during intrauterine development is self driven, self regulated, and controlled by a developmental timing mechanism. The conversion of SMCs from the embryonic to the late fetal/adult growth phenotype will likely be found to be an important component of a developmental system controlling vascular morphogenesis.

Animals↗

Comparisons between oral and intraperitoneal 1,25-dihydroxyvitamin D3 therapy in children treated with peritoneal dialysis.

Recent studies in adults have suggested that parenteral 1,25-dihydroxyvitamin D3 (1,25[OH]2D3) may have advantages over oral therapy in the management of renal osteodystrophy. The purpose of this study was to determine whether there were clear differences between oral and IP 1,25(OH)2D3 treatments in children who did not pose a treatment problem. Seven children (5 males, 2 females, aged 1.8 to 16 years, median 4.8 years) undergoing peritoneal dialysis were initially treated with oral 1,25(OH)2D3 for a one month equilibration period They were randomly assigned to 3 months of either oral or intraperitoneal (IP) therapy with 1,25(OH)2D3 followed by 3-months-treatment using the alternative route. No significant differences in serum creatinine, phosphate, or parathyroid hormone concentrations were found between the different routes of administration in the patients. No significant differences in height standard deviation scores or renal osteodystrophy scores were found over the six-month study. Paired oral and IP pharmacokinetic studies were performed on these 7 patients and 2 other children who had been treated for at least 2 months using either oral or IP 1,25(OH)2D3. Serum was taken prior to one of the usual 1,25(OH)2D3 doses and 0.5, 1.5, 3, 6, and 24 h afterward. The highest measured concentrations of 1,25(OH)2D3 were found at 1.5 h for both oral and IP treatments (mean Cmax [SD]: oral 116 [23] pmol/l, IP 121 [24] pmol/l, p > 0.05). The AUC's for oral and IP therapy were similar (1701 [276] and 1645 [301] pmol/h/l, respectively). In the paired pharmacokinetic studies no significant differences were found between oral and IP treatments for the serum half life (27.4 [11.6] h and 19.2 [8.1] h, respectively) and total body clearance (15.3 [2.1] h and 18.4 [3.3] h, respectively) of 1,25(OH)2D3. In children who respond appropriately to oral 1,25(OH)2D3 there is no biological advantage to the use of IP 1,25(OH)2D3.

Administration, Oral↗

Primary right atrial chondrosarcoma in a dog.

Primary right atrial chondrosarcoma was diagnosed in a dog that had clinical signs of right-sided heart failure. The tumor was attached to the intra-atrial septum, dorsal to the septal leaf of the tricuspid valve. Tumor cells were not identified elsewhere.

Animals↗

Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children.

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulness of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.

Antibodies, Antineutrophil Cytoplasmic↗