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Biomedical subjects

R J Hogg

Publications and source records attributed to R J Hogg.

At least 19 recordsLinked to original sources

Acquired renal cystic disease in children prior to the start of dialysis.

This report describes the clinical course and serial sonographic findings in three children who developed acquired renal cystic disease (ARCD) prior to the institution of dialysis. The children were aged from 3 years to 13 years and their estimated glomerular filtration rate varied from 8 to 13 ml/min per 1.73 m2 when ARCD was diagnosed. Their primary renal disorders, which included hemolytic-uremic syndrome and focal segmental glomerulosclerosis, had been present for 1.5-11.5 years prior to the cysts being discovered. These patients show that ARCD may develop in children with chronic progressive renal parenchymal disease prior to the institution of specific therapy for end-stage renal disease.

Adolescent

Accelerated growth in short children with chronic renal failure treated with both strict dietary therapy and recombinant growth hormone.

In a 12-month study, nine boys, aged 4.8-15.6 years, with bone ages 4.6-13 years, with moderate to severe chronic renal failure and resultant growth failure were treated with daily recombinant human growth hormone (rhGH), in conjunction with a strict low-protein/low-phosphate diet supplemented with keto and amino forms of the essential amino acids, histidine and additional energy. Improved growth had previously been observed with this dietary management over that obtained with conventional treatment for chronic renal failure. Each child had been on this diet for at least 2 years before rhGH was commenced. Mean height velocity increased from 4.6 +/- 1.3 to 9.0 +/- 1.3 cm/year (P < 0.001) in the pre-pubertal group, and in the pubertal group from 5.4 +/- 1.4 to 10.4 +/- 1.8 cm/year (P < 0.01). The mean height velocity standard deviation scores (SDSs) increased from -1.2 +/- 0.6 to +2.3 +/- 0.9 (P < 0.001) in the pre-pubertal group and from -0.4 +/- 0.6 to +1.9 +/- 1.1 (P < 0.01) in the pubertal group. Mean height SDS for chronological age increased from -2.2 +/- 0.7 to -1.5 +/- 0.5 (P < 0.01) in the pre-pubertal group and from -1.9 +/- 0.7 to -1.3 +/- 0.9 in the pubertal group (P < 0.02). There was no significant deterioration in renal function or renal bone disease, and bone age did not advance more than chronological age over the 12-month period.

Adolescent

Trials and tribulations of multicenter studies. Lessons learned from the experiences of the Southwest Pediatric Nephrology Study Group (SPNSG).

Multicenter studies in Pediatric Nephrology have been acknowledged in recent years as an important mechanism for studying renal disease in children. The purpose of this review is to describe some of the experiences of the Southwest Pediatric Nephrology Study Group (SPNSG) in order to assist others in developing their own multicenter studies. The importance of protocol development, including adequate attention to study design, data management, and data analysis, is emphasized. Mechanisms for facilitating the frequency and productivity of study group meetings that are so essential for the success of multicenter studies, are described in some detail. The need and some of the methods for achieving ongoing collaboration within a climate of critical peer review are also discussed. Controversial issues such as authorship and the question of institutional credit for involvement in multicenter studies are discussed in brief. Finally, some of the features of the SPNSG that have permitted us to maintain a relatively high rate of productivity are described. The two most important of these, ongoing commitment to the group and willingness to collaborate across differences of opinion, are stressed throughout the review.

Authorship

Long term observations in a patient with pseudohypoaldosteronism.

This paper describes a patient with severe pseudohypoaldosteronism (PHA) for over 12 years. The patient presented at 10 days of age with a serum sodium of 118 mEq/l and potassium of 12 mEq/l. After failing to maintain normal fluid and electrolyte status with standard therapy, including maximal mineralocorticoid stimulation, he was given a special formula containing minimal potassium plus salt supplements which normalized his electrolyte status. However, when he was 4.5 years of age, an acute gastrointestinal illness led to severe volume depletion, hyperkalemia, and cardiopulmonary arrest. This resulted in significant neurological impairment. At 12.5 years of age, the patient continues to require massive sodium supplements and his diet contains less than 0.5 mEq/kg potassium daily; his height and weight are at the 95th percentile, thus demonstrating that normal growth may be achieved with strict dietary manipulation in a patient with persistent, severe PHA. Serial studies to further define the lesion in this patient have demonstrated: (1) normal binding of aldosterone to aldosterone binding globulin (5.1% bound); (2) normal mineralocorticoid "activity"; (2) suppressible renin and aldosterone levels; (4) increased prostaglandin excretion (3.15 micrograms/g creatinine); (5) lack of benefit of prostaglandin inhibition with indomethacin; (6) normal proximal tubule function (CNa + CH2O = 18.0 ml/100 ml glomerular filtration rate; (7) impaired distal tubule function (CH2O/CNa + CH2O = 79.8%) during water diuresis.

Aldosterone

A monoclonal antibody exhibiting reactivity with both X-hapten- and lactose-bearing glycolipids.

The tissue reactivity of a monoclonal antibody raised against human granulocytes, FMC 34, was shown to overlap with antibodies including FMC 10, which is directed against the X-hapten, and with T5A7, which is directed against lactose-bearing antigens. Reactivity of FMC 34 on sections of human cornea was more widespread than that of either FMC 10 or T5A7. High sensitivity fluorescence testing revealed that both FMC 34 and T5A7 reacted weakly with the T-lymphocyte cell line JM, unlike the CD15 antibody FMC 10. Competitive binding experiments showed that binding of FMC 34 to granulocytes was inhibited by preincubation with T5A7, while FMC 34 itself was able to inhibit the binding of both FMC 10 and T5A7. The binding of FMC 10 was not inhibited by preincubation of cells with T5A7. Saturation-binding analyses revealed that each human granulocyte could bind 17.5 x 10(5) molecyles of FMC 34, which was more than three times the number of molecules of FMC 10 which could be bound to each cell. The additional reactivity of FMC 34 appears to be due to its capacity to bind fucosylated and non-fucosylated lactose-bearing structures.

Antibodies, Monoclonal

Evaluation of long-term aggressive dietary management of chronic renal failure in children.

Ten children with chronic renal failure (CRF) were managed for 3 years using a strict low-protein and low-phosphorus diet supplemented by a mixture of the keto and amino forms of the essential amino acids and histidine (phase II). All of these children were previously managed for at least 2 years with a less rigorous diet of limited protein intake with no specific reduction of phosphorus (phase I). Energy, vitamin D, bicarbonate, phosphate binders and vitamin and mineral mixtures were added as required during both dietary phases. Data on dietary intake showed a significant fall in protein and phosphorus intake and a rise in calcium intake during phase II compared with phase I. Plasma calcium increased and phosphate fell, with an associated fall in intact parathyroid hormone levels. There was a marked improvement in urea creatinine ratios, which suggested an improved anabolic state. Cholesterol and triglyceride levels were improved. Height and weight velocity were increased, becoming significant after 3 years of phase II. Renal function deteriorated at a slower rate than predicted. The diet was well tolerated by the children, with fitness and school performance showing improvement. We conclude that long-term strict dietary management of children with CRF is feasible. Our data suggest an overall improvement in general health and an apparent reduction in the rate of deterioration of renal function.

Adolescent

Spontaneous remission of nephrotic syndrome in a patient with IgA nephropathy.

A patient with spontaneous remission of nephrotic syndrome (NS) associated with IgA nephropathy is described. The patient presented at the age of 8 years with asymptomatic proteinuria, and at the age of 11 years developed classical features of NS. A percutaneous renal biopsy showed mild mesangial prominence without significant hypercellularity, electron-dense deposits within the mesangium, and 3+ mesangial staining with IgA and IgG. NS resolved 6 weeks after onset without any form of therapy; absence of proteinuria persisted 6 months later. This report demonstrates clearly that patients with NS associated with IgA nephropathy may undergo spontaneous resolution of their proteinuria.

Biopsy

A clinicopathological study of 24 children with hemolytic uremic syndrome. A report of the Southwest Pediatric Nephrology Study Group.

This study reports the pattern of renal injury in 24 North American children with hemolytic uremic syndrome (HUS), and the extent of extrarenal involvement in 9 of these children examined at autopsy. Fifteen of the 24 children were studied during the first 16 days of hospitalization; their renal specimens demonstrated glomerular thrombotic microangiopathy (TMA) in 8 children, cortical necrosis in 1, and varying degrees of glomerular TMA and cortical necrosis in 6 children. Nine of the children were studied after 16 or more days of hospitalization; these patients had prominent renal arterial lesions. Of 9 children examined at autopsy, extrarenal microthrombi were identified in 8. In 4 children who died during the acute phase of the disease, hemorrhagic colonic necrosis (3 children) and pancreatic islet cell necrosis (2 children) were the principal extrarenal lesions encountered. Rare microthrombi were present in the brains of the 3 children who manifested seizures.

Aging

Blood pressure in schoolchildren measured under standardized conditions.

The blood pressures of 6346 children who were between the ages of seven and 17 years were measured under standardized conditions. Blood pressures were similar in prepubertal boys and girls. After puberty, the systolic blood pressures of the girls remained unchanged whereas those of the boys continued to rise. The difference between the fourth and fifth Korotkoff sounds was 2 mm at both the 50th and 95th percentiles. The systolic blood pressure was observed to fall over a 10-min period by 4-9 mmHg at the 50th percentile and 9-15 mmHg at the 95th percentile. Over the same period of time, the diastolic blood pressure was seen to fall by 2-3 mmHg and 3-6 mmHg at the 50th and 95th percentiles, respectively. There was no further fall in blood pressure after 10 min. The conditions and timing of measurement are important in blood-pressure evaluation and may explain the differences in blood pressure that have been reported for different populations.

Adolescent

IgA nephropathy: clinical features and natural history--a pediatric perspective.

This review describes the spectrum of clinical features observed in pediatric patients with IgA nephropathy (IgAN) in different parts of the world. The typical clinical presentation consists of an episode of macroscopic hematuria within 24 to 48 hours of an upper respiratory infection. However, many children who present with macroscopic hematuria are subjected to a battery of urologic studies before the appropriate procedure is performed. This sequence highlights the lack of awareness of IgAN among pediatricians, family practitioners, and urologists. The finding of microscopic hematuria or, less commonly, proteinuria, in a urinalysis carried out as part of a school screening program is the most frequent "presentation" of IgAN in Japanese children. However, it is possible that many children with IgAN expressed as microscopic hematuria and/or mild proteinuria remain undiagnosed in this country because routine urinalysis is not done and many pediatric nephrologists are reluctant to perform renal biopsies when such children are identified. It is now recognized that some patients with IgAN and nephrotic range proteinuria exhibit a state of steroid responsiveness. The renal biopsy in such patients often reveals "minimal change." Several recent studies have shown progressive deterioration in approximately 10% of all pediatric patients found to have IgAN and in 15% to 30% of the subset of patients with more severe histologic findings. Hypertension and proteinuria are observed frequently in patients who progress to chronic renal failure. It is proposed that multicenter collaborative studies be designed to evaluate proposed therapies for children with IgAN associated with proliferative glomerular lesions, particularly those in whom hypertension, proteinuria, and depressed glomerular filtration rate are found.

Child

Antigen expression during early human granulocyte development studied with immuno-electron microscopy.

Expression of the hapten fucosyl-N-acetyllactosamine was correlated with ultrastructural development in human granulocyte precursors using the monoclonal antibody FMC 10 with immunogold techniques. The antigen was detectable from the myeloblast/early neutrophilic promyelocyte stage onwards and was associated with striking development of the rough endoplasmic reticular system. In addition, low levels of labelling were seen on monocytes, eosinophils and some basophil precursors. Contraction and alignment of the cisternae of the rough endoplasmic reticulum during the promyelocyte stage of neutrophilic differentiation gave the appearance of a plasma cell. However, on closer examination it was apparent that true plasma cells did not react with this antibody.

Antibodies, Monoclonal

Renal transplantation after prolonged dwell peritoneal dialysis in children.

Thirty-three children received a total of 38 renal transplants (18 living related donor, and 20 cadaveric) after being on CAPD and/or CCPD (PDPD). Ten patients (12 transplants) were converted to hemodialysis pre-transplant in order to be free of the risk of peritonitis and off antibiotics, whereas 23 patients (26 transplants) were on PDPD at the time of transplant. The latter group of patients are described in greater detail. Within this group there was one episode of catheter colonization with Flavobacterium, and only three patients developed ascites post-transplant. Of the 26 transplants, catheters were removed at the time of transplant in the 13 LRD allografts but left in situ for a mean of 3.8 weeks in the 13 cadaveric transplant recipients. Peritoneal dialysis was required post-transplant in seven patients (two LRD recipients requiring a new catheter placement) without complications. Our policy of removing PD catheters at the time of transplant in LRD recipients and prior to hospital discharge in cadaveric transplant recipients has resulted in the avoidance of additional hospitalizations in 19 of the 26 transplants and avoided extra surgery in 11 of the 13 LRD transplants. We conclude that children who have been on PDPD are suitable candidates for renal transplantation and that the early removal of PD catheters, including removal at the time of transplantation in LRD recipients, is associated with a significant reduction in operative procedures for the patients.

Adolescent