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Biomedical subjects

M McVicar

Publications and source records attributed to M McVicar.

At least 19 recordsLinked to original sources

Transient urodynamic dysfunction of infancy: relationship to urinary tract infections and vesicoureteral reflux.

PURPOSE: Urinary tract infections and vesicoureteral reflux are more common in male than female infants. Since these problems can result from voiding dysfunction, we obtained a detailed history of voiding patterns and urodynamic testing in infants with urinary tract infections in the first year of life. MATERIALS AND METHODS: We evaluated 39 male and 22 female infants, including 40 with primary vesicoureteral reflux and 21 with no reflux or obstruction. RESULTS: Voiding abnormalities were noted in 97% of the male and 77% of the female infants, including high voiding detrusor pressure of greater than 40 cm. water in 92% of the male and 66% of the female infants, residual urine greater than 2 ml./kg. in 13% of the male and 23% of the female infants, and detrusor hyperreflexia with filling pressure greater than 40 cm. water in a third of the male infants. Voiding detrusor pressure was significantly higher in male than female infants and in male infants with grade IV to V reflux than those with lower grades of reflux or no reflux. Followup urodynamic testing in 15 infants with high voiding detrusor pressure revealed resolution of detrusor hyperreflexia and improvement in post-void residual in all and decreased voiding detrusor pressure in 14. CONCLUSIONS: We coined the term transient urodynamic dysfunction of infancy to describe this constellation of abnormalities, which predisposes infants to urinary tract infections and vesicoureteral reflux but improves spontaneously. The higher incidence of urinary tract infections and reflux in male infants may be related to higher intravesical pressures.

Female↗

Intraperitoneal production of erythropoietin with continuous ambulatory peritoneal dialysis.

Higher hematocrit and serum erythropoietin (EPO) levels have previously been shown in end-stage renal disease patients treated with continuous ambulatory peritoneal dialysis (CAPD) compared with hemodialysis. We investigated whether EPO was produced intraperitoneally in CAPD patients. EPO concentration was 3.5 +/- 0.3 mU/ml by radioimmunoassay in 26 samples of peritoneal dialysis effluent obtained from 15 CAPD patients. EPO was not detectable in the fresh unused dialysate. No correlation was observed between EPO levels in the serum and dialysis effluent. Peritoneal macrophages were isolated from the dialysis effluent of 9 CAPD patients after an overnight dwell. The culture supernatant obtained after 24 h of in vitro culture of a million cells yielded EPO of 3.5 +/- 0.3 mU/ml. Our study demonstrated that peritoneal macrophages from CAPD patients produce EPO on in vitro stimulation, and EPO is present in the dialysis effluent of CAPD patients.

Adolescent↗

Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases.

A 15-year-old girl with severe high renin hypertension caused by a juxtaglomerular cell tumor (JCT) was successfully treated with the calcium channel blocker nifedipine until surgical removal effected a permanent cure. This case was incorporated into a review of the 37 cases previously published. Comparison of the children and adolescents with the adult population showed that the features of JCT were similar in the two groups except for the average duration of symptoms prior to diagnosis (pediatric group 2.6 years vs. 6.0 years for the adult group). Analysis of all 38 cases demonstrated the following: 1. Teenagers constituted the largest single population with JCT (39%) and approximately two-thirds of the entire population were female. 2. Many patients failed to show persistent hypokalemia despite high plasma renin activity and secondary hyperaldosteronism. 3. Renal angiography was initially negative in more than half the cases. 4. Renal vein renin failed to show lateralization to the affected kidney in 52% of the cases. 5. Computerized tomography demonstrated a renal mass in all of the cases in which it was performed, even when other imaging studies were negative. 6. Calcium channel blockers may evolve as the preferred treatment for the high renin hypertension of JCT.

Adenocarcinoma↗

Diagnosis and imaging of the fetal and neonatal abdominal mass: an integrated approach.

Almost all of the renal causes of NAM and adrenal neuroblastoma, the most common nonrenal cause, can be categorized as cystic or solid by sonography. Evaluation with CT is usually the most appropriate next step for assessment of solid renal tumors lacking clinical features of RVT. Renal scintigraphy and diuretic renal scintigraphy offer valuable information about relative individual kidney GFR and excretory function, whether normal, partially, or completely obstructed. Many dilated urinary tracts, especially those diagnosed in utero, are found to resolve spontaneously when serial studies are performed. The informed pediatrician should play a major role in the selection of diagnostic procedures and determination of management strategy. Only he or she has the detailed clinical knowledge of the patient that can give appropriate direction to the consultants. A tentative algorithm for imaging studies in the evaluation of NAM is presented in Figure 1 and emphasizes the functionally more informative radionuclide studies 15,25,26 over the traditional radiocontrast studies.

Abdominal Neoplasms↗

Idiopathic arterial calcification of infancy: a case with prolonged survival.

We describe a patient with idiopathic arterial calcification of infancy and the following unusual features of the disease: (1) prolonged survival until age 11 years, (2) discordance between the extent of vascular calcification and clinical manifestations of arterial luminal occlusion, (3) a large area of myocardial calcification, (4) symptoms of cerebral-vascular insufficiency, and (5) spontaneous resolution of hypertension.

Calcinosis↗

Serum erythropoietin levels and hematocrit in end-stage renal disease: influence of the mode of dialysis.

Serum erythropoietin (Ep) levels were measured by radioimmunoassay in 70 patients with end-stage renal disease (ESRD) to evaluate the influence of the mode of dialysis on the relationship between serum Ep levels and the severity of anemia. Thirty-five patients were on hemodialysis (HD), seven were on intermittent peritoneal dialysis (IPD), and 28 were on continuous ambulatory peritoneal dialysis (CAPD). Compared to HD, CAPD patients had higher serum Ep (CAPD), 46.1 +/- 13.4 v HD, 16.9 +/- 2.2 mU/mL) and hematocrit (CAPD, 33.9 +/- 2.5 v HD, 24.8 +/- 1.4%; P less than 0.05). The Ep and Hct values for IPD patients were intermediate between the other two groups. Serum Ep levels were higher in CAPD patients in the first 4 weeks of initiation of CAPD (144 +/- 35 mU/mL, n = 6) than later (39 +/- 6.4 mU/mL, n = 24). A significant fluctuation in serum Ep and Hct values was noted in patients on all three modes of dialysis, when multiple samples were obtained at different time intervals. There was a weak correlation between serum Ep and Hct in the three groups of dialysis patients; r = 0.36, P less than 0.005. The data suggest that CAPD provides a better biochemical milieu for Ep production and responsiveness than HD treatment of ESRD.

Adolescent↗

Pathogenesis of the anemia of chronic renal failure: the role of erythropoietin.

In summary, the anemia of CRF results from several interactive processes, chief among these inadequate Ep production relative to the degree of anemia. The anemia of renal failure can be regarded as an endocrine deficiency state, which is corrected by the specific replacement therapy. The advances of molecular biology have provided a biosynthetic Ep, a potent tool for correction and prevention of the anemia of renal failure. However, new problems often arise as new treatments become available. The rapid improvement in hematocrit and the resultant lowering in plasma volume may affect dialysis clearances in hemodialysis patients. Since the well-being and appetite of the patients improves as the hematocrit rises, newer methods to increase the weekly dialysis clearances will be needed to prevent the complication of underdialysis in these patients.

Anemia↗

Renal-hepatic-pancreatic dysplasia: a syndrome reconsidered.

Five infants, three dying neonatally and two later in the first year of life, had renal, hepatic, and pancreatic dysplasia, a combination of abnormalities first described by Ivemark et al [1959]. The renal malformation consisted of cystic dysplasia, with abnormally differentiated ducts, deficient nephron differentiation, and glomerular cysts. The hepatic abnormality consisted of enlarged portal areas containing numerous elongated biliary "profiles," with a tendency to perilobular fibrosis. Serial liver biopsies in one child with cholestasis from birth showed a progression from bile duct paucity at 1 1/2 wk to typical biliary "dysgenesis" at 7 mo. Four of the five children had intrahepatic ductal dilatation, diagnosed ante mortem in the two older children as Caroli disease. The pancreatic abnormality consisted of fibrosis and cysts, with a diminution of parenchymal tissue. The clinical and functional reflection of these abnormalities in the two children surviving the newborn period included renal insufficiency, chronic jaundice, and insulin-dependent diabetes mellitus. Similar renal, hepatic, and pancreatic abnormalities occur in other syndromes, including trisomy 9, Meckel syndrome, Jeune, Saldino-Noonan, and Elejalde types of chondrodysplasia, and glutaric aciduria II. After exclusion of identifiable syndromes, the remaining cases of renal-hepatic-pancreatic dysplasia do not necessarily constitute a homogeneous group.

Abnormalities, Multiple↗

Role of erythropoietin in the reversal of anemia of renal failure with continuous ambulatory peritoneal dialysis.

We report serum erythropoietin levels in a patient who showed significant improvement in hematocrit when switched from hemodialysis to continuous ambulatory peritoneal dialysis (CAPD) treatment. This 22-year-old woman had severe anemia and low serum immunoreactive erythropoietin levels (8.0 +/- 1.2 mU/ml; n = 5) while on hemodialysis for 7 years. Serum erythropoietin levels were 80 and 177 mU/ml, 2 and 3 weeks, respectively, after starting CAPD. This was followed by an increase in reticulocyte count from 3.9 to 22% and hematocrit from 19 to 48%. The serum erythropoietin concentration obtained on CAPD treatment (62.7 +/- 15.2 mU/ml; n = 9) was significantly higher than that obtained on hemodialysis. Our findings indicate that CAPD facilitates increased erythropoietin production compared to hemodialysis and that the anemia of uremia may reverse if sufficient erythropoietin is available.

Adult↗

Upper- vs lower-limb systolic blood pressure in full-term normal newborns.

The difference between upper- vs lower-limb systolic blood pressure (BP) was investigated in 100 normal full-term newborns younger than 24 hours of age by the oscillometric method. The mean systolic BP of both upper limbs was 72.3 +/- 7.6 mm Hg (mean +/- SD). The mean +/- SD systolic BP of both lower limbs was 71.3 +/- 8.2 mm Hg. Systolic BP in the upper limb was greater than that in the lower limb in 66% of newborns by as much as 20 mm Hg (mean +/- SD, 3.5 +/- 3.1). Systolic upper-limb BP was lower than systolic lower-limb BP in 28% of newborns by as much as 21 mm Hg (mean +/- SD, 5.1 +/- 5.1). Systolic BP did not correlate with birth weight. Follow-up evaluation of BP in 25 of the infants up to 3 years of age revealed higher systolic BP in the lower extremities in 24 of the 25 infants. We conclude that (1) it is normal to have a higher systolic BP in either the upper limb or the lower limb in newborns younger than 24 hours of age, and (2) there is no correlation of systolic BP with birth weight in full-term normal neonates younger than 24 hours of age.

Arm↗

Serum immunoreactive erythropoietin levels in patients with polycystic kidney disease as compared with other hemodialysis patients.

Serum erythropoietin levels were randomly collected and measured by a sensitive radioimmunoassay in a hemodialysis population. For analysis, the patients were divided into two groups: those with polycystic kidney disease and those with other kidney diseases. In 12 polycystic kidney disease patients, serum erythropoietin was 22.6 +/- 2.4 mU/ml, hematocrit 29.7 +/- 1.0%, and absolute reticulocyte count 17.0 +/- 4.1 X 10(4)/microliters. In 24 other kidney disease patients, serum erythropoietin was 12.4 +/- 0.7 mU/ml, hematocrit 21.2 +/- 0.8%, and reticulocyte count 7.5 +/- 1.5 X 10(4)/microliters. Serum erythropoietin was 18.5 +/- 0.7 mU/ml in normal controls. Polycystic kidney disease patients manifested higher hematocrit, reticulocyte counts, and serum erythropoietin levels when compared to other kidney disease patients (p less than 0.01). The data suggest (1) an inappropriately low serum erythropoietin level for the severity of anemia in uremic hemodialysis patients and (2) that greater availability of erythropoietin results in more effective erythropoiesis, even in the uremic environment.

Adolescent↗

Experimental focal segmental glomerulosclerosis: correlation with protein excretion, glomerular filtration rate, and renal plasma flow.

A rat model of focal segmental glomerulosclerosis (FSGS) produced by repeated injections of aminonucleoside (AMN) of puromycin was used to evaluate the relative roles of hemodynamic alterations and AMN-induced glomerular visceral epithelial cell injury in the development of FSGS. Twenty rats received three intraperitoneal injections of AMN on days 1, 21, and 28 and developed significant proteinuria. On day 50, 14 rats (group 1) underwent selective left renal perfusion with AMN and six rats (group 2) received left renal perfusion with saline. At sacrifice on day 70 or 110, group 2 rats had similar values in left and right kidneys for glomerular filtration rate (GFR), effective renal plasma flow (ERPF), and the amount of FSGS (13.1 +/- 5.6% in left and 12.9 +/- 7.8% in right). In contrast, group 1 rats manifested a significantly higher amount of FSGS in right kidneys as compared to left kidneys (3.1 +/- 1.3% in left and 6.3 +/- 2.0% in right, as well as significantly diminished GFR and ERPF in left as compared to right kidneys. A higher degree of FSGS was seen in kidneys with a higher GFR and ERPF. A positive correlation was observed between the mean 24-h protein excretion of the rats and the percentage of FSGS in left and right kidneys (r = 0.66, p less than 0.01).

Animals↗

Jejunal transport in experimental nephrotic syndrome.

In vivo jejunal transport of amino acids, monosaccharides, sodium, and electrolytes were studied in rats made nephrotic with puromycin aminonucleoside (PAN) and in pair-fed controls. Studies were performed 14 days after a single intravenous dose of PAN when rats were no longer edematous, but were still hypoproteinemic. There was decreased absorption of glucose, 3-0-methyl glucose, glycine, phenylalanine, histidine, water, and sodium in the nephrotic animals but transport of fructose, lysine and potassium was similar in the nephrotic and control animals. Enzyme kinetic studies for glucose transport showed a mixed type of inhibition affecting both Vm and Km. The jejunal mucosa of nephrotic and control rats had similar ATP content and enzyme activity for lactase, sucrase, maltase and (Na-K)-ATPase and the ratios of RNA to DNA were similar in the nephrotic and control rats. No abnormality of the jejunum was detected by light or electron microscopy. The data suggest that the impairment of absorption is a result of decreased activity of jejunal membrane carrier mechanisms. The altered transport may be secondary to effects related to the metabolic consequences of nephrotic syndrome and does not appear to be related to acute purine aminonucleoside toxicity, edema or malnutrition.

3-O-Methylglucose↗

Nephrotic syndrome and multiple tubular defects in children: an early sign of focal segmental glomerulosclerosis.

The nephrotic syndrome is rarely associated with renal tubular defects, and the combination has been reported only in association with advanced renal insufficiency. We report here five children with nephrotic syndrome and multiple tubular defects which evolved when glomular filtration rate ranged between 56 and 90 ml/minute/1.73 m2. The tubular defects were first noted at 3, 4, 4, 7, and 22 months after the onset of the nephrotic syndrome, and renal glycosuria was the first sign in all five children. Glycosuria was intermittent in three patients, constant in two, and ceased with loss of kidney function. Four patients had hyperaminoaciduria and renal tubular acidosis (two of four tested had distal renal tubular acidosis). Three patients had decreased tubular reabsorption of phosphorus and defective maximum concentrating capacity. All five had focal segmental glomerulosclerosis proven by renal biopsy. Over a follow-up period of seven years, all of the children have developed advanced renal insufficiency, four of the five have required dialysis or transplantation within 21 to 72 months after onset, and one has stabilized renal function at 35 ml/minute/1.73 m2. The one patient receiving a kidney transplant has had recurrence of focal segmental glomerulosclerosis in the transplanted kidney and became nephrotic with three subsequent transplants. Our experience suggests that the nephrotic syndrome associated with tubular defects in children forms a subgroup of focal segmental glomerulosclerosis, with rapid progression to renal insufficiency and the potential for recurrence of the lesion in the transplanted kidney.

Acidosis, Renal Tubular↗