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R W Baehler

Publications and source records attributed to R W Baehler.

At least 19 recordsLinked to original sources
PubMed1998

Epidemiology of IgA nephropathy in central and eastern Kentucky for the period 1975 through 1994. Central Kentucky Region of the Southeastern United States IgA Nephropathy DATABANK Project.

Population-based incidence data for IgA nephropathy (IgAN) are available for some countries but not for the United States. The purpose of this study was to determine the incidence of IgAN in central and eastern Kentucky for 5- and 10-yr periods between 1975 and 1994 and to examine differences among patient groups between those periods. The incidence of IgAN was 5.4 cases per one million population per year (MPPY) for period 1A (1975 through 1979), increasing to 12.4 cases per MPPY for period 2B (1990 through 1994) (P < 0.001). Males had a 2.7 times higher incidence than females for period 1 (1975 through 1984) and 2.2 times higher for period 2 (1985 through 1994). For period 1A, the incidence for Fayette County, which includes the city of Lexington, was lower than that of the rest of the study area (P=0.26), whereas for period 2 the incidence was higher for Fayette County (P=0.052). During period 1, the highest incidence of IgAN for any age and gender group was 24.3 cases per MPPY for males ages 30 through 39. For period 2, the incidence for males was similar for each decade between ages 20 and 59 (approximately 19 cases per MPPY). No African-American was diagnosed during period 1, but in period 2 incidences for blacks and whites were similar (10.7 and 10.2 cases per MPPY, respectively). For the last 5 yr of the study (1990 through 1994), the incidence of end-stage renal disease (ESRD) due to IgAN was 5.5 cases per MPPY: 8.4 for males and 2.7 for females. The incidence of IgAN in Kentucky for period 2B was still much lower than that in European studies, but the incidence of ESRD due to IgAN may be similar. Thus, IgAN may be as important a condition with respect to ESRD in Kentucky as it is in other regions of the world.

Adolescent↗
PubMed1988

Familial clustering and immunogenetic aspects of IgA nephropathy.

IgA nephropathy (IgAN) has been assumed to develop sporadically in individuals. We review the recent clinical and laboratory evidence that supports a genetic influence in the immunopathogenesis in some patients. These data include (1) families with multiple members with the disease not sharing a known nephrotoxic factor, (2) clustering of the birthplaces of ancestors of large pedigrees with multiple affected members, suggesting a "founder effect," (3) DNA alleles and protein phenotypes associated with IgAN (or a clinical subset) in some patient populations or that cosegregate with the disease in families with multiple affected members, and (4) immunologic abnormalities of patients shared by relatives with renal disease. However, the lack of understanding the fundamental pathogenetic mechanisms hinders progress in the pursuit of genetically controlled process in this disease. Furthermore, the diagnosis requires a renal biopsy, and no therapy has been proven effective. Therefore, asymptomatic family members with IgAN may forego the invasive diagnostic procedure necessary to establish the diagnosis, and instances of familial disease may not be investigated. Nonetheless, the increasing awareness of families with multiple affected members offers an opportunity to investigate the hypothesized genetic influence in the pathogenesis of IgAN.

Genetic Markers↗
PubMed1987

Morphologic and immunohistochemical observations in granulomatous glomerulonephritis.

Eight renal biopsies of Wegener's granulomatosis and other vasculitic syndromes with periglomerular granulomatous reactions (granulomatous glomerulonephritis) are studied. Controls consist of 57 biopsies of crescentic and focal necrotizing glomerulonephritis (of various diseases, excluding systemic lupus erythematosus). Both groups have in common the morphologic alterations of inflammatory tuft necrosis, fibrin exudation into the urinary space, crescent formation, and breaks of Bowman's capsule. In the study group, the unique feature of glomeruli with surrounding granulomatous reactions was the presence, within the urinary space, of an exudate of fibrin mixed with immune reactants (immunoglobulins and usually complement) in association with gaps of Bowman's capsule. This contrasts with an exudate of fibrin alone in glomeruli of the control group and other glomeruli of the study group. The study group also had a significantly greater incidence of immune deposits in Bowman's capsule (P less than 0.001). Giant cells may result from the interaction of macrophages of the granuloma with Bowman's capsule that is permeated with immune reactants.

Fluorescent Antibody Technique↗
PubMed1987

Regionalization in hereditary IgA nephropathy.

The genealogies of 80 patients with IgA nephropathy who were born in central or eastern Kentucky or whose parents were born in this region were researched. At a minimum, 48 of these patients were related to at least one other patient. On the basis of presence or absence of established kinships, the patients were divided into three groups. Twenty-nine patients in group 1 belonged to one large pedigree. Their birthplaces and those of their parents, grandparents, and great-grandparents clustered in the extreme eastern portion of the state. Seventeen other patients, group 2, were related to at least one other patient but not to a patient in group 1. Their birthplaces and those of their ancestors did not show significant clustering. With the exception of two siblings, the 34 patients of group 3 had no family members with IgA nephropathy. The birthplaces for these patients and ancestors were widely scattered. These data suggest that one or more genetically determined factors are important in the pathogenesis of IgA nephropathy in some patients. A founder effect, whereby a gene(s) conveying susceptibility to IgA nephropathy was carried into eastern Kentucky by one or more of the early settlers, would explain the geographic clustering of the birthplaces of the patients in group 1 and their ancestors. The characteristic immunopathology of IgA nephropathy may represent the histologic result of separate disease processes, one or more of which could be genetically influenced.

Consanguinity↗
PubMed1985

Focal sclerosis of hypertrophied glomeruli in solitary functioning kidneys of humans.

Morphometric data on glomerular size are presented on three patients with solitary functioning kidneys and one with bilateral oligomeganephronic hypoplasia. Renal biopsy of each of two patients with a congenitally absent kidney (unilateral renal agenesis) and a patient with oligomeganephronie, all with proteinuria and renal insufficiency, reveal increases of mean glomerular diameters of at least 1.75X and mean glomerular volumes greater than 5X. These dimensions, which are in the range of maximal increases recorded for man, are associated in all three biopsies with focal sclerosis of the hypertrophied glomeruli. By contrast, the functionally fully-compensated solitary kidney of a patient who lost function of the contralateral kidney from acquired disease, is characterized by the absence of focal glomerulosclerosis and by glomerular enlargement of significantly lesser degree (increased mean diameter 1.24X and mean volume less than 2X). These observations correlate glomerular injury with glomerular size and suggest that in the setting of reduced nephron numbers, nephron destruction via focal glomerulosclerosis may be initiated when compensatory glomerular hypertrophy has reached its limits.

Adult↗
PubMed1980

Studies on the pathogenesis of Bartter's syndrome.

There is no agreement concerning the primary pathogenetic event leading to Bartter's syndrome. Free water clearance and distal fractional chloride reabsorption were abnormally low in our patient with Bartter's syndrome. This series of investigations in this patient with Bartter's syndrome and hypomagnesemia was undertaken to determine if the defect in chloride transport in the ascending limb and the associated renal potassium wasting was specifically related to potassium depletion, increased prostaglandin production or magnesium depletion. Neither potassium repletion, indomethacin administration nor magnesium repletion had an effect on the defect in free water clearance or in distal fractional chloride reabsorption. However, magnesium infusion eliminated renal potassium wasting. These observations suggest that the proximate cause of Bartter's syndrome in this patient is a primary defect in the reabsorption of sodium chloride in the ascending limb and not renal potassium wasting. however, hypomagnesemia may contribute to the renal potassium wasting seen in this syndrome.

Adult↗
PubMed1980

Studies on the natural history of the norepinephrine model of acute renal failure in the dog.

Studies on the natural history of the norepinephrine (NE) model of acute renal failure (ARF) in the dog. Group I (n = 6) was infused with NE into the renal artery (0.75 microgram/kg/min x 80 min) and glomerular filtration rate (GFR) determined sequentially. 21 days post-NE infusion (I) the GFR of the I kidneys (20 + or - (SE) 5ml/min) remained less (p less than 0.001) than the pre-I control value (44 plus or minus 2.9). Group II (n = 7) was treated in an identical fashion except that a contralateral nephrectomy (CN) was done on day 7 and the animals were prepared for micropuncture on day 16. In the animals with residual nephron function at CN, the GFR increased (p less than 0.02) from 26 plus or minus 6.4 ml/min on day 7 to 38 plus or minus 6.6 ml/min on day 14. Considerable interanimal heterogeneity of single nephron GFR (SNGFR) was demonstrated and morphologic studies revealed a very patchy lesion. Therefore, the model is reversible, heterogeneity of SNGFR is present, and the residual functioning nephrons have the capacity to undergo an increase in GFR after CN.

Acute Kidney Injury↗
PubMed1980

Charcoal hemoperfusion in the therapy for methsuximide and phenytoin overdose.

Two patients suffered overdoses of anticonvulsant drugs. The first patient took methsuximide and the second, phenytoin. Because of profound CNS depression, both patients underwent hemoperfusion with a cellulose-activated charcoal hemoperfusion column. The primary metabolite of methsuximide, N-desmethylmethsuximide, primarily was responsible for the CNS depression in the first patient. The clearance of N-desmethylmethsuximide by the charcoal column was high, and clinical improvement became apparent during the hemoperfusion period. The clearance of phenytoin, on the other hand, was much lower than that of N-desmethylmethsuximide, and there was no apparent clinical improvement in that patient's condition.

Adult↗
PubMed1979

IgA antibasement membrane nephritis with pulmonary hemorrhage.

Goodpasture's syndrome has characteristically been described as being mediated by IgG antibodies. We have recently seen a 55-year-old man who developed renal failure and hemoptysis; a renal biopsy showed linear deposits of IgA and C3 involving glomerular and tubular basement membrane. Serologic tests for detecting (IgG) antiglomerular basement membrane antibodies were negative. Elution studies of kidney and lung showed the presence of an IgA antibasement membrane antibody only. The patient's serum contained IgA, but not IgG, antibodies reactive with glomerular and tubular basement membrane of normal human kidney and alveolar basement membrane of normal human lung. Attempts to transfer disease with the patient's IgA antibody to a monkey and to Lewis and Brown-Norway rats were unsuccessful. Immunoglobulin A antibasement membrane antibody must be considered in the design of immunoserologic procedures for the diagnosis of Goodpasture's syndrome.

Animals↗
PubMed1978

Failure of chronic sodium chloride loading to protect against norepinephrine-induced acute renal failure in dogs.

We previously have shown that chronic sodium chloride (NaCl) loading protects against HgCl2-induced acute renal failure (ARF) in dogs. To determine whether NaCl loading protects against an ischemic model of ARF, unilateral oliguric renal failure was produced by the infusion of norepinephrine (NE) into the renal artery of both saline-expanded (SE) and water-drinking (WD) dogs (n = 7). The renal renin content (30 U/g kidney) of SE dogs was suppressed (P less than 0.001) compared to that of WD dogs (132 +/- 18). Forty-eight hours after infusion of NE (1.5 microgram/kg per min X 100 min), inulin clearances from the infused kidney of SE (6 ml/min +/- 2) and WD dogs (7 +/- 2) did not differ; in both groups, respective clearances from the noninfused kidney (43 ml/min +/- 3) and (36 +/- 5) also did not differ from each other. The present fall in renal blood flow to the infused kidney 48 hours after NE in SE (44%) and WD dogs (38%) did not differ. Because of failure to demonstrate protection, a lower dose of NE (0.75 microgram/kg per min X 40 min) was infused into SE and WD animals (n = 6). Forty-eight hours after low dose NE, inulin clearances of the infused kidney of SE (17 ml/min +/- 5) and WD dogs (17 +/- 4) did not differ. Respective clearances in the noninfused kidney of SE (46 ml/min +/- 6) and WD dogs (35 +/- 4) did not differ. Therefore, despite suppression of renal renin content, NaCl loading failed to protect against this ischemic model of ARF. In conclusion, unlike HgCl2-induced ARF, it is unlikely that the renin angiotensin system contributes to the pathogenesis of this ischemic model of ARF.

Acute Kidney Injury↗
PubMed1978

A cystostomy cannula for dogs.

A practical, durable cystostomy cannula for the collection of urine samples for prolonged periods in awake dogs with intact bladder or hemibladder has been devised. The specifications, method of implantation, complications, and results of its use in nine dogs are described.

Abdominal Muscles↗
PubMed1976

Conservative management of chronic renal failure.

Failing kidneys can play havoc with other parts of the body. Specific treatment of these associated problems may help ward off uremia and preserve whatever renal function remains. Sodium levels may drop if too much water is mistakenly given to counteract kidney failure. Hyperkalemia can lead to cardiac arrest if potassium levels aren't reduced without delay. Acidosis also may reach life-threatening proportions, especially if diarrhea occurs. Almost all patients with chronic renal failure have a bleeding tendency and anemia, with the hematocrit dipping as low as 20 percent. Over half have decreased tolerance to carbohydrares, although severe hyperglycemia is rare. Disorders of calcium metabolism also are common, ranging from asymptomatic hypocalcemia to osteomalacia. The kidneys' impaired filtration ability should be kept in mind when drugs are prescribed. Dosages may need to be cut to avoid an adverse reaction.

Acidosis, Renal Tubular↗
PubMed1975

Plasma renin and aldosterone in an abdominal pregnancy with toxemia.

The first reported measurement of plasma renin and aldosterone in toxemia with an abdominal pregnancy is presented. In contrast to toxemia where plasma renin and aldosterone are either normal or low, extraordinarily high levels of both were found in this patient which returned to normal after delivery. The role of extrarenal renin in the hypertension of toxemia is discussed and the possibility raised that the elevated renin in this case was of placental origin.

Adult↗