PubMed Health⌕ Search

Biomedical subjects

K J Sheth

Publications and source records attributed to K J Sheth.

At least 37 records · Page 2Linked to original sources
PubMed1983

Stenosing ureteritis in Henoch-Schönlein purpura.

We report on a boy with Henoch-Schönlein purpura in whom flank pain and gross hematuria developed during the early phase of the disease. Urologic investigations revealed hydronephrosis and ureteral stenosis. Pyeloplasty was done to relieve ureteropelvic junction obstruction. Severe hemorrhagic ureteritis and vasculitis were noted on the ureteral biopsy. Recovery was slow and residual hydronephrosis persisted. Although abdominal pain usually accompanies Henoch-Schönlein purpura, colicky flank pain associated with hematuria should alert the physician to the presence of ureteritis. Recognition and early surgical treatment of this urologic complication of Henoch-Schönlein purpura may prevent a potentially serious outcome.

Child↗
PubMed1983

Early renal failure in Fabry's disease.

Two young males were observed with renal failure from Fabry's disease. These two patients had progressive decrease of renal function and required hemodialysis at ages 16 and 24. Diagnosis was confirmed by low plasma galactosidase levels and by skin and kidney biopsy. Each has had a successful kidney transplant with good graft function, the 16-year-old patient for five years and the 24-year-old for two years. Although the renal impairment usually does not appear until the fourth or fifth decade of life. Fabry's disease should be considered in the differential diagnosis of renal failure in young males.

Adolescent↗
PubMed1982

Electrocardiograms in Fabry's disease.

We studied the ECGs of 47 kindred members from three families with Fabry's disease. The control group (19) with normal plasma alpha-galactosidase levels had normal ECGs. Of the 12 affected males (mean age 15 years, plasma alpha-galactosidase levels less than 10% of normals), ten had ECG evidence of LVH, and five had ST-T wave changes. The conduction time of the cardiac impulse was accelerated (decreased PR index) in one male. Of the 16 heterozygous females (mean age 19 years, plasma alpha-galactosidase level and alpha/beta galactosidase ratio in the carrier range) none had ECG evidence of LVH, but six had conduction abnormalities--decreased PR index (four) and delayed conduction (two). The ECG abnormalities were uniformly distributed in different age groups.

Adolescent↗
PubMed1981

Heterozygote detection in Fabry disease utilizing multiple enzyme activities.

In Fabry disease, as in other X-linked traits, identification of all heterozygotes is difficult. Reduced plasma alpha-galactosidase activities will correctly identify 60-70% of the carriers. The identification rate improves when an alpha/beta-galactosidase activity enzyme ratio is used. We measured alpha-galactosidase activity in reference to several other enzyme activities, beta-galactosidase, beta-hexosaminidase, and alpha-fucosidase in plasma and leukocytes from 22 suspected and 9 obligate carriers from 4 kindreds of Fabry disease patients. Utilizing such ratios or various combinations of ratios in plasma we have correctly identified the carrier state in 91% of heterozygotes. Leukocyte alpha/beta-galactosidase identified one more female than leukocyte alpha-galactosidase activities alone. We recommend the use of such multiple biochemical tests to identify carriers of Fabry disease.

Adolescent↗
PubMed1981

Gastrointestinal structure and function in Fabry's disease.

We evaluated gastrointestinal structure and function in 13 hemizygous males and 17 heterozygous females, five to 67 years old, from four kindreds with Fabry's disease. Gastrointestinal symptoms, noted in 62% (8/13) of the males and 29% (5/17) of the females, were present prior to the diagnosis of Fabry's disease in five patients; were discovered at the time of study in six patients and were associated with multiple other symptoms in two patients. Serum protein, albumin, folate, Vitamin B12, calcium, phosphorous, cholesterol and iron were normal in all 30 patients. Xylose absorption was normal in 2/2 males and 13/13 females studied. HLA B8 antigen was present in none of the males and 2/17 females. Peroral duodenal (one male), jejunal (six males, two females) and rectal (one male) biopsies on light microscopy demonstrated a normal villous pattern and luxol-fast blue positive "foamy" cell deposits in all males, while no deposits were visualized in the females. In all males and females studied, electron microscopic examination showed electron dense, intralysosomal "zebra-like" (0.5-0.75 micrometer.) bodies in the vascular endothelial and perithelial cells and in the cytoplasm of the small unmyelinated neurons, and perineurial cells. Despite the frequency of gastrointestinal symptoms, both malabsorption and celiac disease were absent.

Adolescent↗
PubMed1980

Peripheral nerve conduction in Fabry disease.

Peripheral nerve conduction was studied in 34 members, aged 3 to 54 years, from three separate kindreds with Fabry disease. Nerve conduction velocity was prolonged in one-third (11 of 34) of the study group. Of the 9 control family members with normal plasma alpha-galactosidase levels, 8 had normal velocity. A 6-year-old girl with normal plasma and leukocyte alpha-galactosidase levels and absent corneal deposits had slightly lower than normal nerve conduction velocity. One of the 3 obligate female carriers and 1 of 10 suspected carriers of Fabry disease had prolonged conduction velocity but normal distal latency. On the other hand, 8 of 12 males affected with Fabry disease demonstrated slow nerve conduction velocity while 4 of the 8 had prolonged distal latency. Frequently, only one of the two nerves studied showed abnormalities. These abnormalities were not related to patient age. Interfamilial variations in the abnormalities were present.

Adolescent↗
PubMed1979

Hemagglutination-inhibiting antibodies in vaccinated children with renal disease.

One year after immunization with a single 0.5-mL dose of influenza virus vaccine, serum samples from 30 children with renal diseases were tested for serum hemagglutination-inhibiting (HI) antibody titers to A/New Jersey/76, A/Victoria/75, and A/USSR/77. Eleven unvaccinated children with renal diseases formed a comparison group. In contrast to the comparison group (0/11), 53% (16/30) of the vaccinated group had a protective level of serum HI titers (greater than or equal 1:40) against A/New Jersey. A protective level of serum HI titer against A/Victoria was noted in 83% (25/30) of the vaccinated group, while 54% (6/11) of the unvaccinated group had similar HI titers. None had a detectable HI titer against A/USSR. A minor common cold-like illness occurred in seven of the 30 vaccinated children; one of these had exacerbation of nephrotic syndrome. The data suggest a good protection against influenza one year after vaccination in children with renal disease.

Age Factors↗
PubMed1979

Fabry's disease in a black kindred.

In a 16-member black kindred with Fabry's disease, four hemizygous males had plasma alpha-galactosidase levels less than 6% and seven heterozygous females had plasma alpha-galactosidase levels between 10% and 50% of normal. A 16-year-old index male had hypertension with left ventricular hypertrophy, abnormal renal function, tortuous retinal veins, "myelin" inclusions in bone marrow macrophages, and intraepithelial inclusion bodies in the kidney. Scrotal angiectasia developed a year after diagnosis. The three other affected males had left ventricular hypertrophy and retinal vein tortuosity. Of the seven carrier females, five had frequent headaches, four had retinal vessel changes, three had proteinuria with normal renal function, and two had bundle-branch blocks on ECGs. There was no deuteranomalopia in this family, although the inheritance pattern of the Fabry gene is X-linked recessive.

Adolescent↗
PubMed1979

Concurrent linear scleroderma and systemic lupus erythematosus: a report of two cases.

Two patients with linear scleroderma (en coup de sabre) developed systemic lupus erythematosus (SLE). This association has been well documented in only one previous case. The presence of high titer antibodies to ribonucleoprotein (RNP) initially led to the diagnosis of the mixed connective tissue disease. Development of more serious clinical involvement and antibodies to Sm (case 1) or native deoxyribonucleic acid (nDNA) (case 2) helped establish a diagnosis of SLE. Use of these studies in the differential diagnosis of systemic rheumatic diseases is disucssed briefly. The presence of anti-RNAP antibodies in patients with localized scleroderma may herald a more serious rheumatic disease.

Adult↗
PubMed1978

Influenza virus immunization. Antibody response and adverse effects in children with renal disease.

Before and four weeks after immunization with a single 0.5-ml dose of influenza virus vaccine, sera from 36 children with renal diseases were tested for serum hemagglutinating-inhibiting antibody (HAI) titers to A/New Jersey/76, A/Victoria/75, and A/Port Chalmers/73. Before immunization, 1:40 HI antibodies to A/New Jersey were noted in one child only, to A/Victoria in ten children (27%), and to A/Port Chalmers in 25/34 children (68%). Serum HAI titers increased fourfold or more (P less than .01) in 31/36 children (86%) after immunization. Neither the type of the renal disease nor therapy with prednisone had any effect on the rise of serum HAI titers (P less than .05). Of the seven children with preimmunization proteinuria, four had a transient rise in protein levels following immunization. None required an increased prednisone dose for exacerbation of nephrotic syndrome. Children with chronic renal problems should be protected against influenza.

Adolescent↗
PubMed1978

Polydipsia, polyuria, and hypertension associated with renin-secreting Wilms tumor.

A 16-month-old black male infant had unusual thirst, polyuria, hyponatremia, and hypertension. His polyuria was unresponsive to vasopressin therapy, and his high blood pressure was not effectively controlled by antihypertensive drugs. Radiographic examinations revealed an occult Wilms tumor in the right kidney. After removal of the tumor, the signs and symptoms were relieved. The tumor had a renin activity about 280 times that of the adjacent renal cortex, and many intracytoplasmic secretory granules were found on electron microscopy. The pathogenesis of these clinical manifestations appears to be mediated through the physiologic pathways of renin-angiotensin II and renin-aldosterone.

Humans↗
PubMed1978

Scintigraphy in pediatric urinary tract lymphoma.

Excretory urography is the standard examination used to evaluate structural abnormalities of the urinary tract in children. However, renal imaging offers advantages over excretory urography in the functional evaluation and in certain morphologic patterns of lymphoma.

Child↗
PubMed1978

Urinary acidic hydrolases in renal diseases in children.

Acidic hydrolases were assayed in urines of 19 normal children, 33 children with idiopathic nephrotic syndrome of childhood (INS), 21 children with glomerulonephritides (GN) and 7 children with persistent proteinuria/hematuria, and in plasma of 10 children each with INS or GN. Both plasma and urinary acidic hydrolases were studied in intermittent orthostatic proteinuria. Cbeta-galactosidase and Cbeta-N-hexosaminidase were done in normals and children with active renal disease. Significantly (P less than 0.01) elevated urinary acidic hydrolases excretion in active renal diseases, both in INS and GN, returned to a normal range with regression of the diseases. Increased postural proteinuria was associated with normal urinary acidic hydrolases. Both beta-galactosidase and beta-N-hexosaminidase excretion was higher than similar mol wt proteins in normals and increased further in active renal diseases. The data suggests that increased urinary acidic hydrolases is related to the activity of the renal disease, and not to urinary WBC, hematuria or proteinuria. The likely source of urinary acidic hydrolases thus appears to be the injured renal parenchyma itself.

Arylsulfatases↗