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Biomedical subjects

W R Griswold

Publications and source records attributed to W R Griswold.

At least 19 recordsLinked to original sources

End-stage renal disease and primary hypogonadism associated with a 46,XX karyotype.

OBJECTIVE: To determine the cause of absent sexual development in a 17-year-old girl with end-stage renal disease. DESIGN: Case study. PARTICIPANT: Seventeen-year-old girl with end-stage renal failure. INTERVENTIONS: None. MEASUREMENTS/MAIN RESULTS: The patient had phenotypically normal external female genitalia, müllerian duct hypoplasia, and no ovaries. Her serum gonadotropin levels were in the castrate range at baseline and after gonadotropin-releasing hormone stimulation. Her karyotype, in lymphocytes and cultured fibroblasts, was 46,XX. Analysis of genomic DNA, following polymerase chain reaction-amplication with oligonucleotide primers corresponding to the Y-encoded zinc finger protein ZFY and the testis-determining SRY gene, showed Y chromosome material in a male control but none in the patient. CONCLUSIONS: The results suggest a diagnosis of Frasier syndrome, a disorder characterized by true gonadal dysgenesis and end-stage renal disease occurring in normal phenotypic girls. Although previously reported only in individuals with a 46,XX karyotype, our studies indicate that Frasier syndrome may also occur in 46,XX girls. Delayed puberty is not uncommon in renal failure. This case illustrates the importance of measuring gonadotropin levels in teenage girls with delayed puberty and renal failure, particularly if the origin of the renal disease is obscure.

Adolescent

Symptomatic cholelithiasis in pediatric renal transplant recipients.

We report two cases of symptomatic cholelithiasis in pediatric renal transplant recipients immunosuppressed with cyclosporine (CsA), prednisone and azathioprine. CsA was present in the bile and in the cholesterol gallstones of one patient. The diagnosis of cholelithiasis was established in both cases by abdominal ultrasound examination.

Child

Peritoneal dialysis for acute renal failure in children.

Fifty infants and children with acute renal failure were treated with acute peritoneal dialysis between 1987 and 1990. The patients were dialyzed using either a catheter introduced percutaneously over a guide-wire (n = 40) or a Tenckhoff catheter (n = 10). The cause of the acute renal failure was primary renal disease in 17 children, cardiac disease in 19, and trauma/sepsis in 14. Peritoneal dialysis succeeded in controlling metabolic abnormalities, improving fluid balance, and relieving the complications of uremia. The procedure had few major complications. Overall mortality was 50%, reflecting the serious nature of the underlying diseases. We conclude that acute peritoneal dialysis is a safe and effective treatment in most pediatric patients with acute renal failure. Our series of patients treated with acute peritoneal dialysis serves as a basis of comparison for the evaluation of new modalities of therapy in childhood acute renal failure.

Acute Kidney Injury

Theoretical analysis of the accuracy of calibrated immunoassays for measuring antibody concentration.

The accuracy of calibrated immunoassays for measuring antibody concentration was analysed from a theoretical perspective. The study shows that there are theoretical limits on the accuracy of antibody immunoassays, which are determined by the affinity of the standard and unknown antibodies and the conditions chosen for the assay. As a result of these limits, calibration of an immunoassay with a standard antibody does not automatically ensure accurate measurements of antibody concentration. Extremely large errors may develop in affinity-dependent assays when the affinities of the standard and unknown antibodies are different. Assay conditions and the affinity of the standardizing antibody must be chosen carefully to measure antibody concentration accurately.

Antibodies

Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents.

In children, steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) is frequently a progressive condition resulting in end-stage renal disease. There have been no reports of effective treatment for this condition. For the past several years, the Pediatric Nephrology services at the University of California, San Diego and Stanford University Schools of Medicine have treated these patients with a protocol involving infusions of high doses of methylprednisolone, often in combination with oral alkylating agents. Twenty-three children have been treated in this manner with a follow-up of 46 +/- 5 months. Twelve of these children are in complete remission. Six have minimal to moderate proteinuria. Four children remain nephrotic. Each of these children has a normal glomerular filtration rate. One child developed chronic renal failure and subsequently died while on dialysis. These results appear significantly better than previous series of children with FSGS. A controlled, multi-center trial of this protocol has been proposed.

Adolescent

Trimethoprim/sulfamethoxazole-induced renal tubular acidosis.

A child was evaluated for growth failure at the University of California, San Diego. On two occasions the patient had renal bicarbonate wasting, acidosis, and growth failure associated with trimethoprim/sulfamethoxazole (TMP/SMZ) administration. On both occasions, the acidosis resolved and the growth rate normalized following a period without receiving TMP/SMZ. Renal tubular acidosis and growth failure may occur as a result of TMP/SMZ therapy in children.

Acidosis, Renal Tubular

Severe bilateral renal disease: correlation of antenatal and autopsy findings.

We report 20 infants with severe bilateral renal disease examined by prenatal ultrasound and by autopsy. In 17, the prenatal and pathologic diagnoses correlated well. Although the prenatal and autopsy findings differed in the three remaining cases, the autopsy confirmed the presence of severe bilateral renal abnormalities. All 20 pregnancies were complicated by oligohydramnios, which was severe in 60 per cent. Most of these fetuses had malformation of other organ systems. This series supports the utility of prenatal ultrasound examinations, but emphasizes the need for postnatal evaluation of congenital renal disease including pathologic examination of tissue when possible for correct classification and genetic counselling.

Female

Follow-up of infants with obstructive uropathy detected in utero and treated surgically postnatally.

Thirty-four infants with obstructive uropathy detected by prenatal ultrasonography at 34 +/- 0.56 weeks (SE) had surgery during the first 2 years of life. The growth and renal function of the 25 children after being followed for over 1 year are described. Twelve children had unilateral disease, and 13 had bilateral disease. All 12 children with unilateral disease grew at or above the fifth percentile and had normal renal function (glomerular filtration rate, 101 +/- 7 mL/min/1.73 m2). Thirteen children with bilateral disease were followed for 26.1 +/- 3.4 months. Growth was good: 10 of the 13 grew at or above the fifth percentile. The serum creatinine was 0.7 +/- 0.2 mg/dL, and the glomerular filtration rate was 91 +/- 10 mL/min/1.73 m2. One child required chronic dialysis. The prenatal diagnosis of urinary tract anomalies followed by early intervention may improve the long-term outcome of children with obstructive uropathy.

Child, Preschool

Functional affinity of antibody to the Haemophilus influenzae type b polysaccharide.

The functional affinity, or avidity, of antibody to the capsular polysaccharide of Haemophilus influenzae (Hib-PS) was measured in serum samples from 12 adult male subjects before and after immunization with Hib-PS vaccine. Mean avidity increased from 0.74 nM-1 to 1.5 nM-1 after immunization (P less than .05, paired Student's t test). The Bureau of Biologics reference antiserum had an avidity constant of 12 nM-1, which was the highest of all 25 serum samples studied.

Antibodies, Bacterial

Theoretical analysis of the sensitivity of the solid phase antibody assay (ELISA).

The sensitivity of the solid phase ELISA antibody assay was analyzed from a theoretical perspective. Results show that the investigator may choose either affinity dependent or affinity independent performance by adjusting the assay conditions. High antigen concentration (concn) and small interepitope distance favor affinity independence, while low antigen concn and large interepitope distance favor affinity dependent assay behavior. The maximum sensitivity will occur in the affinity independent region. Antibody titers obtained under affinity independent conditions will reflect true antibody concn, but measurements performed under affinity dependent conditions will underestimate antibody concn.

Antibody Affinity

Theoretical analysis of the reaction of multivalent antigen with heterogeneous antibody: a model of soluble phase antibody assays.

The theoretical characteristics of soluble phase antibody assays were analyzed using a computer model of antigen-antibody interaction which allowed antibody to be heterogeneous and antigen multivalent. Studies show that when antibody is heterogeneous with respect to affinity, the high affinity antibody will usually mask the low affinity antibody. If the reaction of multivalent antigen with multispecific antibody is studied in antigen excess, the reaction closely approximates a one to one binding reaction. Several characteristics of the soluble phase antibody assay are determined by the affinity-antigen product or the product of the antibody affinity constant multiplied by the molar antigen concentration. When the product is high, the sensitivity of the assay will vary with antigen concentration and titers will be independent of antibody affinity. If the affinity-antigen product is low, sensitivity is dependent upon antibody affinity and titers will reflect antibody affinity as well as antibody concentration.

Antibodies

Intrauterine diagnosis of renal anomalies by ultrasonography.

The diagnosis of renal disease was made antenatally in three patients by ultrasonographic examination. In two cases a multicystic kidney was detected in utero and removed shortly after delivery. In another case, a hydronephrotic kidney was detected in utero; repair was then performed early, before symptoms developed in the infant. Antenatal diagnosis of renal disease by ultrasonography may help reduce morbidity and mortality in some cases.

Female

The Cockayne syndrome: an evaluation of hypertension and studies of renal pathology.

Three children with renal disease, hypertension, and the Cockayne syndrome were evaluated. All patients had severe hypertension; peripheral vein renin was elevated in two patients. Renal biopsy specimens from two patients were studied by light microscopy, electron microscopy, and immunofluoresence. Immunohistologic studies demonstrated deposits of immunoglobulin and complement in the vessels and glomeruli of the first patient; deposits of immunoglobulin and complement were seen in the glomeruli of the third patient. Also electron-dense deposits were seen in the glomerular basement membrane of the third patient. Circulating immune complexes were detected by the Raji cell and Clq binding techniques in this child as well. Both hypertension and renal disease are frequent complications of the Cockayne syndrome.

Abnormalities, Multiple

Propranolol as an antihypertensive agent in children.

The antihypertensive effect of oral propranolol was studied in 9 children with hypertension. After treatment with propranolol, systolic blood pressure fell by an average of 26 mmHg (P less than 0.01). Diastolic pressure decreased by 20 mmHg on average (P less than 0.01). The mean propranolol dose was 2.5 mg/kg per day. Side effects included bradycardia and anorexia. There was no correlation between pretreatment plasma renin activity and fall in blood pressure. Propranolol is an effective and well tolerated antihypertensive agent in children.

Adolescent