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Biomedical subjects

R M Ehrlich

Publications and source records attributed to R M Ehrlich.

At least 73 records · Page 4Linked to original sources

Pyelovesicostomy as a form of urinary reconstruction in renal transplantation.

The development of ureteral obstruction or ureteral fistula formation in the renal transplant recipient usually requires surgical repair. This involves reconnecting the donor ureter to either the recipient ureter (ureteroureterostomy) or bladder (ureteroneocystostomy), or creating an anastomosis between the renal pelvis and recipient native ureter (pyeloureterostomy). Occasionally, the donor or recipient ureter is absent, necrotic or diseased so that a ureteroureterostomy, ureteroneocystostomy or pyeloureteral anastomosis cannot be performed. In 8 such cases we have performed a direct anastomosis between the donor renal pelvis and recipient bladder (pyelovesicostomy) with a followup of between 2 months and 11 years. In all 8 patients there has been no deterioration in renal function attributed to obstruction at the anastomotic site or to the free reflux between the bladder and renal pelvis. Because of the excellent short-term and long-term results of pyelovesicostomy, this procedure should be considered as an excellent alternative to pyeloureterostomy, ureteroureterostomy and ureteroneocystostomy in the reconstruction of the upper urinary tract of the renal transplant patient.

Adult↗

Thyrotrophin blocking antibodies in the sera of mothers with congenitally hypothyroid infants.

We examined IgG from 15 mothers of infants with congenital hypothyroidism for their ability to block TSH-stimulated iodine organification in vitro. Only one out of 15 of these IgGs exhibited TSH-blocking activity. This IgG could inhibit TSH action by 30-50%. It could not block radiolabelled TSH from binding to its receptor but did inhibit dibutyryl cyclic AMP stimulation of iodine organification implying a unique effect on post-receptor processes via a membrane binding site distant from the TSH binding site. Interestingly there was no evidence of autoimmune thyroid disease in the mother. These results suggest that the transplacental passage of TSH-blocking antibodies plays little, if any, role in the development of congenital hypothyroidism.

Antibodies↗

Congenital hypothyroidism. The effect of stopping treatment at 3 years of age.

Fifty-six children with congenital hypothyroidism diagnosed by neonatal screening were reviewed at 3 years of age or older. The presence or absence of the thyroid gland was determined by radionuclide scanning prior to treatment in the newborn period. Thyroxine therapy was discontinued in those children who did not have anatomic defects or a secondary rise in their thyrotropin (thyroid-stimulating hormone [TSH]) level once it was suppressed by thyroid hormones. Sixteen of 17 children developed a low thyroxine and an elevated TSH level within three to six weeks. One child was not receiving thyroxine for nine months and was clinically and biochemically euthyroid. We conclude that (1) newborn thyroid scans are useful to determine the cause of hypothyroidism, (2) a secondary rise in the TSH level indicates permanent hypothyroidism, (3) only about one third of infants whose condition is diagnosed by newborn screening will qualify for a trial off therapy at 3 years of age, (4) only 1% to 2% of infants whose condition is diagnosed by newborn screening have transient hypothyroidism, and (5) a three-week period of hormone withdrawal after the age of 3 years seems adequate and safe to confirm permanent hypothyroidism.

Child, Preschool↗

The ureteral folding technique for megaureter surgery.

The folding technique for megaureter surgery achieved a success rate of 94.6 per cent in 74 megaureters. In this series failure to correct reflux was noted in only 3 ureters and only 1 obstruction resulted. This operation theoretically preserves lower ureteral blood supply, eliminates suture line leakage and prolonged stenting, and is a model of cost-effectiveness.

Adult↗

The relationship of metabolic control to growth and pubertal development in children with insulin-dependent diabetes.

Growth and pubertal development were studied in 122 children aged 13.4 +/- 2.9 yr (mean +/- SD) with IDDM of 6.1 +/- 3.8 yr duration. Height and weight, pubertal status, insulin dose (u/kg), frequency of insulin administration and HbA1 were measured every 3 months for a minimum period of 1 yr for each child. The mean and distribution of height and height velocity percentiles were normal for boys and girls. The mean weight percentile for boys was increased (62 +/- 27, p less than 0.01), but was normal for girls. The mean and distribution of weight velocity percentiles were normal for both sexes. The mean and distribution of age of onset of Tanner 2 in both boys (11.9 +/- 1.4 yr) and girls (11.2 +/- 1.3) was normal, as was the age of menarche (13.2 +/- 1.2 yr). The mean HbA1 level was 11.1 +/- 2.0% (normal less than 7.5%). There was no correlation of mean HbA1 levels (calculated from 4 or more measurements for each child) with height velocity or with weight velocity percentiles or with mean height or weight percentiles. These results indicate that diabetic control, as reflected by HbA1 levels, was not a major determinant of growth in this group of children with IDDM, who displayed normal growth patterns and pubertal development despite a wide range of HbA1 levels. Normal growth may not reflect optimal metabolic control but "average" control.

Adolescent↗

Bilateral renal cell carcinoma and renal cell carcinoma in the solitary kidney.

We report on 43 patients with renal cell carcinoma in a solitary kidney, 39 of whom underwent a potentially curative resection. Of 36 patients who had a nephron salvaging procedure only 4 required ex vivo surgical resection. The survival curves of patients with solitary or bilateral lesions are similar, and depend more on the adequacy of tumor resection and tumor stage than on the fate of the contralateral kidney. Crude survival in this series was 64 per cent for patients followed for more than 1 year.

Adenocarcinoma↗

Prevention of cyclophosphamide cystitis with 2-mercaptoethane sodium sulfonate: a histologic study.

The ability of 2-mercaptoethane sodium sulfonate to prevent histologic damage to the bladder from cyclophosphamide was studied. Male rats receiving 2-mercaptoethane sodium sulfonate in conjunction with cyclophosphamide had a statistically significant decrease in ulceration, inflammation and edema of the bladder compared to those treated with cyclophosphamide alone. Most bladders of animals given prophylactic 2-mercaptoethane sodium sulfonate with a dose of cyclophosphamide were histologically indistinguishable from controls receiving neither drug. The relevance of these findings to the short and long-term effects of cyclophosphamide on the urothelium is discussed.

Animals↗

The use of sodium 2-mercaptoethane sulfonate to prevent cyclophosphamide cystitis.

In 2 patients receiving bone marrow transplantation sodium 2-mercaptoethane sulfonate has proved efficacious in preventing the serious problem of cyclophosphamide cystitis by regional detoxification of acrolein. We detail the first use of sodium 2-mercaptoethane sulfonate in the United States. We are cautiously enthusiastic and optimistic that the simultaneous administration of sodium 2-mercaptoethane sulfonate and cyclophosphamide will decrease if not eliminate cyclophosphamide-induced carcinoma.

Bone Marrow Transplantation↗

Sustained normoglycemia in newly diagnosed type I diabetic subjects. Short-term effects and one-year follow-up.

The impact on remission of normalizing blood glucose levels immediately after diagnosis of type I diabetes was studied in 14 adolescents. Accordingly, in this randomized prospective primary intervention study, 7 of the subjects (i.v. group) received insulin by continuous intravenous (i.v.) infusion via a portable preprogrammed system for 28-62 days and 7 (s.c. group) received conventional subcutaneous (s.c.) therapy. Before therapy, the two groups did not differ significantly with respect to glycosylated hemoglobin, fasting plasma C-peptide, or 24-h urinary C-peptide excretion. During the infusion period, the overall mean fasting plasma glucose (FPG) concentration for the i.v. group was 84 mg/dl with a mean coefficient of variation of 18 +/- 4% (mean +/- SD). During the comparable period for the s.c. group, the mean FPG was 253 mg/dl with a coefficient of variation of 30 +/- 20%. Twenty-four-hour urinary glucose excretions for the two groups were 0.29 +/- 0.06 (mean +/- SEM) and 59 +/- 11 g/day, respectively. Daily insulin requirements in the i.v. group decreased from 1.47 +/- 0.19 U/kg body wt/day at the start to 0.47 +/- 0.10 U/kg/day at the end of the infusion period. Notably, 10-25 days after the infusion period, 5 of 7 subjects experienced a further decrease to a low of 0.27 +/- 0.01 U/kg/day. The mean peak and low requirements in the s.c. group were 0.71 +/- 0.15 and 0.33 +/- 0.13 U/kg/day, respectively, with the only peak requirements being significantly different (P less than 0.01). No patient was able to discontinue insulin therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Renal transplantation update.

Various factors affect the outcome of renal transplants in humans. Matching for HLA-A, -B, and -DR histocompatibility antigens improves survival rates for renal allografts from first cadaver donors. Zero-HLA-A- and -B-antigen-mismatched grafts and two-HLA-DR-antigen-matched grafts do better, although results differ depending on the recipient's primary renal disease. Pretransplant third-party blood transfusions significantly improve survival rates of cadaver donor allografts. The mechanism of this beneficial effect has not been identified; however, blood transfusions probably do not "select out" high responders among potential recipients by stimulating the production of lymphocytotoxic antibodies. Cyclosporine has been heralded as a potent, nonspecific immunosuppressive agent that will significantly improve renal allograft survival rates. The selectivity of cyclosporine's effect on T lymphocytes is advantageous; however, its side effects, especially nephrotoxicity, may limit its usefulness. Attention to the potential surgical complications of renal transplantation can significantly reduce morbidity and mortality.

Blood Transfusion↗

Calycovesicostomy for total ureteral obstruction after renal transplantation.

Surgical reconstruction for ureteral obstruction is necessary in 1 to 10 per cent of renal transplants. On an acute basis edema, ischemia, lymphocele and hematoma formation cause ureteral obstruction. Chronic etiologies include ureterovesical obstruction and retroperitoneal or ureteral fibrosis. Options for repair are myriad and include repeat ureteral reimplantation, pyeloureterostomy, ureteroureterostomy, pyelocystostomy and calycovesicostomy. We report on the desirability of calycovesicostomy as a last resort option for total ureteral obstruction after renal transplantation.

Adult↗

HLA haplotype segregation in families of type 1 diabetics.

The hypothesis of linkage between HLA and a disease susceptibility (DS) locus (or loci) for type 1 diabetes was tested. HLA segregation was random among 57 non-diabetic sibs but not among 39 diabetic sibs, suggesting that susceptibility to type 1 diabetes may be due to an HLA-linked gene(s). The data did not fit a genetic model involving either a single recessive or dominant gene. The excess of HLA-identical diabetic sibs and the reduced number who were HLA-discordant compared to expected numbers indicated that factors from both paternal and maternal haplotypes were necessary for DS. In 1 of the 3 families with a diabetic parent and more than one diabetic sib, the diabetic sibs inherited different haplotypes from the affected parent, suggesting that either of these haplotypes conferred DS. HLAB 8, B 18 and B 40 were increased in frequency among 97 unrelated type 1 diabetics compared with 238 controls, especially among those with onset age less than 10 years. This early onset group may represent a subtype of type 1 diabetes.

Adolescent↗