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Biomedical subjects

R D Jeffs

Publications and source records attributed to R D Jeffs.

At least 109 records · Page 6Linked to original sources
PubMed1985

Cloacal exstrophy: a 22-year experience.

Our 22-year experience in the management of 12 patients with cloacal exstrophy is discussed. All patients underwent functional bladder closure. Of 7 evaluable patients 3 (43 per cent) have continent intervals of 3 to 4 hours and constitute the first report of satisfactory urinary continence following functional bladder closure in patients with cloacal exstrophy. Of 8 patients with an XY karyotype 6 were raised as girls with satisfactory genitoplasty, while 2 raised as boys had functionally inadequate external genitalia. The management of the rudimentary hindgut varied and the alternative approaches are discussed. It appears that this patient population can be rehabilitated to lead productive lives.

Abnormalities, Multiple↗
PubMed1984

The inheritance of the exstrophy-epispadias complex.

The inheritance pattern of the exstrophy-epispadias complex was investigated by a combined literature review and a survey of the personal experience of pediatric urologists in North and South America, and Europe. Bladder exstrophy recurred in only 9 of approximately 2,500 families (1 in 275) with bladder exstrophy or complete epispadias. The recurrence of the exstrophy-epispadias complex in offspring of parents with bladder exstrophy or complete epispadias has never been described previously. We identified 215 offspring produced by parents with bladder exstrophy or epispadias, and bladder exstrophy was inherited in 3 of the offspring (1 in 70 live births). The recurrence of bladder exstrophy in offspring of parents with the exstrophy-epispadias complex is greater than previously assumed.

Abnormalities, Multiple↗
PubMed1984

Urethral reconstruction in boys with classical bladder exstrophy.

A total of 24 boys with classical bladder exstrophy underwent initial urethral reconstruction at our hospital between 1975 and 1982. Penile reconstruction in male patients with classical bladder exstrophy includes penile lengthening, release of the dorsal chordee and reconstruction of the urethra. The former 2 procedures are performed during the primary bladder closure and urethroplasty usually follows bladder neck reconstruction. A modified Young urethroplasty was done in 22 of the 24 patients. Preputial pedicle grafts of free full thickness skin grafts were used for urethroplasty in 2 boys with insufficient penile skin. Fistulas requiring surgical revision developed after urethroplasty in 21 per cent of the patients. A prior osteotomy was associated with a decreased fistula rate. The cosmetic and preliminary functional results of the penile reconstruction were assessed by parental interviews. The definitive assessment of the penile reconstruction will be determined when these boys reach sexual maturity.

Bladder Exstrophy↗
PubMed1984

Fetal hydronephrosis: considerations regarding urological intervention.

Real-time ultrasonic imaging during pregnancy has made it possible to diagnose accurately fetal hydronephrosis as early as at 17 weeks of gestation. The ultrasonic finding of oligohydramnios in conjunction with megacystis and bilateral hydronephrosis is the most reliable indicator of marked permanent urinary tract obstruction and, therefore, is an absolute requirement for fetal urological intervention. Fetal intervention by means of percutaneous catheter drainage of the bladder into the amniotic space is undertaken to preserve renal and pulmonary function. It appears that urinary tract decompression at 17 weeks of gestation is unable to prevent renal dysplasia. The degree to which renal cortical function is preserved by fetal drainage of the hydronephrotic kidney is unknown. Experimental evidence has suggested that early urinary tract decompression promotes improved pulmonary development, which may allow for normal pulmonary function postnatally. We report 6 cases that illustrate important considerations with regard to urological intervention for fetal hydronephrosis.

Female↗
PubMed1983

Wound dehiscence in bladder exstrophy: an examination of the etiologies and factors for initial failure and subsequent success.

During the last 8 years 20 patients were seen for secondary or repeat closure for exstrophy of the bladder following dehiscence after initial closure. Various factors that may have contributed to the initial problems have been investigated. Wound infection (42 per cent) and bladder prolapse (46 per cent) were the major etiologies for initial failures. In 6 patients (30 per cent) colon conduits were required for bladder augmentation or colocolostomy, which emphasized the importance of achieving a good result at initial closure. Our successful protocol (only 5 per cent reclosure rate for 60 closures) is outlined.

Anti-Bacterial Agents↗
PubMed1983

Ileal trigonal conduits: 10-year followup.

Nonrefluxing ileal trigonal conduits were constructed in 21 patients with neurogenic bladder to prevent the long-term sequelae of standard ileal conduit urinary diversion. Although the initial results at 5 years were encouraging the findings at 10 years were not as promising. Of the patients 50 per cent had evidence of pyelographic deterioration. Reflux occurred in 41 per cent of the renal units. Over-all, 8 patients (45 per cent) have done well. Today, these patients probably would not have undergone diversion.

Adolescent↗
PubMed1983

Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy.

The surgical results of 28 consecutive initial bladder closures and 25 consecutive initial bladder neck reconstructions performed for classical bladder exstrophy at our hospital between 1975 and 1982 are presented. Partial bladder prolapse occurred in 2 cases and complete wound dehiscence never occurred following the initial primary bladder closure. Urinary continence following bladder neck reconstruction was assessed from parental interviews. An excellent surgical result was defined either as achievement of a daytime dry interval for more than 3 hours or less than 1 incontinent episode per day. According to these parameters, an excellent surgical result was achieved in 86 and 80 per cent of children, respectively. In 21 children evaluated with excretory urograms between 1/2 and 6 years after bladder neck reconstruction 10 per cent of the renal units showed significant hydronephrosis and deterioration of function. The 2 patients who had upper tract deterioration were not followed postoperatively at our institution and the diagnosis of bladder outlet obstruction was delayed when excretory urograms were not obtained during the first postoperative year. This review of the surgical results following primary bladder closure and bladder neck reconstruction for classical bladder exstrophy demonstrates that secure abdominal wall closure and urinary continence can be achieved with minimal morbidity and with infrequent deterioration of renal function following staged functional bladder closure.

Bladder Exstrophy↗
PubMed1982

Undiverting the ileal conduit.

From 1975 to 1981, 46 patients were evaluated for urinary undiversion. Of these patients 27 completed urinary reconstruction. The patients have been followed for 6 to 80 months, with a mean followup of 32 months and a median followup of 23 months. There has been no death and no rediversion has been necessary, although in 1 patient renal function has deteriorated significantly following urinary reconstruction. Postoperative bladder function was normal in patients diverted for dysfunctional voiding or bladder outlet obstruction but children with meningomyelocele required intermittent catheterization postoperatively. With careful patient selection, meticulous surgical technique and compulsive followup urinary undiversion is a safe procedure.

Adolescent↗
PubMed1982

The urological evaluation and management of patients with congenital lower urinary tract anomalies prior to renal transplantation.

Previously, patients with chronic renal failure and major congenital anomalies of the lower urinary tract (often with urinary diversion) were thought to be poor candidates for renal transplantation. Pre-transplant evaluation and possible urinary reconstruction are essential in these patients to achieve successful renal transplantation. Ten patients, including 7 adults, presented with congenital anomalies of the lower urinary tract that were responsible for renal failure. Percutaneous suprapubic cystostomy aided in the assessment of bladder function. Undiagnosed posterior urethral valves were found in 2 adults. Patients with exstrophy, neurogenic bladder or a contracted bladder (with augmentation cystoplasty) had urinary drainage into the bladder at the time of renal transplantation. Sometimes an imperfect bladder can be used for urinary drainage with transplantation but, otherwise, intestinal conduits are still a viable alternative.

Adult↗
PubMed1981

Control of prolonged, benign, renal hematuria by silver nitrate instillation.

Four patients with protracted benign hematuria underwent silver nitrate instillation of the upper collecting system. The doses varied from 10 ml. of 0.25 per cent silver nitrate to 14 ml. of 1 per cent silver nitrate. In all 4 cases the hematuria resolved within four days without significant complications. At follow-up extending from three to twenty-four months there have been no recurrences of gross hematuria. Silver nitrate instillation appears to be a safe and effective method of treating benign renal hematuria, thus obviating the need for extirpative surgery.

Adult↗
PubMed1980

Phenotypic variation in a family with partial androgen insensitivity syndrome.

A family with partial androgen insensitivity syndrome exhibited considerable variation in phenotypic expression of their androgen resistance. One subject died at 2 1/2 years of age of a Wilms' tumor. In the two living members, one had a micropenis with otherwise normal genitalia, while the other had a small phallus, perineoscrotal hypospadias, bifid scrotum, and persistence of a vaginoutricular pouch. At puberty, plasma androgens and serum gonadotropins increased to normal or elevated values. However, despite adequate endogenous plasma testosterone levels and testosterone therapy, these patients showed poor virilization and were sterile. Studies of cultured sexual skin fibroblasts showed adequate 5 alpha-reductase activity and normal receptor affinity and capacity for dihydrotestosterone. An X-linked mode of inheritance is postulated, although autosomal dominance cannot be ruled out.

Child↗