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Biomedical subjects

G T Klauber

Publications and source records attributed to G T Klauber.

At least 19 recordsLinked to original sources
PubMed1996

Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management.

PURPOSE: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated. METHODS: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery. RESULTS: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%. CONCLUSION: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.

Abortion, Therapeutic↗
PubMed1996

Renal cell carcinoma in association with tuberous sclerosis in children.

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

Adult↗
PubMed1996

Minimal hydronephrosis in the fetus: clinical significance and implications for management.

PURPOSE: We further define the natural history and management of minimal fetal hydronephrosis. MATERIALS AND METHODS: Experience with minimal fetal hydronephrosis (renal pelvic diameter 4 to 10 mm. before 20 weeks of gestation and 5 to 10 mm. from 20 to 24 weeks) was reviewed for 18 months at New England Medical Center. RESULTS: The incidence of minimal fetal hydronephrosis was 2.2% in the 5,900 fetuses screened by ultrasound. In 63% of cases prenatal ultrasound was done more than once, and revealed that dilatation of the urinary tract was stable during gestation in 31 (25%), and became normal in 35 (29%) and worse in 11 (9%). A total of 63 patients (103 renal units) followed for up to 1 year underwent postnatal sonography. Those with abnormalities were screened with a voiding cystourethrogram and renal scan, including 40 with normal postnatal ultrasound and no changes on subsequent evaluation. Of the 23 patients with abnormal findings 4 had functionally significant ureteropelvic junction obstruction and 1 had severe vesicoureteral reflux. A retrospective analysis of the sonograms of patients with minimal fetal hydronephrosis and persistence or progression of hydronephrosis revealed calicectasis, progression of minimal fetal hydronephrosis in utero and/or abnormal renal echogenicity in each fetus. CONCLUSIONS: Our study shows that in a small but significant number of fetuses minimal fetal hydronephrosis will progress, whereas in most it will resolve.

Disease Progression↗
PubMed1995

Simple testicular cyst in the neonate.

The authors report the first case of a simple testicular cyst presenting in a neonate. Diagnostic criteria are outlined and theories concerning etiology are discussed. The authors show an absence of elastin staining in the specimen, and suggest this precludes germinal tubules as a source. The rationale for testicular-sparing surgery is discussed.

Cysts↗
PubMed1995

The biological behavior of autologous collagen injected into the rabbit bladder.

Growing interest in the endoscopic treatment of vesicoureteral reflux and urinary incontinence has led to the development of safe reliable techniques that use an injectable bulking agent. A variety of injectable materials has been studied, such as polytetrafluoroethylene, fat and bovine collagen. We evaluated autologous collagen extracted from the dermis of skin for potential use in the urinary tract. Therefore, an animal model was developed to study the biological behavior of autologous collagen within the bladder. Collagen was extracted from the skin of rabbits and radiolabeled with 125iodine. An aliquot of radiolabeled autologous collagen was injected submucosally in the bladder. The animals were monitored weekly under a gamma camera. The decay of the radiolabeled collagen was recorded during 50 days. The animals were then sacrificed and the bladder wall at the site of injection was studied histologically and on electron microscopy. Decay of the injected collagen within the urinary tract was similar to control material, indicative of good stability within the bladder. A mild inflammatory response with fibroblastic and vascular invasion of the collagen implant was observed. These preliminary data suggest that autologous collagen injected endoscopically can provide a safe reliable bulking material that could be used to treat urinary incontinence and lower grades of vesicoureteral reflux in children.

Animals↗
PubMed1994

Urological aspects of intra-abdominal desmoplastic small round cell tumor of childhood: a preliminary report.

To our knowledge intra-abdominal desmoplastic round cell tumor has not previously been described in the pediatric urological literature. This lesion has only recently been recognized as a clinicopathological entity with predilection for adolescent boys. We report on 2 patients who presented with urological symptoms and a large abdominal or pelvic tumor that was biopsied at exploratory laparotomy. Complete surgical excision was impossible in both patients, who subsequently underwent multiagent chemotherapy. Histological and immunohistochemical staining are distinctive in this condition. The tumor is associated with a poor prognosis, despite multidisciplinary, multimodal therapy.

Abdominal Neoplasms↗
PubMed1992

Von Hippel-Lindau disease and renal cell carcinoma in a 16-year-old boy.

Von Hippel-Lindau disease is a rare autosomal dominant disorder. Kidney lesions occur in the majority of cases, with renal cell carcinoma noted in 40% and renal cysts in 60%. Renal cell carcinoma in von Hippel-Lindau disease is usually bilateral and occurs at an earlier age than in patients with sporadic renal cell carcinoma. We report on a 16-year-old boy who, to our knowledge, is the youngest patient to present with von Hippel-Lindau disease and renal cell carcinoma. Controversy currently exists regarding the nature of renal cysts in von Hippel-Lindau disease and the optimal therapeutic approach (that is radical versus parenchymal sparing surgery). We review the histology of renal cysts and carcinoma, and discuss the rationale for selecting parenchymal sparing surgery.

Adolescent↗
PubMed1986

Dermoid tumors occurring at the site of previous myelomeningocele repair.

Four children with previously repaired myelomeningoceles presented toward the end of the first decade or early in the second decade of life with deteriorating lower-extremity and bladder function. Myelography and computerized tomography scanning demonstrated irregular filling defects at the area of the myelomeningocele repair, and surgical exploration disclosed dermoid tumors that were adherent to the placode and adjacent roots. Dermoid tumors should be considered in the differential diagnosis of neurological deterioration in children with a repaired myelomeningocele.

Adolescent↗
PubMed1985

Congenital ureteral valves--an abnormality of ureteral embryogenesis?

Congenital ureteral valves are a rare cause of obstructive uropathy and the majority of cases are diagnosed only at surgery or autopsy. A case of congenital ureteral valve associated with an incompletely duplicated kidney is reported and the literature on ureteral valves is reviewed. Modern uroradiological and endoscopic diagnostic techniques should result in a more precise preoperative diagnosis. The high incidence of associated genitourinary anomalies, particularly duplication anomalies, suggests abnormal ureteral embryogenesis as an etiological factor in congenital ureteral valve formation.

Adolescent↗
PubMed1983

Cell surface coat of human and rat bladder urothelium. I. Ruthenium-red studies in non-neoplastic and neoplastic cells.

We have studied the ultrastructure of glycocalyx at the luminal surface of normal and diseased urothelium from humans and rats with ruthenium red staining. A correlation between the thickness and staining intensity of the glycocalyx and the surface topography of the luminal surface was observed. An intensely stained thick glycocalyx was associated with prominent surface microvilli seen in the following conditions in humans: some control urothelium, inverted papilloma, well and moderately differentiated transitional cell carcinomas and mucin producing adenocarcinomas. These changes were also present in rats with FANFT-induced preneoplastic and neoplastic changes. A thin glycocalyx was associated with a scalloped luminal surface containing asymmetric unit membrane plaques and was found in some control humans urothelium and in normal rat urothelium. A thin glycocalyx was also associated with the relatively smooth surface seen in poorly differentiated transitional cell carcinomas as well as in some mucin producing adenocarcinomas. We suggest that urothelial glycocalyx, as demonstrated by ruthenium red staining, correlates with the luminal surface topography rather than specific pathological conditions of the bladder.

Adenocarcinoma↗
PubMed1983

Acute leukemia: diagnosis and management of testicular involvement.

Contemporary therapy of acute leukemia frequently achieves long-term continued complete remission (CCR) of bone marrow disease and prevents central nervous system relapse. However, accompanying improved survival is an increasing incidence of overt testicular relapse either during CCR or associated with bone marrow relapse. In 7 boys testicular abnormalities developed during CCR, 6 had open biopsy and 5 had histologically confirmed leukemic infiltration. Despite local therapy of orchiectomy or irradiation and chemotherapy reinduction, 2 of 6 had testicular relapse and 4 of 6 died. Three boys with coexistent overt testicular and systemic relapse died. Nine boys with normal testes had testicular biopsy during CCR prior to discontinuation of chemotherapy. Results of all biopsies were benign, but one boy had a relapse. The diagnosis of occult testicular leukemia prior to discontinuation of chemotherapy allows selection of high-risk boys requiring prolonged, intensive, and possibly alternative therapy. The indication for testicular biopsy in boys with acute leukemia is documented, and appropriate clinical management of testicular leukemia is presented.

Antineoplastic Agents↗
PubMed1983

Critical evaluation of the Credé maneuver: a urodynamic study of 207 patients.

The efficacy of Credé's maneuver to promote voiding was evaluated prospectively in 207 patients with a variety of lower urinary tract disorders. The normal response to Credé's maneuver was a contraction of the external urethral sphincter (the Credé effect), an increase in urethral pressure and maintained closure of the vesical neck. Relaxation of the external urethral sphincter and synchronous opening of the bladder neck during Credé's maneuver were seen in only 4 patients (2 per cent). We conclude that Credé's maneuver is an inefficient method of bladder emptying in the majority of patients and even when voiding is accomplished with Credé's maneuver it usually is grossly inefficient and associated with significant urethral obstruction.

Adolescent↗