[Rare variations in exstrophy-epispadias complex].
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Adult↗
Biomedical subjects
G Schott
Publications and source records attributed to G Schott.
Management of failed bladder exstrophy closure in adolescence and adulthood.
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Adolescent↗
Development of an oncology data network in Germany.
In the 1990ies, an oncology data network has been set up mainly in the New States of Germany. Although not formally planned and established as a whole, it consists of a number of initiatives, that co-operate well and gain added value from this co-operation. From the technological view, the centre of the network is the Giessener Tumordokumentationssystem (GTDS), that was developed at Giessen University. We present important basic conditions in which this development took place, show some results and describe future directions of the development.
Computer Communication Networks↗
[Extravesical correction of the uretero-vesicle junction].
In correction of the ureterovesical junction, the pathology can be corrected using an extravesical or intravesical technique. We performed an extravesical antireflux procedure with the dismembered or non-dismembered technique in 117 ureters between March 1991 and March 1996 at our Department of Pediatric Urology. The success rate was 94.9% (111 renal units). Postoperative morbidity and complications were minimal, hematuria and bladder spasms were not seen. Associated pathology like paraostial diverticula, megaureter with the necessity for modeling and ureter duplex do not compromise the success rate.
Anastomosis, Surgical↗
[Ventral urethral diverticulum. Case report of a 10-year-old girl].
In total urethral diverticula in children represent a very rare urological disorder. Primary localisation is the dorsal side of the urethra, ventral located diverticula are extremely rare. We report on a ten year old girl with a ventral, urethral diverticulum presenting with the symptoms of persisting enuresis. The diverticulum was excised through a median laparatomy. Placement of suprapubic catheter facilitated adequate postoperative drainage and postoperative cystourethrography confirmed the absence of extravasation.
Child↗
Percutaneous renal biopsy: three years of experience with the biopty gun in 761 cases--a survey of results and complications.
From 1.1.1993 to 12.31.1995 we performed 761 consecutive biopsies on 509 non-selected patients. The most frequent diagnoses in 351 biopsies (39.4%) on native kidneys were 262 cases of glomerulonephritis (74.6%) and 167 of so-called benign nephrosclerosis (47.6%). With 410 biopsies (60.6%) on allograft kidneys 219 cases (78%) showed signs of interstitial rejection, 14 cases (5%) vascular and 49 cases (17%) interstitial as well as vascular rejection. Only after 5 biopsies (0.66%) clinical relevant complications (3 perirenal haematomas, 1 AV fistula, 1 vesical tamponade) appeared. Again percutaneous renal biopsy is proven to be a diagnostically efficient and safe tool at the same time even when used in a large number of non-selected patients, so that an ambulant performance may be discussed. The relatively frequent diagnosis of a so-called benign nephrosclerosis seems to indicate the need for an intensified examination of this disease.
Adult↗
Immunophenotype and clinical characteristics of CD45-negative and CD45-positive childhood acute lymphoblastic leukemia.
To evaluate the expression pattern of the leukocyte common antigen CD45 in acute leukemias and to investigate whether the lack of CD45 expression in childhood acute lymphoblastic leukemia (ALL) is associated with other immunophenotypic features and a distinct clinical behavior, we have carried out extensive immunophenotypic analyses of bone marrow and peripheral blood samples from 638 patients with childhood B-cell precursor (n=529) or T-lineage ALL (n=109). All 638 patients were enrolled in the German ALL-BFM 90 and ALL-BFM 95 trials. CD45 was detected on the surface of childhood ALL cells (cut-off > or = 20% positive cells) in only 88.7% (n=566) of all cases. Among 529 patients with childhood B-cell precursor ALL, 12.9% (n=68) did not express CD45, compared with only 3.7% (n=4) of patients with childhood T-lineage ALL (p < 0.001). In the B-cell precursor ALL subtypes, the highest frequency of CD45- cases (15.1%) was observed in common ALL (56/372) compared with only 7.2% in pro-B ALL (3/41) and 7.8% in pre-B ALL (9/116). Assessment of clinical parameters (age, organ enlargement, WBC, etc.) and event-free survival did not reveal significant differences between CD45- and CD45+ patients. Myeloid antigen coexpression was not correlated with CD45 expression. The mean percentage of antigen expression for CD34, CD10, TdT, CD22, and CD24 was significantly higher in children with CD45- B-cell precursor ALL than in those with CD45+ B-cell precursor ALL. In 28 patients with B-cell precursor ALL, cell cycle analyses of freshly isolated leukemic cells were performed with propidium iodide (PI) staining and flow-cytometric analysis. The percentage of cells in S-phase was inversely correlated to the percentage of CD45+ cells (r=-0.48, p < 0.05). With two-parameter analysis of CD45-fluorescein isothiocyanate (FITC)- and PI-stained cells in nine patients with a percentage of CD45+ cells between 40 and 60%, two populations were distinguishable in a single patient. It was shown that the CD45- subpopulation had a higher percentage of cells in S-phase than the CD45+ subpopulation (10.7 +/- 4.0 vs. 2.7 +/- 1.8, p < 0.007). We conclude that the lack of CD45 expression contributes to the identification of a distinct functional and immunological subgroup of B-cell precursor ALL, but that it has no significant impact on clinical behavior or on therapy outcome in childhood ALL.
Antineoplastic Agents, Hormonal↗
Down's syndrome in childhood acute lymphoblastic leukemia: clinical characteristics and treatment outcome in four consecutive BFM trials. Berlin-Frankfurt-Münster Group.
Clinical characteristics, treatment response and outcome were evaluated in children with Down's syndrome (DS) and acute lymphoblastic leukemia (ALL) as compared to other children with ALL (NDS). Sixty-one DS and 4049 NDS patients, receiving intensive antileukemic treatment during four consecutive trials (ALL-BFM 81, 83, 86 and 90) of the Berlin-Frankfurt-Münster Group (BFM), were retrospectively analyzed. DS and NDS children did not differ with respect to sex, leukocyte count, CNS leukemia and cytogenetic translocations. The DS cohort was slightly older (P=0.04), presented predominantly with the common while lacking the T immunophenotype (P=0.005), had a lower frequency of hyperdiploidy (P=0.004) and tended to have a better initial steroid response (P=0.057). Therapy-associated morbidity especially during high-dose methotrexate and a subsequent need for treatment modification occurred in 43% of all DS patients. Event-free survival (EFS) was slightly worse in children with DS (58+/-8% vs 70+/-1%, P=0.14), mainly due to rather late bone marrow recurrences. However, EFS in DS patients was comparable to the NDS group once they either received treatment with no major modifications (65+9% vs 70+/-1%, P=0.66) or were <6 years of age, irrespectively of therapy modifications (73+/-9% vs 74+/-1%, P=0.7). Cox regression analysis revealed that DS was an adverse prognostic factor for patients having completed therapy (P=0.0107), but was not prognostic at diagnosis (P=0.103). Age > or = 6 years, suboptimal treatment and infectious problems contributed to the slight inferior EFS in children with ALL and Down's syndrome. Therefore, most of these patients can be successfully treated if receiving intensive antileukemic treatment with no major modifications, but they require more sophisticated management of toxicity.
Antineoplastic Combined Chemotherapy Protocols↗
Immunophenotypic and clinical features of T-cell receptor gammadelta+ T-lineage acute lymphoblastic leukaemia.
The immunophenotypic and clinical features of TCR-gammadelta+ T-lineage acute lymphoblastic leukaemia (T-ALL) were prospectively analysed in 52 children with membrane CD3+ T-ALL. We observed a relatively high incidence of TCR-gammadelta+ T-ALL (26/52 patients). Leukaemic blasts from 22 children demonstrated TCR-alphabeta positivity, and simultaneous expression of the TCR-beta and -delta chain was found in four children. Clinical and haematological features of TCR-alphabeta and gammadelta+ T-ALL did not differ significantly, except that haemoglobin levels were significantly lower in TCR-gammadelta+ cases. Event-free survival at 4 years was significantly better in TCR-gammadelta+ compared with TCR-alphabeta+ T-ALL, but expression of TCR molecules did not emerge as an independent prognostic factor in multivariate analysis.
Acute Disease↗
Effective biomodulation by leucovorin of high-dose infusion fluorouracil given as a weekly 24-hour infusion: results of a randomized trial in patients with advanced colorectal cancer.
PURPOSE: To determine whether high-dose infusional fluorouracil (FU) is effectively modulated by leucovorin (LV), interferon (IFN) alpha-2b, or both when given to patients with metastatic colorectal cancer. PATIENTS AND METHODS: Patients (n = 236) with progressive, measurable disease were randomized to three groups and received FU 2,600 mg/m2 as a 24-hour continuous infusion (CI) weekly for 6 weeks with 2 weeks rest (FU24h) and LV 500 mg/m2 as a 2-hour infusion before FU or IFN 3 x 10(6) U subcutaneously 3 times weekly or both. Treatment continued until progressive disease or unacceptable toxicity was observed. Pairs of treatment arms were analyzed sequentially to detect equivalence or a 25% difference in response rates. RESULTS: The rate of objective remission in patients who received FU24h/LV (44%; 40 of 91) was significantly higher than in patients who received FU24h/IFN (18%; 16 of 90; P < .05). The response rates of patients who received FU24h/LV versus FU24h/LV/IFN (27%; 13 of 49) were statistically equivalent. Significant differences were observed for time to tumor progression (TTP) (FU24h/LV, 7.1 months; FU24h/IFN, 3.9 months; FU24h/LV/IFN, 6.3 months; global P value < .009) and survival (16.6 months, 12.7 months, 19.6 months, respectively; global P value < .04). Unpredictable and life-threatening toxicity in the FU24h/LV/IFN arm required dose reduction of FU to 2,000 mg/m2/day and early stoppage of this arm. Toxicity was manageable in patients who received both FU24h/LV (grade 3 to 4 diarrhea, 21%) and FU24h/IFN (grade 3 to 4 diarrhea, 15%). CONCLUSION: Response rate, TTP, and overall survival were superior for LV-containing regimens compared with IFN modulation alone. The addition of IFN to high-dose infusional FU plus LV offers no advantage and may increase toxicity. The regimen of high-dose infusional FU24h/LV warrants further evaluation in patients with metastatic colorectal cancer.
Adult↗
[Tramadol infusion for pain therapy following bladder exstrophy surgery in pediatric wards].
We investigated in 17 children (mean 7.1 years) the continuous administration of tramadol following augmentation cystoplasty or exstrophy reconstruction. Mean duration of the tramadol administration on the pediatric ward was 3.8 +/- 1.1 days (initial dosage 0.25 mg/kg/per hour, dose adjustment by the nursing staff). Mean tramadol consumption was 0.21 mg/kg/h on day 1 and was reduced to 0.08 mg/kg/per hour on day 4. Median pain score (assessed with ten-step scales) was 5 before treatment and between 2.5 (day 1) and 0.5 (day 5) during therapy. Lowest oxygen saturations (mean) ranged from 93.8% to 95.2%. Three patients (17.6%) suffered from nausea/vomiting on 3 of 64 treatment days (4.7%). Pruritus and extreme sedation did not occur. The continuous administration of tramadol is a simple and safe procedure following major urological surgery in children.
Adolescent↗
Is there still a need for Tc-99m DMSA renal imaging?
Scintigraphy with Tc-99m MAG3 is, to date, the most commonly used technique for evaluating separate renal function (SRF). In this study, the performance of this technique is compared to Tc-99m DMSA. A total of 37 patients with various kidney disorders (12 children and 25 adults) underwent both Tc-99m DMSA and Tc-99m MAG 3 scintigraphy. In children and adults of normal weight the correlation coefficient between the two methods was 0.99 (P < 0.001), whereas in individuals who were more than 10% overweight according to Broca, or who had abnormal anatomical positions of the kidneys, the correlation coefficient was 0.82 (P < 0.05). Scintigraphy with Tc-99m DMSA was more accurate in renal parenchyma lesions. These results indicate that Tc-99m DMSA scintigraphy is preferable for the evaluation of SRF in patients who are more than 10% overweight, or have abnormal positions of the kidneys.
Adolescent↗
[Follow-up of kidney function in children with former posterior urethral valves].
Severe forms of posterior urethral valves involve the risk of upper urinary tract damage and impaired renal function. For this reason, 26 boys formerly (between January 1980 and December 1989) treated for high-grade urethral obstruction were re-examined with reference to renal function. Results were compared with observations in a control group of 26 boys matched for age and height undergoing minimal urological surgery. While there were no significant differences in haemoglobin, height, blood pressure or kidney volume, in 14 of 20 patients with normal glomerular filtration rate pathologic findings were recorded, most of which related to urine concentrating capacity, tubular phosphate handling and hyperparathyroidism. These observations necessarily have implications for treatment of such children even if they are assumed to have unimpaired renal function. In future, monitoring of renal function in children with former posterior urethral valves should include different tests, and in particular tests of concentration capacity and measurement of parathyroid hormone.
Child↗
[Suspected kidney tumor--diagnostic-therapeutic procedure].
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Diagnostic Imaging↗
[Kidney transplantation in patients with anomalies of the lower urinary tract].
In the transplant centre in Erlangen-Nuremberg, 1242 patients underwent renal transplantation between 1966 and 1993. In 4.5% of cases (56 patients) the renal end stage disease had been caused by severe abnormalities or functional disturbance of the lower urinary tract. Despite long-standing defunctionalization, urinary diversions and multiple operative procedures, it was possible to use the original bladder for ureterocystoneostomy in all patients. Only 1 patient needed an enterocystoplasty after transplantation. Altogether 72 transplantations have been performed in 56 patients ranging from 10.2 to 62.7 years of age. At follow up, 40 patients (71%) had a functioning graft with a mean serum creatinine level of 1.5 mg%. The 5-year transplant survival rate is 57.3%. Our results suggest that carefully planned renal transplantation in urological patients has results comparable to those obtained in other, non-risk, kidney transplant recipients. The methods of bladder reconstruction and augmentation, intermittent self-catheterization as well as anticholinergics and continuous antibiotic prophylaxis combine to make the ileal or colonic conduits needed earlier unnecessary.
Adolescent↗
[Intraoperative cortical PO2 measurement in kidney transplantation. the effect of the calcium antagonist diltiazem].
A local measurement of the partial pressure of oxygen in the renal cortex was performed during renal transplantation in 40 patients aged between 10 and 62 years. During the measurement, 20 of the patients received the calcium channel blocker diltiazem. Accurate knowledge of the renal microcirculation in the postischemic phase became possible when PO2 tissue polarography was performed. Moreover, measurement in four live donors before removal of the kidneys allowed an exact comparison between the postischemic microcirulation and the native in situ perfusion. A good postischemic baseline histogram (similar to the situation in a live donor) or quick stabilization of the histogram during the course was found to correlate with a prompt initial renal function. Intra-arterial administration of diltiazem led to an insignificant improvement of the primary function rate. In the case of kidneys with longer cold ischemic periods and initially indifferent baseline histograms there was an especially pronounced benefit of diltiazem administration. Not only macroscopic examination, but also polarographic measurements revealed an improvement in the renal microcirculation throughout, with higher mean values for PO2 and homogeneity of all PO2 values measured after administration of diltiazem. In keeping with this, in such cases the incidence of primary renal function was distinctly higher. In cases with improved cortical circulation the a-v oxygen difference was less pronounced, so that evaluation of the avDO2 determinations suggests distinct perfusion improvement following diltiazem.
Adolescent↗
[Immunophenotyping of acute lymphatic leukemia: diagnostic aspects and clinical relevance].
During the past two decades immunophenotyping has yielded significant new information regarding the biological heterogeneity of ALL and has provided a solid basis for a biologically oriented and reliable classification of this disease. At present, lineage commitment of acute leukaemias can be achieved in more than 98% of cases by applying a standardized panel of mAbs to pan-B-cell (CD19, cyCD22), pan-T-cell (cyCD3, CD7) and pan-myeloid antigens (CD13, CD33, MPO) that are expressed either on the surface or in the cytoplasm of the earliest progenitors of the respective cell lineage. Further subclassification of ALL based on the analysis of antigens more closely associated with different maturational stages of B- and T-cell lineage has proven useful for the identification of biologically and clinically distinct entities in both B-cell precursor and T-lineage ALL. Immunophenotyping in about 2800 patients recruited for the German multicentre trials has shown that children and adults differ markedly in frequency distribution of immunological subgroups, with a higher adult incidence of immature B-cell precursor (i.e., pre-pre-B ALL) and T-lineage ALL immunophenotypes (i.e., pre-T ALL). Detailed immunological analyses using a broad panel of mAbs have recently documented typical ALL cases inappropriately expressing myeloid antigens (My+ ALL) as well as morphologically/cytochemically defined acute myeloid leukaemia (AML) with lymphoid-associated markers (Ly+ AML). Based on our own results and a critical review of published data, leukaemic blasts in 5-20% of ALL patients disclose My+ ALL, whereas a coexpression, mostly of T-cell-associated antigens, can be identified in 10-25% of AML cases.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Clinical significance and histological classification of atrophy/hypertrophy complexes of the liver].
Biliary, portal, biliary/portal and hepatovenous obstruction have been recognized as the etiological factors leading to the atrophy/hypertrophy complex (AHC) of the liver. From 1987 to 1990 30 patients were evaluated in our department with documented significant AHC of the liver. The evaluation of these patients was performed at an interdisciplinary meeting of surgeons, gastroenterologists, radiologists and hepatologists. All patients were studied in regard to clinical, radiological and gastroenterological investigations, intraoperative findings and histology. We present the diagnostic pathway, which included ultrasound in 29 patients as the first diagnostic step and 26 patients in whom computed tomography was added. In 21 patients hepato-iodide scintigraphy was performed, in 14 patients ERCP and angiography, and in 8 patients PTC. Etiologically we found in 12 patients a post-cholecystectomy stricture (PCS) in 12 patients, a tumour obstruction in 9 patients, different etiologies (Echinococcus, Budd-Chiari syndrome, primary biliary cirrhosis [PBC], Mirizzi-syndrome) in 9 patients and an unclear etiology in 4 patients. On the basis of our study we present a new histological classification of the AHC. Severe histological changes were prognostically worse, even in benign diseases. Therapy planning is always dependent on a very precise diagnostic pathway and early recognition of AHC is essential for the planning of therapy and for the prognosis of the disease.
Adult↗