Unilateral Multicystic Kidney Dysplasia: Follow-Up during the First Two Years of Life

Author(s):  
H. Bachmann ◽  
J. Winkielman ◽  
H. Olbing
Keyword(s):  
2003 ◽  
Vol 14 (3) ◽  
pp. 177-186 ◽  
Author(s):  
F. Eckoldt ◽  
R. Woderich ◽  
S. Wolke ◽  
K.-S. Heling ◽  
B. Stöver ◽  
...  

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Teresa Bada Bosch ◽  
Angel Sevillano ◽  
Sara Afonso ◽  
Eduardo Gutierrez ◽  
Teresa Cavero Escribano ◽  
...  

Abstract Background and Aims Thin basement membrane nephropathy (TBMN), the most common cause of persistent microhaematuria (mH), is due to mutations in genes codifying alfa-3 and alfa-4 collagen IV chains (COL4A4/COL4A3). Initially considered as a benign condition, subsequent studies have shown that an important number of patients develop proteinuria and CKD. We reported in a previous small study the presence of multicystic kidney disease (MCD) in some TBMD patients. In this study we aimed to evaluate the presence of MCD in a larger cohort of TBMD patients and analyze its association with renal outcomes. Method We collected 50 patients with a diagnosis of TBMD based on the presence of persistent mH (>5 erythocytes per high power field in more than 90% of urinary sediments and radiological examinations to exclude other causes of mH) and at least one first-degree relative with persistent mH. TBMD diagnosis was confirmed by renal biopsy (glomerular basement membrane thickness less than 150nm) in 18 patients and by genetic test (pathogenic mutations in COL4A3/COL4A4) in 6 patients. MCD was diagnosed by the presence of uncountable cysts on renal ultrasonography. Results Mean age at diagnosis was 43.7 years, 34% were males and 18% had hypertension. At baseline, serum creatinine (SCr) was 0.9 mg/dL, proteinuria 0.48 gr/24h and 9 patients (18%) had CKD (estimated glomerular filtration rate -eGFR- lower than <60 mL/min/1.73m2). 7 patients (14%) had CKD G3 and 2 (4%) CKD G4. Kidney cysts were found in 34 patients (68%) and 19 (38%) met MCD criteria. After a mean follow-up of 14.7±11.5 years, 23 patients (46%) had CKD. Among them, 17 patients (34%) had CKD G3, 2 (4%) CKD G4, and 4 (8%) CKD G5. Hypertension was more frequent among CKD patients as compared with no-CKD patients (39 vs 0%, p 0.00), proteinuria was higher (0.58±0.68 vs 0.39±0.58 g/24h, p 0.05) and MCD more frequent (65.2% vs 14.8%, p 0.00). Patients with MCD had higher SCr (2.1 vs 1.1 mg/dL, p 0.004) and lower eGFR (41.7 vs 77.2 mL/min/1.73m2, p 0.00) at the end of follow-up, and MCD was the only risk factor for the occurrence of CKD (OR 6.49, 95% CI 1.3-31.6) by multivariable analysis that included age, hypertension and proteinuria. Conclusion MCD is frequently observed in TBMD patients and is a risk factor for the progression of CKD.


2010 ◽  
Vol 63 (3-4) ◽  
pp. 262-266 ◽  
Author(s):  
Sonja Pop-Trajkovic ◽  
Aleksandar Ljubic ◽  
Vesna Kopitovic ◽  
Vladimir Antic ◽  
Jelena Milosevic ◽  
...  

Introduction Congenital fetal anomalies are the great problem and one of the main causes of increased perinatal mortality and morbidity. The aim of this study is to determine the outcome of prenataly detected multicystic dysplastic kidney and to point to the necessity of postnatal diagnostic procedures. Material and methods The retrospective-prospective study encompasses 38 cases of the prenatally diagnosed unilateral fetal multicystic dysplastic kidney. The associated anomalies were revealed either by autopsy findings when the pregnancy was terminated, or when the pregnancy continued, by clinical and operative findings the newborns. Results The autopsy finding revealed bilateral multicystic displastic kidney or unilateral mylticystic displastic kidney and the agenesis of the contralateral kidney. The postnatal evaluation of the newborns with unilateral multicistic disease revealed that 84.3% of them had some concomitant anomaly of the urinary tract, most of them had an anomaly of the contralateral kidney (31.4%). The surgery was performed in 73.6% of children, in 17% of children the kidney function deteriorated after the surgery. Conclusion The findings of bilateral multicystic kidney disease and unilateral multicystic kidney disease and anamnion are the indication to terminate the pregnancy. The finding of an isolated unilateral multicystic dysplastic kidney require thorough examination, both prenatally and postnatally. We propose obligatory serial prenatal ultrasound examinations, followed by postnatal ultrasound, isotope scan, and urinary cystourethography.


2019 ◽  
Vol 42 ◽  
Author(s):  
John P. A. Ioannidis

AbstractNeurobiology-based interventions for mental diseases and searches for useful biomarkers of treatment response have largely failed. Clinical trials should assess interventions related to environmental and social stressors, with long-term follow-up; social rather than biological endpoints; personalized outcomes; and suitable cluster, adaptive, and n-of-1 designs. Labor, education, financial, and other social/political decisions should be evaluated for their impacts on mental disease.


1999 ◽  
Vol 173 ◽  
pp. 189-192
Author(s):  
J. Tichá ◽  
M. Tichý ◽  
Z. Moravec

AbstractA long-term photographic search programme for minor planets was begun at the Kleť Observatory at the end of seventies using a 0.63-m Maksutov telescope, but with insufficient respect for long-arc follow-up astrometry. More than two thousand provisional designations were given to new Kleť discoveries. Since 1993 targeted follow-up astrometry of Kleť candidates has been performed with a 0.57-m reflector equipped with a CCD camera, and reliable orbits for many previous Kleť discoveries have been determined. The photographic programme results in more than 350 numbered minor planets credited to Kleť, one of the world's most prolific discovery sites. Nearly 50 per cent of them were numbered as a consequence of CCD follow-up observations since 1994.This brief summary describes the results of this Kleť photographic minor planet survey between 1977 and 1996. The majority of the Kleť photographic discoveries are main belt asteroids, but two Amor type asteroids and one Trojan have been found.


Author(s):  
D.G. Osborne ◽  
L.J. McCormack ◽  
M.O. Magnusson ◽  
W.S. Kiser

During a project in which regenerative changes were studied in autotransplanted canine kidneys, intranuclear crystals were seen in a small number of tubular epithelial cells. These crystalline structures were seen in the control specimens and also in regenerating specimens; the main differences being in size and number of them. The control specimens showed a few tubular epithelial cell nuclei almost completely occupied by large crystals that were not membrane bound. Subsequent follow-up biopsies of the same kidneys contained similar intranuclear crystals but of a much smaller size. Some of these nuclei contained several small crystals. The small crystals occurred at one week following transplantation and were seen even four weeks following transplantation. As time passed, the small crystals appeared to fuse to form larger crystals.


Author(s):  
C. Wolpers ◽  
R. Blaschke

Scanning microscopy was used to study the surface of human gallstones and the surface of fractures. The specimens were obtained by operation, washed with water, dried at room temperature and shadowcasted with carbon and aluminum. Most of the specimens belong to patients from a series of X-ray follow-up study, examined during the last twenty years. So it was possible to evaluate approximately the age of these gallstones and to get information on the intensity of growing and solving.Cholesterol, a group of bile pigment substances and different salts of calcium, are the main components of human gallstones. By X-ray diffraction technique, infra-red spectroscopy and by chemical analysis it was demonstrated that all three components can be found in any gallstone. In the presence of water cholesterol crystallizes in pane-like plates of the triclinic crystal system.


1979 ◽  
Vol 10 (3) ◽  
pp. 139-144
Author(s):  
Cheri L. Florance ◽  
Judith O’Keefe

A modification of the Paired-Stimuli Parent Program (Florance, 1977) was adapted for the treatment of articulatory errors of visually handicapped children. Blind high school students served as clinical aides. A discussion of treatment methodology, and the results of administrating the program to 32 children, including a two-year follow-up evaluation to measure permanence of behavior change, is presented.


2020 ◽  
Vol 29 (2) ◽  
pp. 688-704
Author(s):  
Katrina Fulcher-Rood ◽  
Anny Castilla-Earls ◽  
Jeff Higginbotham

Purpose The current investigation is a follow-up from a previous study examining child language diagnostic decision making in school-based speech-language pathologists (SLPs). The purpose of this study was to examine the SLPs' perspectives regarding the use of evidence-based practice (EBP) in their clinical work. Method Semistructured phone interviews were conducted with 25 school-based SLPs who previously participated in an earlier study by Fulcher-Rood et al. 2018). SLPs were asked questions regarding their definition of EBP, the value of research evidence, contexts in which they implement scientific literature in clinical practice, and the barriers to implementing EBP. Results SLPs' definitions of EBP differed from current definitions, in that SLPs only included the use of research findings. SLPs seem to discuss EBP as it relates to treatment and not assessment. Reported barriers to EBP implementation were insufficient time, limited funding, and restrictions from their employment setting. SLPs found it difficult to translate research findings to clinical practice. SLPs implemented external research evidence when they did not have enough clinical expertise regarding a specific client or when they needed scientific evidence to support a strategy they used. Conclusions SLPs appear to use EBP for specific reasons and not for every clinical decision they make. In addition, SLPs rely on EBP for treatment decisions and not for assessment decisions. Educational systems potentially present other challenges that need to be considered for EBP implementation. Considerations for implementation science and the research-to-practice gap are discussed.


2019 ◽  
Vol 28 (3) ◽  
pp. 1039-1052
Author(s):  
Reva M. Zimmerman ◽  
JoAnn P. Silkes ◽  
Diane L. Kendall ◽  
Irene Minkina

Purpose A significant relationship between verbal short-term memory (STM) and language performance in people with aphasia has been found across studies. However, very few studies have examined the predictive value of verbal STM in treatment outcomes. This study aims to determine if verbal STM can be used as a predictor of treatment success. Method Retrospective data from 25 people with aphasia in a larger randomized controlled trial of phonomotor treatment were analyzed. Digit and word spans from immediately pretreatment were run in multiple linear regression models to determine whether they predict magnitude of change from pre- to posttreatment and follow-up naming accuracy. Pretreatment, immediately posttreatment, and 3 months posttreatment digit and word span scores were compared to determine if they changed following a novel treatment approach. Results Verbal STM, as measured by digit and word spans, did not predict magnitude of change in naming accuracy from pre- to posttreatment nor from pretreatment to 3 months posttreatment. Furthermore, digit and word spans did not change from pre- to posttreatment or from pretreatment to 3 months posttreatment in the overall analysis. A post hoc analysis revealed that only the less impaired group showed significant changes in word span scores from pretreatment to 3 months posttreatment. Discussion The results suggest that digit and word spans do not predict treatment gains. In a less severe subsample of participants, digit and word span scores can change following phonomotor treatment; however, the overall results suggest that span scores may not change significantly. The implications of these findings are discussed within the broader purview of theoretical and empirical associations between aphasic language and verbal STM processing.


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