scholarly journals Fetal multicystic kidney disease: Outcome and follow up

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.


2009 ◽  
Vol 66 (9) ◽  
pp. 733-737
Author(s):  
Sonja Pop-Trajkovic ◽  
Aleksandar Ljubic ◽  
Vladimir Antic ◽  
Milan Trenkic

Background/Aim. Multicystic dysplastic kidney represents a disorder in the fetus development presented prenatally of postnatally, this deserving special attention due to a risk of additional anomalies in children with this disorder. The aim of this study was to determine the incidence and type of common anomalies of the urinary system in the prenatal diagnosis of unilateral multicystic dysplastic kidney, and point out the necessity of postnatal diagnostic procedures in order to evaluate the state of the urinary system. Methods. This retrospective-prospective study encompassed 38 cases of prenatally diagnosed unilateral fetal multicystic dysplastic kidney, presented to the Council for Fetal Anomalies from the Institute for Gynecology and Obstetrics of the Clinical Centre of Serbia and the University Children's Clinic within a three-year period. Associated anomalies were revealed by autopsy findings when pregnancy was terminated, ie resumed with clinical and operative findings of born children. Results. In every case of terminated pregnancy and death after birth the autopsy revealed additional renal or exstrarenal anomaly which were not prenatally detected. Postnatal evaluation of survived children with unilateral multicistic disease revealed that 31,4% of them have an anomaly of the contralateral kidney, 26,3% anomaly of the ipsilateral side, 13,2% anomaly of the lower portions of the urinary system and the same percent an additional extrarenal anomaly. The surgery was performed in 73,6% of children, more than half of the interventions were related to extrarenal anomaly. In 17% of children the kidney function was deteriorated after surgery. Conclusion. Children suffering from unilateral multicystic dysplastic kidney have a greater chance of exhibiting an anomaly of the contralateral kidney and the urinary system in general. Therefore, they require thorough examination, both prenatally and postnatally. We propose obligatory serial professional prenatal ultrasound examinations, followed by postnatal ultrasound, isotope scan, and we especially emphasize the need for performing urinary cystouretherography, bearing in mind the high incidence of the vesicoureteral reflux of the contralateral kidney. In addition to nephrectomy, cytoscopy and colposcopy also need to be performed for the purpose of discovering possible hidden anomalies of the urogenital system.



2008 ◽  
Vol 23 (1) ◽  
pp. 111-116 ◽  
Author(s):  
Adam Weinstein ◽  
T. Rob Goodman ◽  
Sandra Iragorri


1995 ◽  
Vol 25 (4) ◽  
pp. 275-277 ◽  
Author(s):  
K. Kaneko ◽  
Y. Suzuki ◽  
Y. Fukuda ◽  
K. Yabuta ◽  
T. Miyano


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.





Radiology ◽  
1967 ◽  
Vol 89 (5) ◽  
pp. 857-860 ◽  
Author(s):  
Anthony F. Lalli


Renal Failure ◽  
2005 ◽  
Vol 27 (2) ◽  
pp. 189-192 ◽  
Author(s):  
Said Al-Ghwery ◽  
Abdulrahman Al-Asmari


2006 ◽  
Vol 175 (4S) ◽  
pp. 186-186
Author(s):  
Bulent Onal ◽  
Barry A. Kogan


2017 ◽  
Vol 27 (06) ◽  
pp. 533-537 ◽  
Author(s):  
Vijai Upadhyaya ◽  
Manish Gupta ◽  
Laxmi Bharti ◽  
Ram Rao ◽  
Sheo Kumar ◽  
...  

Objective Surgery is still indicated as the treatment of choice in subset of patients with unilateral multicystic dysplastic kidney (UMCDK) because of its potential complications and malignant change. The purpose of this study is to present our observation that early nephrectomy may cure hypertension early in children with UMCDK and review the literature. Materials and Methods We report here four children (two males and two females) with antenatally diagnosed UMCDK with hypertension, treated in the past 4 years. All have antenatal diagnosis of UMCDK and referred to us after their birth. Diagnosis of hypertension (blood pressure > 95th percentile) was made after 3 months of follow-up in all patients. Nephrectomy was performed in all children in variable time duration. Hypertension, urinary tract infection, and desire of parents were the main indication of surgery. Results All children showed decrease in the size of their dysplastic kidney with time, but hypertension was persistent and needed antihypertensive drugs; even increase in the doses in successive follow-up. After nephrectomy, three children who were operated early got cured having normal blood pressure within variable time duration, while a child, operated late, still had high blood pressure and needed antihypertensive drugs. Conclusion Early nephrectomy in recently diagnosed hypertension in UMCDK is advisable and can cure hypertension early. Levels of Evidence The level of evidence is IV (case series with no comparison group).





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