Welche Therapie erfordert die pränatal diagnostizierte multizystische Nierendysplasie?

Abstract

Multicystic dysplastic kidneys (MCD) were found in 17 of 114 neonates with prenatal diagnosis of urinary tract malformations. Distribution of side and of sex was not different. Contralateral malformations were present in 3 infants. One of them with contralateral renal dysplasia and cardiac malformation died at the age of 4 weeks. All other children so far have a normal renal function. Three neonates presented with a palpable abdominal mass, 2 infants had urinary infections during the first year of life. Two neonates had obstruction of the contralateral kidney caused by the giant MCD which relieved after nephrectomy. Hypertension or development of malignancy were not noted. Nephrectomy was performed in 10 infants at the mean age of 3.2 months. Six infants had conservative treatment and a complete regression was noted in 5 of them within a period of 8 to 18 months. Prenatal diagnosis of MCD enables early recognition of contralateral urinary malformations and of problems caused by the MCD itself. Conservative treatment is recommended in all asymptomatic patients. Studies of the natural history may show that regression of MCD is the rule and could account for many cases with apparent unilateral renal agenesis.

Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney

“…Hypertension is a potential complication associated with MCDK [12,13,16,17,19,22,26]. There are only several case reports on hypertension in children with MCDK in the literature [2±4, 11,16,23].…”

“…Arterial hypertension is a potential complication of MCDK. The frequency of hypertension in patients with MCDK is considered to be very low but the estimated incidence in several studies varies widely from 0% to 8% [8,13,16,17,22]. In the two largest studies published so far, hypertension has been found only in 6 of 137 and in 4 of 260 patients [19,26] although neither study has focused primarily on blood pressure (BP).…”

Multicystic kidney dysplasia: a prospective study on the natural history of the affected and the contralateral kidney

“…As shown in this study and by others [3] the natural history is mostly a decrease in volume of the aected kidney with occasional total regression and compensatory hypertrophy of the contralateral kidney. Until recently, nephrectomy was recommended [6], whereas nowadays conservative management is almost generally accepted with US follow up [13].…”

Hypertension in Children with Cystic Kidney Diseases

“…There are only several case reports on hypertension in children with MCDK in the literature [60][61][62][63][64][65][66] but systematic investigations of BP in patients with MCDK are rare and based on clinic BP measurements only. The frequency of hypertension in children with MCDK in these studies has been shown to be relatively low with the prevalence ranging between 0 -8% [4,58,59,64,[67][68][69][70][71][72]. However Emmert and Webb [73,74] suggested in their papers that the risk of hypertension secondary to MCDK is underestimated and many children therefore receive suboptimal follow-up.…”