Multicystic dysplastic kidney disease and hypertension: Clinical and pathological correlation

Neonatal hypertension is increasingly recognized as dramatic improvements in neonatal intensive care, advancements in our understanding of neonatal physiology, and implementation of new therapies have led to improved survival of premature infants. A variety of factors appear to be important in determining blood pressure in neonates, including gestational age, birth weight, and postmenstrual age. Normative data on neonatal blood pressure values remain limited. The cause of hypertension in an affected neonate is often identified with careful diagnostic evaluation, with the most common causes being umbilical catheter-associated thrombosis, renal parenchymal disease, and chronic lung disease. Clinical expertise may need to be relied upon to decide the best approach to treatment in such patients, as data on the use of antihypertensive medications in this age group are extremely limited. Available data suggest that long-term outcomes are usually good, with resolution of hypertension in most infants. In this review, we will take a case-based approach to illustrate these concepts and to point out important evidence gaps that need to be addressed so that management of neonatal hypertension may be improved.

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“…In infants with MCDK with severe HTN, nephrectomy may be considered if BP control remains challenging. [81]…”

Section: Approach To Treatmentmentioning

confidence: 99%

Neonatal hypertension: cases, causes, and clinical approach

Starr

Flynn

2018

Pediatr Nephrol

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“…The site of renin production is usually the vascular polar macula densa; in our case, renin was partially localized in the proximal tubules and the parietal epithelium of the Bowman’s capsule in the immature glomeruli. Abdulhannan et al reported a similar case of a 2-month-old girl; however the serum renin and the renin expression in the renal specimen were not evaluated [ 30 ]. Furthermore, proximal tubular production of renin in the transgenic mice can result in a systemic increase in arterial pressure [ 31 ].…”

Section: Discussionmentioning

confidence: 99%

Pathophysiological clinical features of an infant with hypertension secondary to multicystic dysplastic kidney: a case report

et al. 2021

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Background The association of hypertension with congenital renal hypoplasia has been established. We report a case of an infant who underwent nephrectomy for hypertension. Case presentation Magnetic resonance imaging for the mother revealed fetal renal masses, and fetal multicystic dysplastic kidney was suspected. Following birth, the baby developed hypertension. Numerous investigations revealed that the left kidney was non-functional, and she was initiated on benazepril hydrochloride. However, because the drug response was poor, the left kidney was removed at the age of 7 months. Examination of the renal specimen revealed abrupt transition from normal to atrophic cortex with lobar atrophy and cysts. Tubular atrophy, marked abnormal blood vessels with wall thickening, gathered immature glomeruli, and parenchymal destruction were observed. Renin was partially localized in the proximal tubules and the parietal epithelium of the Bowman’s capsule in the immature glomeruli. We speculated that an abnormal vascular structure and irregular renin localizations may be the cause of hypertension. Serum renin and aldosterone levels gradually reduced post-surgery, reaching normal levels on the 90th postoperative day. A long follow-up is needed due to the possibility of the child developing hypertension in the future. Conclusion This is a case of an infant with MCDK, which discusses the clinicopathological features based on the pathophysiological analysis, including renin evaluation.

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“…Usually, the resolution of the cysts begins in the postnatal period and, in majority of the cases, the resolution is completed by the end of the first 18 months [5]. Therefore, the surgery for MCDK is rarely indicated and a surgery before 2 years of age is usually not indicated in an asymptomatic baby [8,10,12,13,14]. At the University Children's Hospital in Skopje the surgical extirpation of the MCDK was indicated only in one case in the last ten years -that was a baby with extremely large MCDK which filled the whole abdomen and seriously comprised the breathing.…”

Section: Discussionmentioning

confidence: 99%

Posterior Urethral Valve and Prenataly Resolved Multicystic Dysplastic Kidney

Aluloska

Palchevska²,

Симеонов³

et al. 2021

Prilozi

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Multicystic dysplastic kidney is a rare congenital anomaly of the kidney and urinary tract. The association with the posterior urethral valve is also very rare. Here we present a patient with both entities and prenatal resolution of the cysts. A 10-week old baby was referred for nephrourological work up due to prenatal diagnosis of the left multicystic kidney. He had serial US scans during the pregnancy. Immediately before delivery the cysts were not seen (prenatal resolution). There were no extrarenal anomalies. The first postnatal ultrasound scan revealed normal sized right kidney without dilatation of the pelvicalyceal system. The bladder had normal thickness of the wall. Technetium-99m dimercaptosuccinic acid scan showed no activity on the left side, and the right kidney appeared normal. At two months of age, a poor urinary steam was observed and additional urologic work up was indicated on clinical suspicion of PUV. Voiding urethrocystography revealed posterior urethral valve and the baby underwent cytoscopic valve resection. Conclusion: We present a rare association of two congenital anomalies of the kidney and urinary tract with prenatal involution of the multicystic dysplastic kidney that is extremely rare event as seen in our case. Presence of posterior urethral valve must be suspected in a male baby with a poor urinary stream even when his ultrasound scan of urinary system appears normal.

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Multicystic dysplastic kidney disease and hypertension: Clinical and pathological correlation

Cited by 16 publications

References 5 publications

Neonatal hypertension: cases, causes, and clinical approach

Neonatal hypertension: cases, causes, and clinical approach

Pathophysiological clinical features of an infant with hypertension secondary to multicystic dysplastic kidney: a case report

Posterior Urethral Valve and Prenataly Resolved Multicystic Dysplastic Kidney

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