Eosinophilic cystitis-an obscure case of obstructive uropathy in infancy.

We report on an infant with features of intermittent obstructive uropathy, acute kidney injury, hypertension and type 4 renal tubular acidosis (RTA) despite urethral catheterisation and fluid resuscitation. Radiological findings showed upper tract dilatation, likely bilateral vesicoureteric junction obstruction and bladder base thickening which was concerning for possible malignancy. Renal biopsy demonstrated eosinophilic infiltrate, suggestive of kidney involvement. Bladder biopsy was diagnostic for eosinophilic cystitis (EC) showing mature degranulating eosinophils. EC is a rare, easily treatable and important differential of bladder mass in children which may present with an atypical obstructive uropathy. This report adds to the limited literature of this condition within the paediatric population. EC should be considered early in children presenting with eosinophilia, urinary tract obstruction and kidney dysfunction, with uncertain aetiology. This case also highlights the need for detailed imaging, including visualisation of the bladder base, in cases of likely obstructive uropathy.

Unilateral Multicystic Dysplastic Kidney Management: A National Survey.

Risks of contralateral kidney abnormalities and chronic kidney disease necessitate follow-up for unilateral multicystic dysplastic kidneys (MCDK). A nationwide survey of senior UK pediatricians was conducted. Of the 60 responses obtained, 62% routinely perform a dimercaptosuccinic acid scan to confirm diagnosis. Eight percent routinely perform a cystogram to investigate contralateral vesicoureteric reflux. Sixty-two percent would routinely measure renal function (frequency ranging from once only to "every 2 years"). Twenty-five percent recalled MCDK nephrectomy being performed within the previous 5 years. Respondents voiced concerns that national guidance may result in an overcautious approach but could balance consensus and safe variation, and offer families choice and reassurance. The mean estimated cost of follow-up from birth to 18 years ranged from £258 to £3854. Results demonstrate significant variation in management, highlighting the need for a clear pathway to decrease unwanted variability and to ensure those at high risk of renal sequelae are recognized early, without undue investigatory burden.

Variation in paediatric 24-h ambulatory blood pressure monitoring interpretation by Canadian and UK physicians.

Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is widely accepted as a more accurate method for measurement of blood pressure (BP) compared to a single office-based measurement of BP. However, it is unclear how physicians interpret ABPM and make management decisions. This study's goal is to investigate variation in ABPM interpretation among paediatric nephrologists (Canada and UK) and paediatric cardiologists (Canada only) via an online survey. The survey content included baseline demographics, questions on the use and indications for ABPM, interpretation of results, and subsequent management decisions in various clinical scenarios. The survey was sent to 196 Canadian physicians, with 69 (35.2%) total responses. Thirty-five UK clinicians also completed the survey. Most respondents were >44 years old, were in practice for at least 11 years, and were university-based. There were substantial differences among clinicians in ABPM interpretation for isolated systolic, diastolic, and night-time hypertension. For example, only 53.1% of physicians would initiate or modify treatment in those with diastolic HTN in CKD. Further, even for the same abnormal ABPM parameter, the decision to start or alter treatment was influenced by the underlying medical condition. There is significant variation in clinical practice among physicians for interpretation and management of hypertension when using ABPM. Differences in guidelines among various jurisdictions, as well as knowledge gaps in the research on which guidelines are based, create ambiguity regarding ABPM interpretation and management decisions. A more protocolized approach and further insight into the reasoning behind the variation in physicians' interpretation may help to standardise practice.

Middle aortic syndrome in children and adolescents.

Middle aortic syndrome is a rare form of renovascular disease that may present with severe hypertension during childhood. Narrowing of the abdominal aorta is often associated with narrowing of the renal and/or other visceral arteries and may be secondary to specific genetic syndromes. Following the optimization of blood pressure control, significant aortic narrowing often requires invasive management, including endovascular and surgical intervention. In younger children, endovascular therapy may be attempted in the first instance to acutely reduce the pressure gradient across the narrowing; however, a sustained benefit is rare. Once the child has grown to accommodate a graft of an adequate size, surgical therapy is indicated for patients in whom medical and/or endovascular management has not resulted in adequate blood pressure control. It is critical that individuals with middle aortic syndrome be managed by an experienced multidisciplinary team that includes medical, endovascular, and surgical expertise that can provide long-term care to monitor for recurrent hypertension and evidence of end-organ damage.

Hyperuricemia and Hypertension: Links and Risks.

Hyperuricemia has long been recognized to be associated with increased cardiovascular risk, including risk of developing hypertension. Epidemiological findings suggest that the link with hypertension is stronger in children and adolescents. Uric acid acts as a strong antioxidant compound in the extracellular environment but has pro-inflammatory effects within the intracellular setting. A chronic phase of microvascular injury is known to occur after prolonged periods of hyperuricemia. This is proposed to contribute to afferent arteriolopathy and elevation of blood pressure that may become unresponsive to uric acid-lowering therapies over time. Studies have struggled to infer direct causality of hyperuricemia due to a vast number of confounders including body mass index. The aim of this review is to present the available data and highlight the need for large scale prospective randomized controlled trials in this area. At present, there is limited evidence to support a role for uric acid-lowering therapies in helping mitigate the risk of hypertension.