Background Autosomal recessive polycystic kidney disease (ARPKD) is a significant cause of morbidity and mortality in infants and children. In severe cases bilateral nephrectomies are considered but may be associated with significant neurological complications and life-threatening hypotension. Case presentation We describe a case of a 17 months old boy with genetically confirmed ARPKD who underwent sequential bilateral nephrectomies at the age of 4 and 10 months. Following the second nephrectomy the boy was started on continuous cycling peritoneal dialysis with blood pressure on the lower range. At the age of 12 months after a few days of poor feeding at home the boy experienced a severe episode of hypotension and coma of Glasgow Come Scale of three. Brain magnetic-resonance imaging (MRI) showed signs of hemorrhage, cytotoxic cerebral edema and diffuse cerebral atrophy. During the subsequent 72 h he developed seizures requiring anti-epileptic drug therapy, gradually regained consciousness but remained significantly hypotensive after discontinuation of vasopressors. Thus, he received high doses of sodium chloride orally and intraperitoneally as well as midodrine hydrochloride. His ultrafiltration (UF) was targeted to keep him in mild-to-moderate fluid overload. After two months of stable condition the patient started to develop hypertension requiring four antihypertensive medications. After optimizing peritoneal dialysis to avoid fluid overload and discontinuation of sodium chloride the antihypertensives were discontinued, but hyponatremia with hypotensive episodes reoccurred. Sodium chloride was reintroduced resulting in recurrent salt-dependent hypertension. Conclusions Our case report illustrates an unusual course of blood pressure changes following bilateral nephrectomies in an infant with ARPKD and the particular importance of tight regulation of sodium chloride supplementation. The case adds to the scarce literature about clinical sequences of bilateral nephrectomies in infants, and as well highlights the challenge of managing blood pressure in these patients. Further research on the mechanisms and management of blood pressure control is clearly needed.

The global prevalence of arterial hypertension (AH) in the pediatric population is increasing, but therapeutic approaches and the choice of the most suitable antihypertensive medications remains challenging. The study aimed to estimate the prevalence, treatment rates, and pharmacological treatment patterns of children and adolescents with AH in Lithuania during 2019 using real-world data. The study population consisted of citizens of Lithuania aged 0 to 17 years, who were diagnosed with AH according to the International Classification of Diseases (ICD). The analysis of reimbursed antihypertensive medication prescriptions was performed according to AH etiology and age. The overall prevalence of AH by diagnostic ICD codes in 2019 was 0.29%:0.24% for primary and 0.05% for secondary. Treatment rates were 39.8% for primary AH and 66.3% for secondary AH. Angiotensin-converting enzyme inhibitors (ACEi) were the most popular medications irrespective of the etiology of AH or age. Beta-blockers were in the second place and used more often in older children. Calcium channel blockers were the third and angiotensin receptor blockers were the fourth most frequent choices. Enalapril was the most popular agent in the ACEi group and metoprolol in the beta-blocker group. Nearly forty percent of Lithuanian children with primary AH receive pharmacological therapy compared to two-thirds with secondary AH. Although ACEi are the predominant class of antihypertensive medications, discordances with available guidelines are evident, particularly in the overuse of beta-blockers and underuse of diuretics.

Kidney cysts are the most common kidney lesion, while congenital kidney cysts are mostly found in pediatric population. Neonatal kidney cysts can develop due to fetal malformations, rare genetic disorders or can be acquired which is very rare. Kidney cysts may be the only isolated finding or be part of the overall phenotype. They can be asymptomatic, found by ultrasound accidentally or can manifest from mild to life-threatening symptoms. Therefore, early diagnosis is very important. Autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease are the most common causes of kidney cysts in the neonatal population. This review highlights the most common kidney cystic diseases during the neonatal period and a rare clinical case of HNF1B-associated disease.