Background Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease.

The concentration of leptin and apelin in HT-OB children was significantly higher than in the C and HT-NW group. A similar finding for leptin level was demonstrated in comparison of HT-NW with C group. In children with HT-OB, the lack of decline in nocturnal DBP was associated with significantly higher BMI and the Cole's Index value. Children with HT-OB and lack of decline in nocturnal SBP showed lower leptin and insulin concentrations, and lower values of HOMA-IR as compared with dipping children. Positive correlation in the whole study group was found between adipokines levels and BP measurements. In conclusion, children with primary HT show higher concentration of adipokines compared with their healthy peers. The data on relationship of serum adipokines levels with anthropometric and BP parameters in children may be helpful to clarify the sequence of disturbances in the cardiovascular system in adults, which requires further examination.

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Serious renal involvement in systemic diseases is common and generally constitutes a pivotal prognostic factor, making those pathology frequently seen in nephrology departments. A recent study even states that, among different medical subspecialists, nephrologists deal with the most complex patients, in terms of comorbidities and other complexity markers [2]. From this somehow eclectic nephrologist's perspective, it seems important to be aware of and keep a high level of suspicion for rare, non-renal, but potentially devastating complications of systemic diseases, like the one highlighted in this c l i n i c a l c a s e : t h e s e c o n d a r y h e m o p h a g o c y t i c lymphohistiocytosis (HLH). When HLH complicates a

A 14-year-old Caucasian girl with a history of primary hypoparathyroidism and unstable calcium and phosphorus levels and on ongoing treatment was admitted to the Department of Pediatric Nephrology because of the onset of nephrocalcinosis and difficulties achieving normocalcemia. Coexistence of hypoparathyroidism, oral candidiasis, dental enamel hypoplasia, and subclinical Hashimoto's disease was strongly suggestive for autoimmune polyglandular syndrome (APS) type I. One of the clinical implications of this diagnosis is the high probability of future occurrence of adrenal insufficiency and hence the importance of maintaining a high level of suspicion in case of the onset of symptoms like weakness, fainting, hypotonia, or hyperkaliemia. Addison's disease would, in fact, be quite challenging for the future management of this patient.This clinical quiz highlighted the importance of careful evaluation of all multiorgan symptoms occurring in a patient to prevent further complications.Keywords Nephrocalcinosis . Autoimmune polyendocrine syndrome type 1 . Hypoparathyroidism . Children Case presentationA 14-year-old Caucasian girl with a history of primary hypoparathyroidism and unstable calcium and phosphorus levels and on ongoing treatment was admitted to the Department of Pediatric Nephrology because of the onset of nephrocalcinosis and difficulties achieving normocalcemia. The obstetric, neonatal, and developmental history was unremarkable. The child was born at full term after a normal seventh pregnancy and fifth delivery, with a birth weight of 3150 g and an Apgar score of 10. An adenoidectomy was performed at the age of 6 years. The first symptoms of the disease appeared at the age of 9 years and consisted of several episodes of syncope and seizures. At presentation, apart from a white-coated tongue, dental caries, and enamel hypoplasia, the clinical, neurological, cardiovascular, and ophthalmic examinations were norm a l . L a b o r a t o r y t e s t s r e v e a l e d h y p o c a l c e m i a , hyperphosphatemia, and low serum parathyroid hormone (sPTH). Computed tomography (CT) and magnetic resonance (MR) imaging revealed bilateral calcifications in the basal ganglia and the frontal lobes (Fig. 1). The thyroid gland was slightly heterogeneous on ultrasound, and antithyroid peroxidase (anti-TPO) antibodies were present, without any other clinical or biochemical features of hypothyreosis. A treatment of primary hypoparathyroidism based on calcium supplementation and 1-α-hydroxycholecalciferol administration was prescribed. During a period of 3 years (2011)(2012)(2013)(2014), the dosage had to be progressively increased due to persisting hypocalcemia, to a maximum of 4.0 g of calcium carbonate and 1.75 μg of 1-α-hydroxycholecalciferol daily. In January 2015, the patient was hospitalized because of dyspnea, generalized weakness, polydipsia, nausea, and tachycardia. She was severely hypercalcemic at that moment but recovered rapidly after intensive fluid therapy, loop diuretics, and temporary withdrawal of th...

IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is the most common form of systemic vasculitis in children. Nephrological symptoms in the course of the disease are observed in 30-50% of children. The prognosis for maintaining normal kidney function is good in most patients; however, it depends on the primary clinical manifestation. Our aim was to present a case of a girl with nephropathy in the course of IgAV in the form of nephritic-nephrotic syndrome, who, despite early and intensive immunosuppressive treatment, revealed development of end-stage renal failure (ESRF) within two years. We also discuss the treatment of severe nephropathy in the course of IgAV, in accordance with current recommendations, which still remains a great challenge and does not guarantee slower progression of the disease.

Background: The most common disease associated with the presence of kidney cysts in the population is autosomal dominant polycystic kidney disease (ADPKD), which finally leads to end-stage renal disease. Method: The study evaluated serum and urinary concentration of angiotensinogen (AGT) and interleukin 18 (IL-18) in a group of 39 children with renal cysts of different aetiology. Results: Serum and urinary AGT concentration in children with renal cysts was higher compared to controls, regardless of the underlying background and gender. Serum IL-18 concentration was lower, in contrast, and the concentration of IL-18 in the urine did not differ between affected and healthy children. Negative correlation between urinary IL-18 concentration and systolic and mean arterial blood pressure was noted. Conclusions: Higher AGT levels in serum and urine in children with renal cysts may indicate the activation of the renin–angiotensin–aldosterone system, including its intrarenal part, even before the onset of hypertension. Lower serum concentration of IL-18 in children with kidney cysts may indicate the loss of the protective role of this cytokine with the occurrence of hypertension.

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