Springer Science and Business Media LLC

Renal hypertension causes left ventricular (LV) hypertrophy leading to cardiomyopathy. Nephrectomy has been utilized to improve blood pressure and prepare for kidney transplantation in the pediatric population. We sought to investigate antihypertensive medication (AHM) requirement and LV mass in patients undergoing nephrectomy with renal hypertension. We performed a single institution retrospective review from 2009 to 2021 of children who have undergone nephrectomy for hypertension. Primary outcome was decrease in number of AHM. Secondary outcomes included change in LV mass and elimination of AHM. LV mass was measured using echocardiogram area-length and linear measurements. Non-parametric analyses were utilized to assess significance. Thirty-one patients underwent nephrectomy. Median age was 12.5 years (0.8–19 years). Median of 3 AHM (range 1–5 medications) were used pre-operatively and patients had been managed for median 2.5 years. Twenty-nine had preoperative echocardiogram. Forty-eight percent of patients had LVH at nephrectomy. Median AHM after surgery was 1 (range 0–4 medications) at 30 days and 12 months, (p < 0.001). By 12 months after nephrectomy, 79.2% of patients had decreased the number of AHM. Eight (26%) patients were on no AHM 30 days after surgery, and 13 (43%) at 12 months. Systemic vascular disease and multicystic dysplastic kidney were the only factors associated with lack of improvement in AHM (p = 0.040). Fourteen patients had pre- and post-operative echocardiogram and 11 (79%) had improvement in LV mass (p = 0.016, 0.035). Nephrectomy is effective in improving LV mass and reducing AHM for children with renal hypertension. Improvement is less likely in patients with systemic vascular disease and multicystic dysplastic kidneys.

The origin of proteinuria is found in either the glomerular filtration device or the proximal tubular reabsorption machinery. During equilibrium, small amounts of predominantly low molecular weight proteins are filtered and reabsorbed by the receptor complex megalin/cubilin/amnionless. This results in a protein-free filtrate passing further down the tubule. During glomerular damage, the reabsorption machinery in the proximal tubule is challenged due to elevated amounts of proteins passing the glomerular filtration slits. Even though it is considered to be a high-capacity system, several conditions result in proteinuria, thus exposing the cells in the rest of the nephron to a protein-rich environment. The impact on cells in the more distal part of the nephron is uncertain, but studies support an involvement in fibrosis development. Protein accumulation in lysosomes of the proximal tubule, due to increased protein internalization, is thought to mediate inflammation and fibrosis, eventually leading to renal failure. In contrast, low molecular weight proteinuria develops when the endocytic machinery is malfunctioning either by direct or indirect causes such as in Imerslund-Gräsbeck syndrome (IGS) or Dent’s disease, respectively. This review discusses the origin of proteinuria and describes the structural fundament for protein reabsorption in the proximal tubule as well as conditions resulting in low molecular weight proteinuria.

Studies have demonstrated that cyclin-dependent kinase inhibitors (CDKI) that inhibit cell-cycle progression have a protective effect against acute kidney injury (AKI). Most studies have focused on the CIP/KIP family members of CDKI; only a few have explored the role of INK4 family members in AKI. Because INK4 family members block the G1-S transition, we postulated that they should have protective effects against AKI. The most conserved INK4 member is p18, so we selected it to explore its effects on cisplatin-induced renal cell injury. We overexpressed p18 in renal tubular epithelial cells (LLC-PK1) by transient transfection and investigated its effects on the cell cycle and proliferation. After transfection, cell injury was induced by cisplatin (100 μM) incubation for 24 h in a standard medium. The effect of p18 was assayed by assessing cell necrosis and apoptosis in transfected cells. The endoplasmic reticulum stress (ERS) pathway was evaluated to interpret the possible mechanism of p18 action in cisplatin-induced renal cell injury. Overexpression of p18 arrested cell cycle progression in the G1 phase and inhibited proliferation. Compared with vehicle transfection, p18 overexpression did not affect cisplatin-induced necrosis, but it reduced the percentage of apoptotic cells significantly. The severity of ERS induced by cisplatin was also decreased by p18 overexpression. P18 protects against cisplatin-induced renal cell injury. The mechanism of p18 protection may lie in its effect on the cell death pathway.

Wilson’s disease (WD) is an autosomal recessive disorder, and has a variety of presentations. We reported a case of 9-year-old girl who presented with a history of recurrent gross hematuria, renal histological changes of IgA nephropathy, and finally had been confirmed to be Wilson’s disease-associated IgA nephropathy.

Nephrotic syndrome (NS) consists of the clinical triad of hypoalbuminaemia, high levels of proteinuria and oedema, and describes a heterogeneous group of disease processes with different underlying drivers. The existence of circulating factor disease (CFD) as a driver of NS has been epitomised by a subset of patients who exhibit disease recurrence after transplantation, alongside laboratory work. Several circulating factors have been proposed and studied, broadly grouped into protease components such as soluble urokinase-type plasminogen activator (suPAR), hemopexin (Hx) and calcium/calmodulin-serine protease kinase (CASK), and other circulating proteases, and immune components such as TNF-α, CD40 and cardiotrophin-like cytokine-1 (CLC-1). While currently there is no definitive way of assessing risk of CFD pre-transplantation, promising work is emerging through the study of ‘multi-omic’ bioinformatic data from large national cohorts and biobanks.

Capnocytophaga canimorsus, a bacterium rarely encountered by clinicians, was responsible for the development of peritonitis in an 18-year-old white male on automated peritoneal dialysis following the puncture of his dialysis tubing by a domestic cat. Although more than 100 cases of septicemia caused by C. canimorsus have been reported, this is the first report of the organism causing peritonitis in a patient receiving peritoneal dialysis. Of interest, the patient had a prior episode of peritonitis secondary to Pasteurella multocida, also following transmission from the same cat.

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