No abstract

Patient preparationPatients should refrain from drinking coffee, smoking cigarettes, and using other stimulants for at least 30 minutes before the measurement Measurement should be performed after at least 5 minutes of rest, in a sitting position with the back supported, in a quiet room at comfortable ambient temperature. The arm on which BP is measured should be flexed at the elbow and loosely supported at the level of the heart. The upper arm should be free from any restrictive clothing Measurement techniqueWhen measuring using the classical (auscultatory) technique, the cuff should be inflated to 30 mm Hg above the audible sounds (palpable pulse). The cuff should be deflated at a rate of 2 mm Hg/s Initially, BP should be measured on both arms, with further measurements on the arm with the higher BP reading for long-term BP monitoring and evaluation of the effectiveness of antihypertensive therapy With the auscultatory technique, systolic blood pressure (SBP) is defined as the appearance of the first tone during cuff deflation -Korotkoff phase I, and diastolic blood pressure (DBP) is defined as the disappearance of the last tone during cuff deflation -Korotkoff phase V BP should be calculated as the mean of 2 last measurements, the standard being at least 3 BP measurements performed 1-2 minutes apart during the same visit. If BP values differ between subsequent measurements (> 10 mm Hg), additional measurements should be performed BP measurements in patients with arrhythmia (e.g., atrial fibrillation) should be performed using the auscultatory technique At the initial evaluation, orthostatic challenge (active standing) test should be performed in all patients, with BP measurements at 1 and 3 minutes after standing up from the sitting position. Orthostatic hypotension is defined as SBP fall by ≥ 20 mm Hg or to < 90 mm Hg or DBP fall by ≥ 10 mm Hg. Active lying-to-standing test (standing up from the lying position) should be considered in the elderly, diabetic patients, and patients with other conditions associated with an increased risk of orthostatic hypotension. Extending orthostatic BP measurements to 5 minutes should be considered in these groups Pulse rate should be measured to exclude significant arrhythmia. Resting heart rate is also used for cardiovascular risk evaluation If a BP difference is found between the arms, the higher value should be taken as actual BP (preferred simultaneous BP measurement, and if not available -sequential BP measurement) BP -blood pressure; DBP -diastolic blood pressure; SBP -systolic blood pressure

BackgroundRegional citrate anticoagulation (RCA) is one of the methods used to prevent clotting in continuous renal replacement therapy (CRRT). The aim of this study was to describe the outcomes and complications of RCA-CRRT in comparison to heparin anticoagulation (HA)-CRRT in critically ill children.MethodsThis study was a retrospective review of 30 critically ill children (16 on RCA- and 14 on HA-CRRT) who underwent at least 24 h of CRRT. The mean body weight of the children was 8.69 ± 5.63 kg. RCA-CRRT was performed with a commercially available pre-dilution citrate solution (Prismocitrate 18/0).ResultsThe mean time on RCA-CRRT and HA-CRRT was 148.73 ± 131.58 and 110.24 ± 105.38 h, respectively. Circuit lifetime was significantly higher in RCA-CRRT than in HA-CRRT (58.04 ± 51.18 h vs. 37.64 ± 32.51 h, respectively; p = 0.030). Circuit clotting was observed in 11.63 % of children receiving RCA-CRRT and 34.15 % of those receiving HA-CRRT. Episodic electrolyte and metabolic disturbances were more common in children receiving RCA-CRRT. The survival at discharge from the hospital was 37.5 and 14.3 % among children receiving RCA-CRRT and HA-CRRT, respectively.ConclusionsIn critically ill children with a low body weight, RCA appeared to be safe and easy to used. Among our patient cohort, RCA was more effective in preventing circuit clotting and provided a better circuit lifetime than HA.

We report one of the largest cohorts of FHHNC cases caused by CLDN16 mutations. A missense variant of CLDN16, Leu151Phe, is the most common mutation responsible for FHHNC in Poland. Additionally, we found that normomagnesaemia does not exclude FHHNC and the calculation of fractional excretion of Mg can be diagnostic in the setting of normomagnesaemia. We also demonstrate the efficacy of a treatment with thiazides in terms of hypercalciuria in the majority of patients.

The role of substance P (SP) in physiological haematopoiesis is well established. However, it also seems to be important in the neoplastic transformation of bone marrow, leading to the development of acute leukaemia in children, and also metastases to bone marrow of solid tumours (particularly neuroblastoma and breast cancer) in early stages of these diseases. This review summarises the available data on SP involvement in both processes. In the future, SP antagonists may be used as anti-neoplastic drugs, for example by direct or indirect blocking of tumour cell proliferation through inhibition of growth factor production and interleukin-1b synthesis.

P < 0.001 (Fisher exact probability test).c l i n i c a l i n v e s t i g a t i o n AM _ Zurowska et al.: Mild Alport syndrome due to founder COL4A5 p.G624D AM _ Zurowska et al.: Mild Alport syndrome due to founder COL4A5 p.G624D c l i n i c a l i n v e s t i g a t i o n Kidney International (2021) -, ---AM _ Zurowska et al.: Mild Alport syndrome due to founder COL4A5 p.G624Dc l i n i c a l i n v e s t i g a t i o n

Chronic renal failure (CRF) is usually accompanied by abnormalities of both humoral and cellular immune response. The aim of the study was to investigate the influence of N-acetyl-cysteine (NAC) on intracellular oxidative stress and apoptosis rate of T lymphocytes in children with CRF. Twenty-two children (aged 4-16, mean 7.4) with CRF treated with dialysis were enrolled in the study. Intracellular reactive oxygen species (ROS) production was quantified by mean rhodamine 123 (RHO) fluorescence intensity with flow cytometry. Annexin V FITC was used for identifying apoptotic cells. Mean fluorescence intensity (MFI), which reflected intracellular oxidative stress in T lymphocytes, was increased in patients with CRF compared with the controls (CD3+: 31.58+/-11.58 vs 22.55+/-4.97, p = 0.043; CD3+CD4+: 32.50+/-8.59 vs 27.75+/-12.76, NS; CD3+CD8+: 32.10+/-11.85 vs 20.77+/- 4.89, p =0.012). Apoptotic T lymphocytes occurred more frequently in patients with CRF treated with hemodialysis (HD) (11.36+/-6.96%) than in the controls (6.14%+/-3.36%; p = 0.025). After 24 h incubation with NAC MFI and apoptosis rate decreased significantly in all subpopulations of lymphocytes. NAC, as a strong antioxidant, has a favorable effect on intracellular oxidative stress and apoptosis rate of T lymphocytes in patients with CRF. A decreased apoptosis rate may have positive effect on functional abnormalities of T cells already found in patients with CRF.

The increased permeability of the filtration barrier in steroid-resistant glomerulopathies may be a consequence of subcellular changes in podocytes resulting from decreased expression of synaptopodin. Moreover, impaired permeability of endothelium could be secondary to increased expression of podocyte-derived VEGF-C1.