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

Aim of the study To compare analgesia and adverse effects during oral morphine and oxycodone and transdermal fentanyl and buprenorphine administration in cancer patients with pain. Patients and methods Cancer patients treated at home and in outpatient clinics with severe pain (numerical rating scale score 6–10) fail to respond to non-opioids and/or weak opioids. All patients were randomized to either morphine, oxycodone, fentanyl or buprenorphine and divided into subgroups with predominant neuropathic and nociceptive pain component. Doses of opioids were titrated to satisfactory analgesia and acceptable adverse effects intensity. Patients were assessed at baseline and followed for 28 days. In all patient groups, immediate-release oral morphine was the rescue analgesic and lactulose 10 mL twice daily was the prophylaxis of constipation; no antiemetics were used as prophylaxis. Results A total of 62 patients participated and 53 patients completed the study. Good analgesia was obtained for all 4 opioids, for both nociceptive and neuropathic pain. The use of co-analgesics was greater in patients with neuropathic pain. Morphine treatment was associated with less negative impact of pain on ability to walk, work and activity (trend) according to Brief Pain Inventory-Short Form scores and less consumption of rescue morphine. The most common adverse effects included nausea and drowsiness, which increased at the beginning of the treatment and gradually decreased over the days to come. Appetite, well-being, anxiety, depression, and fatigue improved. There was no constipation (the Bowel Function Index scores were within normal range) during the treatment with all opioids. No changes were seen for constipation, vomiting and dyspnea. Conclusion All opioids were effective and well-tolerated. Morphine was the most effective in the improvement in some of the Brief Pain Inventory-Short Form items regarding negative impact of pain on patients’ daily activities. Prophylaxis of constipation was effective; antiemetics may be considered for nausea prevention.

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This study was performed to explore serum tumor necrosis factor-like weak inducer of apoptosis (TWEAK) and its dependent cytokines urinary excretion: monocyte chemoattractant protein-1 (MCP-1) and regulated on activation, normal T cell expressed and secreted chemokine (RANTES) with their relation to the kidney function parameters in children with solitary functioning kidney (SFK). The study included 80 children and adolescents (median age 9.75 year) with congenital and acquired (after surgical removal) SFK. Serum TWEAK and urinary MCP-1 and RANTES levels were significantly higher in SFK patients (p < 0.05). The serum TWEAK was positively related to serum creatinine (r = 0.356; p < 0.001). Moreover, in SFK the receiver operating characteristic analyses revealed good diagnostic profile for serum TWEAK with AUC (Area Under The Curve)—0.853, uRANTES—0.757, and for RANTES/cr.: AUC—0.816. Analysis carried out to identify children with impaired renal function (albuminuria and/or decreased estimated glomerular filtration rate < 90 mL/min/1.73 m2 and/or hypertension) showed good profile for TWEAK (AUC—0.79) and quite good profile for uRANTES and RANTES/cr. (AUC 0.66 and 0.631, respectively). This is the first study investigating serum TWEAK and urinary excretion of MCP-1 and RANTES together in children with SFK. Obtained results indicate that TWEAK and RANTES may serve as potential markers of renal impairment.

This is the first comprehensive report on nephropathic cystinosis (NC) from Poland. To date, the prevalence, clinical course, genetic background, treatment and outcome of this rare inherited metabolic disease was unknown in this country. The results of this study indicate that the prevalence of NC in Poland is much lower than in Western countries, and moreover its molecular background appears to be different. The latter is based on infrequent occurrence of the common 57-kb deletion, and higher proportion of other pathogenic variants of the CTNS gene, mainly c.18_c.21delGACT and c.681+1G>A. It should be noted that an unfavorable course in majority of Polish patients was caused by a historically limited accessibility to the specific cystine depletion therapy with cysteamine. Fortunately, better access to diagnostic and therapeutic procedures has significantly improved patients' care in recent years.