ObjectiveLight chain deposition has been shown to be an important histologic hallmark with differences in isotype, characteristics and ratio of kappa and lambda light chains having a significant role ...in pathobiology, pathogenesis and prognosis of several glomerular diseases. However, there is, to the best of our knowledge, no study dedicated to evaluating light chain deposits in patients with lupus nephritis (LN).MethodsWe have conducted a retrospective cohort study to evaluate the characteristics and prognostic significance of light chain deposition profile in the kidney of subjects with LN. We have collected data on demographics, clinical and laboratory parameters and histopathology (light, immunofluorescent and electron microscopy). Lambda domination (LD) was defined as lambda intensity – kappa intensity ≥ +1. SLE was diagnosed using the ACR criteria and renal outcomes per KDIGO.ResultsA total of 56 patients with LN were followed up for at least one year after kidney biopsy (79% women, mean age at biopsy 38±13 years). Mean number of glomeruli per biopsy sample was 26±12. A total of 42 (75%) patients had light chain deposition in the glomerulus with 4 (7%) having restricted lambda chain deposition and none had restricted kappa chain deposition. Mean immunofluorescent intensity was 1.6±1.0 for lambda and 1.8±1.0 for kappa light chain. A total of 12 (21%) patients had LD in the glomerulus. When examining renal outcomes at one year post-biopsy, 55% of patients achieved complete response (CR), 30% achieved partial response (PR) and 15% had no response. There were no differences in achievement of remission (CR or PR) between patients with vs. without light chain deposition (88% vs. 71%, p=0.60) as well as between those with vs. without LD (90% vs. 83%, p>0.99).ConclusionLight chain deposition is prevalent in LN, but LD is much lower than in IgA nephropathy. While their deposition did not affect renal outcomes in our patients, light chains are an important factor to consider in LN patients, especially where restriction is present and further work-up, primarily for hematologic disease, is needed. Further investigation of the potential effect of pathobiologic characteristics of light chains in LN is warranted.
Introduction: Intrauterine growth restriction (IUGR) is linked to a higher incidence of cardiovascular and renal diseases. Methods: A total of 91 healthy individuals were included, 40 women and 51 ...men, born below the 10th percentile of birth weight for gestational age. Anthropometric parameters, arterial pressure (AP), blood glucose, estimated glomerular filtration rate (eGFR), albumin/creatinine ratio, lipid profile, uric acid, renal volume by ultrasound, pulse wave velocity, central arterial pressure (cAP), and augmentation index (Aix) were measured. Results: Men have higher body mass index (BMI), waist circumference, ambulatory and continuous AP, lower eGFR, pulse, higher uric acid and LDL cholesterol, lower HDL cholesterol, higher cAP and Aix, higher corrected renal volume, and birth weight than females. Overweight men had hypertension, lower eGFR, and dyslipidemia more often. Systolic pressure correlated positively with BMI in men. In women, systolic pressure correlated positively with heart rate and negatively with gestational age. BMI affected the systolic pressure in men and eGFR in women. Conclusion: Results indicate the more unfavourable effect of IUGR on men. Higher AP, vascular dysfunction, poorer renal function, and dyslipidemia predispose men to earlier chronic disease development. Key words: intrauterine growth restriction, arterial pressure, renal function
This study aimed to analyze blood pressure (BP) patterns, assess blood pressure variability (BPV), and its possible determinants in children with essential hypertension. The study group included 132 ...children with essential hypertension without antihypertensive therapy. Anthropometric and laboratory parameters were evaluated, office and ambulatory BP were measured. BPV was defined as the standard deviation of BP for the day and nighttime periods. In addition to classical statistical analysis, an unsupervised machine learning approach using the expected maximization algorithm was implemented to find groups of patients with similar characteristics. No differences in BPV were observed between sexes; however, boys had higher levels of creatinine, serum glucose, and uric acid despite similar body mass index values. There was a significant correlation between the Z-score for body mass index and daytime systolic BPV (r=0.19, p<0.05). Nighttime BPV significantly correlated with total cholesterol and uric acid levels. Within the male population, two clusters were found. The subjects in Cluster 2 had higher daytime and nighttime systolic and diastolic BP values, total cholesterol, triglycerides, and nighttime systolic and diastolic BPV. Our results suggest that the clustering of metabolic factors influences BPV in untreated children with essential hypertension, which may be a sex-specific effect in males. Key words: Blood pressure variability; Hypertension; Ambulatory blood pressure monitoring
Lupus nephritis (LN) is one of the most severe features of systemic lupus erythematosus (SLE). Data on LN is scarce in the Croatian population. We analysed the characteristics of LN patients ...diagnosed at our tertiary referral centre. In this retrospective study, we analysed the following features of patients with biopsy-proven LN diagnosed between 2011 and 2020: demographics, renal laboratory parameters, renal histopathology, and treatment. A total of 38 patients were included (30 females; mean age 39+ or -15 years).The most common indication for kidney biopsy was proteinuria (89%). The proportion of LN classes was: class I (2.6%), II (5.3%), III (18.4%), IV (42.1%), V (13.2%), III+V (10.5%), IV+V (5.3%).The median time from SLE diagnosis to histologic confirmation of LN was 1.0 year. All patients were treated with methylprednisolone (MP), 68% received MP pulses. Induction treatment included intravenous (IV) cyclophosphamide (CYC) (71%) (15 patients treated per Euro-Lupus and 9 per the National Institutes of Health regimen), oral CYC (3%), or mycophenolate mofetil (11%). 79% of patients received antimalarials. While there is heterogeneity between different populations, our patient profile was similar to that from other European studies. Further follow-up of this group is necessary to assess outcomes in our population. Key words: systemic lupus erythematosus, lupus nephritis, treatment, population characteristics
•Fetal epigenetics determines telomere dynamics and health span in adulthood.•Environmental factors contributs to telomere length and dynamics.•Fetal telomere programing responds to environmental and ...physiological factors.
Advances in epigenetics now enable us to better understand environmental influences on the genetic background of human diseases. This refers especially to fetal development where an adverse intrauterine environment impacts oxygen and nutrient supply to the fetus. Recently, differences in telomere length and telomere loss dynamics among individuals born with intrauterine growth restriction compared to normal controls have been described. In this paper we propose possible molecular mechanisms that (pre)program telomere epigenetics during pregnancy. This programming sets differences in telomere lengths and dynamics of telomere shortening in adulthood and therefore dictates the dynamics of aging and morbidity in later life.
Concomitant treatment with drugs that inhibit drug metabolising enzymes and/or transporters, such as commonly prescribed statins and nonsteroidal anti-inflammatory drugs (NSAIDs), has been associated ...with prolonged drug exposure and increased risk of adverse drug reactions (ADRs) due to drug-drug interactions. The risk is further increased in patients with chronic diseases/comorbidities who are more susceptible because of their genetic setup or external factors. In that light, we present a case of a 46-year-old woman who had been experiencing acute renal and hepatic injury and myalgia over two years of concomitant treatment with diclofenac, atorvastatin, simvastatin/fenofibrate, and several other drugs, including pantoprazole and furosemide. Our pharmacogenomic findings supported the suspicion that ADRs, most notably the multi-organ toxicity experienced by our patient, may be owed to drug-drug-gene interactions and increased bioavailability of the prescribed drugs due to slower detoxification capacity and decreased hepatic and renal elimination. We also discuss the importance of CYP polymorphisms in the biotransformation of endogenous substrates such as arachidonic acid and their modulating role in pathophysiological processes. Yet even though the risks of ADRs related to the above mentioned drugs are substantially evidenced in literature, pre-emptive pharmacogenetic analysis has not yet found its way into common clinical practice.
Hemodijaliza (HD) je najčešća metoda nadomještanja bubrežne funkcije u svijetu. Tunelirani dijalizni kateteri (TDK) predstavljaju jednu od najčešće korištenih mogućnosti u osiguravanju žilnog ...pristupa za HD. Cilj retrospektivnog istraživanja bio je prikazati desetogodišnje iskustvo u postavljanju dijaliznih katetera u našem središtu. U razdoblju od 2012. do 2021. godine postavljeno je 1.660 dijaliznih katetera, od kojih su 80% bili TDK. Najčešće mjesto pristupa bila je desna unutarnja jugularna vena, potom lijeva unutarnja jugularna vena, femoralne vene te subklavijske vene. Svim bolesnicima dijalizni kateter je uspješno postavljen, a u postupcima je sudjelovalo dvanaest liječnika Zavoda za nefrologiju, od čega je sedam liječnika postavilo 89% svih katetera, a četvero
liječnika ima više od 300 postavljenih dijaliznih katetera po osobi. Zaključno možemo reći da organizirani programi postavljanja katetera osiguravaju velik broj bolesnika i iskustvo operaterima za učinkovito postizanje žilnog pristupa i najsloženijim bolesnicima.
Chronic kidney disease (CKD) is a serious illness with important consequences for patients and health systems. Estimation of prevalence and incidence, especially in early stages, is difficult due to ...a lack of epidemiological studies and consolidated registries. In general, the disease awareness is low, and thus CKD is not timely diagnosed in most cases. Robust screening programs are not implemented in Eastern European countries. A panel consisting of Primary Care Physicians and Nephrologists from Bulgaria, Croatia, Serbia, and Slovenia virtually met in December 2021 to discuss current CKD awareness and diagnostic approaches in the Balkan area The meeting resulted in specific calls to action in the region to improve the number and quality of epidemiology studies and the level of awareness among patients and medical communities, as well as implementation of screening programs in high-risk populations. Collaboration between specialists was acknowledged as a crucial driver for optimal management of patients with CKD. Joint efforts are required to persuade healthcare authorities to establish specific policies for better care of kidney patients.
The most common cause of rapidly progressive glomerulonephritis in elderly, antineutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN), demands immunosuppressive therapy (IS) regimen ...in a multi-morbid disease burdened population. Our aim was to assess outcome differences in two age groups. The study included a total of 38 ANCA-GN renal limited patients (18 men) treated from 1990 to 2018, of which 11 were 65 years of age and older (median 70, min. - max. 66 - 79 years), and 27 younger than 65 (median 55, min. - max. 23 - 64 years). All patients were treated with mono/combination of IS. Most commonly applied IS in elderly was combination of IV cyclophosphamide and corticosteroids (CS) (in 9 81.8%), while in younger it was a combination of CS and cyclophosphamide or rituximab (59.2%). Older patients had comparable mortality (3, 14.8% vs. 4, 27.3%; P = 0.369), malignancies (1, 3.7% vs. 1, 9.1%; P = 0.5) and infectious complications (10, 46.7% vs. 7, 63.6%; P = 0.388). Ten patients at the end of the follow up were at renal replacement therapy (RRT), with no difference between age groups (6, 22.2% vs. 4, 36.4%; P = 0.369). Interestingly, from initial need for RRT, half of the younger and older patients recovered with IS. Our findings give more credit to the current paradigm to treat elderly ANCA-GN patients with IS therapy due to the similar outcome of elderly as younger ones. Key words: Antineutrophil Cytoplasmic antibodies, Glomerulonephritis, elderly, Immunosuppression, Mortality, Kidney failure
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