Elderly men are underscreened for primary aldosteronism even in Hypertension Excellence Centre.

Purpose The Endocrine Society (ES) guidelines recommend screening for primary aldosteronism (PA) in high risk hypertensive patients presenting with at least one of seven criteria (resistant HTN, hypokalaemia, adrenal nodule, etc.) Although guidelines are clear and screening is simple, compliance rates among clinicians are extremely low. This results in underdiagnosis of early disease, leading to cadiovasculaer complications and the extra-burden of advanced chronic kidney disease. We aimed to evaluate the screening rates in our Nephrology and Hypertension clinics, as an example of a dedicated Hypertension Excellence Centre. Materials and methods Data on adult hypertensive patients was retrieved from January 2018 to December 2020. Included in the study were hypertensive patients who had at least one of the ES criteria for PA screening. Of all suitable patients, we compared those who were screened for PA to patients who were not screened. Univariate and multivariate cox regression analyses were used for comparison between groups. Results Of 661 patients with HTN, 218 patients (33%) met the ES guidelines for PA screening. Forty-six of them (21.1%) were referred for screening. Advanced age and male gender were associated with lower screening referral rates. Odds ratio for age was 0.945 for every year (95% CI 0.915 - 0.975). There was a trend towards decreased referral rate in advanced kidney disease. Conclusions A 21% screening rate, suggests that many cases of PA are likely missed, more often in older patients. We therefore advocate for PA screening of all hypertensive patients, especially elderly patients with CKD, in whom clinicians' awareness is low but the absolute risk is high.

Aldosterone is a hormone secreted from the adrenal gland.Oversecretion of aldosterone (Primary Aldosteronism [PA]) causes salt retention, urinary loss of potassium and difficult to control hypertension.Both hypertension and hyperaldosteronism act synergistically and cause, over time, severe cardiac, vascular and renal damage.Different guidelines support doctors' decision-making algorithm, suggesting who should be evaluated for aldosterone hypersecretion.Our study demonstrates that even in an expert hypertension centre, many candidates for screening are missed. Elderly men are specifically underscreened.Since PA is not as rare as once thought, and can have a devastating impact on patients' health, we suggest screening all hypertensive patients for autonomous hypersecretion of aldosterone.

Comparison of blood pressure measurements on the bare and sleeved arms - what does it uncover?

Accurate office blood pressure (BP) measurement remains crucial in diagnosing and managing hypertension. In this study, we aimed to compare BP measurements done over a bare arm versus a sleeved arm, while controlling all other possible sources of variance. We collected BP measurements of 100 hypertensive patients visiting a nephrology and hypertension clinic between January 2019 and December 2023. Measurements were taken by a single operator and according to the updated guidelines. BP measurements were performed first with one arm bare, and the other arm sleeved, with measurements taken simultaneously. Then, measurements were again taken simultaneously after exposing the arm which was first sleeved, and dressing the arm which was bare at first. A nonparametric Wilcoxon test was performed to compare each patient's measurements on each arm. No statistically significant differences were found between the sleeved and the bare arm measurements, with one exception of SBP measured on the left arm (slightly lower SBP on the bare arm). While looking at the absolute value of differences, the median difference was impressive with a 7-8 mmHg systolic difference and 5.5 mmHg diastolic difference. Our study revealed a robust and unpredicted effect of clothing on BP; in some patients, BP was increased while in others decreased. Therefore, we believe there is importance in measuring BP on bare skin, regardless of clothing or sleeve type.

Critical Stepwise Decline of Antibodies against SARS-CoV-2 among Chronic Hemodialysis Patients 180 Days Post Comirnaty Vaccine.

BACKGROUND: The reduced immune response of maintenance hemodialysis patients to coronavirus disease 2019 (COVID-19) vaccines is a major concern. OBJECTIVES: To analyze the late (6 months after full vaccination) antibody response and compare it to early post-vaccination titer. METHODS: We conducted a multicenter prospective study of 13 hemodialysis units in Israel. RESULTS: We demonstrated that the low titers observed among ESRD patients 2-3 months after vaccination with the Comirnaty vaccine (median 63.8 AU/ml) declined to critically lower values 6 months after full vaccination. (Mediananti S antibodies, 31 AU/ml). Seropositivity significantly declined among hemodialysis patients from 89% to 74% (P < 0.0001), although it did not significantly change among controls. CONCLUSIONS: We recommend all patients on hemodialysis receive a booster COVID-19 vaccine 6 months after the second dose.

Diminished and waning immunity to COVID-19 vaccination among hemodialysis patients in Israel: the case for a third vaccine dose.

BACKGROUND: Humoral responses to coronavirus disease 2019 (COVID-19) vaccines in hemodialysis (HD) patients can direct vaccination policy. METHODS: We compared 409 COVID-19-naïve HD patients from 13 HD units in Israel to 148 non-dialysis-dependent COVID-19-naïve controls. Twenty-four previously infected (antinucleocapsid positive) HD patients were analysed separately. Blood samples were obtained ≥14 days post-vaccination (BNT162b2, Pfizer/BioNTech) to assess seroconversion rates and titers of anti-spike (anti-S) and neutralizing antibodies. RESULTS: The median time from vaccination to blood sample collection was 82 days [interquartile range (IAR) 64-87] and 89 days (IQR 68-96) for HD patients and controls, respectively. Seroconversion rates were lower in HD patients compared with controls for both anti-S and neutralizing antibodies (89% and 77% versus 99.3%, respectively; P < 0.0001). Antibody titers were also significantly lower in HD patients compared with controls {median 69.6 [IQR 33.2-120] versus 196.5 [IQR 118.5-246], P < 0.0001; geometric mean titer [GMT] 23.3 [95% confidence interval (CI) 18.7-29.1] versus 222.7 [95% CI 174-284], P < 0.0001, for anti-S and neutralizing antibodies, respectively}. Multivariate analysis demonstrated dialysis dependence to be strongly associated with lower antibody responses and antibody titers waning with time. Age, low serum albumin and low lymphocyte count were also associated with lower seroconversion rates and antibody titers. HD patients previously infected with sudden acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had no difference in their seroconversion rates or antibody titers compared with COVID-19-naïve patients. CONCLUSION: This study demonstrates diminished and waning humoral responses following COVID-19 vaccination in a large and diverse cohort of HD patients, including those previously infected with SARS-CoV-2. Considering these results and reduced vaccine effectiveness against variants of concern, in addition to continued social distancing precautions, a third booster dose should be considered in this population.

A multidisciplinary nephrogenetic referral clinic for children and adults-diagnostic achievements and insights.

BACKGROUND: Genetic kidney diseases contribute a significant portion of kidney diseases in children and young adults. Nephrogenetics is a rapidly evolving subspecialty; however, in the clinical setting, increased use of genetic testing poses implementation challenges. Consequently, we established a national nephrogenetics clinic to apply a multidisciplinary model. METHODS: Patients were referred from different pediatric or adult nephrology units across the country if their primary nephrologist suspected an undiagnosed genetic kidney disease. We determined the diagnostic rate and observed the effect of diagnosis on medical care. We also discuss the requirements of a nephrogenetics clinic in terms of logistics, recommended indications for referral, and building a multidisciplinary team. RESULTS: Over 24 months, genetic evaluation was completed for a total of 74 unrelated probands, with an age range of 10 days to 72 years. The most common phenotypes included congenital anomalies of the kidneys and urinary tract, nephrotic syndrome or unexplained proteinuria, nephrocalcinosis/nephrolithiasis, tubulopathies, and unexplained kidney failure. Over 80% of patients were referred due to clinical suspicion of an undetermined underlying genetic diagnosis. A molecular diagnosis was reached in 42/74 probands, yielding a diagnostic rate of 57%. Of these, over 71% of diagnoses were made via next generation sequencing (gene panel or exome sequencing). CONCLUSIONS: We identified a substantial fraction of genetic kidney etiologies among previously undiagnosed individuals which influenced subsequent clinical management. Our results support that nephrogenetics, a rapidly evolving field, may benefit from well-defined multidisciplinary co-management administered by a designated team of nephrologist, geneticist, and bioinformatician. A higher resolution version of the Graphical abstract is available as Supplementary information.

Association of Nutrition Status at Dialysis Start With Long-Term Survival: A 10-Year Retrospective Study.

OBJECTIVE: The objectives of the study are to explore the association between nutritional status at the initiation of dialysis and the improvement or worsening of nutrition status during first 3 months of dialysis and first 5 years of survival on dialysis. METHODS: Two hundred ninety-seven patients who started dialysis between March 2009 and March 2019 were enrolled in the study. The nutritional status of the patients at dialysis commencement was evaluated by the method of The Integrative Clinical Nutrition Dialysis Score (ICNDS). Improvement or worsening of nutrition status was monitored by calculating the ICNDS slope for each patient enrolled in the study from 3 ICNDS values from the first 3 months on dialysis. The baseline ICNDS and the slope of 3 subsequent monthly ICNDS values were tested for correlation with the odds of all-cause mortality for each of the first 5 years on dialysis. RESULTS: There was a significant difference between the survival odds of patients who started dialysis with an ICNDS at 75 and those who started dialysis with an ICNDS <75 (hazard ratio [HR] 2.505, confidence interval [Cl] 1.235-5.079, P = .011 after 1 year on dialysis;, HR 1.543, Cl 1.083-2.198, P = .016 after 5 years). Deterioration of nutritional status (a negative ICNDS slope) during the first 3 months of dialysis was associated with increased mortality during 1-3 years after dialysis start, compared to a positive ICNDS slope indicating a stable or improved nutritional status (HR 1.732, Cl 1.151-2.607, P = .008 after 3 years on dialysis). CONCLUSIONS: Nutritional status at initiation of dialysis is associated with long-term (5 years) survival. Deterioration of nutritional status during the first 3 months on dialysis significantly increases the risk of death during the first 3 years on dialysis.

[HYPERTENSION IN A YOUNG WOMAN WITH ANXIETY DISORDER: THE CHICKEN OR THE EGG?]

INTRODUCTION: This is a case study of a thirty-five year old woman with a past medical history of anxiety disorder and hypertension which has been elevated up to 180/100 mmHg during the previous year. She had no cardiovascular risk factors or family history of hypertension. Her high blood pressure was initially attributed to emotional stress, however, she was later referred for additional evaluation for secondary causes of hypertension. Her lab test results demonstrated significantly elevated plasma aldosterone levels and suppressed renin levels. A computed tomography scan demonstrated a left adrenal mass consistent with adrenal adenoma, with a normal adrenal gland on the right. Immediately after left adrenalectomy, plasma aldosterone level normalized and blood pressure was controlled with only minimal pharmacotherapy. Approximately 10 days post-surgery, her blood pressure values were measured in a range of 125/90 and anxiety significantly improved, under treatment only with 12.5mg Atenolol.

[HYPERTENSION IN A PATIENT WITH AN ADEQUATELY TREATED COARCTATION OF THE AORTA - WHAT IS THE REASON FOR UNCONTROLLED HYPERTENSION?]

INTRODUCTION: A 35 year old patient, who had a successful surgical repair of coarctation of the aorta in early childhood, was referred for investigation regarding the cause for resistant hypertension. He underwent a full workup which was negative. Due to elevated renin levels his medications were altered with corresponding normalization of the renin levels. Symptomatic palpitations subsided after stopping treatment with a calcium channel blocker (lercanidipine), which implies reflex tachycardia secondary to lercanidipine. After all the investigations and interventions were performed, it appears that the etiology of resistant hypertension in his case is secondary to the coarctation, in spite of prior successful therapeutic interventions.

The effect of poly (ADP-ribose) polymerase inhibition on aminoglycoside-induced acute tubular necrosis in rats.

INTRODUCTION: Aminoglycosides (AG) cause nephrotoxicity in 10 - 20% of patients. One of the mechanisms is by generating reactive oxygen species (ROS), leading to DNA destruction and activation of poly(ADPribose) polymerase (PARP) causing necrotic tubular cell death. PARP inhibition on gentamicin-induced nephrotoxicity was studied. METHODS: 19 female Wistar-Kyoto rats divided into 3 groups: control (3 rats receiving no treatment); gentamicin-treated group (8 rats); and 8 rats treated with gentamicin combined with 3-aminobenzamide (3 AB). Kidney functions, protein, and gentamicin levels as well as urinary trypsin inhibitory activity (TIA) were measured. Tissue microscopic examination and immunohistochemical study for proliferative cell nuclear antigen (PCNA) were determined. The effect of PARP inhibitor on the bactericidal activity of gentamicin was also assessed. RESULTS: The following results were statistically significant: urea (mg/dL) 39.9 ± 5.86, 88.3 ± 50.3, and 48.5 ± 12.7 (p = 0.048); serum creatinine (mg/dL): 0.6 ± 0.26, 1.05 ± 0.7, 0.6 ± 0.06 (p = 0.043); proteinuria (mg/24-hours): 7.27 ± 3.65, 41.2 ± 18.1, and 17.6 ± 13.9 (p = 0.050); the number of tubular macronuclei (per 10 mm2): 18.33 ± 16.07, 218 ± 101.8, 41.7 ± 36.2 (p = 0.012); the number of dilated tubes (per 10 mm2): 61.67 ± 12.58, 276.3 ± 112.7, 140.0 ± 90.9 (p = 0.04); and the number of PCNA positive nuclei (per 10 mm2): 223.3 ± 95.69, 3,585 ± 2,215.3, 626.7 ± 236.9 (p = 0.034) in the control, gentamicin, and gentamicin+3AB-treated groups, respectively. The following biochemical and histologic parameters were also examined, however, they showed no statistically significant difference: TIA (p = 0.055), mitoses (p = 0.14), mononuclear infiltrate (p = 0.188), and intratubular cast formation (p = 0.084). No effect on bactericidal activity was observed. CONCLUSION: This study illustrates that PARP inhibitor significantly attenuates gentamicin-induced nephrotoxicity in rats with no effect on the bactericidal activity.

Nutrition assessment and risk prediction in dialysis patients-a new integrative score.

OBJECTIVE: We developed a quantitative nutritional score, based on biochemical measures, taken as part of monthly routine care. The score can be accomplished within a short time after routine laboratory results completion and identify a monthly change in nutritional status. DESIGN: A longitudinal observational cohort study SETTING: The Institute of Nephrology, Wolfson Medical Center, Holon, Israel. SUBJECTS: A total of 179 hemodialysis patients were followed up for up to 2.5 years after study baseline. INTERVENTION: The Integrative Clinical Nutrition Dialysis Score (ICNDS) is based on the biochemical measures of albumin, creatinine, urea, cholesterol, C-reactive protein, dialysis adequacy, and weight change. Each parameter is ranked between 1 and 5, with the higher rank derived from recommended National Kidney Foundation Kidney Disease/Dialysis Outcomes and Quality Initiative values and the lower rank indicating deviation from those values. The final ICNDS is the sum of ranks over 7 parameters. MAIN OUTCOME MEASURE: The Pearson correlation coefficient was calculated for association between subjective global assessment and ICNDS in 63 randomly selected patients. In 179 dialysis patients, the baseline ICNDS, the slope of 3 subsequent monthly ICNDS values, were tested for their correlation with odds of all-cause mortality, hospitalization frequency, length of stay, after 31 months. Spline Cox regression was used to select the best cutoff point, associated with severe mortality risk. RESULTS: Score results were significantly correlated with nutrition evaluation by subjective global assessment (r = 0.842, P < .01). For a unit increase in baseline score, death odds were significantly decreased (hazard ratio [HR] = 0.929, 95% confidence interval [CI] 0.88-0.974, P < .002). Each unit increase in slope significantly reduced mortality risk (HR = 0.485, 95% CI 0.278-0.847, P < .011). Hospitalization frequency was significantly increased across worsening baseline score (HR = 0.935, 95% CI 0.906-0.964, P < .0001). A 1-unit increase in slope significantly decreased hospitalization (HR = 0.799, 95% CI 0.726-0.881, P < .0001). CONCLUSIONS: Results confirm that ICNDS is a useful prognostic tool that serves to detect nutrition deterioration at its very beginning.

C-reactive protein and carotid and femoral intima media thickness: predicting inflammation.

BACKGROUND: C-reactive protein (CRP) is a recognized marker of systemic inflammation. Its association with carotid and femoral intima media thickness (surrogate measures of atherosclerosis) may explain excess cardiovascular disease risk in hemodialysis patients. OBJECTIVES: To estimate the association between CRP and both carotid and femoral IMT in hemodialysis (HD) patients; to predict CRP in these patients. METHODS: The present cross-sectional study is nested in the Sevelamer hydrochloride and ultrasound-measured femoral and carotid intima media thickness progression in end-stage renal disease (SUMMER) clinical trial. Carotid (common, internal, and bifurcation) and femoral arteries were visualized in B-mode ultrasonography. CRP was measured in serum. RESULTS: The study cohort included 144 HD patients (39.5% female, mean age 67.8 ± 11.5 years). All measures of both carotid and femoral IMT were significantly positively associated with CRP. Subjects with a history of smoking or coronary revascularization had significantly higher CRP levels, while subjects treated with sevelamer hydrochloride had significantly lower CRP. CRP was significantly positively associated with serum phosphorus, calcium, alkaline phosphatase, and PTH, and significantly inversely associated with HDL and albumin. CONCLUSIONS: CRP is significantly positively associated with both femoral and carotid IMT. Treatment with sevelamer hydrochloride is associated with lower CRP in HD patients.

Effect of atorvastatin on IgA nephropathy in the rat.

BACKGROUND: IgA nephropathy (IgAN) is the most common chronic glomerulonephritis in humans and is a major cause of end-stage kidney disease worldwide. There is no agreement on the exact underlying mechanism or therapeutic intervention for this disorder. Mesangial proliferation typifies the renal histopathology in IgAN. Statin drugs, as prenylationinhibitors, have been shown to have an antiproliferative effect on renal mesangial cells and to reduce IgAN-associated glomerulusclerosis and proteinuria. The aim of this study is to examine the effect of atorvastatin on kidney function, proteinuria and kidney histology changes in IgANinduced rats. METHODS: IgAN was induced in Wistar-Kyoto rats by bovine γ-globulin (BGG). Four groups of rats were treated in metabolic cages: 1) control; 2) atorvastatin (2 mg/kg body weight/day through nasogastric tube) - treated rats; 3) IgAN-rats; 4) IgAN-rats treated with atorvastatin. Urine volume, urine protein excretion, blood urea and creatinine concentrations in addition to creatinine clearance were examined every 14 days, throughout the duration of the study (56 days). All kidneys from sacrificed rats were examined for histology including glomerular cell nuclei count and immunofluorescence. RESULTS: There were no differences in blood creatinine concentrations between the groups. Creatinine clearance was lower on the 42nd day and proteinuria was higher on Days 14, 42 and 56, in rats in Group 3 compared to all others; additionally, histology examination revealed a higher glomerular cell nuclei count in this group. Immunofluorescence was equally positive for IgA in mesangial cells in the kidneys from rats of Groups 2, 3 and 4. CONCLUSIONS: Atorvastatin attenuates kidney-function impairment, proteinuria and mesangial cell proliferation in BGG model of IgANinduced rats.

Evidence for tissue iron overload in long-term hemodialysis patients and the impact of withdrawing parenteral iron.

BACKGROUND/AIMS: Erythropoiesis in long-term hemodialyzed (LTH) patients is supported by erythropoietin (rHuEpo) and intravenous (IV) iron. This treatment may end up in iron overload (IO) in major organs. We studied such patients for the parameters of IO in the serum and in major organs. METHODS: Patients were treated with rHuEpo (6-8 x 10(3) units × 1-3/wk) and IV 100 mg ferric saccharate. RESULTS: Of 115 patients, 21 had serum ferritin (SF) > 1000 ng/mL. This group was further analyzed. Their SF and transferrin saturation (TSAT) were 2688 ± 1489 ng/mL and 54.2 ± 32.7%, respectively (vs. 125-360 ng/mL and 20-50% in normal controls). Serum hepcidin was 60.1 ± 29.5 nm (vs. 10.61 ± 6.44 nm in controls) (P < 0.001). Nineteen patients had increased malonyldialdehyde, a product of lipid peroxidation, indicating oxidative stress. T2* MRI disclosed in 19 of 21 patients moderate to severe IO in the liver and spleen, in three of eight patients in the pancreas, but in no patient in the heart. After stopping IV iron for a mean of 12 months, while continuing rHuEpo, the mean SF decreased in 11 patients to 1682 ng/mL and the mean TSAT decreased to 28%, whereas hemoglobin did not change indicating that tissue iron was utilized. CONCLUSION: High SF correlates with IO in the liver and spleen, but not in the heart.

Medication apprehension and compliance among dialysis patients--a comprehensive guidance attitude.

BACKGROUND: Compliance with treatment regimens is a continuing challenge for chronic dialysis patients and their medical caregivers. Poor patient adherence to prescribed medications can adversely affect treatment outcome. STUDY DESIGN: In this pre- versus post-intervention study, 89 chronic dialysis patients [75 hemodialysis (HD), 14 continuous ambulatory peritoneal dialysis (CAPD); mean age 62.7 +/- 12.39 years, 34 females] responded to a written questionnaire designed to assess knowledge about and compliance with 5 groups of prescribed medications: metabolic drugs, antihypertensives, cardiac-supporting agents, peptic disease therapy and hematological replacement therapy. Mode of intake, storage, means of supply and source of information for each class of drug were also assessed. Patients then received both oral and written instructions regarding their prescribed medications (intervention). This information was repeated 3 months later. Six months after the intervention, patients were re-administered the questionnaires. Response to the questionnaires and laboratory data were compared prior to and following the intervention. RESULTS: Overall, compliance with prescribed medications significantly improved following the intervention, from 89 to 95.7%, p = 0.0007. This relative improvement was greater in HD than CAPD patients (27 vs. 2%, p < 0.0001). Improvement in compliance was associated with lower initial scores, fewer years of education, and longer dialysis vintage. Compared to baseline values, post-intervention blood hemoglobin, hematocrit, mean corpuscular volume, ferritin and Ca levels were significantly improved. CONCLUSIONS: Dialysis patients appear to benefit from receiving comprehensive guidance about medications, in terms of compliance with medications and blood chemistry and hematology measures.

A randomized controlled clinical trial comparing the efficacy of dead sea mineral-enriched body lotion versus two types of placebo in the treatment of cutaneous dryness, itching, peeling and tightness in hemodialysis patients (EDIT).

BACKGROUND/AIMS: The present study was designed to investigate the short-term safety and efficacy of topical application with body lotion enriched with minerals from the Dead Sea versus 2 different placebo treatments in reducing symptoms of uremic pruritus. METHODS: In this single-center, randomized, double placebo-controlled clinical trial, 78 hemodialysis patients with self-reported uremic pruritus were randomized to twice-daily topical treatment with body lotion enriched with minerals from the Dead Sea (DS) or to each of 2 types of placebo: (1) lotion with no Dead Sea minerals but otherwise identical to DS (P1) or (2) lotion with no active ingredients (P2). Symptoms of uremic pruritus (itching, dryness, peeling, tightness) were evaluated at baseline and 2 weeks (14 days) after treatment intervention using a 5-point Likert scale. RESULTS: Following treatment, significant differences in symptom severity scores between DS and P1 and, separately, between group DS and P2, were not detected. Additionally, when DS was compared to the combined placebo groups (P1 and P2 together), significant post-treatment differences in symptom severity scores were not observed. Symptoms were less severe post-treatment regardless of treatment assignment. CONCLUSIONS: DS was not superior to either of the placebo treatments in the symptomatic relief of uremic pruritus.

Familial pure proximal renal tubular acidosis--a clinical and genetic study.

BACKGROUND: Inherited proximal renal tubular acidosis (pRTA) is commonly associated with more generalized proximal tubular dysfunctions and occasionally with other organ system defects. Inherited combined pRTA and distal RTA with osteopetrosis and pure pRTA associated with ocular abnormalities, a rare disease which has been recently described. Only one family with pure isolated pRTA has been reported so far and the genetic cause for this disease is unknown. Objectives. We report a unique family with isolated pRTA. The aim of the project was to define the phenotype and to try to find the gene defect causing the disease. METHODS: Clinical and metabolic evaluation of all family members was performed and a family pedigree was constructed. DNA was extracted from blood samples of affected and unaffected family members. We amplified by PCR and sequenced the coding areas and splice-sites of the genes that contribute to HCO(-)(3) reclamation in the proximal tubule. The genes studied were as follows: CA II, CA IV, CA XIV, NCB1, Na(+)/H(+) exchanger (NHE)-3, NHE-8, the regulatory proteins of NHE3, NHRF1 and NHRF2 and the Cl(-)/HCO(-)(3) exchanger, SLC26A6. RESULTS: The father and all four children had RTA with blood HCO(-)(3) levels of 11-14 meq/l and urine pH of 5.3-5.4. Increased HCO(-)(3) fractional excretion after bicarbonate loading to 40-60% confirmed the diagnosis pRTA. No other tubular dysfunction was found, and no organ system dysfunction was detected, besides short stature. No mutation was found in all candidate genes studied. CONCLUSIONS: We presented a second family in the literature with familial isolated pure pRTA. The mode of inheritance is compatible with an autosomal dominant disease. Because of the small size of the family, wide genome search was not applicable and the gene candidate approach was chosen. Nine important candidate genes were extensively studied but the molecular basis of the disease was not yet found and genotyping nine important gene candidates were negative.

Antihypertensive effect of low calcium dialysis.

BACKGROUND: During maintenance hemodialysis acute elevation in serum calcium is common. Low calcium dialysis is advocated as a therapy for prevention of dialysis-induced hypercalcemia. Approximately 16% of our chronic hemodialysis patients experience elevated arterial blood pressure during the hemodialysis session, becoming hypertensive by the end of the treatment. All these patients exhibited post-dialysis hypercalcemia. OBJECTIVES: To investigate the effect of low calcium dialysis on post-dialysis hypertension in view of an evident link between serum calcium and blood pressure in both normal renal function and chronic renal failure patients. METHODS: We evaluated 19 chronic hemodialysis patients in whom both post-dialysis hypertension and PDHCa were observed. We investigated changes in serum total calcium, ionized calcium, intact parathormone levels and arterial blood pressure in response to 4 weeks low calcium dialysis as a treatment for PDHCa. RESULTS: When PDHT patients were treated with low calcium dialysis, post-dialysis blood pressure was significantly decreased compared to pre-dialysis values (155.3 +/- 9.7/82.2 +/- 7.9 mmHg pre-dialysis vs. 134.1 +/- 20.8/80 +/- 8.6 mmHg post-dialysis, P = 0.001). Additionally, post-dialysis blood pressure was significantly lower than post-dialysis blood pressure prior to the low calcium dialysis treatment (176.1 +/- 15/86 +/- 10.8 mmHg post-standard dialysis, 134.1 +/- 20.8/80 +/- 8.6 mmHg after low calcium dialysis, P = 0.001). A decline in post-dialysis serum calcium (2.34 +/- 0.2 vs. 2.86 +/- 0.12 mmol/L, P= 0.04) and ionized calcium (1.17 +/- 0.12 vs. 1.3 +/- 0.06 mmol/L, P = 0.03) compared to pre-dialysis levels was also achieved by this treatment, with no significant changes in iPTH levels. CONCLUSIONS: These data suggest a role for low calcium dialysis in treating acute serum calcium elevation and post-dialysis hypertension in patients receiving maintenance hemodialysis.

Neonates with extra-renal pelvis: the first 2 years.

Extra-renal pelvis (ERpel) is a common ultrasonographic finding among neonates who have undergone recurrent ultrasound examinations for a better definition of prenatal renal pelvic dilatation. This study tries to determine whether or not ERpel has important prognostic implications. Seventy-nine neonates (17 female) were examined. All had a diagnosis of prenatal renal pelvis dilatation, which was shown by postnatal ultrasound to be ERpel. Sixty ERpel neonates were examined 1.5 months to 2.5 months after the ultrasound (US) diagnosis by both Tc-99m diethylene triamine penta-acetic acid (DPTA) dynamic renal scanning and (99m)Tc-pertechnetate direct cystography. Clinical assessment, urine cultures and renal ultrasound follow-up were maintained for 2 years. The proportion of urinary tract infections (UTIs) in patients with ERpel was compared with that of the total neonatal and infantile population with normal US scans in the region of our hospital. Associated minor congenital malformations were found in 12 of 79 neonates (15.2%). Four had a family history of ERpel. Among 60 neonates who underwent renal scanning, 36 (60%) were found to have urinary retention in the collecting system. Another nine (15%) had vesico-ureteral (VU) reflux, of which seven had urinary retention. Fifteen (25%) showed normal isotope imaging. Urinary tract infection was diagnosed in 16 ERpel neonates in whom only one exhibited VU reflux (grade 2). The incidence of neonatal UTI in the ERpel group was more than that of either neonatal or infantile UTI in those with normal US scans in the local population (20.2% vs 1.2% and 4.3%, respectively). Fifty-three infants completed a 2-year follow-up. Repeat renal ultrasonography indicated that one infant (1.8%) had developed bilateral hydronephrosis, 12 (22.6%) had unchanged findings, 18 (40%) showed an improvement (decrease of ERpel width or resolution in one side) and, in 22 (41.5%) infants, the condition had resolved. No clinical or kidney function deterioration was observed. Seven patients (13.2%) each had one episode of UTI during the 2-year follow-up period; none of them had VU reflux. Neonatal ERpel is more frequent in male infants. It is associated with greater rates of minor congenital malformations, VU reflux and UTI than in the general population of the same ages. The increased UTI incidence is not attributed to VU reflux.