Magnetic resonance imaging based kidney volume assessment for risk stratification in pediatric autosomal dominant polycystic kidney disease

IntroductionIn the pediatric context, most children with autosomal dominant polycystic kidney disease (ADPKD) maintain a normal glomerular filtration rate (GFR) despite underlying structural kidney damage, highlighting the critical need for early intervention and predictive markers. Due to the inverse relationship between kidney volume and kidney function, risk assessments have been presented on the basis of kidney volume. The aim of this study was to use magnetic resonance imaging (MRI)-based kidney volume assessment for risk stratification in pediatric ADPKD and to investigate clinical and genetic differences among risk groups.MethodsThis multicenter, cross-sectional, and case-control study included 75 genetically confirmed pediatric ADPKD patients (5–18 years) and 27 controls. Kidney function was assessed by eGFR calculated from serum creatinine and cystatin C using the CKiD-U25 equation. Blood pressure was assessed by both office and 24-hour ambulatory measurements. Kidney volume was calculated from MRI using the stereological method. Total kidney volume was adjusted for the height (htTKV). Patients were stratified from A to E classes according to the Leuven Imaging Classification (LIC) using MRI-derived htTKV.ResultsMedian (Q1-Q3) age of the patients was 6.0 (2.0–10.0) years, 56% were male. There were no differences in sex, age, height-SDS, or GFR between the patient and control groups. Of the patients, 89% had PKD1 and 11% had PKD2 mutations. Non-missense mutations were 73% in PKD1 and 75% in PKD2. Twenty patients (27%) had hypertension based on ABPM. Median htTKV of the patients was significantly higher than controls (141 vs. 117 ml/m, p = 0.0003). LIC stratification revealed Classes A (38.7%), B (28%), C (24%), and D + E (9.3%). All children in class D + E and 94% in class C had PKD1 variants. Class D + E patients had significantly higher blood pressure values and hypertension compared to other classes (p > 0.05 for all).DiscussionThis study distinguishes itself by using MRI-based measurements of kidney volume to stratify pediatric ADPKD patients into specific risk groups. It is important to note that PKD1 mutation and elevated blood pressure were higher in the high-risk groups stratified by age and kidney volume. Our results need to be confirmed in further studies

Diyabetik hastalarda asimetrik dimetil arjinin ile otonomik disfonksiyon arasındaki ilişki

Amaç: Diabetes Mellitus toplumda yaygın prevelansa sahip bir hastalıktır. Endotel disfonksiyonu ve otonomik disfonksiyon bu hastalığın morbidite ve mortalitesinin altında yatan onemli nedenlerdendir. Bizim bu calışmadaki amacımız endotel disfonksiyonu belirteci olarak kabul goren asimetrik dimetil arjinin ile otonomik disfonksiyon belirteci olarak kabul goren kalp hızı değişkenliği ve kalp hızı turbulansı parametreleri arasında ilişki olup olmadığını incelemektir. Yöntem: Calışmaya Kardiyoloji ve Endokrinoloji Bolumlerine başvuran diyabetik olan hastalar ve diyabetik olmayan bireyler alındı. Calışmaya toplam 142 birey dahil edildi. Bunlardan 67&#8217;si oral antidiyabetik ilac ile takipli diyabetik grubu, 33&#8217;u insulin ile takipli diyabetik grubu, 42&#8217;si de kontrol grubunu oluşturdu. Hastalardan en az sekiz saat aclık sonrası, serum asimetrik dimetil arjinin seviyesi bakmak icin kan orneği ile hemogram, bobrek ve karaciğer fonksiyon testleri ile lipit profili goruldu. Otonomik fonksiyonları değerlendirmek uzere 24 saatlik Holter elektrokardiyografik inceleme yapıldı. Kalp hızı değişkenliği ve kalp hızı turbulansı hesaplandı. Bulgular: Her iki diyabet grubunda da kalp hızı değişkenliği parametreleri, kontrol grupla karşılaştırıldığında azalmış olarak bulundu (p<0.05). Gruplar arasında kalp hızı turbulansı parametrelerine bakıldığında, turbulans başlangıcı gruplar arasında faklılık gostermiyordu (-0.0095, -0.0107, -0.0135, p=0.8). Turbulans eğimi diyabetik gruplarda kontrol grubuna gore azalmakla beraber, bu azalma istatistiki olarak anlamlı bir duzeye ulaşmıyordu (6.1 }4, 4.6 }3, 7.3 }5, 0.057). Serum asimetrik dimetil arjinin seviyeleri diyabetik gruplarda kontrol grubuna gore yuksek idi (0.706, 0,708, 0,645, p=0.007). Asimetrik dimetil arjinin duzeyleri diyabetik gruplar arasında faklılık gostermiyordu. Korelasyon analizi ile değerlendirildiğinde serum asimetrik dimetil arjinin seviyeleri ile kalp hızı değişkenliği ve kalp hızı turbulansı parametreleri arasında istatistiki olarak anlamlı bir ilişki saptanmadı. Sonuç: Serum asimetrik dimetil arjinin duzeyleri diyabetik hastalarda diyabetik olmayan bireylere gore artmıştır. Kalp hızı değişkenliği ve kalp hızı turbulansı diyabetik hastalarda diyabetik olmayan bireylerle karşılaştırıldığında azalmıştır. Serum asimetrik dimetil arjinin duzeyleri ile kalp hızı değişkenliği ve kalp hızı turbulansı parametreleri arasında anlamlı bir ilişki gosterilememiştirObjective: Diabetes Mellitus is a highly prevelant disease in community. Endothelial dysfunction and autonomic dysfunction are crucial reasons that underlie the morbidity and mortality of disease. Our aim in present study was to look for if any relationship exist between asymmetric dimethyl arginine which is widely accepted as a marker of endothelial dysfunction and heart rate variability and heart rate turbulence parameters which are accepted as marker of autonomic dysfunction. Method: Diabetic patients and non-diabetic individuals admitted to Departments of Cardiology and Endocrinology were included in the study. Totally 142 individuals were admitted to study. 67 of these formed the group of diabetic patients followed up with oral antidiabetics, 33 patients formed the group of diabetic patients that followed up with insulin, and 42 individuals formed the control group. Blood samples were taken to evaluate serum asymmetric dimethyl arginine, hemogram, kidney and liver functions and lipid profile at least after eight hours of fasting. To evaluate autonomic functions 24 hour Holter electrocardiographic monitorization was performed. Heart rate variability and heart rate turbulence were calculated. Results: In both of diabetic groups, heart rate variability parameters were reduced when compared to control group (p<0.05). When heart rate turbulence parameters were evaluated, turbulence onset was not differing between groups (-0.0095, -0.0107, -0.0135, p=0.8). Although turbulence slope was reduced in diabetic groups compared to control group, it did not reached to statistical significance (6.1 }4, 4.6 }3, 7.3 }5, 0.057). Serum asymmetric dimetil arginin levels were higher in diabetic groups compared to control group (0.706, 0,708, 0,645, p=0.007). Asymmetric dimetil arginin levels were not differing between diabetic goups. When correlation analysis was performed, no statistically significant relationship was found between serum asymmetric dimetil arginin levels with heart rate variability and heart rate turbulence parameters. Conclusion: Serum asymmetric dimetil arginin levels are increased in diabetic patients compared to non diabetic individuals. Heart rate variability and heart rate turbulence parameters are decreased in diabetic patients when they are compared with non diabetic individuals. Significant relationship between serum asymmetric dimetil arginine levels with heart rate variability and heart rate turbulence parameters couldn&#8217;t be found.

Wpływ leczenia statynami na morfologię załamka P i czas przewodzenia przedsionkowego

Background and aim: The aim of this study was to evaluate the effect of statin treatment on P-wave morphology, dispersion, and tissue Doppler imaging-derived atrial conduction time (PA-TDI), which are known to be predictors of atrial fibrillation (AF). Methods: A total of 132 patients with guideline-directed statin indications but no clinical atrial tachyarrhythmias were studied. P-wave duration, P-wave dispersion, and P-wave amplitude on surface 12-lead electrocardiogram and PA-TDI were evaluated before and after three months of statin (either atrovastatin 10–40 mg/d or rosuvastatin 10–20 mg/d) treatment. Results: Total and low-density lipoprotein cholesterol were significantly reduced after statin therapy. P-wave dispersion significantly decreased from 39.6 ± 9.4 to 36.9 ± 9.6 ms. Statin treatment significantly decreased both the maximum (from 1.5 ± 0.36 to 1.45 ± 0.33 mV, p = 0.001) and the minimum (from 1.07 ± 0.28 to 1.04 ± 0.27 mV, p = 0.01) P-wave amplitude. The PA-TDI value was found to be significantly shorter after statin treatment (121.7 ± 18.7 vs. 118.7 ± 15.8 ms, p = 0.016) Conclusions: Short-term statin therapy was shown to significantly affect P-wave amplitude, P-wave dispersion, and atrial conduction time in a broad range of patients without any clinical atrial tachyarrhythmia.Wstęp i cel: Celem niniejszego badania była ocena wpływu leczenia statynami na morfologię i dyspersję załamka P oraz czas przewodzenia przedsionkowego określonego w obrazowaniu metodą doplera tkankowego (PA-TDI) uznawany za czynnik predykcyjny migotania przedsionków (AF). Metody: Przeanalizowano dane 132 chorych ze wskazaniami do stosowania statyn, zgodnie z wytycznymi terapeutycznymi, ale bez klinicznych objawów tachyarytmii przedsionkowej. Przed rozpoczęciem terapii i po 3-miesięcznym leczeniu statynami (atorwastatyna 10–40 mg/d. lub rosuwastatyna 10–20 mg/d.) oceniano czas trwania, dyspersję i amplitudę załamka P w powierzchniowym 12-odprowadzeniowym elektrokardiogramie oraz określano czas PA-TDI. Wyniki: Stężenie cholesterolu całkowitego i cholesterolu frakcji LDL było istotnie niższe po leczeniu statynami. Nastąpiło istotne zmniejszenie dyspersji załamka P z 39,6 ± 9,4 do 36,9 ± 9,6 ms. Terapia statynami spowodowała znamienną redukcję maksy­malnej (z 1,5 ± 0,36 do 1,45 ± 0,33 mV; p = 0,001) i minimalnej amplitudy załamka P (z 1,07 ± 0,28 do 1,04 ± 0,27 mV; p = 0,01). Czas PA-TDI był istotnie krótszy po leczeniu statynami (121,7 ± 18,7 vs. 118,7 ± 15,8 ms; p = 0,016) Wnioski: Wykazano, że krótkotrwała terapia statynami istotnie wpływa na amplitudę i dyspersję załamka P oraz czas przewodzenia przedsionkowego w zróżnicowanej grupie chorych bez objawowej tachyarytmii przedsionkowej

Korelacje między wskaźnikiem czynności mięśnia sercowego i osoczowym stężeniem peptydu natriuretycznego typu B u chorych na cukrzycę typu 2 lub z nieprawidłową tolerancją glukozy

Background and aim: In this study, we aimed to evaluate myocardial functions in patients with diabetes mellitus (DM) and impaired glucose tolerance (IGT). We also aimed to investigate the relationship between B-type natriuretic peptide (BNP) levels and myocardial performance index (Tei index) in these patients. Methods: A total of 38 patients with DM, 34 patients with IGT, and 40 healthy volunteers were recruited to the study. Basal clinical and laboratory findings were recorded. BNP levels of all individuals were measured. Both conventional transthoracic and tissue Doppler echocardiogaphy were performed to all study participants. Results: B-type natriuretic peptide levels of the diabetic group were greater than in patients with IGT and the control group. BNP levels of the IGT group were also higher than the control group. Myocardial performance index values, measured by both the conventional method and tissue Doppler echocardiography, were significantly higher in the diabetic group than in the control group. There was a significant relationship between myocardial performance index and BNP levels. Conclusions: Myocardial functions are disturbed in patients with DM and also in patients with IGT. BNP and myocardial performance index can be used in diabetic patients and in patients with IGT to define myocardial dysfunction.Wstęp i cel: Niniejsze badanie przeprowadzono w celu oceny czynności mięśnia sercowego u chorych na cukrzycę (DM) i u osób z nieprawidłową tolerancją glukozy (IGT) oraz ustalenia zależności między stężeniem peptydu natriuretycznego typu B (BNP) w osoczu i wskaźnikiem czynności mięśnia sercowego (wskaźnik Tei) u tych chorych. Metody: Do badania włączono 38 chorych na DM, 34 osoby z IGT i 40 zdrowych ochotników. Zgromadzono dane z wyjściowego badania klinicznego i wyniki badań laboratoryjnych. U wszystkich uczestników zmierzono stężenie BNP w osoczu oraz przeprowadzono badanie echokardiograficzne, zarówno konwencjonalne przezklatkowe, jak i z zastosowaniem tkankowego doplera. Wyniki: Stężenie BNP w osoczu było wyższe u chorych na DM niż u osób z IGT i w grupie kontrolnej. Z kolei u osób z IGT stężenie BNP było wyższe niż w grupie kontrolnej. Wartości wskaźnika Tei, oznaczonego zarówno techniką echokardiografii konwencjonalnej, jak i tkankowego doplera, były istotnie wyższe u chorych na DM niż w grupie kontrolnej. Stwierdzono istotną zależność między wskaźnikiem Tei a stężeniem BNP w osoczu. Wnioski: U chorych na DM i u osób z IGT czynność mięśnia sercowego jest upośledzona. Stężenie BNP w osoczu i wskaźnik Tei mogą być przydatne w określeniu zaburzeń czynności miokardium u tych chorych

Evaluation of PSMA PET/CT imaging using a Ga-68-HBED-CC ligand in patients with prostate cancer and the value of early pelvic imaging

PurposeThe aim of the study was to evaluate the diagnostic value of the prostate-specific membrane antigen (PSMA) ligand Ga-68-HBED-CC (PSMA PET/CT) in patients with prostate cancer and evaluate the value of early imaging of the pelvis.Materials and methodsThe files of 28 patients were retrospectively evaluated. All patients had a histopatological confirmation of prostate cancer. PSMA PET/CT images were obtained at 5 and 60min after injection from all patients.ResultsIntense pathologic radiotracer uptake was observed in 23 patients (77%) at the site of primary tumour. Lymph node metastases were detected in 10 patients (36%) and bone metastases were detected in seven patients (25%). Bone scan (n=25) results revealed metastatic bone lesions in four patients, equivocal results in nine patients and normal results in 12 patients. PSMA PET/CT confirmed bone metastases in all four patients. Pathologic radiotracer uptake in PSMA PET/CT scans was observed only in one patient among those who had equivocal bone scans. PSMA PET/CT showed additional bone lesions in two patients who had a normal bone scan. When we compared early and late pelvic images we found no difference in the number of lesions detected. The maximum standardized uptake value (SUVmax) for primary tumour, lymph nodes and bone metastases was significantly higher in late images.ConclusionPSMA PET/CT imaging seems to be a valuable imaging modality for evaluation of primary prostate cancer and it seems to have potential for the detection of lymph node and bone metastases. Early images 5min p.i. can help to better distinguish between urinary bladder (before tracer accumulation occurs) and tumour lesions

Atrial Electromechanical Delay and Diastolic Dysfunction in Primary Sjögren Syndrome

Purpose: In this study we aimed to investigate myocardial function and atrial electromechanical properties by conventional and tissue doppler echocardiography in patients with primary Sjögren syndrome. Methods: Forty patients with Sjögren syndrome (SS) and 25 age- and sex-matched healthy volunteers were enrolled in the study. Using transthoracic echocardiography, myocardial performance index and atrial electromechanical properties were measured. Results: Basal characteristics were similar between two groups. Myocardial performance index values were disturbed in patients with Sjögren syndrome (0.41 vs. 0.32, p < 0.01). There was significant intraatrial (16.4±6.4, 5.0±4.5, p < 0.01) and interatrial (30.6±10.1, 15.4±5.9, p < 0.01) electromechanical delay in this patient group. Conclusion: Myocardial function is disturbed and there is significant atrial electromechanical delay in patients with primary SS. This study is the first to show altered myocardial function and atrial electromechanical properties in primary SS