Background and objectives: Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease. Endothelial dysfunction, an early and reversible feature in the pathogenesis of atherosclerosis, is associated with increased vascular smooth muscle tone, arterial stiffening, and increased intima-media thickness. Coronary flow velocity reserve is a noninvasive test showing endothelial function of epicardial coronary arteries and coronary microcirculatory function. The aim of the study was to investigate the carotid intima-media thickness and coronary flow velocity reserve in patients with autosomal dominant polycystic kidney disease.Design, setting, participants, & measurements: Thirty normotensive patients with autosomal dominant polycystic kidney disease (10 male, 20 female) with well-preserved renal function and 30 healthy subjects (12 male, 18 female) were included in the study. Coronary flow velocity reserve was measured at baseline and after dipyridamole infusion by echocardiography. Coronary flow velocity reserve was calculated as the ratio of hyperemic to baseline diastolic peak velocities.Results: Carotid intima-media thickness was significantly higher in patients than in control subjects (0.80 ؎ 0.29 versus 0.54 ؎ 0.14 mm, respectively; P < 0.001). Moreover, coronary flow velocity reserve was significantly lower in patients than in control subjects (1.84 ؎ 0.39 versus 2.65 ؎ 0.68, respectively; P < 0.001).Conclusions: Normotensive patients with autosomal dominant polycystic kidney disease with well-preserved renal function have significantly increased carotid intima-media thickness and significantly decreased coronary flow velocity reserve compared with healthy subjects. These findings suggest that atherosclerosis starts at an early stage in the course of their disease in patients with autosomal dominant polycystic kidney disease.
Both intrarenal and intravenous applications of NAC failed to show any benefit over placebo in the prevention of CIN. This result shows that NAC application does not have any prophylactic effect, dose dependent or otherwise, on CIN, as previously reported. Our results suggest that more attention should be paid to optimize hemodynamic variables for the prevention of CIN.
A 19-year-old female patient with hypercalciuria and recurrent nephrolithiasis/urinary tract infection unresponsive to thiazide type diuretics is presented. The patient first experienced nephrolithiasis at the age of 4 years. Afterwards, recurrent passages of stones and urinary tract infection occurred. On diagnostic evaluation at the age of 19 years, she also had hypocitraturia and hypomagnesemia. Her serum calcium concentrations were near the lower limit of normal (8.5–8.8 mg/dl; normal range: 8.5–10.5), her serum magnesium concentrations were 1.15–1.24 mg/dl (normal range: 1.4–2.5) and urinary calcium excretion was 900 mg/24 h. PTH concentrations were increased (110–156 pg/ml; normal range: 10–65). We tried to treat the patient with hydrochlorothiazide at a dose of 50 mg/day. During treatment with thiazide diuretics, PTH concentration remained high and the patient had recurrent urinary tract infections and passages of stones. Serum magnesium concentration did not normalize even under the parenteral magnesium infusion. Her mother had a history of nephrolithiasis 20 years ago. Severe hypomagnesemia in association with hypercalciuria/urinary stones is reported as a rare autosomal recessive disorder caused by impaired reabsorption of magnesium and calcium in the thick assending limp of Henle’s loop. Recent studies showed that mutations in the CLDN16 gene encoding paracellin-1 cause the disorder. In exon 4, a homozygous nucleotide exchange (G679C) was identified for the patient. This results in a point mutation at position Glycine227, which is replaced by an Arginine residue (G227R). The mother was heterozygous for this mutation. G227 is located in the fourth transmembrane domain and is highly conserved in the claudin gene family. This case indicates the pathogenetic role of paracellin-1 mutation in familial hypomagnesemia with hypercalciuria and nephrocalcinosis and further underlines the risk of stone formation in heterozygous mutation carriers.
Patient: Male, 28Final Diagnosis: Posterior reversible encephalopathy syndromeSymptoms: Headache • pain around umblical region • seizures • visual disturbancesMedication: Mycophenolate mofetilClinical Procedure: Treatment of parasitosis • antiepileptic treatment • control of hypertension • changing mycophenolate mofetil to everolimusSpecialty: TransplantologyObjective:Unusual or unexpected effect of treatmentBackground:Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible neurological findings with clinical hallmarks such as headache, confusion, seizures, cortical visual disturbances, and other focal neurological signs.Case Report:A 28-year-old male patient was hospitalized secondary to diarrhea and abdominal pain. He had renal transplantation due to renal amyloidosis secondary to familial Mediterranean fever (FMF). In his clinical follow-up, he had seizures, hemiparesis, blurred vision, and vomited an Ascaris lumbricoides. MRI results led to diagnosis of PRES. Mycophenolate mofetil was changed to everolimus, his systolic blood was pressure kept below 140 mm hg, and his intestinal parasitosis was treated. During follow-up, he had no pain and no diarrhea. His neurological symptoms turned to normal within 48 hours and neuroradiological findings returned to normal within 2 weeks.Conclusions:PRES is a rare disorder of unknown incidence in renal transplantation patients. Early diagnosis is very important to prevent irreversible neurological sequelae. PRES is totally reversible with cessation of the offending agent, rapid control of hypertension, and treatment of the underlying disease. For early diagnosis and to reduce morbidity and mortality, stool sample examination should be made in patients taking immunosuppressive drugs.
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