Inherited renal cystic diseases

Kim, Bohyun; King, Bernard F.; Vrtiska, Terri J.; Irazabal, María V.; Torres, Vicente E.; Harris, Peter C.

doi:10.1007/s00261-016-0754-3

Cited by 12 publications

(17 citation statements)

References 30 publications

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“…1 Currently, the classification of diseases related to the presence of cysts in the kidneys is of an etiological basis and includes, apart from the family history, also the clinical picture, the location and morphology of the cysts as well as the presence of extrarenal symptoms. 2 Genetically caused cystic kidney diseases were systematized according to a new classification developed by Kim et al 2 Of these, ADPKD is the most common. [3][4][5][6] Diagnosis of ADPKD due to the typical symptoms, initially before confirmation by genetic testing, is based on family history and imaging.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of liver-type fatty acid binding protein (L-FABP) and interleukin 6 in children with renal cysts

Plesiński¹,

Świętochowska²,

Morawiec-Knysak³

et al. 2019

Adv Clin Exp Med

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Background. Renal cysts, according to their etiology, can be divided into genetic and acquired cysts. This is of great importance in patients with cystic kidney disease with a possible poor prognosis to identify markers of early kidney damage. Objectives. The objective of this study was to evaluate the concentration of serum and urine liver-type fatty acid binding protein (L-FABP) and interleukin 6 (IL-6) in children with kidney cysts. Material and methods. The study was conducted on a group of 39 children with kidney cysts including 20 subjects with autosomal dominant polycystic kidney disease (ADPKD). Results. Serum and urine L-FABP concentration in children with renal cysts was significantly higher compared to the controls, regardless of the underlying type of cystic degeneration, number of cysts and gender. Also, serum and urinary IL-6 concentration was significantly higher than in the control group. There was a significant negative correlation between serum L-FABP concentration and standard deviation score (SDS) for diastolic blood pressure (DBP). A significant negative correlation was found between serum IL-6 concentration and systolic blood pressure (SBP), DBP and mean arterial pressure (MAP) values as well as SDS for SBP and DBP. In addition, a significant positive correlation was found between urinary IL-6 concentration and estimated glomerular filtration rate (eGFR). Conclusions. Higher concentration of L-FABP in serum and urine in children with kidney cysts indicates the early damage to the renal parenchyma, detectable before the onset of hypertension and other organ damage. Significantly higher serum and urinary IL-6 levels in children with cystic kidney disease compared to healthy children may suggest the role of this cytokine in chronic kidney disease development.

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Section: Introductionmentioning

confidence: 99%

Evaluation of liver-type fatty acid binding protein (L-FABP) and interleukin 6 in children with renal cysts

Plesiński¹,

Świętochowska²,

Morawiec-Knysak³

et al. 2019

Adv Clin Exp Med

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“…Renal cysts, according to their aetiology, can be divided into acquired and genetically determined cysts. 1,2 A positive family history, clinical picture, location and morphology of the cyst, the presence of extra-renal symptoms are important clues in the diagnostic process. 2 The most common kidney cystic disease is autosomal dominant polycystic kidney disease (ADPKD), which occurs at a frequency of 1/500–1000 of live births and affects about 12.5 million people worldwide.…”

Section: Introductionmentioning

confidence: 99%

Angiotensinogen and interleukin 18 in serum and urine of children with kidney cysts

Plesiński¹,

Adamczyk

Świętochowska

et al. 2019

J Renin Angiotensin Aldosterone Syst

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Background: The most common disease associated with the presence of kidney cysts in the population is autosomal dominant polycystic kidney disease (ADPKD), which finally leads to end-stage renal disease. Method: The study evaluated serum and urinary concentration of angiotensinogen (AGT) and interleukin 18 (IL-18) in a group of 39 children with renal cysts of different aetiology. Results: Serum and urinary AGT concentration in children with renal cysts was higher compared to controls, regardless of the underlying background and gender. Serum IL-18 concentration was lower, in contrast, and the concentration of IL-18 in the urine did not differ between affected and healthy children. Negative correlation between urinary IL-18 concentration and systolic and mean arterial blood pressure was noted. Conclusions: Higher AGT levels in serum and urine in children with renal cysts may indicate the activation of the renin–angiotensin–aldosterone system, including its intrarenal part, even before the onset of hypertension. Lower serum concentration of IL-18 in children with kidney cysts may indicate the loss of the protective role of this cytokine with the occurrence of hypertension.

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“…The renal cystic kidney diseases most commonly diagnosed in adults are ADPKD and acquired renal cystic disease. While renal cystic disease commonly involves both kidneys (with the exception of multicystic dysplastic kidney), unilateral multiple cystic lesions have been reported and described as unilateral polycystic kidney disease, localized cystic disease of the kidney, or unilateral renal cystic disease [11, 12]. Previous reports suggest unilateral renal cystic disease most often demonstrates imaging features similar to ADPKD except that it involves a single kidney or a segmental area of a kidney [13, 14].…”

Section: Introductionmentioning

confidence: 99%

Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic

et al. 2019

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Purpose The aim of this study was to clarify the radiologic and clinical characteristics of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney. Method Fourteen patients with unique and characteristic multiple hemorrhagic subcapsular cortical cysts of the kidney, not categorized in any existing renal cystic diseases, were retrospectively reviewed. The clinical information including age, sex, symptom, family history of renal or renal cystic disease, and laboratory data were collected. CT and MRI findings including distribution, number and size of cysts, and CT attenuation and signal intensity on T1- and T2-weighted MRI of cysts were analyzed. Results All patients except one were young and none had a family history of renal or renal cystic disease. Common clinical symptoms were flank or abdominal pain and hematuria. In all cases, only the left kidney was involved at initial presentation. Cysts were small (median cyst size, 4–15 mm), numerous, and distributed mainly along the subcapsular cortex of the kidney. Cysts were hyper-attenuated on unenhanced CT, extremely hypointense on T2-weighted MRI, and mildly hyperintense on T1-weighted MRI. All patients except one had normal renal function. Imaging follow-up revealed stable or mildly progressive disease in seven patients. Two patients developed several hemorrhagic subcapsular cortical cysts in the right kidney at follow-up. Three of five patients with a renal pathology specimen showed concurrent IgA nephropathy. Conclusion We have identified a unique renal cystic disease with multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney that has a characteristic manifestation both radiologically and clinically.

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Inherited renal cystic diseases

Cited by 12 publications

References 30 publications

Evaluation of liver-type fatty acid binding protein (L-FABP) and interleukin 6 in children with renal cysts

Evaluation of liver-type fatty acid binding protein (L-FABP) and interleukin 6 in children with renal cysts

Angiotensinogen and interleukin 18 in serum and urine of children with kidney cysts

Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic

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