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Last updated: 2019-07-11 22:10
Cystic Kidney
A kidney cyst is a small sac of fluid that develops in the kidneys.
Advanced polycystic kidney disease with multiple cysts
CT of peripelvic cysts with non-contrast and urography
Presentation
- Cystic disorders can present during infancy, childhood, or adulthood.[ncbi.nlm.nih.gov]
- The patient presented with a subcutaneous nodule, which led to discovery of a large primary tumor in the left kidney. ACKD was present in the atrophic right kidney.[ncbi.nlm.nih.gov]
- The proband, a 33-year-old woman, presented with renal cysts, mild renal failure and a renal tumor. One of the proband's two sons had hyperechogenic kidneys and the other had renal cortical microcysts.[ncbi.nlm.nih.gov]
- A 19-year-old African female presented with a chronic severe debilitating right lower quadrant abdominal pain refractory to analgesics.[ncbi.nlm.nih.gov]
- At present, ce-US in children can only be performed off-license, since the only approved US contrast agent (CA) for children has been taken off the market.[ncbi.nlm.nih.gov]
Entire Body System
Dysostosis
- Ischiospinal dysostosis (ISD) is a recently described entity characterized by ischial hypoplasia and spinal segmental anomalies.[ncbi.nlm.nih.gov]
Respiratoric
Cheyne-Stokes Respiration
- He was admitted at that age because of progressive respiratory problems, with Cheyne-Stokes respiration and bilateral vocal cord paralysis. Terminal renal failure with cystic renal disease was diagnosed at the age of 14 years.[ncbi.nlm.nih.gov]
Anosmia
- The clinical features associated with cilia dysfunction can include such phenotypes as polycystic kidneys, skeletal abnormalities, blindness, anosmia, and obesity.[ncbi.nlm.nih.gov]
Gastrointestinal
Abdominal Pain
- A patient with end-stage renal disease on maintenance hemodialysis developed sudden severe abdominal pain and distension.[ncbi.nlm.nih.gov]
- The patient reported nausea, vomiting, severe abdominal pain, and loose stools.[revistanefrologia.com]
- Flank and abdominal pain was the presenting symptom in both patients neither of whom had hematuria. Renal ultrasound detected cystic lesions consistent with ACKD; malignant masses were ultimately identified by CT scan.[ncbi.nlm.nih.gov]
- A 19-year-old African female presented with a chronic severe debilitating right lower quadrant abdominal pain refractory to analgesics.[ncbi.nlm.nih.gov]
- The most common mode of presentation was abdominal pain followed by hypertension, urinary tract infection, chronic renal failure and palpable abdominal mass, in decreasing order.[ncbi.nlm.nih.gov]
Abdominal Mass
- The condition is usually found during a prenatal ultrasound or when a doctor discovers an abdominal mass during a routine physical examination. The diagnosis of multicystic dysplastic kidney is usually made with an ultrasound examination.[nationwidechildrens.org]
- The most common mode of presentation was abdominal pain followed by hypertension, urinary tract infection, chronic renal failure and palpable abdominal mass, in decreasing order.[ncbi.nlm.nih.gov]
- masses; presents as flank mass or pyelonephritis In bilateral disease, neonates may also display oligohydramnios and pulmonary hypoplasia If blastema present, patient is at a slightly higher risk for Wilms tumor Segmental dysplasia is seen in children[pathologyoutlines.com]
- In the past, before we were able to make this diagnosis before birth, many of these kidneys were removed in the operating room simply to be able to see the abdominal mass.[chw.org]
Skin
Subcutaneous Nodule
- The patient presented with a subcutaneous nodule, which led to discovery of a large primary tumor in the left kidney. ACKD was present in the atrophic right kidney.[ncbi.nlm.nih.gov]
Urogenital
Hematuria
- Transplant patients with flank pain, hematuria, or other suspicious symptoms should have imaging studies of their native kidneys.[ncbi.nlm.nih.gov]
- Flank and abdominal pain was the presenting symptom in both patients neither of whom had hematuria. Renal ultrasound detected cystic lesions consistent with ACKD; malignant masses were ultimately identified by CT scan.[ncbi.nlm.nih.gov]
- […] hemodialysis for less than 3 years only (14 months prior to receiving a functional renal transplant which lasted 8 years, followed by 18 additional months of dialysis), before the diagnosis of ACKD was made following an episode of flank pain with gross hematuria[ncbi.nlm.nih.gov]
- Hematuria is also managed conservatively with bedrest and intravenous hydration, and most episodes of hematuria are self-limiting and last 2 to 7 days.[mdedge.com]
- Although, routine screening of family members with cystic kidney disease still remains a contentious issue because the knowledge may evoke anxiety in terms of employment and insurance, screening of symptomatic cases or those that develop hypertension, hematuria[ncbi.nlm.nih.gov]
Workup
- Standard workup includes Bosniak classification with contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). However, these tests are costly and not without risks.[ncbi.nlm.nih.gov]
- Therefore, ultrasound is the very first diagnostic tool and will guide further diagnostic workup with additional imaging studies or genetic testing.[link.springer.com]
- Simple cysts fall into the Bosniak category 1 and do not require further workup or imaging.[ncbi.nlm.nih.gov]
Biopsy
Renal Biopsy showing Juxtaglomerular Hyperplasia
- Renal biopsy showed juxtaglomerular hyperplasia, heavy subintimal deposits and C3 and IgG in preglomerular arteriolar walls, and degenerative changes in the tubules, including loss of brush border and "macula densa-like" lesions.[ncbi.nlm.nih.gov]
Treatment
- AH was defined as systolic blood pressure or 150 mmHg, diastolic blood pressure or 90 mmHg and/or antihypertensive treatment.[ncbi.nlm.nih.gov]
- Embryonic kidney cyst formation of Sclt1-/- mice was effectively reduced by an anti-STAT3 treatment using pyrimethamine.[ncbi.nlm.nih.gov]
- Consequently, D-penicillamine treatment was discontinued, after which the progression of cystic kidney disease gradually ceased, along with the other systemic manifestations of toxicity.[ncbi.nlm.nih.gov]
- Therefore, it may represent a treatment of choice in MCKD1 patients with ESRD.[ncbi.nlm.nih.gov]
- Treatment for cystic kidney disease There is no cure for any of the three cystic kidney diseases. Medical treatment aims to manage symptoms and reduce the risk of complications.[betterhealth.vic.gov.au]
Prognosis
- Unilateral renal cystic disease with an ipsilateral non-functional kidney and an atretic pelvicalyceal system is a very rare condition that needs to be distinguished from multicystic dysplastic kidney in order to guide management and set prognosis.[ncbi.nlm.nih.gov]
- These RCCs seems to have a relatively good prognosis.[ncbi.nlm.nih.gov]
- The results were analyzed with specific reference to age, gender, annual incidence, type of cystic disease, location of cyst, mode of presentation, complications and prognosis.[ncbi.nlm.nih.gov]
- "Polycystic kidney disease: inheritance, pathophysiology, prognosis, an IJNRD" . International Journal of Nephrology and Renovascular Disease : 69. doi : 10.2147/IJNRD.S6939 . Retrieved 2015-08-01 .[en.wikipedia.org]
- Prognosis is highly unpredictable due to an inability to quantify residual functional parenchyma. Treatment is surgical correction of any associated GU abnormalities and, if renal insufficiency or renal failure is present, renal replacement therapy .[msdmanuals.com]
Etiology
- There were no significant differences in the frequency of ACKD in the men and the women (P .59) and in the etiology of end-stage renal disease (P .64).[ncbi.nlm.nih.gov]
- [ edit ] Two main types Autosomal Dominant PKD (adult PKD) Autosomal Recessive PKD (infantile PKD) ADPKD [ edit ] Etiology and Natural History gene PKD1 mutated in about 85% of cases gene PKD2 mutated in about 15% of cases symptoms usually develop beginning[en.wikibooks.org]
- […] renal calices possibly caused by secondary hyperparathyroidism References: [3] [2] Pathology Differential diagnoses Multicystic dysplastic kidneys Definition : renal dysplasia with multiple cystic dilatation of nephrons during embryonic development Etiology[amboss.com]
- We also propose that hypokalemia induced tissue proliferation may, at least in some cases, be the etiological factor for the often described association of renal tubular acidosis and cystic disease of the kidney.[bmcnephrol.biomedcentral.com]
- Etiology Two genes have been linked to the disease: MCKD1 (1q21) is associated with end-stage renal disease at the mean age of 62 years, and MCKD2 (in 16p12, where the gene UMOD , encoding uromodulin or Tamm-Horsfall protein, has been identified as responsible[orpha.net]
Epidemiology
- Epidemiology Incidence ADPKD : 1/1,000 ; one of the most common inherited kidney diseases in humans ARPKD : 1/20,000 References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
- Bioinformatics and Genomics Programme, Centre for Genomic Regulation (CRG), Barcelona, Catalonia, Spain. 5 Universitat Pompeu Fabra, Barcelona, Catalonia, Spain. 6 Hospital del Mar Medical Research Institute (IMIM), Barcelona, Catalonia, Spain. 7 CIBER in Epidemiology[ncbi.nlm.nih.gov]
- Definition / general Three or more cysts per kidney in patients on longstanding hemo- or peritoneal dialysis for end stage renal disease (unrelated to underlying renal pathology) Epidemiology Occurs in 10% - 20% of patients within the first three years[pathologyoutlines.com]
- […] by week 20 in utero Enlarged, cystic kidneys with disorganized parenchyma and numerous cysts Immature tubules surrounded by mesenchymal collars and islands of immature mesenchyme are present between the cysts Also known as multicystic renal dysplasia Epidemiology[pathologyoutlines.com]
- Summary Epidemiology Less than 60 families affected by ADMCKD have been described. Prevalence is estimated to be 1/100 000. Clinical description Clinical onset and course are insidious.[orpha.net]
Sex distribution
Age distribution
Pathophysiology
- Recently, several promising trials have further extended our understanding of the pathophysiology of CKD and may have the potential for rational personalized therapies in future years.[ncbi.nlm.nih.gov]
- "Polycystic kidney disease: inheritance, pathophysiology, prognosis, an IJNRD" . International Journal of Nephrology and Renovascular Disease : 69. doi : 10.2147/IJNRD.S6939 . Retrieved 2015-08-01 .[en.wikipedia.org]
- […] dialysis Males females during first ten years of dialysis Not restricted to adults; occurs in children and young adults on dialysis ( Pediatr Nephrol 1997;11:447 ) Frequency and severity not affected by online hemodiafiltration ( Ren Fail 2009;31:555 ) Pathophysiology[pathologyoutlines.com]
- Conclusion The association of cystic renal disease with hypokalemia, and the possible pathophysiological basis of the development of renal cysts in patients with severe hypokalemia, are discussed.[bmcnephrol.biomedcentral.com]
- Pathophysiology The exact pathophysiology for renal cysts is unclear. Diverticula on the distal tubule of the nephron may be the starting point for cyst formation.[ncbi.nlm.nih.gov]
Prevention
- The National Institute of Diabetes and Digestive and Kidney Diseases recently asked the community to identify research objectives, which, if addressed, could improve understanding of basic kidney function and aid in prevention, treatment, and reversal[ncbi.nlm.nih.gov]
- There is no known way to prevent either kidney cysts or polycystic kidney disease.[healthcommunities.com]
- Frequent turning of the infant or bedridden child helps to prevent decubitus ulcers and lessens the danger of pneumonia, a constant threat to these children.[medical-dictionary.thefreedictionary.com]
- There is no way to prevent multicystic dysplastic kidney from occurring early in a baby's development, and there is no known way to prevent it from happening.[nationwidechildrens.org]