Progression of autosomal-dominant polycystic kidney disease in children

Fick-Brosnahan, Godela M.; Tran, Zung Vu; Johnson, Ann; Strain, John D.; Gabow, Patricia A.

doi:10.1046/j.1523-1755.2001.0590051654.x

Cited by 151 publications

(108 citation statements)

References 32 publications

(6 reference statements)

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“…Much of the data on the natural history of ADPKD comes from longitudinal studies (23)(24)(25)(26)(27)(28), which show that renal function and rate of decline in renal function in patients with ADPKD correlate with renal volume and the rate of increase in renal volume. Moreover, the Consortium of Radiologic Imaging Study of PKD (CRISP) cohort (23) confirmed the notion that cystic growth and renal enlargement is significant before impairment of renal function.…”

Section: Discussionmentioning

confidence: 99%

Glomerular Hyperfiltration and Renal Progression in Children with Autosomal Dominant Polycystic Kidney Disease

Helal¹,

Reed²,

McFann³

et al. 2011

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives The purpose of this study was to determine whether glomerular hyperfiltration (GH) occurring early in autosomal dominant polycystic kidney disease (ADPKD) is indicative of more rapid disease progression in children.Design, setting, participants, & measurements One hundred eighty children with ADPKD (ages 4 to 18 years) with normal renal function were examined by renal ultrasound. Renal volume was calculated using a standard formula for a modified ellipsoid. Creatinine clearance was calculated from serum creatinine and 24-hour urine creatinine. GH was defined as creatinine clearance Ն140 ml/min per 1.73 m 2 .Results Thirty-two children had GH (mean age 11.4 Ϯ 3.6 years) and 148 had normal renal function (mean age 10.8 Ϯ 3.9 years). Patients with GH at baseline demonstrated an increased rate of total renal volume growth (␤: rate of change ϭ ϩ19.3 Ϯ 10.8 cm 3 /year) over 5 years compared with those without GH at baseline (␤ ϭ Ϫ4.3 Ϯ 7.7 cm 3 /year), P ϭ 0.008. Those with GH at baseline experienced a faster decline in creatinine clearance in subsequent years (␤ ϭ Ϫ5.0 Ϯ 0.8 ml/min per 1.73 m 2 per year) compared with those without GH at baseline (␤ ϭ ϩ1.0 Ϯ 0.4 ml/min per 1.73 m 2 per year), P Ͻ 0.0001. ConclusionsThis study revealed that occurrence of GH in ADPKD children is associated with a significantly faster decline in renal function and higher rate of kidney enlargement over time. GH combined with the increased renal volume may therefore be used as an early marker for a more severe progression of ADPKD in children.

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Section: Discussionmentioning

confidence: 99%

Glomerular Hyperfiltration and Renal Progression in Children with Autosomal Dominant Polycystic Kidney Disease

Helal¹,

Reed²,

McFann³

et al. 2011

Clinical Journal of the American Society of Nephrology

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“…6,7 Clinical features depend on the age of onset and the predominance of hepatic and renal involvement. 8 In our patient the antenatal renal ultrasound was considered normal and the diagnosis was made after birth.…”

Section: Discussionmentioning

confidence: 99%

Pyloric stenosis in a newborn baby with polycystic kidneys

2007

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Patients with infantile hypertrophic pyloric stenosis (IHPS) have an increased incidence of renal anomalies. Association of IHPS and polycystic kidney disease (PKD) has been reported in three case histories. It is uncertain whether the association of PKD and IHPS is due to genetics, a pathophysiological link or a chance phenomenon. One hypothesis linking the two conditions is nitric oxide synthetase (NOS) deficiency. NOS deficiency leads to lack of locally available nitric oxide causing a failure of smooth muscle relaxation. It is therefore plausible that NOS deficiency plays a part in the pathogenesis of pyloric stenosis, hypertension and cyst development in autosomal-dominant PKD.

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“…The optimal BP target for patients with hypertension and ADPKD has not been defined. Rates of cyst growth are higher in the presence of hypertension, but the independent relevance of achieved BP values is unclear (30,31). A subanalysis of 200 participants in the Modification of Diet in Renal Disease (MDRD) study with ADPKD did not show a beneficial effect of lowering the BP to below 125/75 mmHg on the rate of progression of kidney disease during the 2.2 years of the study compared with the conventional target of 140/90 mmHg (32).…”

Section: Treatment Of Hypertension In Pkdmentioning

confidence: 99%

A Young Patient with a Family History of Hypertension

Peixoto¹

2014

Clinical Journal of the American Society of Nephrology

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The evaluation of causes of hypertension in young adults with a family history of hypertension needs to be methodical to identify potentially treatable causes. Renal-and renovascular imaging and measurement of plasma aldosterone and plasma renin activity are at the core of this evaluation in most patients. Pertinent aspects of hypertension in autosomal dominant polycystic kidney disease are discussed with a focus on the role of the endothelium in mediating early hypertension and a review of treatment strategies. Finally, the possibility that autosomal dominant polycystic kidney disease and primary aldosteronism are connected beyond coincidence is explored; evidence to support it is scant, although there is a likely role for aldosterone excess and the resultant hypokalemia in promoting cyst growth.Clin J Am Soc Nephrol 9: 2164-2172, 2014. doi: 10.2215/CJN.02240314 Case PresentationA 36-year-old white man presented for the evaluation of hypertension, hypokalemia, and biochemical evidence of aldosterone excess. His hypertension was first diagnosed at 22 years of age while in the military, and we do not have records of his evaluation at that time. He reported that he had BP levels in the 150/100-mmHg range and that his weight at that time was approximately 180 lb (body mass index [BMI] approximately 24.5 kg/m 2 ). He was treated with lisinopril with prompt and sustained achievement of BP control.He left the military when he was 26 years old, and his new physician performed an evaluation of his hypertension. He was asymptomatic. He reported a family history of hypertension starting in early adulthood in his brother, mother, one maternal uncle, and maternal grandfather. He was not aware of any history of strokes, kidney disease, endocrine tumors, or hypokalemia, although he had been estranged from his parents since early childhood, and therefore, the information about his family was not complete.His BP was 128/84 mmHg. Other than being overweight (218 lb; BMI529.4 kg/m 2

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Progression of autosomal-dominant polycystic kidney disease in children

Abstract: The progression of ADPKD clearly occurs in childhood and manifests as an increase in cyst number and renal size. This study identifies children at risk for rapid renal enlargement who may benefit the most from future therapeutic interventions.

Cited by 151 publications

References 32 publications

Glomerular Hyperfiltration and Renal Progression in Children with Autosomal Dominant Polycystic Kidney Disease

Glomerular Hyperfiltration and Renal Progression in Children with Autosomal Dominant Polycystic Kidney Disease

Pyloric stenosis in a newborn baby with polycystic kidneys

A Young Patient with a Family History of Hypertension

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