Maximum urine concentrating ability in children with Hb SC disease: Effects of hydroxyurea

Iyer, Rathi V.; Baliga, Radhakrishna; Nagel, Ronald L.; Brugnara, Carlo; Kirchner, Kent A.; Hogan, Shirley; Steinberg, Martin H.

doi:10.1002/(sici)1096-8652(200005)64:1<47::aid-ajh8>3.0.co;2-1

Cited by 26 publications

(9 citation statements)

References 27 publications

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“…They need to be interpreted with some caution for urine osmolality. We observed no significant difference in urine osmolality (or albuminuria) in patients that were on hydroxyurea versus those not on hydroxyurea, consistent with an earlier report on lack of effect of hydroxyurea on albuminuria in Hb SC patients [35]. However, it is likely that the hydroxyurea group of patients is a biased set of patients with more severe disease.…”

Section: Discussionsupporting

confidence: 90%

Biomarkers for early detection of sickle nephropathy

et al. 2011

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Renal complications affect nearly 30–50% of adults with sickle cell anemia (SCA), causing significant morbidity and mortality. Standard renal function tests like serum creatinine and glomerular filtration rate become abnormal in this disease only when renal damage has become extensive and largely irreversible. Moreover, not all patients develop sickle nephropathy (SN). Therefore, noninvasive biomarkers that predict early onset of SN are necessary. We performed a cross-sectional analysis for nephropathy in 116 patients with sickle cell disease, analyzing urinary kidney injury molecule-1 (KIM-1), liver-type fatty acid binding protein (L-FABP), N-acetyl-b-D-glucosaminidase (NAG), neutrophil gelatinase-associated lipocalin (NGAL) and transforming growth factor-β1 (TGF-β), together with conventional renal biomarkers (urine albumin and osmolality, and serum creatinine and cystatin C estimated GFR) during routine clinic visits when patients were at steady-state/baseline. We observed a distinct biomarker pattern: KIM-1 and NAG emerged as biomarkers with a strong association with albuminuria. Surprisingly, and in contrast to other acute/chronic renal disorders, NGAL, L-FABP, and TGF-β levels did not show any relationship with albuminuria in patients with SCA. Our study identifies potential biomarkers for SN, and suggests longitudinal validation of these biomarkers for early detection of SN, so that therapeutic interventions can be applied before renal damage becomes irreversible.

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Section: Discussionsupporting

confidence: 90%

Biomarkers for early detection of sickle nephropathy

et al. 2011

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“…Notably, in children, RBC transfusions can reverse the UCD, but the defect is irreversible after the age of 10–15 yrs [554, 555]. Hydroxyurea therapy results in an increase in fetal hemoglobin production and dramatically reduces the frequency of pain and acute chest episodes in individuals with SCD [561]. However, hydroxyurea showed no improvement in UCD in children with hemoglobin SC disease [561].…”

Section: Renal/genitourinary Complicationsmentioning

confidence: 99%

“…Hydroxyurea therapy results in an increase in fetal hemoglobin production and dramatically reduces the frequency of pain and acute chest episodes in individuals with SCD [561]. However, hydroxyurea showed no improvement in UCD in children with hemoglobin SC disease [561]. A recently completed multicenter BABY-HUG study [562] should provide useful prospective data on the usefulness of hydroxyurea in alleviating the UCD in early SN.…”

Section: Renal/genitourinary Complicationsmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

158

144

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The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

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“…Microalbuminuria occurs in 30% of patients with HbSC, and ESRD develops in 2-3% by their 5th decade (Powars et al, 1991(Powars et al, , 2002Lionnet et al, 2012;Drawz et al, 2016). Children with HbSC typically develop irreversible hyposthenuria at 10-15 years of age and have evidence of glomerular damage and medullary injury (Iyer et al, 2000;Drawz et al, 2016). Acute HbSC renal complications include renal papillary necrosis and renal infarction.…”

Section: Renalmentioning

confidence: 99%

Knowledge insufficient: the management of haemoglobin SC disease

2016

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Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC.

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Maximum urine concentrating ability in children with Hb SC disease: Effects of hydroxyurea

Cited by 26 publications

References 27 publications

Biomarkers for early detection of sickle nephropathy

Biomarkers for early detection of sickle nephropathy

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Knowledge insufficient: the management of haemoglobin SC disease

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