Bennhold's analbuminemia: A follow-up study of the first two cases (1953–1992)

Kallee, E

doi:10.1016/s0022-2143(96)90064-5

Cited by 38 publications

(35 citation statements)

References 14 publications

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“…CAA is a rare autosomal recessive disorder [4,9,18,21] leading to defective albumin synthesis in the liver [23] with subsequent very low serum albumin concentration (<0.2 g/dl). If albumin is administered intravenously to patients with CAA, its biologic half-life is prolonged to 38 -50 days (reference range: 16 -20 days) [21,23]; on the contrary, the half-life in our patients was very short (<5 days), raising the question of albuminuria.…”

Section: Discussionmentioning

confidence: 99%

“…A few publications have reviewed the clinical presentation and long-term outcome of patients with CAA [8,9,12,16,21]. However, follow-up data are incomplete and inconsistent in some cases.…”

Section: Discussionmentioning

confidence: 99%

“…However, follow-up data are incomplete and inconsistent in some cases. Thus, despite the very high serum concentrations of total and LDL cholesterol, the risk of progressive atherosclerosis and cardiovascular events is still not well defined; yet, an increased risk of atherosclerosis cannot be excluded [8,9,12,16,21]. Well documented complications were lipodystrophy (mainly in adult females) and osteoporosis.…”

Section: Discussionmentioning

confidence: 99%

“…Congenital analbuminemia (CAA) is a rare autosomal recessive disorder [9] in which subjects have little or no circulating serum albumin due to defective albumin synthesis in the liver [23]. Fewer than 50 patients have been recorded so far in the continuously updated Register of Analbuminemia Cases [18].…”

Section: Introductionmentioning

confidence: 99%

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A boy with congenital analbuminemia and steroid-sensitive idiopathic nephrotic syndrome: an experiment of nature

2007

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A boy with congenital analbuminemia and steroid-sensitive idiopathic nephrotic syndrome: an experiment of nature

2007

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“…These data are consistent with generalized hyperlipidemia and other changes reported for human analbuminemia. 1,26,[28][29][30][31][32][33] Despite these serum changes, both Alb -/-strains are healthy, maintaining normal body weights and breeding similarly to their Alb C/C parental strains. Gross examinations by necropsy of »8 wks mice from both Alb -/-strains were unremarkable, lacking overt differences compared with the background strains, B6 and Tg32.…”

Section: Blood Chemistry and Gross Histopathologymentioning

confidence: 99%

Albumin-deficient mouse models for studying metabolism of human albumin and pharmacokinetics of albumin-based drugs

Roopenian

Low

Christianson³

et al. 2015

mAbs

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(2015) Albumin-deficient mouse models for studying metabolism of human albumin and pharmacokinetics of albumin-based drugs, mAbs, 7:2, 344-351, DOI: 10.1080DOI: 10. /19420862.2015 To link to this article: https://doi.org/10. 1080/19420862.2015 Serum albumin is the major determinant of blood colloidal osmotic pressure acting as a depot and distributor of compounds including drugs. In humans, serum albumin exhibits an unusually long half-life mainly due to protection from catabolism by neonatal Fc receptor (FcRn)-mediated recycling. These properties make albumin an attractive courier of therapeutically-active compounds. However, pharmaceutical research and development of albumin-based therapeutics has been hampered by the lack of appropriate preclinical animal models. To overcome this, we developed and describe the first mouse with a genetic deficiency in albumin and its incorporation into an existing humanized FcRn mouse model, B6.Cg-Fcgrt tm1Dcr Tg(FCGRT)32Dcr/DcrJ (Tg32). Albumin-deficient strains (Alb -/-) were created by TALEN-mediated disruption of the albumin (Alb) gene directly in fertilized oocytes derived from Tg32 mice and its nontransgenic background control, C57BL/6J (B6). The resulting Alb -/-strains are analbuminemic but healthy. Intravenous administration of human albumin to Tg32-Alb -/-mFcRn -/-hFcRn Tg/Tg ) mice results in a remarkably extended human albumin serum half-life of »24 days, comparable to that found in humans, and in contrast to half-lives of 2.6-5.8 d observed in B6, B6-Alb -/-and Tg32 strains. This striking increase can be explained by the absence of competing endogenous mouse albumin and the presence of an active human FcRn. These novel albumin-deficient models provide unique tools for investigating the biology and pathobiology of serum albumin and are a more appropriate rodent surrogates for evaluating human serum albumin pharmacokinetics and albumin-based compounds.

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Basics of Fluid Physiology

Magder

2021

Cardiopulmonary Monitoring

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Bennhold's analbuminemia: A follow-up study of the first two cases (1953–1992)

Cited by 38 publications

References 14 publications

A boy with congenital analbuminemia and steroid-sensitive idiopathic nephrotic syndrome: an experiment of nature

A boy with congenital analbuminemia and steroid-sensitive idiopathic nephrotic syndrome: an experiment of nature

Albumin-deficient mouse models for studying metabolism of human albumin and pharmacokinetics of albumin-based drugs

Basics of Fluid Physiology

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