Growing Old Too Fast: A Rare Case of Werner Syndrome

Kaur, Anahat; Grover, Punita; Albawaliz, Anas; Chauhan, Mahak; Barthel, Brandon

doi:10.7759/cureus.4743

Cited by 3 publications

(3 citation statements)

References 10 publications

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“…Hypertriglyceridemia should also be noted. It was reported that a 29-year-old patient with Werner syndrome and hypertriglyceridemia (triglyceride level of 3900 mg/dL) had advanced three-vessel disease requiring coronary artery bypass graft surgery [ 45 ]. Therefore, in addition to statins, management of hypertriglyceridemia may also be needed.…”

Section: Discussionmentioning

confidence: 99%

Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry

Maeda

Koshizaka

Shoji

et al. 2023

Aging

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Werner syndrome is an adult-onset progeria syndrome that results in various complications. This study aimed to clarify the profile and secular variation of the disease. Fifty-one patients were enrolled and registered in the Werner Syndrome Registry. Their data were collected annually following registration. A cross-sectional analysis at registration and a longitudinal analysis between the baseline and each subsequent year was performed. Pearson's chi-squared and Wilcoxon signed-rank tests were used. Malignant neoplasms were observed from the fifth decade of life (mean onset: 49.7 years) and were observed in approximately 30% of patients during the 3-year survey period. Regarding renal function, the mean estimated glomerular filtration rate calculated from serum creatinine (eGFRcre) and eGFRcys, which were calculated from cystatin C in the first year, were 98.3 and 83.2 mL/min/1.73 m 2 , respectively, and differed depending on the index used. In longitudinal analysis, the average eGFRcre for the first and fourth years was 74.8 and 63.4 mL/min/1.73 m 2 , showing a rapid decline. Secular changes in Werner syndrome in multiple patients were identified. The prevalence of malignant neoplasms is high, and renal function may decline rapidly. It is, therefore, necessary to carry out active and detailed examinations and pay attention to the type and dose of the drugs used.

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Section: Discussionmentioning

confidence: 99%

Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry

Maeda

Koshizaka

Shoji

et al. 2023

Aging

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“…The average life expectancy in patients with WS is around 54 years [13]. The cause of death in these patients is a myocardial infarction, or malignant…”

Section: Case Discussionmentioning

confidence: 99%

Werner syndrome associated with acroosteolysis

Khalid¹,

Inam²,

Iqbal³

2022

DOJ

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“…The causative gene is WRN on the short arm of chromosome 8. About 70% of the cases reported to date are Japanese, and 1 in 150 Advance Publication Journal of Atherosclerosis and Thrombosis Accepted for publication: August 16, 2021 Published online: September 11, 2021 disease requiring coronary artery bypass surgery 78) . In addition to statins to hypercholesterolemia, adequate management of hypertriglyceridemia may also be required.…”

Section: Werner Syndromementioning

confidence: 99%

Atherosclerosis and Cardiovascular Diseases in Progeroid Syndromes

Kato

Maezawa

2022

JAT

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Hutchinson-Gilford progeria syndrome (HGPS) and Werner syndrome (WS) are two of the representative genetic progeroid syndromes and have been widely studied in the field of aging research. HGPS is a pediatric disease in which premature aging symptoms appear in early childhood, and death occurs at an average age of 14.5 years, mainly due to cardiovascular disease (CVD). Conversely, WS patients exhibit accelerated aging phenotypes after puberty and die in their 50s due to CVD and malignant tumors. Both diseases are models of human aging, leading to a better understanding of the aging-associated development of CVD. In this review, we discuss the pathogenesis and treatment of atherosclerotic diseases presented by both progeroid syndromes with the latest findings. Hutchinson-Gilford Progeria SyndromeHGPS is an ultra-rare autosomal dominant genetic premature aging syndrome that occurs in one in four to eight million births and causes death at an average age of 14.5 years due to myocardial infarction or stroke 4, 5) (Table 1). Because of the abnormal splicing of the LMNA gene on chromosome 1, the lamin A protein encoded by this gene cannot be normally produced, and an abnormal protein called progerin accumulates in the nucleus. The main symptoms are scleroderma-like skin, joint contractures, bone abnormalities, hair loss, growth retardation, and atherosclerotic diseases, such as myocardial infarction and stroke. Clinical Characteristics of HGPSInfants with HGPS are normal at birth, but the mean weight is slightly small for gestational age 4) . The first pathognomonic signs are prominent veins on the nose bridge, followed by growth retardation, hair loss, and reduced subcutaneous fat around 6 to 12 months, and the diagnosis is often made between 2 and 3 years Copyright©2021 Japan Atherosclerosis Society This article is distributed under the terms of the latest version of CC BY-NC-SA defined by the Creative Commons Attribution License.

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Growing Old Too Fast: A Rare Case of Werner Syndrome

Cited by 3 publications

References 10 publications

Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry

Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry

Werner syndrome associated with acroosteolysis

Atherosclerosis and Cardiovascular Diseases in Progeroid Syndromes

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