Sagliker syndrome: A case report of a rare manifestation of uncontrolled secondary hyperparathyroidism in chronic renal failure

Yu, Yu; Zhu, Chenfang; Fu, Xiao; Xu, Hua

doi:10.12998/wjcc.v7.i22.3792

Cited by 8 publications

(13 citation statements)

References 20 publications

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“…Laboratory values that are dominant in this syndrome are high serum PTH and ALP. High PTH (2815.07 pg/mL) and ALP (2350 U/L) with lower range of calcium (2.31 mmol/L) were presented for the patient in the case report done by Yu Yu et al in China 5 . Moreover, similar laboratory results were given in the research done by A. M. Pineda et al 7 .…”

Section: Discussionmentioning

confidence: 88%

“…Cholakova et al explains that commensurate increased serum alkaline phosphatase is responsible for this type of skeletal changes 4 . Yu Yu et al hypothesized that intramembranous ossification is hyperactivated in this syndrome, which then leads to thickening of the maxilla, anterior mandible and skull 5 . Moreover, in the international research of Yahya Sagliker et al they concluded that the GNAS1 gene missense mutations, which are possibly activated during the hemodialysis, might be responsible for genesis of SS 6 .…”

Section: Discussionmentioning

confidence: 99%

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Sagliker Syndrome – A Case Report

Smajić

Zibar

2023

Med. Flum. (Online)

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Aim: To present a case of Sagliker syndrome (SS), a rare syndrome caused by longterm heavy tertiary hyperparathyroidism (HPT) in end-stage renal disease (ESRD). Case report: In the year 2000, the 38-year-old man was diagnosed with ESRD. He is currently of low height, paraplegic, pigeon and barrel chested, with elongated upper extremities, deformed fingers, mandibular and maxillary asymmetric deformities with teeth malformations. Due to extremely high serum parathormone (PTH), resistant to pharmacological treatment, subtotal parathyroidectomy (PTx) was performed in 2007. However, he underwent parathyroid resurgery in 2010 for persistently high PTH of more than 2500 pg/mL (upper normal limit 69). In 2012, imaging found two suspected parathyroid glands and one of them was surgically removed. Postoperatively, the expected decrease in calcium and PTH serum concentrations did not occur. Ten years after the diagnosis of ESRD, the patient began to notice more pronounced skeletal deformities (upper, lower jaw, extremities, deformities of fingers, kyphoscoliosis) along with depressive disorder. Laboratory findings still show extremely high PTH (1994 pg/mL), low calcium, 1.89 mmol/L (normal range 2.14–2.53), and high alkaline phosphatase, 837 U/L (normal range 60–120), despite continuous pharmacological treatment. Conclusion: SS was firstly recognized by Yahya Sagliker in 2004 and it has not been described in Croatia to date. Pervasiveness and knowledge of the syndrome is still poor. The most efficient way of treating/preventing SS is early total PTx in severe secondary HPT related to ESRD. However, it can only stop progress of the disease, but cannot return skeletal deformities.

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Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Sagliker Syndrome – A Case Report

Smajić

Zibar

2023

Med. Flum. (Online)

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“…Los hallazgos clínicos, bioquímicos, estudios de imagen e histopatológicos son las principales herramientas para establecer el diagnóstico de tumores pardos en el HPTS en individuos con síndrome de Sagliker 5,9 . Los niveles séricos de calcio, fósforo, fosfatasa alcalina y hormona paratiroidea en la evaluación inicial son de importancia para el manejo adecuado 13 . Así mismo, los estudios de imágenes permiten identificar alteraciones óseas no solo en los maxilares, sino también en otras áreas anatómicas 13 .…”

Section: Discussionunclassified

“…Los niveles séricos de calcio, fósforo, fosfatasa alcalina y hormona paratiroidea en la evaluación inicial son de importancia para el manejo adecuado 13 . Así mismo, los estudios de imágenes permiten identificar alteraciones óseas no solo en los maxilares, sino también en otras áreas anatómicas 13 . En el síndrome de Sagliker es frecuente observar lesiones con aspecto de sal y pimienta en los huesos craneales 8 .…”

Section: Discussionunclassified

Abordaje quirúrgico de tumor pardo de maxilar en síndrome de Sagliker

Fuentes

Cruz

2022

Alerta (San Salvador)

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Presentación del caso. Se trata de una paciente femenina de 20 años de edad con enfermedad renal crónica estadio 5 en tratamiento de hemodiálisis que notó aumento progresivo de la región palatina en los últimos siete meses, que le generaba dislalia y dificultad para la masticación con expansión del hueso maxilar, separación de las piezas dentales superiores y cambios en la configuración y perfil facial compatibles con un tumor pardo. Los resultados de laboratorio confirmaron un hiperparatiroidismo e hipocalcemia secundarios a la enfermedad renal. Los hallazgos ecográficos fueron compatibles con hiperplasia de paratiroides del lado izquierdo. Intervención terapéutica. Se administró carbonato de calcio y paricalcitol durante las dos semanas previas a la cirugía. El tratamiento consistió en una paratiroidectomía subtotal y la resección quirúrgica de la tumoración en el maxilar y los suplementos de calcio y vitamina D3 por vía oral. Evolución clínica. Se obtuvo normalización de los niveles de calcio y de fósforo y disminuyeron los niveles de la paratohormona. Después de un seguimiento de más de dos años, no se identificaron recurrencias.

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“…Brown tumors secondary to hyperparathyroidism can also occur in the mandible and though typically asymmetric and focal along the mandible as opposed to diffuse as seen in uremic leontiasis ossea, they too will often have this alternating pattern of lucency and sclerosis which is indicative of rapid osteoclastic bone turnover and repair thought to result in this marbled appearance. [ 5 , 6 ]…”

Section: Discussionmentioning

confidence: 99%

Uremic Leontiasis Ossea

Heard

Raj

et al. 2021

JCIS

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While renal osteodystrophy is a common complication of chronic renal failure which is caused by secondary hyperparathyroidism, it is rare that the bony changes result in a severe progressive overgrowth of the bones of the face such that the patient is at risk for breathing and feeding difficulties. When this occurs, it is called uremic leontiasis ossea and patients who suffer from this rare, severe complication of renal osteodystrophy may go undiagnosed or be misdiagnosed resulting improper management due to its limited discussion in the literature. We report a case of a 42-year-old man with end-stage renal disease who was unable to receive dialysis consistently for many years who was found to have a large hard mass on the palate and palate ulcers.

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Sagliker syndrome: A case report of a rare manifestation of uncontrolled secondary hyperparathyroidism in chronic renal failure

Cited by 8 publications

References 20 publications

Sagliker Syndrome – A Case Report

Sagliker Syndrome – A Case Report

Abordaje quirúrgico de tumor pardo de maxilar en síndrome de Sagliker

Uremic Leontiasis Ossea

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