Sagliker syndrome in patients with secondary hyperparathyroidism and chronic renal failure: Case report

Pineda, Ana Mejía; Aguilera, María Lorena; Meléndez, Héctor Julio; Lemus, José A.; Peñalonzo, Marco Antonio

doi:10.1016/j.ijscr.2015.01.044

Cited by 15 publications

(11 citation statements)

References 9 publications

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“…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 . In the both researches total PTx was performed which resulted in rapid decrease in PTH level.…”

Section: Discussionsupporting

confidence: 87%

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: Discussionsupporting

confidence: 87%

Sagliker Syndrome – A Case Report

Smajić

Zibar

2023

Med. Flum. (Online)

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“…Although she had received medical care since childhood, her renal disease had been inadequately managed due to missed hemodialyses, which had directly contributed to her recurrent fluid overload and respiratory failure. She also had not undergone parathyroidectomy, the only procedure that is known to halt the progression of her disease [ 4 , 8 , 9 ], which had resulted in her difficult airway anatomy [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…Genes associated with plasma calcium-sensing and bone dysplasia have also been implicated in disease pathogenesis [ 5 , 6 ]. These bony changes will irreversibly worsen if the parathyroid hormone (PTH) level is inadequately controlled [ 7 ], and parathyroidectomy is the only procedure known to halt the disease progression [ 4 , 8 , 9 ]. Furthermore, given the physical deformity and debility as the disease continues to evolve, SS patients are associated with a higher prevalence of psychiatric disorders than patients with ESRD alone [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Perioperative Care and Airway Management for a Patient With Sagliker Syndrome

Chen

Lorenzo

2020

Cureus

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In this report, we present a case of a patient with a history of complex airway anatomy secondary to Sagliker syndrome (SS) who presented with acute exacerbation of chronic respiratory failure. The patient’s difficult airway, complicated medical comorbidities, and poor psychosocial status posed a unique challenge for providing safe care during an emergency. The perioperative anesthesia service (PAS), led by critical care anesthesiologists, coordinated a multidisciplinary airway management plan. The PAS team also assisted this medically complex patient with her decision-making process. A 37-year-old female with SS, which is characterized by irreversible disfiguring of head and neck anatomy secondary to end-stage renal disease (ESRD) and poorly controlled hyperparathyroidism, presented with acute exacerbation of chronic respiratory failure due to hypervolemia. The patient’s respiratory status rapidly deteriorated despite aggressive hemodialysis, requiring transfer to the ICU. Given the challenging anatomy and poor respiratory reserve in this patient, the PAS team helped coordinate a comprehensive airway plan that involved transnasal fiberoptic intubation, and in case of emergency, extracorporeal membrane oxygenation (ECMO) as a bridge to a surgical airway. During the decision-making process, the patient was found to be in psychological distress and had limited insights into her condition. The PAS team helped facilitated multidisciplinary goals-of-care discussions for the patient and her family. Fortunately, the patient’s oxygenation improved with noninvasive oxygen support and aggressive hemodialysis without the need for intubation. She was discharged with outpatient follow-up appointments arranged to discuss long-term management. This is the first reported case of SS in the United States. The early involvement by the PAS team helped coordinate a multidisciplinary care plan for this patient with a difficult airway and complex comorbidities. This report highlights an innovative airway algorithm for a potentially “cannot-intubate, cannot ventilate" complex airway, and the PAS team’s role in providing support for the patient’s physical and psychological needs, suggesting that a comprehensive perioperative service can improve the quality and safety of care, not only for surgical patients but also for medically complex patients as well.

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“…Совокупность данных признаков в 2004 году была выделена Сагликером в одноименный синдром [1]. По состоянию на 2012 год в литературе сообщалось о шестидесяти случаях данного заболевания [9]. В отечественной литературе описано всего два клинических случая наблюдения данного синдрома [10,11].…”

Section: клинический случайunclassified

“…Неконтролируемое течение ВГПТ и развившийся синдром Сагликера следует считать показанием к паратиреоидэктомии, которую рекомендуют выполнять без задержек в специализированных центрах [16][17]. Тотальная паратиреоидэктомия является предпочтительной, для избежания персистирования и рецидива заболевания [9,17]. В большинстве случаев хирургическое лечение ВГПТ приводит к нормализации лабораторных показателей, однако сформировавшиеся костные деформации необратимы [1].…”

Section: описание случаяunclassified

Case report of Sagliker syndrome in a young patient with secondary hyperparathyroidism and chronic renal failure

et al. 2019

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Sagliker syndrome is a rare complication of renal osteodystrophy, characterized by severe skeletal and cranium deformities, neurologic and soft tissue abnormalities in patients with chronic renal failure (CRF) and untreated secondary hyperparathyroidism. This article reports a 29-year-old female patient with end-stage CRF after 9 years of hemodialysis. She had severe secondary hyperparathyroidism, hyperplasia of three parathyroid glands and cranium and skeletal bone structure deformation. The first changes appeared after 4 years of therapy with peritoneal dialysis. They included uglifying face appearances, short stature, severe maxillary changes, chest deformity. During the examination we revealed severe tomographical and X-ray changes: maxillary and mandibular hyperplasia, temporomandibular articulation changes, affected cheekbones, sphenoid bone and bones of the cranial vault, fingertip changes, vertebral body compression. Although surgical parathyroidectomy was effective at biochemical abnormalities, severe bone deformities were not regressed. This case highlights the importance of clinicians’ attention for early monitoring and appropriate treatment of secondary hyperparathyroidism in patients with end-stage CRF.

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Sagliker syndrome in patients with secondary hyperparathyroidism and chronic renal failure: Case report

Abstract: HighlightsSagliker syndrome is assumed to result from insufficient treatment of secondary hyperparathyroidism in early stages of chronic renal failure.Sagliker syndrome should be added to current surgical indications for parathyroidectomy.

Cited by 15 publications

References 9 publications

Sagliker Syndrome – A Case Report

Sagliker Syndrome – A Case Report

Perioperative Care and Airway Management for a Patient With Sagliker Syndrome

Case report of Sagliker syndrome in a young patient with secondary hyperparathyroidism and chronic renal failure

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