“As Black as Ink”

Donaldson, Callum J.; Mitchell, Stuart; Riley, Lee H.; Kebaish, Khaled M.

doi:10.1097/brs.0000000000002755

Cited by 9 publications

(3 citation statements)

References 19 publications

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“…In most patients, this myelopathy is falsely attributed to age-related degeneration. Some radiological features might aid in diagnosis, especially in the absence of typical clinical findings, including involvement of multiple intervertebral levels, progressive calcification of the discs within a relatively short period of time, and the presence of large numbers of osteophytes [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…In addition to the involvement of large joints, such as the knees and hips, the spine is one of the most common areas of the musculoskeletal system in which ochronotic pigment is deposited, leading to a condition called ochronotic spondyloarthropathy. Myelopathy is a very rare presentation, with a recent literature review finding that only 11 patients with AKU presented with myelopathy and only 5 with cervical myelopathy [ 4 ]. Few reports have described patients with lumbar disc herniation but no signs of AKU preoperatively who were diagnosed with AKU post-operatively after intraoperative findings of disc material with black discoloration [ 5 – 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Cervical Myelopathy Due to Ochronosis: An Intraoperative Suspicion

et al. 2020

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Patient: Female, 55-year-old Final Diagnosis: Alkaptonuria Symptoms: Neck pain Medication:— Clinical Procedure: — Specialty: Neurosurgery • Orthopedics and Traumatology Objective: Rare disease Background: Alkaptonuria (AKU) is a rare metabolic disease caused by a deficiency in homogentisic acid oxidase, which leads to the accumulation of homogentisic acid dark pigments in tissues such as bones, ligaments, and ten-dons. Long-term duration of this condition, termed ochronosis, can result in degenerative arthropathy involving the spine and large joints. Case Report: This report describes a 55-year-old Jordanian woman presenting with chronic neck and lower back pain. History, physical examination, and radiological imaging indicated cervical myelopathy and lumbar spine degeneration. Two-level anterior cervical discectomy and fusion was performed successfully. Intra-operatively, the cervical discs were observed to be black, suggesting a diagnosis of alkaptonuria, which was later confirmed by genetic testing. A detailed history and physical examination revealed the absence of classical features of AKU. Conclusions: Intraoperative detection of black disc material suggests the need for further tests to diagnose AKU, especially in indolent patients who did not have classical clinical features. Surgical management may improve outcomes in patients with cervical myelopathy due to ochronosis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cervical Myelopathy Due to Ochronosis: An Intraoperative Suspicion

et al. 2020

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“…HGA being a reducing agent is oxidized by a slow spontaneous irreversible process imparting black color to the urine and was first explained as “as black as ink” by Scribonius in 1584 [ 9 , 10 ]. In addition to the blackening of urine, the eyes and ears also develop pigmentation, but it takes two to three decades to be visible externally [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

Identification of Potential Inhibitors for the Treatment of Alkaptonuria Using an Integrated In Silico Computational Strategy

et al. 2023

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Alkaptonuria (AKU) is a rare genetic autosomal recessive disorder characterized by elevated serum levels of homogentisic acid (HGA). In this disease, tyrosine metabolism is interrupted because of the alterations in homogentisate dioxygenase (HGD) gene. The patient suffers from ochronosis, fractures, and tendon ruptures. To date, no medicine has been approved for the treatment of AKU. However, physiotherapy and strong painkillers are administered to help mitigate the condition. Recently, nitisinone, an FDA-approved drug for type 1 tyrosinemia, has been given to AKU patients in some countries and has shown encouraging results in reducing the disease progression. However, this drug is not the targeted treatment for AKU, and causes keratopathy. Therefore, the foremost aim of this study is the identification of potent and druggable inhibitors of AKU with no or minimal side effects by targeting 4-hydroxyphenylpyruvate dioxygenase. To achieve our goal, we have performed computational modelling using BioSolveIT suit. The library of ligands for molecular docking was acquired by fragment replacement of reference molecules by ReCore. Subsequently, the hits were screened on the basis of estimated affinities, and their pharmacokinetic properties were evaluated using SwissADME. Afterward, the interactions between target and ligands were investigated using Discovery Studio. Ultimately, compounds c and f were identified as potent inhibitors of 4-hydroxyphenylpyruvate dioxygenase.

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