Acute intermittent porphyria precipitated by atazanavir/ritonavir

Bharti, Sheena; Malhotra, Prashant; Hirsch, Bruce Elliot

doi:10.1177/0956462416633981

Cited by 7 publications

(4 citation statements)

References 9 publications

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“…However, true-positive results of urinary urobilinogen caused by the elevated serum total bilirubin were found in several of the patients with cholecystitis and gallstones. Furthermore, we found that most of the AHP patients showed normal serum bilirubin levels, consistent with the literature (12,18,(20)(21)(22). This indicated that urinary urobilinogen combined with serum total bilirubin could be used to distinguish the false-positive result from the true-positive result.…”

Section: Discussionsupporting

confidence: 91%

A high urinary urobilinogen / serum total bilirubin ratio reported in abdominal pain patients can indicate acute hepatic porphyria

Song

Liu

2022

Preprint

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Background: Due to its variable symptoms and nonspecific laboratory test results during routine examinations, acute hepatic porphyria (AHP) has always been a diagnostic dilemma for physicians. Misdiagnoses, missed diagnoses, and inappropriate treatments are very common. Correct diagnosis mainly depends on the detection of a high urinary porphobilinogen (PBG) level, which is not a routine test performed in the clinic and highly relies on the physician’s awareness of AHP. Therefore, identifying a more convenient indicator for use during routine examinations is required to improve the diagnosis of AHP. Results: In the present study, we retrospectively analyzed laboratory examinations in 12 AHPpatients and 100 patients with abdominal pain of other causes as the control groups between 2015 and 2022. Compared with the control groups, AHP patients showed a significantly higher urinary urobilinogen level during the urinalysis (P < 0.05). However, we showed that the higher urobilinogen level was caused by a false-positive result due to a higher level of urine PBG in the AHP patients. Moreover, a remarkable increase in the urinary urobilinogen/serum total bilirubin ratio was observed in AHP patients when compared to the control groups. The area under the ROC curve of this ratio for AHP was 1.000 (95% confidence interval, 1.000–1.000, P < 0.01). A cutoff value of 3.22 for the urinary urobilinogen/serum total bilirubin ratio yielded a sensitivity of 100% and a specificity of 100% to distinguish AHP patients from the controls. Conclusion: A reported high urinary urobilinogen level that was adjusted by the serum total bilirubin level (urinary urobilinogen/serum total bilirubin ratio) could be used as a sensitive and specific screening marker for AHP in patients with abdominal pain.

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Section: Discussionsupporting

confidence: 91%

A high urinary urobilinogen / serum total bilirubin ratio reported in abdominal pain patients can indicate acute hepatic porphyria

Song

Liu

2022

Preprint

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“…In the present study, we reported that the AHP patients showed a high level of apparent urinary urobilinogen for the first time. However, the serum bilirubin level was normal or only slightly elevated, consistent with the literature 10 , 13 , 15 , 17 , 18 . Urobilinogen is a product of bilirubin metabolism by anaerobic bacteria in the intestine.…”

Section: Discussionsupporting

confidence: 91%

A high urinary urobilinogen/serum total bilirubin ratio indicates acute hepatic porphyria in patients with abdominal pain

Song,

Liu

2023

Sci Rep

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Acute hepatic porphyria (AHP) has always been a diagnostic dilemma for physicians due to its variable symptoms. Correct diagnosis mainly depends on the detection of an elevated urinary porphobilinogen (PBG), which is not a routine test and highly relies on the physician’s awareness of AHP. In the present study, we identified a more convenient indicator during routine examinations to improve the diagnosis of AHP. We found that AHP patients showed a significant higher “FALSE” urinary urobilinogen level caused by urinary PBG during the urinalysis when detected by strips impregnated with Ehrlich reagent (P < 0.05). And a remarkable increase in the urinary urobilinogen/serum total bilirubin ratio was observed in AHP patients. The area under the ROC curve of this ratio for AHP was 1.000 (95% confidence interval 1.000–1.000, P < 0.01). A cutoff value of 3.22 for this ratio yielded a sensitivity of 100% and a specificity of 100% to distinguish AHP patients from the controls. Thus, we proved that a “falsely” high urinary urobilinogen level that was adjusted by the serum total bilirubin level (urinary urobilinogen/serum total bilirubin ratio) could be used as a sensitive and specific screening marker for AHP in patients with abdominal pain.

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“…Thus, a positive result shows in the urinalysis [18,19]. However, our study demonstrated that most of the AHP patients showed normal serum bilirubin levels, consistent with the literature [9,15,[20][21][22]. Therefore, we believe that the elevated urinary urobilinogen level in AHP group was a false-positive result caused by the urinary PBG, which was proved by Watson-Schwartz test ( gure 1).…”

Section: Discussionsupporting

confidence: 85%

A High Urinary Urobilinogen / Serum Total Bilirubin Ratio Reported in Abdominal Pain Patients Can Indicate Acute Hepatic Porphyria

Song

Sang

Liu

2021

Preprint

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Background: Due to its variable symptoms and nonspecific laboratory test results during routine examinations, acute hepatic porphyria (AHP) has always been a diagnostic dilemma for physicians. Correct diagnosis mainly depends on the elevated urinary porphobilinogen (PBG) level, which is not a routine test performed in the emergency department. Therefore, identifying a more convenient indicator during routine examinations is important to improve the diagnosis of AHP.Methods: In this retrospective study, we enrolled 12 AHP patients and 100 patients with abdominal pain of other causes as the control groups in Qilu hospital of Shandong University between 2015 and 2021. The clinical manifestations and laboratory result data including urinary urobilinogen/serum total bilirubin ratio were compared between these two groups. The diagnostic performance of urinary urobilinogen/serum total bilirubin ratio was measured as sensitivity, specificity, and accuracy. The cut-off for optimal clinical performance was determined by the receiver operator characteristic (ROC) curve. Results were considered significant at a P < 0.05.Results: Compared with the control groups, AHP patients showed a significantly higher urinary urobilinogen level (P < 0.05). However, we showed that the higher urobilinogen level was caused by a false-positive result due to elevated urine PBG. Hence, we used serum total bilirubin, an upstream substance of urinary urobilinogen synthesis, for calibration. A remarkable increase in the urinary urobilinogen/serum total bilirubin ratio was observed in AHP patients. The area under the ROC curve of this ratio for AHP was 1.000 (95% confidence interval, 1.000–1.000, P < 0.01). A cutoff value of 3.22 for the urinary urobilinogen/serum total bilirubin ratio yielded a sensitivity of 100% and a specificity of 100% to distinguish AHP patients from the controls.Conclusion: A reported high urinary urobilinogen level that was adjusted by the serum total bilirubin level (urinary urobilinogen/serum total bilirubin ratio) could be used as a sensitive and specific indicator for AHP in patients with abdominal pain.

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Acute intermittent porphyria precipitated by atazanavir/ritonavir

Cited by 7 publications

References 9 publications

A high urinary urobilinogen / serum total bilirubin ratio reported in abdominal pain patients can indicate acute hepatic porphyria

A high urinary urobilinogen / serum total bilirubin ratio reported in abdominal pain patients can indicate acute hepatic porphyria

A high urinary urobilinogen/serum total bilirubin ratio indicates acute hepatic porphyria in patients with abdominal pain

A High Urinary Urobilinogen / Serum Total Bilirubin Ratio Reported in Abdominal Pain Patients Can Indicate Acute Hepatic Porphyria

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