The Hoesch Test: Bedside Screening for Urinary Porphobilinogen in Patients with Suspected Porphyria

Lamon, Joel M.; With, Torben K.; Redeker, Allan G.

doi:10.1093/clinchem/20.11.1438

Cited by 47 publications

(14 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Before application of ALA, porphyria was excluded in all patients anamnestically and by the Hoesch test 19. Five hours before laparoscopic surgery was performed, the patients received a sterile, 1% ALA solution (pH 6.5) (Medac, Wedel, Germany), intraperitoneally.…”

Section: Methodsmentioning

confidence: 99%

Laparoscopic fluorescence detection of ovarian carcinoma metastases using 5‐aminolevulinic acid‐induced protoporphyrin IX

Löning

Diddens²,

Küpker

et al. 2004

Cancer

110

View full text Add to dashboard Cite

BACKGROUND The aim of the current clinical study was to evaluate the in vivo fluorescence detection of ovarian carcinoma metastases in a second‐look laparoscopic procedure after intraperitoneally applied 5‐aminolevulinic acid (ALA). METHODS Five hours before laparoscopic surgery, ALA was applied intraperitoneally via short infusion in a concentration of 30 mg/kg bodyweight in a sterile, 1% solution. Application of ALA resulted in the endogenous production of the fluorescent photosensitizer, protoporphyrin IX (PP IX). The Combilight PDD 5133 system served as a light source, permitting the switch from white light mode to blue light mode to excite the PP IX accumulated in the ovarian tissue specimens. By means of blue light illumination, intraperitoneally located red fluorescent lesions, which were suspected to be metastases, underwent a biopsy. In addition, several biopsy specimens were taken from nonfluorescent areas of the peritoneal cavity. RESULTS In 13 of 29 patients, ovarian carcinoma was confirmed histologically or cytologically. In 12 of these patients, metastases were visible by red fluorescence. In total, 123 biopsies were performed. Comparison of histologic assessment of the biopsy specimens with the fluorescence detection showed that strong red fluorescence had a sensitivity of 92% for detecting tumor tissue on specimens. In only 2% of all biopsy specimens was endometriosis observed in benign tissue specimens using fluorescence. In four of 13 patients with ovarian carcinoma, lesions were detected under fluorescence, which were not observed under white light illumination. CONCLUSIONS Laparoscopic fluorescence detection of endogenous PP IX after intraperitoneal application of ALA may provide a higher sensitivity of finding peritoneal metastases of epithelian ovarian carcinoma compared with conventional laparoscopy. Direct visualization of in vivo fluorescence after ALA application may improve the early detection of intraperitoneal ovarian carcinoma micrometastases. The high tissue selectivity of PP IX accumulation in tumor tissue specimens also offers the opportunity for therapeutic approaches using photodynamic therapy in the future. Cancer 2004. © 2004 American Cancer Society.

show abstract

Section: Methodsmentioning

confidence: 99%

Laparoscopic fluorescence detection of ovarian carcinoma metastases using 5‐aminolevulinic acid‐induced protoporphyrin IX

Löning

Diddens²,

Küpker

et al. 2004

Cancer

110

View full text Add to dashboard Cite

show abstract

“…The withdrawal from the market of the Trace Õ PBG kit by Thermo Scientific has left a gap in the methodology for rapid urinary PBG analysis. Previously widely used qualitative methods such as the Watson-Schwartz test 18 the Rimington method 19 and the Hoesch test 20 are all simple procedures with a lower detection limit of approximately 12 mol/L. However, these methods have been criticized for low sensitivity and poor specificity 21,22 and EQA data from WEQAS showed that at a concentration of 10.7 mol/L (upper reference limit) the percentage assessed as positive was 40% vs. 80% positive using the Trace Õ PBG kit (WEQAS porphyrin guide).…”

Section: Methodsmentioning

confidence: 99%

Best practice guidelines on first-line laboratory testing for porphyria

et al. 2017

View full text Add to dashboard Cite

The porphyrias are disorders of haem biosynthesis which present with acute neurovisceral attacks or disorders of sun-exposed skin. Acute attacks occur mainly in adults and comprise severe abdominal pain, nausea, vomiting, autonomic disturbance, central nervous system involvement and peripheral motor neuropathy. Cutaneous porphyrias can be acute or chronic presenting at various ages. Timely diagnosis depends on clinical suspicion leading to referral of appropriate samples for screening by reliable biochemical methods. All samples should be protected from light. Investigation for an acute attack: • Porphobilinogen (PBG) quantitation in a random urine sample collected during symptoms. Urine concentration must be assessed by measuring creatinine, and a repeat requested if urine creatinine <2 mmol/L. • Urgent porphobilinogen testing should be available within 24 h of sample receipt at the local laboratory. Urine porphyrin excretion (TUP) should subsequently be measured on this urine. • Urine porphobilinogen should be measured using a validated quantitative ion-exchange resin-based method or LC-MS. • Increased urine porphobilinogen excretion requires confirmatory testing and clinical advice from the National Acute Porphyria Service. • Identification of individual acute porphyrias requires analysis of urine, plasma and faecal porphyrins. Investigation for cutaneous porphyria: • An EDTA blood sample for plasma porphyrin fluorescence emission spectroscopy and random urine sample for TUP. • Whole blood for porphyrin analysis is essential to identify protoporphyria. • Faeces need only be collected, if first-line tests are positive or if clinical symptoms persist. Investigation for latent porphyria or family history: • Contact a specialist porphyria laboratory for advice. Clinical, family details are usually required.

show abstract

“…determination and quantification of porphyrins and non-porphyrin precursor patterns in biological samples) are mandatory for an accurate diagnosis, and for starting an appropriate clinical management [3,25,58,[90][91][92]94,95]. According to current knowledge and consensus, an APA is invariably associated with an increased urinary excretion of non-porphyrin precursors [ALA and PBG] [6,25,92,96,97] (Figure 2). For this reason, in case of suspected APA, a fresh light-protected urine sample should be assessed for ALA and PBG concentrations [to date, HPLC assays are the most accurate, but rapid, ion-exchange column tests (column-chromatographic screening tests) are also available] (first-line test) [66,[98][99][100][101][102].…”

Section: The Acute Porphyric Attack: Diagnosis and Differential Diagnmentioning

confidence: 99%

The Acute Porphyric Attack: A Difficult Diagnosis for a Potential Lethal Event in Emergency Medicine

Ventura¹

2015

ETS

View full text Add to dashboard Cite

The porphyrias are a heterogeneous group of metabolic disorders due to an inherited (but in some forms the disturbance may also be acquired) enzymatic deficiency in the metabolic pathway of heme biosynthesis. The variable degree of block in the heme biosynthetic pathway due to the enzyme deficiency results in accumulation of different metabolic intermediates, whose toxicity is responsible for the peculiar (cutaneous and/or neurovisceral) clinical pictures observed in each of these diseases. According to the clinical features, the porphyrias are classified as "acute" (or neuropsychic) [characterized by acute neurovisceral crises (the acute porphyric attack) involving the autonomic and/or central nervous system, but also the liver and the kidney] and "on acute" (or dermatological) (mostly presenting with cutaneous lesions, due to photosensitivity).

show abstract

The Hoesch Test: Bedside Screening for Urinary Porphobilinogen in Patients with Suspected Porphyria

Cited by 47 publications

References 0 publications

Laparoscopic fluorescence detection of ovarian carcinoma metastases using 5‐aminolevulinic acid‐induced protoporphyrin IX

Laparoscopic fluorescence detection of ovarian carcinoma metastases using 5‐aminolevulinic acid‐induced protoporphyrin IX

Best practice guidelines on first-line laboratory testing for porphyria

The Acute Porphyric Attack: A Difficult Diagnosis for a Potential Lethal Event in Emergency Medicine

Contact Info

Product

Resources

About