The power of the Hoesch test

Authors

  • Juan José Nava Mateos Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Vicente Gómez del Olmo Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Marta Rosas Cancio-Suárez Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Raquel Besse Díaz Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Olivia Sánchez Sánchez Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Diego Cebrian Novella Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid, España
  • Rafael Enriquez de Salamanca Llorente Departamento de Medicina, Universidad Complutense. Laboratorio de Porfirias del Hospital Universitario 12 de Octubre, Madrid.

DOI:

https://doi.org/10.19230/jonnpr.2016.1.1.927

Keywords:

Acute porphyria, porphyric crisis, Hoesch test, hemine, porphobilinogen.

Abstract

Acute porphirias are a rare group of diseases in which the main clinical expression are abdominopsychoneurological crisis. The most typical symptom is abdominal pain.
If left untreated, acute porphyria attacks are associated with a high mortality rate (about 10%). Early diagnosis is very important. A rapid test to detect porphobilinogen (PBG) called the Hoesch test can be used for this purpose. If we have a positive test we can affirm that the clinical issues are induced by a porphyric attack. With this background, early treatment must be started with human hemin.
Our purpose is to prove the utility of the Hoesch test in the treatment period. Therefore, we made daily Hoesch tests in a porphyria crisis. We noticed that the Hoesch test remains positive without symptoms. It seems that the Hoesch test does not offer information to take the decision to stop treatment. More patients are necessary to prove this conclusion.

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References

Raili Kauppineu. Porphyrias. Lancet 2005; 365: 241-52.

R. Enriquez de Salamanca Lorente. Porfirias. Farreras-Rozman, Medicina Interna. Volumen 1. Capítulo 4,

páginas 22-29. 17ª edición. Barcelona: Elsevier, 2012.

Lamon JM, Fryklhom BC, Hess RA, Tschudy DP. Hematin therapy for acute porphyria. Medicine (Baltimore)

; 58 (3); 252-69.

No authors listed. Treatment of acute hepatic porphyria. Lancet 1978, Jun 24; 1 (8078): 1361-2.

Mustajki P, Nordmann Y. Early administration of heme argininato for acute porphyria attacks. Arch Internal Med

;13; 153 (17): 2004-8.

Walderburg M, Bonnot O, Mocellini R, Velakoulis D. The neuropsychiatrics of inborn errors of metabolism. J

Inherited Met Dis 2013; 36: 687-702.

Lamon J, With TK, Redeker AG. The Hoesch test: Bedside Screening for urinary porphobilinogen in patients with

suspected pophyria. Clin Chem 1979;20/11, 1438-1440.

A S Winkler, T J Peters, R D C Elwes. Neuropsychiatric porphyria in patients with refractory epilepsy: report of

three cases. J Neurol Neurosurg Psychiatry 2005;76:380–383.

Ylva Floderus, Eliane Sardh, Christer Möller, Claes Andersson, Lillan Rejkjaer, Dan E.H. Andersson et al.

Variations in Porphobilinogen and 5-Aminolevulinic Acid Concentrations in Plasma and Urine from Asymptomatic

Carriers of the Acute Intermittent Porphyria Gene with Increased Porphyrin Precursor Excretion. Clinical

Chemistry 2006;52:4 701–707.

F. Sedel, N. Baumann, J.-C. Turpin, O. Lyon-Caen, J.-M. Saudubray, D. Cohen. Psychiatric manifestations

revealing inborn errors of metabolism in adolescents and adults. J Inherit Metab Dis 2007; 30:631–641.

Besur S, Hou W, Schmeltzer P, Bonkovsky HL. Clinically important features of porphyrin and heme metabolism

and the porphyrias. Metabolites. 2014;3:4(4):977-1006.

Published

2016-05-16