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Protocol for patients with suspected acute porphyria
Protocolo de actuación en pacientes con sospecha de porfiria aguda
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Castelbón Fernández, I. Solares Fernandez, E. Arranz Canales, R. Enríquez de Salamanca Lorente, M. Morales Conejo" "autores" => array:5 [ 0 => array:3 [ "nombre" => "F.J." "apellidos" => "Castelbón Fernández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:3 [ "nombre" => "I." "apellidos" => "Solares Fernandez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "E." "apellidos" => "Arranz Canales" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "R." "apellidos" => "Enríquez de Salamanca Lorente" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 4 => array:4 [ "nombre" => "M." "apellidos" => "Morales Conejo" "email" => array:1 [ 0 => "moralmon@yahoo.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Grupo CSUR de errores congénitos del metabolismo en el adulto, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Protocolo de actuación en pacientes con sospecha de porfiria aguda" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 4023 "Ancho" => 2917 "Tamanyo" => 526969 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">xxx.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Porphyria are a group of diseases resulting from congenital errors in porphyrin metabolism and in the heme biosynthetic pathway. Eight enzymatic steps are needed from the conversion of glycine and succinyl-CoA to heme biosynthesis. Each type of porphyria is the result of a pathway enzyme disorder, and the accumulation of intermediate products can cause the characteristic clinical manifestations. The increase in porphyrin precursors (delta-aminolevulinic acid [ALA] and porphobilinogen) is related to acute neurovisceral crises (acute porphyria), while the overproduction of porphyrin photosensitizers is associated with skin lesions (cutaneous porphyria).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">There are 4 types of acute porphyria: acute Doss porphyria (of which only some 8 cases have been detected), acute intermittent porphyria, hereditary coproporphyria and porphyria variegata. The latter two are called mixed porphyria because they can also manifest with skin symptoms. Acute intermittent porphyria is the most common porphyria subtype, with a prevalence of the mutation in population studies of approximately 1 case per 1700 individuals, although with very low penetrance (<1%), with an estimated prevalence of 6 cases per 100,000 inhabitants.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4–6</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical presentation</span><p id="par0015" class="elsevierStylePara elsevierViewall">Acute porphyria are expressed in the form of crises characterized mainly by intense abdominal pain (>95%), vomiting, anxiety, constipation, tachycardia and hypertension. Severe crises can be complicated by the onset of hyponatremia, convulsions, neuropsychiatric disorders and peripheral neuropathy, which can progress to tetraparesis and respiratory muscle impairment.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The age range for presenting acute crises varies from adolescence to the eighth decade of life. Crises are rare before puberty and manifest most often in women.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Most patients (80%–90%) who carry a mutation associated with porphyria will never present symptoms. The phenotypic expression depends on the type of causal mutation and on the presence of precipitating factors.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Triggers</span><p id="par0030" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1</span><p id="par0035" class="elsevierStylePara elsevierViewall">Fasting.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2</span><p id="par0040" class="elsevierStylePara elsevierViewall">Cytochrome p450 inducers (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>), which include pyrazolones (metamizole) and the “day after pill”, given its high concentration of progestogens.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3</span><p id="par0045" class="elsevierStylePara elsevierViewall">For more information on other drugs, consult <a href="http://www.drugs-porphyria.org">http://www.drugs-porphyria.org</a>.</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">4</span><p id="par0050" class="elsevierStylePara elsevierViewall">Toxins: alcohol, tobacco and other drugs.</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">5</span><p id="par0055" class="elsevierStylePara elsevierViewall">Hormonal changes: premenstrual phase, pregnancy.</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">6</span><p id="par0060" class="elsevierStylePara elsevierViewall">Others: infections, stress.</p></li></ul></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Diagnosing an acute porphyric crisis</span><p id="par0065" class="elsevierStylePara elsevierViewall">During acute crises, urinary elimination of porphobilinogen and ALA precursors is always very high. Excessive porphobilinogen concentration in urine is easily identified using the Hoesch test, a simple test available in emergency departments: 2 drops of urine recently emitted by the patient are added to a 1-mL aliquot of modified Ehrlich’s reagent (previously stored in a refrigerator or freezer). The solution is then gently shaken; a reddish tint then develops in the reagent. A negative test rules out the abdominal pain being the result of a current porphyric crisis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9,10</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0070" class="elsevierStylePara elsevierViewall">For all allegedly positive tests, a urine sample should be frozen for later testing and study. Photographic evidence is recommended both of the urine and the test performed.</p><p id="par0075" class="elsevierStylePara elsevierViewall">A positive test requires subsequent more complex biochemical studies that can confirm the diagnosis of porphyria and establish its variety.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Marked positivity in the Hoesch test is highly suggestive of acute porphyria in crisis phase. An early start to specific therapy (hemin) is therefore advisable, if the clinical situation recommends it, to prevent the onset of neurological complications.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Managing an acute porphyric crisis</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Initial assessment</span><p id="par0085" class="elsevierStylePara elsevierViewall">When reviewing the medical history of a patient in the emergency department with suspected acute porphyria and a positive Hoesch test, it is essential to investigate the highly likely involvement of the previously mentioned potential factors and triggers.</p><p id="par0090" class="elsevierStylePara elsevierViewall">During the physical examination, special attention should be paid to the presence of arterial hypertension and tachycardia. In the event of low oxygen saturation or dyspnea, the ventilatory function (peak flow) should be assessed. If the patient presents signs of motor neuropathy or respiratory muscle paralysis is suspected, the intensive care unit should be notified.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11,12</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">The most common fluid and electrolyte disorder is hyponatremia, caused mainly by a syndrome of inappropriate antidiuretic hormone secretion (SIADH). Moderate to severe hyponatremia is an indication for hemin treatment. For patients with severe hyponatremia (<120 mEq/L), the recommendation is an intensive care assessment.</p><p id="par0100" class="elsevierStylePara elsevierViewall">The onset of acute renal failure and rhabdomyolysis are rare complications in acute porphyria crises but should be taken into account.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11,12</span></a> Obviously, other additional tests should be performed based on the patient’s clinical data and to identify potential triggers.</p><p id="par0105" class="elsevierStylePara elsevierViewall">A crisis should be considered moderate-severe if it is associated with the following:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">•</span><p id="par0110" class="elsevierStylePara elsevierViewall">Neurological symptoms (encephalopathy, muscle weakness, respiratory impairment).</p></li><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">•</span><p id="par0115" class="elsevierStylePara elsevierViewall">Moderate-severe hyponatremia.</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">•</span><p id="par0120" class="elsevierStylePara elsevierViewall">Abdominal pain that requires hospitalization and major opioids to control it.</p></li></ul></p><p id="par0125" class="elsevierStylePara elsevierViewall">Acute porphyric crises are very uncommon in childhood; less than 5% of acute crises occur in children younger than 14 years. At this age, the crises are more common in boys than in girls. Acute porphyria should probably be included in the differential diagnosis of abdominal pain of unknown origin in late childhood and adolescence.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">13,14</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Suppression of potential triggers</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Specific treatment</span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Hemin: heme arginate (Normosang®), Panhematin® in countries other than Spain</span><p id="par0130" class="elsevierStylePara elsevierViewall">The drug’s mechanism of action consists of suppressing the hyperactivity of the first pathway enzyme (delta-aminolevulinic acid synthase [ALAS1]) through the supply of heme. The suppression of ALAS1 causes a reduction in the synthesis of the toxic precursors ALA and porphobilinogen. The drug is indicated for all moderate-severe crises.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–4,7,11,12</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">The administration regimen is 3–4 mg/kg of body weight (maximum dosage, 250 mg/day), dissolved in 100 mL of 0.9% physiological saline solution (PSS) (best in glass container) administered intravenously, preferentially by the central pathway, in a single 30-min daily dose for 4 days.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–4,7,11,12</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> In the event a peripheral pathway is employed, a different vein should be used each day (given the high risk of phlebitis), and the solution should be diluted in 100 mL of 5%–20% albumin, with subsequent flushing with PSS. The diluted solution should be protected from light (e.g., inside an opaque or aluminum-covered bag) and should be administered within 1 h of its preparation. The drug may be administered during pregnancy.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,11</span></a></p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Carbohydrate overload</span><p id="par0140" class="elsevierStylePara elsevierViewall">Glucose and other carbohydrates have a repressive effect on the induction of hepatic ALAS1, thereby reducing the excretion of porphyrin precursors.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,4</span></a> However, this effect is lower than that obtained with hemin. The plasma precursors are barely altered after the administration of glucose.</p><p id="par0145" class="elsevierStylePara elsevierViewall">In acute mild crises, intravenous therapy may be started with 200 g of glucose (10% glucose serum, 500 mL infusion every 6 h). In these cases, it is important to perform an ionogram check, especially exhaustive for natremia readings, because glucose serum can exacerbate the SIADH presented by these patients during acute crises.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,11,12</span></a></p></span></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Symptomatic treatment</span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Pain</span><p id="par0150" class="elsevierStylePara elsevierViewall">Patients often present intense and difficult-to-control abdominal pain. Opioids, both major and minor, are the basis of analgesic treatment for porphyria crises and are safe and effective. Paracetamol is safe but has little efficacy. Metamizole (Nolotil®) should be avoided due to its porphyrinogenic potential. Once the acute crises is over, the opioid-based therapy should be withdrawn. Marked improvement in the abdominal pain is expected after the third dose of hemin.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,11,12</span></a></p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Hyponatremia</span><p id="par0155" class="elsevierStylePara elsevierViewall">In terms of the need for and speed of sodium replenishment, the condition is treated like any hyponatremia secondary to SIADH. As mentioned previously, the presence of moderate-severe hyponatremia is an indication for treatment with human hemin.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11,12</span></a></p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Nausea and vomiting</span><p id="par0160" class="elsevierStylePara elsevierViewall">These symptoms are often present during acute porphyria crises and can be controlled with ondansetron or chlorpromazine, which are preferred over metoclopramide.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,11,12</span></a></p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Seizures</span><p id="par0165" class="elsevierStylePara elsevierViewall">These can be due to a direct central dysfunction or secondary to the hyponatremia associated with SIADH. Management consists on treating the underlying cause; however, the use of benzodiazepines or levetiracetam is appropriate.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Hypertension and tachycardia</span><p id="par0170" class="elsevierStylePara elsevierViewall">To control AHT and tachycardia, beta blockers (atenolol, propranolol, labetalol) may be employed. Patients should be monitored closely for the onset of hypotension or bradycardia because the patients are frequently dehydrated and have autonomic dysfunction.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,11,12</span></a></p></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Anxiety and insomnia</span><p id="par0175" class="elsevierStylePara elsevierViewall">Short-acting low-dose benzodiazepines (lorazepam) may be administered.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">11,12</span></a></p></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Infection</span><p id="par0180" class="elsevierStylePara elsevierViewall">Decompensation secondary to infection should be treated early and with appropriate antibiotherapy, avoiding the family of sulfonamides (cotrimoxazole).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–4,7,11,12</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p></span></span></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1432567" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1307724" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1432568" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1307723" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Clinical presentation" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Triggers" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Diagnosing an acute porphyric crisis" ] 7 => array:3 [ "identificador" => "sec0020" "titulo" => "Managing an acute porphyric crisis" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0025" "titulo" => "Initial assessment" ] 1 => array:3 [ "identificador" => "sec0030" "titulo" => "Suppression of potential triggers" "secciones" => array:2 [ 0 => array:3 [ "identificador" => "sec0035" "titulo" => "Specific treatment" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0040" "titulo" => "Hemin: heme arginate (Normosang®), Panhematin® in countries other than Spain" ] ] ] 1 => array:2 [ "identificador" => "sec0045" "titulo" => "Carbohydrate overload" ] ] ] 2 => array:3 [ "identificador" => "sec0050" "titulo" => "Symptomatic treatment" "secciones" => array:7 [ 0 => array:2 [ "identificador" => "sec0055" "titulo" => "Pain" ] 1 => array:2 [ "identificador" => "sec0060" "titulo" => "Hyponatremia" ] 2 => array:2 [ "identificador" => "sec0065" "titulo" => "Nausea and vomiting" ] 3 => array:2 [ "identificador" => "sec0070" "titulo" => "Seizures" ] 4 => array:2 [ "identificador" => "sec0075" "titulo" => "Hypertension and tachycardia" ] 5 => array:2 [ "identificador" => "sec0080" "titulo" => "Anxiety and insomnia" ] 6 => array:2 [ "identificador" => "sec0085" "titulo" => "Infection" ] ] ] ] ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2019-09-20" "fechaAceptado" => "2019-10-01" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1307724" "palabras" => array:3 [ 0 => "Porphyria" 1 => "Acute" 2 => "Protocol" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1307723" "palabras" => array:3 [ 0 => "Porfiria" 1 => "Aguda" 2 => "Protocolo" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Porphyria are a group of congenital errors in porphyrin metabolism and in the heme biosynthetic pathway. Accumulation of porphyrin precursors (delta-aminolaevulinic acid and porphobilinogen) is responsible for the neurovisceral crises of acute porphyria, which, when expressed clinically, start with intense abdominal pain. During crises, the urinary elimination of porphobilinogen and delta-aminolaevulinic acid is always very high. Excessive porphobilinogen concentration in urine is easily identified using the simple Hoesch test. A negative test rules out a current porphyric crisis. The clinical protocol for patients with acute abdominal pain of unknown origin in whom a positive Hoesch test leads to the suspicion of acute porphyria is based on the following aspects: initial clinical assessment in the emergency department, suppression of potential triggers, specific treatment for the crisis with hemin and/or glucose overload and symptomatic treatment.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Las porfirias son errores congénitos del metabolismo de las porfirinas o ruta biosintética del hemo. El acúmulo de los precursores de las porfirinas, ácido delta aminolevulínico (ALA) y/o porfobilinógeno (PBG) es responsable de las crisis neuroviscerales de las porfirias agudas que cuando se expresan clínicamente se inician con intenso dolor abdominal. Durante las crisis la eliminación urinaria de PBG y ALA siempre es muy elevada. La excesiva concentración de PBG en orina es fácilmente identificable mediante el sencillo test de Hoesch. Un test negativo descarta crisis porfírica actual. El protocolo de actuación en pacientes con dolor abdominal agudo no filiado en los que el test de Hoesch positivo permite la sospecha de porfiria aguda se basa en los siguientes aspectos: valoración clínica inicial en el servicio de urgencias, supresión de los posibles factores desencadenantes, tratamiento específico de la crisis con hemina y/o sobrecarga de glucosa y tratamiento sintomático.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Castelbón Fernández FJ, Solares Fernandez I, Arranz Canales E, Enríquez de Salamanca Lorente R, Morales Conejo M. Protocolo de actuación en pacientes con sospecha de porfiria aguda. Rev Clin Esp. 2020;220:592–596.</p>" ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 4023 "Ancho" => 2917 "Tamanyo" => 526969 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">xxx.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Barbiturates \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Carbamazepine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Carisoprodol \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ergotamines \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hydantoins \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Griseofulvin \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Glutethimide \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Alpha-methyldopa \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Methyprylon \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Meprobamate \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pentazocine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Primidone \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pyrazolones (metamizole) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pyrazinamide \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Progestogens \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Succinimides \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Sulfonamides (cotrimoxazole) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2463172.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Porphyrinogenic drugs.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:15 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute hepatic porphyrias: Current diagnosis & management" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "K.E. 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