Interval Hemin Therapy: Efficacy in Reducing Hospitalizations for Acute Attacks of Acute Intermittent Porphyria.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3771-3771
Author(s):  
Rene Rothstein-Rubin
Keyword(s):  
Abdominal Pain ◽  
Hospice Care ◽  
Aminolevulinic Acid ◽  
Caloric Intake ◽  
Acute Intermittent Porphyria ◽  
Care Patient ◽  
Cytochrome P450 Enzymes ◽  
Biochemical Basis ◽  
Life Threatening ◽  
Acute Attacks

Abstract Background Acute intermittent porphyria (AIP), is one of the acute porphyrias resulting from deficient activity of a distinct enzyme in the heme biosynthetic pathway. Porphobilinogen deaminase, is the enzyme in AIP with approximately 50% activity1. This predisposes individuals to factors exacerbating the disease, including drugs inducing heme synthesis and cytochrome P450 enzymes, steroids, dieting, smoking, stress, and infection. Clinically, AIP is characterized by visceral, autonomic, peripheral, and CNS involvement, leading to varying degrees of intermittent and life-threatening symptoms. Despite avoidance of these factors, frequent attacks may persist due to unidentified modifier genes or environmental/endogenous factors1. Recurrent noncyclic attacks may be prevented by weekly or biweekly infusions of hemin2. Objective To report the results of the prevention of acute life-threatening attacks of AIP by a multidisciplinary team leading to a 50–100% decrease in patient hospitalizations. Methods Three patients were diagnosed with AIP on the biochemical basis of increased urinary porphobilinogen and aminolevulinic acid levels. All patients required hospitalization over 3–5 years due to severe abdominal pain and inability to maintain caloric intake and hydration. Due to recurrent attacks, hemin 313 mg was initiated on a prophylactic basis and frequency of administration was dependent on activity of their disease. All 3 patients served as their own control and the outcome of hemin prophylaxis was measured by patient symptoms, narcotic requirements, physical examination, and hospitalizations. Results Three patients (1 female, 2 male) with a mean age of 58 years had recurrent attacks of AIP. Patient #1 was hospitalized monthly over 5 years and received hemin for 10 days during acute attacks. Hemin infusions 1/month was initiated, and hospitalizations decreased by 50% until discontinued due to severe cardiomyopathy (unknown if related to porphyria). She expired in hospice care. Patient #2 was classified as a drug seeker for 3 years. After diagnosis, he was hospitalized almost monthly for 3–5 days during acute attacks over 2–3 years. Hemin was infused 1/month and symptoms persisted. Infusions were increased to 2/month. Symptoms and narcotic requirements have decreased and he has not been hospitalized. Patient #3 experienced acute attacks during stress, increasing due to business travel abroad. He was hospitalized 4 times in 3 years. As a nurse, he self-administers 1/month and in the office approximately every 2–3/months. 2 of 3 patients experienced phlebitis during infusions and receive via a portacath with no further adverse events. 2 of 3 patients have not required hospitalizations for acute attacks, symptoms have decreased and/or resolved, as well as narcotic requirements. Conclusions The experience in management of these three patients demonstrates the safety and effectiveness of hemin as a prophylactic agent in AIP. Most patients will require treatment 1–2 times/month and can avoid painful crisis and hospitalizations. PATIENT DATA, TREATMENT, AND RESULTS Patient Age Sex Yr Diagnosis Presenting Sxs Txment Frequency Hospitalizations 1 66 F 1990 Abdominal Pain, Difficulty Urinating 1 month/5yrs 50% ↓ 2 52 M 1996 Abdominal Pain, Neuropathic Sxs 2 month/2 yrs 100%↓ 3 56 M 2002 Abdominal Pain, Paresthesias 1 month/8 mos 100%↓

A Case Report of Prophylactic Hemin Treatments in the Management of Patients with Life-Threatening Attacks of Acute Intermittent Porphyria.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3757-3757
Author(s):  
Richard E. Mills
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Motor Function ◽  
Mental Status ◽  
Acute Intermittent Porphyria ◽  
Acute Attack ◽  
Precipitating Factors ◽  
Presenting Symptoms ◽  
Life Threatening ◽  
Acute Attacks

Abstract Background The acute porphyrias are a group of 4 genetic disorders resulting from a deficiency in a specific enzyme of the heme biosynthetic pathway (1). Without prompt treatment, these disorders can cause acute life-threatening attacks of neurovisceral symptoms, the most common being abdominal pain, nausea, vomiting, mental symptoms, paresis, and tachycardia (1). Frequent, non cyclic attacks can sometimes be prevented with weekly or bi-weekly infusions of hemin (2). Case Report A 25-year-old female was initially diagnosed with acute intermittent porphyria (AIP) at 16 years of age. Her initial presenting symptoms included acute abdominal pain, nausea, vomiting, and a seizure. The patient was subsequently diagnosed by the evidence of elevated urinary porphobilinogen levels. She was initially treated with fluids (D5W) and then with hemin (313mg) for 3 days. The patient is a smoker and occasionally drinks alcohol. At 22 years of age, she was hospitalized for her fifth acute attack of AIP and received treatment with fluids and a course of hemin (313mg) for three days. Her condition improved and she was discharged. Two days later, her condition deteriorated and she was readmitted. She developed pneumonia, hyponatremia, seizures, and mental-status changes. The patient became tetraplegic and developed progressive respiratory failure requiring ventilatory support. She remained comatose for a period of 48 hours. Diagnostic studies included a noncontrast CT scan of the abdomen/pelvis which was unremarkable and EMG nerve conduction studies demonstrating polyneuropathies. Treatment included D5W, then increased to D10W. Following placement of a PICC line, hemin (313mg) was administered daily. Hemin therapy was continued daily for a period of three weeks and then stepped down to twice/week (313mg) every week in a prophylactic fashion. Her hospital course was prolonged as a result of the following: MRSA, VRE, superventricular tachycardia, arrhythmias, fungal infection, pulmonary infection and a clot in her right subclavian vein. She also experienced intermittent mental status changes. She was discharged to a rehabilitation center following her 11 month hospital stay. Upon discharge, her paresis had resolved, although a loss of motor function persisted. The patient was discharged with hemin therapy for prophylaxis twice/week (313mg with albumin 25%) via a portacath. She has experienced one acute attack of AIP during her course of prophylaxis during a three year period. The patient’s current medical status is that she is alert and oriented with the ability to work from home utilizing a telephone and computer. She remains wheel chair bound with loss of motor function. Discussion: Delay of treatment, and the delay in treating the pathophysiology of the disease itself can cause life-threatening attacks. Acute intermittent Porphyria is a disease that is challenging to manage in this particular patient. It is important for patients to recognize the triggers that exacerbate an attack. This particular patient still has 2 precipitating factors for acute attacks, smoking and alcohol. The prophylactic dosing regimen for this patient has been effective despite the presence of these precipitating factors. This case report is an example of the effectiveness of hemin therapy in the prevention of acute attacks of AIP.

Porphyrias

2016 ◽  
Author(s):  
Janneke G. Langendonk ◽  
Timothy M. Cox
Keyword(s):  
Abdominal Pain ◽  
Aminolevulinic Acid ◽  
Acute Intermittent Porphyria ◽  
Heme Biosynthesis ◽  
Reproductive Age ◽  
Systemic Complications ◽  
Skin Symptoms ◽  
Excess Production ◽  
Acute Attacks ◽  
Acute Porphyrias

The porphyrias are disorders caused by overproduction of metabolites involved in heme biosynthesis. The four acute porphyrias— acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), and Doss Porphyria—present with severe abdominal pain, often accompanied by agitation, hypertension, and tachycardia associated with neuropathy and sometimes paralysis. Painful and disabling neurovisceral attacks are due to excess production of the heme precursor ALA (delta-aminolevulinic acid).While 90% of individuals with an inherited defect in heme biosynthesis will never develop symptoms, acute attacks in those affected are provoked by drugs, fasting, and alcohol; in women of reproductive age, they usually occur in the progestagenic phase of the menstrual cycle. All other porphyrias are considered cutaneous porphyrias. They present with blisters or pain on light exposed areas, toxic porphyrins accumulate and give rise to skin symptoms. The cutaneous porphyrias (PCT, EPP, XLEPP, and HEP) do not present with acute neurovisceral attacks (e.g., abdominal pain). However, severe systemic complications can occur.

scholarly journals Hypertensive Crisis in Patients with Acute Intermittent Porphyria

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Olivera Andrejic ◽  
Rada Vucic ◽  
Violeta Iric Cupic ◽  
Goran Davidovic
Keyword(s):  
Abdominal Pain ◽  
Aminolevulinic Acid ◽  
Beta Blockers ◽  
Hypertensive Crisis ◽  
Acute Intermittent Porphyria ◽  
Gas Analysis ◽  
Urine Test ◽  
Arterial Blood Gas ◽  
Arterial Blood ◽  
Clinical Centre

AbstractIntroductionAcute intermittent porphyria (AIP) is the most common and the most severe form of acute hepatic porphyria.Case reportPatient, 39 years old, was admitted to the Emergency Department because of abdominal pain. Abdominal pain started 5 days before the admission. The diagnostic research in his hospital showed presence of a stone in the right kidney, and the patient was transported to the other Clinical Centre, where a common urine test showed: high values of delta - aminolevulinic acid and porphobilinogen. The patient was transported to our Clinical Centre.At the admission, abdominal pain decreased, but the patient had a hypertensive crisis with a headache, tearing eyes, swelling, anxiety. Common laboratory tests were in reference range, except creatinine, CRP, arterial blood gas analysis, urine test. The hypertensive crisis was treated by beta blockers and diuretics in maximal doses, but without a positive effect, so we decided to try with Glyceryl trinitrate intravenously. Control blood pressure was 170/100mmHg….130/80mmHg.DiscussionPorphyria can be a diagnostic problem, because one of the manifestations can be abdominal pain.ConclusionsComorbidities can be critical in the therapy of life threating conditions.

scholarly journals Secret Underlying Unexplained Abdominal Pain, Neurological Symptoms and Intermittent Hypertension: Acute Intermittent Porphyria

2017 ◽  
Vol 15 (1) ◽  
pp. 35-38
Author(s):  
Andac Komac ◽  
Elif Gram ◽  
Feray Gulec ◽  
Harun Akar
Keyword(s):  
Abdominal Pain ◽  
Aminolevulinic Acid ◽  
Abdominal Cavity ◽  
Gene Mutations ◽  
Acute Intermittent Porphyria ◽  
Recurrent Abdominal Pain ◽  
Neurological Symptoms ◽  
Heterozygous Mutation ◽  
Abdominal Organs ◽  
Mediterranean Fever

AbstractA 21-year-old female patient with abdominal pain, vomiting and constipation was admitted to the hospital with the possible diagnosis of diabetic ketoacidosis. Due to increased abdominal pain and constipation the patient underwent a surgery with the diagnosis of ileus. However, no pathological findings were found in the abdominal organs apart from serous fluid in the abdominal cavity. The patient became hypertensive, tachycardic and had an episode of seizures postoperatively. Neurological manifestations with unexplained abdominal pain indicated a diagnosis of acute intermittent porphyria (AIP). Acute intermittent porphyria diagnosis is based on elevated urinary δ-aminolevulinic acid (ALA) and porphobilinogen (PBG) levels as well as hydroxymethylbilane synthase (HMBS) IVS13-2 A>G heterozygous mutation. Familial Mediterranean Fever (FMF) gene mutations were not confirmed. Porphyria should be considered in the differential diagnosis of patients with recurrent abdominal pain, neurological symptoms and lack of FMF gene polymorphism.

scholarly journals Hyponatremia and Isolated Free T4 Elevation in a Patient With Acute Intermittent Porphyria

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A585-A586
Author(s):  
Anand Gandhi ◽  
Michael Mortensen ◽  
Mahmoud Alsayed ◽  
Aditi Kumar ◽  
Jerome H Targovnik
Keyword(s):  
Abdominal Pain ◽  
Thyroid Function ◽  
Aminolevulinic Acid ◽  
Acute Intermittent Porphyria ◽  
Laboratory Evaluation ◽  
Porphobilinogen Deaminase ◽  
Thyroid Function Tests ◽  
Free T4 ◽  
Deiodinase Activity ◽  
Wide Range

Abstract Background: Porphyrias represent a spectrum of diseases that stem from dysfunction within the heme biosynthetic pathway. Acute intermittent porphyria (AIP) is the most common type of porphyria due to a genetic deficiency of porphobilinogen deaminase which results in a wide range of neurovisceral symptoms. Hyponatremia and abnormalities with thyroid function have been found in AIP but the mechanisms behind these processes are unclear. Clinical Case: A 26-year-old male with a history of chronic, recurrent abdominal pain presented with 10 days of progressively worsening periumbilical abdominal pain and constipation. He describes that preceding the onset of symptoms he had been binging 5-10 standard alcoholic drinks each day for a few days. Initial laboratory workup demonstrated hyponatremia with Na 114 mmol/L (n: 134 – 137 mmol/L), hypochloremia with Cl 76 mmol/L (n: 95 – 108 mmol/L), and hyperbilirubinemia with total bilirubin 2.5 mg/dL (n: 0.2 – 1.3 mg/dL). CT abdomen/pelvis was negative for any concerning pathology. Further studies showed low serum osmolality at 243 mOsm/kg (n: 275 – 295 mOsm/kg), urine Na 62 mmol/L (n > 20mmol/L), and urine osmolality at 394 mOsm/kg (n: 300 – 900 mOsm/kg) consistent with SIADH. The patient was treated with 3% NaCl and free water restriction to 1.2L/day with improvement in Na levels. Further laboratory workup demonstrated normal TSH, but persistently elevated free T4 with maximum free T4 of 2.77 ng/dL (n: 0.80 – 1.70 ng/dL) which downtrended to 1.99 ng/dL by discharge. Thyroid ultrasound was unremarkable. Pituitary evaluation via hormonal workup and MRI brain was negative for any abnormalities. Given the symptomatology and laboratory findings, the patient was evaluated for porphyria. Laboratory evaluation demonstrated severe elevations in urine porphyrins, urine delta aminolevulinic acid (56.8 mg/24h, n < 4.5 mg/24h), and urine porphobilinogen (82.5 mg/g, n < 2.3 mg/g) consistent with AIP. The patient was treated with four days of hematin infusions which resolved his abdominal pain and was discharged in an improved state. Conclusion: AIP is a rare entity brought out by a deficiency in porphobilinogen deaminase, a key enzyme in the heme biosynthesis pathway. Various metabolic disturbances have been described in AIP including hyponatremia and alterations in thyroid function tests suggestive of thyrotoxicosis. Hyponatremia in our patient was likely due to SIADH from neurovisceral pain. Our patient displayed isolated free T4 elevation as well. We hypothesize this developed due to his acute illness causing a greater decrease in D2 deiodinase activity compared to any concomitant increase in D3 deiodinase activity. This was supported by his free T4 level downtrending following treatment of his AIP attack. More research is needed to further elucidate the mechanism behind these derangements in biochemical markers and their impact on patient prognosis.

scholarly journals MON-067 A Perplexing Case of Hyponatremia and Abdominal Pain

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Olga Yeliosof ◽  
Sadana Balachandar
Keyword(s):  
Urinary Tract Infection ◽  
Abdominal Pain ◽  
Urinary Tract ◽  
Tract Infection ◽  
Serum Sodium ◽  
Acute Intermittent Porphyria ◽  
Unknown Etiology ◽  
Fluid Restriction ◽  
Abdominal Symptoms ◽  
Acute Attacks

Abstract Previously healthy 20-year-old female presented with diffuse lower abdominal pain, cramping in nature, multiple episodes of emesis as well as urinary frequency. On day 2 of symptoms, she was treated for a urinary tract infection with antibiotics, as well as NSAIDs and opiates for pain relief. Her serum sodium was 133 mmol/L at this time. On day 3 of symptoms, a CT scan of the abdomen was performed however did not reveal pathology. Her serum sodium was 129 mmol/L at this time. She presented to our ED, on day 5 of symptoms, where serum sodium was down to 122 mmol/L. Despite IV fluids, her sodium continued to decrease to a nadir of 117 mmol/L. Further testing, including a serum osmolality of 242 mOsm/kg, urine osmolality of 540 mOsm/kg, and urine sodium of 207 mmol/L, was consistent with a diagnosis of SIADH. Given persistence of abdominal symptoms along with SIADH further imaging studies, including US abdomen, CT brain and Chest XR, were ordered and returned unremarkable. The constellation of SIADH along with persistent abdominal pain, with negative imaging, lead to consideration of acute intermittent porphyria as a diagnosis. Random urine porphobilinogen was found to be elevated to 147.2 mcmol/L (≤ 2.4) leading to the presumptive diagnosis of acute intermittent porphyria presenting as a neurovisceral attack. Biochemical and genetic testing is being pursued to confirm her diagnosis. Acute intermittent porphyria is an autosomal dominant hematologic disorder characterized by deficiency in porphobilinogen deaminase, an enzyme in the heme synthesis cascade. Acute attacks are caused by accumulation of porphyrin resulting in autonomic and peripheral neuropathy which can present as abdominal pain, urinary retention, polyneuropathy, dark urine and psychiatric disturbance. Hyponatremia is present in 25–60% of cases which is caused by SIADH or sometimes renal and gastrointestinal sodium loss. Triggers for acute attacks include medications, starvation, infections, hormonal changes and alcohol. Treatment includes avoidance of triggers, IV dextrose and high carbohydrate diet. In severe attacks, IV hemin is used. Our patient’s urinary tract infection likely triggered her acute symptoms, which was further exacerbated by treatment with NSAIDS and opiates. She developed SIADH which improved with hypertonic saline and fluid restriction. This case illustrates the need to consider acute intermittent porphyria in the differential diagnosis of SIADH presenting with abdominal pain of unknown etiology.

Hemin Prophylaxis for Patients with Frequently Recurring Acute Porphyric Attacks: Experience with Five Cases.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3545-3545
Author(s):  
Harry S. Griffith
Keyword(s):  
Aminolevulinic Acid ◽  
Hospital Setting ◽  
Acute Intermittent Porphyria ◽  
Motor Neuropathy ◽  
Acute Porphyria ◽  
Psychological Disturbances ◽  
Disease Management Strategy ◽  
Acute Attacks ◽  
Acute Porphyrias ◽  
One Year

Abstract Background The acute porphyrias are a group of four clinically indistinguishable diseases characterized by sudden, acute episodes of neurovisceral symptoms that can be life-threatening. Attacks are precipitated by a genetic deficiency in one of the enzymes involved in heme biosynthesis, acting in concert with one or more endogenous or exogenous triggers. The resultant stimulation of hepatic heme results in overproduction of highly reactive heme intermediaries - porphyrins and porphyrin precursors - which accumulate in the liver, blood, and bone marrow. A small percentage of patients have attacks that recur every several weeks, even after all known triggers are eliminated. Although hemin prophylaxis has been recommended for such patients (Anderson K, et al. Recommendations for the Diagnosis and Treatment of the Acute Porphyrias. Annals of Internal Medicine. 2005 Mar; 142(6):439–50), no adequate and well-controlled clinical studies have been conducted. Methods We report on five patients, all diagnosed with acute porphyria on the basis of elevated levels of urinary aminolevulinic acid and porphobilinogen (4 with acute intermittent porphyria, 1 with hereditary coproporphyria). All had a history of recurring acute porphyric attacks and received hemin prophylaxis for at least one year in an out-patient setting and were previously treated for a minimum of one year in a hospital setting. The dosage and frequency of prophylactic hemin was based on each patient’s clinical picture, and ranged from a one-day course of 1–4mg/kg/day every 10 to 40 days to a four day-course of 1–4 mg/kg/day every 30 days. The outcome of prophylaxis hemin treatment was determined on the basis of frequency of severe attacks requiring hospitalization and both physician and patient assessment of the severity of symptoms. Results Five patients (3 female, 2 male), ranging in age from 25–61 years old suffered from frequently recurring acute porphyric attacks, with symptoms that included abdominal pain (5), psychological disturbances (2), peripheral motor neuropathy (2), and nausea (2). Two patients had a significant reduction in the frequency of their acute attacks and three reported a moderate decrease with prophylactic hemin treatment. All five patients experienced a marked improvement in the reduction of the severity of their attacks. Overall, the occurrence of severe acute attacks requiring hospitalization declined by 66%. Three of the five patients did not require hospitalization over the 12 month period of time. One patient experienced phlebitis during one course of hemin treatment. Conclusions Hemin prophylaxis can be an effective disease management strategy in acute porphyria patients with frequently recurring acute attacks.

scholarly journals Feigning Acute Intermittent Porphyria

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Rania Elkhatib ◽  
Modupe Idowu ◽  
Gregory S. Brown ◽  
Yasmeen M. Jaber ◽  
Matthew B. Reid ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
English Language ◽  
Autosomal Dominant ◽  
Psychiatric Symptoms ◽  
Genetic Defect ◽  
Acute Intermittent Porphyria ◽  
Acute Attack ◽  
Neurological Deficits ◽  
Life Threatening ◽  
Language Literature

Acute intermittent porphyria (AIP) is an autosomal dominant genetic defect in heme synthesis. Patients with this illness can have episodic life-threatening attacks characterized by abdominal pain, neurological deficits, and psychiatric symptoms. Feigning this illness has not been reported in the English language literature to date. Here, we report on a patient who presented to the hospital with an acute attack of porphyria requesting opiates. Diligent assessment of extensive prior treatment records revealed thirteen negative tests for AIP.

Presentation of Gall Stone Ileus as Gut Obstruction

JMS SKIMS ◽  
2019 ◽  
Vol 21 (2) ◽  
pp. 117-119
Author(s):  
Munir Ahmad Wani ◽  
Mubarak Ahmad Shan ◽  
Syed Muzamil Andrabi ◽  
Ajaz Ahmad Malik
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Ct Scan ◽  
Bowel Obstruction ◽  
Emergency Surgery ◽  
Gallstone Ileus ◽  
Gall Stone ◽  
Life Threatening ◽  
Life Threatening Complication ◽  
Gall Stone Ileus

Gallstone ileus is an uncommon and often life-threatening complication of cholelithiasis. In this case report, we discuss a difficult diagnostic case of gallstone ileus presenting as small gut obstruction with ischemia. A 56-year-old female presented with abdominal pain and vomiting. A CT scan was performed and showed an evolving bowel obstruction with features of gut ischemia with pneumobilia although no frank hyper density suggestive of a gallstone was noted. The patient underwent emergency surgery and a 60 mm obstructing calculus was removed from the patient's jejunum, with a formal tube cholecystostomy. JMS 2018: 21 (2):117-119

scholarly journals Biochemical and hematological analysis in acute intermittent porphyria (AIP): a case report

2013 ◽  
Vol 85 (3) ◽  
pp. 1207-1214 ◽  
Author(s):  
ANNA R.R. DOS SANTOS ◽  
RAFAELA R. DE ALBUQUERQUE ◽  
MARIA J.R. DORIQUI ◽  
GRACIOMAR C. COSTA ◽  
ANA PAULA S.A. DOS SANTOS
Keyword(s):  
Biochemical Parameters ◽  
Contraceptive Use ◽  
Aminolevulinic Acid ◽  
Neuropsychiatric Symptoms ◽  
Acute Intermittent Porphyria ◽  
Signs And Symptoms ◽  
Acute Porphyria ◽  
Reactive Protein ◽  
Hematological Analysis ◽  
Hematological And Biochemical Parameters

Acute intermittent porphyria is the most common acute porphyria caused by a decrease in hepatic porphobilinogen deaminase activity, resulting in an accumulation of delta-aminolevulinic acid and porphobilinogen. This disease shows nonspecific signs and symptoms that can be confused with other diseases, thereby making the diagnosis difficult. We report a case of acute intermittent porphyria, reviewing clinical and laboratory aspects, highlighting the hematological and biochemical parameters during and after the crisis. A female patient, aged 28 years, suffered two crises, both presenting gastrointestinal disorders. The second presented neuropsychiatric symptoms. The analysis of hematological and biochemical parameters during the second crisis showed anemia, leukocytosis, hyponatremia, mild hypokalemia, uremia and elevated C-reactive protein. The initial treatment included glucose infusion, a diet rich in carbohydrates and interruption of porphyrinogenic drugs. Subsequently, treatment was maintained with oral contraceptive use. According to the observed data, signs and symptoms of gastrointestinal, neurological and psychiatric disorders, associated with laboratory results presented in this paper can be applied to screen acute porphyria, contributing to early diagnosis.

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