Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

1. A therapeutic trial of intravenous hematin is presented. Eleven cases of AIP and one of VP who did not improve with conventional treatment (high carbohydrate intake) received this new agent. 2. Urinary ALA, PBG and, when possible, uroporphyrin and coproporphyrin were used to monitor the chemical response to the treatment. Objective clinical parameters of hypertension and tachycardia were followed when present in addition to subjective estimates of acute porphyric symptomatology (abdominal pain, backache, extremity pain and paresthesias, weakness, depression, etc.). 3. At a dosage of approximately 3 mg/kg, diminution of urinary ALA and PBG excretion was achieved in every patients. Hypertension and tachycardia improved in those instances where they were observed in association with the attack. Also, subjective improvements in the clinical status of the patients were observed frequently. 4. Hematin appears to be a promising therapeutic agent for the treatment of acute attack forms of porphyria.
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PMID:Hematin therapy for acute porphyria. 44 61

A review is presented of the more recent biochemical findings in the hepatic porphyrias. The clinical aspects of acute porphyria are re-emphasized and illustrated with a case history. In particular the differentiation between porphyria and surgical causes of abdominal pain is stressed. The anaesthetic management is discussed and details of diagnostic and screening tests are given.
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PMID:Porphyria in relation to surgery and anaesthesia. 111 85

An elevation of serum amylase and lipase has not been reported previously to occur with porphyria. In this report, we describe a patient who presented with the clinical and laboratory picture of pancreatitis: elevated amylase, lipase, amylase-creatinine clearance ratio, and with abdominal pain. Only after extensive evaluation, was the patient found to have porphyria. On two separate occasions, with hematin therapy, her serum amylase decreased, as did her clinical symptoms of porphyria and her urinary quantitative porphyrins. This suggests an association between elevation of the serum amylase and lipase with acute porphyria. Moreover, this association can lead to delay in establishing the diagnosis of acute porphyria.
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PMID:Acute porphyria presenting with hyperamylasemia. 172 Oct 20

A fatal case of variegate (mixed) porphyria in an 11-year-old Nigerian girl is reported. She presented with severe abdominal pain, vomiting, constipation, quadriplegia and cutaneous bullous dermatosis. Remission was temporarily achieved with chlorpromazine, high carbohydrate diet and physiotherapy. She finally died of respiratory paralysis 17 months after diagnosis.
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PMID:Variegate (mixed) porphyria in a Nigerian girl. 171 2

In a retrospective study covering 411 acute intermittent porphyria patients, four cases of a coincidence with Crohn's disease or ulcerative colitis were found. Their courses of disease confirmed that patients with chronic inflammatory bowel disease have a higher risk for acute porphyria manifestation. Both malnutrition (glycopenic induction) and sulphasalazine (drug-induced exacerbation) are known as triggering factors for acute porphyric states. Furthermore, diagnosis of acute intermittent porphyria tends to be much more difficult in such cases, as the acute phases of abdominal pain are likely to be associated with the enteral disease process. A delay of diagnosis and therapy of acute hepatic porphyria, however, may endanger the patient by pareses, which could be irreversible or even lethal. Therefore, whenever there is suspicion of a coinciding acute porphyria, urinary screening tests for porphyria should immediately be performed and, if a coinciding acute hepatic porphyria is diagnosed, porphyrogenic drugs like sulphasalazine should be avoided in treatment of chronic inflammatory bowel disease.
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PMID:Manifestation of acute intermittent porphyria in patients with chronic inflammatory bowel disease. 177 36

A 36-year-old woman had for two months experienced progressively more marked diffuse abdominal pain, at times colicky, as well as nausea, vomiting and severe constipation. In addition, paraesthesias and motor weakness developed in the thighs. This was accompanied by a normochromic, normocytic anaemia with a haemoglobin concentration of 9.6 g/l. A short time later her mother and daughter also fell ill with similar symptoms. After symptomatic treatment had failed, secondary coproporphyria due to lead poisoning was found. The poisoning had resulted from criminal contamination of food, especially of cocoa powder, with lead acetate. Raised lead concentrations in serum were found in two other members of the family. In all the patients treatment was undertaken with sodium calcium edetate (20 mg/kg body-weight) in several three-day cycles, achieving a gradual fall in serum lead concentration. When the level had fallen to below 4 mumol/l the symptoms disappeared. Below 3 mumol/l porphyria was no longer demonstrable and the anaemia regressed. It is pointed out that, as lead poisoning may be fatal, it should be considered in the differential diagnosis of acute abdominal colic of unclear cause.
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PMID:[Acute lead poisoning]. 189 43

We report a 16-year-old girl with acute intermittent porphyria who had abdominal pain, generalized tonic-clonic and simple partial seizures, and inappropriate antidiuretic hormone secretion. Because most antiepileptic drugs are contraindicated in porphyria, she was treated with magnesium sulfate i.v. Soon after starting treatment, seizures stopped, returned, and then again responded in several trials with discontinuation and reinstitution of i.v. magnesium sulfate. Our experience encourages the use of magnesium sulfate for treatment of seizures in patients with porphyria.
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PMID:Treatment of porphyric convulsions with magnesium sulfate. 191 81

A 15-year-old girl of Turkish descent had for one year complained of severe recurrent fever-associated deep back pains. Since she was three years of age she had suffered from repeated attacks of fever and severe abdominal pain which ceased spontaneously in 1-3 days. On physical examination the sacrum and iliosacral joints were very painful to percussion, and she limped. Radiography revealed symmetric destructive sacroiliitis. Despite the unusual location of the arthritis, the triad of fever, abdominal pain and arthritis, as well as her belonging to an ethnic "at risk" group, pointed to the diagnosis of familial mediterranean fever (FML) or recurrent hereditary polyserositis. This diagnosis was confirmed by a positive metaraminol provocation test in that infusion of metaraminol reproduced the typical pains. Collagen diseases, rheumatic disease, acute porphyria and chronic infectious processes were excluded. The sacroiliitis quickly responded to long-term administration of colchicine, 0.5 mg twice daily. The patient also has Hageman factor deficiency whose significance remains unclear.
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PMID:[Sacroiliitis in familial Mediterranean fever]. 193 71

A 49-year-old man with a 1-month history of episodic, severe abdominal pain sought medical attention. The patient's history was remarkable for type II diabetes, for which glipizide therapy had been initiated 2 months earlier. No other medications were being taken at the time the paroxysms of pain began. During the episodes of pain, both examination of the abdomen and abdominal roentgenograms revealed normal findings. Initial assessment, including ultrasonography and computed tomographic scanning of the abdomen, upper gastrointestinal and colon roentgenograms, and esophagogastroduodenoscopy, revealed no cause of the pain. Empiric trials of famotidine, sucralfate, and antacids failed to relieve the pain. Both urine and fecal specimens collected after an attack demonstrated substantially increased coproporphyrins. The glipizide regimen was discontinued; 2 months later, the stool coproporphyrins had decreased to normal levels. At follow-up more than 1 year later, the patient had had no recurrence of abdominal pain. Although other orally administered hypoglycemic agents and other sulfa compounds have been reported to precipitate acute attacks of porphyria, to our knowledge this is the first such case associated with glipizide. We suggest that glipizide be added to the list of medications to be avoided in patients with porphyria.
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PMID:A coproporphyria-like syndrome induced by glipizide. 200 89

Patients with acute hepatic porphyria present with abdominal pain and neurologic abnormalities. Although the disease is uncommon, the emergency physician will occasionally encounter a patient with porphyria. The relevant pathophysiology of acute hepatic porphyria and the treatment of the patient with acute hepatic porphyria are reviewed.
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PMID:Acute porphyria in the emergency department. 266 71


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