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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sudden permanent blindness of cerebral origin, in addition to severe abdominal pain, hypertension, convulsions, and peripheral neuropathy developed in a 21-year-old woman, a victim of acute intermittent porphyria. Findings of the pathological examination of the brain showed extensive infarction in both occipital lobes. The pathological changes were consistent with anoxia. We discuss and review the literature of the possibility of "vasospasm" of both posterior cerebral arteries. Follow-up studies with serial EEG showed either focal epileptogenic activity or diffuse slow waves. The most consistent epileptic discharges were found in the occipital regions. The favorable response to the treatment of seizures with carbamazepine in this patient might encourage further clinical trials.
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PMID:Blindness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination. 19 74

Three patients with acute intermittent porphyria were noted to have retinal branch vessel occlusion. Branch "vein" occlusion, segmental optic atrophy, and soft exudate were the most common ocular manifestation. Two patients had labile elevated hypertension. When patients present with retinal branch vessel occlusion and a constellation of bizarre symptoms that might include hypertension, abdominal pain, acute psychotic behavior and/or cutaneous photosensitivity, the diagnosis of acute intermittent porphyria should be considered.
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PMID:Retinal branch vessel occlusion in acute intermittent porphyria. 55 59

A 36-year-old woman was admitted to hospital with a first attack of acute intermittent porphyria. At the same time increased serum levels of amylase and lipase as well as an increased amylase clearance to creatinine clearance ratio were observed, permitting the diagnosis of acute pancreatitis. The etiology of the latter could not be determined. In addition, elevation of indirect bilirubin without evidence of hemolysis was observed Gilbert's syndrome was suspected. 40 weeks after the first episode, a second attack of identical abdominal pain was noted, with elevation of pancreatic enzymes in the serum. There is evidence that acute intermittent porphyria and acute relapsing pancreatitis may have some etiological connection in this patient.
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PMID:Acute intermittent porphyria with relapsing acute pancreatitis and unconjugated hyperbilirubinemia without overt hemolysis. 95 Jan

In 17 patients (15 women, 2 men) with acute intermittent porphyria in the incidence of 23 clinical symptoms during 49 attacks was calculated. The most frequent symptoms in percentage of attacks were: Red colour of the urine 100%, abdominal pain 92%, tachycardia 88%, hypertension 75%, vomiting 54%, peripheral neuropathy 50%. In 35% of acute attacks a transient normochromic, normocytic anemia developed which is probably due to a disturbance of heme synthesis. Oliguria was found in 25%, azotemia in 12.5% of attacks. 4 patients with an average of 5 preceding acute attacks showed a persistent reduction of renal function during the symptom-free interval, in contrast to 12 patients with an average of 1.7 previous attacks and normal renal function. During the observation period from 1960-1974 3 (= 18%) of the 17 patients died.
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PMID:[Acute intermittent porphyria: report on 17 patients with 49 attacks (author's transl)]. 99 30

Clinical symptoms in 26 patients with acute intermittent porphyria (AIP) were correlated with light and electron microscopic findings. Abdominal pain, neuropsychiatric disorders and pathologic urine tests were predominant. Light and electron microscopic pictures of the central nervous system, the liver and the kidney were shown.
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PMID:Special clinical, light and electron microscopic aspects of acute intermittent porphyria. 100 93

We report the case of a 58-year-old woman affected by polyneuropathy, vegetative disturbances and abdominal pain. A provisional diagnosis of acute intermittent porphyria was made and was confirmed by the increased levels of urinary delta-aminolevulinic acid (ALA) and porphobilinogen (PBG).
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PMID:Acute intermittent porphyria. A perplexing case. 160 36

Among many metabolic disorders, porphyrias and Fabry disease are known to affect autonomic nervous system. In patients with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria, autonomic symptoms such as abdominal pain, vomiting, hypertension and tachycardia are among the most prominent clinical manifestations. Fabry disease is clinically characterized by severe limb pain, hypohidrosis, angiokeratomas and various autonomic symptoms. In both porphyrias and Fabry disease, pathological changes in the central and peripheral autonomic nervous system have been documented. In porphyrias, a loss of myelinated fibers, axonal degeneration, and segmental demyelination in peripheral autonomic nerves as well as chromatolysis of several brain stem nuclei have been found. In Fabry disease, abnormal amount of the substrates of alpha-galactosidase, i.e. ceramide di- and trihexoside, are found to be accumulated in the central and peripheral autonomic nerves.
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PMID:[Autonomic dysfunction in metabolic diseases]. 161 65

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

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 young woman with acute intermittent porphyria is described. She was admitted in a prolonged attack and had developed a flaccid quadriplegia. During the course she showed various manifestations of the autonomic nervous system, including pupils, gastrointestinal tract, cardiovascular system and others. On admission her pupils were equally mydriatic, and reacted to light sluggishly. Dilation of the pupils was seen when cocaine was instilled, but not when adrenalin. It was suggested that the parasympathetic control of pupils was disturbed. She complained repeatedly abdominal pain, nausea, vomiting, and constipation. However, diarrhea was rarely found. Radiological examinations revealed that her bowel movements were markedly impaired. Sinus tachycardia and elevation of blood pressure were frequently observed with attacks, and they correlated with the clinical course. With tachycardia the coefficient variance of R-R interval was markedly decreased, and large dose of atropine failed to accelerate the heart rate. These indicate that the vagal function was markedly impaired with attacks. The effects of isoproterenol and of propranolol on the heart rate were normal. Phenylephrine and phentolamine changed the blood pressure normally. From these it was concluded that the sympathetic nervous function was not so impaired at the time examined. However, with the elevation of blood pressure plasma and urinary noradrenaline were markedly increased. Other autonomic and related manifestations observed during the course included disorders of sweating, loss of sphincter control, fever of unknown cause and amenorrhea.
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PMID:[Autonomic dysfunctions in acute intermittent porphyria]. 258 92


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