Stress and the precipitation of acute intermittent porphyria.

نویسندگان

  • E D LUBY
  • J G WARE
  • R SENF
  • C E FROHMAN
چکیده

:UTE INTERMITTENT porphyria, following Watson's classification, is a subgroup of porphyria hepatica, which is probably inherited as a non-sex-linked Mendelian dominant. Porphyria hepatica can present in any of four ways: intermittent acute, cutania tarda, mixed or combined type, and latent. In the United States the most common form is the intermittent acute, with the prime mode of onset characterized by symptoms referable to the abdomen and central nervous system. Apparently the incidence varies greatly from one country to another. In the United States it has been estimated as 1 in 150.000. The ratio reported by Dean and Barnes,' in South Africa, is much higher at 1 in 100. Acute intermittent porphyria has been mistaken for so many diverse syndromes that, in the words of Watson, "it well deserves the sobriquet of 'little simulator'." Psychiatric symptoms are an integral part of the clinical picture of the disease. Hysteroid behavior, conversion reactions, acting out, and schizophrenic-like phenomena have been described as occurring in porphyric patients.' 9 Abdominal pain is the most frequent presenting complaint, but paralysis of extremity musculature asso

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Acute Intermittent Porphyria: A Reversible Cause of Cachexia

Acute Intermittent Porphyria (AIP), the most common of acute porphyrias, is due to deficient activity of the enzyme porphobilinogen deaminase. Clinically presents with acute attacks, which comprise a variety of neuropsychiatric and visceral symptoms. The attacks may be precipitated by drugs, alcohol, smoking, reduced caloric intake, infection, surgery, psychological stress, or hormonal changes....

متن کامل

Acute intermittent porphyria: case report and review of the literature.

Acute intermittent porphyria is an unusual pathology with potentially severe consequences when not early detected. Among the possible causes of porphyric crises decrease of caloric intake has been described. A case of acute intermittent porphyria in the late postoperative period of a bariatric surgery performed for treatment of obesity is reported. A review of the diagnostic aspects and managem...

متن کامل

De Novo mutation found in the porphobilinogen deaminase gene in Slovak acute intermittent porphyria patient: molecular biochemical study.

The porphyrias are group of mostly inherited disorders in which a specific spectrum of accumulated and excreted porphyrins and heme precursors are associated with characteristic clinical features. There are eight enzymes involved in the heme synthesis and defects in seven of them cause porphyria. Four of them are described as acute hepatic porphyrias, which share possible precipitation of acute...

متن کامل

Acute Intermittent Porphyria: A Diagnostic Challenge

Acute intermittent porphyria is a metabolic disorder rarely seen in prepubertal children. A delay in diagnosis of acute intermittent porphyria is common because of variable and nonspecific symptoms. We report an 8-year-old boy with right hemimegalencephaly and intractable seizures, who presented with dark-colored urine, hypertension, increasing lethargy, fluctuating seizures, and poor oral inta...

متن کامل

Acute intermittent porphyria with transient cortical blindness.

Acute intermittent porphyria is a hereditary disorder characterized by deficient activity of the enzyme porphobilinogen deaminase. It manifests with occasional neurovisceral crises due to overproduction of porphyrin precursors. We report a 12 year old male child with acute intermittent porphyria, who presented with encephalopathy and transient blindness of cerebral origin.

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Psychosomatic medicine

دوره 21 1  شماره 

صفحات  -

تاریخ انتشار 1959