[Ingested medication blister packs]. / Ingeslikte blisterverpakkingen.

BACKGROUND: Single dose blister packs (BP) are commonly used in pharmaceutical packaging. Accidental ingestion of medication BPs can cause serious harm as the sharp edges can severely damage the esophageal wall. CASE DESCRIPTIONS: We describe 2 cases of accidental BP ingestion. An 88-year-old man self-administered his medication during hospital admission. Afterwards, he started to complain about dysphagia. Endoscopic examination the next day revealed a BP stuck in the esophageal wall, which was successfully removed. A 66-year-old man presented to the emergency department with acute onset hematemesis and dysphagia for one week. Upper endoscopy showed a deep tear in the esophageal mucosa and an intact BP in the stomach. The BP was removed and the patient recovered. CONCLUSION: Patients are often not aware of the ingested BP. Urgent endoscopic intervention is needed in order to prevent further damage to the esophageal wall. Supervision during specific moments of intake could help to prevent accidental ingestion.

Value of molecular analysis of Wilson's disease in the absence of tissue copper deposits: a novel ATP7B mutation in an adult patient.

Wilson's disease (WD) is a disorder of copper metabolism leading to copper accumulation in the liver and in extrahepatic organs, such as brain and cornea. We present a patient with liver disease who did not fulfil the biochemical criteria for WD. Mutational analysis was necessary to make the diagnosis and show a new mutation. Our case supports the use of mutation analysis in cases with unclear liver disease and suggests that the spectrum of WD is broader than currently assumed.

Heterozygous alpha-I antitrypsin deficiency as a co-factor in the development of chronic liver disease: a review.

Alpha-I antitrypsin (AIAT) is an acute-phase protein that is produced in liver cells. AIAT deficiency is a hereditary disease which is defined by the hepatic production of an abnormal protein that can not be released into the plasma. This leads to deficiency of plasma AIAT and subsequently to an impaired protection against proteases, resulting in pulmonary disease. Accumulation of the abnormal protein in hepatocytes can lead to liver damage. Serum level measurement, phenotyping and liver biopsy can be used for establishing the diagnosis. Homozygous AIAT deficiency can cause neonatal hepatitis; in adults end-stage liver disease, cirrhosis and hepatocellular carcinoma can develop. There are strong arguments to consider heterozygous AIAT deficiency as an important co-factor in the aetiology of chronic liver disease. Studies have shown that AIAT heterozygosity can be considered a modifier for hepatitis C virus, end-stage liver disease, cirrhosis and hepatocellular carcinoma. The accumulation of AIAT in the hepatocytes occurs more profoundly in a diseased liver, and as a consequence it affects the natural course of the liver disease. Therapeutic options include augmentation therapy (infusion of purified human plasma AIAT) in pulmonary disease; in end-stage liver disease liver transplantation is an option. For the future, other interventions such as gene therapy or strategies to inhibit polymerisation are promising.

[Acute pancreatitis in a hepatitis C positive patient following treatment with peginterferon alfa-2b and ribavirin]. / Acute pancreatitis bij een hepatitis C-positieve patient na behandeling met peginterferon alfa-2b en ribavirine.

A 59-year-old man, treated with peginterferon alfa-2b and ribavirin because of a chronic Hepatitis C virus infection presented in the emergency department with acute epigastric pain. An acute pancreatitis, probably toxic, was diagnosed. A literature search confirmed that acute pancreatitis may develop as a result of treatment with (peg)interferon whether or not in combination with ribavirin. It is important to be aware of pancreatitis when patients treated with these medications present with acute epigastric pain. Discontinuing the medication will lead to a rapid reduction of the symptoms and recovery.

[Heterozygosity for alpha1-antitrypsin deficiency as a cofactor in the development of chronic liver disease]. / Heterozygotie voor alpha1-antitrypsinedeficiëntie als cofactor bij de ontwikkeling van een chronische leveraandoening.

In 3 patients, 2 men aged 51 and 40 years and a 50-year-old woman, with liver-function disorders due to excessive consumption of alcohol, the liver function deteriorated rapidly resulting in the patients' death. All 3 were found to be heterozygous for alpha1-antitrypsin (AT) deficiency. Alpha1-AT deficiency can lead to cirrhosis of the liver and pulmonary emphysema. There are indications that heterozygous alpha1-AT deficiency can contribute to the development of a chronic liver disease, even when the serum level of alpha1-AT is within the normal range, especially in association with other risk factors such as alcohol abuse or chronic viral hepatitis. Persons with this mutation also have an increased risk for the development of cryptogenic cirrhosis and primary liver-cell carcinoma. Determination of the alpha1-AT phenotype should perhaps be recommended for all patients with a chronic liver disease, especially if the liver function deteriorates more rapidly than expected, even in the presence of a normal alpha1-AT serum level. A liver biopsy remains the gold standard for establishing the presence of alpha1-AT deposits in the liver.