Currarino syndrome  is an inherited congenital disorder where either the sacrum (the fused vertebrae forming the back of the pelvis) is not formed properly, or there is a mass in the presacral space in front of the sacrum, and there are malformations of the anus or rectum. It occurs in approximately 1 in 100,000 people.Anterior sacral meningocele is the most common presacral mass in patients with Currarino syndrome, occurring in 60% of cases. Its presence may significantly affect the surgical management of these patients. Other potential presacral masses include presacral teratoma and enteric cyst. Presacral teratoma usually is considered to be a variant of sacrococcygeal teratoma. However, the presacral teratoma that is characteristic of the Currarino syndrome may be a distinct kind. Genetics
The disorder is an autosomal dominant genetic trait caused by a mutation in the HLXB9 homeobox gene. In 2000 the first large series of  Currarino cases was genetically screened for HLXB9 mutations, and it was shown that the gene is specifically causative for the syndrome, but not for other forms of sacral agenesis. The study was published in the American Journal of Human Genetics. Diagnosis
Diagnosis of Currarino syndrome is usually clinical, detecting all three elements of the triad. However, genetic testing is often used as the confirmation of diagnosis and genetic analysis of patients family members. Treatment
Surgery of an anterior myelomeningocele is  only indicated in the rare case in which the space-occupying aspect is expected to cause constipation or problems during pregnancy or delivery.
Zieves syndrome is an acute metabolic condition that can occur during withdrawal from prolonged heavy alcohol use. It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinaemia (excessive blood lipoprotein), jaundice (elevation of unconjugated bilirubin), and abdominal pain. The underlying cause is liver delipidization. This is distinct from alcoholic hepatitis which, however, may present simultaneously or develop later.Diagnosis of Zieves syndrome should be considered in patients with prolonged alcohol use (especially after an episode of binge drinking) with an elevation of unconjugated bilirubin and without obvious signs of gastrointestinal bleeding. Pathogenesis
The proposed mechanism of the characteristic haemolytic anaemia in Zieves syndrome is due to alteration of the red cell metabolism, namely pyruvate kinase instability leaving them susceptible to circulating hemolysin such as lysolecithin. Changes in membrane lipid compositions such as increased cholesterol and polyunsaturated fatty acid (PUFA) have been reported during the hemolytic phase. Diagnosis
The diagnosis is demonstrated by the triad of alcoholic hepatitis or cirrhosis, hemolytic anemia, and hyperlipidemia. Treatment
Definitive treatment for Zieves syndrome is alcohol cessation. Individuals with markedly elevated triglycerides, particularly with a history of pancreatitis or intracerebral hemorrhage, may require plasmapharesis to avoid complications associated with hypertriglyceridemia. History
Zieves syndrome was initially described in 1958. Dr. Leslie Zieve described patients with a combination of alcoholic liver disease, hemolytic anemia and hypertriglyceridemia. References


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