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Liver Diseases
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
The relative amount of conjugated and unconjugated bilirubin can be used for the differential diagnosis of various liver diseases. Early investigations described a test involving the coupling of bilirubin with diazotized sulfanilic acid in the presence of alcohol. In the serum of patients with hemolytic jaundice, this reaction gave a red color. Van der Bergh found that alcohol is not necessary in estimating bilirubin in the serum of patients with obstructive jaundice. These two types of reactions are known as indirect and direct bilirubin indicating that the former reaction required alcohol, but the latter did not. The difference between the two measurements represents the relative amounts of bilirubin conjugates (direct) and nonconjugated bilirubin (indirect) present in the serum of jaundiced patients.
Preoperative assessment
Published in Prem Puri, Newborn Surgery, 2017
John Gillick, Dawn Deacy, Prem Puri
Hyperbilirubinemia in the newborn may have a pathological basis such as severe sepsis, Rh and ABO incompatibilities, and congenital hemolytic anemias. Neonatal hemolytic jaundice usually appears during the first 24 hours of life, whereas physiological jaundice, as mentioned before, reaches a peak between 2 and 5 days of life. Other causes for prolonged hyperbilirubinemia, including those often associated with surgical conditions, are as follows: biliary obstruction, hepatocellular dysfunction, and upper intestinal tract obstruction. The diagnosis of extrahepatic biliary obstruction should be done as early as possible, because early operations for biliary atresia are essential to obtain good short-term as well as long-term results.45 The major concern in neonatal hyperbilirubinemia (high levels of unconjugated bilirubin) is the risk of kernicterus (bilirubin deposition in the brain), which can result in brain damage.
Management of poisoning
Published in Bev-Lorraine True, Robert H. Dreisbach, Dreisbach’s HANDBOOK of POISONING, 2001
Bev-Lorraine True, Robert H. Dreisbach
Laboratory findings in hemolytic jaundice due to poisons (castor beans, naphthalene, methylene blue, primiquine, sulfonamides): Bilirubin is present in the urine. Urinary and fecal urobilinogen are increased. Serum bilirubin is increased, indicating the inability of the liver to remove bilirubin as fast as it is formed. The degree of the accompanying anemia indicates the severity of the process.
Microwave ablation versus transcatheter arterial embolization for large hepatic hemangiomas: clinical outcomes
Published in International Journal of Hyperthermia, 2020
Yaoping Shi, Jingjing Song, Min Ding, Xiaoyin Tang, Zhi Wang, Jiachang Chi, Tao Wang, Jiansong Ji, Bo Zhai
The main disadvantage of MWA for the treatment of large hepatic hemangiomas is hemolysis attributable to the generous blood supply of hepatic hemangiomas. Hemolysis can lead to various degrees of hemoglobinuria, hemolytic jaundice, anemia or even renal damage accordingly, depending on its severity. It is worth noting that intraoperative quick infusion followed by intravenous use of 125 mL of sodium bicarbonate solution and 20 mg of furosemide was effective for preventing acute renal dysfunction. In our study, three early patients who did not receive preventive measures experienced acute renal dysfunction. However, none of the later patients suffered acute renal dysfunction. Furthermore, to prevent injury to the surrounding organisms, such as the diaphragm, gastrointestinal tract, gall bladder and biliary tract, it is not necessary to pursue complete ablation of a hemangioma. In this study, all nine patients with incomplete ablation had tumors located abutting either the diaphragmatic dome or hepatic hilum. However, none of them need further treatment because of the small size and slow growth of the residual tissue.
Advances in understanding disease mechanisms and potential treatments for Crigler–Najjar syndrome
Published in Expert Opinion on Orphan Drugs, 2018
Giulia Bortolussi, Andrés Fernando Muro
Crigler and Najjar were the first to characterize the syndrome in 1952, as severe congenital familiar non-hemolytic jaundice [22]. The CNS is a recessively inherited metabolic disorder of the liver with an estimated frequency of 1 in a million live births and is equally distributed in both genders [23,24]. It has been reported that CNS is relatively more frequent in specific populations such as the Amish and Mennonite communities, and in the sub-Mediterranean region (Tunis) [25,26]. Two distinct forms of the disease have been characterized according to the severity of the outcome: type I (CNS-I) and type II (CNS-II). Untreated CNS-I patients have total serum bilirubin levels exceeding 20 mg/dl (340 µM) and can increase up to 50 mg/dl (850 µM). This is the most severe form of the disease since patients completely lack UGT1A1 enzyme activity. Bile acid test in these patients showed that bile is almost completely composed by UCB with only traces of mono-glucuronide bilirubin [27]. If not promptly treated, these patients develop severe neurological damage and are at constant risk of kernicterus. Genetic mutations in CNS-I patients are well distributed along all the exons and are point missense or nonsense mutations, single base deletions, insertions or in few cases promoter polymorphisms (see [13] for a detailed description).
Recent advances in screening and diagnosis of hemoglobinopathy
Published in Expert Review of Hematology, 2020
Kanjaksha Ghosh, Kinjalka Ghosh, Reepa Agrawal, Anita H. Nadkarni
Clinical presentations like anemia, hemolytic jaundice, failure to thrive, splenomegaly, presence of similar conditions in other family members, gall stones, epigastric discomfort or heaviness due to splenomegaly, methemoglobinemia, sudden drop in Hb concentrations without any apparent cause, hemoglobinuria, reticulocytosis, polycythaemia are the presenting manifestations of hereditary hemoglobinopathies. Diagnosis of hemoglobinopathy should start following the abovementioned clinical presentations along with a family history of the disease.