Coexistence of Gilbert Syndrome and Hereditary Spherocytosis in a Child Presenting with Extreme Jaundice

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are se-lectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyper-bilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are se-lectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyper-bilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings

Serum Liver Enzyme Pattern in Birth Asphyxia Associated Liver Injury

Purpose: To study temporal pattern of serum liver enzymes levels in newborns with hepatic injury associated with birth asphyxia (BA). Methods: Singleton term newborns with BA and ≤72 hours of age admitted to neonatal intensive care unit were prospectively enrolled. Term newborns with physiological jaundice and without BA were studied as controls. Serum liver enzymes were measured at ＜24 hours, 24-72 hours, and at 6-12 days of age for cases and at 1-6 days of age for controls. BA was defined by 1 minute Apgar score ＜7 or delayed or absent cry with hypoxic ischemic encephalopathy. BA-associated liver injury was defined as serum alanine aminotransferase (ALT) elevation beyond +2 standard deviation (ALT > +2 SD) above the mean of control subjects at any of the three time points. Results: Sixty controls and 62 cases were enrolled. Thirty-five cases (56%) developed BA-associated liver injury (ALT＞81 IU/L). They had higher serum levels of ALT, aspartate aminotransferase, lactate dehydrogenase than the control infants, with peak at 24-72 hours. In controls, serum liver enzyme levels were significantly higher in appropriate- for-date (AFD) babies than small-for-date (SFD) babies. Serum enzyme pattern and extent of elevation were comparable between SFD and AFD babies. Degree of serum liver enzyme elevation had no relationship with severity of hypoxic encephalopathy. Conclusion: Serum liver enzyme elevation is common in BA; it peaks at 24-72 hours followed by a sharp decline by 6-12 days of age. Pattern and extent of enzyme elevation are comparable between SFD and AFD babies.Purpose: To study temporal pattern of serum liver enzymes levels in newborns with hepatic injury associated with birth asphyxia (BA). Methods: Singleton term newborns with BA and ≤72 hours of age admitted to neonatal intensive care unit were prospectively enrolled. Term newborns with physiological jaundice and without BA were studied as controls. Serum liver enzymes were measured at ＜24 hours, 24-72 hours, and at 6-12 days of age for cases and at 1-6 days of age for controls. BA was defined by 1 minute Apgar score ＜7 or delayed or absent cry with hypoxic ischemic encephalopathy. BA-associated liver injury was defined as serum alanine aminotransferase (ALT) elevation beyond +2 standard deviation (ALT > +2 SD) above the mean of control subjects at any of the three time points. Results: Sixty controls and 62 cases were enrolled. Thirty-five cases (56%) developed BA-associated liver injury (ALT＞81 IU/L). They had higher serum levels of ALT, aspartate aminotransferase, lactate dehydrogenase than the control infants, with peak at 24-72 hours. In controls, serum liver enzyme levels were significantly higher in appropriate- for-date (AFD) babies than small-for-date (SFD) babies. Serum enzyme pattern and extent of elevation were comparable between SFD and AFD babies. Degree of serum liver enzyme elevation had no relationship with severity of hypoxic encephalopathy. Conclusion: Serum liver enzyme elevation is common in BA; it peaks at 24-72 hours followed by a sharp decline by 6-12 days of age. Pattern and extent of enzyme elevation are comparable between SFD and AFD babies

Nutritional State of Children in the Democratic People’s Republic of Korea (DPRK): Based on the DPRK Final Report ofthe National Nutrition Survey 2012

A nationwide nutrition survey began when the Democratic People’s Republic of Korea (DPRK) requested assistance from international relief organizations in 1997 due to flooding in 1995-1996, followed by the worsening food shortage peak in 1997. According to reported data for the 15 years since the active intervention and assistance from interna-tional societies, the malnutrition and mortality rates of children in the DPRK have improved. However, the prevalence of the stunting reported in the latest 2012 report is 27.9%, which is still a moderate−severe level, and worrisome in terms of international standards. In particular, the prevalence in Ryangangdo, which is regarded as the worst region in the DPRK, is 39.6%, which is a very high level of stunting. To alleviate such regional deviation will be a major task for future assistance. In addition, one cannot emphasize too highly the importance of early nutritional assistance for pregnant women and infants, considering that the recovery from stunted growth is low after two years of age, and the aftereffects would continue for the rest of their lives.A nationwide nutrition survey began when the Democratic People’s Republic of Korea (DPRK) requested assistance from international relief organizations in 1997 due to flooding in 1995-1996, followed by the worsening food shortage peak in 1997. According to reported data for the 15 years since the active intervention and assistance from interna-tional societies, the malnutrition and mortality rates of children in the DPRK have improved. However, the prevalence of the stunting reported in the latest 2012 report is 27.9%, which is still a moderate−severe level, and worrisome in terms of international standards. In particular, the prevalence in Ryangangdo, which is regarded as the worst region in the DPRK, is 39.6%, which is a very high level of stunting. To alleviate such regional deviation will be a major task for future assistance. In addition, one cannot emphasize too highly the importance of early nutritional assistance for pregnant women and infants, considering that the recovery from stunted growth is low after two years of age, and the aftereffects would continue for the rest of their lives

Omega-3 Polyunsaturated Fatty Acid for Cholestasis due to BileDuct Paucity

Omega (ω)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nu-trition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (ω)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including amino-transferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effec-tiveness of ω-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.Omega (ω)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nu-trition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (ω)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including amino-transferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effec-tiveness of ω-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease