BACKGROUND:Acute intermittent porphyria (AIP) is the most common hepatic porphyria.Its clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric symptoms.These s...BACKGROUND:Acute intermittent porphyria (AIP) is the most common hepatic porphyria.Its clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric symptoms.These symptoms result from the overproduction and accumulation of porphyrin precursors,5-aminoleuvulinic acid (ALA) and porphobilinogen (PBG).The effect of medical treatment is transient and is not effective once irreversible neurological damage has occurred.Liver transplantation (LT) replaces hepatic enzymes and can restore normal excretion of ALA and PBG and prevent acute attacks.METHOD:Two cases of LT for AIP were identified retrospectively from a prospectively maintained LT database.RESULT:LT was successful with resolution of AIP in two patients who suffered from repeated acute attacks.CONCLUSION:LT can correct the underlying metabolic abnormality in AIP and improves quality of life significantly.展开更多
The Impact of the Indian Ocean Dipole(IOD)and the El Nino Southern Oscillation(ENSO)event for Indonesian rainfall has been investigated for the period from 1950 to 2011.Inter-annual change of IOD and ENSO indices are ...The Impact of the Indian Ocean Dipole(IOD)and the El Nino Southern Oscillation(ENSO)event for Indonesian rainfall has been investigated for the period from 1950 to 2011.Inter-annual change of IOD and ENSO indices are used to investigate their relationship with Indonesian rainfall.By using the wavelet transform method,we found a positive significant correlation between IOD and Indonesian rainfall on the time scale of nearly 2.5–4 years.Furthermore,the positive significant correlation between ENSO(sea surface temperature anomaly at Nino3.4 area indices)and Indonesian rainfall exists for shorter than 2 years and between 5.5 to 6.5-year time scales.展开更多
文摘BACKGROUND:Acute intermittent porphyria (AIP) is the most common hepatic porphyria.Its clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric symptoms.These symptoms result from the overproduction and accumulation of porphyrin precursors,5-aminoleuvulinic acid (ALA) and porphobilinogen (PBG).The effect of medical treatment is transient and is not effective once irreversible neurological damage has occurred.Liver transplantation (LT) replaces hepatic enzymes and can restore normal excretion of ALA and PBG and prevent acute attacks.METHOD:Two cases of LT for AIP were identified retrospectively from a prospectively maintained LT database.RESULT:LT was successful with resolution of AIP in two patients who suffered from repeated acute attacks.CONCLUSION:LT can correct the underlying metabolic abnormality in AIP and improves quality of life significantly.
基金Marine Computation Laboratory, Faculty of Marine Sciences and Fisheries-Udayana University, Bali which supported our research in Collaboration with Yamaguchi University, Japan
文摘The Impact of the Indian Ocean Dipole(IOD)and the El Nino Southern Oscillation(ENSO)event for Indonesian rainfall has been investigated for the period from 1950 to 2011.Inter-annual change of IOD and ENSO indices are used to investigate their relationship with Indonesian rainfall.By using the wavelet transform method,we found a positive significant correlation between IOD and Indonesian rainfall on the time scale of nearly 2.5–4 years.Furthermore,the positive significant correlation between ENSO(sea surface temperature anomaly at Nino3.4 area indices)and Indonesian rainfall exists for shorter than 2 years and between 5.5 to 6.5-year time scales.
