• 한국동물위생학회지
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• 제16권1호
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• pp.57-64
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• 1993

This survey was performed to report rare outbreak of liver cirrhosis in Korean native goat (KNG) which was died of Yangpyeong's goat farm on Feb. 1992. The examination for the KNG was carried out by clinical signs, necropsy and various lab-oratory test including parasitic, bacterial and histological test. The KNG looked jaundice, ascite, hemorrhage of lumen, abomasum and intestine, and brownish smooth cirrhotic liver at necropsy. Histological examination for liver revealed considerable proliferation of connective tissue and piecemeal necrosis which was caused by chronic active inflammation in interlobules and intralobules. There were atrophic micro and macro nodules which were sur-rounded by connective tissue. The lobular structure lack almost all central vein. The portal areas appearred proliferation of bile ducts, blood vessels and connective tissues. These connective tissue infiltrated heavily with plasma cells, Iymphocytes and histocytes. Histological examination for brain proved to be hepatic encephalopathy by virture of congestion and edema in cerebral medullary. From these results were demonstrated miked nodular, active, postnecrotic liver cirrhosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권3호
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• pp.162-169
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• 2014

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 ${\leq}72$ 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.

• 대한임상독성학회지
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• 제11권1호
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• pp.28-30
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• 2013

A 66-year-old male with chronic alcoholism presented with tremor, gait disturbance, memory impairment, insomnia, decreased appetite, and confusion. The patient had been taking lithium daily for treatment of bipolar disorder. Brain CT showed no specific abnormality, and serum lithium and ammonia levels were 3.63 mEq/L (therapeutic range, 0.6~1.2 mEq/L) and $85{\mu}g/dL$ (reference range: $19{\sim}54{\mu}g/dL$), respectively. Therefore, the initial differential diagnosis included chronic lithium intoxication, hepatic encephalopathy, Wernicke encephalopathy, or alcohol withdrawal syndrome. Even with the provision of adequate hydration, the patient's neurologic status did not show improvement, so that lactulose enema, thiamine replacement, and continuous venovenous hemodiafiltration (CVVHDF) were started on the third admission day. By the fifth admission day he had made a rapid neurologic recovery, and was discharged on the 20th admission day. Therefore, CVVHDF might be a treatment for patients with chronic lithium intoxication, because, even if serum lithium concentration is normal, lithium concentration in the brain may be different from that of the serum.

• Neonatal Medicine
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• 제15권2호
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• pp.176-182
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• 2008

드문 선천성 문맥전신성 단락에 대한 표준화된 치료는 없으나 저자들은 출생 직후부터 심부전 증상을 보인 환아에서 심실 중격 결손증을 동반한 선천성 문맥전신성단락을 4회의 코일 색전술 및 심 교정술로 치료한 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제34권2호
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• pp.265-269
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• 2017

Chylothorax or chylous ascites are rare manifestations of liver cirrhosis. We report a rare case of simultaneous chylothorax and chylous ascites in a patient with hepatitis B virus-related liver cirrhosis. A 76-year-old woman was referred to our hospital with a pleural effusion on her right side. She had no history of recent medical procedures, trauma or tumor. There was no evidence of mass or thoracic duct obstruction in a computed tomography scan. Pleural fluid and ascites were confirmed as chylothorax and chylous ascites by chemistry analysis. Despite thorough conservative care, there was no improvement. Pleurodesis was planned, but hepatic encephalopathy developed suddenly and she did not recover.

• Pediatric Infection and Vaccine
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• 제19권2호
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• pp.79-83
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• 2012

라이 증후군은 간부전을 동반하며 급속히 진행하는 뇌증으로, 인플루엔자와 수두 바이러스 등에 감염된 어린이에게 발병한다. 아스피린이 주된 원인 인자로 알려진 이후 소아에서 아스피린의 사용을 줄이면서 현재는 매우 드물게 보고 되고 있다. 저자들은 가와사키병 치료를 위해 정맥내 면역글로불린을 투여받고 아스피린을 복용하던 중 보챔, 강직, 활동량 저하, 식욕 저하, 처짐, 구토, 간수치 상승, 혈액 응고 장애와 고암모니아혈증으로 치료받은 5개월 여아를 보고하는 바이다.

• 한국환경보건학회지
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• 제19권1호
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• pp.66-70
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• 1993

저 및 고단백식이로 성장한 흰쥐에 methanethiol 투여시 체중당 간무게 및 혈청 alanine aminotransferase활성의 증가율은 고단백식이군(HP군)보다 저단백식이군(LP군)이 높았으며, 이 때 혈청 보다 xanthine oxidase 활성도는 HP군보다 LP군이 높게 나타났다. 또한 혈청 중 요산함량 역시 HP군보다 LP군에서 높게 나타났으며, 간조직의 uricase 활성은 LP군이 HP군보다 높게 나타났다. 따라서 LP군이 HP군보다, methanethiol 투여로 인한 혈청 xanthine oxidase 활성이 높게 나타남은 식이중 단백함량을 낮출 때 methanethiol 에 의한 간독성이 심하게 나타나기 때문이며, 또한 단백영양 부족시 methanethiol 의 폭로가 고뇨산혈증을 유도함을 시사해 주고 있다.

• Toxicological Research
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• 제33권4호
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• pp.299-304
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• 2017

Thallium and its compounds are a class of highly toxic chemicals that cause wide-ranging symptoms such as gastrointestinal disturbances; polyneuritis; encephalopathy; tachycardia; skin eruptions; hepatic, renal, cardiac, and neurological toxicities; and have mutagenic and genotoxic effects. The present research aimed to evaluate the efficacy of the chelating agents deferasirox (DFX) and deferiprone (L1) in reducing serum and tissue thallium levels after the administration of thallium (III), according to two different dosing regimens, to several groups of Wistar rats for 60 days. It was hypothesized that the two chelators might be more efficient as a combined therapy than as monotherapies in removing thallium (III) from the rats' organs. The chelators were administered orally as either single or combined therapies for a period of 14 days. Serum and tissue thallium (III) and iron concentrations were determined by flame atomic absorption spectroscopy. Serum and tissue thallium (III) levels were significantly reduced by combined therapy with DFX and L1. Additionally, iron concentrations returned to normal levels and symptoms of toxicity decreased.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권1호
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• pp.80-85
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• 2019

Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

• 대한유전성대사질환학회지
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• 제16권1호
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• pp.34-41
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• 2016

성인기에 나타난 운동으로 인한 스트레스와 과량의 단백질 투여에 의한 의식 변화, 기억 장애, 행동 장애등의 고암모니아혈증성 뇌병증 소견을 토대로 저자들은 성인형 제 2형 시트룰린혈증을 의심하였다. 검사 결과에서 고암모니아혈증, 혈중 시트룰린 상승, 요중 오로트산 경도 상승을 보였으며, 이를 통해 성인형 제 2형 시트룰린혈증을 진단하였다. SLC25A13 유전자 분석 결과를 통해 환자에게서 복합 이형 접합성 돌연변이(IVS11+1G>A, c.674C>A)를 확인하였다. 환자의 가족에서도 유전자 분석 검사를 진행하였고, 아버지, 어머니, 남동생에게서 성인형 제 2형 시트룰린혈증 보인자를, 언니에게서 성인형 제 2형 시트룰린혈증 환자임을 확인하였다. 그 동안 보고된 성인형 제 2형 시트룰린혈증의 임상 경과를 고려했을 때, 증상이 없던 언니에게서 질환을 발견하고 보존적 치료를 선제적으로 시작함으로써, 신경학적 장애 없이 일상 생활을 영위하고, 추가적인 뇌 손상을 방지하기 위한 간 이식 등 장기적인 치료 계획을 수립했다는 점에서 의의가 있다. 본 환자의 증례를 통해 고암모니아혈증성 뇌병증이 발생한 성인에서, 간질환 및 뇌질환의 증거가 없으며 다른 뚜렷한 원인이 없는 경우에는, 성인형 제 2형 시트룰린혈증을 고려해야 한다는 점을 인지하였다. 빈도가 드문 질환이지만 간성 혼수로 흔히 오인되고 있으며, 조기 진단 및 적절한 치료가 이루어지지 않으면 비가역적인 신경학적 후유증이 발생할 수 있다. 또한 다른 요소 회로 대사 질환 및 간성 혼수와는 달리, 고탄수화물 식이가 질병의 경과를 인위적으로 악화시킬 수 있기에 적절한 대사 이상 검사 및 유전자 검사가 시행되어야 하겠다. 본 증례는 반복적인 고암모니아혈증성 뇌병증 소견을 보인 37세 여성과 뇌병증 소견이 없었던 언니에게서 성인형 제 2형 시트룰린혈증을 진단하고, 즉각적인 치료를 통해 심각한 신경학적 장애 없이 일상 생활을 지속하고 있는 환자들을 문헌 고찰과 함께 보고하는 바이다.