Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

Dai Jung,서고훈,김윤명,최진호,유한욱 대한소아내분비학회 2018 Annals of Pediatirc Endocrinology & Metabolism Vol.23 No.1

Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.

Hepatopulmonary Syndrome in Poorly Compensated Postnecrotic Liver Cirrhosis by Hepatitis B Virus in Korea

(Jae Ho Lee),(Dong Ho Lee),(Joo Hee Zo),(Tae Ho Kim),(Kook Lae Lee),(Hee Soon Chung),(Cheol Ho Kim),(Sung Ku Han),(Young Soo Sim),(Hyo Suk Lee),(Yong Bum Yoon),(In Sung Song),(Chung Yong Kim) 대한내과학회 2001 The Korean Journal of Internal Medicine Vol.16 No.2

N/A Background : Hepatopulmonary syndrome (HPS) refers to the association of hypoxemia, intrapulmonary shunting and chronic liver disease. But there is no clear data about the prevalence of HPS in postnecrotic liver cirrhosis by hepatitis B virus(HBV), the most common cause of liver disease in Korea. The aim of this study was to investigate the prevalence of HPS in poorly compensated postnecrotic liver cirrhosis by HBV, and the correlation of the hepatopulmonary syndrome with clinical aspects of postnecrotic liver cirrhosis by HBV. Methods : Thirty-five patients underwent pulmonary function test, arterial blood gas analysis and contrast-enhanced echocadiography. All patients were diagnosed as HBV-induced Child class C liver cirrhosis and had no evidence of intrinsic cardiopulmonary disease. Results : Intrapulmonary shunt was detected in 6/35 (17.1%) by contrast- enhanced echocariography. Two of six patients with intrahepatic shunts had significant hypoxemia (PaO2 < 70 mmHg) and four showed increased alveolar- arterial oxygen gradient over 20 mmHg. Only cyanosis could reliably distinguish between shunt positive and negative patients. Conclusions : The prevalence of intrapulmonary shunt in poorly compensated postnecrotic liver cirrhosis by HBV was 17.1% and the frequency of hepatopulmonary syndrome was relatively low (5.7%). ‘Subclinical’ hepatopulmonary syndrome (echocardiographically postive intrapulmonary shunt but without profound hypoxemia) exists in 11.4% of cases with poorly compensated postnecrotic liver cirrhosis by HBV. Cyanosis is the only reliable clinical indicator of HPS of HBV- induced poorly compensated liver cirrhosis. Further studies are required to determine if the prevalence and clinical manifestations of HPS varies with etiology or with geographical and racial differences.

The effects of flavanoid on the treatment of hepatopulmonary syndrome

Talha Atalay,Murat Cakir,Ahmet Tekin,Tevfik Kucukkartallar,Suleyman Kargin,Adil Kartal,Adnan Kaynak 대한외과학회 2013 Annals of Surgical Treatment and Research(ASRT) Vol.85 No.5

Purpose: Hepatopulmonary syndrome is an arterial oxygenation disorder brought about by advanced liver failure and pulmonary vascular dilatations. The reason why hypoxia develops in hepatopulmonary syndrome depends on the broadening of perialveolar capillary veins. Our study aims to investigate the effects of Flavanoid on hepatopulmonary syndrome through its inhibition of nitric oxide. Methods: Three groups, each having 8 rats, were formed within the scope of our study. Group I (the control group) only received laparatomy, group II received choledoch ligation, and group III was administered Flavanoid (90% flavonoid diosmin, 10% flavonoid hesperidin) following choledoch ligation. The rats were administered Flavanoid at week two following choledoch ligation. The rats’ livers and lungs were examined histopathologically following a five-week follow-up and the perialveolar vein diameters were measured. Arterial blood gases and biochemical parameters were evaluated. Results: It was seen that fibrosis and oxidative damage in the liver with obstructive jaundice as well as hypoxia with pulmonary perialveolar vein sizes were significantly lower than the other group with cirrhosis formed through the administration of Flavanoid. Conclusion: We have concluded that Flavanoid administration might be useful in the treatment of hypoxia in hepatopulmonary syndrome and the delay of cirrhosis contraction.

곤봉지를 동반한 간폐증후군 1예

김호경 ( Ho Kyung Kim ),김원기 ( Won Ki Kim ),김주한 ( Joo Han Kim ),이정우 ( Jeong Woo Lee ),정욱진 ( Wook Jin Chung ),권오상 ( Oh Sang Kwon ),김주현 ( Ju Hyun Kim ) 대한내과학회 2021 대한내과학회지 Vol.96 No.3

간폐증후군은 폐내 혈관 확장으로 발생하는 간경변의 합병증 중의 하나로 동맥혈 산소 포화도 저하를 유발한다. 호흡곤란이 환자가 호소하는 가장 흔한 증상이며 입위 시 악화소견을 보인다. 신체 검사에서 곤봉지와 청색증이 발견되기도 하며 유일하게 간이식이 현재까지는 가장 효과적인 치료법으로 알려져 있다. 본 증례에서는 호흡곤란을 호소하는 간경변 환자에서 곤봉지를 동반한 간폐증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다. Hepatopulmonary syndrome is a rare lung complication of liver cirrhosis, caused by pulmonary microvascular vasodilation that induces abnormal arterial oxygenation. Typical findings on physical examination are finger clubbing and cyanosis. Dyspnea is a common symptom and is worse in the upright position. Contrast echocardiography is a useful diagnostic test. Currently, the only effective treatment is liver transplantation. We report the case of a woman with cirrhosis who has hepatopulmonary syndrome with finger clubbing, confirmed by contrast echocardiography. The patient is waiting for a liver transplant. (Korean J Med 2021;96:236-240)

Case Report : Improved severe hepatopulmonary syndrome after Liver transplantation in an adolescent with end-stage Liver disease secondary to biliary atresia

( Tae Jun Park ),( Keun Soo Ahn ),( Yong Hoon Kim ),( Hyungseop Kim ),( Ui Jun Park ),( Hyoung Tae Kim ),( Won Hyun Cho1 ),( Woo Hyun Park ),( Koo Jeong Kang ) 대한간학회 2014 Clinical and Molecular Hepatology(대한간학회지) Vol.20 No.1

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period. (Clin Mol Hepatol 2014;20:76-80)

Aggravation of Hepatopulmonary Syndrome after Sildenafil Treatment in a Patient with Coexisting Portopulmonary Hypertension

정승민,이경호,장성아,김덕경 대한심장학회 2015 Korean Circulation Journal Vol.45 No.1

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. Their pathophysiological mechanisms clearly differ. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vasculardilatation. In contrast, PPHTN is predominantly due to excessive pulmonary vasoconstriction and vascular remodeling, but is rarely associatedwith hypoxia. We report a case of a patient who had both HPS and PPHTN at the time of presentation. HPS was aggravated after sildenafiladministration for the treatment of PPHTN. We demonstrated increased amount of intrapulmonay shunt after sildenafil challengeby using agitated saline contrast transthoracic echocardiography.

Hepatopulmonary Syndrome Is Related to the Development of Acute-on-Chronic Liver Failure and Poor Prognosis in Cirrhotic Patients

( Han Seul Ki ),( Moon Young Kim ),( Seong Hee Kang ),( Ki Tae Suk ),( Soon Koo Baik ) 대한간학회 2021 춘·추계 학술대회 (KASL) Vol.2021 No.1

HBV 에 의한 후괴사성 간경화환자들에서 조영 심초음파도를 이용한 폐내 단락에 관한 연구

이재호(Jae Ho Lee),이경미(Kyung Mi Lee),조주희(Joo Hee Zo),이동호(Dong Ho Lee),정경해(Kyung Hae Jung),김원배(Won Bae Kim),이국래(Kook Lae Lee),김철호(Cheol Ho Kim),정희순(Hee Soon Chung) 대한내과학회 1998 대한내과학회지 Vol.54 No.6

N/A Background: Liver cirrhosis is a common disease and mainly postnecrotic by the infection of hepatitis B virus (HBV) in Korea, and arterial hypoxemia is not a rare finding in liver cirrhosis. But there were few studies about hepatopulmonary syndrome which is defined as liver dysfunction, hypoxemia and intrapulmonary vascular dilatation, and one of the causes of arterial hypoxemia. Method: We examined the presence of dyspnea and stigmata of chronic liver disease in 35 patients who were diagnosed as Child class C, liver cirrhosis by HBV and had no evidence of intrinsic cardiopulmonary disease at Seoul Municipal Boramae Hospital (from January 1996 to October 1996). To diagnose hepatopulmonary syndrome, contrast-enhanced echocardiography was done for detecting intrapulmonary shunt in association with pulmonary function test, arterial blood gas analysis, chest roentgenogram and EKG. Result: 1) Twenty nine patients (82.9%) complained of exertional dyspnea, and the six from these patients also complained of resting dyspnea. Tachypnea was observed in 15 patients (42.9%/normal respiration rate defined as 12 to 16 per min), while platypnea was not found. 2) Splenomegaly and ascites were universal findings in all while clubbing of finger and cyanosis were observed in only 1 case (2.9%) and 2 cases (5.7%), respectively, and esophageal varix was found in the thirty two (91.4% / grade I in 9, grade II in 20 and grade III in 3 patients). Spider angioma was observed in the twenty one (70.0% among 30 patients). 3) Seven patients (20.0%) showed mild ventilatory defect of restrictive type since forced vital capacity decreased to 65-79% of predicted value. Diffusing capacity for carbon monoxide decreased below 70% of predicted value in 4 patients (11.4%). In addition, there were small amount of pleural effusion in 5 patients (14.3%) and nonspecific arrhythmia in 3 patients (8.6%). 4) Six cases (17.1%) had intrapulmonary shunt, which showed significantly lower arterial oxygen tension (72.2±15.1 vs 90.2±7.4 mmHg, p<0.05) and increased alveolar- arterial oxygen difference (43.1±16.2 vs 22.4±7.4 mmHg, p<0.05). 5) Only the cyanosis had a significant correlation with intrapulmonary shunt. Conclusion - 1) Intrapulmonary shunt was detected by contrast- enhanced echocardiography in the six from 35 cases of Child class C, liver cirrhosis by HBV 2) But only two patients of six positive intrapulmonary shunt patients showed significant hypoxemia (PaO2<70mmHg). Our results suggest that subclinical hepatopulmonary syrdrome may be exist.

Pulmonary Artery Embolotherapy in a Patient with Type I Hepatopulmonary Syndrome after Liver Transplantation

이해원,서경석,김주현,신우영,이남준,제환준,정진욱,오소원,강건욱,이건욱 대한영상의학회 2010 Korean Journal of Radiology Vol.11 No.4

Although liver transplantation (LT) is the only effective treatment option for hepatopulmonary syndrome (HPS), the post-LT morbidity and mortality have been high for patients with severe HPS. We performed post-LT embolotherapy in a 10-year-old boy who had severe type I HPS preoperatively, but he failed to recover early from his hypoxemic symptoms after an LT. Multiple embolizations were then successfully performed on the major branches that formed the abnormal vascular structures. After the embolotherapy, the patient had symptomatic improvement and he was discharged without complications.

Child-Pugh 분류 A군 간경화에 수반된 간폐증후군

김정선 ( Jung Sun Kim ),김창환 ( Chang Hwan Kim ),김계수 ( Gye Su Kim ),임달수 ( Dal Soo Lim ),황흥곤 ( Hweung Kon Hwang ),노영무 ( Young Moo Ro ) 대한결핵 및 호흡기학회 2009 Tuberculosis and Respiratory Diseases Vol.66 No.1

Hepatopulmonary syndrome (HPS) is characterized by a defect in arterial oxygenation that`s induced by pulmonary vascular dilatation in the setting of liver disease. Some studies have shown the relationship between the presence of the HPS and the severity of liver disease, but there are only rare cases of HPS inpatient with Child-Pugh class A liver cirrhosis. We report here on a case of a 58 years-old male who suffered from progressive dyspnea for the previous few years. He was diagnosed with alcoholic liver cirrhosis 5 years previously. There was no significant abnormality on the chest radiograph and transthoracic echocardiography, but the arterial blood gas analysis revealed severe hypoxemia. Contrast-enhanced transesophageal echocardiograpy with agitated saline demonstrated a delayed appearance of microbubbles in the left cardiac chambers. Thus, he was finally diagnosed with HPS. This case suggests that we should consider HPS when a patient with compensated liver cirrhosis has unexplained dyspnea.