[Effect of growth hormone supplementation on liver and lung function in patients with hypopituitarism].

To analyze the clinical features of patients with anterior hypopituitarism (HP) complicated with cirrhosis, and to explore the effects of growth hormone supplementation on liver and lung function. A total of 11 patients with HP complicated with cirrhosis admitted to Peking Union Medical College Hospital from January 2016 to December 2022 were included in the study, including 8 males and 3 females, aged [M(Q1, Q3)]31 (20, 37) years. There were 6 patients with pituitary stalk interruption syndrome, 4 patients after craniopharyngioma resection, and 1 patient after germinal cell tumor chemoradiotherapy. Cirrhosis appeared at [M(Q1, Q3)]7 (1, 16) years after the diagnosis of HP. There were 7 cases complicated with hepatopulmonary syndrome (HPS). The liver and lung function of 5 patients were improved significantly after the addition of growth hormone, and the arterial partial pressure of oxygen increased from (47±11) mmHg(1 mmHg=0.133 kPa) to (84±12) mmHg. Timely supplementation of growth hormone can improve the symptoms of fatty liver, cirrhosis and HPS, and postpone or even avoid the transplantation of liver and other organs.

Diagnosis of Hepatopulmonary Syndrome in a Large Integrated Health System.

BACKGROUND & AIMS: Data on the accuracy of the diagnosis of hepatopulmonary syndrome (HPS) in cirrhosis is limited. We evaluated the clinical characteristics of patients with International Classification of Diseases (ICD) codes for hepatopulmonary syndrome (HPS) in a large integrated health system. METHODS: A retrospective review of encounters was performed of all patients with ICD-9-CM and/or ICD-10-CM diagnosis of cirrhosis and HPS from 2014-2019 in a multi-state health system. Demographics and cardiopulmonary testing closest to the time of HPS diagnosis were recorded. HPS was defined using standard criteria. RESULTS: A total of 42,749 unique individuals with cirrhosis were identified. An ICD diagnosis of HPS was found in 194 patients (0.45%), of which 182 had clinically confirmed cirrhosis. 143 (78.5%) underwent contrast-enhanced transthoracic echocardiography, and 98 (54%) had delayed shunting. Among them, 61 patients had a documented arterial blood gas, with 53 showing abnormal oxygenation (A-a gradient of >15 mm Hg). 12 were excluded due to significant pulmonary function test abnormalities and abnormal oxygenation from other cardiopulmonary diseases. Ultimately, 41 (22.5%) fulfilled the criteria for HPS. When stratifying those with an ICD code diagnosis of HPS into HPS, no HPS and indeterminate HPS groups, based on standard diagnostic criteria for HPS, we found that the confirmed HPS patients had similar complications except for less portopulmonary hypertension, worse gas exchange, less cardiopulmonary disease and were more often diagnosed in transplant centers. CONCLUSIONS: The diagnosis of HPS by ICD code is made in an extremely small subset of a sizeable cirrhotic cohort. When made, only a minority of these patients meet diagnostic criteria. Our findings highlight the need for improved education and more effective screening algorithms.

Sorafenib in Hepatopulmonary Syndrome: A Randomized, Double-Blind, Placebo-Controlled Trial.

The tyrosine kinase inhibitor sorafenib improves hepatopulmonary syndrome (HPS) in an experimental model. However, the efficacy and adverse effect profile in patients with HPS are unknown. We aimed to determine the effect of sorafenib on the alveolar-arterial oxygen gradient (AaPO2 ) at 3 months in patients with HPS. We performed a randomized, double-blind, placebo-controlled parallel trial of sorafenib in patients with HPS at 7 centers. A total of 28 patients with HPS were randomized to sorafenib 400 mg by mouth daily or a matching placebo in a 1:1 ratio. We found no statistically significant difference in the median change in AaPO2 from baseline to 12 weeks between the patients allocated to sorafenib (4.5 mm Hg; IQR, -3.8 to 7.0 mm Hg) and those allocated to placebo (-2.4 mm Hg; IQR, -4.8 to 8.2 mm Hg; P = 0.70). There was also no difference between the groups in terms of degree of intrapulmonary shunting by contrast echocardiography. Sorafenib significantly reduced circulating levels of angiogenic markers, including vascular endothelial growth factor receptors (P < 0.01) and TIE2-expressing M2 monocytes (P = 0.03), but it reduced the mental component scores of the Short Form 36 (P = 0.04), indicating a worse quality of life. In conclusion, sorafenib did not change the AaPO2 or other disease markers at 3 months in patients with HPS. Alternative antiangiogenic therapies or treatments targeting other pathways should be investigated.

Hepatopulmonary syndrome: an unusual cause of dyspnea in the pulmonology ward - case presentation.

Hepatopulmonary syndrome is one of the possible complications of chronic liver disease, defined clinically by impaired oxygenation. The underlying cause of the respiratory failure is the presence of intrapulmonary shunting, as a result of abnormal vascular dilatations in the lungs. We report the case of 52-year-old male, exsmoker, with a history of pulmonary TB and also of heavy drinking, who was admitted to the pulmonology ward for dyspnea at rest and limb cyanosis. His clinical exam was suggestive of liver cirrhosis, with signs of pneumonia, but also chronic lung disease. Variations in SaO2 with posture were noted: platypnea and orthodeoxia. Arterial gas assessment revealed severe hypoxemia, only partially corrected by high-flow oxygen therapy, while plethysmography showed only a mild obstructive syndrome, but with severely impaired alveolar-capillary diffusion. The suspicion of a hepatopulmonary syndrome was raised and a contrast echocardiography confirmed the diagnosis by revealing the presence of an intrapulmonary shunt. Although it is believed to be a fairly common complication of chronic liver disease, it is possible for a case of hepatopulmonary syndrome to be admitted solely for respiratory symptoms. The patient's poor socio-economic status is the main reason for both the lack of proper followup for his liver disease and the limited therapeutic options. Keywords: Hepatopulmonary syndrome, liver cirrhosis, respiratory failure, contrast echocardiography

Treatment of hepatopulmonary syndrome with Allium sativum L. (garlic): a pilot trial.

No medical therapy exists for subjects with hepatopulmonary syndrome (HPS). A patient with HPS was reported to have improvement in arterial oxygenation while self-administering garlic. Our goal was to determine whether a standardized garlic powder improves arterial oxygenation and dyspnea in subjects with HPS. A prospective, open label uncontrolled pilot study in 15 subjects with HPS were administered garlic powder capsules daily for a minimum of 6 months. Arterial blood gases were determined every 4-8 weeks, in the same position on room air, and a subjective dyspnea transition index was reported. Six of 15 subjects (40%, confidence interval: 0.15-65) had at least a 10 mmHg increase in the P(O2) or decrease in the alveolar-arterial gradient. The mean pre- and postarterial difference in these patients were: P(O2) (14+/-4 mmHg) and alveolar-arterial gradient (18+/-5 mmHg). All 6 subjects who responded to garlic had less dyspnea on exertion. Garlic improved arterial oxygenation in younger subjects (mean 40 versus 56 years old; p = 0.021) or those with lower macroaggregated albumin shunt fractions (mean 21 versus 44%, p = 0.058). Garlic may improve arterial oxygenation and symptoms in patients with hepatopulmonary syndrome and warrants further investigation.