Portal vein thrombosis (PVT) is a rare condition, particularly in non-cirrhotic patients. Anticoagulation remains the mainstay of the treatment. Extensive PVT can lead to variceal bleeding, ascites, bowel ischemia, and hypersplenism. The role of thrombolysis and thrombectomy in these patients remains unclear. However, there is evidence that local thrombolysis and thrombectomy should be considered in those who remain symptomatic on anticoagulation and are at risk of complications with acute PVT.

This case report describes a patient who received hormone replacement therapy for secondary panhypopituitarism and subsequently developed diabetes. His physician decided to discontinue growth hormone (GH) replacement, which was previously deemed contraindicated. Following the diagnosis of fatty liver, the patient began to exhibit liver damage that progressed over the ensuing years, ultimately leading to cirrhosis. Common factors linked to cirrhosis were excluded, leading to the belief that GH deficiency over several years was the primary contributor to cirrhosis. Therefore, when treating patients with GH insufficiency and diabetes, clinicians should carefully consider the potential implications of GH replacement therapy.

A 78-year-old woman with liver cirrhosis due to chronic hepatitis C visited our department for treatment of a thoracic aortic aneurysm. Her Child-Pugh classification was class A, and her model for end-stage liver (MELD) disease score was 8. As she also had thrombocytopenia associated with splenomegaly and esophageal varices, endoscopic injection sclerotherapy and partial splenic embolization were performed before total arch replacement surgery for treating esophageal varices to reduce the bleeding risk during transesophageal echocardiography and for thrombocytopenia, respectively. After endoscopic injection sclerotherapy and partial splenic embolization, the platelet count increased; hence, total arch replacement surgery was performed. By combining partial splenic embolization and endoscopic injection sclerotherapy, we were able to safely perform transesophageal echocardiography and total arch replacement surgery in the perioperative period.

Pseudomonas putida (P. putida) is a rare pathogen that primarily causes nosocomial infection. It is usually seen in immune dysfunction or immunocompromised patients and patients with invasive medical devices. Here, we present a rare case of P. putida bacteremia in a patient with cirrhosis of the liver.

BACKGROUND: Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This neurological dysfunction is almost always due to cirrhosis and portocaval shunt, either surgical or spontaneous.
METHODS: We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSIONS: Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.

BACKGROUND: Congenital hepatic fibrosis (CHF) is a rare autosomal recessive genetic disease, which is often diagnosed in children and young adults. The clinical manifestations of CHF were lack of specificity, mainly including portal hypertension related symptoms and signs, and normal or mildly abnormal liver function. When no obvious varices are indicated under endoscope, it can easily lead to misdiagnosis or missed diagnosis. We report this case in the hope of raising awareness of this disease.
METHODS: A 31 years old male patient with major clinical manifestations of unexplained thrombocytopenia for 5 years.
METHODS: Results of ultrasound, magnetic resonance imaging (MRI) and computed tomography portal venography (CTV) showed that patient had liver cirrhosis with portal hypertension and liver biopsy revealed CHF.
METHODS: Patient received ursodeoxycholic acid tablets, fuzheng huayu capsule, ganshuang granule, etc for liver protection treatment.
RESULTS: The condition of patient stabilized after symptomatic treatment. Spleen resection will be considered during follow-up.
CONCLUSIONS: This case reminds us that in case of patients with negative endoscopic evaluation, ultrasonic, computed tomography (CT) and MRI examination should be performed at the same time to determine whether patients have portal hypertension. When patients with normal or mildly abnormal liver function had unexplained liver cirrhosis complicated with portal hypertension, the possibility of CHF should be considered.

暂无摘要。

Esophagojejunal varices occurring after total gastrectomy are rare but potentially fatal in cases of variceal bleeding. Owing to their rarity, treatment strategies for this condition are not well established. Here, we describe the case of a 48-year-old woman who presented with hematemesis and melena. Four years prior, she underwent a total gastrectomy for gastric cancer. Esophagojejunal variceal bleeding supplied by a dilated jejunal vein, along with liver cirrhosis, was diagnosed as per endoscopy and computed tomography findings. Initial attempts at endoscopic therapy were unsuccessful. Subsequently, transjugular intrahepatic portosystemic shunt placement was performed to reduce the portal pressure gradient, resulting in the cessation of bleeding. At the 1-month follow-up endoscopy, the varices had resolved, and no rebleeding occurred during 6 months of follow-up. Transjugular intrahepatic portosystemic shunt placement may be considered as an effective treatment option for esophagojejunal variceal bleeding.

BACKGROUND: This study aims to evaluate clinical outcomes among severely injured trauma patients presenting with isolated blunt abdominal solid organ injuries with a pre-diagnosis of liver cirrhosis (LC) undergoing emergency laparotomy vs nonoperative management (NOM).
METHODS: This retrospective cohort study utilized the American College of Surgeons Trauma Quality Program Participant Use File (ACS-TQIP-PUF) dataset from 2017 to 2021. Adults (≥18 years) with a pre-existing diagnosis of LC who presented with severe blunt (ISS ≥ 16) isolated solid organ abdominal injuries and underwent laparotomy or NOM were included. Outcomes of interest included in-hospital mortality, intensive care unit length of stay (ICU-LOS), and in-hospital complications such as acute renal failure and deep vein thrombosis.
RESULTS: 929 patients were included in this analysis, with 355 undergoing laparotomy and 574 managed nonoperatively. Laparotomy patients suffered greater in-hospital mortality (n = 186, 52.3% vs n = 115, 20.0%; P < .01), required significantly more blood within 4 hours (8.9 units vs 4.3 units, P < .01), and had a significantly longer ICU-LOS (10.2 days vs 6.7 days, P < .01). In the 1:1 propensity score matched analysis of 556 matched patients, in-hospital mortality was greater for laparotomy patients (52.3% vs 20.0%, P < .01).
CONCLUSIONS: Laparotomy was associated with significantly higher in-hospital mortality in propensity-matched trauma patients, longer ICU-LOS, and more blood products given at 4 hours compared to NOM. These findings illustrate that NOM may be a safe approach in managing severely injured trauma patients with isolated blunt abdominal solid organ injuries and a pre-diagnosis of LC.

先天性肝纤维化目前仍被认为是一种罕见的常染色体隐性遗传性疾病，该病与胆管板畸形所致的肝内胆管遗传发育障碍有关。现以1例多囊肾/多囊肝病变1基因突变致胆管炎型先天性肝纤维化患者为例，探讨该病发病原因、临床表现、诊断要点以及治疗进展，以期能够在一定程度上提高肝胆科医师对该病的认识，从而有效提高早期诊断率。.