Annuloaortic ectasia in a 16 year-old boy with Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is a rare genetic disorder with an autosomal dominant inheritance due to mutations in the transforming growth factor beta-receptor type 1 or type 2. The disease is characterised by the triad of hypertelorism, bifid uvula or cleft palate, arterial tortuosity and aortic aneurysms. These phenotypic characteristics distinguish LDS from other connective tissue disorders related to transforming growth factor beta-receptor. Patients with LDS have a high risk of aortic dissection or rupture at a younger age and smaller aortic diameters. So, clinical suspicion of LDS followed by genotyping is important to prevent aortic dissection, leading cause of death, by surgical treatment.

Complete response in hepatocellular carcinoma with lymph node metastasis by combination therapy of atezolizumab and bevacizumab: a case report.

Sorafenib is the oldest first line systemic treatment in patients with advanced hepatocellular carcinoma (HCC) and has been used exclusively for nearly 10 years. The superiority of administering a combination of atezolizumab plus bevacizumab (AteBeva) compared to sorafenib as first line systemic treatment for unresectable HCC was recently proven during the IMbrave150 Phase III randomized trial. While clinicians can expect improved responses and treatment outcomes due to the good results of the IMbrave 150 trial, they must also consider that atezolizumab can cause various immune-related adverse events (IrAEs). Based on the above suggestions, we herein present a case of HCC with lymph node metastasis who achieved complete remission following treatment with AteBeva and developed an IrAE (adrenal insufficiency). Further study of real-life data regarding combination therapy with AteBeva is needed to manage patients with advanced HCC.

The Effectiveness of Personalized Bowel Preparation Using a Smartphone Camera Application: A Randomized Pilot Study.

BACKGROUND: We aimed to investigate the effectiveness of a smartphone application that analyzes and judges the optimal dosage of polyethylene glycol (PEG) for bowel preparation. METHODS: Patients were assigned to use the smartphone camera application (app group) or written instructions (non-app group). The smartphone camera application was programmed to analyze the bowel preparation quality and automatically determine the dosage of PEG from an analysis of stool images. In contrast, the non-app group consumed PEG solution according to the manual. RESULTS: The primary outcome was the quality of the bowel preparation based on blinded ratings using the Ottawa bowel preparation scale (OBPS). There was no statistically significant difference in the mean OBPS scores between the two groups (P = 0.950). However, the app group consumed a lower dose of PEG than the non-app group (mean dosage (mL): 3713.2 ± 405.8 versus 3979.2 ± 102.06, P = 0.001). The app group (5-point Likert scale; mean score 4.37 ± 0.895) had high acceptance of the application. CONCLUSIONS: Although the app group consumed a lower PEG dose, the bowel preparation quality was similar in the two groups. Moreover, use of the smartphone camera application enhanced compliance with the bowel preparation.

Two Cases of Stress Cardiomyopathy during Esophagogastroduodenoscopy.

Esophagogastroduodenoscopy (EGD) is considered a relatively safe procedure. However, the procedure and the materials used in EGD with conscious sedation can cause stress to the patient. Adverse events during EGD have been reported, represented by cardiopulmonary complications. To date, five cases have reported worldwide to be associated with gastrointestinal endoscopy. Stress cardiomyopathy (SCMP) is a reversible cardiomyopathy that typically occurs in postmenopausal women due to stress and may resolve within a few weeks. SCMP resembles acute myocardial infarction but differs in terms of treatment and prognosis. Here, we describe two cases of SCMP with shock during EGD with conscious sedation.

[A Case of Lead Poisoning with Drug-induced Liver Injury after Ingestion of Herbal Medicine].

A 61-year-old male patient was admitted because of unexplained abdominal pain and anemia. His past medical history was unremarkable except for having taken herbal medicine to treat facial palsy two months ago. The result of health examination performed about a month ago showed increased serum aspartate and alanine aminotransferase level, and he was diagnosed with toxic hepatitis by herbal medicine. When the patient presented to the outpatient department three weeks ago, follow-up liver function test results showed improvement but he complained of abdominal pain. Despite extensive blood chemistry tests and computed tomography, the cause of pain could not be found. After much deliberation, serum lead level and herbal medicines analysis was performed based on the fact that he took herbal medicine two months ago, and he could finally be diagnosed with lead poisoning. Since the serum lead level was high enough to be indicated for lead chelating therapy, conservative management was given. When a patient with toxic hepatitis due to herbal medication presents with abdominal pain, the possibility of lead poisoning should always be taken into consideration.

Acute Stroke and Limb Ischemia Secondary to Catastrophic Massive Intracardiac Thrombus in a 40-Year-Old Patient With Dilated Cardiomyopathy.

Dilated cardiomyopathy has been associated with left ventricular (LV) thrombosis which leads to substantial morbidity and mortality as a site for systemic emboli. We report an interesting case of a stroke and acute limb ischemia secondary to a large mobile pedunculated LV thrombus in 40-year-old patient with dilated cardiomyopathy.

A rare combination of left ventricular noncompaction, patent ductus arteriosus, and persistent left superior vena cava demonstrated by multidetector computed tomography and echocardiography.

Noncompaction of the ventricular myocardium (NCM) is a disorder characterized by numerous prominent ventricular trabeculations and deep intertrabecular recesses. It may present in an isolated form or in association with other cyanotic heart diseases, obstructions of the ventricular outflow tract, and anomalies of coronary arteries. We report on a rare combination of NCM, patent ductus arteriosus, and persistent left superior vena cava in a 27-year-old man examined via echocardiography and multidetector computed tomography.