Severe mitral regurgitation in a young female with pansinusitis and bronchiectasis

SummaryPrimary ciliary dyskinesia (PCD) is a disease characterized by symptoms of upper and lower respiratory tract infections due to abnormal structure and function of cilia.Cardiac involvement is characterized by situs inversus (Kartagener's syndrome in PCD) and other congenital cardiovascular abnormalities. We describe a 34-year-old female with a history of recurrent sinusitis and bronchiectasis but without situs inversus or other congenital cardiac anomalies in whom an association between mitral regurgitation secondary to myxoid degeneration and primary ciliary dyskinesia was suggested

Evolution of ribosomal DNA-derived satellite repeat in tomato genome

<p>Abstract</p> <p>Background</p> <p>Tandemly repeated DNA, also called as satellite DNA, is a common feature of eukaryotic genomes. Satellite repeats can expand and contract dramatically, which may cause genome size variation among genetically-related species. However, the origin and expansion mechanism are not clear yet and needed to be elucidated.</p> <p>Results</p> <p>FISH analysis revealed that the satellite repeat showing homology with intergenic spacer (IGS) of rDNA present in the tomato genome. By comparing the sequences representing distinct stages in the divergence of rDNA repeat with those of canonical rDNA arrays, the molecular mechanism of the evolution of satellite repeat is described. Comprehensive sequence analysis and phylogenetic analysis demonstrated that a long terminal repeat retrotransposon was interrupted into each copy of the 18S rDNA and polymerized by recombination rather than transposition via an RNA intermediate. The repeat was expanded through doubling the number of IGS into the 25S rRNA gene, and also greatly increasing the copy number of type I subrepeat in the IGS of 25-18S rDNA by segmental duplication. Homogenization to a single type of subrepeat in the satellite repeat was achieved as the result of amplifying copy number of the type I subrepeat but eliminating neighboring sequences including the type II subrepeat and rRNA coding sequence from the array. FISH analysis revealed that the satellite repeats are commonly present in closely-related <it>Solanum </it>species, but vary in their distribution and abundance among species.</p> <p>Conclusion</p> <p>These results represent that the dynamic satellite repeats were originated from intergenic spacer of rDNA unit in the tomato genome. This result could serve as an example towards understanding the initiation and the expansion of the satellite repeats in complex eukaryotic genome.</p

Stent insertion and balloon angioplasty for portal vein stenosis after liver transplantation: long-term follow-up results

PURPOSEIt is not easy to determine whether balloon angioplasty or stenting should be performed in patients with portal vein stenosis after liver transplantation. We aimed to propose appropriate indication by evaluating long-term outcomes of balloon angioplasty and stent insertion in adult liver transplant patients.METHODSWe retrospectively reviewed 31 patients with portal vein stenosis among 1369 patients who underwent adult liver transplantation from January 2001 to December 2015. When stenosis was confirmed by venography, angioplasty was performed first. When there was no flow improvement or pressure gradient was not decreased after angioplasty, stent insertion was performed. We also performed primary stent insertion without angioplasty for diffuse stenosis, kinking, external compression, and near occlusion of portal vein in venography. We assessed patency in patients who underwent percutaneous transluminal angioplasty and stent insertion through regular outpatient follow-up and evaluated technical and clinical success and long-term results.RESULTSTechnical success was 85% and 100% in balloon angioplasty and stent insertion, respectively. Clinical success was achieved in 78% of balloon angioplasties and in 100% of stent insertions. At 1, 5, and 10 years after balloon angioplasty, patency rates were 87%, 82%, and 68% respectively, and the rates of stent patency were all 100%. Portal vein size measured during the operation of patients with and without recurrence were 19±4.2 mm and 19±3.0 mm (P = 0.956), respectively. The balloon size of patients with and without recurrence were 11±1.95 mm and 14±1.66 mm, respectively (P = 0.013), when balloon angioplasty was performed after stenosis diagnosis.CONCLUSIONStent insertion can be considered when fibrotic changes are expected due to repeated inflammation and when the balloon size to be used is small. Balloon angioplasty seems less risky for anastomotic ruptures in portal vein stenosis in the early post liver transplantation period

Pharmacokinetics and diuretic effect of furosemide after single intravenous, oral tablet, and newly developed oral disintegrating film administration in healthy beagle dogs

Background Furosemide, a diuretic that acts on the loop of Henle, is commonly used to treat congestive heart failure in veterinary medicine. Some owners have difficulty in administering oral tablet medication to animal patients, which leads to noncompliance, especially during long-term administration. Oral disintegrating film (ODF) has the advantages of easy administration via a non-invasive route, rapid dissolution, and low suffocating risk. The objective of this study was to research the pharmacokinetic (PK) profiles and diuretic effect of furosemide after intravenous (IV), orally uncoated tablet (OUT), and newly developed ODF administration in healthy beagle dogs. In this study, a furosemide-loaded ODF (FS-ODF) formulation was developed and five beagle dogs were administered a single dose (2 mg/kg) of furosemide via each route using a cross-over design. Results The most suitable film-forming agent was sodium alginate; thus, this was used to develop an ODF for easy drug administration. No significant differences were detected in the PK profiles between OUT and FS-ODF. In the blood profiles, the concentration of total protein was significantly increased compared to the baseline (0 h), whereas no significant difference was detected in the concentration of creatinine and hematocrit compared to the baseline. FS-ODF resulted in a similar hourly urinary output to OUT during the initial 2 h after administration. The urine specific gravity was significantly decreased compared to the baseline in each group. The peak times of urine electrolyte (sodium and chloride) excretion per hour were 1 h (IV), 2 h (OUT), and 2 h (FS-ODF). Conclusions These results suggest that the PK/PD of furosemide after administration of newly developed FS-ODF are similar to those of OUT in healthy dogs. Therefore, the ODF formulation has the benefits of ease and convenience, which would be helpful to owners of companion animals, such as small dogs (< 10 kg), for the management of congestive heart failure

Acute Idiopathic Hemorrhagic Pericarditis with Cardiac Tamponade as the Initial Presentation of Acquired Immune Deficiency Syndrome

This paper presents a case of cardiac tamponade with idiopathic hemorrhagic pericarditis as the initial symptom of human immunodeficiency virus (HIV) infection. A 29-year-old male came to the emergency room with a sudden onset of dizziness. Upon arrival, he was hypotensive although not tachycardic, and his jugular venous pressure was not elevated. His chest X-rays revealed a mild cardiomegaly. Transthoracic echocardiography revealed a large amount of pericardial effusion with a diastolic collapse of the right ventricle, a dilated inferior vena cava with little change in respiration, and exaggerated respiratory variation of mitral inflow velocities, representing echocardiographic evidence of cardiac tamponade. After pericardiocentesis, his blood pressure improved to 110/70 mmHg without inotropics support. Serial 12-lead electrocardiograms during hospitalization revealed upwardly concave diffuse ST-segment elevation followed by a T-wave inversion suggestive of acute pericarditis. Pericardial fluid cytology and cultures for bacteria, mycobacteria, adenovirus, and fungus were all negative. HIV enzyme-linked immunosorbent assay (ELISA) was positive and confirmed by Western blot. The CD4 cell count was 168/mm3. Finally, the diagnosis of cardiac tamponade due to HIV-associated hemorrhagic pericarditis was made. It was concluded that HIV infection should be considered in the diagnosis of unexplained pericardial effusion or cardiac tamponade in Korea

Impact of immunosuppressant therapy on early recurrence of hepatocellular carcinoma after liver transplantation

Background/AimsThe most commonly used immunosuppressant therapy after liver transplantation (LT) is a combination of tacrolimus and steroid. Basiliximab induction has recently been introduced; however, the most appropriate immunosuppression for hepatocellular carcinoma (HCC) patients after LT is still debated.MethodsNinety-three LT recipients with HCC who took tacrolimus and steroids as major immunosuppressants were included. Induction with basiliximab was implemented in 43 patients (46.2%). Mycophenolate mofetil (MMF) was added to reduce the tacrolimus dosage (n=28, 30.1%). The 1-year tacrolimus exposure level was 7.2 ± 1.3 ng/mL (mean ± SD).ResultsThe 1- and 3-year recurrence rates of HCC were 12.9% and 19.4%, respectively. Tacrolimus exposure, cumulative steroid dosages, and MMF dosages had no impact on HCC recurrence. Induction therapy with basiliximab, high alpha fetoprotein (AFP; >400 ng/mL) and protein induced by vitamin K absence/antagonist-II (PIVKA-II; >100 mAU/mL) levels, and microvascular invasion were significant risk factors for 1-year recurrence (P<0.05). High AFP and PIVKA-II levels, and positive 18fluoro-2-deoxy-d-glucose positron-emission tomography findings were significantly associated with 3-year recurrence (P<0.05).ConclusionsInduction therapy with basiliximab, a strong immunosuppressant, may have a negative impact with respect to early HCC recurrence (i.e., within 1 year) in high-risk patients

Peutz-Jeghers Syndrome with Multiple Genital Tract Tumors and Breast Cancer: A Case Report with a Review of Literatures

We report here on the multiple genital tract neoplasms in a 41-yr-old Korean woman with Peutz-Jeghers Syndrome (PJS). The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer. Pelvic ultrasound showed a multilocular cyst at the right adnexal region, diagnosed as bilateral ovarian mucinous borderline tumors. An ovarian sex cord tumor with annular tubules was incidentally diagnosed together with a minimal deviation adenocarcinoma of the uterine cervix and mucinous metaplasia of both the Fallopian tubal mucosa and the endometrium. Although the cases of multiple genital tract tumors with PJS has rarely been reported, the present case appears to be the first in Korea in which the PJS syndrome was complicated by multiple genital tract tumors and infiltrating carcinoma of the breast. The clinical significance of the multiple genital tract tumors and breast cancer associated with PJS is reviewed

Biliary complications in living donor liver transplantation: imaging findings and the roles of interventional procedures

PURPOSE: To describe the incidence, types, and findings of biliary complications in living donor liver transplantation (LDLT) and to determine the roles of interventional procedures. MATERIALS AND METHODS: Twenty-four biliary complications among 161 LDLT patients (24/161, 14.9%) were identified. These complications were divided into two groups according to the initial manifestation time, i.e., "early" (<60 days) or "late". The CT and cholangiographic findings were reviewed regarding the presence of a stricture or leak and the location, and length, shape, and degree of the stricture. Both groups were categorized into three subgroups: leak, stricture, and both. The type of interventional procedures used and their roles were determined. RESULTS: Early complications were identified in 14 of the 24 patients (58%) and late complications in 11 (46%). One patient showed both early and late complications. Biliary stricture was detected in 10 patients, leak in 10, and both in 5. By cholangiography, all strictures were irregular and short (mean length 15 +/- 6 mm) at the anastomotic site and complete obstruction was observed in 2 patients with late stricture. Twenty-three of the 24 patients were treated using percutaneous and/or endoscopic drainage procedures with or without balloon dilatation. Seventeen (74%) showed a good response, but reoperations were inevitable in 6 (26%). All patients except those with complete obstruction showed a favorable outcome after interventional management. CONCLUSION: Biliary leaks and strictures are predominant complications in LDLT. Most show good responses to interventional treatment. However, complete obstruction needs additional operative management

Cardiac tamponade caused by tuberculosis pericarditis in renal transplant recipients

A 50-year-old male, renal transplant recipient, was admitted with fever and chest discomfort. At admission, chest radiologic finding was negative and echocardiography showed minimal pericardial effusion. After 2 days of admission, chest pain worsened and blood pressure fell to 60/40 mmHg. Emergency echocardiography showed a large amount of pericardial effusion compressing the entire heart. Pericardiocentesis was performed immediately. Mycobacterium tuberculosis was isolated from pericardial fluid. Tuberculosis pericarditis should be considered as the cause of cardiac tamponade in renal transplant recipients, even with the absence of pericardial effusion in the initial study or suggestive history