Objectives: This study investigated the 24-hour ambulatory blood pressure monitoring (ABPM) and Holter parameters for evaluating their prognostic significance of cardiovascular events including stroke in population without atrial fibrillation (AF). Methods: Among 3,199 patients that underwent ABPM, 335 who also underwent Holter recordings were selected in a tertiary hospital. Seventeen patients who had been documented with AF on Holter monitoring or diagnosed with AF were excluded, and finally 318 patients were analyzed. The association between cardiovascular events and ABPM/Holter parameters was analyzed by a logistic regression model, and the risk factors were estimated by a Cox hazard model. Age, sex, and histories of cardiovascular disease were adjusted by a multivariable analysis, and the cut-off values were suggested by a Kaplan-Meyer analysis. Results: During the total follow-up (28.5±1.7 months), 13 (4.1%) stroke, 6 (1.9%) heart failure, and 12 (3.8%) acute coronary syndrome incidences were observed. In the univariate analysis of the ABPM parameters, an increment in the night systolic BP (hazard ratio=1.034, P=0.020) and night diastolic BP (hazard ratio=1.063, P=0.031) significantly elevated the risk of a stroke occurrence. According to the Kaplan-Meyer analysis, there was a significant difference in the stroke incidence between the groups divided by a cut-off value of the night systolic BP of 120 mmHg (P=0.014) and night diastolic BP of 75 mmHg (P=0.023). Conclusion In a population without AF, the nocturnal BP was a significant predictor of a stroke incidence. At this point, the cut-off value of mean 120/75 mmHg in 24 ABPM was advisable.

Catastrophic antiphospholipid syndrome (APS) is defined as a rare, life-threatening autoimmune disorder leading to multiorgan failure. Probable APS, with clinical manifestations similar to APS without antiphospholipid antibodies, was suggested to be seronegative catastrophic APS. The triggering factors of catastrophic APS are various, including infection, trauma, malignancy, and surgery. In approximately 40% of patients, catastrophic APS develops from an unknown cause. We report a case of seronegative catastrophic APS due to an unknown origin. A 20-year-old man presented with cough, abdominal pain, skin lesions, tunnel vision, and watery diarrhea without fever. His symptoms and laboratory test suggested disseminated intravascular coagulation. Considering seronegative catastrophic APS, we treated with intravenous steroid and intravenous immunoglobulin, but the effects were limited. After weekly treatment with rituximab, an immune-modulating agent, his laboratory findings including thrombocytopenia and coagulation tests, returned to normal. We conclude that rituximab can be an effective treatment for seronegative catastrophic APS.
Abdominal Pain ; Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Cough ; Diarrhea ; Disseminated Intravascular Coagulation* ; Fever ; Humans ; Immunoglobulins ; Skin ; Thrombocytopenia ; Young Adult ; Rituximab

Giant cell reparative granuloma (GCRG) is a rare benign bone disease of unknown causes. Trauma is a suspected cause or contributor to the onset of GCRG, of which only a few cases have been reported worldwide. We report a case of temporal bone GCRG in a 4-month-old male newborn, who was born at full term via spontaneous vaginal delivery with recent presentation of right facial palsy. He had a right temporo-occipital craniectomy due to GCRG 1 month earlier. There had been no history of trauma. During the workup of the facial palsy complaint, we identified a lobulated mass in the right temporal bone by computed tomography and magnetic resonance imaging. The mass was mainly in the petrous portion of the temporal bone with extracranial extension through the occipital bone. This was consistent with a GCRG recurrence. The mass was removed via the transcrusal approach, and the final histopathology report confirmed it as recurrent GCRG.

Giant cell reparative granuloma (GCRG) is a rare benign bone disease of unknown causes. Trauma is a suspected cause or contributor to the onset of GCRG, of which only a few cases have been reported worldwide. We report a case of temporal bone GCRG in a 4-month-old male newborn, who was born at full term via spontaneous vaginal delivery with recent presentation of right facial palsy. He had a right temporo-occipital craniectomy due to GCRG 1 month earlier. There had been no history of trauma. During the workup of the facial palsy complaint, we identified a lobulated mass in the right temporal bone by computed tomography and magnetic resonance imaging. The mass was mainly in the petrous portion of the temporal bone with extracranial extension through the occipital bone. This was consistent with a GCRG recurrence. The mass was removed via the transcrusal approach, and the final histopathology report confirmed it as recurrent GCRG.
Bone Diseases ; Bone Neoplasms ; Facial Paralysis ; Giant Cells ; Granuloma ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Occipital Bone ; Recurrence ; Temporal Bone

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract. Most NCMH has a benign nature and can be cured by complete resection. Only a few NCMH cases have been reported worldwide due to its rarity. Here, we report three cases of successfully treated NCMH, of which one case was diagnosed during preliminary ultrasound. All three cases were consistent with the benign symptoms of NCMH. Tumor in all of the cases were successfully treated through an endoscopic approach surgery and no evidence of aggressive recurrence was reported.

Background and Objectives: Middle ear implants (MEI) have been reported to be an effective and safe alternative for the treatment of sensorineural hearing loss (SNHL) and conductive hearing loss (CHL). This study aimed to compare the functional outcomes between SNHL and CHL in terms of audiological gains.Subjects and Method The medical records of 14 consecutive SNHL and CHL patients who underwent MEI surgeries from 2015 to 2019 by a single surgeon were retrospectively reviewed. Audiological changes using hearing aids (HA) and MEI were compared. Results: In SNHL, the mean unaided air-conduction pure tone audiometry (PTA), (57.7 dB HL) decreased significantly using HA and MEI (44.7 and 41.4 dB HL), but with no significant difference from each other. Unaided word recognition score (WRS) at 65 dB HL (45.1%) was significantly improved using HA and MEI (72.6% and 76.6%), with no significant difference. In CHL, the mean unaided air-conduction and bone-conduction PTA were 77.1 (57.5-93.8) and 44.1 (26.3-57.5) dB HL. Three patients could not use conventional HAs due to otorrhea and deformity of the external auditory canal by previous surgeries and 4 patients had used conventional HAs before MEI. Though both HA and vibrant soundbridge (VSB) showed improvement of hearing thresholds (32.0 and 48.8 dB HL) and WRS (80.0% and 94.9%), it was significant only when using VSB. There was no significant difference between HA and VSB. Conclusion MEI can be an effective treatment option for both SNHL and CHL patients, especially for those who suffered from problems using conventional HA.

Background and Objectives: In this study, we investigated the effects of hearing loss on mental health and quality of life (QoL) using survey data in adults aged >40 years. Subjects and Methods: We obtained data from 10,921 individuals who responded to the fifth Korean National Health and Nutrition Examination Survey. Primary outcomes were measured using questionnaires that recorded stress perception, depressive mood, and suicidal ideation to evaluate mental health and motor ability, self-management, activities of daily living, pain or discomfort, and anxiety or depression to evaluate QoL. Results: On multivariate analysis, suicidal ideation was more prevalent among older adults with hearing loss than in older adults without hearing loss. Motor ability was lower in individuals with hearing loss of >20 dB than in older adults with normal hearing. Categorization of the EuroQol-5 Dimension (EQ-5D) health state into upper and lower groups showed that the EQ-5D values were lower in the moderate-tosevere hearing loss group than in the mild hearing loss group. Conclusions Among adults aged >40 years, those with hearing loss experienced more suicidal ideation, had lower motor ability, and lower overall QoL compared with these variables in older adults with normal hearing. Additionally, QoL scores were low in individuals with poor hearing.

PURPOSE: Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is a rare genetic syndrome resulting from germline mutations in fumarate hydratase. The combination of bevacizumab plus erlotinib showed promising interim results for HLRCC-associated RCC. Based on these results, we analyzed the outcome of bevacizumab plus erlotinib in Korean patients with HLRCC-associated RCC. MATERIALS AND METHODS: We retrospectively reviewed the efficacy and safety of bevacizumab plus erlotinib in patients with HLRCC-associated RCC who were confirmed to have germline mutations in fumarate hydratase. The primary endpoint was the objective response rate (ORR), while the secondary endpoints were progression-free survival (PFS) and overall survival (OS). RESULT: We identified 10 patients with advanced HLRCC-associated RCC who received bevacizumab plus erlotinib. Median age at diagnosis was 41 years, and five of the patients had received the combination as first- or second-line treatments. The ORR was 50% and the median PFS and OS were 13.3 and 14.1 months, respectively. Most adverse events were predictable and manageable by conventional measures, except for one instance where a patient died of gastrointestinal bleeding. CONCLUSION: This is the first real-world outcome of the treatment of advanced HLRCC-associated RCC. Bevacizumab plus erlotinib therapy showed promising activity with moderate toxicity. We should be increasingly aware of HLRCC-associated RCC and bevacizumab plus erlotinib should be a first-line treatment for this condition, unless other promising data are published.
Bevacizumab ; Carcinoma, Renal Cell ; Diagnosis ; Disease-Free Survival ; Erlotinib Hydrochloride ; Fumarate Hydratase ; Germ-Line Mutation ; Hemorrhage ; Humans ; Leiomyomatosis ; Retrospective Studies

Objectives: . This study aimed to evaluate long-term changes after balloon dilation of the Eustachian tube (BDET) in chronic otitis media (COM) patients with Eustachian tube (ET) dysfunction that persisted after tympanomastoidectomy (TM). Methods: . We retrospectively reviewed the medical records of consecutive patients who were diagnosed with COM and ET dysfunction and underwent TM at our tertiary hospital from 2016 to 2017. The tympanic membrane status, the presence of a ventilation tube, ability to perform the Valsalva maneuver, and audiologic changes after dilation of the ET were analyzed. Results: . This study included 20 patients (with 21 ears) who underwent TM but could not perform the Valsalva maneuver, showed a persistent air-bone gap, and eventually underwent BDET (male:female, 8:13; right:left, 11:10). Four ears showed perforation of the tympanic membrane after TM. Among the remaining 17 ears, 15 ears underwent ventilation tube insertion before BDET, while two ears underwent ventilation tube insertion and BDET simultaneously. Although none of the patients were capable of the Valsalva maneuver before BDET, 13 (62%) were able to perform the Valsalva maneuver successfully after BDET. When evaluating the tympanic membrane status at the latest follow-up, ventilation tubes were still present in eight ears. In the other 13 ears, intact tympanic membranes were present in nine out of 11 ears n the successful Valsalva group, whereas none of them were intact in the unsuccessful Valsalva group (P=0.014). The successful Valsalva group after BDET showed an improved air-bone gap of 8.9±12.4 dB, while the unsuccessful Valsalva group showed an aggravated air-bone gap of 3.8±11.8 dB at 1 year after BDET; this difference was statistically significant (P=0.031). Conclusion The Valsalva maneuver could be performed successfully after BDET by 62% of patients with COM and ET dysfunction. BDET is helpful for successful hearing improvement and improved tympanic aeration in COM patients with ET dysfunction.