BACKGROUND: To explore an interaction(s) of endothelin with other blood pressure regulating systems, effects of either a high concentration of sodium chloride vehicle or pretreatment with atrial natriuretic peptide (ANP) on the pressor response to endothelin were investigated in rats. METHOD: Sprague-Dawley rats were anesthetized with thiopental (50mg/kg, IP) and were cannulated into left lateral cerebral ventricle. The left femoral artery and vein were also catheterized to measure blood pressure and to serve as an infusion route, respectively. Endothelin (10pmol) dissolved in either normal (0.15M) or high concentration (3.0M) of NaCl vehicle with ANP (1 nmol, ICV). For intravenous (IV) infusion, endothelin(1pmol/min) dissolved either in normal or high concentration of NaCl vehicle was infused for 20min. RESULT: ICV endothelin caused an increase of arterial pressure, in which the NaCl did not affect but ANP-pretreatment significantly attenuated the pressor magnitude. IV endothelin also increased the arterial pressure, which was potentiated by NaCl, ie, the maximum increase caused by the IV endothelin in the high NaCl vehicle was greater than the sum of that induced by endothelin (infused in normal NaCl vehicle) and that induced by high NaCl vehicle only. CONCLUSION: These results suggest that endothelin has separate central and peripheral pressor actions, in which the former is not affected by high NaCl but is attenuated by the ANP system and the latter is potentiated by high NaCl.
Animals ; Arterial Pressure ; Atrial Natriuretic Factor ; Blood Pressure ; Catheters ; Cerebral Ventricles ; Endothelins* ; Femoral Artery ; Rats* ; Rats, Sprague-Dawley ; Sodium Chloride* ; Sodium* ; Thiopental ; Veins

Compression of the airway is relatively common in pediatric patients, although it is often an unrecognized complication of congenital cardiac and aortic arch anomalies. Aortopexy has been established as a surgical treatment for tracheobronchial obstruction associated with vascular anomaly, aortic arch anomaly, esophageal atresia, and tracheoesophageal fistula. The tissue-to-tissue arch repair technique could result in severe airway complication such as compression of the left main bronchus which was not a problem before the correction. We report three cases of corrective open heart surgery monitored by intraoperative bronchoscopy performed during prebypass, and performed immediately before weaning from bypass, to evaluate tracheobronchial obstruction caused by congenital, complex cardiac anomalies in the operating room.
Airway Obstruction* ; Aorta, Thoracic ; Aortic Coarctation ; Bronchi ; Bronchoscopy* ; Esophageal Atresia ; Humans ; Operating Rooms ; Thoracic Surgery ; Tracheoesophageal Fistula ; Weaning

Background: Scars in children are highly concerning to most parents who usually seek prompt treatment for these lesions. However, compared with adults, children show a greater tendency to develop hypertrophic scars and a higher likelihood of scar widening with increasing age. Objective: We investigated the role of laser treatment for scars in pediatric patients in view of the fact that this approach is challenging in this age group. Methods: This retrospective study included patients aged ＜17 years who visited the Samsung Medical Center between 2013 and 2018 for scar treatment. Of the 28 children who visited the center during this time, 14 presented within 4 to 5 weeks from scar onset and received laser therapy and 14 presented after 4 to 5 weeks and received topical treatment. The Stony Brook Scar Evaluation Scale (SBSES) was used to assess scar formation. Results: The mean initial SBSES scores were lower in the laser than in the topical group (1.93±0.92 vs. 2.71±0.83, p=0.0363). No intergroup difference was observed in SBSES scores upon treatment completion (4.50±0.94 vs. 4.21±1.19, p=0.4673). Multivariable analysis after adjustment for age, sex, and initial SBSES scores showed that the type (laser vs. topical) of treatment was not significantly associated with an SBSES score=5 or duration of treatment. Conclusion Laser intervention may be useful for scar therapy in children who present early and can receive prompt treatment before scar maturation; however, further studies are warranted to validate our results.

Background: Scars in children are highly concerning to most parents who usually seek prompt treatment for these lesions. However, compared with adults, children show a greater tendency to develop hypertrophic scars and a higher likelihood of scar widening with increasing age. Objective: We investigated the role of laser treatment for scars in pediatric patients in view of the fact that this approach is challenging in this age group. Methods: This retrospective study included patients aged ＜17 years who visited the Samsung Medical Center between 2013 and 2018 for scar treatment. Of the 28 children who visited the center during this time, 14 presented within 4 to 5 weeks from scar onset and received laser therapy and 14 presented after 4 to 5 weeks and received topical treatment. The Stony Brook Scar Evaluation Scale (SBSES) was used to assess scar formation. Results: The mean initial SBSES scores were lower in the laser than in the topical group (1.93±0.92 vs. 2.71±0.83, p=0.0363). No intergroup difference was observed in SBSES scores upon treatment completion (4.50±0.94 vs. 4.21±1.19, p=0.4673). Multivariable analysis after adjustment for age, sex, and initial SBSES scores showed that the type (laser vs. topical) of treatment was not significantly associated with an SBSES score=5 or duration of treatment. Conclusion Laser intervention may be useful for scar therapy in children who present early and can receive prompt treatment before scar maturation; however, further studies are warranted to validate our results.

Cowden syndrome is caused by mutations in the phosphatase and tensin homolog (PTEN) gene and is part of the PTEN hamartoma tumor syndrome. Skin lesions including trichilemmomas, acral keratosis, mucocunateous neuromas and oral paillomas are the most prevalent feature found in patients with Cowden syndrome. It also possesses an increased risk of developing malignancies including breast, thyroid, endometrial, and colorectal cancers.Due to the increased risk of cancer, early diagnosis and regular surveillance are important for Cowden syndrome patients. Herein, we report a case of Cowden syndrome with diverse cutaneous manifestations and thyroid cancer.

A capillary hemangioma is a vascular tumor with small capillary sized vascular channel. Multiple capillary hemangioma in relation with drugs have been rarely reported. Here in, we report a case of multiple capillary hemangioma in patient diagnosed with chronic myeloid leukemia who received tyrosine kinase inhibitors (TKIs). Histopathological findings have shown capillary proliferation in the upper dermis, which is consistent with capillary hemangioma. Since TKIs can paradoxically activate the MEK/ERK pathway which is required for angiogenesis, we presumed that the lesions as the cutaneous side effects of TKIs.

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is rare Epstein-Barr virus (EBV)-associated disease. The classic form of HVLPD is a self-resolving disease, whereas the systemic form can progress to malignant lymphoma, resulting in fatal outcomes. However, the prognostic factors remain unclear. Objective: This study aimed to evaluate the clinical characteristics of HVLPD and the association between whole blood EBV DNA and clinical outcomes. Methods: We retrospectively reviewed our 25-year experience involving 11 patients with HVLPD from a single tertiary center in South Korea and evaluated the clinical characteristics of HVLPD and the correlation between whole blood EBV DNA and clinical outcomes. Results: Of the total 11 patients, 54.5% (6/11) manifested classic HVLPD that resolved with conservative treatment, while 45.5% (5/11) patients had systemic HVLPD, four of whom died of progressive disease or hemophagocytic syndrome. Five patients with systemic HVLPD manifested severe skin lesions such as prominent facial edema, deep ulcers and necrotic skin lesions involving sun-protected areas. Median EBV DNA levels at initial diagnosis were higher in three dead patients than in those alive (2,290 vs. 186.62 copies/μl). Conclusion When EBV DNA levels were high, patients showed severe skin lesions and when EBV DNA levels were low, skin lesions tended to improve. Thus, patients with high EBV DNA levels showed an increased risk of severe skin lesions and disease progression.

A capillary hemangioma is a vascular tumor with small capillary sized vascular channel. Multiple capillary hemangioma in relation with drugs have been rarely reported. Here in, we report a case of multiple capillary hemangioma in patient diagnosed with chronic myeloid leukemia who received tyrosine kinase inhibitors (TKIs). Histopathological findings have shown capillary proliferation in the upper dermis, which is consistent with capillary hemangioma. Since TKIs can paradoxically activate the MEK/ERK pathway which is required for angiogenesis, we presumed that the lesions as the cutaneous side effects of TKIs.

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is rare Epstein-Barr virus (EBV)-associated disease. The classic form of HVLPD is a self-resolving disease, whereas the systemic form can progress to malignant lymphoma, resulting in fatal outcomes. However, the prognostic factors remain unclear. Objective: This study aimed to evaluate the clinical characteristics of HVLPD and the association between whole blood EBV DNA and clinical outcomes. Methods: We retrospectively reviewed our 25-year experience involving 11 patients with HVLPD from a single tertiary center in South Korea and evaluated the clinical characteristics of HVLPD and the correlation between whole blood EBV DNA and clinical outcomes. Results: Of the total 11 patients, 54.5% (6/11) manifested classic HVLPD that resolved with conservative treatment, while 45.5% (5/11) patients had systemic HVLPD, four of whom died of progressive disease or hemophagocytic syndrome. Five patients with systemic HVLPD manifested severe skin lesions such as prominent facial edema, deep ulcers and necrotic skin lesions involving sun-protected areas. Median EBV DNA levels at initial diagnosis were higher in three dead patients than in those alive (2,290 vs. 186.62 copies/μl). Conclusion When EBV DNA levels were high, patients showed severe skin lesions and when EBV DNA levels were low, skin lesions tended to improve. Thus, patients with high EBV DNA levels showed an increased risk of severe skin lesions and disease progression.

Bullous pemphigoid (BP) is a chronic and recurrent bullous disorder that may be associated with the administration of certain drugs. Recently, bullous cutaneous adverse events after immunotherapy (IT) or targeted therapy have been increasingly reported. Here, we report a case of BP in a patient diagnosed with metastatic melanoma after treatment with pembrolizumab, dabrafenib, and trametinib. Histopathological examination showed a subepidermal blister with perivascular lymphocytic and eosinophilic infiltration; the accompanying findings of linear immunoglobulin G and C3 deposition by immunofluorescence microscopy were consistent with BP. Since IT agents may initiate immune dysregulation and pathologic autoantibody production, which are required for the pathogenesis of BP, the lesions were thought to be cutaneous adverse events caused by IT.