1.Regenerative Capacity of Alveolar Type 2 Cells Is Proportionally Reduced Following Disease Progression in Idiopathic Pulmonary Fibrosis-Derived Organoid Cultures
Hyeon Kyu CHOI ; Gaeul BANG ; Ju Hye SHIN ; Mi Hwa SHIN ; Ala WOO ; Song Yee KIM ; Sang Hoon LEE ; Eun Young KIM ; Hyo Sup SHIM ; Young Joo SUH ; Ha Eun KIM ; Jin Gu LEE ; Jinwook CHOI ; Ju Hyeon LEE ; Chul Hoon KIM ; Moo Suk PARK
Tuberculosis and Respiratory Diseases 2025;88(1):130-137
Background:
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease that culminates in respiratory failure and death due to irreversible scarring of the distal lung. While initially considered a chronic inflammatory disorder, the aberrant function of the alveolar epithelium is now acknowledged as playing a central role in the pathophysiology of IPF. This study aimed to investigate the regenerative capacity of alveolar type 2 (AT2) cells using IPF-derived alveolar organoids and to examine the effects of disease progression on this capacity.
Methods:
Lung tissues from three pneumothorax patients and six IPF patients (early and advanced stages) were obtained through video-assisted thoracoscopic surgery and lung transplantation. HTII-280+ cells were isolated from CD31-CD45-epithelial cell adhesion molecule (EpCAM)+ cells in the distal lungs of IPF and pneumothorax patients using fluorescence-activated cell sorting (FACS) and resuspended in 48-well plates to establish IPF-derived alveolar organoids. Immunostaining was used to verify the presence of AT2 cells.
Results:
FACS sorting yielded approximately 1% of AT2 cells in early IPF tissue, and the number decreased as the disease progressed, in contrast to 2.7% in pneumothorax. Additionally, the cultured organoids in the IPF groups were smaller and less numerous compared to those from pneumothorax patients. The colony forming efficiency decreased as the disease advanced. Immunostaining results showed that the IPF organoids expressed less surfactant protein C (SFTPC) compared to the pneumothorax group and contained keratin 5+ (KRT5+) cells.
Conclusion
This study confirmed that the regenerative capacity of AT2 cells in IPF decreases as the disease progresses, with IPF-derived AT2 cells inherently exhibiting functional abnormalities and altered differentiation plasticity.
2.Long-Term Surgical Outcome of Canal-Tympanoplasty in Patients With Schuknecht Type B Congenital Aural Stenosis
Yoonjae LEE ; Moo Kyun PARK ; Jun Ho LEE ; Sang-Yeon LEE ; Myung-Whan SUH
Clinical and Experimental Otorhinolaryngology 2025;18(1):14-20
Objectives:
. Congenital aural atresia (CAA) is a complex condition that manifests in various forms, including Schuknecht type B stenosis, which is characterized by a congenitally narrow bony external auditory canal (EAC). This study aims to evaluate the long-term surgical outcomes of canal-tympanoplasty in patients with Schuknecht type B CAA.
Methods:
. The study included 21 ears diagnosed with Schuknecht type B CAA, all of which underwent canal-tympanoplasty. Reconstruction of the large EAC and tympanic membrane involved conchal flap meatoplasty, bony EAC drilling, ossicular reconstruction, and overlay tympanoplasty. Standard pure-tone audiometry was analyzed at multiple post-surgery time points: 3, 6, 12, and 24–60 months. Additionally, the incidence of postoperative complications was assessed.
Results:
. The hearing outcomes of canal-tympanoplasty in this case series were satisfactory. Serviceable hearing was achieved in all patients after 3 months and was maintained in 85% of patients for 1 year. The air-bone gap improved from a preoperative average of 39.3±15.1 dB to 13.7±7.4 dB at 3 months, 16.4±10.5 dB at 6 months, 19.1±11.7 dB at 12 months, and 21.5±16.1 dB at 2–5 years postoperatively. Three patients required revision canal-tympanoplasty due to hearing deterioration, representing 14.3% of the cases. There were no instances of postoperative infection, facial nerve weakness, vertigo, deterioration of bone conduction thresholds, or complete restenosis of the EAC.
Conclusion
. Canal-tympanoplasty proved to be a satisfactory surgical intervention for patients with Schuknecht type B CAA. This procedure led to significant improvements in hearing outcomes, providing serviceable hearing that lasted for several years. Moreover, the risk of postoperative hearing deterioration and/or the need for revision surgery within 1 year was considered acceptable.
3.Regenerative Capacity of Alveolar Type 2 Cells Is Proportionally Reduced Following Disease Progression in Idiopathic Pulmonary Fibrosis-Derived Organoid Cultures
Hyeon Kyu CHOI ; Gaeul BANG ; Ju Hye SHIN ; Mi Hwa SHIN ; Ala WOO ; Song Yee KIM ; Sang Hoon LEE ; Eun Young KIM ; Hyo Sup SHIM ; Young Joo SUH ; Ha Eun KIM ; Jin Gu LEE ; Jinwook CHOI ; Ju Hyeon LEE ; Chul Hoon KIM ; Moo Suk PARK
Tuberculosis and Respiratory Diseases 2025;88(1):130-137
Background:
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease that culminates in respiratory failure and death due to irreversible scarring of the distal lung. While initially considered a chronic inflammatory disorder, the aberrant function of the alveolar epithelium is now acknowledged as playing a central role in the pathophysiology of IPF. This study aimed to investigate the regenerative capacity of alveolar type 2 (AT2) cells using IPF-derived alveolar organoids and to examine the effects of disease progression on this capacity.
Methods:
Lung tissues from three pneumothorax patients and six IPF patients (early and advanced stages) were obtained through video-assisted thoracoscopic surgery and lung transplantation. HTII-280+ cells were isolated from CD31-CD45-epithelial cell adhesion molecule (EpCAM)+ cells in the distal lungs of IPF and pneumothorax patients using fluorescence-activated cell sorting (FACS) and resuspended in 48-well plates to establish IPF-derived alveolar organoids. Immunostaining was used to verify the presence of AT2 cells.
Results:
FACS sorting yielded approximately 1% of AT2 cells in early IPF tissue, and the number decreased as the disease progressed, in contrast to 2.7% in pneumothorax. Additionally, the cultured organoids in the IPF groups were smaller and less numerous compared to those from pneumothorax patients. The colony forming efficiency decreased as the disease advanced. Immunostaining results showed that the IPF organoids expressed less surfactant protein C (SFTPC) compared to the pneumothorax group and contained keratin 5+ (KRT5+) cells.
Conclusion
This study confirmed that the regenerative capacity of AT2 cells in IPF decreases as the disease progresses, with IPF-derived AT2 cells inherently exhibiting functional abnormalities and altered differentiation plasticity.
4.Long-Term Surgical Outcome of Canal-Tympanoplasty in Patients With Schuknecht Type B Congenital Aural Stenosis
Yoonjae LEE ; Moo Kyun PARK ; Jun Ho LEE ; Sang-Yeon LEE ; Myung-Whan SUH
Clinical and Experimental Otorhinolaryngology 2025;18(1):14-20
Objectives:
. Congenital aural atresia (CAA) is a complex condition that manifests in various forms, including Schuknecht type B stenosis, which is characterized by a congenitally narrow bony external auditory canal (EAC). This study aims to evaluate the long-term surgical outcomes of canal-tympanoplasty in patients with Schuknecht type B CAA.
Methods:
. The study included 21 ears diagnosed with Schuknecht type B CAA, all of which underwent canal-tympanoplasty. Reconstruction of the large EAC and tympanic membrane involved conchal flap meatoplasty, bony EAC drilling, ossicular reconstruction, and overlay tympanoplasty. Standard pure-tone audiometry was analyzed at multiple post-surgery time points: 3, 6, 12, and 24–60 months. Additionally, the incidence of postoperative complications was assessed.
Results:
. The hearing outcomes of canal-tympanoplasty in this case series were satisfactory. Serviceable hearing was achieved in all patients after 3 months and was maintained in 85% of patients for 1 year. The air-bone gap improved from a preoperative average of 39.3±15.1 dB to 13.7±7.4 dB at 3 months, 16.4±10.5 dB at 6 months, 19.1±11.7 dB at 12 months, and 21.5±16.1 dB at 2–5 years postoperatively. Three patients required revision canal-tympanoplasty due to hearing deterioration, representing 14.3% of the cases. There were no instances of postoperative infection, facial nerve weakness, vertigo, deterioration of bone conduction thresholds, or complete restenosis of the EAC.
Conclusion
. Canal-tympanoplasty proved to be a satisfactory surgical intervention for patients with Schuknecht type B CAA. This procedure led to significant improvements in hearing outcomes, providing serviceable hearing that lasted for several years. Moreover, the risk of postoperative hearing deterioration and/or the need for revision surgery within 1 year was considered acceptable.
5.Regenerative Capacity of Alveolar Type 2 Cells Is Proportionally Reduced Following Disease Progression in Idiopathic Pulmonary Fibrosis-Derived Organoid Cultures
Hyeon Kyu CHOI ; Gaeul BANG ; Ju Hye SHIN ; Mi Hwa SHIN ; Ala WOO ; Song Yee KIM ; Sang Hoon LEE ; Eun Young KIM ; Hyo Sup SHIM ; Young Joo SUH ; Ha Eun KIM ; Jin Gu LEE ; Jinwook CHOI ; Ju Hyeon LEE ; Chul Hoon KIM ; Moo Suk PARK
Tuberculosis and Respiratory Diseases 2025;88(1):130-137
Background:
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease that culminates in respiratory failure and death due to irreversible scarring of the distal lung. While initially considered a chronic inflammatory disorder, the aberrant function of the alveolar epithelium is now acknowledged as playing a central role in the pathophysiology of IPF. This study aimed to investigate the regenerative capacity of alveolar type 2 (AT2) cells using IPF-derived alveolar organoids and to examine the effects of disease progression on this capacity.
Methods:
Lung tissues from three pneumothorax patients and six IPF patients (early and advanced stages) were obtained through video-assisted thoracoscopic surgery and lung transplantation. HTII-280+ cells were isolated from CD31-CD45-epithelial cell adhesion molecule (EpCAM)+ cells in the distal lungs of IPF and pneumothorax patients using fluorescence-activated cell sorting (FACS) and resuspended in 48-well plates to establish IPF-derived alveolar organoids. Immunostaining was used to verify the presence of AT2 cells.
Results:
FACS sorting yielded approximately 1% of AT2 cells in early IPF tissue, and the number decreased as the disease progressed, in contrast to 2.7% in pneumothorax. Additionally, the cultured organoids in the IPF groups were smaller and less numerous compared to those from pneumothorax patients. The colony forming efficiency decreased as the disease advanced. Immunostaining results showed that the IPF organoids expressed less surfactant protein C (SFTPC) compared to the pneumothorax group and contained keratin 5+ (KRT5+) cells.
Conclusion
This study confirmed that the regenerative capacity of AT2 cells in IPF decreases as the disease progresses, with IPF-derived AT2 cells inherently exhibiting functional abnormalities and altered differentiation plasticity.
9.Regenerative Capacity of Alveolar Type 2 Cells Is Proportionally Reduced Following Disease Progression in Idiopathic Pulmonary Fibrosis-Derived Organoid Cultures
Hyeon Kyu CHOI ; Gaeul BANG ; Ju Hye SHIN ; Mi Hwa SHIN ; Ala WOO ; Song Yee KIM ; Sang Hoon LEE ; Eun Young KIM ; Hyo Sup SHIM ; Young Joo SUH ; Ha Eun KIM ; Jin Gu LEE ; Jinwook CHOI ; Ju Hyeon LEE ; Chul Hoon KIM ; Moo Suk PARK
Tuberculosis and Respiratory Diseases 2025;88(1):130-137
Background:
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease that culminates in respiratory failure and death due to irreversible scarring of the distal lung. While initially considered a chronic inflammatory disorder, the aberrant function of the alveolar epithelium is now acknowledged as playing a central role in the pathophysiology of IPF. This study aimed to investigate the regenerative capacity of alveolar type 2 (AT2) cells using IPF-derived alveolar organoids and to examine the effects of disease progression on this capacity.
Methods:
Lung tissues from three pneumothorax patients and six IPF patients (early and advanced stages) were obtained through video-assisted thoracoscopic surgery and lung transplantation. HTII-280+ cells were isolated from CD31-CD45-epithelial cell adhesion molecule (EpCAM)+ cells in the distal lungs of IPF and pneumothorax patients using fluorescence-activated cell sorting (FACS) and resuspended in 48-well plates to establish IPF-derived alveolar organoids. Immunostaining was used to verify the presence of AT2 cells.
Results:
FACS sorting yielded approximately 1% of AT2 cells in early IPF tissue, and the number decreased as the disease progressed, in contrast to 2.7% in pneumothorax. Additionally, the cultured organoids in the IPF groups were smaller and less numerous compared to those from pneumothorax patients. The colony forming efficiency decreased as the disease advanced. Immunostaining results showed that the IPF organoids expressed less surfactant protein C (SFTPC) compared to the pneumothorax group and contained keratin 5+ (KRT5+) cells.
Conclusion
This study confirmed that the regenerative capacity of AT2 cells in IPF decreases as the disease progresses, with IPF-derived AT2 cells inherently exhibiting functional abnormalities and altered differentiation plasticity.
10.Long-Term Surgical Outcome of Canal-Tympanoplasty in Patients With Schuknecht Type B Congenital Aural Stenosis
Yoonjae LEE ; Moo Kyun PARK ; Jun Ho LEE ; Sang-Yeon LEE ; Myung-Whan SUH
Clinical and Experimental Otorhinolaryngology 2025;18(1):14-20
Objectives:
. Congenital aural atresia (CAA) is a complex condition that manifests in various forms, including Schuknecht type B stenosis, which is characterized by a congenitally narrow bony external auditory canal (EAC). This study aims to evaluate the long-term surgical outcomes of canal-tympanoplasty in patients with Schuknecht type B CAA.
Methods:
. The study included 21 ears diagnosed with Schuknecht type B CAA, all of which underwent canal-tympanoplasty. Reconstruction of the large EAC and tympanic membrane involved conchal flap meatoplasty, bony EAC drilling, ossicular reconstruction, and overlay tympanoplasty. Standard pure-tone audiometry was analyzed at multiple post-surgery time points: 3, 6, 12, and 24–60 months. Additionally, the incidence of postoperative complications was assessed.
Results:
. The hearing outcomes of canal-tympanoplasty in this case series were satisfactory. Serviceable hearing was achieved in all patients after 3 months and was maintained in 85% of patients for 1 year. The air-bone gap improved from a preoperative average of 39.3±15.1 dB to 13.7±7.4 dB at 3 months, 16.4±10.5 dB at 6 months, 19.1±11.7 dB at 12 months, and 21.5±16.1 dB at 2–5 years postoperatively. Three patients required revision canal-tympanoplasty due to hearing deterioration, representing 14.3% of the cases. There were no instances of postoperative infection, facial nerve weakness, vertigo, deterioration of bone conduction thresholds, or complete restenosis of the EAC.
Conclusion
. Canal-tympanoplasty proved to be a satisfactory surgical intervention for patients with Schuknecht type B CAA. This procedure led to significant improvements in hearing outcomes, providing serviceable hearing that lasted for several years. Moreover, the risk of postoperative hearing deterioration and/or the need for revision surgery within 1 year was considered acceptable.

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