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Article: Characteristics of terminal hemimelia: What is the difference between terminal hemimelia and classic fibular hemimelia?

Song, Mi Hyun / Shin, Chang-Ho / Choi, In Ho / Cho, Tae-Joon

2024 Volume 18, Issue 2, Page(s) 179–186

Abstract: Purpose: Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to ... ...

Abstract	Purpose: Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia. Methods: In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared. Results: Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years. Conclusion: Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening. Level of evidence: level IV.
Language	English
Publishing date	2024-01-30
Publishing country	England
Document type	Journal Article
ZDB-ID	2279410-4
ISSN	1863-2548 ; 1863-2521
ISSN (online)	1863-2548
ISSN	1863-2521
DOI	10.1177/18632521241227830
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Article: Elucidation of the Interactions of Reactive Oxygen Species and Antioxidants in Model Membranes Mimicking Cancer Cells and Normal Cells.

Cho, Geonho / Lee, Deborah / Kim, Sun Min / Jeon, Tae-Joon

Membranes

2022 Volume 12, Issue 3

Abstract: Photosensitizers (PSs) used in photodynamic therapy (PDT) have been developed to selectively destroy tumor cells. However, PSs recurrently reside on the extracellular matrix or affect normal cells in the vicinity, causing side effects. Additionally, the ... ...

Abstract	Photosensitizers (PSs) used in photodynamic therapy (PDT) have been developed to selectively destroy tumor cells. However, PSs recurrently reside on the extracellular matrix or affect normal cells in the vicinity, causing side effects. Additionally, the membrane stability of tumor cells and normal cells in the presence of reactive oxygen species (ROS) has not been studied, and the effects of ROS at the membrane level are unclear. In this work, we elucidate the stabilities of model membranes mimicking tumor cells and normal cells in the presence of ROS. The model membranes are constructed according to the degree of saturation in lipids and the bilayers are prepared either in symmetric or asymmetric form. Interestingly, membranes mimicking normal cells are the most vulnerable to ROS, while membranes mimicking tumor cells remain relatively stable. The instability of normal cell membranes may be one cause of the side effects of PDT. Moreover, we also show that ROS levels are controlled by antioxidants, helping to maintain an appropriate amount of ROS when PDT is applied.
Language	English
Publishing date	2022-03-01
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2614641-1
ISSN	2077-0375
ISSN	2077-0375
DOI	10.3390/membranes12030286
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Article ; Online: The role of

Shin, Chang Ho / Whi, Wonseok / Cho, Yoon Joo / Yoo, Won Joon / Choi, In Ho / Cheon, Gi Jeong / Cho, Tae-Joon

BMC musculoskeletal disorders

2023 Volume 24, Issue 1, Page(s) 668

Abstract: Backgrounds: Determining the precise localization of diseased physes is crucial for guiding the treatment of growth disturbances. Conventional radiography, computed tomography (CT), and magnetic resonance imaging only provide information on physeal ... ...

Abstract	Backgrounds: Determining the precise localization of diseased physes is crucial for guiding the treatment of growth disturbances. Conventional radiography, computed tomography (CT), and magnetic resonance imaging only provide information on physeal anatomy. Planar bone scintigraphy and bone single-photon emission computed tomography (SPECT) resolutions are suboptimal for clinically managing growth disturbances. Bone SPECT/CT, which provides high-resolution functional information, can be a useful tool for evaluating growth disturbances. The purposes of this study were to identify the conditions in which bone SPECT/CT outperforms planar scintigraphy or SPECT for evaluating the location and activity of diseased physes and to assess surgical outcomes using bone SPECT/CT findings in pediatric patients experiencing long bone growth disturbances. Methods: Fifty-nine patients who underwent bone SPECT/CT between January 2018 and January 2021 to evaluate physeal activity using technetium-99 m-labeled 2,3-dicarboxypropane-1,1-diphosphonate ( Results: Bone SPECT/CT was sufficient for selecting a treatment plan, but planar scintigraphy or SPECT alone was insufficient in every 10 patients with diseased physes inside the femoral head (p = 0.002) and in every six with physes that were severely deformed or whose locations were unclear on conventional radiography (p = 0.03). In the proximal or distal tibia, where the tibial and fibular physes often overlapped on planar scintigraphy due to leg rotation, bone SPECT/CT was sufficient in 33/34 patients (97%), but planar scintigraphy and SPECT were sufficient in 10/34 (29%) (p < 0.001) and 24/34 (71%) patients, respectively (p = 0.004). No progression or deformity recurrence occurred. Conclusions: Bone SPECT/CT may be indicated in proximal femoral growth disturbance, when the physis is unclear on conventional radiography or severely deformed, the leg exhibits rotational deformity, or the patient is noncompliant.
MeSH term(s)	Humans ; Child ; Single Photon Emission Computed Tomography Computed Tomography ; Tomography, Emission-Computed, Single-Photon ; Tomography, X-Ray Computed ; Bone Development ; Diphosphonates/therapeutic use
Chemical Substances	Diphosphonates
Language	English
Publishing date	2023-08-24
Publishing country	England
Document type	Observational Study ; Journal Article
ZDB-ID	2041355-5
ISSN	1471-2474 ; 1471-2474
ISSN (online)	1471-2474
ISSN	1471-2474
DOI	10.1186/s12891-023-06777-0
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Acute correction of proximal tibial coronal plane deformity in small children using a small monolateral external fixator with or without cross-pinning.

Lim, Chaemoon / Shin, Chang Ho / Yoo, Won Joon / Cho, Tae-Joon

Journal of children's orthopaedics

2021 Volume 15, Issue 3, Page(s) 255–260

Abstract: Purpose: Surgical correction of proximal tibia deformity in small children can be challenging. We present the surgical technique and outcome of proximal tibia osteotomy fixed with small monolateral external fixator in this patient group.: Methods: A ... ...

Abstract	Purpose: Surgical correction of proximal tibia deformity in small children can be challenging. We present the surgical technique and outcome of proximal tibia osteotomy fixed with small monolateral external fixator in this patient group. Methods: A total of 17 cases in eight patients younger than nine years of age were study subjects. A proximal tibia osteotomy was fixed with a small monolateral external fixator with or without cross-pinning. Outcome was evaluated by changes of radiographic parameters such as medial proximal tibia angle (MPTA), metaphyseal diaphyseal angle (MDA) and clinical findings of complications, time interval until weight bearing and fixator removal time. Results: MPTA improved from a preoperative mean of 73° (sd 4°; 66° to 78°) to an immediate postoperative mean of 90° (sd 3°; 85° to 96°) in varus tibiae, and from 104° (sd 1°; 103° to 105°) to 89° (sd 1°; 88° to 89°) in valgus tibiae. In all, 15 of the 17 cases (88.3 %) achieved postoperative MPTA within the normal range (85° to 90°). MDA improved from a preoperative mean of 19° (sd 5°; 11° to 24°) to an immediate postoperative mean of 0° (sd 4°; -6° to 7°) in varus tibiae, and from -25° (sd 2°; -22° to -24°) to 2° (SD 1°; 1° to 3°) in valgus tibiae. Full weight bearing was possible at mean 1.7 months (0.5 to 3.0). Mean follow-up period was 6.5 years (sd 5.4; 1.0 to 16.0). No complications developed during the follow-up. Conclusion: Proximal tibia osteotomy fixed with small monolateral external fixator provides accurate, safe and efficient correction in the management of coronal plane angular deformity in small children. Level of evidence: Level IV.
Language	English
Publishing date	2021-06-18
Publishing country	England
Document type	Journal Article
ZDB-ID	2279410-4
ISSN	1863-2548 ; 1863-2521
ISSN (online)	1863-2548
ISSN	1863-2521
DOI	10.1302/1863-2548.15.200187
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Article: Clinical characteristics and effects of enzyme replacement therapy with elosulfase alfa in Korean patients with mucopolysaccharidosis type IVA.

Lee, Seung Hoon / Kim, Hwa Young / Cho, Tae-Joon / Kim, Hyoungmin / Ko, Jung Min

Molecular genetics and metabolism reports

2022 Volume 31, Page(s) 100869

Abstract: Mucopolysaccharidosis type IVA (MPS IVA) is a rare autosomal recessive disorder caused by a deficiency ... ...

Abstract	Mucopolysaccharidosis type IVA (MPS IVA) is a rare autosomal recessive disorder caused by a deficiency in
Language	English
Publishing date	2022-04-15
Publishing country	United States
Document type	Journal Article
ZDB-ID	2821908-9
ISSN	2214-4269
ISSN	2214-4269
DOI	10.1016/j.ymgmr.2022.100869
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Article: Clinical and radiological outcomes of surgically treated osteochondral lesions of the talus in children and adolescents.

Kim, Dae-Yoo / Park, JiSu / Kang, Ho Won / Shin, Chang Ho / Lee, Dong Yeon / Cho, Tae-Joon / Yoo, Won Joon

Journal of children's orthopaedics

2023 Volume 17, Issue 3, Page(s) 224–231

Abstract: Background: Osteochondral lesions of the talus are uncommon in children and adolescents. Surgical procedures differ from those used for adults to avoid iatrogenic physeal injuries. This study aimed to evaluate the clinical and radiological outcomes of ... ...

Abstract	Background: Osteochondral lesions of the talus are uncommon in children and adolescents. Surgical procedures differ from those used for adults to avoid iatrogenic physeal injuries. This study aimed to evaluate the clinical and radiological outcomes of surgical treatment in pediatric patients with osteochondral lesions, specifically investigating the patient age and the status of distal tibial physis as factors associated with surgical success. Methods: We retrospectively reviewed 28 patients who had symptomatic osteochondral lesions of the talus that were treated surgically between 2003 and 2016. If the lesion was stable and articular cartilage was intact, retrograde drilling was performed under fluoroscopic guidance. Lesions with detached overlying cartilages were treated by debridement of the cartilage combined with microfracture and drilling. Radiographic outcomes, American Orthopaedic Foot & Ankle Society ankle-hindfoot score, and skeletal maturity were evaluated. Results: Radiological improvement was observed in 24 (24/28, 86%) patients and complete and incomplete healing in 8 and 16 patients, respectively. Changes in pain grades, American Orthopaedic Foot & Ankle Society scores, and radiological healing after surgery were significant (pain grade, p < 0.001; American Orthopaedic Foot & Ankle Society, p = 0.018; radiological healing, p < 0.001). In addition, patients in the younger age group (≤13 years) showed greater improvements in pain grades than older patients (p = 0.02). Improvement in pain grade after surgery was better in the skeletally immature group than in the skeletally mature group (p = 0.048). Conclusion: Clinical and radiological improvements were observed after surgical treatment. The younger age group and open physis group showed more pain improvement. Level of evidence: Therapeutic level IV.
Language	English
Publishing date	2023-05-16
Publishing country	England
Document type	Journal Article
ZDB-ID	2279410-4
ISSN	1863-2548 ; 1863-2521
ISSN (online)	1863-2548
ISSN	1863-2521
DOI	10.1177/18632521231152277
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Article ; Online: Articulated Hip Distraction for Impingement of the Deformed Femoral Head in a Patient with Multiple Epiphyseal Dysplasia: A Case Report.

Jang, Woo Young / Cho, Tae-Joon

JBJS case connector

2018 Volume 8, Issue 3, Page(s) e52

Abstract: Case: A 14-year-old boy with multiple epiphyseal dysplasia (MED) presented with right hip pain and stiffness. Radiographs revealed a deep notch at the superolateral part of the femoral head, which abutted the lateral corner of the acetabulum. ... ...

Abstract	Case: A 14-year-old boy with multiple epiphyseal dysplasia (MED) presented with right hip pain and stiffness. Radiographs revealed a deep notch at the superolateral part of the femoral head, which abutted the lateral corner of the acetabulum. Articulated hip distraction (AHD) was used to reduce the femoral head and obliterate the notch. At the 4-year follow-up, the radiographs showed a well-preserved joint space, and the patient had functional improvement. Conclusion: AHD may be an effective way to address and obliterate a superolateral femoral head notch and promote regeneration of the femoral head in a patient with MED.
MeSH term(s)	Adolescent ; Humans ; Male ; Orthopedic Procedures/methods ; Osteochondrodysplasias/diagnostic imaging ; Osteochondrodysplasias/surgery
Language	English
Publishing date	2018-07-11
Publishing country	United States
Document type	Case Reports ; Journal Article
ISSN	2160-3251
ISSN (online)	2160-3251
DOI	10.2106/JBJS.CC.17.00328
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Article ; Online: When to Combine Acetabular Osteotomy in Patients With Proximal Femoral Deformity Causing Residual Hip Dysplasia/Subluxation After Reduction of Developmental Dysplasia of the Hip.

Cho, Yoon Joo / Chae, Ihn Seok / Song, Mi Hyun / Shin, Chang Ho / Chung, Chin Youb / Choi, In Ho / Cho, Tae-Joon

Journal of pediatric orthopedics

2023 Volume 43, Issue 9, Page(s) 560–566

Abstract: Background: After the successful reduction of developmental dysplasia of the hip, residual hip dysplasia may persist and lead to early osteoarthritis. Femoral and/or acetabular osteotomy has been used to address this problem. The purpose of this study ... ...

Abstract	Background: After the successful reduction of developmental dysplasia of the hip, residual hip dysplasia may persist and lead to early osteoarthritis. Femoral and/or acetabular osteotomy has been used to address this problem. The purpose of this study is to determine the indication of femoral versus combined femoral-acetabular osteotomy in the management of residual hip dysplasia. Methods: Fifty-five patients with unilateral dislocated-type dysplasia of the hip, who had residual hip dysplasia after reduction, underwent femoral osteotomy with or without acetabular osteotomy before 8 years of age, and were followed for more than 2 years and over 8 years of age, were the subjects of this retrospective study. Twenty-eight patients underwent femoral osteotomy only at a median age of 34 months (group F), and 27 underwent combined femoral-Dega osteotomy at a median age of 49 months (group C). Seventeen patients in group F and 4 in group C had an additional osteotomy due to persistent hip dysplasia. Acetabular index (AI), lateral center-edge angle, and center-head distance difference were measured on serial radiographs. The z-value of AI (Z AI ) was calculated. At the latest follow-up, patients in group F with Severin I/II who did not have an additional osteotomy were considered satisfactory, and patients with Severin III/IV or those who had an additional osteotomy were considered unsatisfactory. Preoperative variables were tested for the difference between satisfactory and unsatisfactory cases. Receiver operating characteristic analysis was performed to delineate a cutoff value of a significant parameter dividing the outcome. Results: AI and Z AI before index osteotomy were significant parameters predicting a satisfactory outcome in group F. Receiver operating characteristic analysis returned a cutoff value of Z AI 2.6 (Area Under the Curve=0.86, P =0.001). Eight of 12 cases (66.7%) with Z AI <2.6 in group F achieved a satisfactory outcome, whereas only 2 of 14 cases with Z AI ≥2.6 in group F did ( P =0.02). Conclusion: Z AI 2.6 may serve as a threshold to combine acetabular osteotomy with femoral osteotomy in the management of residual hip dysplasia before 8 years of age. Level of evidence: Therapeutic III.
MeSH term(s)	Humans ; Child, Preschool ; Hip Dislocation, Congenital/diagnostic imaging ; Hip Dislocation, Congenital/surgery ; Hip Dislocation/diagnostic imaging ; Hip Dislocation/etiology ; Hip Dislocation/surgery ; Retrospective Studies ; Developmental Dysplasia of the Hip/surgery ; Acetabulum/diagnostic imaging ; Acetabulum/surgery ; Osteotomy ; Treatment Outcome ; Hip Joint/surgery
Language	English
Publishing date	2023-07-27
Publishing country	United States
Document type	Journal Article
ZDB-ID	604642-3
ISSN	1539-2570 ; 0271-6798
ISSN (online)	1539-2570
ISSN	0271-6798
DOI	10.1097/BPO.0000000000002476
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Article ; Online: Elucidating the Molecular Interactions between Lipids and Lysozyme: Evaporation Resistance and Bacterial Barriers for Dry Eye Disease.

Lee, Deborah / Song, Seoyoon / Cho, Geonho / Dalle Ore, Lucia C / Malmstadt, Noah / Fuwad, Ahmed / Kim, Sun Min / Jeon, Tae-Joon

Nano letters

2023 Volume 23, Issue 20, Page(s) 9451–9460

Abstract: Dry eye disease (DED) is a chronic condition characterized by ocular dryness and inflammation. The tear film lipid layer (TFLL) is the outermost layer composed of lipids and proteins that protect the ocular surface. However, environmental contaminants ... ...

Abstract	Dry eye disease (DED) is a chronic condition characterized by ocular dryness and inflammation. The tear film lipid layer (TFLL) is the outermost layer composed of lipids and proteins that protect the ocular surface. However, environmental contaminants can disrupt its structure, potentially leading to DED. Although the importance of tear proteins in the TFLL functionality has been clinically recognized, the molecular mechanisms underlying TFLL-protein interactions remain unclear. In this study, we investigated tear protein-lipid interactions and analyzed their role in the TFLL functionality. The results show that lysozyme (LYZ) increases the stability of the TFLL by reducing its surface tension and increasing its surface pressure, resulting in increased TFLL evaporation and bacterial invasion resistance, with improved wettability and lubrication performance. These findings highlight the critical role of LYZ in maintaining ocular health and provide potential avenues for investigating novel approaches to DED treatment and patient well-being.
MeSH term(s)	Humans ; Lipids/chemistry ; Muramidase ; Dry Eye Syndromes/drug therapy ; Dry Eye Syndromes/metabolism ; Physical Phenomena ; Tears/chemistry ; Tears/metabolism
Chemical Substances	Lipids ; Muramidase (EC 3.2.1.17)
Language	English
Publishing date	2023-10-16
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1530-6992
ISSN (online)	1530-6992
DOI	10.1021/acs.nanolett.3c02936
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Clinical Manifestations and Outcomes of 20 Korean Hypochondroplasia Patients with the FGFR3 N540K variant.

Kim, Hwa Young / Lee, Young Ah / Shin, Choong Ho / Cho, Tae-Joon / Ko, Jung Min

Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association

2022 Volume 131, Issue 3, Page(s) 123–131

Abstract: Background: Hypochondroplasia is a skeletal dysplasia caused by activating pathologic variants of : Methods: Medical records of 20 unrelated patients with genetically confirmed N540K-related hypochondroplasia were retrospectively reviewed. All ... ...

Abstract	Background: Hypochondroplasia is a skeletal dysplasia caused by activating pathologic variants of Methods: Medical records of 20 unrelated patients with genetically confirmed N540K-related hypochondroplasia were retrospectively reviewed. All individuals were diagnosed with hypochondroplasia by Sanger sequencing for Results: Among 20 patients (7 men, 13 women), the mean age at first visit was 3.5±1.0 years, and the mean follow-up duration was 6.8±0.6 years. The patients presented with a short stature and/or short limbs. Genu varum, macrocephaly, and developmental delay were observed in 11 (55.0%), 9 (45.0%), and 5 (25.0%) patients, respectively. Of the 12 patients who underwent neuroimaging, five (41.7%) showed abnormal findings (one required operation for obstructive hydrocephalus). Among 16 growth-hormone-treated patients (two were growth-hormone deficient), the increase in height standard deviation scores was significant after a mean 5.4±0.7 years of treatment (+0.6 and+1.8 using growth references for healthy controls and achondroplasia children, respectively). Four patients underwent surgical limb lengthening at a mean age of 8.8±3.3 years. Conclusions: Neurodevelopmental abnormalities are frequently observed in patients with N540K-related hypochondroplasia. Close monitoring of skeletal manifestations and neurodevelopmental status is necessary for hypochondroplasia.
MeSH term(s)	Male ; Child ; Humans ; Female ; Child, Preschool ; Retrospective Studies ; Achondroplasia/drug therapy ; Achondroplasia/genetics ; Achondroplasia/diagnosis ; Osteochondrodysplasias/genetics ; Human Growth Hormone ; Republic of Korea ; Mutation ; Receptor, Fibroblast Growth Factor, Type 3/genetics
Chemical Substances	Human Growth Hormone (12629-01-5) ; FGFR3 protein, human (EC 2.7.10.1) ; Receptor, Fibroblast Growth Factor, Type 3 (EC 2.7.10.1)
Language	English
Publishing date	2022-11-28
Publishing country	Germany
Document type	Journal Article
ZDB-ID	1225416-2
ISSN	1439-3646 ; 0947-7349
ISSN (online)	1439-3646
ISSN	0947-7349
DOI	10.1055/a-1988-9734
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