Purpose: We present the case of a 4-month-old boy with a large orbital cyst that caused lateral displacement of the optic nerve resulting in progressive exophthalmos of the left eye since birth.Case summary: A 4-month-old boy presented with progressive exophthalmos of the left eye since birth. Physical examination revealed marked left eye proptosis. Magnetic resonance imaging revealed microphthalmia and a 2.2 × 1.9 cm orbital cyst in the retrobulbar region. Subsequently, an anterior orbitotomy of the left eye was performed to dissect and excise the cyst. Histopathological examination revealed an orbital cyst containing neuroectodermal tissue. No complications or recurrences were observed during the 3-month post-surgery period. Conclusions Congenital microphthalmia with orbital cysts is a rare condition that does not always require surgical intervention. However, timely surgical intervention in cases of severe exophthalmos can result in favorable functional and cosmetic outcomes. We present a case that exhibited significant improvement following surgical removal of the orbital cyst.

OBJECTIVES: Restless legs syndrome (RLS) is a sleep disorder characterized by uncomfortable and unpleasant sensations in the legs and an urge to move the legs, usually at night. The aim of this study is to investigate the incidence of RLS in patients with late life depression and its influence on various clinical outcomes such as severity of depression, sleep quality, cognitive function, and quality of life and accordingly, to elucidate the clinical significance of RLS in patients with late life depression (LLD). METHODS: This study enlisted 170 depressive patients aged 65 years or older from an outpatient clinic. Structured diagnostic interviews were performed using the Korean version of the Mini-International Neuropsychiatric Interview. All patients completed the questionnaires, including the International RLS Severity Scale, the Korean version of Short-Form 36-Item Health Survey (SF-36), and the Pittsburgh Sleep Quality Index (PSQI). The severity of depression was evaluated by the Korean form of the Geriatric Depression Scale (KGDS) and the level of global cognition was assessed by the Mini-Mental State Examination in the Korean version of The Consortium to Establish a Registry for Alzheimer's Disease Assessment Packet (MMSE-KC). RESULTS: The incidence of RLS was 17.6% in LLD patients. RLS was more prevalent among the subjects with major depressive disorder (MDD) than those with minor depressive disorder or subsyndromal depressive disorder. The RLS group showed higher score in the KGDS than the Non-RLS group but the difference did not reach the statistical significance (p = 0.095, Student t-test). The mean PSQI score was significantly higher in the RLS group than in the Non-RLS group (p = 0.001, Student t-test). The MMSE-KC score was also lower in the RLS group than in Non-RLS group (p = 0.009, analysis of covariance). But, there was no difference in the score of SF-36 between the RLS group and the Non-RLS group. CONCLUSIONS: RLS is common in LLD patients, especially in the patients with MDD and is associated with poor sleep quality and cognitive dysfunction, indicating that RLS is clinically significant in patients with LLD. Therefore, RLS should be considered as an important clinical issue in the management of LLD.

Purpose: We report an endocrine mucin-producing sweat gland carcinoma diagnosed by biopsy in a patient who presented with an eyelid mass.Case summary: A 64-year-old male presented with a 3 × 3 mm solitary, painless pinkish mass on the right lower eyelid that had developed over the past year. The mass was excised and a biopsy was performed. The pathological findings included basaloid nodules composed of cells with eosinophilic cytoplasm, focal mucin production, and occasional glandular structures. Immunohistochemical examination was positive for cytokeratin 7 (CK-7), tumor protein 63 (P63), and the androgen receptor (AR). The patient was thus diagnosed with an endocrine mucin-producing sweat gland carcinoma. The lesion healed, and there has been no sign of recurrence over 6 months of follow-up. Conclusions An endocrine mucin-producing sweat gland carcinoma is a very rare low-grade glandular malignancy that has not been reported in Korea previously. The prognosis is good (i.e., there is no recurrence) when the lesion is completely surgically excised. We thought it would be useful to report this very rare case.

Purpose: To document changes in the optical coherence biometry data over 1 year of Korean children aged 6 to 9 years with myopia and hyperopia. Methods: We used fluorescein angiography to retrospectively study changes in refractive errors over 1 year in 60 children who visited our hospital between December 2019 and June 2022. The IOL master 700 platform was used to derive axial length, corneal curvature (the K value), central corneal thickness, anterior chamber depth, lens thickness, and the white-to-white size at 1-year intervals. The Mann-Whitney and Wilcoxon signed-ranked tests were used to compare the values. A p-value < 0.05 was taken to indicate a significant difference. Results: The mean ages of hyperopic and myopic children were 6.27 ± 0.87 and 6.93 ± 0.87 years at initial presentation. Significant 1-year changes in axial length, anterior depth, and lens thickness were apparent. The mean corneal curvature, and central corneal and lens thicknesses were significantly higher in hyperopic than myopic children but the axial length and anterior chamber depth were significantly greater in myopic children. However, after 1 year, the lens thicknesses did not significantly differ between the two groups. Conclusions Over 1 year of early life, changes in the mean corneal curvature and lens thickness were significant in hyperopic children and changes in the axial length and anterior depth were significant in myopic children. Thus, the eye growth pattern may differ between hyperopic and myopic children.

Purpose: To compare the short-term clinical outcomes (up to 3 months) after three different secondary intraocular lens (IOL) implantations in dislocated and aphakic eyes with insufficient capsular support. Methods: We retrospectively analyzed the medical records of 97 patients who underwent secondary IOL implantation (32 eyes with sutured scleral fixation, 21 with sutureless scleral fixation, and 44 with retropupillary IOL implantation) from March to December 2018. Uncorrected visual acuity (UCVA), and best-corrected visual acuity (BCVA), spherical equivalent (SE), prediction error (PE), mean absolute error (MAE), surgically induced astigmatism (SIA), ocular residual astigmatism (ORA), and complications in the three groups were assessed before and 1 week and 1 and 3 months after surgery. Results: All groups had an improved BCVA beginning 1 month after surgery. The pre- and postoperative SE (p = 0.857, p = 0.263, and p = 0.163) and PE (p = 0.479, p = 0.848, and p = 0.128) did not differ in the sutured scleral fixation, sutureless scleral fixation, and retropupillary IOL implantation groups, respectively; MAE differed significantly among the procedures 1 week after surgery (1.33 ± 1.25, 1.40 ± 1.54, and 0.85 ± 1.25, p = 0.044), but not 1 month after surgery (p = 0.965, p = 0.731). 3 months after surgery, there was no significant difference in SIA (p = 0.140) or ORA (p = 0.178) among the 3 groups. As a complication, intraocular pressure rise occurred more often in the sutured fixated group, while the retropupillary group had a higher dislocation rate. Conclusions There was no significant difference in the SE, PE, MAE, SIA, ORA, or complications among the three procedures. Surgical skill is still required to minimize the chance of complications regardless of the implantation method.

Distal renal tubular acidosis (RTA) is characterized by a decreased net H+ secretion in the collecting tubules, which results in a failure of urine acidification and results in metabolic acidosis, hypokalemia, and nephrocalcinosis. The acquired form of distal RTA is associated with tubulointerstitial involvement of immune-mediated disorders such as Sjogren's syndrome and systemic lupus erythematosus (SLE). Only a few case reports have indicated that distal RTA precedes SLE by months to years. We present a 39-year-old woman who had manifestations of distal RTA for 21 years before the development of overt symptoms of SLE.
Acidosis ; Acidosis, Renal Tubular ; Adult ; Female ; Humans ; Hypokalemia ; Lupus Erythematosus, Systemic ; Nephrocalcinosis ; Sjogren's Syndrome

Livedoid vasculitis (LV) is a chronic recurrent cutaneous disease that is characterized by a netlike vascular pattern of purpuric lesions that progress to ulcers and atrophic white scars. The etiology of LV is unknown. Various therapies have been used for LV with inconsistent results, including antiplatelet agents, fibrinolytics, anticoagulants, prostaglandin E1, and immunosuppressive agents, such as systemic corticosteroids, azathioprine, methotrexate, cyclosporine, and mycophenolate mofetil. We present a patient with idiopathic LV refractory to immunosuppressive therapy who had a good response to treatment with rituximab and cyclophosphamide.
Adrenal Cortex Hormones ; Alprostadil ; Antibodies, Monoclonal, Murine-Derived ; Anticoagulants ; Azathioprine ; Cicatrix ; Cyclophosphamide ; Cyclosporine ; Humans ; Immunosuppressive Agents ; Methotrexate ; Mycophenolic Acid ; Platelet Aggregation Inhibitors ; Ulcer ; Vasculitis ; Rituximab

OBJECTIVE: This study sought to investigate independent predictive factors for subclinical atherosclerosis in Korean patients with rheumatoid arthritis (RA). METHODS: We used high-resolution B-mode ultrasonography to measure the carotid artery intima-media thickness (IMT) and carotid plaque in 367 patients with RA. Detailed information on the demographic characteristics, cardiovascular (CV) risk factors, and RA disease characteristics were collected on all subjects. The relationship of the carotid artery IMT and carotid plaque to relevant clinical and laboratory variables were examined. RESULTS: Old age and male sex had the most significant association with increased IMT and presence of plaque than other factors. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and mKHAQ (Korean version of modified health assessment questionnaire) were significantly associated with both increased IMT and presence of plaque after univariate analysis adjusting for age and sex. A multivariable logistic regression analysis revealed that ESR and TJC68 were independent factors associated with the presence of plaque (p<0.001 and p=0.019, respectively). There was a significant linear correlation between the number of plaques and ESR (p<0.001 and R2=0.07). CONCLUSION: Our results indicated that markers of systemic inflammation contributed significantly to subclinical atherosclerosis in patients with RA. We emphasize the need for aggressive control of RA disease activity in patients who persistently demonstrate highly elevated ESR levels.
Arthritis, Rheumatoid ; Atherosclerosis ; Blood Sedimentation ; C-Reactive Protein ; Cardiovascular Diseases ; Carotid Arteries ; Cohort Studies ; Humans ; Inflammation ; Logistic Models ; Male ; Risk Factors

OBJECTIVE: betaig-h3 is a 68kDa extracellular matrix protein which is overexpressed in synovial tissues of rheumatoid arthritis (RA). Previous results proved that betaig-h3 fragments are relevant to adhesion and migration of synovial fibroblast and angiogenesis through interaction with alphavbeta 3 integrin. We designed a recombinant betaig-h3 protein consisting of a fas-1 domain and RGD motif and evaluated the therapeutic efficacy in RA. METHODS: Inhibitory effect of adhesion and migration of NIH3T3 cell line was evaluated in 96 well microtiter and transwell plates coated with betaig-h3. Clinical arthritis index was evaluated after treating CIA mice with MFK12. Immunohistochemical staining in synovial tissues were performed. Expression of transcripts and proteins of inflammatory mediators were analyzed by semi-quantitative RT-PCR and immunoblotting. RESULTS: Recombinant protein consisted of 4th fas-1 domain truncated for H1 and H2 sequences and RGD peptide (MFK12), had M.W. of 10.4kDa. betaig-h3 mediated adhesion and migration of NIH3T3 cell line were significantly inhibited in a dose-dependent manner. Arthritis severity and incidence were efficiently reduced when CIA mice were treated with MFK12 at 30 mg/kg/day compared with the control. Immunohistochemical staining of joint tissues in MFK12 treated mice exhibited reduced angiogenesis. In treated mice, expression of transcripts regarding inflammatory mediators was markedly suppressed and immunoblotting of ICAM-1 and RANKL from whole extract of hind paws also showed a significant reduction. CONCLUSION: This study shows that MFK12 is effective in treating RA, although further study is warranted to improve the therapeutic efficacy.
Animals ; Arthritis ; Arthritis, Experimental ; Arthritis, Rheumatoid ; Cell Line ; Extracellular Matrix ; Extracellular Matrix Proteins ; Fibroblasts ; Immunoblotting ; Incidence ; Inflammation ; Intercellular Adhesion Molecule-1 ; Joints ; Mice ; Oligopeptides ; Proteins ; Transforming Growth Factor beta

May-Thurner syndrome, which refers to an iliofemoral venous thrombosis caused by chronic compression of the left common iliac vein by the right common iliac artery, usually manifests as left lower extremity pain and swelling. The syndrome is particularly evident in patients with health conditions including obesity, smoking, pregnancy, surgery, or prolonged immobility. Antiphospholipid syndrome consists of arterial or venous thrombosis and the association of lupus anticoagulant or anticardiolipin antibodies. Most common clinical manifestations include pregnancy loss and deep vein thrombosis. We experienced a rare case of May-Thurner syndrome concurrent with antiphospholipid syndrome, secondary to systemic lupus erythematosus. The patient was treated successfully by catheter-directed percutaneous thrombectomy, venous thrombolysis, and stent insertion, followed by oral anticoagulant therapy.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome ; Humans ; Iliac Artery ; Iliac Vein ; Lower Extremity ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; May-Thurner Syndrome ; Obesity ; Postthrombotic Syndrome ; Pregnancy ; Smoke ; Smoking ; Stents ; Thrombectomy ; Venous Thrombosis