• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.18 no.3
• /
• pp.87-94
• /
• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.

• Journal of the Korean Orthopaedic Association
• /
• v.55 no.3
• /
• pp.253-260
• /
• 2020

Purpose: Soft tissue defects of the distal lower extremity are commonly accompanied by a fracture of the lower extremities. Theses defects are caused by the injury itself or by complications associated with surgical treatment of the fracture, which poses challenging problem. The reverse superficial sural artery flap (RSSAF) is a popular option for these difficult wounds. This paper reviews these cases and reports the clinical results. Materials and Methods: Between August 2003 and April 2018, patients who were treated with RSSAF for soft tissue defects of the lower third of the leg and ankle related to a fracture were reviewed. A total of 16 patients were involved and the mean follow-up period was 18 months. Eight cases (50.0%) of the defects were due to an open fracture, whereas the other eight cases (50.0%) were postoperative complication after closed fracture. The largest flap measured 10×15 cm² and the mean size of the donor sites was 51.9 cm². The flap survival and postoperative complications were evaluated. Results: All flaps survived without complete necrosis or failure. One case with partial necrosis of the flap was encountered, but the wound healed after debridement and repair. One case had a hematoma with a pseudoaneurysmal rupture of the distal tibial artery. On the other hand, the flap was intact and the wound healed after arterial ligation and flap advancement. A debulking operation was performed on three cases for cosmetic reasons and implant removal through the flap was performed in three cases. No flap necrosis was encountered after these additional operations. Conclusion: RSSAF is a relatively simple and safe procedure for reconstructing soft tissue defects following a fracture of the lower extremity that does not require microsurgical anastomosis. This can be a useful treatment option for soft tissue defects on the distal leg, ankle, and foot.

• Journal of the Korean Orthopaedic Association
• /
• v.54 no.1
• /
• pp.52-58
• /
• 2019

Purpose: Fungal periprosthetic joint infection (PJI) is a rare but devastating complication following total knee arthroplasty (TKA). On the other hand, a standardized procedure regarding an accurate treatment of this serious complication of knee arthroplasty is lacking. The clinical progress of staged reimplantation in patients who had fungus-related PJI after TKA was reviewed retrospectively. Materials and Methods: Ten patients who had a fungal related PJI after TKA between 2006 and 2017 using staged reimplantation surgery were reviewed. These patients were compared with 119 patients who had a PJI in the same period. The failure rate of infection control, intravenous antimicrobial using the period, and the clinical results were evaluated by comparing the range of motion and Korean knee score (KKS) between pre-staged reimplantation and the last follow-up. Results: In the fungal infection group, 7 out of 10 cases (70.0%) had failed in infection control using staged reimplantation and in the non-fungal group, 7 out of 119 cases (5.9%) had failed (p=0.04). In the non-fungal group, the mean duration of antibiotics was 6.2 weeks. In the fungus group, the mean duration of antibiotics was 15.3 weeks, which was 9.1 weeks longer (p<0.001). The range of motion of the knee was increased in the two groups (p=0.265). At the last follow-up, the KKS was 71.01 points in the non-fungal group and 61.3 points in the fungal group (p=0.012). Erythrocyte sedimentation rate and C-reactive protein (CRP) decreased in the two groups, but the CRP was significantly different in the two groups (p=0.007). Conclusion: The treatment of fungus-related PJIs using staged reimplantation showed uneven clinical progress and unsatisfactory clinical improvements compared to non-fungal PJI. Therefore, it is necessary to consider the use of an antifungal mixed cement spacer at resection arthroplasty and oral antifungal agent after reimplantation.

• Journal of the Korean Orthopaedic Association
• /
• v.52 no.1
• /
• pp.1-6
• /
• 2017

Purpose: To retrospectively compare minimally invasive percutaneous plate osteosynthesis (MIPPO) with open plate fixation for the treatment of clavicle midshaft fracture. Materials and Methods: Between November 2011 to May 2014, 40 cases that were followed for more than 1 year-among all cases of MIPPO and open plate fixation-were analyzed. The study population was divided into two groups: group A included 20 cases of MIPPO and group B included 20 open plate fixation cases. The comparative analysis between the two groups was based on the operative time, bone union, functional evaluation (American Shoulder and Elbow Society score), incision length, pain relief (visual analogue scale, VAS), and complication. Results: The bone union was successful for all the cases, and the functional evaluation scores of the shoulder joint were satisfactory for both groups (p>0.05). The operative time was 47.5 minutes and 58.7 minutes for group A and B, respectively (p<0.05). The incision length for group A was 6.2 cm and that for group B was 10.7 cm with statistical significance (p<0.05). Pain relief (VAS) after surgery for group A showed a quick recuperation in the early stages. For complications, there were 2 cases of dysesthesia and 1 case of malunion due to metal fixation failure in group A. There were 6 cases of dysesthesia and 2 cases of cosmetic problem due to hypertrophic scar in group B. Conclusion: Surgical results of clavicle shaft fracture were satisfactory for both groups, but group A had advantages of shorter operative time, minimal incision length, and better pain relief in the early stages compared with group B. However, we need further evaluations with long-term follow-up results and complications, such as malunion and exposure on radiation.

• The Journal of Korean Society for Radiation Therapy
• /
• v.24 no.2
• /
• pp.95-106
• /
• 2012

Purpose: The objective of this study was to investigate the usefulness of an IMRT treatment plan according to whether there was a shell-type pseudo target during radiation therapy for patients in Stage III or IV of non-small cell lung cancer (NSCLC). Materials and Methods: After setting an IMRT (Intensity-Modulated Radiation Therapy, IMRT) plan for when there was a shell-type pseudo target (SPT) and when there was none (WSPT) with 22 patients in Stage III or IV of NSCLC, the investigator analyzed dose-volume histograms (DVHs) and made assessment with dosimetric comparisons such as homogeneity index (HI) inside the tumor target, conformity index (CI) of the tumor target, spinal cord maximum dose, Esophagus $V_{50%}$, mean lung dose (MLD), and $V_{40%}$, $V_{30%}$, $V_{20%}$, $V_{10%}$, $V_{5%}$. Results: The mean CI of WSPT and SPT was $1.22{\pm}0.04$ and $1.16{\pm}0.032$ ($.000^*$), respectively, and the mean HI of WSPT and SPT was $1.06{\pm}0.015$ and $1.07{\pm}0.014$ ($.000^*$), respectively. In SPT, the mean of each CI difference decreased by $-5.16{\pm}2.54%$, while HI increased by average $0.81{\pm}0.47%$. Esophagus $V_{50%}$ recorded $14.54{\pm}12.01%$ (WSPT) and $12.14{\pm}11.09%$ ($.000^*$, SPT) with the mean of SPT differences dropping by $-26.37{\pm}25.05%$. Mean spinal cord maximum dose was $3,898.44{\pm}1,075.0$ cGy (WSPT) and $3,810.8{\pm}1,134.9$ cGy ($.004^*$, SPT) with SPT dropping by average $-3.36{\pm}5.81%$. As for lung $V_{X%}$, the mean of $V_{5%}$ and $V_{10%}$ differences was $-1.62{\pm}2.29%$ ($.006^*$) and $-1.98{\pm}5.02%$ ($.005^*$), respectively with SPT making a decrease. The mean of V20%, V30%, and V40% differences was $-3.51{\pm}3.07%$ ($.000^*$), $-4.84{\pm}6.01%$ ($.000^*$), and $-6.16{\pm}8.46%$ ($.001^*$), respectively, with SPT making a decrease with statistical significance. In MLD assessment, SPT also dropped by average $-2.83{\pm}2.41%$ ($.000^*$). Those results show that SPT allows for mean 169 cGy (Max: 547 cGy, Min: 6.4 cGy) prescription dose. Conclusion: An IMRT treatment plan with SPT during radiation therapy for patients in Stage III or IV of NSCLC will help to reduce the risk of lung toxicity and radiation-induced pneumonia by cutting down radiation doses entering the normal lung, reduce the local control failure rate during radiation therapy due to increasing prescription doses to a certain degree, and increase treatment effects.

• Radiation Oncology Journal
• /
• v.24 no.3
• /
• pp.171-178
• /
• 2006

[ $\underline{Purpose}$ ]: To determine the efficacy and safety of concurrent chemotherapy and radiation therapy with high-dose-rate brachytherapy for cervical cancer. $\underline{Materials\;and\;Methods}$: From January 2001 to December 2002, 30 patients with cervical cancer were treated with concurrent chemotherapy (cisplatin and 5-FU) and definitive radiation therapy. The median age was 58 (range $34{\sim}74$) year old. The pathology of the biopsy sections was squamous cell carcinoma in 29 patients and one was adenocarcinoma. The distribution to FIGO staging system was as follows: stage IB, 7 (23%); IIA, 3 (10%); IIB, 12 (40%); IIIA, 3 (10%); IIIB, 5 (17%). All patients received pelvic external beam irradiation (EBRT) to a total dose of $45{\sim}50.4\;Gy$ (median: 50.4 Gy) over $5{\sim}5.5$ weeks. Ir-192 HDR intracavitary brachytherapy (ICBT) was given after a total dose of 41.4 Gy. HDR-ICBT was performed twice a week, with a fraction point A dose of 4 Gy and median dose to point A was 28 Gy (range: $16{\sim}32\;Gy$) in 7 fractions. The median cumulative biologic effective dose (BED) at point A (EBRT+ICBT) was $88\;Gy_{10}$ (range: $77{\sim}94\;Gy_{10}$). The median cumulative BED at ICRU 38 reference point (EBRT+ICBT) was $131\;Gy_3$ (range: $122{\sim}140\;Gy_3$) at point A, $109\;Gy_3$ (range: $88{\sim}125\;Gy_3$) at the rectum and $111\;Gy_3$ (range: $91{\sim}123\;Gy_3$) at the urinary bladder. Cisplatin ($60\;mg/m^2$) and 5-FU ($1,000\;mg/m^2$) was administered intravenously at 3 weeks interval from the first day of radiation for median 5 (range: $2{\sim}6$) cycles. The assessment was performed at 1 month after completion of radiation therapy by clinical examination and CT scan. The median follow-up time was 36 months (range: $8{\sim}50$ months). $\underline{: The complete response rate after concurrent chemoradiation therapy was 93.3%. The 3-yr actuarial pelvic control rate was 87% and 3-yr actuarial overall survival and disease-free survival rate was 93% and 87%, respectively. The local failure rate was 13% and distant metastatic rate was 3.3%. The crude rate of minor hematologic complications (RTOG grade 1-2) occurred in 3 patients (10%) and one patient had suffered from severe leukopenia (RTOG grade 4) during concurrent treatment. Acute minor enterocolitis (RTOG grade 1-2) occurred in 11 patients (37%) and one patient (3%) was suffered from colon perforation during radiation therapy. Late colitis of RTOG grade 1 occurred in 5 patients (15%). Acute cystitis of RTOG grade 1 occurred in 12 patients (40%) and late cystitis of RTOG grade 2 occurred in one patient (3%). No treatment related death was seen. $\underline{Conclusion}$: The results of this study suggest that the concurrent chemoradiation therapy with HDR brachytherapy could be accepted as an effective and safe treatment for cervical cancer.

• Tuberculosis and Respiratory Diseases
• /
• v.45 no.6
• /
• pp.1143-1153
• /
• 1998

Background : Recent outbreaks of pulmonary disease due to drug-resistant strains of Mycobacterium Tuberculosis have resulted in significant morbidity and mortality in patients worldwide. We reviewed our experience to evaluate the effects of pulmonary resection on the management of multidrug-resistant tuberculosis. Method : A retrospective review was performed of 41 patients undergoing pulmonary resection for multidrug-resistant tuberculosis between January 1993 and December 1997. We divided these into 3 groups according to the radiologic findings : (1) patients who have reasonably localized lesion (Localized Lesion Group ; LLG) (2) patients who have cavitary lesions after pulmonary resection on chest roentgenogram (Remained Cavity Group : RCG) (3) patients who have Remained infiltrative lesions postoperatively (Remained infiltrative group : RIG). We evaluated the negative conversion rate after resection and overall response rate of the groups. Then they were compared with the results of the chemotherapy on the multi drug-resistant tuberculosis which has been outcome by Goble et al. Goble et al reported that negative conversion rate was 65% and overall response rate, 56% over a mean period of 5.1 months. Results : Seventy five point six percent were men and 24.4% women with a median age of 31 years (range, 16 to 60 years). Although the patients were treated preoperatively with multidrug regimens in an effort to reduce the mycobacterial burden, 22 of 41 were still sputum culture positive at the time of surgery. 20 of 22 patients(90.9%, p<0.01) responded which is defined as negative sputum cultures within 2 months postoperative. Of 26 patients with the sufficient follow up data, 19 have Remained sputum culture negative for a mean duration of 25.7 months (73.1%, p<0.05). The bulk of the disease was manifest in one lung, but lesser amounts of contralateral disease were demonstrated in 15, consisted of 8 in RIG and 7 in RCG, of 41. 12 of 12 patients (100%, p<0.01) who were sputum positive at the time of surgery in LLG converted successfully. 14 of 15 patients (93.3%, p<0.05) with the follow up have completed treatment and not relapsed for a mean period of 25. 7 months. The mean length of postoperative drug therapy of LLG was 12.2 months. In RIG, postoperative negative conversion rate was 83.3% which was not significant statistically. There was a statistical significance in overall response rate (100%, p<0.05) of RIG for a mean period of 24.4 months with a mean length of postoperative chemotherapy, 11.8 months. In RCG a statistically lower overall response rate (14.3%, p<0.01) has been revealed for a mean duration of follow up, 24.2 months. A negative conversion rate of RCG was 75% which was not significant statistically. Conclusion : Surgery plays an important role in the management of patients with multidrug-resistant Mycobacterium tuberculosis infection. Aggressive pulmonary resection should be performed for resistant Mycobacterium tuberculosis infection to avoid treatment failure or relapse. Especially all cavitary lesions on preoperative chest roentgenogram should be resected completely. If all of them could not be resected perfectly, you should not open the thorax.

• Radiation Oncology Journal
• /
• v.20 no.1
• /
• pp.53-61
• /
• 2002

Purpose : To determine the optimal scheme of postoperative chemoradiotherapy in rectal cancer by comparing survival, Patterns of failure, toxicities in early and late radiotherapy groups using a Phase III randomized prospective clinical trial. Materials and Methods : From January 1996 to March 1999, 307 patients with curatively resected AJCC stage II and III rectal cancer were assigned randomly to an 'early (151 patients, arm 1)' or a 'late (156 patients, arm II)' and were administered combined chemotherapy (5-FU $375\;mg/m^2/day$, leucovorin $20\;mg/m^2$, IV bolus daily, for 3 days with RT, 5 days without RT, 8 cycles with 4 weeks interval) and radiation therapy (whole pelvis with 45 Gy/25 fractions/5 weeks). Patients of arm I received radiation therapy from day 1 of the first cycle of chemotherapy and those of arm II from day 57 with a third cycle of chemotherapy. The median follow-up period of living patients was 40 months. Results : Of the 307 patients enrolled, fifty patients did not receive scheduled radiation therapy or chemotherapy. The overall survival rate and disease free survival rate at 5 years were $78.3\%\;and\;68.7\%$ in arm I, and $78.4\%\;and\;67.5\%$ in arm II. The local recurrence rate was $6.6\%\;and\;6.4\%$ (p=0.46) in arms I and II, respectively, no significant difference was observed between the distant metastasis rates of the two arms ($23.8\%\;and\;29.5\%$, p=0.16). During radiation therapy, grade 3 diarrhea or more, by the NCI common toxicity criteria, was observed in $63.0\%\;and\;58.2\%$ of the respective arms (p=N.S.), but most were controlled with supportive care. Hematologic toxicity (leukopenia) greater than RTOG grade 2 was found in only $1.3\%\;and\;2.6\%$ of patients in each respective arm. Conclusion : There was no significant difference in survival, patterns of failure or toxicities between the early and late radiation therapy arms. Postoperative adjuvant chemoradiation was found to be a relatively safe treatment but higher compliance is needed.

• Journal of Chest Surgery
• /
• v.32 no.7
• /
• pp.621-627
• /
• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

• Tuberculosis and Respiratory Diseases
• /
• v.42 no.1
• /
• pp.58-66
• /
• 1995

Background: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. Method and Purpose: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. Results: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test Conclusion: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.