Kim Ji-Hee;Shin Hye-Kyung;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
Childhood Kidney Diseases
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v.6
no.2
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pp.266-271
/
2002
There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome Is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.
The Journal of the Korean bone and joint tumor society
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v.4
no.1
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pp.44-52
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1998
We treated 19 cases of unicameral bone cysts with methylprednisolone acetate (MPA) from January 1988 to December 1995. We evaluated the effect of MPA injections through simple follow-up radiographs according to Oppenheim's, classification and retrospectively reviewed the sites of cyst, age of the patients at diagnosis, the incidence of pathologic fracture, cystic nature, and cystic proximiy to the growth plate, We then analyzed the relationship between these variables with the results of MPA injections. According to Oppenheim's classification, the results with the use of MPA injections were as follows: healed in six cases, improved in seven cases, incomplete obliteration in five cases and recurred in one case, If healed and improved were considered satisfactory results, then 13 cases(68.4%) were satisfactory at the last follow-up. Sites of cyst, age of the patients at diagnosis, incidence of pathologic fracture, cystic nature and cystic proximiy to the growth plate appeared not to influence the results of MPA injections statistically. On these data, we thought that the treatment of unicameral bone cysts with MPA injection was the most effective first choice of treatment before operative options such as curettage and bone graft.
The Korean Journal of Oral and Maxillofacial Pathology
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v.42
no.6
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pp.189-198
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2018
A 31 years old female had been suffered from a bony swelling in right premolar region of the mandible for 12 years, recently grown rapidly. A fistula tract developed on the right anterior mandibular border, but the lesion was relatively asymptomatic. In the radiological examination, the tumor mass was irregularly mixed with radiolucent and radiopaque areas, forming multiple cystic spaces. Under the diagnosis of calcifying odontogenic cyst, the mandibular mass was resected and examined pathologically. After decalcification, the dissected tumor mass showed multiple small cystic spaces and calcifying fibrous tissue, mimicking calcifying odontogenic cyst or ameloblastoma. Histological observation showed many calcifying cementoid materials and ossifying trabeculae. The cystic spaces were turned out to be dilated vascular channels lined by endothelial cells, containing plasma fluid. However, the main lesion was diagnosed as cemento-ossifying fibroma (COF), and the atypical vascular channels were greatly dilated and gradually expanded the whole tumor mass. The present COF was examined through immunohistochemical (IHC) array, and investigated for tumor cell characteristics, exhibiting abnormal ossification and aneurysmal cystic changes. IHC array disclosed that the tumor cells grew progressively in the lack of apoptosis, and that they showed lower expression of RUNX2 than BMP-2, RANKL, and OPG, and increases of protein expression in $HIF-1{\alpha}$, VEGF-A, and CMG2. These data suggested that the reduced expression of RUNX2, osteoblast differentiation factor, be relevant to abnormal ossification of COF, and that the consistent expressions of angiogenesis factors be relevant to de novo angiogenesis in COF, subsequently resulted in aneurysmal cystic changes.
Purpose: Group A streptococcus (GAS) is a common pathogen in pediatric patients and often causes acute pharyngotonsillitis and skin and soft tissue infections. In addition, bacteremia with significant morbidity and mortality can also occur. This study was conducted to describe the clinical manifestations and treatment outcomes of pediatric GAS bacteremia patients in Korea. Methods: This was a single-center, retrospective study. From January 2000 to December 2016, pediatric patients aged ≤18 years with GAS bacteremia were studied. Clinical manifestations, underlying diseases, intensive care unit stay, and antibiotic susceptibility were evaluated. Results: During the study period, 19 patients had GAS bacteremia. Ten (53%) were male, and the median age was 7.4 years (range, 0.3-17.4 years). Fourteen (74%) had chronic underlying diseases. Five (26%) were immunocompromised (leukemia and chronic kidney disease). Eight (42%) had lymphatic or vascular malformations, of which seven had lesions with signs of inflammation. Three (16%) developed pneumonia, and two of them received ventilator care. The 30-day mortality rate was 6% (1/19), and the cause of death was bacteremic pneumonia. All GAS isolates were sensitive to penicillin. Fifteen (79%) were sensitive to both erythromycin and clindamycin. Conclusions: This study identified various clinical manifestations of GAS bacteremia. GAS should be considered as a potential pathogen that can cause bacteremia and result in a serious clinical course.
Digital Video Library System which manages a large amount of multimedia information requires efficient and effective retrieval methods. In this paper, we propose and implement a new video search and retrieval algorithm that compares the query video shot with the video shots in the archives in terms of foreground object, background image, audio, and its context. The foreground object is the region of the video image that has been changed in the successive frames of the shot, the background image is the remaining region of the video image, and the context is the relationship between the low-level features of the adjacent shots. Comparing these features is a result of reflecting the process of filming a moving picture, and it helps the user to submit a query focused on the desired features of the target video clips easily by adjusting their weights in the comparing process. Although the proposed search and retrieval algorithm could not totally reflect the high level semantics of the submitted query video, it tries to reflect the users' requirements as much as possible by considering the context of video clips and by adjusting its weight in the comparing process.
The aim of this retrospective study was to determine whether a nephron sparing surgery might be feasible in patients with a small solid renal tumor. Materials and methods: Between 1988 and 1999, 21 patients with a radiologically detectable small solid renal tumor underwent enucleoresection, wedge resection and polar segmental nephrectomy. The mean age of the 11 men and 10 women in this study was 43 years (range 14 to 68). According to the preoperative radiological diagnosis, 15 among the 21 patients were considered to have renal cell carcinoma, 4 were considered to have angiomyolipoma, and in the remaining 2 patients, radiological differentiation of renal tumors was difficult. Among 15 patients considered to have renal cell carcinoma, 14 were found to have renal cell carcinoma and the remaining one patient was diagnosed as having oncocytoma on pathologic examination. Radiological determination of angiomyolipoma in four patients was confirmed to be correct on pathological examination. The 2 patients whose radiological diagnose was difficult were found to have cavernous hemangioma and angiomyolipoma. One patient with renal cell carcinoma developed arteriocaliceal fistula, the only immediate complication in this series and underwent nephrectomy on postoperative 10th day. The mean follow up duration for the 14 patients with renal cell carcinoma was 18.6 months (range:1-103). There was no other tumor involvement in the resection margins following the nephron sparing surgery. These results suggest that nephron sparing surgery provides an effective treatment for patients with a single, small, unilateral, localized renal tumor. Longer follow-up is suggested for more definite verification of the role of nephron sparing surgery.
Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.
Purpose : The purpose was to assess the result of arthroscopic management of the septic arthritis on the knee in compromised patients. Materials and Methods : Fourteen patients with septic knee were analyzed. The mean age was 55 years and the mean follow-up period was 14.6 months. Underlying diseases included 4 cases of diabetes, and history of direct acupuncture in 4 cases. Clinical stage of septic arthritis was judged by $G\ddot{a}chter's$ classification, which was determined by arthroscopic findings. After arthroscopic irrigation and debridement, we observed the results of laboratory data and improvement of clinical findings. Results : Causative organism was identified in 7 cases and no organism was detected in the remaining 7 cases. Stage I was 1, stage II 8, stage III 4, and stage IV 1, respectively. Eleven of 14 cases were improved by one stage operation. Two cases of stage III were recurred and additional arthroscopic management was done. In 1 case of stage IV, symptom was not improved and needed arthrotomy. The result was unsatisfactory in patients with stage III and IV. Serum erythrocyte sedimentation rate and C-reactive protein were normalized after 29.3 and 20.8 days following the operation, respectively. Clinical symptoms disappeared average 2 days following the operation. Conclusion : Arthroscopic management of acute septic arthritis of the knee would be an effective and satisfactory treatment modality in that its postoperative pain and complications are minimal, and it can be done with ease repeatedly.
Newborns with ileal atresia freqently present with abdominal distension, bilious vomiting. and failure to pass meconium. Diagnosis is usually established on plain x-ray of the abdomen by the findings of distended small bowel loops and air-fluid levels. In the period of October 1988 to February 1994, 8 patients with congenital ileal atresia were operated and the following results were obtained. 1. Eight patients were comprised of 4 males and 4 females, the ratio of male and female was 1 : 1. 2. Six patients(75%) had been admittted to our hospital during three days of life. 3. Congenital ileal atresia was in 8 cases : Type I in two(25%), Type II in two(25%), Type IIIa in three(37.5%), Type IIIb in one(12.5%). 4. There was one premature patient who was small for gestational age. 5. Overall, abdominal distension and bilious vomiting occurring in seven patients, were frequent presenting complaints. 6. Diagnosis was possible with clinical symptom and simple abdomen. 7. Operative treatment was undertaken as soon as the diagnosis was made. In seven cases a primary end-to-end anastomosis was performed after resection of dilated proximal loop. 8. A total of four associated congenital anomalies were found in one patient. 9. Postoperative complications occurred in three cases(37.5%).
Yoo Ji Hyung;Yook Jinwon;Kim Ji Hong;Kim Pyung-Kil;Han Sang Won;Kim Myung Joon
Childhood Kidney Diseases
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v.4
no.1
/
pp.63-68
/
2000
Purpose: MCDK is regarded as a common cause of abdominal masses in children. And the presentation of the MCDK is usually a unilateral flank mass in the a newborn. Bialteral disease results in either fetal demise or necessity fer renal replacement therapy at birth. This study is designed to assess the clinical features and natural history of the unilateral multicystic dysplastic kidney. Patients and Methods: From January 1987 to January 2000 data were obtained retrospectively on 57 patients (28 boys and 29 girls, age ranged 1day-11years) who had a diagnosis of multicystic dysplastic kidney. The diagnosis of multicystic dysplastic kidney was confirmed by a combination of ultrasonography and radionuclide scan. Voiding cystourethrogram study in 31 patients were done to determine the condition of the contalateral kidney. Restllts: $84\%$ of the patients were diagnosed before birth by antenatal ultrasonography Clinical manifestations of children with postnatal diagnoses were palpable abdominal mass($3.5\%$), abdominal distension($17\%$), and incidental($10.5\%$). The abnormalities in contralateral kidney were hydronephrosis($21\%$), compensatory hypertrophy($12\%$), simple cyst($2\%$), bifid pelvis($2\%$). Surgical management was performed in 20 patients($35\%$) due to recurrent infection, for diagnostic purpose to differentiate from malignancy and abdominal distention. Follow-up in the remaining 37 patients continued (mean 18 months) and results of sonogram findings were involution change in 23 patients($40\%$) and no interval changes in 13 patient($23\%$). Conclusions : The apparent tendency to regression of the dysplastic kidney and no difference in the number of complications justify a conservative management rather than operative intervention except in associated severe complications such as urinary tract infection or rupture of cysts.
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