• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.812-819
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• 2008

Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

• The Korean Journal of Nuclear Medicine
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• v.30 no.4
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• pp.524-531
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• 1996

Purpose : To evaluate the pattern- of skeletal metastases and to classify the pattern of renal uptakes on bone scans in renal cell carcinoma. Materials and Methods : We reviewed the bone scans of 158 patients with RCC established pathologically. In order to identify individual scan lesion as a bone metastasis, we reviewed all available correlative radiological studies, follow-up bone scans, and biopsies for each lesion. The metastatic bone lesions were divided into seven anatomic regions; skull, spine, shoulder girdle, sternum, ribs, pelvis, and long bones of extremities. The individual scan lesions were divided into two groups as the pattern of uptakes, hot and cold lesion. In addition, the contours and uptakes of kidneys with RCC were classified into 6 groups ; normal uptake, photon-deficient lesion, faint up-take with enlargement, uneven uptake with enlargement, lateralization with crescentic shape, and increased uptake. Results : Twenty out of 158(12.7%) patients with RCC at varying stages showed 71 metastatic bone lesions at presentation and on follow- up bone scans. Nearly 80% of all metastatic lesions were in the axial skeleton with predominantly increased uptake of the radioactivity However a considerable number(22.5%) showed cold lesions on bone scan. A half of bone scans revealed abnormal uptake of involved kidney and much more(82.4%) in case of bone metastases. Two common patterns of abnormal renal uptake were photon-deficient lesion (50%) and faint uptake with enlargement(24.3%). In four patients with bone pain or pathologic fracture, bone scans were useful for the serendipitious localization of previously unrecognized primary lesion of RCC as well as for the detection of bone metastases from RCC. Conclusion : The understanding of the pat-terns of skeletal metastases and renal uptakes on bone scans in RCC is important for the useful information about primary lesion(RCC) as well as detection of bone metastases.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.14-21
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• 2014

Purpose: To analyze the clinical features and treatment outcome of Langerhans' cell histocytosis. Materials and Methods: From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively. Results: A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow-up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3-6 months observation, intralesional steroid injection was performed. Conclusion: Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3-6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.2
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• pp.148-154
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• 2003

Purpose: To correlate the significant MRI findings and histologic features of the Schwannoma of the extremities and to review the clinical characteristic and the result of the surgical enucleation. Materials and Methods: 67 patients with pathologically proven Schwannoma of the extremities, who were surgically treated at our institutes between January 1996 and June 2002, were selected for this study. The clinical records, EMG, MRI and histologic findings were reviewed. Age of the patients ranged from 8 to 75 years with average of 44.7 years. Mean follow-up period was 9.7 months with raging from 3 months to 46 months. Results: On MRI, Schwannoma shows a well-demarcated fusiform mass with a low to intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images, which is connected to parent nerve. A target pattern with peripheral hyperintensive rim and central low intensity on T2-weighted images was seen in 6 cases (15%), and fasciculation pattern with inhomogenous intensity in the hyperintensity on T2-weighted images was observed in 24 cases (62%). Various degree of cystic degeneration was discovered in 25 cases (64%). Postoperative complications include tingling sense or radiating pain in 5 patients, paresthesia in 2 patients, nerve palsy in 2 patients, but all of the complications were recovered during followup period. There were no local recurrence or malignant change. Conclusion: MRI demonstrates characteristic findings of Schwannoma, and very useful tool for preoperative diagnosis and planning of surgery. Exact preoperative diagnosis and meticulous enucleation are enough option of treatment.

• Tuberculosis and Respiratory Diseases
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• v.45 no.6
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• pp.1154-1166
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• 1998

Background : In the management of patients whose primary chemotherapy has failed, careful assessment is essential. It is important to find out as accurate a chemotherapy history as possible. Preferably it should contain the drugs which has never used before. The purpose of present study is establishment of retreatment regimen for pulmonary tuberculosis. The present report concerns the results of retreatment of pulmonary tuberculosis patients treated at National Masan Tuberculosis Hospital. Methods : Retrospective cohort study was made of 104 drug-resistant pulmonary tuberculosis patients who were treated by five regimens between Jan. 1994 and Nov. 1996. All the patients taken medicine for second anti-tuberculosis regimens for the first time. We separated the patients by three groups(Group I ; OFX+PTA+CS+PAS+Aminoglycoside, Group II : PZA+PTA+CS+PAS+Aminoglycoside, Group III : PZA+OFX+PTA+PAS+Aminoglycoside). Results : The age distribution was most frequent in fourth decade(36patients, 34.6%) and the mean age was 42.6 year. The sex distribution was more frequent in the males(81 patients, 85.7%). There was 31 patients(29.8%) with combined diseaes, 18 patients with complication and 24 patients(27.9%) with family history. Primary chemotherapy regimens were HERZ(S or K) in 48 patients (46.2%), HER(S or K) in 41 patients(39.4%) and others in 15 patients(14.4%). Result of drug sensitivity test showed that the resistance to INH and RFP is in 68 patients(65.4%), RFP is 12 patients(11.5%), INH is in 3 patients(2.9%) and all sensitive to INH and RFP is 3 patients(2.9%). The clinical symptoms on admission were coughing(89.4%), sputum(69.2%), dyspnea on exertion(37.5%), weight loss(33.7%) blood tinged sputum(15.4%) and others. The extent of disease on the radiograph was far-advanced in 73 patients(70.2%), moderate in 28 patients(26.9%) and minimal in 3 patients(2.9%). The side effects for drugs were gastrointestinal troubles in 31 patients(29.8%), arthralgia in 22 patients(21.2%), skin rash in 12 patients(11.5%) and others. The negative conversion rate on sputum AFB smear was 85.6%(87.5% in Group I, 80.0% in Group II and 90.5% in Group III). The average negative conversion time on sputum was 4 month(4.0 month in Group I, 4.6 month in Group II and 3.0 month in Group III). Conclusion : In the retreatment of pulmonary tuberculosis, ofloxacin is useful drug for the patients who are not available to use PZA and combination of PZA and OFX can be use effectively substituting for CS.

• Tuberculosis and Respiratory Diseases
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• v.58 no.3
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• pp.295-298
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• 2005

We report a case of a 48-year-old man with a paradoxic upper airway obstruction and central sleep apnea that developed after an anterior cervical spinal fusion. Nine months before being admitted to this hospital, he was diagnosed with a herniated intervertebral disc between the 5th and 6th cervical spine, and the first operation was carried out. Two months later, a pseudoarthrosis has developed and a second operation, an anterior interbody fusion of the C5 and C6 using autogenous strut bone graft, was performed. After the second operation, he began to complain of snoring, excessive daytime sleepiness, insomnia, and a bizarre sound heard near the upper airway during breathing. Nasopharyngoscope and magnetic resonance imaging disclosed a paradoxical narrowing of the nasopharynx during expiration. On the overnight polysomnography, the apnea index was 8.7/h (central apnea, 7.0/h; obstructive apnea, 1.7/h). Nasal continuous positive airway pressure was applied, but he complained of pressure-intolerance, and laser-assisted uvulopalatoplasty was then performed. Two months after surgery, clinical symptoms as well as the apneas had improved markedly. We suggest that this paradoxic upper airway obstruction might be associated with the anterior cervical spinal surgery even though the mechanism is unclear. This case also emphasizes that an upper airway obstruction can contribute to the development of central sleep apnea.