Clear cell chondrosarcoma, first described by Unni in 1976, is distinguished from classical chondrosarcoma by a typical histological picture, mostly an epiphyseal site of origin, and relatively a benign clinical course. We present a case of clear cell chondrosarcoma arising from hyoid bone in a 70-year-old male. Histologically, large areas of closely packed cells with characteristic clear cytoplasm were seen in addition to the usual elements of a conventional chondrosarcoma. Our search and review of the literature did not reveal any reported case of clear cell chondrosarcoma arising from hyoid bone.
Aged ; Chondrosarcoma* ; Cytoplasm ; Humans ; Hyoid Bone* ; Male

Angiomyolipoma is considered by many authors to be a hamartoma, occurring in a sporadic form or in association with tuberous sclerosis. This lesion consists of thick walled blood vessels, smooth muscle, and mature adipose tissue in varying amounts. We have experienced a case of the angiomyolipoma composed of monotypic epithelioid cells. The patient was a 69-year-old female. Clinically, there was no evidence of tuberous sclerosis. Microscopically, the tumor was composed of polygonal cells with abundant eosinophilic granular or clear cytoplasm, pleomorphic nuclei, prominent nucleoli, and multinucleated giant cells. The tumor cells showed positive reaction for HMB45, CD68, smooth muscle actin, and S-100, and negative reaction for epithelial membrane antigen, cytokeratin, vimentin, desmin, CD34, estrogen receptor, and progesterone receptor. Ultrastructual analysis showed the presence of glycogen, mitochondria, and other microorganelles in neoplastic cells. Melanosome or premelanosome was not identified.
Actins ; Adipose Tissue ; Aged ; Angiomyolipoma* ; Blood Vessels ; Cytoplasm ; Desmin ; Eosinophils ; Epithelioid Cells ; Estrogens ; Female ; Giant Cells ; Glycogen ; Hamartoma ; Humans ; Keratins ; Kidney* ; Melanosomes ; Mitochondria ; Mucin-1 ; Muscle, Smooth ; Receptors, Progesterone ; Tuberous Sclerosis ; Vimentin

We evaluated the prevalence of vitamin B12 deficiency and associated factors in type 2 diabetes patients using metformin. A total of 799 type 2 diabetes patients using metformin was enrolled. Vitamin B12 and folate levels were quantified by chemiluminescent enzyme immunoassay. Vitamin B12 deficiency was defined as vitamin B12 < or = 300 pg/mL without folate deficiency (folate > 4 ng/mL). The prevalence of vitamin B12 deficiency in metformin-treated type 2 diabetes patients was 9.5% (n = 76), and the mean vitamin B12 level was 662.5 +/- 246.7 pg/mL. Vitamin B12 deficient patients had longer duration of metformin use (P < 0.001) and higher daily metformin dose (P < 0.001) than non-deficient patients. Compared with daily metformin dose of < or = 1,000 mg, the adjusted odds ratio for 1,000-2,000 mg, and > or = 2,000 mg were 2.52 (95% CI, 1.27-4.99, P = 0.008) and 3.80 (95% CI, 1.82-7.92, P < 0.001). Compared with metformin use of < 4 yr, the adjusted odds ratios for 4-10 yr, and > or = 10 yr were 4.65 (95% CI, 2.36-9.16, P < 0.001) and 9.21 (95% CI, 3.38-25.11, P < 0.001), respectively. In conclusion, our study indicates that patients with type 2 diabetes treated with metformin should be screened for vitamin B12 deficiency, especially at higher dosages (> 1,000 mg) and longer durations (> or = 4 yr) of treatment.
Aged ; Area Under Curve ; Diabetes Mellitus, Type 2/complications/diagnosis/*drug therapy ; Female ; Folic Acid/blood ; Humans ; Hypoglycemic Agents/adverse effects/*therapeutic use ; Immunoassay ; Male ; Metformin/adverse effects/*therapeutic use ; Middle Aged ; Odds Ratio ; Patients ; Prevalence ; ROC Curve ; Time Factors ; Vitamin B 12/blood ; Vitamin B 12 Deficiency/diagnosis/epidemiology/*etiology

Neonatal ascites is an uncommon problem with many etiologies. The common causes include hematologic diseases, bowel perforation, obstructive uropathy, cardiovascular diseases, chylous ascites, intrauterine infection, and meconium peritonitis. Recently, the wide application of sonography has greatly narrowed the list of differential diagnosis of neonatal ascites. Meconium peritonitis is readily diagnosed if calcification in the abdomen or scrotum can be seen radiologically or sonographically in a neonate with abdominal distension at birth. We report a case of generalized meconium peritonitis without intraabdominal calcification by radiologic and sonographic study and notable meconium hydrocele at birth.
Abdomen ; Ascites ; Cardiovascular Diseases ; Chylous Ascites ; Diagnosis, Differential ; Hematologic Diseases ; Humans ; Infant, Newborn ; Meconium* ; Parturition ; Peritonitis* ; Scrotum ; Ultrasonography

PURPOSE: The clinical findings of early neonatal hypocalcemia are variable and it is difficult to find relationship between the symptoms and hypocalcemia due to complex causes. The purpose of this study is to establish the relationship between early neonatal hypocalcemia and clinical manifestations and to propose a guideline for appropriate treatment of early neonatal hypocalcemia, especially in asymptomatic cases. METHODS: Study subjects were all sick babies admitted to nursery and NICU and randornly selected 43 healthy babies at Sun General Hospital from January 1996 to December 1996. We examined serum calcium level within 72 hours after birth. Then we evaluated prospectively clinical findings according to each disease category in hypocalcemic cases and analysed the relationship of time course of clinical findings with hypocalcemia and compared serum calcium concentration followed by each therapy after 3 days. RESULTS: The results were as follows. 1) The incidence of early neonatal hypocalcernia was high in premature infants, low birth weight infants, infants with neonatal asphyxia, hyaline membrane disease and transient tachypnea. 2) Tremor, seizure, apnea, dyspnea, abdominal distension, cyanosis, and vomiting were frequently presented symptoms in early neonatal hypocalcemia. 3) In the cases of early hypocalcemia with symptoms, these symptoms persisted continuously after norrnalization of serum calcium concentration. 4) Among asymptomatic hypocalcernic group, mean serum calcium levels changed from 6.7 mg/dL to 8.7 mg/dL in 23 cases of no treatment, from 5.4 mg/dL to 10.3 mg/dL in 4 cases of calcium gluconate infusion, and from 6.3 mg/dL to 8.7 mg/dL in 7 cases of feeding low phosphorus containing milk. None persisted in hypocalcemic state irrespective of treatment methods. CONCLUSION: It is difficult to regard these symptoms as a rule to treatment because these symptoms were present after normalizaton of serum calcium concentration. In addition, asymptomatic hypocalcemia was improved shortly without any treatment without any problem. We conclude that for asymptomatic hypocalcemia, withholding dangerous calcium gluconate infusion would be perrnissible.
Apnea ; Asphyxia ; Calcium ; Calcium Gluconate ; Cyanosis ; Dyspnea ; Hospitals, General ; Humans ; Hyaline Membrane Disease ; Hypocalcemia* ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Milk ; Nurseries ; Parturition ; Phosphorus ; Prospective Studies ; Seizures ; Solar System ; Tachypnea ; Tremor ; Vomiting

Thanatophoric dysplasia (TD) is a sporadic lethal type of skeletal dysplasia featuring micromelia, decreased thoracic dimension and macrocephaly. To date, several kinds of mutation in fibroblast growth factor receptor 3 (FGFR3) has been identified in TD. We experienced a case of TD type I and underwent sequencing of the exon 7, 10 and the stop codon of FGFR3 to identify the type of mutation. TDI was diagnosed by the prenatal ultrasound at 25 weeks of gestation. The pregnancy was terminated and the diagnosis was confirmed by radiological and histologic examinations. The genomic DNA was extracted and the sequences of the exon 7, 10 and the stop codon of FGFR3 were amplified by PCR. The sequencing was performed for the each PCR products by dideoxyterminator method. The nucleotide transition from G to T was found in the nucleotide 1108, which is a part of the transmembrane domain, exon 10. To date, only one type of mutation (nucleotide 742) in the FGFR3 was identified in TD1 among Asian. This case firstly reveals the mutation of FGFR3 other than mutation at nucleotide 742 in TD1.
Asian Continental Ancestry Group ; Codon, Terminator ; Diagnosis ; DNA ; Exons* ; Fibroblast Growth Factors* ; Fibroblasts* ; Humans ; Macrocephaly ; Polymerase Chain Reaction ; Pregnancy ; Receptor, Fibroblast Growth Factor, Type 3* ; Receptors, Fibroblast Growth Factor* ; Thanatophoric Dysplasia* ; Ultrasonography

A nephropathy following urinary tract infection is usually referred to as renal scarring. The main radiologic features are an overall reduction in the size of the kidney, with coarse scar, deformity of calyces and indentation of the surface. If adequately treated, the progressive renal scarring by urinary tract infection could be prevented. Therefore, the early radiologic detection of renal damage following urinary tact infection or vesicoureteral reflux is great importance for the evaluation of the pathogenesis of renal scarring and for the planning of the therapy. To evaluate the renal damage, we must have the normal data of the kidneys. Many reports discussed the renal size in normal children, but there are no reports in the Korean children. We estimate the renal length, width, several focal parenchymal thicknesses for renal size evaluation and segmental lumbar vertebral length at the intravenous pyelography in the normal Korean children. And the linear equations are obtained by the regression analysis between the various renal parameters and segmental vertebral length. Thereafter we make out the nomogram by the obtained equations. The renal length and width are highly correlated to the segmental lumbar vertebral length than various renal parenchymal thicknesses. These result suggest that the renal length and width are reliable parameters for normal renal size evaluation in growing kidney. And then the obtained equations and normograms might be useful in the diagnosis of parenchymal loss in early scarring and follow-up.
Child* ; Cicatrix ; Congenital Abnormalities ; Diagnosis ; Humans ; Kidney ; Nomograms ; Urinary Tract Infections ; Urography ; Vesico-Ureteral Reflux

Guillain-Barr syndrome is rarely complicated by hypertension, which has been ascribed to sympathetic nervous system hyperactivity. We report a 11 years old female with Guillain-Barr syndrome complicated by persistent hypertension associated with elevated renin-angiotensin. So we report this case with brief review of related literatures.
Child ; Female ; Humans ; Hypertension* ; Sympathetic Nervous System

The present study has been conducted to assess the possible mechanism of cultured rabbit lens epithelial cell(LEC) damage and generation of oxygen free radicals after UVB exposure as well as to investigate the role of antioxidants to the survival rate of epithelial cells. Cultured rabbit LECs were exposed to various fluences of UVB radiation. The release of oxygen radicals(superoxide radical and hydrogen peroxide) and the activity of antioxidants(SOD, catalase) were also measured. The protective effects of various antioxidants on LECs damage after UVB exposure were measured. UVB radiation brought about a decrease in the survival fraction of cultured rabbit LECs. Exposure of the cells to fluences of 400 mJ/cm2, 1,600 mJ/cm2 and above 2,400 mJ/cm2 of UVB radiation resulted in 50%, 10%, nearly 0% in cell survival fraction, respectively. Cultured rabbit LECs exposed to UVB and hydrogen peroxide. The activity of intracellular SOD(esp. Cu, Zn-SOD) was significantly increased, but the activity of intracellular catalase was not changed. Antioxidants pretreatment(SOD, catalase, purpurogillin and allopurinol) ameliorated the cytoxoxic effect of UVB on the cultured rabbit LECs. These rusults indicate that the release of oxygen radicals are enhenced by exposure of LECs to UVB radiation. The oxygen radicals seem to play a specific role in the cytotoxic effect of LECs after UVB exposure.
Antioxidants ; Catalase ; Cataract ; Cell Survival ; Epithelial Cells* ; Free Radicals ; Hydrogen ; Hydrogen Peroxide ; Oxygen ; Reactive Oxygen Species ; Survival Rate

No abstract available.
Child* ; Humans