Leiomyosarcoma(LMS) is a malignant tumor from smooth muscle origin that arises most commonly in the gastrointestinal tract and uterus, but rarely in the oral and maxillofacial area which lacks smooth muscles. 63 cases of oral LMS have been reported, especially LMS that occurred in the mandibular gingiva is only 2 cases. Major symptom of oral LMS is painful or painless swelling. Besides it, oral LMS has no specific characteristics. So it is difficult to diagnose the case as LMS of the oral cavity. Certain cases report that LMS of the oral cavity was misdiagnosed as periodontitis and the patients were treated with unnecessary procedures. Conventional treatment of LMS is the radical surgical excision. LMS that is infiltrative and very malignant has poor prognosis despite of well-circumscribed boundary. LMS of the oral cavity is often recurred, has high rate of distant metastasis and 5-year-survival rate is as low as 23%. This article reports LMS of the mandibular gingiva that treated with surgical intervention, had local recurrence and metastasis to the lymph node after 16-month's follow-up examination.
Follow-Up Studies ; Gastrointestinal Tract ; Gingiva* ; Humans ; Leiomyosarcoma* ; Lymph Nodes ; Mouth ; Muscle, Smooth ; Neoplasm Metastasis ; Periodontitis ; Prognosis ; Recurrence ; Unnecessary Procedures ; Uterus

Primary ureteral carcinoma is a rare disease comprising 1% of all urinary tract tumors. Primary adenocarcinoma of the ureter is extremely rare. We report a case of primary ureteral adenocarcinoma associated with long-standing ureteral stone in 40-year-old man, who was treated by nephroureterectomy with bladder cupping, with a brief review of the literatures.
Adenocarcinoma* ; Adult ; Humans ; Rare Diseases ; Ureter* ; Urinary Bladder ; Urinary Tract

The routine screening of patients for hypercalcemia has increased the incidence of hyperpa-rathyroidism, But, Primary hyperparathyroidism is relatively rare disease in korea. Especially, primary hyperparathyroidism caused by parathyroid chief cell hyperplasia has not been reported. The numbers of patients with bone disease, renal stones, other severe complication has declined, but the numbers of patients with psychiatric and neuromuscular disturbance or with asymptomatic disease has increased. We experienced a case with severe bone diseases due to primary hyperparathyroidism caused by parathyroid chief cell hyprepiasia which was confirmed through the mearsurement of serum calcium and parathyroid hormone, neck CT scanning and surgical exploration and which was managed by total parathyroidectomy with autotransplantation.
Asymptomatic Diseases ; Autografts ; Bone Diseases* ; Calcium ; Humans ; Hypercalcemia ; Hyperparathyroidism, Primary* ; Hyperplasia* ; Incidence ; Korea ; Mass Screening ; Neck ; Parathyroid Hormone ; Parathyroidectomy ; Rare Diseases ; Tomography, X-Ray Computed

A 60-year-old male was referred to the ophthalmologic clinic with aggravated anterior uveitis and corneal edema despite the use of topical and systemic steroids. He had undergone cataract surgery in both eyes 15 years previous. Slit lamp examinations revealed a retained lens fragment in the inferior angle of the anterior chamber, with severe corneal edema and mild anterior uveitis. The corneal edema and uveitis subsided following surgical extraction of the lens fragment. That a retained lens fragment caused symptomatic anterior uveitis with corneal edema 15 years after an uneventful cataract surgery is unique. A retained lens fragment should be considered as one of the causes of anterior uveitis in a pseudophakic patient.
Cataract Extraction/*adverse effects ; Corneal Edema/*etiology/*pathology ; Humans ; Lens, Crystalline/*pathology/surgery ; Male ; Middle Aged ; Postoperative Complications/pathology/surgery ; Pseudophakia/pathology ; Reoperation ; Severity of Illness Index ; Uveitis, Anterior/*etiology/*pathology

Idiopathic renal hypouricemia (iRHUC) is a rare hereditary disease caused by a defect in urate handling of renal tubules. Type 1 renal hypouricemia (RHUC1) is diagnosed with confirmation of a mutation in SLC22A12 gene which encodes a renal urate-anion exchanger (URAT1). The majority of iRHUC patients are asymptomatic, especially during childhood, and thus many cases go undiagnosed or they are diagnosed late in older age with complications of hematuria, renal stones, or acute kidney injury (AKI). We report a case of a 7-year-old boy with subtle symptoms such as general weakness and dizziness and revealed hypouricemia and incidental nephrolithiasis. Homozygous mutations were detected in the SLC22A12(c.774G>A) by molecular analysis. The present case suggests that fractional excretion of uric acid (FEUA) screening could be better followed by the coincidental discovery of hypouricemia, to prevent conflicting complications of iRHUC, even with normal urine uric acid to creatinine ratio (UUA/UCr), and sequential genetic analysis if needed.

Idiopathic renal hypouricemia (iRHUC) is a rare hereditary disease caused by a defect in urate handling of renal tubules. Type 1 renal hypouricemia (RHUC1) is diagnosed with confirmation of a mutation in SLC22A12 gene which encodes a renal urate-anion exchanger (URAT1). The majority of iRHUC patients are asymptomatic, especially during childhood, and thus many cases go undiagnosed or they are diagnosed late in older age with complications of hematuria, renal stones, or acute kidney injury (AKI). We report a case of a 7-year-old boy with subtle symptoms such as general weakness and dizziness and revealed hypouricemia and incidental nephrolithiasis. Homozygous mutations were detected in the SLC22A12(c.774G>A) by molecular analysis. The present case suggests that fractional excretion of uric acid (FEUA) screening could be better followed by the coincidental discovery of hypouricemia, to prevent conflicting complications of iRHUC, even with normal urine uric acid to creatinine ratio (UUA/UCr), and sequential genetic analysis if needed.

Head ; Lipoma ; Neck Muscles ; Neck ; Recurrence ; Shoulder ; Subcutaneous Tissue

In order to find out the distribution of fibronectin and fibrinogen in placenta among pregnancy induced hypertensive (PIH) patients, 6 normotensive pregnancies and 17 PIH patients were chosen. The placentas were obtained right after delivery and soaked in the 10% formalin solution. The score was measured in terms of the positiveness of the stain in immunohistochemical stain by using I antibody with the rabbit antihuman fibronectin and the rabbit anti-human fibrinogen. Comparison between two groups are as following: 1.The placenta of PIH patients showed significantly reduced positiveness of fibronectin in their fetal villous vessels and villous stroma. 2. In the PIH patients, the positiveness was reduced in the group giving the birth to intrauterine fetal growth retardation compared to giving birth to normal baby. 3. In both normotensive and PIH patients, the villous basement membrane did not show the staining response to fibronectin, while showing heavy staining response to fibrinogen. 4. The positiveness of fibrinogen in fetal vessels, villous basement membrane, intervillous space and stroma were almost same in both normotensive and PIH patients. From above results, we can conclude that there is abnormality in the distribution of fibronectin especially in the fetal vessels and the villous stroma of placenta among PIH patients. The cause of this result needs further study.
Basement Membrane ; Fetal Growth Retardation ; Fibrinogen* ; Fibronectins* ; Formaldehyde ; Humans ; Parturition ; Placenta* ; Pre-Eclampsia ; Pregnancy*

OBJECTIVE: This study aimed to estimate the association between total and ionized magnesium, and the changes in serum magnesium and calcium levels in patients with preterm labor during magnesium sulfate (MgSO4) administration. METHODS: The study population included 64 women who were candidates for intravenous MgSO4 treatment for preterm labor. Serial blood samples were taken and measured total magnesium (T-Mg), ionized magnesium (I-Mg), total calcium (T-Ca), and ionized calcium (I-Ca) levels every one-week interval (1st, 2nd, 3rd). RESULTS: There was no significant difference in T-Mg and I-Mg levels during MgSO4 administration (P>0.05). There was no significant difference in T-Ca and I-Ca levels during MgSO4 administration (P>0.05). Compared before and after administration of MgSO4, T-Mg and I-Mg levels and T-Ca levels were changed allow statistically significant (P < 0.05). But, there was no significant difference in the I-Ca serum levels before and after MgSO4 administration (P=0.495). The I-Mg levels for patients with adverse effect were higher than other group but did not reach statistical significance (P>0.05). There was significant correlation between levels of I-Mg and T-Mg (I-Mg=0.395×T-Mg+0.144, P < 0.01). CONCLUSION: There were no significant differences in serum Mg and Ca levels during MgSO4 administration for preterm labor. Compared to the before and after administration of MgSO4, only I-Ca levels were not substantially changed. There are significant correlations between I-Mg and T-Mg levels during administration of MgSO4 and I-Mg level seemed to have more correlation with adverse effect than T-Mg.
Calcium ; Female ; Humans ; Magnesium Sulfate ; Magnesium ; Obstetric Labor, Premature ; Pregnancy

Uterine rhabdomyosarcoma (RMS) typically presents as a mixed epithelial and mesenchymal tumors. Pure RMSs of the female genital tract are uncommon. Spindle cell variant of RMS is a rare morphologic subtype of embryonal RMS and mostly occurs in the paratesticular region of children. Here, we present a case of uterine spindle cell RMS in a 76-year-old woman. The tumor, 20x15x7 cm in size, was highly necrotic and adherent to the colon and rectum. Tumor cells were mostly spindle-shaped, and isolated rhabdomyoblasts were scattered. Immunohistochemical stains for myoglobin and myo-D1 showed diffuse positivity for tumor cells. The patient died only of disease three months after diagnosis.
Child ; Colon ; Coloring Agents ; Female ; Humans ; Myoglobin ; Rectum ; Rhabdomyosarcoma ; Uterus