Testicular feminization is an uncommon genetic disorder with considerably familial predisposition and results in total feminization due to end-organ unresponsiveness to androgens. It is characterized by the presence of testes in phenotypically female with adequate breast development, normal extemal genitalia, absence of mullerian structures, and meager or absence of body hair. These patients characteristically have male karyotype(XY) and negative sex chromatin and are at increased risk of undergoing malignant transformation of the undescended gonad. In recent times, the malignant potential of the dysgenetic gonads in the intersex patients with a Y chromosome has been stressed by many authors, but few reports of an association between testicular feminization syndrome and benign tumors such as Sertoli cell adenomas. In the present study, postoperative pathology revealed that the gonads were Sertoli cell adenomas. The main features of clinical presentation and histological studies are briefly discussed with a review of the literature.
Adenoma* ; Androgen-Insensitivity Syndrome* ; Androgens ; Breast ; Female ; Feminization ; Genitalia ; Gonads ; Hair ; Humans ; Male ; Pathology ; Sex Chromatin ; Testis ; Y Chromosome

OBJECTIVE: The objective of this study was to determine the relationship between the fetal doppler flow velocimetry and birth weight in low risk pregnancy population. METHODS: From December 1995 to May 1996, We prospectively performed doppler study in 254 uncomplicated, term pregnant women, who visited Pohang Hospital, Dongguk University. Using pulsed color doppler, we measured umbilical artery RI, middle cerebral artery RI and middle cerebral-umbilical artery RI ratio within one week before delivery. RESULTS: The result was that low birth weight group (below 2500gm) had very significant lationship with umbilical artery RI(P<0.01), middle cerebral artery RI(P<0.05) and middle cerebral-umbilical artery RI ratio(P<0.05), but there was no significant relationship in these blood flow indices between normal birth weight group (2501gm- 3999gm) and macrosomia group (above 4000gm). CONCLUSIONS: We concluded that application of doppler ultrasonopaphy in the assessment of fetal weight is somewhat helpful for identification of low birth weight, not for macrosomia.
Arteries ; Birth Weight ; Female ; Fetal Weight* ; Gyeongsangbuk-do ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Middle Cerebral Artery ; Pregnancy ; Pregnant Women ; Prospective Studies ; Rheology ; Ultrasonography* ; Umbilical Arteries

PURPOSE: Non-accidental intracranial hemorrhage in children is not low in incidence and results in high mortality and serious sequelae. So, the authors have researched the distribution of sex and age, causes, symptoms and signs, hemorrhagic types, mortality rate and sequelae of the patients hospitalized with non-accidental intracranial hemorrhage at Hallym University Sacred Heart Hospital. METHODS: The medical records of twenty patients, aged 15 or younger, and excluding neonatal patients, were analyzed retrospectively. The patients in this study were admitted with non-accidental intracranial hemorrhage from January 1999 to June 2002. RESULTS: Of the twenty cases, the ratio of male to female was 1 : 0.8. The patients aged one or less and between 11 and 15 were discovered to be the most frequent cases. Shaken baby syndrome and arteriovenous malformation were found to be the most frequent causes. Seizure was most frequently found to be a symptom and a sign. Hemorrhagic type was classified into subdural hemorrhage eight, intracerebral hemorrhage five. There were three mortal cases. Twelve surviving patients, excluding five not-followed ones, were reclassified into six cases of complete recovery and six of sequalae. CONCLUSION: Non-accidental intracranial hemorrhage in children is not low in incidence, with a high mortality rate and a high incidence of serious sequelae after survival. Consequently, early diagnosis and appropriate treatment are required. In addition, appropriate rehabilitation after treatment is needed because the high survival rate due to advanced medical treatment results in an increasing number of neurologic sequelae.
Arteriovenous Malformations ; Cerebral Hemorrhage ; Child* ; Early Diagnosis ; Female ; Heart ; Hematoma, Subdural ; Humans ; Incidence ; Intracranial Hemorrhages* ; Male ; Medical Records ; Mortality ; Rehabilitation ; Retrospective Studies ; Seizures ; Shaken Baby Syndrome ; Survival Rate

PURPOSE: The purpose of this study was to identify changes over several years in clinical patterns of patients who underwent hepatectomies for HCC. METHODS: There were 502 patients who underwent hepatectomies for HCC between January 1986 and July 2009 at the Korea Cancer Center Hospital, Seoul, Korea. Period 1 (n=206) extended from the beginning to December 2000. Period 2 from January 2001 to December 2004 (n=146). Period 3 from January 2005 to July 2009 (n=150). The data for the different periods were compared retrospectively or prospectively. RESULTS: Compared to patients from Period 1, Period 2 and Period 3 patients had operations >6 months from diagnosis (p=0.002), high levels of preoperative AST and ALT (p<0.001), and poor scores on the ICG R15 test (p=0.047). The Frequency of Transfusion during the operation was decreased (p<0.001), but there was no difference in postoperative complication rates (p=0.403). In Periods 2 and 3, there was a higher frequency of multiple tumors (p<0.001) and microvascular invasions histologically (p<0.001). Differences between periods in disease free survival rates were not significant. CONCLUSION: The reason for the higher frequency, in more recent periods, of patients presenting at surgery with advanced HCC stages is the longer interval between diagnosis and operative time, and an increase in the number of non-surgical treatments before surgery. Because of differences in follow-up intervals, it was difficult to evaluate the influence of period on survival rates. Hence, a longer follow-up period will be required for an accurate evaluation of changes in survival with time.
Carcinoma, Hepatocellular ; Disease-Free Survival ; Follow-Up Studies ; Hepatectomy ; Humans ; Korea ; Operative Time ; Postoperative Complications ; Prognosis ; Prospective Studies ; Retrospective Studies ; Survival Rate

Mucosa-associated lymphoid tissue (MALT) lymphoma is an uncommon form of non-Hodgkin lymphoma involving the mucosa-associated lymphoid tissue. Most commonly affected organ is the stomach. But, it could affect almost every organ in the whole body. If they have suspicious lesion, excision biopsy could be made. For staging, blood test including blood smear, abdomen and chest tomography or scan will be checked. Also, bone marrow test can be done if it is needed. The patient had visited the clinic for palpable mass on right lower eyelid. With excisional biopsy, it was diagnosed as lymphoid hyperplasia on pathologic test. But 2 years later, the patient came with recurrent symptom for our department with worry. At that time, we recommended excisional soft tissue biopsy under general anesthesia. Unfortunately, it was revealed MALT lymphoma on pathologic finding. It turned out to be stage 3 in Ann Arbor staging system without B symptoms. Hematologic consultation was made and she was treated with adjuvant chemotherapy for eight cycles to complete remission. We report a case of MALT lymphoma on subcutaneous tissue at right lower eyelid previously diagnosed as lymphoid hyperplasia.
Abdomen ; Anesthesia, General ; Biopsy ; Bone Marrow ; Chemotherapy, Adjuvant ; Eyelids ; Hematologic Tests ; Humans ; Hyperplasia ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone ; Lymphoma, Non-Hodgkin ; Stomach ; Subcutaneous Tissue ; Thorax

Malignant pilomatricoma (pilomatrical carcinoma) is a rare, locally occurring malignant tumor with a high rate of recurrence in the case of incomplete excision. This tumor has two characteristics. First, recurrences of pilomatrical carcinoma are common; second, distant metastasis is rare, but if it occurs, it is very fatal. It has characteristic features of high mitotic counts, cellular atypia, and local invasion. Although fine needle aspiration and excisional biopsy could help to confirm this tumor diagnosis, pathologic findings are critical. Pilomatricomas have some characteristic features in histological aspect, such as epithelial islands of basaloid cells and shadow cells or ghost cell. Also, various types of immunohistochemical staining are used to confirm the diagnosis. Despite the lack of clear surgical criteria, treatment is a wide local excision with histologically clear resection margins with or without adjuvant radiotherapy.
Biopsy ; Biopsy, Fine-Needle ; Cheek* ; Diagnosis ; Facial Neoplasms ; Humans ; Infant* ; Islands ; Neoplasm Metastasis ; Pilomatrixoma* ; Radiotherapy, Adjuvant ; Recurrence

Shaken baby syndrome is a serious form of child abuse, mostly involving children younger than 2 years. It results from extreme rotational cranial acceleration induced by violent shaking. The characteristic injuries include subdural hemorrhage, retinal hemorrhage, and fracture of ribs or long bones. If physicians have no recognition of, or suspicion about, shaken baby syndrome, this syndome is difficult to diagnosis because of a lack of obvious external signs and failure of the abuser to admit his or her actions. In addition to the high mortality, 60% of survivors have significant long term neurologic and developmental abnormality. The authors experienced five cases of shaken baby syndrome presented with seizures or vomiting, without external signs of trauma. All of these cases had subdural hemorrhages, and four cases had retinal hemorrahges. In our follow up, two children were found to have sequelae such as quadriplegia, monoplegia, and developmental delay. We emphasize that early recognition and prompt treament are key to overall success of case management. The incidence of shaken baby syndrome can be reduced through public awareness and education of parents not to shake a babies.
Acceleration ; Case Management ; Child ; Child Abuse ; Diagnosis ; Education ; Follow-Up Studies ; Hematoma, Subdural ; Hemiplegia ; Humans ; Incidence ; Mortality ; Parents ; Quadriplegia ; Retinal Hemorrhage ; Retinaldehyde ; Ribs ; Seizures ; Shaken Baby Syndrome* ; Survivors ; Vomiting

PURPOSE: To raise awareness of the clinical importance of, and the need for proper management of acute focal bacterial nephritis(AFBN), we analyzed 22 AFBN patients and 22 other upper urinary tract infection patients by use of comparative studies. METHODS: From January 2000 to May 2002, 22 AFBN patients aged from 1 month to 12 months were selected. As a control group, 22 UTI patients with no radiologic abnormalities were selected and matched by age and sex. RESULTS: The incidence of AFBN was more common in boys than in girls. Since both groups had similar symptoms, it was difficult to diagnose AFBN by clinical presentations alone. ESR and CRP were significantly higher in AFBN patients. The most common causative organism was E. coli in both groups. On the sonographic findings, the most lesions were seen on the upper lobe of the kidney; more frequently, on left kidney. The lesions showed globular or wedge-shaped increased echogenecity. 99mTc-DMSA scan showed the complete coincidence of the location, size and shape in all cases compared to the findings of renal sonography. CONCLUSION: The roles of renal sonography and DMSA scan were very important, and ultrasonography was an excellent initial tool in diagnosing AFBN. Since the degree of infection in AFBN is more severe than other urinary tract infections and evollution into a renal abscess is possible, early diagnosis and appropriate antibiotics therapy is essential.
Abscess ; Anti-Bacterial Agents ; Child* ; Early Diagnosis ; Female ; Humans ; Incidence ; Kidney ; Nephritis* ; Succimer ; Technetium Tc 99m Dimercaptosuccinic Acid ; Ultrasonography ; Urinary Tract Infections

PURPOSE: The aims of this study were to investigate the survival results and the prognostic factors of adjuvant chemotherapy in stage II colon cancer in the sparsity of Korean data. METHODS: From 1993 to 2006, 363 curatively resected pathologic stage II colon cancer patients were enrolled. Six cycles of adjuvant chemotherapy was performed: intravenous bolus 5-fluorouracil (5-FU) 500 mg/m2 with leucovorin 20 mg/m2 for 2 hours daily for 5 days, followed by a 3-week resting period (n = 308). Fifty-five patients received only curative surgery. A high risk of recurrence was defined as the presence of one or more of the following factors: T4 tumor, lympho-vascular invasion, perineural invasion, perforation, obstruction, retrieved lymph node < 12, and poorly differention. The median follow-up period was 68 months (1 to 205 months). RESULTS: The five-year overall survival (OS) rate was 90.1%, and the five-year disease-free survival (DFS) rate was 84.7%. Among high-risk patients, the OS and the DFS rates of the treatment group were significantly higher than those of the non-treatment group (OS: 90.6% vs. 69.1%, P < 0.0001; DFS: 85.9% vs. 54.1%, P < 0.0001). Among low-risk patients, the survival results of the treatment group were also significantly superior (OS: 97.7% vs. 88.2%, P < 0.0001; DFS: 93.0% vs. 80.0%, P = 0.001). In the multivariate analysis, adjuvant chemotherapy was a significantly favorable prognostic factor for overall survival (hazard ratio, 0.41; 95% confidence interval, 0.22 to 0.75; P = 0.004). CONCLUSION: In our population, adjuvant chemotherapy showed superior survival to curative surgery alone and significantly reduced the risk of death. A nationwide multicenter randomized trial is needed.
Chemotherapy, Adjuvant ; Colon ; Colonic Neoplasms ; Disease-Free Survival ; Fluorouracil ; Follow-Up Studies ; Humans ; Leucovorin ; Lymph Nodes ; Multivariate Analysis ; Prognosis ; Recurrence

PURPOSE: The present study was designated to sex, age, etiology of acute symptomatic seizures, which refer to the seizure caused by specific and transient pathophysiologic abnormalities in the central nervous system and other systems, and furthermore to analyze the incidence of acute symptomatic seizures before and during hospitalization. METHODS: The medical records of six hundred and ten convulsive children under fifteen years of age, who visited the Hallym University Sacred Heart Hospital from January 1999 to May 2001, were reviewed. One hundred and fourteen cases out of them were analyzed, and febrile seizures and unprovoked seizures were excluded. RESULTS: Among six hundred and ten children who had seizure during hospitalization, one hundred and fourteen(18.7%) had acute symptomatic seizures exclusive of febrile seizures and unprovoked seizures. The ratio of male to female was 1:2.1 and the peak age was three or less, accounting for 93.9%. Acute symptomatic seizures were caused by acute gastroenteritis(42.0%), metabolic/toxic disturbances(34.1%), cerebrovascular diseases(8.8%), CNS infections(8.0%), hypoxemia(4.4%), brain tumors(1.8%), and others(0.9 %). Remarkably, hypocalcemia and shaken baby syndrom were up to 82.1% of metabolic/ toxic distubances and 30.0% of cerebrovascular diseases, respectively. Among the one hundred and fourteen patients, 41.2% suffered from seizures before and during hospitalization and 11.4% did not before but did during hospitalization. CONCLUSION: Eighteen point seven percent of the cases of convulsions reviewed were classified into acute symptomatic seizures exclusive of febrile seizures and unprovoked seizures with the male to female ratio of 1:2.1 and high incidence age of three or less years. The leading causes of acute symtomatic seizures were acute gastroenteritis and hypocalcemia, comprising 70%. Shaken baby syrome and hyponatremia due to water intoxication can be prevented by public education about the danger, and central nervous system infection can be reduced by vaccine development and nationwide vaccination against the bacteria causing the central nervous system infection. In addition, appropriate prevention and management of seizure attacks are required for the patients with acute symptomatic seizures during hospitalization.
Bacteria ; Brain ; Central Nervous System ; Central Nervous System Infections ; Child* ; Education ; Female ; Gastroenteritis ; Heart ; Hospitalization ; Humans ; Hypocalcemia ; Hyponatremia ; Incidence ; Male ; Medical Records ; Seizures* ; Seizures, Febrile ; Vaccination ; Water Intoxication