Craniofacial clefts are rare among facial anomalies with an incidence of 1.5 to 5 per 100,000 births, and 1 per 100 cases of cleft lip and palate. The Tessier No. 7 clefts are unusual lesions that result from failure of the embryonic mandibular and maxillary processes to properly fuse and form the corners of the mouth. We experienced a case of Tessier No. 7 craniofacial cleft in a 1 day-old female patient who presented with a macrostomia and auricular malformation. The diagnosis was established by clinical and radiographic findings. A brief review of literature was made.
Cleft Lip ; Diagnosis ; Female ; Humans ; Incidence ; Macrostomia ; Mouth ; Palate ; Parturition

PURPOSE: We investigated the epidemiological and clinical characteristics of diabetes mellitus developed in aplastic anemia patients who have had many blood transfusions. METHODS: We retrospectively reviewed medical records of 170 patients with aplastic anemia who were diagnosed before 15 years of age in Department of Pediatrics, The Catholic University of Korea from 1987 to 2001. We obtained their medical history, family history of diabetes mellitus, clinical onset of diabetes mellitus and the successive history, and coexistence of other disorders. RESULTS: Diabetes mellitus was diagnosed in 8 of 107 patients (7.5%) with severe aplastic anemia in childhood. The mean age of diagnosis of diabetes mellitus was 18.5+/-5.2 years, and the mean duration from the start of blood transfusion to the diagnosis of diabetes mellitus was 7.7+/-2.9 years. Duration of multiple blood transfusions was a major risk factor for the development of diabetes mellitus in severe aplastic anemia patients. There was a wide range of symptoms at clinical onset of diabetes mellitus from asymptomatic hyperglycemia to diabetic ketoacidosis. Incidence of other complications, such as hepatic impairment (88%) and cardiac dysfunction (75%), was high in patients who developed diabetes mellitus. CONCLUSION: Severe aplastic anemia patients treated with prolonged, multiple transfusions have a significant risk of developing insulin-deficient diabetes mellitus. These patients are at risk for other complications, such as hepatic, cardiac, or thyroid disorders. Early prevention of iron overload and screening for transfusion-related complications are very important in these patients.
Anemia, Aplastic* ; Blood Transfusion ; Diabetes Mellitus* ; Diabetic Ketoacidosis ; Diagnosis ; Humans ; Hyperglycemia ; Incidence* ; Iron Overload ; Korea ; Mass Screening ; Medical Records ; Pediatrics ; Retrospective Studies ; Risk Factors ; Thyroid Gland

Systemic lupus erythematosus (SLE) is an autoimmune disease which may affect different organs and disclose various clinical manifestations. The clinical manifesations of central nervous system involvement in SLE are highly variable and range from mild cognitive dysfunction, movement disorder, headache, psychosis to life-threatening stroke and coma. Among neuropsychiaric disorders encountered in patients with SLE, cerebrovascular disease has relatively been rare complication. We experienced a case of subdural hematoma (SDH) occurring in a SLE patient which presented with headache. She was diagnosed as SDH by neuropsychiatric symptoms, brain CT, and brain MRI, and showed good response to medical treatment.
Autoimmune Diseases ; Brain ; Central Nervous System ; Coma ; Headache ; Hematoma, Subdural* ; Humans ; Lupus Erythematosus, Systemic* ; Magnetic Resonance Imaging ; Movement Disorders ; Psychotic Disorders ; Stroke

BACKGROUND AND OBJECTIVES: To determine the diagnostic clues to differentiate tuberculous lymph node infection from nodal metastasis in patients with both papillary thyroid carcinoma and cervical tuberculous lymphadenitis. SUBJECTS AND METHOD: We retrospectively reviewed 11 patients suffering concurrently from papillary thyroid carcinomas with cervical tuberculous lymphadenitis. Nine of the 11 patients underwent preoperative neck ultrasonography (US) and seven CT scans. Using the surgical pathology as the reference standards, the results of the preoperative diagnostic tools were re-evaluated according to lymph node level-based analysis. US and CT features were also compared between metastatic nodes and tuberculous lymphadenitis. RESULTS: Preoperative CT could localize the involved lymph node levels and differentiate tuberculous infection from metastasis of thyroid carcinoma in only two of seven patients. The site of the involved lymph nodes, the presence of pulmonary tuberculosis, and the tumor volume of the thyroid carcinoma were the clues to diagnose the lymph node status. However, in five of seven cases, CT could not differentiate tuberculosis from metastasis in the lymph nodes. The morphological characteristics of lymph nodes seen on CT and US did not differ between tuberculous infection and metastasis of papillary thyroid carcinomas. CONCLUSION: Pre-operative CT or US does not provide differential information about lymph node status between tuberculous infection and metastasis in patients with concurrent papillary thyroid carcinomas and cervical tuberculous lymphadenitis. Rather, clinical characteristics such as the site of the involved lymph nodes, the primary tumor burden, and the associated clinical features can help the physician differentiate between them.
Carcinoma ; Humans ; Lymph Nodes ; Lymphatic Metastasis ; Neck ; Neoplasm Metastasis ; Pathology, Surgical ; Retrospective Studies ; Stress, Psychological ; Thyroid Gland ; Thyroid Neoplasms ; Tuberculosis ; Tuberculosis, Lymph Node ; Tuberculosis, Pulmonary ; Tumor Burden

Complications of acute pancreatitis usually occur in pancreas and its contiguous organs. The prevalence of colonic invasion is rare, however, the consequence is fatal, with mortality above 50%. The initial symptoms and onset times are variable and major affected sites are transverse colon and splenic flexure. The spread of inflammatory exudates into the colon is the main mechanism of colonic invasion. If the colonic stenosis develops, it is necessary to manage it surgically. We report a case who arrived at the hospital with watery diarrhea and abdominal distension in the recovery period of acute alcoholic pancreatitis and was diagnosed as a colonic obstruction in the splenic flexure. The patient underwent loop ileostomy instead of the resection of the lesion because of severe adhesion around the splenic flexure. The patient died due to sepsis 5 days after the operation.
Acute Disease ; Colonic Diseases/*complications ; English Abstract ; Humans ; Intestinal Obstruction/*complications ; Male ; Middle Aged ; Pancreatitis/*complications

BACKGROUND/AIMS: Alcohol and the hepatitis B virus (HBV) exert synergistic effects in hepatocelluar carcinogenesis. We aimed to elucidate the clinical significance of the antibody to hepatitis B core antigen (anti-HBc) and occult HBV infection on the development of hepatocellular carcinoma (HCC) in patients with alcoholic liver cirrhosis (LC). METHODS: Patients with alcoholic LC alone (n=193) or combined with HCC (n=36), who did not have HBsAg or antibody to hepatitis C virus were enrolled. Clinical data and laboratory data including anti-HBc were investigated at enrollment. The polymerase chain reaction was applied to HBV DNA using sera of patients with HCC or LC after age and sex matching. RESULTS: Patients with HCC were older (60+/-11 years vs. 53+/-10 years, mean+/-SD, P<0.001), more likely to be male (100% vs. 89%, P=0.03), and had a higher positive rate of anti-HBc (91.2% vs. 77.3%, P=0.067), and a higher alcohol intake (739+/-448 kg vs. 603+/-409 kg, P=0.076) than those with LC. Age was the only significant risk factor for HCC revealed by multiple logistic regression analysis (odds ratio, 1.056; P=0.003). The positive rate of anti-HBc and alcohol intake did not differ in age- and sex-matched subjects between the LC (n=32) and HCC (n=31) groups. However, the detection rate of serum HBV DNA was higher in the HCC group (48.4%) than in the LC group (0%, P<0.001). CONCLUSIONS: Anti-HBc positivity is not a risk factor for HCC. However, occult HBV infection may be a risk factor for HCC in patients with alcoholic LC.
Adult ; Aged ; Antibodies, Viral/blood ; Carcinoma, Hepatocellular/diagnosis/epidemiology/*etiology ; DNA, Viral/analysis ; Female ; Hepatitis B/*complications/diagnosis ; Hepatitis B Core Antigens/*immunology ; Hepatitis B Surface Antigens/immunology ; Hepatitis B virus/genetics/immunology/isolation & purification ; Hepatitis C/complications/diagnosis ; Humans ; Liver Cirrhosis, Alcoholic/*complications/diagnosis/epidemiology ; Liver Neoplasms/diagnosis/epidemiology/*etiology ; Male ; Middle Aged ; Risk Factors

BACKGROUND/AIMS: Alcohol may be a cocarcinogen in patients with chronic viral hepatitis. We investigated the effect of alcohol on the development of hepatocellular carcinoma (HCC) in liver cirrhosis (LC) caused by hepatitis B virus (HBV). METHODS: All patients with LC or HCC associated with HBV or alcohol, admitted between March 2001 and June 2005, were included. Patients were divided into three groups according to the etiology of LC: Alcohol (AL), HBV, or HBV+alcohol (HBV+AL). Age and laboratory data at the enrollment of study were analyzed. The logistic regression coefficiency for the prevalence of HCC was calculated by using variables such as age, gender, serologic markers, and etiology of LC. RESULTS: In LC patients (n=342), the proportions of AL, HBV, and HBV+AL groups were 44%, 39%, and 17%, respectively. The proportions of HCC in AL, HBV and HBV+AL groups were 17%, 55%, and 76%, respectively. Age at the diagnosis of HCC was younger in HBV+AL than in AL group (p=0.036). In logistic regression analysis for the risk factor of HCC, odds ratio of age was 1.056 (p<0.001). Odds ratios of HBV and HBV+AL group comparing AL were 8.449 (p<0.001) and 17.609 (p<0.001), respectively. Therefore, old age and chronic alcohol intake in patients with HBsAg were the risk factors of HCC. CONCLUSIONS: Chronic alcohol intake may be an additive factor for the development of HCC in patient with LC caused by HBV. However, a prospective cohort study is needed to confirm these findings.
Adult ; Aged ; Carcinoma, Hepatocellular/*epidemiology/etiology ; Cross-Sectional Studies ; Female ; Hepatitis B, Chronic/*complications/epidemiology ; Hepatitis, Alcoholic/complications/epidemiology ; Humans ; Liver Cirrhosis/*complications/virology ; Liver Cirrhosis, Alcoholic/*complications/epidemiology/virology ; Liver Neoplasms/*epidemiology/etiology ; Male ; Middle Aged ; Odds Ratio ; Regression Analysis ; Retrospective Studies ; Risk Factors

Obstructive jaundice is most commonly attributed to a malignancy or stones affecting the common bile duct. Biliary tuberculosis and lymphadenitis around the periportal area have also been implicated but cases are quite rare. A 24 year old man presented with jaundice and abdominal pain for 3 days. Abdominal CT and ERCP revealed a stricture of the extrahepatic bile duct with multiple enlarged lymph nodes showing necrotic foci located at the periportal area. The colonoscopic biopsy showed evidence of M. tuberculosis. The patient was treated with ERBD insertion and oral anti-tuberculosis therapy. However, the abdominal pain recurred and there was progressive stenosis of the common bile duct. A bile duct resection with choledochojejunostomy was subsequently performed. Frozen sections revealed granulomatous inflammation with caseation necrosis, which was consistent with tuberculosis. We report a case of tuberculous cholangitis and lymphadenitis with obstructive jaundice that was managed surgically due to the progressive stricture of the bile duct.
Abdominal Pain ; Bile Ducts* ; Bile Ducts, Extrahepatic ; Bile* ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis* ; Choledochostomy ; Common Bile Duct ; Constriction, Pathologic* ; Frozen Sections ; Humans ; Inflammation ; Jaundice ; Jaundice, Obstructive* ; Lymph Nodes ; Lymphadenitis* ; Necrosis ; Tomography, X-Ray Computed ; Tuberculosis ; Tuberculosis, Lymph Node ; Young Adult