Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

PURPOSE: Dendritic cells are antigen presenting cells(APC) that express class II major histocompatibility complex gene products on their surface. Recently, it was proved that dendritic cells activate antitumor immunity for intracranial germ cell tumor. The aim of the present study is to investigate the age-related changes of MHC class II-immunoreactive dendritic cells in the rat brain. METHODS: Male rats(Sprague-Dawley) were sacrificed at 1 month, 12 months and 24 months after birth. Brains were removed and sliced in rat brain matrix. Brain slices were cryosectioned coronally at interaural 5.70-6.70 mm. Brain tissue sections were immunohistochemically reacted with monoclonal MHC class II antibody. RESULTS: MHC class II-immunoreactive dendritic cells were observed in choroid plexuses and white matter(corpus callosum, cerebral peduncle and external capsule). The number of MHC class II-immunoreactive dendritic cells was slightly increased with age. As age increases, shapes of MHC class II-immunoreactive dendritic cells became more complex and aggregated together. CONCLUSION: As age increases, MHC class II-immunoreactive dendritic cells in choroid plexuses and white matter of the brain became not only more complex in shape, but also increased in number to improve immunity.
Aging ; Animals ; Brain* ; Choroid Plexus ; Dendritic Cells* ; Humans ; Major Histocompatibility Complex ; Male ; Neoplasms, Germ Cell and Embryonal ; Parturition ; Rats* ; Tegmentum Mesencephali

PURPOSE: This study was carried out to survey the serum anti-PRP titers after the completion of a primary series with 3 doses of the PRP-T conjugate vaccine(ACT-HIBTM), to evaluate the necessity of booster vaccination. METHODS: One hundred twenty healthy infants who went for consultations at Moon Hwa hospital between December 1999 to May 2001 were vaccinated at two, four and six months after birth. The serum antibody levels were measured at 7-8 months and 19-20 months of age by the "Farr" type of radioimmunological method at Aventis Pasteur International in France. RESULTS: The geometric mean of Anti-PRP titers were 24.6 microgram/mL at 7-8 months and then fell to 2.10 microgram/mL at 19-20 months. Minimum Anti-PRP titer was 0.75 microgram/mL at 7-8 months, and 0.1 microgram/mL at 19-20 months. Maximum Anti- PRP titer was 99.2 microgram/mL at 7-8 months, and 9.1 microgram/mL at 19-20 months. Subjects of Anti-PRP titers more than 0.15 microgram/mL were 100% at 7-8 months, and 97.0% at 19-20 months, and subjects of Anti-PRP titers more than 1.0 microgram/mL were 98.3% at 7-8 months, and 61.6% at 19-20 months. CONCLUSION: The Anti-PRP titers at 7-8 months were very high but rapidly decreased at 19-20 months of age, so the necessity of booster vaccination could be considered in Korean children.
Child ; France ; Haemophilus influenzae* ; Haemophilus* ; Humans ; Infant ; Parturition ; Referral and Consultation ; Vaccination*

PURPOSE: To determine the frequency of complications and diagnostic accuracy of CT-guided percutaneous automated gun biopsy, and to compare the results with those reported for fine needle aspiration. MATERIALS AND METHODS: Using automated biopsy devices, 118 CT-guided percutaneous biopsies of pulmonary lesions were performed. An 18-gauge needle was used. Final diagnosis was made with operation or other methods. We retrospectively analyzed the frequency of complications and diagnostic yields of 118 biopsies. RESULT: Four of 118(3.3%) patients developed pneumothorax and two of these required chest tube insertion. Other complications were resolved spontaneously. 106 biopsies (89.8%) yielded sufficient tissue for pathologic evaluation. For cases of malignant and of benign disease, sensitivity was 91.8% and 87.7% respectively ; the corresponding figures for diagnostic accuracy were 88.5% and 78.9%. CONCLUSION: CT-guided automated gun biopsy of the pulmonary lesions is safe, witha pneumothorax rate comparable to that of fine needle aspiration. In the absence of a trained cytologist at the time of biopsy, the diagnostic accuracy of automated gun biopsy of pulmonary lesions compared favorably with the reported accuracy of fine needle aspiration.
Biopsy* ; Biopsy, Fine-Needle ; Chest Tubes ; Diagnosis ; Needles ; Pneumothorax ; Retrospective Studies

The term myotubular myopathy (MTM) implies a maturational arrest of fetal muscle during the myotubular stage of development at 8-15 weeks of gestation. Characteristic muscle histopathology consists of small hypotrophic muscle fibers with centrally placed nuclei and a surrounding clear area devoid of myofibrils. X-linked recessive inheritance is the most common trait. Autosomal recessive and autosomal dominant forms are less frequently reported. The clinical diagnostic criterion for X-linked MTM has relied on a positive family history and the demonstration of the presence of characteristic biopsy findings from affected male subjects. Additional features may include perinatal onset, severe hypotonia, respiratory failure, dysphagia, thin ribs, contractures of the hips or knees, puffy eyelids and ophthalmoplegia. The prognosis is often fatal, and most patients die within the first year of life from respiratory failure. The authors report a case of presumed X-linked MTM with severe hypotonia, muscle weakness and respiratory failure at birth.
Biopsy ; Contracture ; Deglutition Disorders ; Eyelids ; Hip ; Humans ; Knee ; Male ; Muscle Hypotonia ; Myofibrils ; Myopathies, Structural, Congenital* ; Ophthalmoplegia ; Parturition ; Pregnancy ; Prognosis ; Respiratory Insufficiency ; Ribs ; Wills

Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is usually indolent. Although it was reported recently that about 20-30% cases of MALT lymphoma presented with a disseminated disease at diagnosis, it was described as a disease localized at diagnosis and remaining stable for a prolonged period. However, only a few cases of MALT lymphoma involved the lung and gastrointestinal tract all at once. We report a case of a 73-year-old man with disseminated MALT lymphoma. He presented with non-productive cough, initial chest radiograph showed a nodule in the right lower lobe. The diagnosis of stage IV MALT lymphoma was made by CT scan, video-assisted thoracoscopic excisional biopy, gastrofiberscopic biopsy and bone marrow biopsy. The lymphoma involved the lung, stomach and bone marrow at the time of diagnosis. Because he refused chemotherapy, he discharged after Helicobacter pylori eradication without chemotherapy. Regular follow-up examination did not show any evidence of disease progression over 22 months.
Aged ; Biopsy ; Bone Marrow ; Cough ; Diagnosis ; Disease Progression ; Drug Therapy ; Follow-Up Studies ; Gastrointestinal Tract ; Helicobacter pylori ; Humans ; Lung ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Radiography, Thoracic ; Solitary Pulmonary Nodule* ; Stomach ; Tomography, X-Ray Computed

ESRD patients have higher cardiovascular mortality risk than the general population. Increased QT dispersion has been shown to be a risk factor for cardiac arrhythmia in chronic hemodialysis patients with diabetes or arterial disease. It is known that QT dispersion is significantly prolonged in postdialysis than in predialysis. It is associated with a decrease in serum potassium. During hemodialysis, serum potassium is decreased and QT dispersion increases. Therefore ventricular arrhythmia frequently occurs in the latter half of dialysis. We report 2 cases of ventricular arrhythmia caused by potassium removal during dialysis in chronic hemodialysis patients.
Arrhythmias, Cardiac* ; Dialysis ; Humans ; Hypokalemia ; Kidney Failure, Chronic ; Mortality ; Potassium* ; Renal Dialysis* ; Risk Factors

Using PDC test, peritoneal function in CAPD patients was compared between diabetes and non- diabetes. PDC test were performed in 12 diabetic and 11 nondiabetic CAPD patients. Urea, creatinine, and albumin were measured in each dialysate effluent. Blood urea nitrogen, creatinine, glucose, albumin, and sodium were measured in blood sample at the start and the end of the study. Area parameter(Ao/d x), hydraulic conductance(LpS), final absorption rate (JVAR), large pore fluid flux(JVr.), urea generation rate(UGR), creatinine generation rate (CGR), protein nitrogen appearance/protein catabolic rate(PNA/PCR), residual renal Ccr, total Ccr(PD Ccr+renal Ccr), water removal via PD, total ultrafiltration, and PD clearance of urea, creatinine, vitamin B12 b2 microglobulin, and albumin were calculated. In diabetic patients(n=12), serum albumin was lower in patients on CAPD for less 6 months than patients on CAPD for more 15 months. But total ultrafiltration was lower in patients on CAPD for more 15 months. In non-diabetics(n=ll), protein loss across the peritoneum was lower in patients on CAPD for more 15 months. Other PDC parameters were not different in diabetic and non-diabetic patients according to CAPD duration. In patients on CAPD for less 6 months, serum albumin and PNA/PCR were lower in diabetics than non-diabetics.
Absorption ; Blood Urea Nitrogen ; Creatinine ; Diabetes Mellitus* ; Dialysis* ; Glucose ; Humans ; Nitrogen ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritoneum ; Serum Albumin ; Sodium ; Ultrafiltration ; Urea ; Vitamin B 12 ; Water

Teratomas are tumors composed of derivatives of all three germinal cell layers. They have been described in a variety of organs in both children and adults, most commonly in the gonads. Extragonadal intraperitoneal teratomas are very rare. We here report a case of a large mature teratoma of the gastrohepatic ligament in a 20-year-old woman. To the best of our knowledge, a teratoma of the gastrohepatic ligament has never before been described, although there are a few reports of teratomas arising from the greater and lesser omentum.
Adult ; Child ; Female ; Gonads ; Humans ; Ligaments* ; Omentum ; Teratoma* ; Young Adult

Cytomegalovirus infections (CMV) of the gastrointestinal tract (GI) are common, and most often seen in patients with acquired immunodeficiency syndrome (AIDS), inflammatory bowel disease, or those receiving immunosuppressive therapy. CMV enteritis is uncommon in an immunocompetent individual. A CMV infection of the small bowel accounts for 4.3% of all CMV infections of the GI tract. The GI manifestations of CMV include: diarrhea, bleeding, obstruction and perforation, all of which are usually secondary to discrete erosions or ulceration. High mortality rates have been reported for CMV enteritis. Here, a rare case of CMV enteritis, resulting in segmental ileal ischemia, is reported in a 47-year old man following a traffic accident. On the 17th hospital day, he developed melena, watery diarrhea, fever and abdominal pain. An abdominal computed tomography (CT) on the 23rd hospital day showed an enlarged appendix with mild periappendiceal infiltration and segmental wall thickening in the terminal ileum. An ileocecal resection was performed. Pathological evaluation of the operative specimen revealed CMV inclusion bodies, with ulcerations. The patient was treated with ganciclovir therapy for 3 weeks after which his symptoms improved. If a CMV infection is highly suspected in multiply injured trauma victims, the earlier recognition of potential small bowel involvement can hopefully decrease the incidence of bleeding, ischemic demage to the bowel and perforation, which are usually fatal events.
Abdominal Pain ; Accidents, Traffic* ; Acquired Immunodeficiency Syndrome ; Appendix ; Cytomegalovirus Infections* ; Cytomegalovirus* ; Diarrhea ; Enteritis ; Fever ; Ganciclovir ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Ileum ; Incidence ; Inclusion Bodies ; Inflammatory Bowel Diseases ; Ischemia* ; Melena ; Middle Aged ; Mortality ; Multiple Trauma* ; Ulcer