No abstract available.
Ebstein Anomaly*

This presentation is a case of osteomalacia, the cause of which was a nutritional deficiency. The patient was a 42-year-old female who had pseudofractures of ribs, ulnae, pelvic raml, the right second metacarpai bone and both femoral necks. Following treatment by medication combined with internal fixation of the femoral necks, the result was good.
Adult ; Female ; Femur Neck ; Humans ; Malnutrition ; Osteomalacia ; Ribs ; Ulna

No abstract available.
Diagnosis* ; Hemoglobinuria*

We report a case of primary cardiac angiosarcoma in a 35 year-old woman. She presented with dyspnea, facial edema and neck vein distension during 1 month. The oval round large mass(8x4 cm) filling the right atrium infiltrated into the entire thickness of the lateral wall and extended to the vena cava and ventricle. The interatrial septum and pericardium seemed to be intact. Despite the surgical excision and adjuvant chemo-and radiotherapy, the patient died 8 months later due to tumor recurrence. The histologic findings of tumor varied from benign looking capillary proliferation mimicking granulation tissue to sarcoma composed of spindle cells. But anastomosing vascular channels lined by anaplastic polygonal cells and intracytoplasmic lumen containing red blood cells were characteristic findings. These tumor cells showed positive reaction to endothelial cell antigen and factor VIII-related antigen. The intercellular junctional complex and vascular channels formed by cytoplasmic process of tumor cells were identified by the ultrastructural study.
Female ; Humans

Neonatal Gaves disease is a relatively rare condition due to transplacental passage of Thyroid-stimulating antibody(TSAb) from a mother with active or inactive Graveses disease or autoimmune thyroiditis. A 11-day-old female newborn was referred to our department of pediatrics from a local clinic because of low level T4(3.55microg/dl) concurrent with high level TSH (501.74uIU/ml) on the 5th day neonatal metabolic screening. But, our repeated laboratory data showed very high serum T4(59.6microg/dl), T3(1,600ng/dl), suppressed TSH(0.43uIU/ml), and the presence of TSH receptor antibody. Her mother was treated with propylthiouracil(PTU) for Graves disease during pregnancy. Therefore, we thought it was a delayed-onset neonatal hyperthyroidism, because the fetal thyroid gland was initially suppressed by antithyroid drug taken during pregnancy. After initiating antithyroid drug therapy for the hyperthyroid nature, TSH levels became elevated again, while thyroid hormone levels decreased. Maternal and infant blood samples at the 23th day after birth were examined for serum autoantibodies directed towards the TSH receptor(Thyrotropin-binding inhibitory immunoglobulin:TBII, Thyroid-stimulating antibody:TSAb, Thyroid-stimulating blocking antibody:TSBAb) and high levels of TBII and TSAb were detected. About 2 months after birth, TBII and TSAb decreased within normal limit, and then we could stop antithyroid medication in safety. We report here a case of neonatal Graveses disease with very high level of T4 and T3, but firstly presented as hypothyroid nature on neonatal screening because of the maternally transferred antithyroid drug, PTU.
Autoantibodies ; Drug Therapy ; Female ; Graves Disease ; Humans ; Hyperthyroidism ; Infant ; Infant, Newborn ; Mass Screening ; Mothers ; Neonatal Screening ; Parturition ; Pediatrics ; Pregnancy ; Receptors, Thyrotropin ; Thyroid Gland ; Thyroiditis, Autoimmune

The gross tissue specimens are a valuable aid to the teaching of pathology and anatomy. However, traditional methods for storage and handling of them are discouragingly difficult and, recently, minimal surgical resections as well as preoperative interventions make it more difficult to have instructive gross specimens. Plastination is a process of tissue preservation by impregnation with silicone polymers or epoxy resins. The process in our study involves dehydration by cryosubstitution in aceton, defatting, forced impregnation of silicon polymer in a vacuum, curing and finishing. We submitted 40 surgically resected specimens to plastination. The resulting specimens are odorless, relatively dry, durable, life-like, non-hazardous, maintenance-free, and do not deteriorate with time. Plastinated specimens are a useful adjunct to the teaching of pathology, particularly suited for use in small groups, and appropriate method of tissue preservation. They are much preferred to wet preparation and conventional pots by both students and teachers owing to their accessibility, superior illustrative powers, and comparative ease of interpretation.
Dehydration ; Epoxy Resins ; Humans ; Pathology* ; Polymers ; Silicones ; Tissue Preservation ; Vacuum

No abstract available.
Uterus*

No abstract available.
Cardiac Catheterization* ; Cardiac Catheters* ; Child* ; Cineangiography* ; Heart Diseases* ; Heart* ; Humans ; Statistics as Topic*

Hypothermia is widely acknowledged as fundamental component of myocardial protection during cardiac operations. Although it prolongs the period of ischemic arrest by reducing oxygen demands, hypothermia is associated with a number of major disadvantages, including its detrimental effects on enzymatic function, energy generation, and cellular integrity. The ideal way to rotect the heart is to electromechanically arrest it and perfus it with blood that is aerobic arrest. However alternative technique has been developed, based on the principles of electromechanical arrest and normothermic aerobic perfusion using continuous warm blood cardioplegia. To determine if continuous warm blood cardioplegia was beneficial in clinical practice during valvular surgery, we studied two groups of patients matched by numbers and clinical characteristics. Group included is 31 patients undergoing valvular surgery who received intermittent cold crystalloid cardioplegia. Group II included 30 patients undergoing valvular surgery who received continuous warm blood cardioplegia. Our results suggest that the heartbeat in 100% of patients treated with continuous warm blood cardioplegia converted to normal sinus rhythm spontaneously after the removal of the aortic cross-clamp, compared to only 31% of the cold cardioplegia group. After operation, pericardial closure rate was 90% area in the warm group, compared to 35% area in the cold group. 12 hours after the operation, the total amount of urine output in the warm group was greater than that in the cold group (2863+/-127 ml versus 2257+/-127 ml; p<0.05). After the operation, left diaphragmatic elevation developed in 55% of the cold group but in 0% of the warm group. CK-MB level in the warm group was significantly lower than cold group (2.28+/-0.62 versus 9.96+/-2.12; p<0.01) 1 hour after operation and CK-MB level in the warm group was significantly lower than cold group (1.80+/-1.01 versus 6.00+/-1.74; p<0.05) 12hours after operation. Continuous warm blood cardioplegia is at least as safe and effective as hypothermic technique in patients undergoing cardiac valvular surgery. Conceptually, this represents a new approach to the problem of maintaining myocardial preservation during cardiac operations.
Heart Arrest ; Heart Arrest, Induced* ; Heart* ; Humans ; Hypothermia ; Oxygen ; Perfusion ; Thoracic Surgery*

Acute spontaneous tumor lysis syndrome is a condition resulting from the rapid release of cellular breakdown products into the circulation due to massive cytolysis occurring before start of treatment. It occurs most often in Burkitt's lymphoma and T-cell ALL, both of which have been well described, following cytotoxic chemotherpy and other single agent administrations. However, only a handful of spontaneous acute tumor lysis syndrome cases have recently been reported in the literature. We have recently experienced a case of a T-cell ALL in a 9-year-old girl presented with superior vena cava syndrome(SVC syndrome) and uric acid nephropathy due to acute spontaneous tumor lysis syndrome. On admission, she had marked elevation in serum uric acid, BUN and creatinine. She was given dexamethasone administration as a treatment of SVC syndrome and was supported with allopurinol therapy, hyperhydration and alkalinization of urine. On day 7 following continuous dexamethasone administration, her BUN and creatinine returned to normal and no blast cells was found in peripheral blood. Then, ALL induction chemotherapy (CCG-1882 protocol) was used, resulting in complete remission.
Allopurinol ; Burkitt Lymphoma ; Child ; Creatinine ; Dexamethasone ; Female ; Hand ; Humans ; Induction Chemotherapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Superior Vena Cava Syndrome ; T-Lymphocytes* ; Tumor Lysis Syndrome* ; Uric Acid ; Vena Cava, Superior