We report an autopsy case of secondary hemochromatosis associated with multiple frequent blood transfusion for the treatment of aplastic anemia. A 23-year-old man had been diagnosed as having aplastic anemia at the age of 13. He received a whole blood transfusion, about 1280 ml, every month during the past 10 years. Recently he developed diabetes mellitus and a congestive heart failure. The autopsy revealed that multiple organs were affected by secondary hemochromatosis, including the liver, heart, pancreas, spleen, bone marrow, stomach, thyroid gland, adrenal glands, and testes. The lungs and liver showed gross and microscopic findings consistent with a congestive heart failure in addition to hemochromatosis. The details are presented. This is a case of rare secondary hemochromatosis occurring in a young man and presenting the classic histopathologic changes indistinguishable from those of primary hemochromatosis.
Adrenal Glands ; Anemia ; Anemia, Aplastic* ; Autopsy* ; Blood Transfusion ; Bone Marrow ; Diabetes Mellitus ; Heart ; Heart Failure ; Hemochromatosis* ; Humans ; Liver ; Lung ; Pancreas ; Spleen ; Stomach ; Testis ; Thyroid Gland ; Young Adult

Osteochondrodysplasia is a heterogeneous group of disorders appearing short limbed dwarfism. Because many of these entities are lethal and hereditary, an accurate diagnosis is mandatory. The purpose of this study is to define the clinicopathologic features and radiologic findings of osteochondrodysplasia. We reviewed 29 autopsy cases of congenital short limbed dwarfism, consisting of thanatophoric dysplasia (TD) (12 cases), osteogenesis imperfecta (OI) (12 cases), asphyxiating thoracic dysplasia (ATD) (3 cases), short-rib-polydactyly syndrome (SRPS) (1 case) and hypochondrogenesis (1 case). The gestational age ranged from 16 to 41 weeks. Of 6 fetuses that were born alive, 3 were ATD, 2 were TD and 1 was hypochondrogenesis. TD was frequently complicated by hydramnios. Of 8 cases studied chromosomally, only 1 showed chromosomal abnormality -46XY, inv 9. Intrauterine growth retardation was frequently associated with OI. Pulmonary hypoplasia was present in 23 cases (79%), including all cases of ATD, SRPS and hypochondrogenesis, 11 in TD and 7 in OI. Other associated anomalies were present in 17 cases (59%).
Autopsy* ; Chromosome Aberrations ; Diagnosis ; Dwarfism ; Extremities ; Fetal Growth Retardation ; Fetus ; Gestational Age ; Osteochondrodysplasias* ; Osteogenesis Imperfecta ; Polyhydramnios ; Thanatophoric Dysplasia

This study was carried out to find out specific proteins from different organs of Clonorchis sinensis. Crude extract, organ-specific and excretory-secretory (ES) proteins were analyzed by immunoblot with infected human sera. The bands of 7- and 17-kDa were main component of intestinal fluid and ES protein and commonly found in all organspecific proteins. The 17-kDa protein was observed from ES antigen, intestinal fluid, eggs and sperms, 26- and 28- kDa proteins were from the uterus, vitellaria, and ovary, and 34-, 37-, 43- and 50-kDa proteins were mainly from the testis and sperms. Serum of mice immunized with sperms reacted to the 50-kDa protein by immunoblotting and immunohistochemical staining showed a positive reaction at the seminal receptacle and seminiferous tubule. The present results show that the 7-kDa protein is a common antigen of every part or organ of C. sinensis, but different organs express their specific antigenic protein bands.
Animals ; Antigens, Helminth/*analysis ; Clonorchis sinensis/anatomy & histology/*immunology ; Female ; Humans ; Male ; Mice ; Mice, Inbred BALB C ; Rabbits ; Research Support, Non-U.S. Gov't

Posterior reversible encephalopathy syndrome (PRES) is a transient condition characterized by altered mental status, seizure, headache, and visual disturbance with typical neuro-imaging findings in the bilateral parieto-occipital regions. Clinicians should be aware of this syndrome because delayed diagnosis and treatment result in irreversible neurologic deficits. We present the case of a 77-year-old male diagnosed with PRES in the setting of postoperative critical illness caused by small-bowel strangulation.
Aged ; Critical Illness* ; Delayed Diagnosis ; Headache ; Humans ; Intensive Care Units ; Male ; Neurologic Manifestations ; Posterior Leukoencephalopathy Syndrome* ; Postoperative Period ; Seizures

Anaphylactic reaction during the perioperative period typically exhibits rapid onset, varying clinical manifestations, and an expected mortality rate of 1.5-9%. Neuromuscular blocking agents are the leading cause of perioperative anaphylaxis. Here, we report a severe case of anaphylaxis that developed in a 66-year-old man due to cisatracurium administration. And he was successfully managed by extracorporeal membrane oxygenation. Cardiopulmonary resuscitation was performed by extracorporeal membrane oxygenation, and the patient was successfully weaned off 24 hours later.
Aged ; Anaphylaxis* ; Cardiopulmonary Resuscitation ; Extracorporeal Membrane Oxygenation* ; Heart Arrest* ; Humans ; Mortality ; Neuromuscular Blocking Agents ; Perioperative Period

OBJECTIVES: The present study was performed to investigate whether estrogen and progesterone induce the change of vascular tone in endothelium-denuded human uterine artery and vascular reactivity may be mediated by intracelluar calcium modulation through receptor- and voltage-dependent calcium channels. METHODS: The uterine arteries were obtained at the time of hysterectomy from 28 women followed by denudation of endothelium. After confirmation of functional integrity of endothelium-denuded uterine artery, vascular reactivity was measured by using isometric force transducer and recorded by physiograph. Contraction was induced by 10-6 M norepinephrine and 35mM high concentrated potassium chloride solution which activated receptor-dependent calcium channel and voltage-dependent calcium channel, respectively.Thereafter estradiol of 4 different concentrations from 3x10-11M to 3x10-8M was administered. Progesterone was also administered to endothelium-denuded uterine artery which was contracted by 10-6M norepinephrine and high potassium chloride solution. To evaluate the effect of additional progesterone on vascular smooth muscle relaxation effect of estrogen,4 different progesterones in concentrations from 3x10-8M to 3x10-5M were given to vascular smooth muscle which was initially pretreated with norepinephrine followed by relaxation of estradiol. RESULTS: Estradiols from 3x10-11M to 3x10-8M showed in significant dose-dependent vascular relaxation. Progesterones result in significant decrease in vascular contraction in concentration dependent manner. Additional progesterone on estrogenic effects also results in significant decrease in vascular contraction. CONCLUSION: Estradiol may have endothelium independent vasorelaxation effect in human uterine artery. These vasorelaxant effects may be mediated through antagonistic action for receptor-and voltage-dependent calcium channels in vascular smooth muscle. Progesterone also bring about vasorelaxation by same action in endothelium-denuded vascular smooth muscle. On estrogen induced vascular relaxation, progesterone results in additional vasorelaxation.
Calcium ; Calcium Channels ; Endothelium ; Estradiol ; Estrogens* ; Female ; Humans* ; Hysterectomy ; Muscle, Smooth, Vascular ; Norepinephrine ; Potassium Chloride ; Progesterone* ; Relaxation ; Transducers ; Uterine Artery* ; Vasodilation

No abstract available.
Foreign Bodies* ; Skull Base* ; Skull*

No abstract available.
Sinusitis*

Animal models for human chronic pain syndromes were developed and widely used for pain research. One of thsese neuropathic pain model by Kim and Chung[1992] has many advantages for operation and pain elicitation. We have examined the c-fos protein, substance P, CGRP immunoreactivity in dorsal root ganglia and dorsal horn in this neuropathic model. About 50 Sprague-Dawley rats were used for this study. L5 and L6 spinal nerve were ligated tightly to produce neuropathic pain model. After 2, 4, 8, 16, 24 hours and 1 week of surgery, rats were anesthesized and sacrificed by perfusion through the left ventricle with saline followed by 0.1M phosphate buffer[pH 7.4] containing 3% paraformaldehyde, 3% glutaraldehyde, and 0.1% picric acid. After confirmation of the roots transected by the surgery, the L5 and L6 dorsal root ganglion and spinal cord were removed and processed for immunohistochemistry. All tissue sections were immunohistochemically stained for substance P, CGRP and c-fos by using the peroxidase-antiperoxidase[PAP] method. Count the number of immunostained substance P and CGRP dorsal root ganglion cells and c-fos immunoreactive dorsal horn cells and analyzed statistically with Mann-Whitney U test. The results are as follows. 1. The number of c-fos protein immunoreactive neurons in the superficial layer of dorsal horn were increased markedly at 2 hours after operation, gradually decreased to normal level 1 week after operation. 2. The number of c-fos protein immunoreactive neurons in the deep layer of dorsal horn were gradually increased to the peak 24 hours after operation, decreased to normal level 1 week after operation. 3. The number of substance P and CGRP immunoreactive L5 and L6 dorsal root ganglion neurons were decreased markedly at 1 week after pain model operation. In conclusion, after neuropathic pain model operation, c-fos protein were immediately expressed in the superficial layer of spinal dorsal horn, thereafter c-fos protein in the deep layer of spinal dorsal horn were expressed. CGRP and substance P immunoreactive neurons were decreased markedly 1 week after neuropathic pain model operation.
Animals ; Chronic Pain ; Ganglia, Spinal* ; Glutaral ; Heart Ventricles ; Horns* ; Humans ; Immunohistochemistry ; Models, Animal ; Neuralgia ; Neurons* ; Perfusion ; Peripheral Nervous System Diseases* ; Posterior Horn Cells ; Rats* ; Rats, Sprague-Dawley ; Spinal Cord ; Spinal Nerve Roots* ; Spinal Nerves ; Substance P

No abstract available.
Dermatitis, Atopic* ; Lupus Erythematosus, Systemic*