Holoprosencephaly is a developmental malformation complex of forebrain and midface which arises from incomplete cleavage of the embryonic forebrain. It is subdivided into alobar, semilobar and lobar types based on the degree of growth disturbance within the anterior wall of the telencephalon, particularly in the midline. Cyclopia is the most severe form of alobar holoprosencephaly presenting a single median eye and a blind-ending proboscis usually located above the eye. We report a case of alobar holoprosencephaly with cyclopia and proboscis in premature infant.
Holoprosencephaly* ; Humans ; Infant, Newborn ; Infant, Premature ; Prosencephalon ; Telencephalon

The effect of electroacupuncture (EA) on experimental colitis was investigated in Sprague-Dawley rats. Colitis was induced by intracolonic instillation of 4% acetic acid. EA (2 Hz, 0.05 ms, 2 V for 20min) was applied to bilateral Hoku (LI-4) and Zusanli (ST-36) on 12 hrs and 36 hrs after induction of colitis. EA-treatment significantly reduced the macroscopic damage and the myeloperoxidase activity of colonic samples at 3 days post-induction of colitis. Colitic colon showed a decreased in vitro motility. However, colonic motility of EAtreated group was not significantly different from that of normal group. The anti-inflammatory effect of EA was not inhibited by a glucocorticoid receptor antagonist, RU-486, but suppressed by a beta-adrenoceptor antagonist, propranonol. These results suggest that EA-treatment has a beneficial effect on colitis, and its anti-inflammatory effect is mediated by beta-adrenoceptor activation but not by endogenous glucocorticoiddependent mechanism.
Acetic Acid ; Adrenergic beta-Antagonists/pharmacology ; Animals ; Carbachol/pharmacology ; Cholinergic Agonists/pharmacology ; Colitis/chemically induced/enzymology/pathology/*therapy ; Electroacupuncture/*veterinary ; Enzyme Inhibitors/metabolism ; Gastrointestinal Motility/physiology ; Hormone Antagonists/pharmacology ; Male ; Mifepristone/pharmacology ; Muscle Contraction/physiology ; Muscle, Smooth/drug effects/physiology ; NG-Nitroarginine Methyl Ester/pharmacology ; Peroxidase/metabolism ; Propranolol/pharmacology ; Rats ; Rats, Sprague-Dawley

Gastric adenocarcinoma is the second most common cause of cancer death worldwide, but there are some geographical differences in its incidence. Gastointestinal stromal tumor (GIST) is an uncommon disease with a wide spectrum of aggressive behavior. These two tumors have a distinct pathogenesis. GIST is frequently identified as an incidental lesion found by routine endoscopy or in resection specimens that are removed for other reasons. We report a case of a gastric adenocarcinoma mimicking GIST in a 79-year-old woman. GIST was suggested by endoscopic ultrasonography and computed tomography scanning, but gastric adenocarcinoma was confirmed by gastroduodenoscopic biopsy. We performed a subtotal gastrectomy with Billroth I, lymph node dissection and transverse colon segmentectomy, and a 15.0x7.5x5.5 cm-sized gastric tumor was confirmed pathologically. Immunohistochemistry was positive for carcinoembryonic antigen and focal weakly positive for cytokeratin7 and cytokeratin20.
Adenocarcinoma ; Aged ; Biopsy ; Carcinoembryonic Antigen ; Colon, Transverse ; Endoscopy ; Endosonography ; Female ; Gastrectomy ; Gastroenterostomy ; Gastrointestinal Stromal Tumors ; Humans ; Immunohistochemistry ; Incidence ; Lymph Node Excision ; Mastectomy, Segmental

PURPOSE: Intravenous lipid emulsions have been used to treat the systemic toxicity of local anesthetics. The goal of this in vitro study was to examine the effects of lipid emulsions on the norepinephrine-mediated reversal of vasodilation induced by high doses of levobupivacaine, ropivacaine, and mepivacaine in isolated endothelium-denuded rat aorta, and to determine whether such effects are associated with the lipid solubility of local anesthetics. MATERIALS AND METHODS: The effects of lipid emulsions (0.30, 0.49, 1.40, and 2.61%) on norepinephrine concentration-responses in high-dose local anesthetic (6x10-4 M levobupivacaine, 2x10-3 M ropivacaine, and 7x10-3 M mepivacaine)-induced vasodilation of isolated aorta precontracted with 60 mM KCl were assessed. The effects of lipid emulsions on local anesthetic- and diltiazem-induced vasodilation in isolated aorta precontracted with phenylephrine were also assessed. RESULTS: Lipid emulsions (0.30%) enhanced norepinephrine-induced contraction in levobupivacaine-induced vasodilation, whereas 1.40 and 2.61% lipid emulsions enhanced norepinephrine-induced contraction in both ropivacaine- and mepivacaine-induced vasodilation, respectively. Lipid emulsions (0.20, 0.49 and 1.40%) inhibited vasodilation induced by levobupivacaine and ropivacaine, whereas 1.40 and 2.61% lipid emulsions slightly attenuated mepivacaine (3x10-3 M)-induced vasodilation. In addition, lipid emulsions attenuated diltiazem-induced vasodilation. Lipid emulsions enhanced norepinephrine-induced contraction in endothelium-denuded aorta without pretreatment with local anesthetics. CONCLUSION: Taken together, these results suggest that lipid emulsions enhance the norepinephrine-mediated reversal of local anesthetic-induced vasodilation at toxic anesthetic doses and inhibit local anesthetic-induced vasodilation in a manner correlated with the lipid solubility of a particular local anesthetic.
Amides/adverse effects ; Anesthetics, Local/*adverse effects ; Animals ; Bupivacaine/adverse effects/analogs & derivatives ; Emulsions/*chemistry/*therapeutic use ; Lipids/*chemistry ; Male ; Mepivacaine/adverse effects ; Norepinephrine/*therapeutic use ; Rats ; Rats, Sprague-Dawley ; Vasodilation/*drug effects

BACKGROUND AND PURPOSE: 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome. Epilepsy and other neuropsychiatric (NP) manifestations of this genetic syndrome are not uncommon, but they are also not well-understood. We sought to identify the characteristics of epilepsy and other associated NP manifestations in patients with 22q11.2DS. METHODS: We retrospectively analyzed the medical records of 145 child and adolescent patients (72 males and 73 females) with genetically diagnosed 22q11.2DS. The clinical data included seizures, growth chart, psychological reports, development characteristics, school performance, other clinical manifestations, and laboratory findings. RESULTS: Of the 145 patients with 22q11.2DS, 22 (15.2%) had epileptic seizures, 15 (10.3%) had developmental delay, and 5 (3.4%) had a psychiatric illness. Twelve patients with epilepsy were classified as genetic epilepsy whereas the remaining were classified as structural, including three with malformations of cortical development. Patients with epilepsy were more likely to display developmental delay (odds ratio=3.98; 95% confidence interval=1.5-10.5; p=0.005), and developmental delay was more common in patients with structural epilepsy than in those with genetic epilepsy. CONCLUSIONS: Patients with 22q11.2DS have a high risk of epilepsy, which in these cases is closely related to other NP manifestations. This implies that this specific genetic locus is critically linked to neurodevelopment and epileptogenesis.
Adolescent* ; Child* ; DiGeorge Syndrome* ; Epilepsy* ; Genetic Loci ; Growth Charts ; Humans ; Male ; Malformations of Cortical Development ; Medical Records ; Mental Disorders ; Neurologic Manifestations ; Retrospective Studies ; Seizures

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Pulmonary gangrene is a rare complication of severe pulmonary infection in which a pulmonary segment or lobe is sloughed. It is a part of a spectrum of disease in which lung tissue is devitalized(such as necrotizing pneumonia, pulmonary abscess), but apart from them, pulmonary gangrene has mo re extensive area of necrosis and thrombosis of large vessels plays a prominent role in the pathogenesis. We experienced a case of pulmonary gangrene in 71 year old female obstructive pneumonia patient with non-small cell lung carcinoma. She complained high fever, chill and despite treatment with antibiotics, pneumonia progressed to empyema. At that time chest radiograph showed a large cavity including sloughed lung tissue, freely moving to dependent position at both lateral decubitus view. RML and RLL were resected and compression of pulmonary vessels by enlarged lymph nodes was observed. Defervescence was obtained immediate postoperative period and the patient was discharged after infection control with antibiotics, chest tube drainage. The perivascular ly mph nodes dissected during lobectomy were proved to be reactive hyperplasias. We speculated that the carcinoma caused obstructive pneumonia, in turn, resulted in reactive hyperplasia of the draining lymph nodes surrounding the large vessels and finally the lung tissues supplied by them necrotized and sloughed.
Aged ; Anti-Bacterial Agents ; Chest Tubes ; Drainage ; Empyema ; Female ; Fever ; Gangrene* ; Humans ; Hyperplasia ; Infection Control ; Lung* ; Lymph Nodes ; Necrosis ; Pneumonia* ; Postoperative Period ; Radiography, Thoracic ; Thrombosis

Pulmonary gangrene is a rare complication of severe pulmonary infection in which a pulmonary segment or lobe is sloughed. It is a part of a spectrum of disease in which lung tissue is devitalized(such as necrotizing pneumonia, pulmonary abscess), but apart from them, pulmonary gangrene has mo re extensive area of necrosis and thrombosis of large vessels plays a prominent role in the pathogenesis. We experienced a case of pulmonary gangrene in 71 year old female obstructive pneumonia patient with non-small cell lung carcinoma. She complained high fever, chill and despite treatment with antibiotics, pneumonia progressed to empyema. At that time chest radiograph showed a large cavity including sloughed lung tissue, freely moving to dependent position at both lateral decubitus view. RML and RLL were resected and compression of pulmonary vessels by enlarged lymph nodes was observed. Defervescence was obtained immediate postoperative period and the patient was discharged after infection control with antibiotics, chest tube drainage. The perivascular ly mph nodes dissected during lobectomy were proved to be reactive hyperplasias. We speculated that the carcinoma caused obstructive pneumonia, in turn, resulted in reactive hyperplasia of the draining lymph nodes surrounding the large vessels and finally the lung tissues supplied by them necrotized and sloughed.
Aged ; Anti-Bacterial Agents ; Chest Tubes ; Drainage ; Empyema ; Female ; Fever ; Gangrene* ; Humans ; Hyperplasia ; Infection Control ; Lung* ; Lymph Nodes ; Necrosis ; Pneumonia* ; Postoperative Period ; Radiography, Thoracic ; Thrombosis

Nonspecific interstitial pneumonia (NSIP) was first described as a new category of idiopathic interstitial pneumonia in 1994. This is a disease with a more insidious onset and has a chronic course. The histological findings are unusual for other idiopathic interstitial pneumonia cases (usual interstitial pneumonia, diffuse interstitial pneumonia, and acute interstitial pneumonia). In contrast to NSIP, acute interstitial pneumonia (AIP) has an acute onset and a fulminant course with the rapid development of respiratory failure. A pathological examination demonstrated characteristic diffuse interstitial fibrosis, hyaline membranes, thrombi, and architectural derangement. Here we report a 48-year-old woman who was diagnosed pathologically NSIP, but with a rapid progressive course similar to AIP.
Female ; Fibrosis ; Humans ; Hyalin ; Idiopathic Interstitial Pneumonias ; Lung Diseases, Interstitial* ; Membranes ; Middle Aged ; Respiratory Insufficiency

Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.
Aneurysm ; Arteries ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Coronary Aneurysm* ; Coronary Stenosis ; Coronary Thrombosis ; Coronary Vessels ; Diagnosis ; Humans ; Infant* ; Infarction ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia ; Systemic Vasculitis ; Thromboembolism ; Thrombosis* ; Vasculitis ; Weaning