A twenty four-year-old female patient had suffered progressive dyspnea for 6 years until death. She denied any symptoms suggestive of connective tissue disease, or deep vein thrombosis. She suffered an episode of pontine infarct in 1995. Four years after diagnosis of primary pulmonary hypertension, she died of sudden death during hospitalization. Gross features of pulmonary arteries at autopsy were as follows: left main pulmonary artery showed dilation of the lumen and thickening of the wall, and right main pulmonary artery was markedly dilated and contained fresh thrombus. Hematoxylin and eosin-stained sections of lung tissue showed plexiform lesions of pulmonary arteries, complete luminal obliteration of pulmonary arterioles and dilated lesion of pulmonary arterioles, and capillaries. This patient represents a typical case with a primary pulmonary arteriopathy with plexiform lesions with thrombotic lesion, demonstrating the importance of thrombosis in situ in the pathogenesis of primary pulmonary hypertension. To our knowledge, this is the first autopsy report on the primary pulmonary hypertension in Korea.
Arterioles ; Autopsy ; Capillaries ; Connective Tissue Diseases ; Death, Sudden ; Diagnosis ; Dyspnea ; Female ; Hematoxylin ; Hospitalization ; Humans ; Hypertension, Pulmonary* ; Korea ; Lung ; Phenobarbital ; Pulmonary Artery ; Thrombosis ; Venous Thrombosis

PURPOSE: Anorectal manometry is a way of investigation for anti-rectal sphincters. In this paper we evaluated the usefulness of anorectal manometry in constipation patients and compared the anal spnincter function in control, constipation and encopresis patients. METHOD: We analysed the data of anorectal function studies in normal children (control, n=11), children with constipation (constipation group, n=20) and children with encopresis (encopresis group, n=16). RESULTS: The specific manometric parameters in normal children were like as follows; external anal sphinter pressure 21.0+/-8.00 mmHg, internal anal sphicter pressure 30.0+/- 14.57 mmHg, conscious rectal sensitivity threshold 11.4+/-4.52 mmHg. The above results were not different from that of previous studies except conscious rectal sensitivity threshold, which was slightly lower than that of others. Internal and external anal sphincter pressure were elevated significantly in constipation and encopresis groups than in control, which results was the same in conscious rectal sensitivity threshold. But the values of rectoanal inhibitory threshold and percent relaxation of rectoanal inhibitory reflex were not different among control group, constipation group and encopresis group. External sphincter activity was increased during the act of bearing down for defecation in none of the child in control group, in 6 of 17 children in constipation group and 5 of 12 children in encopresis group. CONCLUSION: With the results of above we could say that complete history taking and physical examination are important in diagnosis of constipation, and we could say also that the anorectal manometry was a valuable tool to understand the physiology of normal defecation and the pathophysiology of constipation and encopresis.
Anal Canal ; Child* ; Constipation* ; Defecation ; Diagnosis ; Encopresis ; Humans ; Manometry* ; Physical Examination ; Physiology ; Reflex ; Relaxation

Ticlopidine is a powerful inhibitor of platelet aggregation which is induced by adenosine diphosphate. Ticlopidine has been shown to be effective in reducing combined stroke, myocardial infarction, reocclusion of coronary stent. The principal side effects of ticlopidine are severe neutropenia, rash and gastrointestinal upset. Recently, we experienced two cases of severe neutropenia associated with ticlopidine. One was administered ticlopidine to prevent subacute thrombosis after intracoronary stent implantation in unstable angina pectoris. Thirty days of therapy, her absolute neutrophil count (ANC) had dropped to 14/mm3 . The other patient was presented with recurrent episodes of cerebral infarction, for which he had undergone right carotid angiogram. The carotid angiogram demonstrated tight stenosis of right internal carotid artery. Carotid artery stenting was performed at right internal carotid artery without any complications. Twenty seven days of ticlopidine therapy, his ANC had dropped to 111/mm3. The ticlopidine was stopped, and they were given granulocyte-colony stim-ulating factor 250 microgram/day subcutaneous injection until their ANC was up to 1000/mm3. They were discharged with normal neutrophil count and no other complications.
Adenosine Diphosphate ; Angina, Unstable ; Carotid Arteries ; Carotid Artery, Internal ; Cerebral Infarction ; Constriction, Pathologic ; Exanthema ; Granulocyte Colony-Stimulating Factor ; Humans ; Injections, Subcutaneous ; Myocardial Infarction ; Neutropenia* ; Neutrophils ; Platelet Aggregation ; Stents ; Stroke ; Thrombosis ; Ticlopidine*

No abstract available.
Head* ; Neck*

No abstract available.

OBJECTIVE: To quantify the effect of botulinum toxin A injection, by the compound muscle action potential (CMAP) measure from the gastrocnemius muscles (GCM) and to compare them with the clinical data. METHODS: Seventeen legs of 10 cerebral palsy (CP) children were studied with botulinum toxin A injection on the motor points of their GCM. Each GCM was injected up to 6 units of the botulium toxin A per kilogram of the body weight. The CMAP were measured at the motor points of GCM with the surface electrodes on the post-injection day 1, day 3, day 7, 2 weeks and at 1 month then monthly thereafter for 6 months. Physician rating scale (PRS) and the angle of passive ankle dorsiflexion were evaluated at the same time. RESULTS: The amplitude and the area of the CMAP decreased from post-injection day 1 to 3 months. The most pronounced decrement was seen at 2 weeks post-injection (p<0.05). The most pronounced increase of the dorsiflexion angle and PRS were seen at 1 and 2 months post-injection, respectively (p<0.05). CONCLUSION: The compound muscle action potential measure can be used for the neurophysiological quantification of the effect of botulinum toxin A, especially for the superficial muscles of extremities.
Action Potentials* ; Ankle ; Body Weight ; Botulinum Toxins* ; Cerebral Palsy ; Child ; Electrodes ; Extremities ; Humans ; Leg ; Muscles

PURPOSE: Recently the role of vitamins, folate in particular, has been emphasized in the maintenance of health. Folate deficiency is known to give rise to developmental delay, immature vascular disease, neural tube defect, acute leukemia, atherosclerotic vascular disease, delivery defects, breast cancer, and particularly gastrointestinal neoplasia. Methylenetetrahydrofolate reductase (MTHFR) is an essential enzyme in folate metaboism, and influences DNA synthesis and DNA methylation. Generally, folate deficiency is associated with gastrointestinal neoplasms. The amino-acid- changing and enzyme-activity-reducing nucleotide polymorphism (766C-->T/ Ala222Val) has been described in the MTHFR polymorphism and leads to low enzyme activity that may reduce the capacity of DNA methylation and possibly uracil mis-incorporation into DNA. These processes may be critical in the oncogenic transformation of human cells, especially in colorectal carcinomas. We investigated the relationship between the MTHFR polymorphism in gastric cancer and the tumor site, the smoking history, and the alcoholic history. MATERIALS AND METHODS: Ninety-six (96) individuals with gastric cancer and 287 healthy persons were analyzed. Blood sampling was performed, PCR-RFLP was analyzed, and MTHFR polymorphism genotypes of C/C, C/T, and T/T were obtained and analyzed statistically for their correlation. RESULTS: In the gastric cancer group there were 69 (72%) males and 27 (28%) females. There were also 58 cases (60%) involving the gastric lower body, 20 cases (21%) the gastric mid-body, and 18 cases (19%) the gastric upper body. In the control group there were 169 (59%) males and 118 (41%) females. Among the gastric cancer, 56 (61%) smoking patients, 40 (39%) non-smoking patients, 45(47%) alcoholic patients, 51 (53%) non-alcoholic patients. In the gastric cancer group, MTHER polymorphisms were C/C in 18 (19%) cases, C/T in 59 (61%) cases, T/T in 19 (20%) cases. In the control group polymorphisms were C/C 116 (40%) cases, C/T 103 (36%) cases, and T/T 68 (24%) cases (P=0.045). In cases of lower gastric body cancer, polymorphisms were C/C in 16 (24%) C/C in 16 (24%) cases and C/T or T/T in 42 (72%) cases. In cases of upper and mid-body cancer, polymorphisms were C/C in 2 (5%) cases and C/T or T/T 36 (95%) cases (P=0.006). In the non-smoking patient group, polymorphisms were C/C in 5 (12%) cases and C/T or T/T in 35 (88%) cases. In the smoking patient group, C/C in 13 (23%) cases and C/T or T/T in 43 (77%) cases (P=0.189). In the non-alcoholic patient group, polymorphisms were C/C in 6 (12%) cases and C/T or T/T in 45 (88%) cases. In the alcoholic patient group, polymorphisms were C/C in 12 (26%) cases and C/T or T/T in 33 (74%) cases (P=0.063) CONCLUSION: MTHFR polymorphisms are associated with gastric cancer and tumor site, but not with smoking and alcohol drinking.
Alcohol Drinking ; Alcoholics ; Breast Neoplasms ; Colorectal Neoplasms ; DNA ; DNA Methylation ; Female ; Folic Acid ; Gastrointestinal Neoplasms ; Genotype ; Humans ; Leukemia ; Male ; Methylenetetrahydrofolate Reductase (NADPH2)* ; Neural Tube Defects ; Polymorphism, Single Nucleotide ; Smoke ; Smoking ; Stomach Neoplasms* ; Uracil ; Vascular Diseases ; Vitamins

It is well known that both acute glomerulonephritis and rheumatic fever are a common sequelae of group A streptococcal infection. However, their simultaneous occurrence is uncommon. Only a few cases have been reported because they are different in epidemiologic, clinical, serologic and immunopathogenic characteristics. We experienced a 10-year-old boy who presented the manifestation of acute rheumatic fever and acute poststreptococcal glomerulonephritis. We report this case with brief review of literature.
Child ; Glomerulonephritis* ; Humans ; Male ; Rheumatic Fever* ; Streptococcal Infections

A five-year-old boy received a blunt trauma on chest by a kindergarten bus on Feb. 29, 1996. Pulmonary hemorrhage and pericardiac effusion were developed, followed by multi-organ failure threatening his life. All symptoms were improving when pansystolic harsh murmur (III/IV) originating from a tiny ventricular rupture with a blood leak to the pericardial space was auscultated on the 12th day after the trauma. This murmur disappeared with the left ventricular healing and progressive aneurysmatic formation. Howeve, on the 61st day after the trauma a continuous murmur (II/III) of aortic dissecting aneurysm on the left upper sternal border and dorsum was newly auscultated. Two-dimensional color doppler echocardiogram and aortogram by femoral artery catheterization revealed aneurysmatic dilatations of both the left ventricular free wall and descending aorta immediately after the origin of the subclavian artery with mild mitral regugitation. The patient underwent successful corrective surgery.
Aneurysm* ; Aneurysm, Dissecting ; Aorta, Thoracic* ; Automobiles* ; Catheterization ; Catheters ; Dilatation ; Femoral Artery ; Heart Ventricles* ; Hemorrhage ; Humans ; Male ; Rupture ; Subclavian Artery ; Thorax

Chronic graft-versus-host disease (GVHD) usually presents 100 days after allogenic bone marrow transplantation. Chronic GVHD cutaneous lesions are characterized by lichenoid or sclerodermoid variants. Vesicles, a common presentation in patients with acute GVHD, rarely appear in chronic GVHD. We report a case of a 49-year-old man who presented with bilateral vesicles on lower extremities. He was diagnosed with myelodysplastic syndrome 2 years before and was taking oral cyclosporine after the allogenic bone marrow transplantation. Six months post-transplantation, lichenoid and sclerodermoid lesions developed on his entire body and he was diagnosed with chronic GVHD and eosinophilic fasciitis. A biopsy of the vesicles revealed detached lower margins of the epidermis, necrotized keratinocytes, and infiltration of lymphocytic inflammatory cells. Administration of oral prednisolone alleviated the patient's symptoms. This is an interesting case showing a new pattern of vesicle appearance after development of typical chronic GVHD lesions.
Biopsy ; Bone Marrow Transplantation ; Cyclosporine ; Eosinophilia ; Eosinophils ; Epidermis ; Fasciitis ; Graft vs Host Disease ; Humans ; Keratinocytes ; Lower Extremity ; Middle Aged ; Myelodysplastic Syndromes ; Prednisolone