No abstract available.
Humans

Superficial angiomyxoma is a comparatively rare dermal and subcutaneous tumor. We report a case of superficial angiomyxoma of the thumb in view of its rarity and typical light and electronmicroscopic features. The patient was a 46-year-old male. who presented with an asymptomatic, slowly enlarging mass that developed in the left thumb over the 5 years. He had a history of trauma and electric burn in the same area 20~30 years ago. Simple X-ray and magnetic resonance imaging revealed 35x30mm, mass with destruction of distal phalangeal bone. On operation, the lesion was moderately well circumscribed and soft with lobulated nodules that elevated the overlying skin and destroyed the underlying bone. The cut surface of the mass was glistening and slimy. The mass was whitish gray and lobulated. Bony involvement was not present. Microscopically, the tumor was composed of stellated and spindle shaped stromal cells which were scattered throughout myxoid ground substance. Neither nuclear hyperchromasia nor plemorphisam was present. Small to medium sized thin walled blood vessels were scattered. There was a scanty infiltrate of inflammatory cells. The S-100 protein immunostaining was negative in tumor cells. On electron microscopy, the cytoplasm of the stromal cells contained well developed rough ednoplasmic reticulums and other features that indicated differentiation toward fibroblasts.

Holoprosencephaly, a grave malformation during cleavage phase of brain development, occurs in association with a variety of clinical syndrome including chromosomal aberration. Among chromosomal anomalies trisomy syndromes, particularly trisomy 18, are often associated with holoprosencephaly. Triploidy with holoprosencephaly had also been described. We report an autopsy case of incomplete triploidy with abnormal sex chromosome, i.e., 63, XXY. Our case showed a marked intrauterine growth retardation, and postmortem examination revealed alobar holoprosencephaly, hypotelorism, bilateral cleft palates and lips, flat nose, microstomia, lowset ears, congenital heart disease and cystic kidney. The brain was microcephalic 5 x 6 cm and was of pancake shape. there was a large dorsal cyst. Olfactory tracts and bulbs were absent. The brain surface was smooth, and only suggestive hippocampal fissure was noted. The basal ganglia and thalami were fused in midline and the aqueductal origin was exposed. The brain stem and cerebellum were unremarkable. Repeated karyotypings revealed 63,XXY consistently. All 21 chromosomes showed trisomy except for D group. The sex chromosome was XXY, and the genital tract and gonad were those of female.
Female ; Humans

A dieulafoy lesion, which is an unusual cause of gastrointestinal bleeding that can be fatal in children. Dieulafoy lesions are characterized by an abnormally large eroded submucosal artery that is commonly located in the lesser curvature of the proximal stomach. In most cases, permanent hemostasis is achieved by endoscopic epinephrine injection, however, some patients require other endoscopic treatment modalities, embolization or surgery. We report here a case of a Dieulafoy lesion in an 11-year-old boy who had recurrent bleeding from the lesion in the duodenal bulb after endoscopic epinephrine injection and surgical ligation, that was successfully treated using coil embolization.
Arteries ; Child* ; Cytochrome P-450 CYP1A1 ; Embolization, Therapeutic ; Epinephrine ; Hemorrhage ; Hemostasis ; Humans ; Ligation ; Male ; Stomach

A dieulafoy lesion, which is an unusual cause of gastrointestinal bleeding that can be fatal in children. Dieulafoy lesions are characterized by an abnormally large eroded submucosal artery that is commonly located in the lesser curvature of the proximal stomach. In most cases, permanent hemostasis is achieved by endoscopic epinephrine injection, however, some patients require other endoscopic treatment modalities, embolization or surgery. We report here a case of a Dieulafoy lesion in an 11-year-old boy who had recurrent bleeding from the lesion in the duodenal bulb after endoscopic epinephrine injection and surgical ligation, that was successfully treated using coil embolization.
Arteries ; Child* ; Cytochrome P-450 CYP1A1 ; Embolization, Therapeutic ; Epinephrine ; Hemorrhage ; Hemostasis ; Humans ; Ligation ; Male ; Stomach

No abstract available.
Hepacivirus ; Hepatitis C* ; Hepatitis*

Double aortic arch is the most common type of symptomatic vascular ring. In most patients, the symptoms are manifested at birth or in early infancy. Double aortic arch usually has more severe symptoms than other types of complete vascular rings. We experienced one case of complete duplicated double aortic arch with left descending aorta and left patent ductus arteriosus. A one-day-old female neonate was transferred to our hospital because of mild dyspnea and stridor. She showed intractable CO2 retention and respiratory difficulty with time in spite of ventilator therapy. She died of respiratory failure 23 hours after birth. On autopsy, we found that the diameter of the vascular ring was 0.7cm in maximum extent. It was ovoid and contained trachea and esophagus. The trachea was flattened due to allowing 0.1x0.2cm. The compression level of the trachea was approximately 1cm from the tracheal bifurcation.
Aorta, Thoracic* ; Autopsy* ; Ductus Arteriosus, Patent ; Dyspnea ; Esophagus ; Female ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Parturition ; Respiratory Insufficiency ; Respiratory Sounds ; Trachea ; Ventilators, Mechanical

Kikuchi-Fujimoto's disease (KFD) is a rare self-limiting necrotizing lymphadenitis found mainly in young women. Patients typically present with lymphadenopathy and often with a high fever. The etiology of the disease remains unknown, but various infection and autoimmune processes have been postulated to be the cause. We report here on a 22-year-old female with 7-year history of systemic lupus erythematosus presented with high fever and many enlarged cervical lymph nodes. Neck computed tomography scan showed multiple lymphadenopathies and KFD was proven by under sonographic guidance percutaneous needle biopsy.
Biopsy, Needle ; Female ; Fever ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Lupus Erythematosus, Systemic* ; Lymph Nodes ; Lymphadenitis ; Lymphatic Diseases ; Neck ; Ultrasonography ; Young Adult

No abstract available.
Down Syndrome* ; Myeloproliferative Disorders*

Leukocytosis and neutrophilia is common during the acute phase of Kawasaki disease whereas leukopenia is not common and severe neutropenia is rare. Severe neutropenia is defined as absolute neutrophil count less than 500/mm3. There are only few publicatons reporting of atypical Kawasaki disease with severe neutropenia. We report a case of atypical Kawasaki disease with severe neutropenia.
Leukocytosis ; Leukopenia ; Mucocutaneous Lymph Node Syndrome* ; Neutropenia* ; Neutrophils