No abstract available.
Breast* ; Female ; Humans ; Milk, Human*

No abstract available.
Peutz-Jeghers Syndrome*

No abstract available.
Child* ; Glomerulonephritis* ; Humans

Ancient schwannoma is a variant of schwannoma and is characterized slowly growing tumor with degenerative change. And it is reported that schwannoma is relatively rare in extensor area. As a rare cause of solitary ancient schwannoma in extensor area of upper arm, we report it.
Arm ; Neurilemmoma

Williams syndrome is a congenital disorder characterized by mental retardation, loquacious personalities, dysmorphic face, and vascular and valvular abnormalities. The etiology of this syndrome was one allelic loss of elastin gene, exhibiting a submicroscopic deletion, at 7q11.23. Sudden death is an infrequently recognized complication. The mechanism of sudden death is explained by myocardial ischemia, decreased cardiac output, and arrhythmia by anatomical abnormality of coronary artery stenosis and severe biventricular outflow tract obstruction. We report an autopsy case of a 80 day-old male with Williams syndrome. Five days before admission, cardiac murmur was detected incidentally on ascultation at a local clinic during a visit for vaccination. He was transferred to our hospital and cardiac catheterization was done. He died suddenly next day. Postmortem examination revealed a dysmorphic face and multiple cardiovascular abnormalities including supravalvular aortic stenosis with narrowed coronary artery ostia, supravalvular pulmonic stenosis, secundum type of atrial septal defect, right ventricular hypertrophy, and renal artery stenosis. Histologically, aorta and pulmonary, bronchial, and renal arteries showed markedly hyperplastic medial elastic laminae approximately three times thick compared to those of age-matched normal artery. The elastic fibers of the innermost two thirds of media were disposed in a normal orderly parallel fashion. In outer third of the media, the elastic fibers had lost the normal orderly arrangement.
Aorta ; Aortic Stenosis, Supravalvular ; Arrhythmias, Cardiac ; Arteries ; Autopsy* ; Cardiac Catheterization ; Cardiac Catheters ; Cardiac Output ; Cardiovascular Abnormalities ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Coronary Stenosis ; Coronary Vessels ; Death, Sudden ; Elastic Tissue ; Elastin ; Heart Murmurs ; Heart Septal Defects, Atrial ; Humans ; Hypertrophy, Right Ventricular ; Infant* ; Intellectual Disability ; Loss of Heterozygosity ; Male ; Myocardial Ischemia ; Pulmonary Valve Stenosis ; Renal Artery ; Renal Artery Obstruction ; Vaccination ; Williams Syndrome*

Williams syndrome is a congenital disorder characterized by mental retardation, loquacious personalities, dysmorphic face, and vascular and valvular abnormalities. The etiology of this syndrome was one allelic loss of elastin gene, exhibiting a submicroscopic deletion, at 7q11.23. Sudden death is an infrequently recognized complication. The mechanism of sudden death is explained by myocardial ischemia, decreased cardiac output, and arrhythmia by anatomical abnormality of coronary artery stenosis and severe biventricular outflow tract obstruction. We report an autopsy case of a 80 day-old male with Williams syndrome. Five days before admission, cardiac murmur was detected incidentally on ascultation at a local clinic during a visit for vaccination. He was transferred to our hospital and cardiac catheterization was done. He died suddenly next day. Postmortem examination revealed a dysmorphic face and multiple cardiovascular abnormalities including supravalvular aortic stenosis with narrowed coronary artery ostia, supravalvular pulmonic stenosis, secundum type of atrial septal defect, right ventricular hypertrophy, and renal artery stenosis. Histologically, aorta and pulmonary, bronchial, and renal arteries showed markedly hyperplastic medial elastic laminae approximately three times thick compared to those of age-matched normal artery. The elastic fibers of the innermost two thirds of media were disposed in a normal orderly parallel fashion. In outer third of the media, the elastic fibers had lost the normal orderly arrangement.
Aorta ; Aortic Stenosis, Supravalvular ; Arrhythmias, Cardiac ; Arteries ; Autopsy* ; Cardiac Catheterization ; Cardiac Catheters ; Cardiac Output ; Cardiovascular Abnormalities ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Coronary Stenosis ; Coronary Vessels ; Death, Sudden ; Elastic Tissue ; Elastin ; Heart Murmurs ; Heart Septal Defects, Atrial ; Humans ; Hypertrophy, Right Ventricular ; Infant* ; Intellectual Disability ; Loss of Heterozygosity ; Male ; Myocardial Ischemia ; Pulmonary Valve Stenosis ; Renal Artery ; Renal Artery Obstruction ; Vaccination ; Williams Syndrome*

We experienced a case of MRKH syndrome in a 24-year-old female which was combined with sacral agenesis, neurogenic bladder, and duplicated ureter associated with vesicoureteral reflux in the right side.
Female ; Humans ; Ureter ; Urinary Bladder, Neurogenic* ; Vesico-Ureteral Reflux ; Young Adult

No abstract available.

The immature teratoma is extremely rare tumor consisting of mature tissues and immature embryonal tissues. The immature teratoma in children occurs more frequently at the sacrococcygeal bone, testis and ovary. Only 9.5 percent of immature teratomas occur in retroperitoneum. Often the presenting manifestation is asymptomatic with increasing abdominal distention or mass discovered during physical examination. We report a case of congenital retroperitoneal immature teratoma in 3-week-old boy who was treated by surgical excision.
Child ; Female ; Humans ; Male ; Ovary ; Physical Examination ; Teratoma* ; Testis

No abstract available.
Female ; HELLP Syndrome* ; Pregnancy