No abstract available.
Chondrosarcoma* ; Nasal Cavity*

We report herein two cases of pachyonychia congenita in 19-month-old and 7-year-old girls. In addition to severely deformed nails, both had white plaques on the tongue and keratotic papules on the elbows and knees. In the 19-month-old girl, her father showed deformity of the nails and severe hyperkeratosis of the palms and soles, The family history was normal in other case.
Child ; Congenital Abnormalities ; Elbow ; Fathers ; Female ; Humans ; Infant ; Knee ; Nails, Malformed* ; Pachyonychia Congenita* ; Tongue

No abstract available.
Free Tissue Flaps*

Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH syndrome) is a neurometabolic disorder with highly variable clinical severity ranging from mild learning disability to severe encephalopathy. Diagnosis of HHH syndrome can easily be delayed or misdiagnosed due to insidious symptoms and incomplete biochemical findings, in that case, genetic testing should be considered to confirm the diagnosis. HHH syndrome is caused by biallelic mutations of SLC25A15, which is involved in the urea cycle and the ornithine transport into mitochondria. Here we report a boy with spastic paraplegia and asymptomatic younger sister who have compound heterozygous mutations of c.535C>T (p.R179*) and c.116C>A (p.T39K) in the SLC25A15 gene. We identified that p.T39K mutation is a novel pathogenic mutation causing HHH syndrome and that p.R179*, which is prevalent in Japanese and Middle Eastern heritage, is also found in the Korean population.
Asian Continental Ancestry Group ; Brain Diseases ; Diagnosis ; Genetic Testing ; Genetics ; Humans ; Learning Disorders ; Male ; Mitochondria ; Ornithine ; Paraplegia ; Siblings ; Urea ; Urea Cycle Disorders, Inborn

During administration of anesthesia, intraoperative blood pressure control is very important. Sometimes a hypertensive or anti-hypertensive agent is used in order to maintain blood pressure at an adequate level; alpha-agonist and beta-blocker are drugs frequently used. Alpha-agonists are used in various ways including their application together with local anesthetics during an operation for the purpose of vasoconstriction, to control systemic absorption of local anesthetics, to extend the duration of action, or to reduce bleeding from the surgical site. In addition, alpha-agonists are used in cardiopulmonary resuscitation. Beta-blockers are used widely as a therapeutic agent for hypertension, angina, and arrhythmia, and to lower portal pressure in liver cirrhosis. Here, we are reporting the case of acute pulmonary edema that occurred after the administration of phenylephrine, in order to maintain blood pressure in a 52-year-old female patient with liver cirrhosis. The patient was underwent emergent decompressive craniectomy for intracranial hemorrhage without acknowledging her long-term use of a beta-blocker medication.
Absorption ; Anesthesia ; Anesthetics, Local ; Arrhythmias, Cardiac ; Blood Pressure ; Cardiopulmonary Resuscitation ; Decompressive Craniectomy ; Female ; Hemorrhage ; Humans ; Hypertension ; Intracranial Hemorrhages ; Liver Cirrhosis ; Middle Aged ; Phenylephrine ; Portal Pressure ; Pulmonary Edema ; Vasoconstriction

No abstract available.
Mucocele*

No abstract available.
Cartilage* ; Fascia* ; Transplants* ; Tympanoplasty*

Penile metastases are rare, and usually secondary to other genitourinary primary tumors. Bladder, prostate and renal tumors are the main sources of penile metastasis. Rectal and respiratory neoplasms also metastasize to the penis. However, metastatic penile cancer originating from the pancreas is extremely rare. Here, a case of pancreatic cancer, with penile metastasis, is reported.
Male ; Neoplasm Metastasis ; Pancreas* ; Pancreatic Neoplasms ; Penile Neoplasms* ; Penis ; Prostate ; Urinary Bladder

A lymphoma of the prostate is very rare, and is usually incidentally discovered. Malignant lymphomas can be divided into either primary or secondary according to their origin. Secondary involvement of the gland is the most common presentation. Most non-Hodgkin's lymphomas that affect the prostate are of the B-cell type, and present with non-specific obstructive symptoms. A variety of methods have been used for their, including prostatectomy, radiotherapy or combinations of chemotherapy and radiotherapy. However, the prognosis remains poor, regardless of the patient's age, histological type, treatment or clinical stage at presentation. Here, the case of a patient with a B-cell lymphoma, who presented with symptoms of a bladder outlet obstruction, is reported. The patient has been in complete remission following the completion of 6 cycles of doxorubicin-based chemotherapy and accompanying radiotherapy.
B-Lymphocytes* ; Drug Therapy ; Drug Therapy, Combination* ; Humans ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, Non-Hodgkin ; Prognosis ; Prostate* ; Prostatectomy ; Radiotherapy* ; Urinary Bladder Neck Obstruction

Trigeminal neuralgia is sudden, usually unilateral, severe brief stabbing recurrent pain in the distribution of one or more branches of the 5th cranial nerve. Treatments of trigeminal neuralgia include systemic trials of medications and surgical procedures such as microvascular decompression, stereotactic radiosurgery, percutaneous glycerol or alcohol rhizolysis, percutaneous radiofrequency rhizotomy, and percutaneous balloon compression (PBC). PBC of the trigeminal ganglion using a balloon catheter was introduced by Mullan and Lichtor in 1983. Since then, many papers have been published describing results of this technique. Typically, this procedure is performed under general anesthesia. However, little is known about the use of PBC for the treatment of trigeminal neuralgia in Korea. We report here our anesthetic management of percutaneous balloon compression of trigeminal ganglion for the treatment of the trigeminal neuralgia by total intravenous anesthesia.
Anesthesia, General ; Anesthesia, Intravenous ; Catheters ; Cranial Nerves ; Glycerol ; Korea ; Microvascular Decompression Surgery ; Radiosurgery ; Rhizotomy ; Trigeminal Ganglion ; Trigeminal Neuralgia