Accidents, Traffic ; Diagnosis ; Facial Injuries ; Female ; Head ; Humans ; Incidence ; Inpatients ; Jeollanam-do ; Lacerations ; Male ; Mandibular Fractures ; Neck ; Postoperative Complications ; Social Environment ; Surgery, Oral ; Violence

Primary amenorrhea due to intrasella arachnoid cyst is a very rare disease and require careful and frequent evaluation because may produce intracranial hemorrhage, elevated intracranial pressure and rapid expansion. Surgical intervention is needed only when visual disturbance, hypopituitarism or enlarging lesion is shown. Thus, we present a case of primary amenorrhea due to intrasella arachnoid cyst which was resected through the transsphenoidal approach.
Amenorrhea ; Arachnoid* ; Female ; Hypogonadism* ; Hypopituitarism ; Intracranial Hemorrhages ; Intracranial Hypertension ; Rare Diseases

Telomerase activity is usually detected in most tumor tissues but not in normal tissues. Recently, there is increasing evidence that telomerase activity is associated with cell proliferation without malignancy, whereas there is little information about telomerase activity and its relationship with cell proliferation in chronic hyperproliferative skin diseases. Thus, we studied telomerase activity in skins from 10 patients with psoriasis and compared telomerase activity with the expression of Ki-67, a proliferation marker, using immunohistochemical staining. The effect of retinoic acid on the telomerase activity in HaCaT cells was also evaluated. Telomerase activity was detected in 7 (70%) of 10 lesional skins of psoriasis and none of the nonlesional skin. Telomerase activity in lesional skin was significantly associated with Ki-67 labelling index. Retinoic acid treatment on HaCaT cells inhibited telomerase activity, which correlated with inhibition of cell proliferation by the agent. The results of our study represent another example that shows telomerase activity correlates with cellular proliferation. Further studies on the regulation of the telomerase are needed to understand the cellular factors involved in controlling telomerase activity.
Cell Division/drug effects ; Cell Line ; Enzyme Inhibitors/*pharmacology ; Human ; Ki-67 Antigen/*analysis ; Psoriasis/*enzymology ; Skin/*enzymology ; Telomerase/antagonists & inhibitors/*metabolism ; Tretinoin/*pharmacology

We describe a 58-year-old woman who was incidentally found to have gastric and colonic polyposis, hypoalbuminemia, cutaneous hyperpigmentation and onychodystrophy (Cronkhite-Canada syndrome). Histology of polyps from the stomach showed features of juvenile or retention type (hamartomatous) polyps with Helicobacter pylori (H. pylori) infection. The large pedunculated colonic polyps showed hamartomatous polyps with adenomatous component and polypectomy was performed. After the treatment with H. pylori eradication and omeprazole, the gastric polyposis, hypoalbuminemia and anemia regressed, and endoscopic polypectomy of gastric polyps were performed. After the continuous use of omeprazole for 14 months, the patient showed complete resolution of clinical features of Cronkhite-Canada syndome. The experience of this case suggests that eradication of H. pylori and proton pump inhibitor treatment might be considered in patients with gastric polyposis combined with Cronkhite-Canada syndome.
Anti-Ulcer Agents/*therapeutic use ; Colonic Polyps/complications/microbiology/pathology ; Female ; Helicobacter Infections/complications/*drug therapy ; *Helicobacter pylori ; Humans ; Hyperpigmentation/pathology ; Middle Aged ; Nails, Malformed/pathology ; Omeprazole/*therapeutic use ; Polyps/*complications/microbiology/pathology ; Proton Pumps/antagonists & inhibitors ; Stomach Neoplasms/*complications/microbiology/pathology ; Syndrome

The term MYH9-related disorders indicates a group of autosomal dominant illnesses, formerly known as May-Hegglin anomaly, Sebastian syndrome, Fechtner syndrome and Epstein syndrome, caused by mutations of MYH9, the gene encoding for the heavy chain of non-muscle myosin IIA (NMMHC-IIA). We experienced a family with macrothrombocytopenia without leukocyte inclusion. A 5-year-old girl was found to have macrothrombocytopenia incidentally. Her father also had macrothromtocytopenia, but had been suffering from hearing loss and chronic renal failure. Meticulous search by light and electron microscopy failed to detect leukocyte inclusions. To our knowledge, these cases seem to be the first description of autosomal dominant Epstein giant platelet syndrome in Korea.
Bernard-Soulier Syndrome* ; Child, Preschool ; Fathers ; Female ; Hearing Loss ; Humans ; Kidney Failure, Chronic ; Korea ; Leukocytes ; Microscopy, Electron ; Nonmuscle Myosin Type IIA

Fibrodysplasia ossificans progressiva is a rare hereditary disorder characterized by progressive heterotopic ossifica-tion in muscle and connective tissue, with few reported cases affecting the head and neck region. Although plain radiographic findings and computed tomography features have been well documented, limited reports exist onmagnetic resonance findings. This report presents 2 cases of fibrodysplasia ossificans progressiva, one with limited mouth opening due to heterotopic ossification of the lateral pterygoid muscle and the other with restricted neck movement due to heterotopic ossification of the platysma muscle. Clinical findings of restricted mouth opening or limited neck movement, along with radiological findings of associated heterotopic ossification, should prompt consideration of fibrodysplasia ossificans progressiva in the differential diagnosis. Dentists should be particularly vigilant with patients diagnosed with fibrodysplasia ossificans progressiva to avoid exposure to diagnostic biopsy andinvasive dental procedures.

Keratosis follicularis squamosa(Dohi) is an acquired keratinizing disorder , which is particularly common among Japanese and rarely reported in other countries . It is characterized by multiple small brownish atrophic patches with follicular plug and collarette scales on the trunk and thighs . The scaly patches, which look like lotus leaves on the water as described by Dohi in the first report, is very characteristic. We report a case of keratosis follicularis squamosa(Dohi) in a 39-year-old woman, who showed a clearance of the skin lesion after 4-week minocycline administration.
Adult ; Asian Continental Ancestry Group ; Darier Disease* ; Female ; Humans ; Keratosis* ; Lotus ; Minocycline ; Skin ; Thigh ; Water ; Weights and Measures

Sirenomelia is rare congenital malformation characterized by fusion of the lower extrimities associated anomalies included absence of urogenital system (bilateral renal agenesis, absent ureter, urinary bladder, absent internal and external genitalia), induced severe oligohydramnios, anorectal atresia, single umbilical artery, pulmonary hypoplasia. The incidence is 1:60000 births. Survival is extremely rare, and only possible, in the absence of bilateral renal agenesis. In view of the dismal prognosis, early diagnosis allows for earlier and less traumatic therapeutic abortion. We report a case of sirenomelia without oligohydramnios diagnosed in early second trimester.
Abortion, Therapeutic ; Early Diagnosis ; Ectromelia* ; Female ; Humans ; Incidence ; Oligohydramnios* ; Parturition ; Pregnancy ; Pregnancy Trimester, Second* ; Prognosis ; Single Umbilical Artery ; Ureter ; Urinary Bladder ; Urogenital System

No abstract available.
Bleomycin* ; Drug Therapy* ; Humans ; Uterine Cervical Neoplasms*

Kerion celsi is an inflammatory type of tinea capitis, which occurs chiefly in children between the ages of 4 and 14 years. We report a case of kerion celsi caused by Trichophyton(T.) mentagrophytes in 69 year-old man, who showed a 8x8 cm sized, erythematous boggy mass with pustules and crusts on the right occipital scalp. Cultures from scalp lesion and infected hair on Sabouraud dextrose agar media showed T. mentagrophytes. Histopathologic findings showed inflammatory cell infiltration in entire dermis and many spores around the hair shaft. The skin lesion was successfully treated with terbinafine (250 mg/ day) for 8 weeks. This case is reported with three interesting facts: Kerion celsi caused by T. mentagrophytes has been rarely reported in Korean literatures; Kerion celsi is occuring in an aged man with decreased cellular immunity after chemotherapy; Kerion celsi caused by T. mentagrophytes responded well to oral terbinafine.
Agar ; Aged ; Child ; Dermis ; Drug Therapy ; Glucose ; Hair ; Humans ; Immunity, Cellular ; Scalp ; Skin ; Spores ; Tinea Capitis* ; Trichophyton*