Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

No abstract available.
Osteomyelitis*

No abstract available.
Leiomyosarcoma* ; Rectum*

Serum specimens from leprosy patients, their contacts and controls were examined for the presence of phenolic glycolipid I (PGL-I), a Mycobacterium leprae specific antigen, and antibodies to the antigen using enzyme-linked immunosorbent assays. Of 12 lepromatous patients with less than 2 years of therapy, 11(91.7%) were seropositive to PGL-l, thus indicating that new lepromatous cases can be identified by detecting anti-PGL-l antibodies. In contrast 88(56.4%) of 156 lepromatous patiens treated more than 2 years were positve. Moreover, only 69(40.8%) were seropositve among 169 lepromatous patients in the leprosy resettlement villages. The mean antibody level also declined significantly in proportion to the duration of chemotherapy. This may suggest the possibility of monitoring chemotherapy by detecting anti-PGL-l antibodies. The prevalence of anti-PGL-l antibodies among 200 controls from a high endemic area for leprosy was 5.5% and was significantly higher than that(1.5%) among 200 controls from a low endemic area. Of 103 household contacts in the resettlement villages, 10(9.7%) were seropositive, reflecting the frequent chance of exposure to M. leprae. However, PGL-l was not detected many in any of the sera from controls, contacts, and inactive lepromatous patients having the anti-PGL-l antibodies; on the other hand, 6(50%) of 12 lepromatous patients treated less than 2 years had detectable PGL-l in their sera. The results thus indicate that PGL-l detection may be more suitable for monitoring the effectiveness of chemotherapy and that it may be necessary to examine for the presence of PGL-l in sera from contacts and normal populations for confirming M. leprae infection.
Antibodies, Bacterial/*analysis ; Glycolipids/*blood ; Human ; Leprosy/*blood/diagnosis ; Serologic Tests ; Support, Non-U.S. Gov't

BACKGROUND: There is a high incidence of pain associated with an intravenous injection of propofol, and different methods have been used in an attempt to minimize the incidence and severity of this pain. This study compared the effect of a ketamine pretreatment with that of a remifentanil pretreatment on the pain associated with a propofol injection. METHODS: 225 ASA physical status 1-2 adult patients scheduled for elective surgery were enrolled into this randomized double blind study. The patients received 2 ml of saline (n = 45), 2 ml of ketamine 20 mg (n = 45), 2 ml of remifentanil 0.01 mg (n = 45), or 2 ml of remifentanil 0.02 mg (n = 45) or 2 ml of remifentanil 0.03 mg (n = 45) 30 seconds prior to administering 2 mg/kg of 1% propofol. An anesthesiologist who was blinded to the study asked the patients to evaluate the pain using a four-point scale (none, mild, moderate, severe). RESULTS: The remifentanil 0.02 mg, remifentanil 0.03 mg and ketamine groups showed significantly less frequent and intense pain than the saline group (P < 0.05). The remifentanil 0.02 mg and 0.03 mg provided as much pain relief as ketamine (P > 0.05), but the remifentanil 0.01 mg did not (P < 0.05). There was a similar incidence of injection pain in the remifentanil 0.02 mg and remifentanil 0.03 mg groups. CONCLUSIONS: An intravenous remifentanil 0.02 mg or remifentanil 0.03 mg pretreatment is as effective in alleviating the pain associated with a propofol injection as a intravenous ketamine pretreatment. The remifentanil 0.03 mg pretreatment had a similar analgesic effect relative to the remifentanil 0.02 mg pretreatment.
Adult ; Double-Blind Method ; Humans ; Incidence ; Injections, Intravenous ; Ketamine* ; Propofol*

BACKGROUND: There is a high incidence of pain associated with an intravenous injection of propofol, and different methods have been used in an attempt to minimize the incidence and severity of this pain. This study compared the effect of a ketamine pretreatment with that of a remifentanil pretreatment on the pain associated with a propofol injection. METHODS: 225 ASA physical status 1-2 adult patients scheduled for elective surgery were enrolled into this randomized double blind study. The patients received 2 ml of saline (n = 45), 2 ml of ketamine 20 mg (n = 45), 2 ml of remifentanil 0.01 mg (n = 45), or 2 ml of remifentanil 0.02 mg (n = 45) or 2 ml of remifentanil 0.03 mg (n = 45) 30 seconds prior to administering 2 mg/kg of 1% propofol. An anesthesiologist who was blinded to the study asked the patients to evaluate the pain using a four-point scale (none, mild, moderate, severe). RESULTS: The remifentanil 0.02 mg, remifentanil 0.03 mg and ketamine groups showed significantly less frequent and intense pain than the saline group (P < 0.05). The remifentanil 0.02 mg and 0.03 mg provided as much pain relief as ketamine (P > 0.05), but the remifentanil 0.01 mg did not (P < 0.05). There was a similar incidence of injection pain in the remifentanil 0.02 mg and remifentanil 0.03 mg groups. CONCLUSIONS: An intravenous remifentanil 0.02 mg or remifentanil 0.03 mg pretreatment is as effective in alleviating the pain associated with a propofol injection as a intravenous ketamine pretreatment. The remifentanil 0.03 mg pretreatment had a similar analgesic effect relative to the remifentanil 0.02 mg pretreatment.
Adult ; Double-Blind Method ; Humans ; Incidence ; Injections, Intravenous ; Ketamine* ; Propofol*

Intranasal corticosteroid injection has been used for the control of allergic rhinitis or polypoid inflammation of the nasal cavity. Transient or permanent visual loss after therapeutic intranasal injection of corticosteroid is an extremely rare but well-known disease. In this report, we present a 54-year-old male patient with chronic sinusitis with nasal polyposis who had right visual loss after bilateral intranasal triamcinolone injection. Clinical examination and fluorescent angiography revealed vascular occlusions, which involved multiple small branches of right retinal circulations. Full recovery of vision occurred 3 months after the onset of the symptom. Extreme care should be exercised to avoid visiual complication when intranasal corticosteroid injection is performed.
Angiography ; Blindness* ; Humans ; Inflammation ; Male ; Middle Aged ; Nasal Cavity ; Retinaldehyde ; Rhinitis ; Sinusitis ; Triamcinolone

Rippling muscle disease (RMD) is caused by dominant mutations of the caveolin-3 gene (CAV3), and presents with overlapping limb-girdle muscle weakness, elevated creatine kinase (hyper- CKemia), RMD, and distal myopathy. We report a patient with a CAV3 mutation who presented with myalgia, exercise-induced muscle stiffness, hyperCKemia, and percussion-induced rapid muscle contraction and muscle mounding. A familial genetic study revealed the same mutation in two family members, with physical examinations showing that both of them had rippling muscles.

No abstract available.
Agaricales ; Dermatitis

Heterotopic salivary gland tissue (HSGT) consists of salivary tissue in places where it does not normally exist. Usually, HSGT presents as a draining sinus or nodule of the neck. The pathogenesis of HSGT of the neck remains uncertain. But HSGT in the neck is most widely thought to arise from defective closures and ectodermal hetroplasia of the precervical sinus of His. HSGT in the neck is rare and only a few cases have been reported in Korea. We recently experienced a case of HSGT in the neck presenting as a fistula of the anterior lower neck. We thought this as a 2nd brachial cleft anomaly; however, biopsy revealed HSGT. HSGT in the neck should be considered as one of the causes of a neck mass or fistula in children. Herein, we report this case of HSGT with a literature review.