No abstract available.
Purpura, Thrombocytopenic, Idiopathic*

No abstract available.
Blood Platelets* ; Diagnosis* ; Flow Cytometry* ; Integrin beta3* ; Thrombasthenia*

The Peutz-Jeghers syndrome is a familial disesse charaeterized by mucocutaneaus pigmentation, gastrointestinal polyposis, and transmission of autosomal dominant trait. This syndrome is clinically important becuase of the coreplieation caused by the polyp, leading to abdominal pain, gastrointestinal bleeding, and intussusception. Authors experienced a case of Peutz-Jeghers syndrome admitted to Walace Memorial Baptist Hospital who was complained of recurrent abdominal pain, melanin pigmentation of lips, oral mucosa, and digits. UGI series, small bowel series, colon cnema, gastrofiberscopy, and colonoseopy revealed multiyle intestinal polyps. Pathologic findings on endoseopic polypectomy showed hamartomatous polyp.
Abdominal Pain ; Colon ; Hemorrhage ; Intestinal Polyps ; Intussusception* ; Lip ; Melanins ; Mouth Mucosa ; Peutz-Jeghers Syndrome ; Pigmentation ; Polyps ; Protestantism

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

No abstract available.
Anencephaly* ; Humans ; Pregnancy, Twin* ; Twins*

Lipid transfer protein (LTP) is a widely cross-reacting allergen in vegetables, fruits, nuts, or cereals. The amino acids sequence of LTP shows high structural similarity in various foods and thus acts as a pan-allergen. LTP syndrome is a new term for the pattern of clinical symptoms and natural course of allergy caused by LTP antigens. It is frequently reported by peaches mainly in the Mediterranean region, and reports in East Asia are rare. Therefore, this case report introduces a 36-year-old female Asian who experienced recurrent anaphylaxis and oral allergy syndrome, and finally diagnosed with LTP syndrome.

Skin grafting is often required for diabetic ulcerative foot lesions. In skin grafting, effective regional or local anesthesia into the donor and recipient areas plays a significant role in continuous control of pain. We report on a technique of ultrasound-guided nerve block on the femoral, sciatic, and lateral femoral cutaneous nerves in large split-thickness skin grafting for ulcer of the foot and leg.
Anesthesia, Local ; Femoral Nerve ; Foot* ; Humans ; Leg* ; Nerve Block* ; Sciatic Nerve ; Skin Transplantation* ; Tissue Donors ; Ulcer*

PURPOSE: To compare the clinical outcomes of ultrasound-guided percutaneous repair (USPR) and open repair in a ruptured Achilles tendon. MATERIALS AND METHODS: The outcomes of 12 patients with USPR (group A) and 18 patients with open repair (group B) from January 2015 to February 2017 were analyzed retrospectively. The postoperative clinical evaluations were performed using the Arner-Lindholm scale, American Orthopedic Foot and Ankle Society (AOFAS) ankle-hindfoot score, Achilles tendon total rupture score (ATRS), and visual analogue scale for the overall satisfaction and cosmetic satisfaction with the scar, and the starting time of single heel raises. The complications were also evaluated. RESULTS: The Arner-Lindholm scale, AOFAS ankle-hindfoot score, ATRS, starting time of single heel raises were similar in both groups (all p>0.05). Group A showed a significantly higher overall patient's satisfaction and cosmetic satisfaction in than group B (all p < 0.05). Two cases of Achilles tendon elongation were encountered in group A, and 1 case of re-rupture with deep infection and 1 case of superficial infection were experienced in group B. CONCLUSION: USPR showed good clinical outcomes and high satisfaction as well as a low rate of complications, such as sural nerve injury. Therefore, USPR can be considered as an effective surgical treatment option for Achilles tendon ruptures.
Achilles Tendon* ; Ankle ; Cicatrix ; Foot ; Heel ; Humans ; Orthopedics ; Retrospective Studies ; Rupture ; Sural Nerve ; Ultrasonography

PURPOSE: There have only been a few studies on optimal usage of injection material in the regional nerve block for lower extremity operations. The purpose of this study was to evaluate the efficacy of different concentrations of ropivacaine. MATERIALS AND METHODS: A total of 339 patients underwent lower extremity surgery under ultrasound-guided nerve block (combined femoral and sciatic nerve block) at a Chungnam National University Hospital between March 2016 and February 2017 and were randomly assigned to three groups: Group A (0.5%, 44 ml), group B (0.6%, 30 ml), and group C (0.75%, 30 ml). The interval between nerve block procedure and onset of the complete anesthetic effect (complete anesthetic time) was investigated. The degrees of intraoperative pain, and postoperative pain were evaluated using a visual analogue scale (VAS) score. Patient's satisfaction (0–10) was investigated. To evaluate the efficacy in accordance with the concentration under the same dose and same volume, group A and B were compared with group C respectively. RESULTS: There were 108, 118, and 113, in groups A, B, and C, respectively; and there were no significant differences with respect to the number, age, sex, and type of operation (p>0.05). The mean complete anesthetic times were 78.5, 76.4, and 58.6 minutes, respectively. The mean intraoperative VAS scores were 2.04, 0.62, and 0.24; and the mean postoperative VAS scores (6 hours/12 hours) were 2.41/4.08, 0.27/1.24, and 0.38/1.54. The mean patient's satisfactory scores were 8.53, 9.38, and 9.40, respectively. Compared with group C, group A showed significantly longer complete anesthetic time (p < 0.05) and higher intra, postoperative VAS scores (all p < 0.05). Group B showed longer complete anesthetic time (p < 0.05), but no significant difference of intra, postoperative VAS scores (all p>0.05). Patient's satisfactory scores in both group A and B were similar to group C (p>0.05, p>0.05). There were no specific adverse reactions in all groups. CONCLUSION: Ropivacaine 0.6% as well as 0.75% are safe and effective anesthetics under the same volume (30 ml) for regional nerve block of the lower extremity. However, taking into account of the longer complete anesthetic time, the operation start time must be adjusted.
Anesthetics ; Chungcheongnam-do ; Femoral Nerve ; Humans ; Lower Extremity* ; Nerve Block* ; Pain, Postoperative ; Prospective Studies* ; Sciatic Nerve ; Ultrasonography

PURPOSE: This study compared the clinical results of an ultrasound (US)-guided regional nerve block with those of general anesthesia in below-knee amputation (BKA) surgery. In addition, the 1-year mortality rate of BKA patients was evaluated in relation to the preoperative comorbidity and postoperative hemoglobin level. MATERIALS AND METHODS: Among 47 patients who underwent BKA between January 2011 and August 2016, 18 patients in the US-guided regional nerve block group (group 1) and 29 patients in the general anesthesia group (group 2) were analyzed retrospectively and compared. For the clinical assessment, the 30-day mortality, 1-year mortality, postoperative hemoglobin level, and postoperative complications of both groups were investigated. The visual analogue scale (VAS) pain scores at postoperative 1, 6, and 12 hours for both groups were evaluated. The 1-year mortality of BKA patients was also evaluated in relation to the preoperative comorbidity and postoperative hemoglobin level (hemoglobin < 7 g/dl). RESULTS: Significant differences in the 30-day mortality were observed between the two groups (p=0.023). Group 1 showed a higher 30-day mortality but the 1-year mortality was similar in both groups (p=0.051). The postoperative hemoglobin level was similar in the two groups (p=0.085). The VAS pain scores for the postoperative 1-hour and 6-hour differed significantly between the two groups (p < 0.001). The VAS pain scores for postoperative 12-hour showed no significant difference (p=0.10). The 1-year mortality rate of both groups was not affected by hypertension, diabetes mellitus, arteriosclerosis obliterans, and postoperative hemoglobin less than 7 g/dl, but was affected by chronic kidney disease (CKD) (Pearson's chi-square=14.39, p < 0.05). CONCLUSION: Although US-guided regional nerve block showed comparable 1-year mortality and postoperative hemoglobin levels compared to general anesthesia in BKA, it showed better results in postoperative 1, 6 hour pain control than general anesthesia. The 1-year mortality of BKA was affected by CKD. Therefore, careful consideration is needed for patients with CKD before undergoing BKA regardless of the anesthetic methods.
Amputation* ; Anesthesia, General ; Arteriosclerosis Obliterans ; Comorbidity ; Diabetes Mellitus ; Humans ; Hypertension ; Mortality ; Nerve Block* ; Postoperative Complications ; Renal Insufficiency, Chronic ; Retrospective Studies ; Ultrasonography