No abstract available.

Severe congenital neutropenia is a heterozygous group of bone marrow failure syndromes that cause lifelong infections. Mutation of the ELANE gene encoding human neutrophil elastase is the most common genetic alteration. A Korean female pediatric patient was admitted because of recurrent cervical lymphadenitis without abscess formation. She had a past history of omphalitis and isolated neutropenia at birth. The peripheral blood showed a markedly decreased absolute neutrophil count, and the bone marrow findings revealed maturation arrest of myeloid precursors at the promyelocyte to myelocyte stage. Her direct DNA sequencing analysis demonstrated an ELANE gene mutation (c.607G > C; p.Gly203Arg), but her parents were negative for it. She showed only transient response after subcutaneous 15 microg/kg/day of granulocyte colony stimulating factor administration for six consecutive days. During the follow-up observation period, she suffered from subsequent seven febrile illnesses including urinary tract infection, septicemia, and cellulitis.
Bacterial Infections ; Base Sequence ; Female ; Granulocyte Colony-Stimulating Factor/administration & dosage/therapeutic use ; Humans ; Infant ; Leukocyte Count ; Leukocyte Elastase/*genetics ; Lymphadenitis ; Neutropenia/blood/*congenital/genetics ; Neutrophils ; Point Mutation ; Republic of Korea ; Sequence Analysis, DNA

Pain-related impairment assessment by the fifth edition of the American Medical Association Guides had many ambiguous points, and therefore, it was not applicable directly in Korea. Several disputable pain disorders were excluded from the list of impairment evaluation, and complex regional pain syndrome was chosen as the first object of impairment evaluation. Scales such as Korean version of modified Barthel index for assessing the activity of daily livings and Beck Depression Inventory for assessing depression were added, and pain severity, pain treatment, pain behavior, etc. were scored. In order to objectify as much as possible and to remove the room for misuse, we develop a new rating system based on the concept of total score.
*Disability Evaluation ; Humans ; Korea ; *Pain Measurement ; Program Development ; Reflex Sympathetic Dystrophy/classification/physiopathology ; Sickness Impact Profile

No abstract available.
Female ; Hemophilia A* ; Humans ; Postpartum Period*

BACKGROUND: Thus far, research studies on community-acquired pneumonia (CAP) have focused on its clinical severity. Recently, it has been determined that procalcitonin (PCT) level is correlated with severity of CAP. A retrospective study conducted at our hospital used risk predictability and PCT to determine whether or no PCT is useful in assessing the severity of CAP. METHODS: This study covered 92 CAP cases that were admitted to the respiratory department at Changwon Fatima Hospital between July 1, 2008 and June 30, 2009. All enrolled subjects were measured for infection markers and risk predictability. RESULTS: Based on hospital admission data, enrolled subjects had Pneumonia Severity Index (PSI) scores serving as risk predictors showed that both PCT and white blood cell (WBC) were statistically significant as infection markers (p=0.001, 0.037). Thus, this study used ROC curves in PSI for data analysis. As a result, it was determined that the area under curve (AUC) of PCT and WBC was 0.694 and 0.593 respectively, indicating that PCT has a higher test value for WBC, when PCT was higher than 0.745 ng/mL. In addition, it was found that PCT levels higher than 0.745 ng/mL had higher PSI scores than the group with PCT lower than 0.745 ng/mL (p=0.032). CONCLUSION: In order to predict risk of pneumonia cases admitted due to symptoms of CAP, it is important to consider PCT as well as PSI, and follow-up monitoring of PCT cases.
Area Under Curve ; Calcitonin ; Community-Acquired Infections ; Follow-Up Studies ; Humans ; Leukocytes ; Pneumonia ; Protein Precursors ; Retrospective Studies ; ROC Curve ; Statistics as Topic

BACKGROUND: Thus far, research studies on community-acquired pneumonia (CAP) have focused on its clinical severity. Recently, it has been determined that procalcitonin (PCT) level is correlated with severity of CAP. A retrospective study conducted at our hospital used risk predictability and PCT to determine whether or no PCT is useful in assessing the severity of CAP. METHODS: This study covered 92 CAP cases that were admitted to the respiratory department at Changwon Fatima Hospital between July 1, 2008 and June 30, 2009. All enrolled subjects were measured for infection markers and risk predictability. RESULTS: Based on hospital admission data, enrolled subjects had Pneumonia Severity Index (PSI) scores serving as risk predictors showed that both PCT and white blood cell (WBC) were statistically significant as infection markers (p=0.001, 0.037). Thus, this study used ROC curves in PSI for data analysis. As a result, it was determined that the area under curve (AUC) of PCT and WBC was 0.694 and 0.593 respectively, indicating that PCT has a higher test value for WBC, when PCT was higher than 0.745 ng/mL. In addition, it was found that PCT levels higher than 0.745 ng/mL had higher PSI scores than the group with PCT lower than 0.745 ng/mL (p=0.032). CONCLUSION: In order to predict risk of pneumonia cases admitted due to symptoms of CAP, it is important to consider PCT as well as PSI, and follow-up monitoring of PCT cases.
Area Under Curve ; Calcitonin ; Community-Acquired Infections ; Follow-Up Studies ; Humans ; Leukocytes ; Pneumonia ; Protein Precursors ; Retrospective Studies ; ROC Curve ; Statistics as Topic

BACKGROUND AND OBJECTIVES: Nasal obstruction is common in patients with sleep disordered breathing (SDB). Polysomnography (PSG) is a standard diagnositc tool for sleep disorders, but it can not evaluate the quality of life (QOL). We tried to assess the efficacy of the septoturbinoplasty on patients with SDB and nasal obstruction by using self reported measures that have been known as a tool for evaluating the general and disease specific QOL. SUBJECTS AND METHOD: Thirty-five patients who had snoring and sleep apnea confirmed by PSG and symptomatic nasal obstruction caused by deviated nasal septum were enrolled. All patients underwent septoturbinoplasty to improve nasal obstruction. QOL was assessed before and 2 months after surgery. Medical Outcome Study Short Form 36 (SF-36) and Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Stanford Sleepiness Scale (SSS), Insomnia Severity Index (ISI), Beck Depression Inventory (K-BDI), Nasal Obstruction Symptom Evaluation (NOSE) scale were included in the measures. A statistical analysis was performed using a paired t-test. RESULTS: Snoring sound level, PSQI, ESS, ISI, and NOSE were improved significantly (p<0.05). SSS, BDI and total SF-36 were improved but not significantly (p>0.05). CONCLUSION: QOL in patients with SDB and nasal obstruction was improved by nasal surgery. Different kinds of self-reported measures could be more informative as they can provide pre and post treatment results with respect to psychosomatic effects in patients with SDB.
Depression ; Humans ; Nasal Obstruction ; Nasal Septum ; Nasal Surgical Procedures ; Nose ; Outcome Assessment (Health Care) ; Polysomnography ; Quality of Life ; Self Report ; Sleep Apnea Syndromes ; Sleep Wake Disorders ; Sleep Initiation and Maintenance Disorders ; Snoring ; Symptom Assessment

BACKGROUND: Dipeptidyl peptidase-4 (DPP-4) inhibitor add-on therapy is a new option for patients with inadequately controlled type 2 diabetes who are taking combined metformin and sulfonylurea (SU). We evaluated the efficacy and safety of this triple therapy and the characteristics of rapid responders and hypoglycemia-prone patients. METHODS: We included 807 patients with type 2 diabetes who were prescribed a newly added DPP-4 inhibitor to ongoing metformin and SU in 2009 to 2011. Glycemia and other metabolic parameters at baseline, 12, 24, and 52 weeks, as well as episodes of hypoglycemia were analyzed. Rapid responders were defined as patients with > or =25% reduction in glycosylated hemoglobin (HbA1c) within 12 weeks. RESULTS: At baseline, while on the submaximal metformin and SU combination, the mean HbA1c level was 8.4%. Twelve weeks after initiation of DPP-4 inhibitor add-on, 269 patients (34.4%) achieved an HbA1c level < or =7%. Sixty-six patients (8.2%, 47 men) were rapid responders. The duration of diabetes was shorter in rapid responders, and their baseline fasting plasma glucose (FPG), HbA1c, C-peptide, and homeostasis model assessment of insulin resistance were significantly higher. Patients who experienced hypoglycemia after taking DPP-4 inhibitor add-on were more likely to be female, to have a lower body weight and lower triglyceride and FPG levels, and to have higher homeostasis model assessment of beta-cells. CONCLUSION: An oral hypoglycemic triple agent combination including a DPP-4 inhibitor was effective in patients with uncontrolled diabetes. Proactive dose reduction of SU should be considered when a DPP-4 inhibitor is added for rapid responders and hypoglycemia-prone patients.
Blood Glucose ; Body Weight ; C-Peptide ; Diabetes Mellitus, Type 2 ; Dipeptidyl-Peptidase IV Inhibitors ; Fasting ; Female ; Hemoglobin A, Glycosylated ; Homeostasis ; Humans ; Hypoglycemia ; Insulin Resistance ; Metformin* ; Sulfonylurea Compounds ; Triglycerides

The mortality of acute pancreatitis complicated by acute renal failure (ARF) remains high. Among several therapeutic modalities, peritoneal dialysis (PD) has advantages due to its ability to remove toxic materials in the peritoneal exudate as well as urotoxic substances in the blood. We report successful treatment with PD in a patient with acute pancreatitis and ARF. A 41-year-old heavy drinker was admitted due to acute pancreatitis complicated by ARF. A therapeutic plan of PD was designed. A Tenckhoff PD catheter was used, considering its lower potential for infection compared to conventional catheters. The frequency of PD was set at 8 times per day, considering the catabolic state in pancreatitis. The clinical symptoms and biochemical parameters showed remarkable improvement during the follow up period. In conclusion, PD is one of the effective treatment modalities for treating ARF in patients with pancreatitis.
Acute Kidney Injury ; Adult ; Catheters ; Exudates and Transudates ; Follow-Up Studies ; Humans ; Pancreatitis ; Peritoneal Dialysis

We report a case of tubulointerstitial nephritis and uveitis (TINU) syndrome in an old age female. A 66-year-old woman presented with nonspecific systemic symptoms and severe renal dysfunction. Renal biopsy showed acute interstitial nephritis and ophthalmologic examination revealed bilateral panuveitis. Evaluations for connective tissue diseases and infectious diseases were negative. She was treated with total eight sessions of hemodialysis, oral steroids and topical steroids. Renal function had improved significantly and remained stable at follow-up, although it did not fully recovered yet. TINU syndrome should be considered in cases of unexplained tubulointerstitial nephritis, especially in the presence of ocular symptom.
Acute Kidney Injury ; Aged ; Biopsy ; Communicable Diseases ; Connective Tissue Diseases ; Female ; Follow-Up Studies ; Humans ; Nephritis, Interstitial ; Panuveitis ; Renal Dialysis ; Steroids ; Uveitis