PURPOSE: In this study, the risk factors of failure of ibuprofen treatment in preterm infants with hemodynamically significant patent ductus arteriosus (hsPDA) were investigated. METHODS: Among 403 preterm infants (<32 weeks gestation) born between January 2010 and December 2012, 125 infants treated with ibuprofen for hsPDA were retrospectively reviewed. The preterm infants were divided into the following groups according to their response to the 1st and 2nd cycles of ibuprofen treatment: responder groups I and II, closure of the ductus arteriosus after the 1st and 2nd cycles of ibuprofen treatment; and non-responder groups I and II, persistency of hsPDA after the 1st and 2nd cycles of ibuprofen treatment. RESULTS: One hundred twenty five infants were enrolled in the study: 74 in responder group I, 51 in non-responder group I, 14 in responder group II, and 22 in non-responder group II. In non-responder group I, the gestational age and birth weight were smaller, the postnatal steroid treatment was more frequent, and the duration of mechanical ventilation and the days spent in the hospital were prolonged.I n non-responder group II, the gestational age and birth weight were smaller, the diameters of the ductus arteriosus were larger, and the inotropics use was more frequent. CONCLUSION: Failure of ibuprofen treatment of hsPDA is associated with the diameter of the ductus arteriosus and with inotropics use. Obtaining data regarding these is expected to help in determining if early direct surgical ligation is needed.
Birth Weight ; Ductus Arteriosus ; Ductus Arteriosus, Patent* ; Gestational Age ; Humans ; Ibuprofen* ; Infant ; Infant, Newborn ; Infant, Premature* ; Ligation ; Respiration, Artificial ; Retrospective Studies ; Risk Factors*

Acanthosis nigricans is a symmetric eruption characterized by the presence of a hyperpigmented, velvety cutaneous thickening, that can develop on any part of the body, but characteristically affects the flexural areas of the body. The velvety hyperkeratotic lesions can be located on the dorsum of the hands and feet in dark-skinned people in the form of a variant of acanthosis nigricans called as acral acanthotic anomaly or acral type acanthosis nigricans. Although acanthosis nigricans is associated with malignant tumors, particularly gastric carcinoma, acral type acanthosis nigricans has never been reported to be associated with gastric adenocarcinoma. In our present study, we describe a case of 58-year-old man with acral type acanthosis nigricans and its association with carcinoma of the stomach; a marked improvement was seen in the skin condition of the patient with chemotherapy.
Acanthosis Nigricans ; Adenocarcinoma ; Foot ; Hand ; Humans ; Middle Aged ; Skin

Glossopharyngeal neuralgia (GPN) is a rare condition characterized by paroxysms of lancinating pain in the regions of the tonsil, ear, larynx and tongue. The pain is typically triggered by activities such as swallowing, speaking or coughing. Fifty-seven years old female patient had suffered paroxysms of severe pain in the unilateral region of the glossopharyngeal innervation for several years, and long term drug therapy result in serious pancytopenia probably due to carbamazepine. The patient with idiopathic glossopharyngeal neuralgia received glossopharyngeal nerve block and her symptoms were relieved completely. Glossopharyngeal nerve block with alcohol was done by extraoral approach and satisfactory results were obtained.
Carbamazepine ; Cough ; Deglutition ; Drug Therapy ; Ear ; Female ; Glossopharyngeal Nerve Diseases* ; Glossopharyngeal Nerve* ; Humans ; Larynx ; Palatine Tonsil ; Pancytopenia ; Tongue

PURPOSE: Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn screening test. METHODS: VLBWIs (January 2010 to December 2012) were divided into two groups according to dysfunction-specific thyroid hormone replacement therapy, and associated factors were evaluated. RESULTS: Of VLBWIs, 246 survivors were enrolled. Only 12.2% (30/246) of enrolled subjects exhibited thyroid dysfunction requiring thyroid hormone replacement. Moreover, only one out of 30 subjects who required thyroid hormone treatment had abnormal thyroid function in the newborn screening test with measured TSH. Most of the subjects in the treatment group (22/30) exhibited delayed TSH elevation. Gestational age, Apgar score, antenatal steroids therapy, respiratory distress syndrome, patent ductus arteriosus, sepsis, intraventricular hemorrhage, postnatal steroids therapy, and duration of mechanical ventilation did not differ between the two groups. Birth weight was smaller and infants with small for gestational age were more frequent in the treatment group. CONCLUSION: Physicians should not rule out suggested hypothyroidism, even when thyroid function of a newborn screening test is normal. We suggest retesting TSH and free thyroxine in high risk preterm infants with an initially normal TSH level using a newborn screening test.
Apgar Score ; Birth Weight ; Congenital Hypothyroidism ; Ductus Arteriosus, Patent ; Gestational Age ; Hemorrhage ; Hormone Replacement Therapy ; Humans ; Hypothyroidism ; Infant ; Infant, Newborn ; Infant, Premature* ; Infant, Very Low Birth Weight* ; Mass Screening ; Neonatal Screening ; Respiration, Artificial ; Respiratory Therapy ; Risk Factors ; Sepsis ; Steroids ; Survivors ; Thyroid Function Tests ; Thyroid Gland* ; Thyrotropin ; Thyroxine

Vertebral, anal, cardiac, tracheo-esophageal, renal, and limb(VACTERL) association is defined as the presence of at least three of the above-mentioned six manifestations. An estimated incidence of the VACTERL association is 1 in 20,000 to 35,000 live births although the diagnostic criteria vary. The VACTERL association is highly heterogeneous in clinical presentation. It may represent a spectrum from the less severely affected to the more severely affected. Diagnosis is difficult because of the number of disorders that have overlapping features with trisomy 13 syndrome, trisomy 18 syndrome, trisomy 21 syndrome, Feingold syndrome, and so on. The incidence of trisomy 18 syndrome, a type of a chromosomal disorder, is estimated to be 1 in 6,000-8,000 live births. It includes characteristic craniofacial anomalies, clenched hand with overlapping of index finger over third, fifth finger over fourth, underdeveloped thumbs, short sternum, cardiac anomalies such as ventricular septal defect, and renal anomalies such as horseshoe kidney. Approximately over 50% of infants with trisomy 18 syndrome live less than one week. In 1983, Khoury et al. reported VACTERL association combined with trisomy 18 syndrome. Here, we report a case of a low birth weight female infant with VACTERL association, whose second diagnosis is Edward syndrome, and that she also has another combined anomaly, meningomyelocele. To the best of our knowledge, this is the first reported case of VACTERL association with meningomyelocele combined with trisomy 18 syndrome in Korea.
Chromosome Disorders ; Diagnosis ; Down Syndrome ; Female ; Fingers ; Hand ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Kidney ; Korea ; Live Birth ; Meningomyelocele* ; Sternum ; Thumb ; Trisomy*

A congenital ureteral valve is a rare disease, with the first case presented in 1887, since when, only 42 cases have subsequently been reported. From a review of the reported cases, this abnormality was often found to be associated with other urological disorders, such as vesicoureteral reflux, ectopic ureter, complete and incomplete duplication of the kidney, and contralateral renal atrophy. Here, the case of an adult patient with multiple congenital ureteral valves and renal atrophy is reported.
Adult* ; Atrophy ; Constriction, Pathologic ; Humans ; Kidney ; Rare Diseases ; Ureter* ; Vesico-Ureteral Reflux

BACKGROUND: Studies using a transcranial Doppler (TCD) to establish cerebral vasoreactivity (CVR) have mostly focused on the anterior circulation. The purpose of this study is not only to evaluate the feasibility of the power motion mode Doppler (PMD) with a probes fixating device, but also to simultaneously measure the CVR between the middle cerebral artery (MCA) and the basilar artery (BA) during hypercapnea simulated by the rebreathing technique. METHODS: Twenty eight healthy volunteers were enrolled. Baseline hemodynamic values (heart rate, blood pressure) were measured while volunteers sat in a comfortable position for 5 minutes. The TCD was performed in two steps. First, velocities and spectra of the MCAs were simultaneously monitored. Then, the velocities and spectra of the MCA and BA were simultaneously monitored by a headset that included an anterior-posterior probes fixating device. The equation for CVR was ([maximum mean velocity baseline mean velocity] x 100/baseline mean velocity). RESULTS: Baseline mean velocities were revealed as follows: (64.0+/-13.7, 65.0+/-11.9 cm/s in right and left MCA; p>0.05; 67.3+/-12.2, -45.3+/-7.6 cm/s in dominant MCA and BA). CVR did not differ between the dominant MCA and the BA (46.1+/-12.1, 46.0+/-15.1%; p>0.05), nor between the right and left MCAs (46.9+/-15.2, 46.4+/-14.8%; p>0.05). There was a positive linear correlation between the CVR of the dominant MCA and that of the BA (r=0.856; p<0.001). CONCLUSIONS: PMD with a probes fixating device for accurate insonation is a useful tool for evaluating the relative CVR between the MCA and BA. Our study suggests that CVR values of the BA are similar to those of the MCA.
Basilar Artery ; Healthy Volunteers ; Hemodynamics ; Middle Cerebral Artery* ; Volunteers

Background: A substantial cardiovascular disease risk remains even after optimal statin therapy. Comparative predictiveness of major lipid and lipoprotein parameters for cardiovascular events in patients with type 2 diabetes mellitus (T2DM) who are treated with statins is not well documented. Methods: From the Korean Nationwide Cohort, 11,900 patients with T2DM (≥40 years of age) without a history of cardiovascular disease and receiving moderate- or high-intensity statins were included. The primary outcome was the first occurrence of major adverse cardiovascular events (MACE) including ischemic heart disease, ischemic stroke, and cardiovascular death. The risk of MACE was estimated according to on-statin levels of low-density lipoprotein cholesterol (LDL-C), triglyceride (TG), highdensity lipoprotein cholesterol (HDL-C), and non-HDL-C. Results: MACE occurred in 712 patients during a median follow-up period of 37.9 months (interquartile range, 21.7 to 54.9). Among patients achieving LDL-C levels less than 100 mg/dL, the hazard ratios for MACE per 1-standard deviation change in ontreatment values were 1.25 (95% confidence interval [CI], 1.07 to 1.47) for LDL-C, 1.31 (95% CI, 1.09 to 1.57) for non-HDL-C, 1.05 (95% CI, 0.91 to 1.21) for TG, and 1.16 (95% CI, 0.98 to 1.37) for HDL-C, after adjusting for potential confounders and lipid parameters mutually. The predictive ability of on-statin LDL-C and non-HDL-C for MACE was prominent in patients at high cardiovascular risk or those with LDL-C ≥70 mg/dL. Conclusion On-statin LDL-C and non-HDL-C levels are better predictors of the first cardiovascular event than TG or HDL-C in patients with T2DM.

Background: Progressive deterioration of β-cell function is a characteristic of type 2 diabetes mellitus (T2DM). We aimed to investigate the relative contributions of clinical factors to β-cell function in T2DM. Methods: In a T2DM cohort of 470 adults (disease duration 0 to 41 years), β-cell function was estimated using insulinogenic index (IGI), disposition index (DI), oral disposition index (DIO), and homeostasis model assessment of β-cell function (HOMA-B) derived from a 75 g oral glucose tolerance test (OGTT). The relative contributions of age, sex, disease duration, body mass index, glycosylated hemoglobin (HbA1c) levels (at the time of the OGTT), area under the curve of HbA1c over time (HbA1c AUC), coefficient of variation in HbA1c (HbA1c CV), and antidiabetic agents use were compared by standardized regression coefficients. Longitudinal analyses of these indices were also performed. Results: IGI, DI, DIO, and HOMA-B declined over time (P<0.001 for all). Notably, HbA1c was the most significant factor affecting IGI, DI, DIO, and HOMA-B in the multivariable regression analysis. Compared with HbA1c ≥9%, DI was 1.9-, 2.5-, 3.7-, and 5.5-fold higher in HbA1c of 8%–<9%, 7%–<8%, 6%–<7%, and <6%, respectively, after adjusting for confounding factors (P<0.001). Conversely, β-cell function was not affected by the type or duration of antidiabetic agents, HbA1c AUC, or HbA1c CV. The trajectories of the IGI, DI, DIO, and HOMA-B mirrored those of HbA1c. Conclusion β-Cell function declines over time; however, it is flexible, being largely affected by recent glycemia in T2DM.

Background: Progressive deterioration of β-cell function is a characteristic of type 2 diabetes mellitus (T2DM). We aimed to investigate the relative contributions of clinical factors to β-cell function in T2DM. Methods: In a T2DM cohort of 470 adults (disease duration 0 to 41 years), β-cell function was estimated using insulinogenic index (IGI), disposition index (DI), oral disposition index (DIO), and homeostasis model assessment of β-cell function (HOMA-B) derived from a 75 g oral glucose tolerance test (OGTT). The relative contributions of age, sex, disease duration, body mass index, glycosylated hemoglobin (HbA1c) levels (at the time of the OGTT), area under the curve of HbA1c over time (HbA1c AUC), coefficient of variation in HbA1c (HbA1c CV), and antidiabetic agents use were compared by standardized regression coefficients. Longitudinal analyses of these indices were also performed. Results: IGI, DI, DIO, and HOMA-B declined over time (P<0.001 for all). Notably, HbA1c was the most significant factor affecting IGI, DI, DIO, and HOMA-B in the multivariable regression analysis. Compared with HbA1c ≥9%, DI was 1.9-, 2.5-, 3.7-, and 5.5-fold higher in HbA1c of 8%–<9%, 7%–<8%, 6%–<7%, and <6%, respectively, after adjusting for confounding factors (P<0.001). Conversely, β-cell function was not affected by the type or duration of antidiabetic agents, HbA1c AUC, or HbA1c CV. The trajectories of the IGI, DI, DIO, and HOMA-B mirrored those of HbA1c. Conclusion β-Cell function declines over time; however, it is flexible, being largely affected by recent glycemia in T2DM.