The Holt-Oram syndrome or cardiomelic syndrome is characterized by the association of upper limb and heart malformations. Most frequently, abnormalities of the thumb and secundum atrial septal defects are associated with the disease. The mode of inheritance is autosomal dominant. The etiology of this disease is unknown but is most likely of multifactorial origin. Here we report a case Holt-Oram syndrome with affected mother which was diagnosed at 18th gestational weeks by prenatal ultrasonograpy. Fetal ultrasonography revealed abnormalities of upper limbs, and heart. The upper limbs were shorter than normal, syndactyly of both hands were seen and both wrists were markedly angulated. Ventricular septal defect was suspicious. After genetic counselling her pregnancy was terminated at 22nd gestational week.
Hand ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Mothers* ; Pregnancy ; Syndactyly ; Thumb ; Ultrasonography* ; Ultrasonography, Prenatal ; Upper Extremity ; Wills ; Wrist

BACKGROUND: Laparoscopic surgery is replacing conventional surgical techniques due to its many advantages. However the possibility of respiratory complications during CO2-induced pneumoperitoneum remain. Tracheal gas insufflation (TGI) has been shown to be a useful adjunct to mechanical ventilation in hypercapneic patients. This study investigated the effectiveness of TGI in reducing the PaCO2 level in hypercapneic patients during laparoscopic surgery without increasing the peak inspiratory pressure (PIP) and usefulness of a Univent tube(R) as a device for TGI. METHODS:Twenty-four patients who were scheduled to undergo gynecological laparoscopic surgery, were enrolled in this study. Anesthesia was induced and maintained with propofol, rocuronium and N2O-O2-sevoflurane. The suction port of the endobronchial blocker of the Univent tube(R) was used for the path of TGI. Data including the ABGA and respiratory parameters were measured three times, the pre-CO2 peritoneum (pre-CO2 pneumoperitoneum point, PCP), 15 min after CO2 peritoneum (after-CO2 pneumoperitoneum point, ACP) and after 15 min TGI (TGI point, TGIP). RESULTS: At ACP, the PaCO2 and PIP had increased more significantly than PCP. After TGI, the PaCO2 was decreased more significantly than ACP, but the PIP did not increased. CONCLUSIONS: TGI is a useful adjunct to mechanical ventilation in hypercapneic patients during laparoscopic surgery, and a univent tube(R) is an economic and convenient device for TGI.
Anesthesia ; Humans ; Insufflation* ; Laparoscopy* ; Peritoneum ; Pneumoperitoneum ; Propofol ; Respiration, Artificial ; Suction

Pylephlebitis, or suppurative thrombophlebitis of the portal venous system, is a rare condition occurring secondary to abdominal infections such as diverticulitis. Pylephlebitis can be diagnosed via ultrasonography or CT scan, and is characterized by the presence of a thrombus in the portal vein and bacteremia. However, the diagnosis may be delayed due to the vague nature of the clinical symptoms, causing morbidity and mortality due to pylephlebitis to remain high. Early diagnosis and immediate antibiotic therapy are important for favorable prognosis. Therefore, pylephlebitis should be considered in the differential diagnosis for cases of nonspecific abdominal pain and fever. We report a case of pylephlebitis secondary to diverticulitis, associated with Pseudomonas aeruginosa sepsis. Such cases have not been widely reported.
Abdominal Pain ; Bacteremia ; Diagnosis ; Diagnosis, Differential ; Diverticulitis* ; Early Diagnosis ; Fever ; Liver Abscess* ; Liver* ; Mortality ; Portal Vein ; Prognosis ; Pseudomonas aeruginosa* ; Pseudomonas* ; Sepsis* ; Thrombophlebitis ; Thrombosis ; Tomography, X-Ray Computed ; Ultrasonography

PURPOSE: Vascular access failure is the most common reason for hospitalization among hemodialysis (HD) patients. Cilostazol, which has antiplatelet action and vasodialtory effects, significantly reduces the risk of restenosis after percutaneous coronary intervention in many patients. We conducted this study to evaluate the relationship between the use of antiplatelet agents, especially cilostazol, and arteriovenous fistula (AVF) patency in HD patients. METHODS: A total of 241 patients underwent native AVF creation from January 2001 to December 2008. Among these patients, we selected 86 patients excluding 38 patients (15.8%) with primary technical failure, 49 patients without complete data and 68 patients used cilostazol less than 1 month. Demographic characteristics, medication history and fistula failure rate were collected and analyzed to elucidate the effect of cilostazol to native AVF. RESULTS: From all groups, AVF failure occurred in 24 patients (27.9%). 28 patients received cilostazol (62.3 %) and mean duration of cilostazol therapy was 229.5+/-115.7 days. All patients were classified into two groups according to cilostazol (Cilostazol [n=28, 32.6%] vs. non-Cilostazol [n=58, 67.4%]. There was no statisticallly significant difference in failure rate between the two groups (32.1% vs. 25.9%, p=0.543). In diabetes group, patients who received statin have much lower AVF failure rate (0% vs. 32.4%, p=0.024). Logistic regression analysis showed that female was independent risk factor for access failure (HR 5.549, CI 1.104-27.877, p=0.037). CONCLUSION: Cilostazol and other antiplatelet agent had a no significant association with AVF patency. Female was an independent risk factor for access failure.
Arteriovenous Fistula ; Female ; Fistula ; Hospitalization ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Logistic Models ; Percutaneous Coronary Intervention ; Platelet Aggregation Inhibitors ; Renal Dialysis ; Risk Factors ; Tetrazoles ; Vascular Patency

BACKGROUND: This study was conducted to evaluate the effects of fentanyl and midazolam when used as adjuvant in a supraclavicular brachial plexus block. METHODS: 100 adult patients with an ASA status of I-II that were scheduled to undergo upper extremity surgery performed under a supraclavicular brachial plexus block were prospectively evaluated in this study.The patients were randomly divided into 4 study groups:Group 1, which received 40 ml of 1.5% lidocaine, Group 2, which received 3 mg of midazolam with 40 ml of 1.5% lidocaine, Group 3, which received 100microgram of fentanyl with 40 ml of 1.5% lidocaine, and Group 4, which received 3 mg of midazolam and 100microgram of fentanyl with 40 ml of 1.5% lidocaine.The onset time, as well as the duration of analgesia and motor blocks, proportion of successful blocks, hemodynamic parameters, and adverse events were then noted. RESULTS: The incidence of successful block was higher in group 4 (92%) than in any other groups (68-72%) (P = 0.185). In addition, the mean duration of analgesia was longer in groups 2 and 4 (165 min and 175 min) than in groups 1 and 3 (114 min and 131 min) (P < 0.05).Furthermore, the mean duration of motor block was longer in groups 2 and 4 (169 min and 180 min) than in groups 1 and 3 (123 min and 126 min) (P < 0.05).No significant difference was observed in the onset time of the sensory block and motor block when the groups were compared. CONCLUSIONS: Although the addition of 3 mg of midazolam and 100microgram of fentanyl to lidocaine in a supraclavicular brachial plexus block does not affect the onset of sensory or motor block, it does prolong the duration of analgesia and motor block.
Adult ; Analgesia ; Brachial Plexus ; Fentanyl ; Hemodynamics ; Humans ; Incidence ; Lidocaine ; Midazolam ; Prospective Studies ; Upper Extremity

BACKGROUND: Loss of bone mass is usually detected after BMT. The causes of bone loss are related with gonadal dysfunction and immunosuppressants. Cytokines, especially IL-6, play an important role in the pathogenesis of postmenopausal osteoporosis. However, the pathogenetic role of cytokines in post-BMT bone loss is unknown and data on the changes of cytokines in accordance with bone turnover markers are scarce. The aim of this study is to assess the relationship of bone turnover markers and cytokines of peripheral blood and bone marrow before and after allogeneic BMT. METHODS: This prospective study included two analyses. The first was a study of 46 BMT recipients, examining the relationship between bone turnover markers and cytokines of serum which were measured before and 1, 2, 3, 4 week and 3 months after BMT. The second was a study of 14 BMT patients, measuring bone marrow plasma cytokines such as IL-6 and TNF-alpha at post-BMT 3 week and bone turnover marker at the same time to assess the relationship between two parameters. RESULTS: Serum ICTP, bone resorption marker, increased progressively until 4 weeks (peak) after BMT and then decreased thereafter. Serum osteocalcin, bone formation marker, decreased progressively until 3 weeks after BMT and then increased thereafter. There was positive correlation between serum ICTP and bone marrow IL-6 levels at the post-BMT 3 week with a statistical significance, but the correlation between bone turnover markers and bone marrow TNF-alpha or peripheral blood cytokines was not found. CONCLUSION: Our data suggest that the progressive increase of bone resorption after BMT is related with the increase of bone marrow IL-6, which is a potent stimulator of bone resorption in vivo.
Bone Marrow Transplantation* ; Bone Marrow* ; Bone Resorption ; Cytokines* ; Female ; Gonads ; Humans ; Immunosuppressive Agents ; Interleukin-6 ; Osteocalcin ; Osteogenesis ; Osteoporosis ; Osteoporosis, Postmenopausal ; Plasma ; Prospective Studies ; Tumor Necrosis Factor-alpha

BACKGROUND AND OBJECTIVES: It is necessary to establish the most efficient diagnostic and therapeutic method for benign paroxysmal positional vertigo (BPPV), which is appropriate for Korean healthcare system. We aimed to evaluate current state of Korean clinician's diagnostic and therapeutic approaches for BPPV. MATERIALS AND METHODS: A 16-item survey was emailed to the members of dizziness department of Otology Research Interest Group in the Korean Otologic Society (n=68). 43 were returned and analyzed. RESULTS: All respondents (100%) used Dix-Hallpike test as a diagnostic tool for vertical canal-BPPV. Supine roll test was used for diagnosing lateral canal BPPV in nearly all the respondents (97.7%). Epley maneuver was chosen as otolith repositioning maneuver (ORM) for posterior canal BPPV in all respondents and barbecue rotation (BBQ) was used for treating lateral canal BPPV with geotropic nystagmus in 95.3% of respondents. Extreme variation was noted for therapeutic approach of lateral canal BPPV with ageotropic nystagmus BBQ, with 4 kinds of ORM and adjunctive measures to liberate otolith from cupula, while BBQ was again the most commonly used ORM (76.7%). CONCLUSION: The development of practical and efficient ORM for lateral canal BPPV with ageotropic nystagmus is necessary.
Surveys and Questionnaires ; Delivery of Health Care ; Dizziness ; Electronic Mail ; Korea ; Otolaryngology ; Otolithic Membrane ; Public Opinion ; Vertigo