Latent polyglandular autoimmune syndrome type 2 case diagnosed during a shock manifestation.

There are many types of polyglandular autoimmune syndrome (PAS). PAS type 2 is the most common type among adults. For PAS type 2 (PAS-2) diagnosis, detection of Addison's disease with autoimmune thyroid disease and/or type 1 diabetes mellitus are required. Premature ovarian insufficiency, pernicious anemia, vitiligo, alopecia, myasthenia gravis, celiac disease and autoimmune diabetes insipidus may be comorbidities of this condition. Contrary to the common belief, latent PAS is more common than the manifest forms. Here, we present a PAS-2 case diagnosed via adrenal crisis. At the time of diagnosis, the case was observed to have thyroid, adrenal and ovarian involvement. Therefore, PAS-2 and possible immunologic disorders were discussed.

Ultrasound-guided supraclavicular brachial plexus block for analgesia during endovascular treatment of dysfunctional hemodialysis fistulas.

PURPOSE: To evaluate prospectively the efficacy and safety of ultrasound (US)-guided supraclavicular brachial plexus block (BPB) for analgesia during endovascular treatment of dysfunctional hemodialysis fistulas. MATERIALS AND METHODS: US-guided supraclavicular BPB was performed before endovascular treatment of dysfunctional hemodialysis fistulas in 40 consecutive patients. After BPB, standard interventional procedures were performed for treatment of dysfunctional hemodialysis fistulas. A visual analog scale (0-10) was used to assess pain related to performance of BPB immediately after the endovascular procedure. Patient satisfaction and operator satisfaction during the procedure were also assessed after the procedure. RESULTS: Satisfactory regional anesthesia and analgesia were achieved in all patients without a need for supplemental intravenous analgesia. The mean onset time for complete block was 5.4 minutes ± 2.6. Pain scores were 0 (no pain) in 26 patients and 1-3 (mild, annoying pain) in 14 patients. The patient's satisfaction with pain control was recorded as satisfied (very well) in all cases. The operator's satisfaction with this anesthetic technique was also recorded as satisfied (very well) in all cases. Complications related to the block procedure did not occur in any patient. CONCLUSIONS: US-guided supraclavicular BPB can be used safely to provide analgesia during endovascular treatment of dysfunctional hemodialysis fistulas in adult patients.

Serum ischemia modified albumin levels in pregnancies with neural tube defects.

OBJECTIVE: To evaluate ischemia-modified albumin (IMA) in women who had been pregnant with a child suffering from neural tube defect. STUDY DESIGN: Samples from 50 women who had been pregnant with an affected child (25 spina bifida, 25 anencephaly) and 25 controls matched for age, gestational age, and body mass index were studied. We measured serum IMA by enzyme-linked immunosorbent assay. RESULTS: Serum IMA was significantly higher in the study group compared to normal pregnancies (p < 0.05). The area under the receiver operating curve was 0.858 for IMA (95% CI, 0.769-0.947), whereas the optimal threshold value of IMA to discriminate between affected children and controls was 0.409 (sensitivity 88%, specificity 80%). The risk for increased IMA in mothers who have conceived a fetus with neural tube defect is 24.5 times higher than in the control group (rr = 24.5, 6.9-86.9, 95% CI) (p = 0.001). CONCLUSION: This study indicates that serum IMA in women who have conceived a fetus with neural tube defect is significantly higher than that in normal pregnant women.

Comparison of Sedoanalgesia Versus Ultrasound-Guided Supraclavicular Brachial Plexus Block for the Prevention of the Pain During Endovascular Treatment of Dysfunctional Hemodialysis Fistulas.

PURPOSE: Although intravenous sedation and analgesia have been widely used as a first choice to relieve pain during treatment of dysfunctional hemodialysis fistulas by interventional radiology, the sedoanalgesic drugs have a considerable risk of respiratory depression, especially in hemodialysis patients. In this study, we compared the utility and efficiency of ultrasound-guided supraclavicular brachial plexus block versus sedoanalgesia for the prevention of pain during endovascular treatment of dysfunctional hemodialysis fistulas MATERIALS AND METHODS: Patients were randomized into two groups: ultrasound-guided supraclavicular brachial plexus block (n = 34) or sedoanalgesia group (n = 34). A visual analogue scale from no pain (= 0) to worst pain possible (= 10) was used to assess the pain intensity. Patient and operator satisfaction were graded from 0 to 2: 0, not satisfied at all; 1, partially satisfied; 2, satisfied (very well or complete satisfaction). Both groups were compared in terms of pain scores, patient and operator satisfaction as well as complications. RESULTS: The median pain score was significantly lower in the block group compared to the sedoanalgesia group, 0 (0-4) versus 6 (2-10), p = 0.0001. Patient satisfaction and operator satisfaction were significantly higher in the block group than in the sedoanalgesia group (p = 0.0001). Severe oxygen desaturation occurred in five (14.7%) patients following the administration of sedoanalgesia. No side effects or complications related to block procedure occurred in any patient. CONCLUSION: Ultrasound-guided supraclavicular brachial plexus block has advantages over the sedoanalgesia during endovascular treatment of dysfunctional hemodialysis fistulas. It can provide safe and efficient analgesia with excellent procedural satisfaction in adult hemodialysis patients. LEVEL OF EVIDENCE: Level 1 (randomized controlled trial).

[A rare anomaly presented with symptoms of acute abdomen: a third kidney located at right iliac fossa]. / Akut karin tablosu ile kendini gösteren nadir bir anomali: Sag pelvik yerlesimli üçüncü böbrek.

Supernumerary kidney is an extremely rare congenital anomaly of the urinary tract. A 39 year-old woman was admitted to the emergency unit with right lower quadrant pain. Physical examination revealed abdominal tenderness and defense on palpation of the right pelvic region. Blood and urine analysis revealed leukocytosis and urinary tract infection. Ultrasound examination demonstrated a suspicious mass which was thought to be a pseudokidney in the pelvis. Native kidneys were found in the normal anatomic position. Further investigation with computed tomography demonstrated a functioning third kidney which has located at the right iliac fossa in addition to normal excreting right and left kidneys. Acute abdomen like symptoms were secondary to the urinary infection of the third kidney and the urinary infection was successfully treated by antimicrobial and anti-inflammatory medication. We conclude that the infection of pelvic supernumerary kidney may create clinical symptoms of acute abdomen. Although extremely rare, congenital anomalies like supernumerary pelvic kidney should be included in the differential diagnosis.

Endovenous laser ablation and sclerotherapy for incompetent vein of Giacomini.

PURPOSE: To retrospectively evaluate the feasibility and effectiveness of endovenous laser ablation or ultrasound-guided foam sclerotherapy for Giacomini vein insufficiency. This is the largest cohort of patients treated for Giacomini vein insufficiency with endovenous laser ablation or ultrasound-guided foam sclerotherapy. MATERIAL AND METHODS: Over a three-year period, 23 females and nine males (age range, 19-67 years) treated for Giacomini vein insufficiency with or without saphenous vein insufficiency were retrospectively reviewed. Diagnosis of venous insufficiency was made by color Doppler ultrasonography. Symptomatic insufficiency of the Giacomini vein or the saphenous veins was treated with endovenous laser ablation. Ultrasound-guided foam sclerotherapy was used for tortuous incompetent Giacomini veins. The venous disease was categorized according to the clinical, etiological, anatomical, and pathological classification, and clinical severity was graded with the venous clinical severity score. Follow-up included clinical examination and color Doppler ultrasonography. RESULTS: Thirty-nine limbs in 32 patients were treated (25 endovenous laser ablation and seven ultrasound-guided foam sclerotherapy). All procedures were technically successful. One patient in the ultrasound-guided foam sclerotherapy group had a recurrence with successful repeated treatment. Recurrence was not seen in the endovenous laser ablation group. No complications were observed. All patients had resolution and improvement in 100% of their symptoms at 12 months of follow-up. CONCLUSION: Giacomini vein insufficiency is mostly seen with insufficiency of the great saphenous vein and can be effectively treated with endovenous laser ablation or ultrasound-guided foam sclerotherapy.

Evaluation of fetal autopsy findings in the Hatay region: 274 cases.

OBJECTIVE: The aim of this study was to present the incidence of fetal anomalies in our region of Hatay, Turkey in order to determine the efficiency of prenatal diagnosis through fetal autopsy, and to compare our statistical data with other national and international studies. MATERIAL AND METHOD: This study was conducted on 274 fetuses from terminated pregnancies due to abnormal prenatal findings and intrauterine deaths from 2005 to September 2010. Fetuses were evaluated through postmortem examination, external measurements, X-rays, Magnetic Resonance Images, Multislice Computerized Tomography and photographs. The autopsy was completed by the histological examination of each organ. RESULTS: Autopsy was conducted on 274 fetuses. A fetal anomaly was detected in 160 (58.39%) cases. The central nervous system contained the most frequent structural defects (79 cases, 49.38%), followed by malformations in the musculoskeletal system in 36 cases (22.5%). The most frequent multiple system anomalies were central nervous system defect and bilateral adrenal agenesis, musculoskeletal system malformations and urinary system defects. Fetal autopsy provided additional findings in 43 cases (26.88%). CONCLUSION: Fetal autopsy is a very important procedure and an integral part of the general prenatal management. New findings through this method may suggest invaluable data for parents about potential risks in future pregnancies.

Fetal cervical neuroblastoma: prenatal diagnosis.

Neuroblastoma is the most frequent extracranial solid tumor in childhood, but it is seldom diagnosed prenatally. It usually presented with adrenal masses. Presentation of other localization is extremely rare. We report a case of cervical neuroblastoma identified at 20 weeks of gestational age. This is the third case diagnosed antenatally on neck region in the literature. Additionally, it is the first case that extended to the brain. We also discussed the literature for cervical neuroblastoma detected prenatally.

CT imaging of superior semicircular canal dehiscence: added value of reformatted images.

CONCLUSION: Superior semicircular canal dehiscence (SSCD) syndrome may present with various symptoms. CT scans previously interpreted as normal may show SSCD, especially if special reconstructions tailored for superior canal evaluation are added. OBJECTIVES: The purpose of this study was to investigate prevalence of SSCD, its length and its correlation with symptoms in patients who had previously undergone temporal bone CT examination that was reported normal and to demonstrate the importance of reformatted images in the diagnosis of SSCD. METHODS: We retrospectively reviewed 108 patients who had undergone temporal bone CT examination for various symptoms and were reported as normal. High-resolution temporal bone CT imaging was performed with 1 mm slice thickness in the transverse plane. Each of the superior semicircular canals was evaluated in the plane of Pöschl and Stenver reformatted images together with axial images. RESULTS: Ninety-three patients were included in the study. Nineteen patients with semicircular canal dehiscence were detected. The mean age of the study group was 45 years. Radiologic evidence of SSCD occurred in 23 of 186 temporal bones with a radiologic prevalence of 12%. The most common symptoms in dehiscent patients were vertigo, hearing loss and tinnitus. Defect lengths varied between 1 mm and 6.5 mm.