Band 5, Heft 2

Wilson’s disease is an autosomal-recessive disorder caused by mutation in the ATP7B gene. Absent or reduced function of ATP7B protein leads to decreased hepatocellular excretion of copper into bile. Subsequent copper accumulation, first in the liver but ultimately in the brain and other tissues, produces different clinical manifestations such as hepatic, neurological, hematological, ophthalmological, and psychiatric problems. Diagnosis is based on clinical suspicion, parameters of copper metabolism, ophthalmic examination (Kayser-Fleischer rings) and a liver biopsy. Genetic studies are of limited use. Early diagnosis and initiation of therapy with chelators and therapeutic plasma exchange therapy are essential for prognosis. Liver transplantation corrects the underlying pathophysiology and can be lifesaving in fulminant hepatic failure. Screening of siblings and 1st degree relatives of the patients is also important.

One of the most important adverse drug reactions that physicians encounter is the life-threatening prothrombotic syndrome known as heparin-induced thrombocytopenia (HIT). In patients with a history of heparin-induced thrombocytopenia and coronary arterial disease, alternative anticoagulatory regimens are needed during cardiac surgery for prevention of thrombosis. Treatment options for such patients now generally include the use of alternative anticoagulants such as lepirudin, bivalirudin, argatroban or danaparoid. In this article, we present a case where heparin-induced thrombocytopenia was properly performed coronary arterial bypass grafting by using lepirudin. (This sentence is confusing)

A 78-year-old woman with a history of recent myocardial infarction was admitted to the coronary care unit because of dyspnea. The baseline ECG revealed sinus rhythm of 90 beats/min. Two hours after her admission, her body temperature raised to 38.8 degrees Celcius accompanied by shaking chills. Wide complex tachycardia runs consistent with polymorphic ventricular tachycardia synchronous with shaking chills were noticed on the monitor. Closer observation of the ECG revealed the presence of normal QRS complexes at the cycle length of baseline rhythm. It was presumed that artifact due to shaking chills was responsible for the ECG abnormalities.

Isolated iliac artery aneuryms are very rare vascular malformations. They can remain unnoticed for long periods of time because of their deep location in the pelvic region. Most of the patients present to the clinic with rupture of the aneurysm, and thus the condition has a very high mortality rate. We report here the case of an 84-year-old man with giant iliac artery aneuryms who was treated successfully by aneurysmectomy and aortoiliac bypass.

When thermal injuries of the hand are managed inappropriately, complex hand contractures are inevitable. Patients with hand contractures may have many difficulties in their daily life because of this deformity. These patients are challenging to reconstructive surgeons. In this study we evaluated reverse-flow dorsal metacarpal artery flaps for the defects occurred after metacarpophalangeal joint contracture release of different fingers. Nineteen patients with defects on the volar surface of metacarpophalangeal joint were reconstructed by the reverse-flow dorsal metacarpal artery flaps. All operations were successful and flap losses were not seen. The DMCA flaps provide one stage coverage and permit primary closure of the recipient site. The aesthetic and functional results are satisfactory without sacrificing main arteries. The only drawback is the residual scar on the dorsum of the hand, which can be treated by conservative means and may improve over time.

The purpose of this study was to evaluate the effects of systemic and intra-arterial application of heparin by measuring tissue levels of inflammatory cytokines. Twenty-one adult male Wistar albino rats were divided into three groups (Group A, B and C). All the rats had undergone ligation of the right femoral artery with 4-0 silk suture to induce limb ischemia. Group A was the control group. In Group B, unfractionated heparin of 1500 U/kg/day was given through the tail vein for 10 days, the same dose was given to distal part of ligated right femoral artery for 10 days in Group C. On the 3rd, 5th, and 10th days, biopsies were taken from rectus femoris muscle on the ischemic extremities. Tumor necrosis factor-α, interleukin-1β, and vascular cell adhesion molecule levels in muscle tissue were measured by a standard enzyme-linked immunoabsorbent assay method. An increase in tumor necrosis factor-α level was found in all three groups throughout the duration of the experiment. The increase in Group C was statistically significant as compared with the other groups. The significant increases that occurred in tumor necrosis factor-α level as a result of intra-arterial application of heparin can be postulated to be one of the results of angiogenesis induced by the heparin in ischemic extremities. This might delay the formation of a necrosis in ischemic extremities, depending on the increased angiogenesis response by means of intra-arterial heparin application and may result in extended vitality of an extremity.

A 13-year-old boy with atrial septal defect and tricuspid valve abnormality was reported. He had crepitan ralles and signs of heart failure. He was treated with digital, diuretic and antimicrobial therapies. After clinical improvement he underwent surgery. The atrial septal defect was closed, and ringplasty was applied to the tricuspid valve. After the operation, he could not be extubated because of respiratory failure. On the seventh day following the surgery, he developed pneumothorax and hyportension and died. Postmortem examination showed bilateral diffuse pulmonary tuberculosis. The aim of this report is to emphasise the association of tuberculosis and congenital heart disease.

We report a case of Type I dual left anterior descending coronary artery (LAD) giving rise to a pulmonary artery fistula via a short LAD branch, causing symptoms of typical angina. This rare coronary artery anomaly is importance to classify; it shows features of both intrinsic coronary artery anatomy anomaly and coronary drainage anomaly.

Recent advancements in curative-intent therapies have led to dramatic improvements in breast cancer-specific mortality but at the direct expense of increased risk of cardiovascular-related mortality. The use of radiation therapy has led to significant improvements in survival for patients treated for breast cancer. However, as patients live longer, the potentially serious adverse effects of radiation on the heart have raised concerns. Coronary artery disease following irradiation is encountered rarely but is one of the most devastating treatable complications.In this article we review the cardiac complications associated with radiation therapy.

The reported incidence of vaginal agenesis varies from 1 in 4,000 to 1 in 80,000 and individuals with congenital absence of vagina usually are first seen by a gynecologist at ages 14 to 15 years, when the absence of menses causes concern. A colon-introitus anastomosis was performed with interrupted absorbable sutures after opening peritoneum on müllerien dimple by sharp dissection using a guide inserted through the created perineo-abdominal tunnel. A rare case of vaginal agenesis with a septate uterus and normal tubes and ovaries is reported. Sigmoid vaginoplasty seemed the most appropriate procedure for the case and the continuity of the genital tract was well established as the patient menstruated vaginally after the operation.

Reconstruction of the large defects that develop after pelvic exenteration with local flaps may result in higher morbidity because of poor perineal wound healing after pelvic radiation. A well vascularised reconstructive flap originating from distant non-irradiated areas is needed. We report two cases of pelvic exenteration and rectus abdominis myocutaneous flap procedure in patients with recurrent vulvar malignancies that had undergone external beam pelvic radiation and subsequently developed pelvic fibrosis, necrosis and fistulas. Both flaps were totally viable postoperatively; the abdominal wound healed without any complication, no perineal wound complications developed with a follow-up of nine months. In conclusions, rectus abdominis myocutaneous flap reconstruction seems to be an ideal option for the large defects resulting from exenteration operations in patients with previous perineal radiation.

Combined antifungal therapy has been suggested to enhance the efficacy and reduce the toxicity of antifungal agents. The aim of the study was to investigate the in vitro synergistic activity of caspofungin, voriconazole, and fluconazole with amphotericin B against ten isolates of Candida parapsilosis and Candida albicans strains which were resistant to azoles or amphotericin B. Three different antifungal combinations (amphotericin B [AP] — caspofungin [CS], amphotericin B — fluconazole [FL], and AP — voriconazole [VO]) were evaluated for in vitro synergistic effect by the microdilution checkerboard and E-test methods. For the majority of strains, the combination test showed indifferent activity. Via the E-test method, synergistic activity was seen in 3 strains in response to AP-CS combination treatment and in one strain after administration of AP-FL; however, no synergy was observed in response to combination treatment with P-VO. Antagonistic activity was the result in 1 strain treated with AP-CS as well as in 6 strains treated with AP-FL and AP-VO combinations. Via the microdilution test, no synergistic activity was seen after treatment with all 3 combinations. Antagonistic activity was the result in 2 strains with AP-CS, in 6 strains with AP-VO and in 5 strains with AP-FL combinations. Agreement between the checkerboard and E-test methods was observed to be approximately 72%. These combinations may be used in the case of antifungal resistance.

The aim of this study is to investigate the in vitro antimicrobial activity of Ankaferd Blood Stopper® against methicillin-resistant Staphylococcus aureus (MRSA), Enterococcus species, Escherichia coli, Pseudomonas species, Acinetobacter species and Klebsiella species of nosocomial origin. Ankaferd inhibited growth in 72.4% to 100% of the bacteria tested, depending on the type of the isolate. As a result, it can be stated that Ankaferd inhibits the in vitro growth of nosocomial bacteria. This is a novel, important finding since severe hospital infections coexist with many hemostatic disorders, and the use of Ankaferd may increase hemostatic potential in such clinical conditions.

The aim of this study was to determine the types nosocomial infections (NIs) and the risk factors for NIs in the central intensive care unit (ICU) of Trakya University Hospital. The patients admitted to the ICU were observed prospectively by the unit-directed active surveillance method based on patient and the laboratory over a 9-month-period. The samples of urine, blood, sputum or tracheal aspirate were taken from the patients on the first and the third days of their hospitalization in ICU; the patients were cultured routinely. Other samples were taken and cultured if there was suspicion of an infection. Infections were considered as ICU-associated if they developed after 48 hours of hospitalization in the unit and 5 days after discharge from the unit if the patients had been sent to a different ward in the hospital. The rate of NIs in 135 patients assigned was found to be 68%. The most common infection sites were lower respiratory tract, urinary tract, bloodstream, catheter site and surgical wound. Hospitalization in ICU for more than 6 days and colonization was found to be the main risk factor for NIs. Prolonged mechanical ventilation and tracheostomy, as well as frequently changed nasogastric catheterization, were found to be risk factors for lower respiratory tract infections. For bloodstream infections, both prolonged insertion of and frequent change of arterial catheters, and for urinary tract infections, female gender, period and repeating of urinary catheterization were risk factors. A high prevalence rate of nosocomial infections was found in this study. Invasive device use and duration of use continue to greatly influence the development of nosocomial infection in ICU. Important factors to prevent nosocomial infections are to avoid long hospitalization and unnecessary device application. Control and prevention strategies based on continuing education of healthcare workers will decrease the nosocomial infections in the intensive care unit.

The aim of this study was to investigate the effect of sevoflurane and desflurane on cardiac arrhythmias in adult patients who will be anesthetized. A total of 40 patients who were in the ASA I–II group and who were to undergo elective surgery were included in the study. No pre-medication was administered to the patients. They were monitored with the Holter equipment at preoperative 24 hours, and also at postoperative 24 hours on condition that Holter monitoring will begin before induction. Following routine monitoring, the patients were randomly divided into two equal groups; group one was given sevoflurane and group two was given desflurane. In this study, desflurane and sevoflurane resulted in both a significant prolongation of QT and QTc and a significant increase in QT and QTc dispersion when compared with the basal values. In the sevoflurane group, there was no significant increase in postoperative ventricular arrhythmia, compared to that during the preoperative period. However, this increase was significant in the desflurane group (p = 0.009). Sevoflurane and desflurane result in an increase in QT dispersion. Postoperative rhythmic control should carefully be monitored, especially in the desflurane group.

Lead exposure is a common public health problem. Exposure to the metal can cause hematological, gastrointestinal, rheumatological, endocrine, neurological and renal problems in humans. However, effects on the thyroid gland are controversial. We retrospectively investigated thyroid function parameters in 65 adult males who had been occupationally exposed to lead. We then compared the findings with those of 60 male patients who had no history of lead exposure or thyroid abnormalities, who served as the control group. The mean ages of the lead-exposed workers and the controls were 34.3 ± 7.9 and 32.9 ± 6.6 years respectively. Blood lead levels in the lead-exposed workers were significantly higher than in the control group. The lead-exposed workers were assigned to one of three groups according to their blood lead levels, as follows: 40–59 μg/dl, 60–79 μg/dl, or 80 μg/dl and above. Thyroid Stimulating Hormone (TSH) levels in the 80 μg/dl and above group were significantly higher than in either the 40–59 μg/dl group or the 60–79 μg/dl group. However, TSH levels in the 40–59 μg/dl group did not differ significantly from those in the 60–79 μg/dl group. These results suggest that high levels of lead in the blood may affect thyroid physiology. Clinicians should be aware of the potential hazardous effects of lead on the thyroid, especially in patients who have been occupationally exposed to lead.

The aim of the present experimental study was to determine and to compare the effects of vitamin E and of selenium on the blood flow to a wound region in rats, using the xenon-133 (133Xe) clearance technique. Burn wounds were made on the right thighs of rats followed by oral administration of vitamin E or selenium for a period of 10 days. The effect of vitamin E and of selenium individually on blood flow in the wound region was assessed before and after oral administration using the 133Xe clearance technique. Both vitamin E and selenium significantly increased the blood flow in the wound region (P<0.05). Our results in rats suggest that both vitamin E and selenium have beneficial effects on the skin wound healing process by increasing the skin blood flow.

Metoclopramide is widely used as an antiemetic and a prokinetic agent. Both the antiemetic properties and side effects of the drug are the result of dopamine receptor antagonism within the central nervous system. Therapeutic doses of metoclopramide can produce adverse effects. A 5-month-old girl was referred to our emergency department with the pre-diagnosis of afebrile convulsion. In her medical history, she was mistakenly given 2 mg/kg metoclopramide within a 24 h period, after which she became hypertonic and exhibited intermittent opisthotonos. Complete blood count, electrolytes, liver and renal function tests, blood gas analysis, and urinalysis were all within normal limits. Electroencephalogram, brain CT and cerebrospinal fluid examination were normal. Metoclopramide treatment was discontinued and she was treated with biperiden, which led to an improvement in symptoms after 15 minutes and complete remission in 60 minutes. Intermittent opisthotonos may be confused with convulsion in infant and thus lead to an unnecessary hospital admission. Physicians should be aware that metoclopramide is widely used in the pediatric population and children are susceptible to the side effects of metoclopramide and the side effects may present as “intermittent opisthotonos” as observed in our patient.

This study has goals of examining whether pre-eclampsia may lead to an increase of elastic tissue fibers in blood vessel walls of placental stem villi or whether there are differences in the thickness of blood vessel walls within these villi when compared to normotensive pregnant women. Non-infarcted placental tissue samples from 28 participants with uncomplicated pregnancies and 26 patients with pre-eclampsia were obtained. After routine histological procedures, the sections were processed either for conventional Verhoeff staining for the demonstration of elastic fiber system. Paraffine sections from placenta biopsies prepared for light microscopic examination were gathered. In uncomplicated pregnancies, terminal villi blood vessels were observed with no stained elastic tissue fibers in most areas. In the pre-eclampsia pregnancy of human placenta, the elastic fibers significiantly increased in terminal villi blood vessel walls which were dark in color, using Verhoeff’s tissue stain, when comparing with the uncomplicated pregnancy group. Our results indicate that an increase of elastic tissue fibers in blood vessels of placental stem villus and terminal villi, and also an increase of wall thickness during pre-eclampsia.

To provide instruction for pregnant women regarding adequate and balanced nutrition and determine whether iron and folic acid supplementation is essential. The research was an experimental clinical intervention. The study was conducted between March 2004 and May 2005 with 80 pregnant volunteers. The study participants were in their 16th to 24th weeks of pregnancy; all participants were healthy, carried only one fetus, and successfully completed their pregnancy. All participants were instructed about adequate and balanced nutrition. Until the participants gave birth, 40 (Group 1) consumed an iron-rich diet that was equivalent to the inclusion of a supplement containing 100 mg Ferro III plus 0,35 mg folic acid; the other group (Group 2) was also instructed in proper nutrition and was given by a gynecologist 1 tablet (100 mg) Ferro III hydroxide polymaltose complex and iron pharmaceutical with 0,35 mg folic acid (Maltofer Fol). In both groups, before and after the instruction, consumption frequency was noted, and the levels of serum ferritin, serum iron, total iron-binding capacity, folic acid, and vitamin B12 in the blood were determined at monthly intervals. Between the two groups, no statistical difference was found with regard to age, number of pregnancies, weight before pregnancy, body mass index (BMI) before pregnancy, and weight of the newborn (p>0,05). At the end of the study, the hemoglobin, hematocrit, and serum ferritin levels decreased considerably in both groups compared to the initial values (p<0.01). No statistically significant difference in serum ferritin levels could be found between the two groups (p>0,05). The comparison of Group I and Group II in terms of nutritional status (average energy and food consumption) in the pre-instruction and post-instruction periods revealed that intake of total protein, heme protein, dietary fiber, folic acid, carotene, vitamins A, B1, B2, B6, C, and B12, potassium, calcium, phosphorus, iron, and zinc was higher in Group I in the post-instruction period (p=0.000); no statistically significant change in nutritional status during pregnancy was observed in Group II. Conclusion: Medical diet programs with iron sources are examined in association with food consumption. Assessment of hematological results suggests that, during pregnancy, each patient should receive a specific dose, rather than a routine dose, of iron and folic acid.

Paraurethral or Skene’s duct cyst is a rare cause of masses located in the inter-labial genitor-urinary region and their etiology is not fully known. These congenital cysts may be diagnosed easily by inspection in the initial physical examination of the new-borne. Radiological evaluation is helpful for differential diagnosis and in determining the proximal extension of the cystic pathology. Treatment alternatives are needle aspiration and non-surgical follow-ups leading to a spontaneous regression and surgery. In this report the radiological findings of a female newborn diagnosed with paraurethral cyst in US and MR examinations have been discussed.

The purpose of this study was to compare the quantitative parameters of salivary gland functions in patients with allergic rhinitis and healthy volunteers using [99mTc] pertechnetate salivary gland scintigraphy. Thirty-six patients with allergic rhinitis (14 males and 22 females, mean age 30.6 ± 5.2 years) and thirty-nine healthy controls (18 males and 21 females, mean age 32.2 ± 5.6 years) were evaluated. Dynamic salivary gland scintigraphy was performed after intravenous administration of 185 MBq (5 mCi) of [99mTc] pertechnetate for 25 minutes. Lemon juice was used to stimulate the salivary glands. On the basis of the time-activity curves, the following glandular function parameters were calculated for the parotid and submandibular salivary glands: uptake ratio, maximum accumulation, and ejection fraction. Statistically, all the functional parameters of the parotid and submandibular glands obtained for the allergic rhinitis patients were significantly lower than those of the healthy controls group (p < 0.05). This study demonstrated that there was a significant difference in salivary gland function between patients with allergic rhinitis and healthy controls, which can easily be evaluated by [99mTc] pertechnetate salivary gland scintigraphy.

There is no consensus regarding the time of antiepileptic drug withdrawal and the relevant risk factors for seizure relapse. In this study, we aimed to determine the seizure relapse rates and the associated risk factors for seizure relapse in childhood epilepsy. Two-hundred sixty-six epileptic patients who discontinued the antiepileptic drug therapy after a seizure-free period of at least two years, were enrolled into the study. The data of the patients regarding sex, febrile convulsion history, family history, age at onset, type of epilepsy, total number of seizures and antiepileptic drugs, seizures during treatment, mental status, first and last electroencephalography, brain imaging findings, etiological factors and seizure relapse in the first two years after antiepileptic drug withdrawal were obtained from the patients’ files. Univariate logistic regression analysis was performed for each variable. The variables which were found to be statistically significant in univariate analysis, were included in multivariate logistic regression analysis. The overall seizure relapse rate after antiepileptic drug withdrawal was 19.2%. There were no significant differences for seizure relapse rate after antiepileptic drug withdrawal between patient groups with respect to sex, family history, type of epilepsy, febrile convulsion history, seizures before treatment, first electroencephalography findings, brain imaging findings and etiology. However, there were statistically significant differences for seizure relapse rate among patient groups concerning age at onset of epilepsy, new seizure during treatment, the total number of antiepileptic drugs, mental status, and last electroencephalography findings. We imply that the clinical status of the patients should be considered before the cessation of drug therapy rather than the etiological factors or laboratory findings.

Severe and composite defects of the nose due to trauma or excision of neoplasms can cause significant functional and aesthetic problems. It has been known that nasal stents have been used to maintain the nasal patency and nostril shape for a long time. Recently, nasal prosthesis was introduced as a method to solve aesthetic problems of the patients who had an amputated nose. In the present study, nasal prosthesis combined with nazal stents was used to camouflage the nasal disfigurement and to maintain the nasal patency in two adult female patients. Functional and aesthetic results was accepted as satisfactory by both the patients and the physicians.

Osteoprotegerin (OPG)/“receptor activator of nuclear factor kappa B-ligand” (RANKL) system has an important role in the remodeling of bone through regulation of osteoclastogenesis. We aimed to detect OPG and RANKL levels, particularly in obese children in the pubertal period and to investigate whether these parameters correlate with insulin resistance in childhood. Our study included 66 obese children ranging in age from 9.1 to 16 years, and 22 non-obese children ranging in age from 10.5 to 16 years. Blood glucose, insulin, total cholesterol, HDL cholesterol, and LDL cholesterol levels were measured for all cases; HOMA-IR, Quicki index and atherogenic index were calculated. Serum OPG and RANKL levels were also measured. OPG and RANKL levels did not show any difference between obese and non-obese children (P>.05). No difference in these 2 parameters were observed among the children with and without insulin resistance (P>.05). No correlation could be established between the OPG, the HOMA-IR, Quick and atherogenic indices. Obesity and insulin resistance are believed to show their effect in the later period of life to become able to change some of the parameters.