Evaluation of pediatricians' awareness about anaphylaxis.

OBJECTIVE: Anaphylaxis is a severe hypersensitivity reaction with a rapid onset and is potentially life-threatening if not treated promptly. This study aimed to determine the level of knowledge of pediatricians in Turkey in recognizing and treating the clinical symptoms of anaphylaxis, compare the previous studies conducted in Turkey chronologically, and show the current trends on awareness of anaphylaxis in developed and developing countries in the world. SUBJECTS AND METHODS: Pediatric residents and specialists from all over Turkey were included in the study. A questionnaire was prepared by compiling the current literature. Questions were sent to pediatricians via online applications. Statistical tests were used to analyze the data. RESULTS: A total of 524 pediatricians participated in the study. All participants accepted that anaphylaxis was a life-threatening condition. Almost all suggested epinephrine as the primary drug used in anaphylaxis. The proportion of pediatricians who knew the appropriate dose, route of administration, and place of administration of epinephrine was 82.8%, 88.9%, and 89.7%, respectively. The rate of pediatricians who recognized the clinical features of anaphylaxis was over 90%. The proportion of pediatricians who knew the epinephrine auto-injector and dose was 74.4% and 53.1%, respectively. Pediatricians with less than 10 years of experience and those working in public hospitals had better knowledge about atypical symptoms of anaphylaxis. CONCLUSIONS: Although there are still inadequacies in identifying atypical symptoms and treating anaphylaxis, our study revealed that the level of awareness of anaphylaxis had shown an increasing trend in Turkey over time. On the other hand, the knowledge on diagnosing and treating anaphylaxis still needs to be improved, especially for physicians working in rural areas of developing countries.

Autosomal recessive multiple pterygium syndrome: a new variant?

Multiple pterygium syndromes include at least 15 different entities characterized by multiple pterygia or webs of the skin and multiple congenital anomalies. We describe a female infant who presented with a distinct constellation of multiple anomalies consisting of pterygia of the inguinal, intercrural and popliteal areas, flexion contractures and arthrogryposis of some joints, craniofacial anomalies including ectropion, medial canthal web, blepharophimosis, hypoplasia of nose, oral and nasopharyngeal cavities, vocal cords and tongue, micrognathia, orolabial synechiae secondary to pterygia, low set ears, alopecia, sad and expressionless face, short neck, asymmetric nipples, anal stenosis, rectal polyp, hypoplastic labia majora, complete syndactyly of all fingers and toes, pes equinovarus, bandlike web between feet, and absence of the nails and phalangeal-palmar creases. Radiological examination showed synostosis, absence or hypoplasia of metacarpal, metatarsal and phalangeal bones on feet and hands, and hypoplasia of pelvic bones and scapulae. This pattern of anomalies does not fit entirely any of the known multiple pterygium syndromes. Autosomal recessive inheritance is most likely due to the presence of three similarly affected siblings and normal parents.

Urinary excretion of deoxypyridinoline in 24-hour and first-void samples in healthy Turkish children.

OBJECTIVES: Collagen cross-links are formed during the maturation process of bone matrix. They have been considered as valuable markers in some metabolic, endocrinologic, and neoplastic bone disorders. As an advantage, it can be measured in urine as well as in serum samples. However, the excretion characteristics remains controversial. DESIGN AND METHODS: We investigated urinary free deoxypyridinoline (f-Dpd) excretion in first-void urine samples and in 24-hour collections in healthy Turkish children. We also evaluated the possible correlations and gender-related differences in Dpd excretion between these sampling methods. Both urine samples of 62 subjects (aged from 31 to 120 months) were analyzed by Immulite chemiluminescent technique. RESULTS: There were no remarkable differences in f-Dpd excretion between first-void and 24-hour urine samples, although f-Dpd values of the first-void samples were slightly higher (Dpd: creatinine, mean +/- SD, 20.5 +/- 5.8 nmol/mmol vs. 19.6 +/- 5.6 nmol/mmol, respectively, p > 0.05). A strong linear correlation was found between 24-hour and first-void urine f-Dpd excretions (r = 0.77, p < 0.05). In addition, f-Dpd showed no gender-related differences between boys and girls in either 24-hour or first-void urine samples (p > 0.05). CONCLUSIONS: Because of difficulties in long-time urine collection in infants and young children, f-Dpd assessment in first-void single urine samples is an easy, safe, and non-invasive method.

Protective role of alpha-tocopherol and caffeic acid phenethyl ester on ischemia-reperfusion injury via nitric oxide and myeloperoxidase in rat kidneys.

BACKGROUND: The aim of this study was to determine the acute effects of antioxidant caffeic acid phenethyl ester (CAPE) and alpha-tocopherol (vitamin E) on nitric oxide (NO) production, neutrophil infiltration, and antioxidant enzyme activities on an in vivo model of renal ischemia-reperfusion injury. METHODS: Rats were divided into five equal groups each consisting six rats: sham operation, ischemia, ischemia-reperfusion (I/R), I/R plus CAPE, and I/R plus vitamin E groups. CAPE or vitamin E was administered intraperitoneally before reperfusion. After experimental procedure, rats were sacrificed and both ipsilateral and contralateral kidneys were removed and prepared for NO concentrations, myeloperoxidase (MPO), catalase (CAT) and superoxide dismutase (SOD) activities. RESULTS: Acute administration of vitamin E decreased NO concentrations in both ipsilateral and contralateral renal tissues compared to I/R group. SOD activity was increased in I/R and I/R + CAPE groups compared to sham operation group. The most prominent results were encountered in MPO activities, which did not change in contralateral kidneys in both ischemia and I/R groups. There was a significant decrease in ipsilateral MPO activity in ischemia group and a significant increase in I/R group compared to sham operation group. Pretreatment with intraperitoneal CAPE significantly diminished the tissue MPO activity indicating the prevention of the neutrophil sequestration into the kidney. CONCLUSION: There is a role for CAPE in attenuation in renal damage after I/R injury of the kidney, in part at least by inhibition of neutrophil sequestration.

Morgagni hernia: an unexpected cause of respiratory complaints and a chest mass.

Morgagni hernia (MH) is the least common type of congenital diaphragmatic hernias. Although its course is often asymptomatic, it may be associated with various respiratory and gastrointestinal symptoms. We describe 7 children with MH during a 5-year period in three pediatric centers in Turkey. All children had acute or chronic respiratory symptoms; cough was the most frequent. The diagnosis was made by posterior-anterior (PA) and lateral chest X-rays. The PA chest X-rays showed a homogenous mass in 2 and a gas-filled cystic image in 3 children in the right cardiophrenic angle. A retrocardiac homogeneous density in one child, and bilateral consolidation in lower lung areas in another child were also seen. All lateral chest X-rays showed gas-filled bowel loops above the diaphragm. The diagnosis was confirmed by barium-contrast radiograph. Four patients had five additional anomalies, i.e., ventricular septal defect, right inguinal hernia, congenital hip dislocation, pectus carinatum, and obstruction of the uretero-pelvic junction. All of the hernias were repaired by an abdominal approach. There were no complications or recurrences during follow-up. In conclusion, MH should be considered in the differential diagnosis of cases of long-standing respiratory symptoms and/or when an unexplained radiological image, especially on the right cardiophrenic area, is present.

Intranasal midazolam for prolonged convulsive seizures.

In order to determine the efficiency of intranasal midazolam in prolonged convulsive episodes, we conducted a prospective study in children with various types of seizures. Nine patients (six boys, three girls; age range 6 months to 9 years) with prolonged convulsions lasting more than 10 min were treated with intranasal midazolam, 0.3 mg/kg. The success rate was 100% with only one case requiring a second dose. Estimated duration of seizures was 12-30 min (mean 18.6) while mean time elapsed until cessation of seizures was 139.6 s (range 60-480). No significant adverse effects were noted except for one patient who had seizures secondary to serious CNS infection and respiratory depression after intranasal midazolam.

Skin suction blister wound exposed to u.v. irradiation: a burn wound model for use in humans.

The effects of exposing blister wounds to u.v. irradiation were assessed in 14 male volunteers, on whose forearms blister wounds (diameter 5 mm, suction set at 200 mmHg below atmospheric for 2 h 15 min, blister roof cut at base), and irradiated blister wounds (as above, in addition u.v. irradiation given selectively for 30 min from a distance of 10 cm), were produced. In non-u.v.-irradiated wounds, flow cessation, assessed by video microscopy (n = 6), was observed in a small proportion of the papillary loop vessels. The oedema adjacent to the wound was poorly developed. Laser Doppler linear scans (n = 8) demonstrated a pronounced hyperaemia in the wound bed and also in the adjacent skin, the reaction subsiding over a few days. The exudation rate, determined by weighing the hydrocolloid dressings applied to the wound, was maximal on day 1 and then rapidly decreased. Epithelialization, assessed evaporimetrically as the time taken for reinstatement of the epidermal water barrier, was complete in 5.1 days. In the u.v.-irradiated wounds the blood flow had ceased in all the papillary loop vessels by day 1, and increased oedema, exudation and hyperaemia at the wound edges were observed. Epithelialization was not significantly retarded by the irradiation injury. After 6 months, slight discolouration of the skin was occasionally observed, but no cosmetically disturbing scars. This benign and standardized wound model in humans--based on a combination of a mechanical suction injury and a superficial radiation burn--may prove to be useful, for instance when studying the effects of burns treatment.

Double layered autogenous vein graft patch reconstruction of the common carotid-internal jugular fistula caused by gunshot wound.

Hereby we present a case with a common carotid-internal jugular fistula caused by gunshot wound. The patient was a 32-year old male who had an entrance hole of a bullet on his right anterior cervical area, at the C4 level with a hematoma surrounding it. The exit hole could be detected at the sublingual area. By palpation a thrill and on auscultation a souffle was noted. Neither crepitation, nor any neurologic deficit or any symptom of Horner's syndrome was present. The emergency digital subtraction angiography (DSA) showed a fistulisation to internal jugular vein (IJV) approximately 0.5 cm below the common carotid artery (CCA) bifurcation level. During the operation a hematoma and a false aneurysm was observed on the CCA. Also, proximally to the bifurcation, a communication of CCA with IJV was noted. The wall of the JJV was rather thinned and the size of the vessel had considerably enlarged. Following the evacuation of the hematoma and debridement, the integration of the artery was achieved by placing a double layered autogenous vein graft patch over the 0.5 x 1.5 cm defect. The 0.3 x 1.5 cm defect laterally over the IJV was primarily sutured. The patient was discharged on the fifth day. The control DSA taken on the twelfth day showed a perfect integration of the vessels. We considered the case noticeable due to its rather rare incidence and the double layered autogen vein patch graft reconstruction.

Roberts SC phocomelia with isolated cleft palate, thrombocytopenia, and eosinophilia.

The Roberts-SC (Pseudothalidomide) syndrome is a rare autosomal recessive disorder. We present a Roberts-SC Syndrome in a 20-day-old girl with phocomelia of the upper limbs, isolated cleft palate, micrognathia, prominent eyes, pectus excavatum, and pes equinovarus. Peripheral blood smear revealed thrombocytopenia and hypereosinophilia. Premature centromere separation in the child and also in her normal mother was noted.

Leukoencephalopathy with a mild clinical course: a case report.

Infantile-onset leukoencephalopathy of van der Knaap type is manifested by initially normal or near normal neurological findings despite infantile-onset megalencephaly and magnetic resonance imaging evidence of severe white matter affection. Until this entity was recently described, these cases were usually presented under the heading of atypical variants of Alexander disease. To date 63 individuals have been reported in English literature. We report a four-year-old boy presented in the first months of life with progressive megalencephaly, delay in walking, clumsiness, convulsions and magnetic resonance imaging evidence of diffuse swelling of white matter, cystic cavitations in frontal, temporal and parietal lobes.

Differences in the cellular and humoral immune system between middle-aged men with different intensity and duration of physically training.

AIM: The effects of acute exercise on immune system and serum magnesium and iron have been investigated in recent years. However, data related to the comparisons of long-term physical training with different intensity and duration are limited. METHODS: The association between long-term physical training and cellular (lymphocyte phenotyping) and humoral immune parameters (serum immunoglobulins) and serum magnesium and iron values in the middle-aged men was investigated. Eleven male master athletes (MA) performing high intensity and long duration training, 11 male recreational athletes (RA) performing moderate intensity and duration training (>10 years) participated. Eleven male sedentary individuals were enrolled as control group (CG). RESULTS: The percentages of total CD3+ T cells, CD4+ T helper, CD8+ T suppressor/cytotoxic, CD19+ B cells, natural killer cells, HLA-DR+ active T cells and CD4/CD8 ratios did not show any significant difference among 3 groups. In MA, VO2max values showed a significant negative correlation with CD4+ T helper cells. There were no significant differences among MA, RA and CG in terms of IgG, IgA, and IgM concentrations. There was a significant correlation between VO2max and IgG in RA. Iron, iron binding capacity and ferritin were found similar in all groups, but serum magnesium level in MA was significantly lower than RA and CG. CONCLUSION: No exact data to support immunosuppression or immunostimulation could be obtained except a significant negative correlation between CD4+ T helper cells and VO2max values in MA and a positive correlation between serum IgG and VO2max ivalues in RA. These findings may be the indirect markers of cellular immune system suppression by intensive exercises and stimulation of IgG production by moderate exercises.

Ventricular diastolic filling indices in pulmonary stenosis.

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

Hemimandibular reconstruction with bipedicled scapular osteocutaneous free flap.

A case report is presented on the successful use of a bipedicled scapular osteocutaneous free flap in a young woman, whose hemimandible was resected because of sarcoma, 1 year prior to presentation. The postoperative course was uneventful and donor site morbidity minimal.

Vertical trapezius myocutaneous flap for covering wide scalp defects.

The vertical trapezius myocutaneous flap is extremely reliable, with its wide arc of rotation and its considerable soft tissue bulk, when resurfacing major defects following radical tumor surgery in the head and neck region. In this paper, the use of the vertical trapezius myocutaneous flap in the reconstruction of major scalp defects will be presented and its advantages discussed.

A rare case of Kaposi's sarcoma; hand localization.

A case is reported of Kaposi's sarcoma in a very rare location, the palmar surface of the hand. Kaposi's sarcoma, which still has an unresolved etiology has recently gained great interest because of its close association with AIDS. In our case there had been recurrences even after two surgical interventions. The result of the histopathologic examination was reported as "fibrous histiocytoma" but was also suspected to be a "dermatofibroma". We considered malignancy and performed a wide and deep total excision of the lesion. The histopathologic findings showed Kaposi's sarcoma. Discussion persists in the literature whether to apply radiotherapy or surgery for the treatment of the solitary Kaposi's sarcoma lesion. We have thus far obtained a cure after excision.

Prefabricated microvascular shoulder free flap for forehead reconstruction.

In a case with a large forehead defect following tight scalp bandaging in childhood, a prefabricated microvascular shoulder free flap was used for reconstruction. A free forearm fascial flap, with the radial artery and the concomitant vein as pedicle, was harvested and inserted under a subcutaneous pocket opened in the left shoulder region which served as the future "prefabricated free flap". In addition, a tissue expander was placed in this pocket to provide the necessary tissue expansion to enable primary donor site closure.

Congenital epulis. Case report.

A newborn baby boy weighing 3100 g was found to have a pinky red solid mass 3 x 2.5 x 2 cm arising from the anterior ridge of the maxilla. This caused difficulty in feeding, and looked ugly. The mass was excised under local anaesthetic when he was 2 days old and histological examination showed congenital epulis. He also had a stage II congenital goitre. His hypothyroidism was treated with L-thyroxine sodium and he was well with no sign of recurrence of the tumour at the age of 1 year.

Cholesterol crystal embolism. Case report.

A 60-year-old man presented with a cholesterol crystal embolism that gave a rather confusing clinical picture. An immediate multidisciplinary approach established the diagnosis and the rapid, dramatic skin loss that exposed vital structures over his thigh and lower leg was reconstructed by an ipsilateral inferior pedicled rectus abdominis musculocutaneous flap with a lateral oblique cephalad fasciocutaneal component. The case, which a plastic surgeon would rarely encounter, is interesting because of the diagnostic approach and the management.

Comparison of blood brain barrier permeability in normal and ovariectomized female rats that demonstrate right or left paw preference.

We explored the relations among paw preference, cerebral asymmetry and asymmetrical disruption of blood-brain barrier (BBB) permeability in normal and ovariectomized female rats with known paw preference. A high dose of pentylenetetrazol was used to disrupt the BBB and induce acute hypertension. To determine the areas of macroscopic infarct, samples were stained with 2,3,5-triphenyltetrazolium chloride. Histological staining techniques were used to show the areas of infarct microscopically on paraffin sections. Sixty-two percent of the rats demonstrated right paw preference, 24% demonstrated left paw preference and 14% were ambidextrous. Areas of infarct, which indicated destruction of the BBB, were determined microscopically and macroscopically in rats that demonstrated right and left paw preference. We found a relation between permeability of the BBB and paw preference. There may be a relation between paw preference, cerebral asymmetry and asymmetrical destruction of the BBB in rats. Asymmetrical destruction of the BBB in experimental rats was similar to the control group, which had asymmetrically disrupted BBB with respect to paw preference. Like the control rats, asymmetrical areas of infarct consistent with cerebral asymmetry were observed in ovariectomized rats.