Cengiz Dilber

Sturge-Weber syndrome involved frontoparietal region without facial nevus

Classic Sturge-Weber syndrome is characterized by a facial nevus and hamartomatous lesion in the brain. Hamartomatous lesions are usually located in the ipsilateral occipital region of the facial nevus. The other lobes may be involved. A few cases of Sturge-Weber syndrome without facial nevus have been reported. A 9-month-old male was admitted with the complaint of afebrile seizures two times. We observed the third seizure, which was complex partial, in the clinic. There was no facial hemangioma. On computed tomography and magnetic resonance imaging of the brain we observed findings concordant with the angioma in the right frontoparietal region, although the occipital region was intact. This patient appears to be the first with Sturge-Weber syndrome without facial nevus, and with involvement of the frontoparietal region but not the occipital region. We do not know the embryologic basis of this association.

Henoch–Schönlein purpura associated with hepatitis A infection

common vasculitis conditions of childhood, and is characterized by nonthrombocytopenic purpura, arthritis and/or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal involvement. Although the etiology of HSP is still unknown, it is considered to be an immune-mediated vasculitic disorder resulting from an immune complex reaction to various antigenic stimuli. Many bacterial and viral organisms such as streptococci, adenovirus, parvovirus, hepatitis B virus (HBV), Epstein–Barr virus, varicella and mycoplasma have been reported as predisposing factors for HSP.1 So far, HSP associated with hepatitis A virus (HAV) infection has only been reported in one other patient, as a letter to the editor.2 In the present report we describe two patients with HSP associated with acute hepatitis A.

Pseudotumor cerebri complicating measles : A case report and literature review

A previously healthy 8-year-old girl patient was referred with the complaints of severe headache associated with nausea and vomiting. Three weeks prior to her admission, she had measles manifested with fever and typical skin eruptions. Fundoscopic examination revealed bilateral swollen optic discs with tortuous blood vessels. Other physical examinations were unremarkable, except for photophobia. Lumbar puncture demonstrated a cerebrospinal fluid opening pressure of 30 cm H(2)O, no cells, normal levels of glucose and protein. Serum measles immunoglobulin M level was elevated (183 AU). Our further investigations revealed that the patient had pseudotumor cerebri (PTC) following measles infection.

Intravenous immunoglobulin for Guillain-Barré syndrome: how effective?

Guillain-Barré syndrome is an acute inflammatory demyelinating neuropathy characterized by progressive symmetric polyradiculoneuritis, predominantly manifested by weakness and areflexia. In this article, we report our findings in 25 children treated with intravenous immunoglobulin and compare them with the remaining 30 children who received supportive care only. Only supportive care was given to 30 children who were not able to receive intravenous gammaglobulin because of shortcomings in intravenous gammaglobulin availability owing to a poor import during those years. Twenty-five patients were treated with intravenous gammaglobulin; they received intravenous gammaglobulin 0.4 g/kg/day for 5 consecutive days. Seventeen of the intravenous gammaglobulin group had received intravenous gammaglobulin within 10 days after the first symptoms, and eight of them had received intravenous gammaglobulin after the first 10 days. The average time elapsed for the symptoms to reach the maximum level was 6.9 (range 4—12) days in patients receiving intravenous gammaglobulin in the first 10 days, and it was significantly shorter than the time elapsed for the supportive care group (6.9 versus 8.8 days, respectively) (P < .05). Admission to the hospital after the first symptom, disability grade, time to improve in disability grade, the period of hospitalization, and mortality were not different in the intravenous gammaglobulin and supportive care groups (P > .05). Our suggestion for intravenous gammaglobulin treatment in Guillain-Barré syndrome is that if the patient has risk factors for respiratory insufficiency, then the treatment should be started. We more confidently carry out the follow-up of these patients after the results of this study. In conclusion, although it has been reported that intravenous gammaglobulin facilitates improvement in the disease and the decrease in mortality in children with Guillain-Barré syndrome, it has been mentioned in some studies that the intravenous gammaglobulin treatment was not better than supportive care, as in our study. However, further studies are essential to determine when intravenous gammaglobulin should be given to patients having which clinical and laboratory findings. (J Child Neurol 2006;21:972—974; DOI 10.2310/ 7010.2006.00214).

Diffuse Cystic Bronchiectasis Associated with Left Microtia and External Auditory Canal Atresia: A Very Rare Coincidence

Objective: To present a case in which diffuse cystic bronchiectasis was associated with left microtia/external auditory canal atresia. Clinical Presentation: A 10-year-old girl suffering from cough, fever, dyspnea and sputum for 6 months was transferred to our clinic due to the diagnosis of bronchopneumonia. She had recurrent episodes of bronchopneumonia. On examination, left microtia and left external auditory canal atresia were detected. Thorax CT revealed diffuse cystic bronchiectasis on the left lung. Radiological examination showed atresia of the left external auditory canal. We could not find any etiopathological reason causing bronchiectasis. Intervention: The patient underwent left pneumonectomy and postoperative histopathology was reported as bronchiectasis. Conclusion: This report shows a unique case in which an association of diffuse cystic bronchiectasis and left microtia/external auditory canal atresia was observed. Hence in newborns with microtia and/or external auditory canal atresia, the probability of development of bronchiectasis should be borne in mind and such patients should be followed up more carefully regarding this rare association.

Acute Mercury Poisoning in a Group of School Children.

Objective Elemental mercury is a toxic liquid element that is used widely in the home, medicine, agriculture, and industry. It is readily vaporized and inhaled at room temperature. Thereby, inhalation can cause acute or chronic poisoning. Mercury can be found in environmental naturally find but some dangers sources give rise to contaminations. It can be very dangerous to all living organisms, especially children. Methods This study presents the features of mercury poisoning in a group of pediatric cases. Data were obtained for 29 pediatric cases exposed to elemental mercury in a high school chemistry laboratory in Turkey. Patients with a blood mercury level exceeding 10 μg/L or a urine mercury level exceeding 15 μg/L were considered to have mercury poisoning. The patients were treated with 2,3-dimercaptopropane sulfonic acid or D-penicillamine. Results Twenty-nine children with mercury poisoning were admitted to the hospital. The median duration of exposure was 58 (range, 15–120) minutes. Ten (29%) children were asymptomatic. Physical and neurological examinations were normal in 19 (65.5%) children. The most common presenting complaint was headache. The most common neurological abnormality, partly dilated/dilated pupils, was present in 9 (31%) children. Mercury levels were measured in blood samples every 5 days, and the median blood mercury level was 51.98 (range, 24.9–86.4) μg/L. There was a positive correlation between the duration of exposure and maximum blood/urine mercury levels (P = 0.001). Conclusions Elemental mercury exposure is potentially toxic; its symptomatology varies, especially in children. Secure storage of mercury and other toxic substances and provision of information about this subject to individuals who might be exposed to mercury and their families might help to prevent mercury poisoning.

A Case of Acute Disseminated Encephalomyelitis Presenting with Decreased Strength in Lower Extremities and Meningitis

Mehmet Davutoglu, Fuat Ozkan, Tahir Dalkiran, Cengiz Dilber, Ekrem Guler, Yalcin Goksugur Kahramanmaras Sutcu Imam Universitesi Tip Fakultesi, Cocuk Yogun Bakim Unitesi, Kahramanmaras, Turkiye Kahramanmaras Sutcu Imam Universitesi Tip Fakultesi, Radyoloji Anabilim Dali, Kahramanmaras, Turkiye Kahramanmaras Sutcu Imam Universitesi Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali, Kahramanmaras, Turkiye Kahramanmaras Sutcu Imam Universitesi Tip Fakultesi, Cocuk Norolojisi Bilim Dali, Kahramanmaras, Turkiye Kahramanmaras Sutcu Imam Universitesi Tip Fakultesi, Cocuk Acil Unitesi, Kahramanmaras, Turkiye

Ophthalmic Findings of Acute Mercury Poisoning in Primary School Students

Purpose: To report ophthalmic findings in acute mercury poisoning in the primary school students. Methods: Seventy two children exposed to mercury vapor and 42 healthy controls were enrolled in the study. Full ophthalmologic examination including best corrected visual acuity, external eye examination, a slit-lamp examination, funduscopy, intraocular pressure measurements, Visual Field (VF), Visual Evoked Potential (VEP) and Color Vision (CV) tests were performed at the presentation and after six months. The parametric values of VF, Mean Deviation (MD) and Pattern Standard Deviation (PSD) were compared between groups. Results: The visual acuity less than two lines in ETDRS chart in 7(9.7%) patients, color vision impairment in 6(8.3%) patients were determined with ophthalmic examination. There were a significant difference in the color confusion index of patients, (p<0.05). The mean parametric VF values of MD and PSD were found statistically, significant difference (p<0.001, p<0,001 respectively), The latency values of VEP were 7% of 20 patients over 100 ms. There were no correlation between ophthalmic findings and mercury levels in urine and blood. Conclusion: While visual acuity minimally affected, advanced visual functions were significantly impaired in a way independent of mercury level. The goal of this paper is to draw attention to the importance of public education on potentially hazardous effects of mercury in terms of preventive community health. In particular, both primary school teachers and students should be trained concerning poisonous gases such as mercury.

Yılan ısırığı sonrası ekstraoküler kas felci

On bir yasinda erkek cocuk yilan isirmasi tanisi ile yatirildi Sol el sirtinda isirik izi sislik ve ekimoz vardi Dorduncu gunde cift gorme sikayeti ortaya cikti Sol gozde yukari duz ve yukari saga bakista kisitlilik saptandi Sol gozde inferior oblik kas felci tanisi kondu ve hasta izleme alindi Hastanin cift gormesi zamanla azalarak dorduncu ayda tamamen kayboldu ve goz hareketleri normale dondu Yilan zehirlenmelerinde nadir gorulen ve genellikle kendiliginden iyilesen gozun dis kaslarinin felclerine dikkat cekmek icin bu olgu sunuldu Turk Ped Ars 2012; 47: 134 6

Evans syndrome related to hepatitis B virus infection: a case that responded only to lamivudine therapy.

Evans syndrome is characterized by simultaneous or sequential development of autoimmune thrombocytopenia (AITP) and autoimmune hemolytic anemia (AIHA) (1). Despite therapeutic interventions, most patients with Evans syndrome have a chronic and relapsing course that is associated with significant morbidity and mortality (2). The underlying problem in Evans syndrome is not exactly clear. Although some viral infections, especially Epstein Barr virus (EBV) (3), measles, rubella, mumps, and varicella viruses (4,5) can induce autoimmune hemolytic anemia, no relationship has been detected between Evans syndrome and viral infections. The association of hepatitis B virus (HBV) infection with aplastic anemia or thrombocytopenia has been reported in adult patients (6–10). We could not find any report of Evans syndrome related to HBV infection. Here, we present a case of Evans syndrome related to HBV infection, which was improved only with lamivudine therapy.