Ilknur Erol

Epidemiologic, Clinical, and Imaging Findings in Brucellosis Patients with Osteoarticular Involvement

OBJECTIVE The aim of this study was to assess the epidemiologic and clinical features, complications, imaging findings, and outcomes for brucellosis patients with osteoarticular involvement. SUBJECTS AND METHODS This prospective study was performed over 4 years (December 2000-December 2004). The subjects were 251 Turkish patients (age range, 2-77 years) who were diagnosed with brucellosis during that period. Joint sonography, radiography, radionuclide bone scintigraphy, and MRI were performed in all patients with osteoarticular and spinal manifestations. RESULTS The disease was acute in 92 patients (36.7%), subacute in 48 patients (19.1%), and chronic in 111 patients (44.2%). Sonography of the joints showed bursitis in 13 patients (5.2%). Radiography, MRI, and scintigraphy revealed 71 patients (28.3%) with sacroiliitis, 26 (10.4%) with spondylodiskitis, three (1.2%) with acute osteomyelitis, and one (0.4%) with avascular necrosis of the femoral head. All patients received combinations of either two or three antibiotics. Surgery was performed in three patients with spinal instability or radiculopathy. CONCLUSION Brucellosis is endemic to some regions. MRI is the method of choice for diagnosing osteoarticular and spinal complications of human brucellosis, especially during the early phase. It is important to differentiate tuberculous spondylodiskitis from brucellar spondylodiskitis because proper treatment for each of these diseases can prevent complications. The radiologic findings for these two forms of spondylodiskitis are similar, so serologic testing for brucellosis is necessary in such cases.

Neurological complications of liver transplantation in pediatric patients: A single center experience

Abstract:  Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 ± 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilsons disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilsons disease had a higher incidence of NCs (60%) than did patients without Wilsons disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.

West syndrome in an infant with vitamin B12 deficiency in the absence of macrocytic anaemia

Vitamin B12 deficiency in infants often produces haematological and neurological deficits, including macrocytic anaemia, neurodevelopmental delay or regression, irritability, weakness, hypotonia, ataxia, apathy, tremor, and seizures. The diagnosis of vitamin B12 deficiency can be difficult when the typical macrocytic anaemia is absent. We report the case of a 10‐month‐old female diagnosed with West syndrome associated with vitamin B12 deficiency but without macrocytic anaemia caused by nutritional inadequacy in the mother. The patients motor skills and cognitive development were normal until she was 9 months old, when she began to exhibit a series of sudden flexions of the head, trunk, arms, and legs. She was exclusively breast‐fed and had received no vitamin supplementation. Results of electroencephalography (EEG) indicated modified hypsarrhythmia and the patient was diagnosed as having West syndrome. Synthetic adrenocorticotropic hormone was administered and although her spasms had resolved, the patient remained apathic and could not sit without assistance. EEG results indicated generalized slow activity. After she was diagnosed as having vitamin B12 deficiency, parenteral treatment with vitamin B12 was initiated. Her symptoms resolved and EEG was completely normal. When she was 20 months old she exhibited an age‐appropriate developmental and neurological profile. To our knowledge, this is the first report of West syndrome as a presenting symptom of vitamin B12 deficiency.

Elevated CK-MB mass and plasma brain-type natriuretic peptide concentrations following convulsive seizures in children and adolescents: Possible evidence of subtle cardiac dysfunction

Purpose:  To evaluate the presence of myocardial injury during convulsive seizures in children and adolescents by determining serum concentrations of cardiac troponin I (cTnI), creatine kinase‐MB mass (CK‐MB mass), and plasma brain‐type natriuretic peptide (BNP).

Clinical Pattern and Abdominal Sonographic Findings in 251 Cases of Brucellosis in Southern Turkey

OBJECTIVE The aim of this study was to report the clinical features, complications, abdominal sonographic findings, and treatment outcomes for a series of brucellosis cases in southern Turkey. SUBJECTS AND METHODS This prospective study involved 251 patients (age range, 2-77 years) who were hospitalized with brucellosis during a 4-year period. Patients were classified as having acute (< 3 months), subacute (3-12 months), or chronic (> 12 months) disease. Physical, laboratory, and abdominal sonographic findings were analyzed. RESULTS The disease was acute in 92 cases (36.7%), subacute in 48 (19.1%), and chronic in 111 (44.2%). Sonographic examination of the abdomen showed enlarged periportal lymph nodes in 23 patients (9.2%), splenomegaly in 21 (8.4%), hepatomegaly in 15 (6%), pleural effusion in 7 (2.8%), splenic abscesses in 4 (1.6%), splenic cysts in 2 (0.8%), acute appendicitis in 2 (0.8%), and acute acalculous cholecystitis in 1 patient (0.4%). The main hematologic and biochemical manifestations were anemia, elevated erythrocyte sedimentation rate, elevated C-reactive protein, and elevated transaminase levels. All patients were treated with combinations of either two or three antibiotics. Surgery was performed in the patients with acute appendicitis, acute cholecystitis, and multiple splenic cysts. CONCLUSION Brucellosis is endemic to Turkey. Sonographic examination is the method of choice for diagnosing abdominal complications of human brucellosis. This disease should be included in the differential diagnosis for any patient with enlarged periportal lymph nodes. The specific treatment regimen and duration of therapy should be based on sites of organ involvement and complications.

Hashimoto's encephalopathy in children and adolescents.

Hashimotos encephalopathy is an underdiagnosed, steroid-responsive, progressive or relapsing encephalopathy associated with high titers of serum antithyroid antibodies. Although Hashimotos encephalopathy is well documented in adults, it is rarely observed or studied in children and adolescents. We describe the clinical and laboratory findings of four children (aged 9-15 years) with Hashimotos encephalopathy. The clinical features of two patients at presentation included epileptic seizures and confusion. The other presenting signs included breath-holding spells, behavioral problems, psychosis, and ataxia (one patient each). During their presentation, three patients were euthyroid, and one was hyperthyroid. All patients manifested increased antithyroid antibodies, and all improved with steroid treatment. Hashimotos encephalopathy is rarely suspected at presentation. Therefore, greater awareness of its signs by clinicians is necessary for proper diagnoses.

Increased Vulnerability to Oxidative Stress in Pediatric Migraine Patients

Little is known about the role of oxidative stress in the pathogenesis of pediatric migraine. The objective of the present study was to investigate, during a headache-free period, the activities of erythrocyte antioxidant enzymes in children and adolescents diagnosed with migraine. In all, 47 migraine patients (age range, 8-17 years; mean, 14.1 +/- 2.4 years, 14 with aura) and 35 control subjects were included. Superoxide dismutase, glutathione peroxidase, and catalase activities were measured in erythrocytes. Although superoxide dismutase activities did not differ between groups, both catalase and glutathione peroxidase activities were significantly lower in migraine patients (P = 0.001 and P = 0.009, respectively). Activities of all three antioxidant enzymes were similar across migraine subgroups; there was no correlation with age and sex. These results confirm vulnerability to oxidative stress in pediatric migraine. Further studies and search for new therapeutic agents with antioxidant properties are needed.

Vici Syndrome Associated With Sensorineural Hearing Loss and Laryngomalacia

The phenotypically heterogeneous, autosomal recessive Vici syndrome was first described in 1988 in a sister and brother with oculocutaneous albinism, agenesis of the corpus callosum, cataract, cardiomyopathy, cleft lip, and immunodeficiency. Only 14 cases of Vici syndrome have yet been reported, several involving morphologic and functional defects in addition to those described in the initial case. We report on a 3-month-old Turkish girl with Vici syndrome associated with laryngomalacia, further expanding the clinical spectrum. We also review clinical features in all 15 Vici syndrome patients, to distinguish general from less common signs. To the best of our knowledge, this report is the first of a Turkish patient with Vici syndrome.

Posterior leukoencephalopathy syndrome in children and adolescents.

Posterior leukoencephalopathy syndrome is a recently identified clinical and radiologic entity. The characteristic radiologic findings are bilateral gray and white matter edema in the posterior regions of the cerebral hemispheres. This article reports clinical and radiologic findings in 10 consecutive episodes of posterior leukoencephalopathy syndrome that were diagnosed in 9 children and adolescents. The causes were immunosuppressive therapy in 7 patients and a combination of renal failure and hypertension in 3. The most common presenting symptoms were seizure and altered consciousness; others included headache, sixth nerve palsy, and cortical blindness. Imaging demonstrated abnormalities in the parietal and occipital lobes in all 10 episodes. The signs and symptoms resolved after immunosuppressive agents were reduced or discontinued, or after uremia and hypertension were corrected. Four patients underwent follow-up cranial imaging, and the images showed nearly complete or complete resolution. The syndrome was clinically reversible in all patients.

Brain Abscess Associated with Isolated Left Superior Vena Cava Draining into the Left Atrium in the Absence of Coronary Sinus and Atrial Septal Defect

A previously healthy 12-year-old girl presented with severe headache for 2 weeks. On physical examination, there was finger clubbing without apparent cyanosis. Neurological examination revealed only papiledema without focal neurologic signs. Cerebral magnetic resonance imaging showed the characteristic features of brain abscess in the left frontal lobe. Cardiologic workup to exclude a right-to-left shunt showed an abnormality of the systemic venous drainage: presence of isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect. This anomaly is rare, because only a few other cases have been reported.