An interesting case of childhood brucellosis with unusual features.

Brucellosis is a zoonotic infection, which is still a major public health concern worldwide. Common clinical findings are usually nonspecific involving fever, arthralgia, myalgia, weakness and malaise. Since none of the symptoms of brucellosis is pathognomonic, it may have a similar course with various multisystemic diseases. In terms of focal involvement, sacroiliitis is the most common musculoskeletal manifestation in adult patients, while it is quite rare in pediatric patients. Blood culture is the gold standard in the diagnosis of brucellosis. In the absence of culture facilities, the diagnosis traditionally relies on serologic testing with a variety of agglutination tests such as the Rose Bengal test and the serum agglutination test. However, these agglutination tests are accompanied by frequent false negative results such as seen in prozone phenomenon, which may lead to diagnostic delays. In this article we present a rarely encountered pediatric brucellosis patient who had sacroiliitis-spondylitis, which are rarely reported in children, and exhibited prozone phenomenon in agglutination tests.

Cardiac troponin-I, brain natriuretic peptide and endothelin-1 levels in a rat model of doxorubicin-induced cardiac injury.

BACKGROUND: Cardiotoxicity, during or after therapy, is the most serious side effect of doxorubicin (DXR). The risk of developing cardiac impairment increases concomitantly with an increase in the cumulative dose of DXR. AIM: The aim was to evaluate the levels of cardiac troponin-I (cTnI), brain natriuretic peptide (BNP) and endothelin-1 (ET-1) in DXR induced cardiac injury. MATERIALS AND METHODS: Thirty-nine Wistar albino rats were divided into three groups; a control group and two-study groups that received low-dose DXR (LDD) and high-dose DXR (HDD) in a weekly schedule for reaching a cumulative dose. RESULTS: Serum cTnI level was significantly increased in both LDD and HDD-treated groups. Although serum BNP was not significantly increased either LDD or HDD-treated groups, ET-1 levels was significantly increased in only HDD-treated groups. Histopathologic injury was more evident in HDD-treated group. CONCLUSIONS: Serum cTnI was increased even in LDD and parallel to it low cardiac injury induced by DXR. In the low-dose group, BNP and ET-1 levels were not elevated significant as cTnI despite cardiac injury. Thus, cTnI may be a predictive marker in of DXR-induced cardiotoxicity.

Subclinical immune reactions to viral infections may correlate with child and adolescent diagnosis of attention-deficit/hyperactivity disorder: a preliminary study from Turkey.

BACKGROUND: Attention-Deficit/Hyperactivity Disorder (ADHD) is one of the most common neuro-developmental disorders of childhood and adolescence. Studies focusing on the relationship of infectious agents and ADHD are scarce. It is also known that cerebellar injury may lead to hyperactive behavior. This study aimed to evaluate the relationship between viral agents of cerebellitis and the diagnosis of ADHD. METHODS: The study group was formed of 60 consecutive ADHD patients and 30 healthy children. IgG levels for VZV; HSV-1, CMV, Measles, Mumps, Rubella and EBV were evaluated. RESULTS: Males were significantly higher among patients with ADHD (65% vs. 40%, p=0.025). Patients with ADHD displayed significantly higher positivity for measles IgG (80% vs. 60%, p=0.044). When patients with ADHD were classified according to their pubertal status, adolescents with ADHD displayed higher positivity for mumps (100% vs. 74.4%, p=0.043). Most of the patients were diagnosed with ADHD-Combined or Hyperactive/Impulsive Subtypes (56.6%) while 43.3% were diagnosed with ADHD-predominantly inattentive type. When patients with subtypes of ADHD were compared in terms of seropositivity, it was found that patients with ADHD-Combined/ Hyperactive-Impulsive subtypes had significantly elevated reactions for Rubella (100% vs. 88.5%, p=0.044). CONCLUSION: Although limited to a single center and may be prone to sampling biases, our results may support the notion that immune reactions may be related with ADHD among children and adolescents. Further, prospective studies from multiple centers are needed to support our findings and establish causality.

¿Existe una relación entre la gastroenteritis rotavirus positivo y los grupos sanguíneos ABO? Estudio retrospectivo de cohorte / Is there a relationship between rotavirus positive gastroenteritis and ABO blood groups?: A retrospective cohort study

Introducción. El rotavirus es la causa más frecuente de diarrea grave en los niños. Son pocos los estudios clínicos sobre la relación entre la gastroenteritis por rotavirus y los grupos sanguíneos ABO. Objetivo. La meta de este estudio fue investigar la función de los grupos sanguíneos, en la incidencia de la gastroenteritis por rotavirus y la gravedad de la gastroenteritis. Métodos. Se realizó la investigación retrospectiva de las historias clínicas de los bebés que nacieron en nuestro hospital y a quienes, durante el seguimiento, se les diagnosticó gastroenteritis aguda. Resultados. En el estudio, se incluyeron 219 (36,3%) pacientes rotavirus positivos y 383 (63,6%) pacientes rotavirus negativos. El grupo sanguíneo A se detectó más frecuentemente en el grupo rotavirus positivo que en el grupo rotavirus negativo (50,6% frente a 42,2%, p= 0,047). Las tasas de hospitalización de los casos con gastroenteritis por rotavirus en los niños con el grupo sanguíneo A (30,6% frente a 8%, p < 0,001) fueron significativamente más altas. Conclusiones. Se determinó que la gastroenteritis por rotavirus fue más frecuente en los niños con el grupo sanguíneo A. Se detectó que las tasas de hospitalización de estos pacientes fueron más altas. Por lo tanto, en los niños con el grupo sanguíneo A, podría ser necesario estudiar cuidadosamente la gastroenteritis por rotavirus.

Introduction. Rotavirus is the most common cause of severe diarrhea in children. There is only a few clinical studies about the relationship between rotavirus gastroenteritis and ABO blood groups. Objective. The aim of this study was to investigate the role of blood groups, if any present, in the incidence of rotavirus gastroenteritis, and the severity of the gastroenteritis. Methods. The file records of babies born in our hospital and diagnosed with acute gastroenteritis in their follow up were investigated retrospectively. Results. The study was conducted with 219 (36.3%) rotavirus positive and 383 (63.6%) rotavirus negative patients. The A blood group was detected more commonly in the rotavirus positive group compared to the rotavirus negative group (50.6 vs. 42.2%, p= 0.047). Hospitalization rates of the cases with rotavirus gastroenteritis among children with the A blood group (30.6 vs. 8%, p=˂0.001) were significantly higher. Conclusions. Rotavirus gastroenteritis was determined to be more common in children with A blood group. The hospitalization rates of such patients were detected to be higher. Therefore, rotavirus gastroenteritis among children with blood group A should be monitored closely.

Is there a relationship between rotavirus positive gastroenteritis and ABO blood groups?: a retrospective cohort study.

INTRODUCTION: Rotavirus is the most common cause of severe diarrhea in children. There is only a few clinical studies about the relationship between rotavirus gastroenteritis and ABO blood groups. OBJECTIVE: The aim of this study was to investigate the role of blood groups, if any present, in the incidence of rotavirus gastroenteritis, and the severity of the gastroenteritis. METHODS: The file records of babies born in our hospital and diagnosed with acute gastroenteritis in their follow up were investigated retrospectively. RESULTS: The study was conducted with 219 (36.3%) rotavirus positive and 383 (63.6%) rotavirus negative patients. The A blood group was detected more commonly in the rotavirus positive group compared to the rotavirus negative group (50.6 vs. 42.2%, p= 0.047). Hospitalization rates of the cases with rotavirus gastroenteritis among children with the A blood group (30.6 vs. 8%, p= 0.001) were significantly higher. CONCLUSIONS: Rotavirus gastroenteritis was determined to be more common in children with A blood group. The hospitalization rates of such patients were detected to be higher. Therefore, rotavirus gastroenteritis among children with blood group A should be monitored closely.

Retinal and choroidal thickness in children with familial Mediterranean fever.

PURPOSE: The aim of the present study was to evaluate retinal and choroidal thicknesses in children with familial Mediterranean fever (FMF). METHODS: Thirty patients with FMF and 28 healthy controls were included in the study. The thicknesses of the retina and choroid of each subject's right eye were measured at the fovea and horizontal nasal and temporal quadrants at 500-µm intervals to 1500 µm from the foveal center using spectral-domain optic coherence tomography. RESULTS: Retinal and choroidal thicknesses at the fovea did not differ between groups (p = 0.32 and p = 0.39, respectively). Horizontal nasal and temporal retinal and choroidal thickness measurements at 500-µm intervals to a distance of 1500 µm from the foveal center were also similar between the groups (all p > 0.05). CONCLUSIONS: The retinal and choroidal thicknesses of children with FMF do not differ from those of age- and sex-matched healthy controls.

Serum hepcidin levels and iron metabolism in obese children with and without fatty liver: case-control study.

UNLABELLED: Hepcidin is a regulator of iron balance that is increased in obesity. It reduces the absorption of iron, reduces the transfer of iron from macrophages to the plasma and/or prevents mobilisation of stored iron. Obese patients with non-alcoholic fatty liver disease (NAFLD) demonstrate adipokine and cytokine release promoting inflammatory response. We aimed to analyse the hepcidin levels and iron metabolism in obese children with and without NAFLD and non-obese healthy controls. The study population consisted of 110 children aged 7-18 years in three groups: 50 obese patients without NAFLD, 30 obese patients with NAFLD, and 30 non-obese healthy controls. Serum hepcidin, ferritin, and iron levels, iron-binding capacity, lipid profile, and liver function tests were measured, and hepatic ultrasonography was performed in all participants. Obese patients' white blood cell counts, total cholesterol, triglyceride levels, and homeostatic model assessment of insulin resistance (HOMA-IR) were significantly higher than those of the control group. Iron-binding capacity was significantly higher in obese patients without NAFLD compared with obese patients with NAFLD (p = 0.002). Hepcidin levels were not significantly different between obese patients and the control group. However, hepcidin levels in obese patients with NAFLD were significantly higher than those in obese patients without NAFLD (p < 0.001). CONCLUSIONS: Hepcidin levels were significantly higher in obese children with NAFLD than those without NAFLD. Obese children with NAFLD should receive attention regarding iron metabolism disorders. Serum hepcidin could be a marker of iron metabolism status and NAFLD in these groups of patients.

Evaluation of children with lympadenopathy.

AIM: To examine children who present with enlargement of lymph nodes in terms of demographic, clinical, serological and radiological aspects. MATERIAL AND METHODS: Ninety-eight patients who presented with a complaint of enlargement of lymph nodes were examined in terms of demographic, clinical, serological and radiological aspects by screening file data retrospectively. The character of lymph nodes (reactive, malign) was evaluated according to the distribution, number, sizes and blood supply determined in ultrasonographic measurements. Fisher's Exact test and Mann-Whitney U Test were used in comparison of the groups. Kappa value was used in assessment of compatibility between the two groups. RESULTS: Cervical lymphadenomegaly was found most frequently in accordance with the complaint of swelling in the neck. Erythrocyte sedimentation rate, ultrasonography, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) IgM were not found to be statistically significatly different between the normal and abnormal physical examination groups (erythrocyte sedimentation rate; p=0.623, USG; p=0.753, EBV and CMV; p=1.00). Cytomegalovirus and EBV IgM were not found to be statistically significatly different between the normal and abnormal ultrasonography groups (CMV; p=0.35, EBV; p=0.36). There was no compatibility between physical examination and ultrasonography (=0.32). CONCLUSION: Lymphadenopathy is a common problem in the childhood and necessitates a careful physical examination and follow-up. Laboratory and imaging methods should be used when necessary. Although lymphadenopathy is mostly related with infections, care should be taken in terms of malignancy and malignancy should be eliminated. The important point is systemic evaluation and follow-up of the patient. It is important to note physical examination findings and clinical follow-up findings, because frequent ultrasonographic investigations may confuse physicians and families with high values of lymph node measurements despite normal physical examination. Therefore, using our fingertips with a good physical examination is still our most sensitive diagnostic tool instead of ultrasonography.

Is there a relationship between rotavirus positive gastroenteritis and ABO blood groups?: a retrospective cohort study. / Is there a relationship between rotavirus positive gastroenteritis and ABO blood groups?: a retrospective cohort study.

INTRODUCTION: Rotavirus is the most common cause of severe diarrhea in children. There is only a few clinical studies about the relationship between rotavirus gastroenteritis and ABO blood groups. OBJECTIVE: The aim of this study was to investigate the role of blood groups, if any present, in the incidence of rotavirus gastroenteritis, and the severity of the gastroenteritis. METHODS: The file records of babies born in our hospital and diagnosed with acute gastroenteritis in their follow up were investigated retrospectively. RESULTS: The study was conducted with 219 (36.3

) rotavirus positive and 383 (63.6

) rotavirus negative patients. The A blood group was detected more commonly in the rotavirus positive group compared to the rotavirus negative group (50.6 vs. 42.2

, p= 0.047). Hospitalization rates of the cases with rotavirus gastroenteritis among children with the A blood group (30.6 vs. 8

, p= 0.001) were significantly higher. CONCLUSIONS: Rotavirus gastroenteritis was determined to be more common in children with A blood group. The hospitalization rates of such patients were detected to be higher. Therefore, rotavirus gastroenteritis among children with blood group A should be monitored closely.

Primary vertebral lymphoma presenting with fracture.

We report a 15 year old girl admitted with back pain and multifocal osteolytic lesions without systemic symptoms at T7, L5, and S1 spinal vertebras. The child was diagnosed as having primary multifocal osseous lymphoma, in which multiple bones are involved in the absence of lymph node or visceral disease for at least 6 months following initial presentation.

Pulmonary tuberculosis mimicking metastasis in a patient with osteosarcoma.

Pulmonary metastasis even at diagnosis or during treatment is an important problem in osteosarcoma treatment and treatment modality varies in patients with metastasis. Metastasectomy and aggressive induction chemotherapy are the 2 most well-known treatment options. However, reactivation of pulmonary tuberculosis can be a problem in developing countries, where the prevalence of tuberculosis is high. Here, we described a patient with osteosarcoma with pulmonary tuberculosis reactivation mimicking metastasis.

Primary gastric Burkitt lymphoma: a rare cause of intraabdominal mass in childhood.

Primary gastric lymphoma in the pediatric population is rare and the role of Helicobacter Pylori (H. Pylori) in its pathogenesis is unclear. In this report, we describe a case of non-Hodgkin's lymphoma (Burkitt's type) coexisting with H. pylori and discuss the potential relationship between H. Pylori and gastric Burkitt lymphoma.

Melatonin ameliorates doxorubicin-induced skin necrosis in rats.

Doxorubicin (DXR), a highly effective chemotherapeutic agent, causes serious injury when extravasated. The injury can sometimes result in skin necrosis and ulceration, requiring surgery. The detrimental effect of DXR on the antioxidant system via free oxygen radicals is one of the mechanisms proposed in its etiology. Thus, we used melatonin, a potent antioxidant, and compared the effects with dimethylsulfoxide (DMSO), which is used in the treatment of patients with DXR-induced extravasation.Twenty-seven Wistar-albino rats were used. After intradermal injection of DXR, DMSO was injected into the extravasated area and melatonin was given intraperitoneally. On day 14 of the experiment, skin ulcers were clearly formed and samples were taken with a punch biopsy. Ulcer sizes were measured. Tissue samples were analyzed for superoxide dismutase, glutathione peroxidase, and malondialdehyde enzymes, and histopathologically evaluated.Melatonin clearly decreased MDA levels, ulcer size, and histopathologic ulcer scores in DXR extravasated tissue. DMSO also decreased MDA levels, ulcer size and histopathologic ulcer score. However, melatonin was remarkably more effective than DMSO in terms of antioxidant enzyme activity, oxidative stress, and histopathologic ulcer scores in rats. Necrosis was evident in the DXR-treated group and some slides showed necrosis involving the fascia. Histopathologic ulcer scores of the necrotic tissue decreased in the DMSO and melatonin groups. The ulcer score in the melatonin group was significantly lower than in the control group. Although the ulcer score in the DMSO group was lower than control, there was no statistically significant difference. The ulcer size in the DMSO group was significantly lower than the control group. The ulcer size in the melatonin group was significantly lower than both the DMSO and control groups.We believe that melatonin, either alone or in combination with DMSO, may be used for treating DXR extravasation. In addition, free oxygen radicals play a crucial role in the etiology of the injury, which should be considered in further studies.

Alpha-fetoprotein producing tumor cells in children with Wilms' tumor.

Alpha fetoprotein (AFP) is generally used as a marker in diagnosis and follow-up of germ cell tumors and hepatoblastomas. However, serum AFP levels were elevated in our three patients with Wilms tumor. The elevated levels could only be decreased completely by surgery and not by chemotherapy. Histopathologically, the tumors consisted of blastemal, stromal, and epithelial cells. Chemotherapy was only effective on stromal and epithelial components of the tumors. In AFP staining, the source of AFP production was identified as blastemal tumor cells. Because the increased AFP levels were decreased after surgery, AFP levels may be used in the follow-up of the patients with Wilms tumor. Herein, we report three patients with Wilms tumor whose serum AFP levels were elevated and who had diffuse WT-1 and focal AFP expression in all tumors, immunohistochemically.

Pediatric abdominal masses: diagnostic accuracy of diffusion weighted MRI.

PURPOSE: To retrospectively identify apparent diffusion coefficient (ADC) values of pediatric abdominal mass lesions, to determine whether measured ADC of the lesions and signal intensity on diffusion-weighted (DW) images allow discrimination between benign and malignant mass lesions. MATERIALS AND METHODS: Approval for this retrospective study was obtained from the institutional review board. Children with abdominal mass lesions, who were examined by DW magnetic resonance imaging (MRI) were included in this study. DW MR images were obtained in the axial plane by using a non breath-hold single-shot spin-echo sequence on a 1.5-T MR scanner. ADCs were calculated for each lesion. ADC values were compared with Mann-Whitney U test. Receiver operating characteristic curve analysis was performed to determine cut-off values for ADC. The results of visual assessment on b800 images and ADC map images were compared with chi-square test. RESULTS: Thirty-one abdominal mass lesions (16 benign, 15 malignant) in 26 patients (15 girls, 11 boys, ranging from 2 days to 17 years with 6.9 years mean) underwent MRI. Benign lesions had significantly higher ADC values than malignant ones (P < .001). The mean ADCs of malignant lesions were 0.84 +/- 1.7x10(-3) mm2/s, while the mean ADCs of the benign ones were 2.28 +/- 1.00x10(-3) mm2/s. With respect to cutoff values of ADC: 1.11x10(-3) mm2/s, sensitivity and negative predictive values were 100%, specificity was 78.6% and positive predictive value was 83.3%. For b800 and ADC map images, there were statistically significant differences on visual assessment. All malignant lesions had variable degrees of high signal intensity whereas eight of the 16 benign ones had low signal intensities on b800 images (P < .001). On ADC map images, all malignant lesions were hypointense and most of the benign ones (n=11, 68.7%) were hyperintense (P < .001). CONCLUSION: DW imaging can be used for reliable discrimination of benign and malignant pediatric abdominal mass lesions based on considerable differences in the ADC values and signal intensity changes.

Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease.

The histiocytoses are a heterogeneous group of disorders characterised by an excessive number of histiocytes. In most cases the pathophysiology is unclear and treatment is nonspecific. Faisalabad histiocytosis (FHC) (MIM 602782) has been classed as an autosomal recessively inherited form of histiocytosis with similarities to Rosai-Dorfman disease (RDD) (also known as sinus histiocytosis with massive lymphadenopathy (SHML)). To elucidate the molecular basis of FHC, we performed autozygosity mapping studies in a large consanguineous family and identified a novel locus at chromosome 10q22.1. Mutation analysis of candidate genes within the target interval identified biallelic germline mutations in SLC29A3 in the FHC kindred and in two families reported to have familial RDD. Analysis of SLC29A3 expression during mouse embryogenesis revealed widespread expression by e14.5 with prominent expression in the central nervous system, eye, inner ear, and epithelial tissues including the gastrointestinal tract. SLC29A3 encodes an intracellular equilibrative nucleoside transporter (hENT3) with affinity for adenosine. Recently germline mutations in SLC29A3 were also described in two rare autosomal recessive disorders with overlapping phenotypes: (a) H syndrome (MIM 612391) that is characterised by cutaneous hyperpigmentation and hypertrichosis, hepatomegaly, heart anomalies, hearing loss, and hypogonadism; and (b) PHID (pigmented hypertrichosis with insulin-dependent diabetes mellitus) syndrome. Our findings suggest that a variety of clinical diagnoses (H and PHID syndromes, FHC, and familial RDD) can be included in a new diagnostic category of SLC29A3 spectrum disorder.

Proanthocyanidin prevents methotrexate-induced intestinal damage and oxidative stress.

Mucositis is an important dose-limiting side effect of methotrexate for which there is no definitive prophylaxis or treatment. This study was designed to investigate whether proanthocyanidin had a protective effect on methotrexate-induced small intestine damage. Twenty-eight albino rats were randomized into four groups. To the first group, methotrexate was applied as a single dose (20mg/kg) intraperitoneally. To the second group, proanthocyanidin (100mg/kg) was given orally every day by gavage in addition to methotrexate application until the rats were killed. To the third group, only proanthocyanidin was administered. The fourth group was the control. All animals were sacrificed 4 days after the intraperitoneal injection of methotrexate for histopathological examination and the assay for tissue malondialdehyde, superoxide dismutase and glutathione peroxidase levels. Methotrexate caused jejunal injury and increased malondialdehyde levels. Administration of proanthocyanidin decreased the jejunal damage and malondialdehyde level, which were caused by methotrexate treatment and increased superoxide dismutase and glutathione peroxidase levels. These results suggest that proanthocyanidin may protect the small intestine of rats from methotrexate-induced damage. The effects of proanthocyanidin could result from its antioxidant properties.

Hepatoblastoma metastatic to the right atrium responding to chemotherapy alone.

An 18-month-old boy presented with abdominal pain and distension. On physical examination there was a 10 x 7-cm mass in the right upper abdominal quadrant. His alpha-fetoprotein level was 175,000 IU/mL. Abdominal magnetic resonance findings revealed hepatomegaly with multiple tumor masses involving nearly all the segments of the liver (PRETEXT IV). The tumor extended through the inferior vena cava and filled 2/3 of the right atrium. Echocardiography revealed normal cardiac function. Histopathologic findings after liver biopsy were consistent with hepatoblastoma. After 6 courses of chemotherapy including cisplatin and doxorubicin (PLADO, SIOPEL protocol), the cardiac tumor regressed completely. The patient's primary tumor was then fully resected; no cardiac surgery was performed. After surgery the AFP level was 4 IU/mL and echocardiography revealed normal cardiac function with no residual tumor. The patient has been in remission for 31 months postdiagnosis.