Celiac disease and hematological abnormalities in children with recurrent aphthous stomatitis.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is one of the most common diseases of the oral mucosa and may be related to vitamin deficiencies or systemic diseases such as celiac disease (CD). The aim of this study was to investigate the frequency of hematinic deficiency and CD in children with RAS. METHODS: The medical records of patients diagnosed with RAS were reviewed for the presence of hematinic deficiencies (hemoglobin, mean corpuscular volume, ferritin, vitamin B12 , folic acid), and CD. The study group included 108 children with RAS and 57 healthy children who were evaluated for hematological abnormalities in routine evaluation. RESULTS: The frequency of a family history of RAS was significantly higher in the RAS group compared to the control group (34.2% vs 7%, respectively; P < 0.001). A hematological abnormality was detected in 32.4% of the RAS group and 10.5% of the control group (P = 0.02). The prevalence of iron deficiency anemia was significantly higher in the RAS group (P = 0.037). Three (2.7%) patients with RAS were diagnosed with CD, which is a significantly higher frequency than that observed in healthy children in Turkey (P < 0.01; OR 6.03, 95% CI [2.37, 4.56]). These children had mild malnutrition, iron deficiency, and iron deficiency anemia. CONCLUSIONS: Children with RAS should be evaluated for nutritional status and hematological indices, and in the case of hematological abnormalities and malnutrition screening for CD should be considered.

An Unusual Presentation of Cytomegalovirus Infection: Generalized Edema.

OBJECTIVE: Cytomegalovirus (CMV) infection may rarely lead to protein-losing gastropathy that presents with nausea, vomiting, abdominal pain, and edema in immunocompetent children, but extremely rarely with only generalized edema. CLINICAL PRESENTATION AND INTERVENTION: A previously healthy 5-year-old boy presented with generalized edema without any other symptoms. He had hypoalbuminemia but no proteinuria. He was evaluated for gastrointestinal protein loss, and hypertrophic gastropathy was revealed on esophagogastroduodenoscopy. Meanwhile, CMV infection was detected by serologic tests and polymerase chain reaction in the blood. He recovered spontaneously within a week. CONCLUSION: CMV-related protein-losing gastropathy may present with generalized edema without any gastrointestinal symptoms.

Pediatric electrical injury in Turkey: Five year retrospective hospital-based study.

BACKGROUND: We aimed to evaluate children who presented with electrical injury to the emergency department. We tried to reveal the complications of these patients and the measures to be taken in this regard. METHODS: Medical records of pediatric patients who were admitted with electrical injury to emergency department between January 2007 and January 2012 were retrospectively reviewed. An information form was completed about demographic data, clinical features, exposure place, electrical current type, clinical course, duration of hospitalization, electrocardiography, echocardiography and other systems findings. RESULTS: Children who were the most exposed to electrical injury were children 4 years and under. While low-voltage current exposure was more common in younger ages, high-voltage current exposure was more common in older ages. Most of our patients (%72) were exposed to low voltage. Patients who exposed to high-voltage electricity had significantly higher levels of CPK, CK-MB, LDH, AST, and ALT compared to patients who were exposed to low voltage electricity. None of the patients have died. CONCLUSION: Young children are often exposed to electric shock at home. Increasing precautions to be taken at home reduces electrical shock injuries. Children and especially families should be educated in this regard.

Clinical and laboratory findings of 97 pediatric brucellosis patients in central Turkey.

Brucellosis is a disease transmitted to humans by consumption of unpasteurized animal milk, or through direct contact with infected animals. The aim of this study was to evaluate clinical, laboratory findings of pediatric patients with brucellosis. Data of 97 patients diagnosed with brucellosis between January 2000 and December 2010 were evaluated retrospectively.

Pseudotumor cerebri/idiopathic intracranial hypertension in children: an experience of a tertiary care hospital.

OBJECTIVE: Pseudotumor cerebri (PTC) is diagnosed at increasing rates probably due to the increase in obesity prevalence all over the world and awareness about the disease. Our aim in this study was to evaluate the PTC clinical picture and etiological factors in children at the present time. METHOD: The records of 53 patients with 32 females, who were diagnosed with PTC in a child neurology department between the years of 2005 and 2012 were retrospectively analyzed. RESULTS: The mean age at presentation was 10.9 years (3-17 years) and approximately half of patients were aged of 11 years or less. While more than half of prepubertal patients were male, girls rate reaches 74% at puberty. An etiological factor such as venous sinus thrombosis, infections, anemia, steroid discontinuation, drugs, slit ventricle syndrome and minor head injury causing the PTC was identified in 43% of the patients. The mean duration of treatment was 6.4 months (3-24 months) and the mean follow-up duration 16.5 months (3-52 months). Visual field constriction was moderate in only two pubertal and obese female patients and mild in four patients. CONCLUSIONS: PTC is seen in prepubertal children as often as in puberty. An etiological factor causing PTC is present in about half the patients in childhood. The main etiological factors of the disease currently consist of cranial venous thrombosis, infections, anemia and drugs. Malnutrition, renutrition and related vitamin deficiencies or excesses commonly seen previously have become less important in PTC etiology. PTC is a disease that requires long-term treatment and follow-up but the prognosis is good in patients who are diagnosed early, receive appropriate treatment and show good compliance with the treatment.

Reactive thrombocytosis in children.

The aim of this study was to evaluate the causes of thrombocytosis, which was defined as a platelet count greater than 500 x 10(9)/L, and to compare the groups with mild and severe thrombocytosis. A total of 484 patients were evaluated for the etiology of thrombocytosis. Patients with a platelet count between 500-800 x 10(9)/L were considered to have mild thrombocytosis, while those with a count of ≥800 x 10(9)/L were considered as having severe thrombocytosis. Of 484 patients included, 63% had thrombocytosis due to an infectious disease, 11.4% had a chronic inflammatory condition, 8.5% had anemia, and 5.2% had tissue injury. The frequency of chronic inflammation was higher in the severe thrombocytosis group compared to the mild thrombocytosis group (p=0.006). In conclusion, severe infections and chronic inflammatory conditions should be considered in the differential diagnosis of a patient with severe thrombocytosis.

Unilateral exudative retinal detachment as the sole presentation of relapsing acute lymphoblastic leukemia.

Ocular findings are rarely the initial symptom of leukemia, although up to 90% of all leukemia patients have fundus changes during the course of the disease. Herein we report a relapsing acute lymphoblastic leukemia patient with thesole presentation of sudden visual loss and exudative retinal detachment. An 8-year-old boy with acute lymphoblasticleukemia developed sudden visual loss during his first remission period. Bullous retinal detachment with total afferentpupillary defect was observed. Orbital magnetic resonance imaging revealed an intraocular mass lesion; simultaneouslyobtained bone marrow and cerebrospinal fluid samples showed no evidence of leukemic cells. Following local irradiation,and systemic and intrathecal chemotherapy the mass disappeared. Local irradiation, and systemic and intrathecalchemotherapy effectively controlled the isolated ocular relapse of acute lymphoblastic leukemia and eliminated the needfor enucleation.

Crimean-Congo hemorrhagic fever in children.

BACKGROUND: Crimean-Congo hemorrhagic fever (CCHF) virus causes a severe disease in humans with a mortality up to 30%. In Turkey there has been an increase in the number of cases during years since 2002. Humans of all ages living in endemic areas,especially those who are working as shepherds and toddlers, have high risk of acquiring CCHF. OBJECTIVES: The epidemiological, clinical, and laboratory characteristics of the children, who were diagnosed as Crimean-Congo hemorrhagic fever (CCHF) were described. STUDY DESIGN: The children infected with CCHF virus between April 2008 and October 2009, and hospitalised in Ankara Diskapi Children's and Research Hospital were included. RESULTS: Laboratory diagnosis was set by detection of CCHF IgM antibodies and/or genetic detection of CCHF virus. Thirtyone cases included to the study, and all were from the northeastern Anatolia and the southern parts of Black sea region. The mean age was 9.45+/-4.9 years, the proportion of females was 38.7%. The majority (87%) of the cases had the history of tick bite. There was no fatal case. All the patients had the history of fever. Malaise,tonsillopharyngitis, nausea-vomiting, headache, diarrhea, myalgia and rash were the most common symptoms. The mean AST and ALT levels on the admission were 116 (range 25-389) and 61 (range 8-180)U/L respectively. The mean platelet count on admission was 125,000/mm3, and the lowest was 23,000/mm3. The mean of the lowest white blood cell count was 2353/mm3 and the mean of the highest lactate dehydrogenase was 861IU/L. CONCLUSIONS: The clinical course of CCHF among children seems to be milder than in adults. Tonsillopharyngitis is a common symptom among children with CCHF.

Cavernous sinus syndrome as the initial presentation of childhood non-hodgkin lymphoma.

A 10-year-old boy with unilateral ophthalmoplegia, ptosis, and proptosis underwent diagnostic examination. Symptoms of headache, nausea, dyspnea, fatigue, weakness, and loss of appetite began 14 days after the onset of ocular manifestations and 7 days before he was examined. Complete blood count showed an increased white blood cell count with 64% blast cells, anemia, and thrombocytopenia. The patient was transferred to a pediatric hematology unit, where he underwent bone marrow aspiration biopsy. Bilateral ocular inflammatory findings and left-sided mild proptosis became evident. He was diagnosed as having B-cell non-Hodgkin lymphoma infiltrating the bilateral cavernous and sphenoid sinuses.

Primary subacute Salmonella osteomyelitis of the navicular bone in a child with normal immunity.

Primary subacute haematogenous osteomyelitis is one of the causes of limp. It usually involves tubular bones. Flat and small bones are affected less commonly. Diagnosis is difficult and usually takes weeks together for completion. Salmonella spp. can be isolated as a cause of primary subacute haematogenous osteomyelitis, if a usually underlying disorder, such as sickle cell anemia is associated. In this study, we present a child with normal immunity diagnosed as Salmonella primary subacute haematogenous osteomyelitis of the navicular bone, which is a rare condition. Primary subacute haematogenous osteomyelitis must be considered as a cause of limp for timely diagnosis and treatment.

Arc syndrome without arthrogryposis, with hip dislocation and renal glomerulocystic appearance: a case report.

INTRODUCTION: Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is a rare multisystem disorder first described in 1979 and recently ascribed to mutation in VPS33B whose product acts in intracellular trafficking. It exhibits wide clinical variability. Arthrogryposis, spillage of various substances in the urine, and conjugated hyperbilirubinemia define an ARC core phenotype, in some patients associated with ichthyosis, central nervous system malformation, deafness, and platelet abnormalities. Many patients with different associations of cholestasis, renal tubular acidosis, and dysmorphic morphology may be underdiagnosed. CASE REPORT: We report the third patient of ARC syndrome from Turkey with an incomplete form with renal tubular dysfunction and cholestasis in the absence of arthrogryposis but exhibiting other rare features. CONCLUSION: Our case highlights that the variability of involvement of different systems in ARC syndrome is well described; however, the renal glomerulocystic appearance has not been reported previously. Knowledge of this rare condition can benefit the practitioner as well as the patient.