Five Years Follow-up of Opsoclonus-Myoclonus-Ataxia Syndrome-Associated Neurogenic Tumors in Children.

AIM: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare autoimmune disorder. Approximately half of the cases are associated with neuroblastoma in children. This study's aim is to review management of our cases with OMAS-associated neuroblastoma for treatment approach as well as long-term follow-up. METHODS: Age at onset of symptoms and tumor diagnosis, tumor location, histopathology, stage, chemotherapy, OMAS protocol, surgery, and follow-up period were evaluated retrospectively in six patients between 2007 and 2022. RESULTS: Mean age of onset of OMAS findings was 13.5 months and mean age at tumor diagnosis was 15.1 months. Tumor was located at thorax in three patients and surrenal in others. Four patients underwent primary surgery. Histopathological diagnosis was ganglioneuroblastoma in three, neuroblastoma in two, and undifferentiated neuroblastoma in one. One patient was considered as stage 1 and rest of them as stage 2. Chemotherapy was provided in five cases. The OMAS protocol was applied to five patients. Our protocol is intravenous immunoglobulin (IVIG) 1 g/kg/d for 2 consecutive days once a month and dexamethasone for 5 days (20 mg/m2/d for 1-2 days, 10 mg/m2/d for 3-4 days, and 5 mg/m2/d for the fifth day) once a month, alternatively by 2-week intervals. Patients were followed up for a mean of 8.1 years. Neuropsychiatric sequelae were detected in two patients. CONCLUSION: In tumor-related cases, alternating use of corticosteroid and IVIG for suppression of autoimmunity as the OMAS protocol, total excision of the tumor as soon as possible, and chemotherapeutics in selected patients seem to be related to resolution of acute problems, long-term sequelae, and severity.

Is very high platelet count always associated with essential thrombocythemia? An unusual presentation in a child.

Myeloproliferative neoplasms are rare in childhood. They are categorized as Philadelphia chromosome-positive and Philadelphia chromosome-negative. Chronic myeloid leukemia (CML) is the most common myeloproliferative disease in which the Philadelphia chromosome is detected as a result of BCR-ABL rearrangements. In others, the most common genetic abnormality is JAK2V617F mutation. The coexistence of these 2 abnormalities in CML is unexpected, and rare cases have recently been reported in adults. We present a child who had a very high platelet count in which we found this coexistence. The clinical presentation, laboratory findings, management, and prognosis of this coexistence is challenging in such a rare condition.

Long-Term Symptoms and Quality of Life in Persons with COVID-19.

Objective: After three years since the first cases of COVID-19, many people suffer from post-COVID symptoms, reducing their quality of life. In this study, we aimed to evaluate the symptoms and prevalence of long COVID and its effect on the quality of life and also the effect of SARS-CoV-2 vaccine on the quality of life. Materials and Methods: Patients with COVID-19 between April 01, 2020, and December 31, 2021, were evaluated with a survey at least three and at most 12 months (mean 7.43±3.3 months) after diagnosis. Patients answered the questions face-to-face or via telephone interview. The survey included questionnaires on demographic features, current complaints, and complaints that persisted or developed after recovery, Short Form 36 Health Survey (SF-36), and European Quality of Life 5 Dimensions 3 Level Version (EQ-5D-3L) Scale. Results: The study was conducted with 521 participants, 81% complaining of post-COVID symptoms. The most common symptoms were fatigue and shortness of breath. Long COVID was found to significantly reduce the quality of life in both sub-dimensions of the scale. Compared to SF-36 Turkish population norms, all participants showed a significant decrease in quality of life subscales. Vaccination against severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) was found to improve quality of life and reduce long-term COVID-19 symptoms. Conclusion: COVID-19 has significantly affected quality of life. Post-illness follow-up of people with COVID-19 is important to provide patients with medical, physical, and psychosocial rehabilitation support. SARS-COV-2 vaccine reduces the negative effects of COVID-19 on quality of life. For this reason, the community should be made aware of vaccination, and COVID-19 vaccination rates should be increased in the community.

Brucellosis in pregnancy: results of multicenter ID-IRI study.

Brucellosis in pregnant women is reported to be associated with obstetric complications (OCs), and adequate data for human brucellosis during pregnancy are largely lacking. We performed this multicenter retrospective cross-sectional study to evaluate the epidemiology, clinical course, treatment responses, and outcomes of brucellosis among pregnant women. The study period comprised a 14-year period from January 2002 to December 2015. All consecutive pregnant women diagnosed with brucellosis in 23 participating hospitals were included. Epidemiological, clinical, laboratory, therapeutic, and outcome data along with the assessment data of the neonate were collected using a standardized questionnaire. Data of 242 patients were analyzed. The OC rate was 14.0% (34/242) in the cohort. Of the 242 women, 219 (90.5%) delivered at term, 3 (1.2%) had preterm delivery, 15 (6.2%) aborted, and 5 (2.1%) had intrauterine fetal demise. Seventeen (7.0%) of the newborns were considered as low birth weight. Spontaneous abortion (6.1%) was the commonest complication. There were no maternal or neonatal deaths and pertinent sequelae or complications were not detected in the newborns. Splenomegaly (p = 0.019), nausea and/or vomiting (p < 0.001), vaginal bleeding (p < 0.001), anemia (blood hemoglobin < 11 g/dL; p < 0.001), high level of serum aspartate aminotransferase (> 41 IU/L; p = 0.025), oligohydramnios on ultrasonography (p = 0.0002), history of taking medication other than Brucella treatment during pregnancy (p = 0.027), and Brucella bacteremia (p = 0.029) were the significant factors associated with OCs. We recommend that pregnant women with OC or with fever should be investigated for brucellosis if they live in or have traveled to an endemic area.

Hepatitis A immunity in Yozgat, Turkey.

BACKGROUND: Since routine immunization could change the epidemiological profile of hepatitis A virus (HAV) infection in the future, it is important to determine the baseline immunity to HAV across Turkey. OBJECTIVE: The aim of this study was to determine the seroprevalence of hepatitis A among individuals 6 years of age and older in Yozgat, Turkey. DESIGN: Cross-sectional. SETTING: Community in central region of Turkey. PATIENTS AND METHODS: Questionnaires and blood specimens were collected and the presence of hepatitis A IgG antibodies against hepatitis A virus was determined quantitatively by ELISA. MAIN OUTCOME MEASURES: The rates of hepatitis A immunity by age group. SAMPLE SIZE: 1862. RESULTS: Immunity to hepatitis A was 79.1% (n=1473). The mean (SD) age was 17.1 (14.7) years in the nonimmune group and 37.8 (19.5) years in the immune group (P less than .001), and immunity increased with age. No significant difference in immunity rate was detected between gen.ders in children and adults. The seropositivity rate for subjects ages 6-19 years was lower than in subjects aged 20-96 years (52.2% versus 93.9%; P less than .001). CONCLUSION: A catch-up vaccination program is needed for persons aged 6-19 years in Yozgat. LIMITATIONS: Single region data which can not be generalized. For this reason, a multi-centered study that can reflect the whole country is recommended. CONFLICT OF INTEREST: None.

MicroRNA-17, MicroRNA-19b, MicroRNA-146a, MicroRNA-302d Expressions in Hepatoblastoma and Clinical Importance.

Hepatoblastoma (HB) is the most common liver malignancy in children. The prognosis changes according to the histologic subtypes of HB. In the present study, we aimed to characterize the expression level of selected microRNAs (miRNAs) in HB as well as in histologic subtypes, and to consider the association with the prognosis. A total of 22 HB tumor samples, subtyped as fetal (n=16) and embryonal (n=6), and 10 nontumorous surrounding liver samples were evaluated in this study. Expressions of miR-17, miR-146a, miR-302d, and miR-19b were analyzed in 22 HB tumor samples and 10 nontumorous surrounding liver samples by quantitative real-time polymerase chain reaction. Lower miRNA-17 expression levels were obtained in tumor samples in comparison with nontumorous surrounding liver samples (P=0.028). Lower miRNA-17 expression was significant for predicting prognosis in HB patients (area under receiver-operator characteristic curve=0.875, P=0.044). A higher-level of miR-19b was found in embryonal samples (P=0.008). Overall and event-free survival was not found to correlate with miRNA expression levels (P>0.05). This research finds miRNA-17 and miRNA-19b expression levels can provide important data on diagnosis and prognosis in HB showing different clinical behaviors.

Hepatitis A immunisation need in nursing students in Turkey.

Hepatitis A is a worldwide vaccine-preventable infection. The aim of our study was to determine the serological status of hepatitis A virus (HAV) among first-year nursing students in Turkey. A sample of 423 students was used and immunoglobulin G antibodies against HAV were determined quantitatively by enzyme-linked immunosorbent assay on each. Overall, 84.6% had no immunity to HAV, making them at risk for HAV, and so susceptible to nosocomial transmission. Nursing students who work in high-risk wards must be vaccinated against hepatitis A.

Measurements of Retinal Nerve Fiber Thickness and Ganglion Cell Complex in Neurofibromatosis Type 1, with and Without Optic Pathway Gliomas: A Case Series.

PURPOSE: The aim of this study was to investigate differences in retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thicknesses in neurofibromatosis 1 (NF1) cases, with and without optic pathway gliomas (OPGs). MATERIALS AND METHODS: In total, 33 eyes of 33 subjects were evaluated in this prospective observational case series. Twenty-one patients with a diagnosis of NF1 were enrolled. Patients with NF1 and OPGs were included in Group 1 (n = 9), and patients with NF1 without OPGs were included in Group 2 (n = 12). The control group (Group 3) was comprised of 12 age- and sex-matched subjects with no history of ophthalmic or systemic diseases. All of the subjects underwent complete ophthalmic examinations, including best-corrected visual acuity (BCVA), slit lamp microscopy, and indirect ophthalmoscopy. Additionally, optical coherence tomography (OCT) measurements were obtained. RESULTS: There were no statistically significant between-group differences in age and sex (p = 0.227 and 0.986, respectively). The average RNFL thickness in Group 1 (NF1 patients with OPGs) was significantly lower than in Groups 2 and 3 (86.6 ± 22.5, 107.4 ± 6.65, and 108.4 ± 5.05 µm, respectively; p = 0.001). Furthermore, the average GCC thickness in Group 1 was significantly lower than in Groups 2 and 3 (78.6 ± 16.3, 94.8 ± 3.55, and 94.9 ± 3.82 µm, respectively; p < 0.001). CONCLUSIONS: Both RNFL and GCC thicknesses were significantly lower in NF1 patients with OPGs. The use of OCT to quantify damage to the visual pathway may enable earlier detection of OPGs in NF1 patients.

A New Perspective for Infantile Hepatic Hemangioma in the Age of Propranolol: Experience at Baskent University.

Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Hemangiomas, like pure arteriovenous malformations, can cause hyperdynamic heart failure, and diffuse nodular-type hemangiomas can present with hypothyroidism. Respiratory problems and hepatic failure can be associated with diffuse nodular-type liver hemangiomas. There is a spectrum of approaches to management, varying from "watchful waiting" to liver transplant. In the age of propranolol, there has been a prominent change in the infantile hepatic hemangioma treatment algorithm. Our suggestion is early treatment with 3 mg/kg/day propranolol plus 1.0 to1.5 mg/kg/day prednisolone in all patients. This protocol is the most effective strategy for type 3 infantile hepatic hemangioma. Approximately one-third of patients with abdominal compartment syndrome in the era before propranolol treatment required liver transplant; this new treatment obviates transplant for many of these patients.

Secondary Hemophagocytic Lymphohistiocytosis: Do We Really Need Chemotherapeutics for All Patients?

Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1.02, P=0.006), pediatric logistic organ dysfunction scores (hazard ratio=1.01, P=0.001) were the predictors of the survival. The hospital survival was 83% for patients with sHLH who were treated with less immunosuppressive therapy. In conclusion initiation of HLH-specific therapy for the patients with hyperferritinemia-associated sHLH should be delayed while awaiting resolution of systemic inflammation with less immunosuppressive therapy.

A different cause for respiratory disorder in children: cases with pulmonary Langerhans cell histiocytosis.

BACKGROUND AND AIMS: In children, complaints of a respiratory disorder are very frequent. Etiology of respiratory illness is a broad spectrum that varies from a simple viral infection to a malignant disorder. Pulmonary Langerhans cell histiocytosis (PLCH) is one of these entities and it is truly rare in children. The aim of this study is to evaluate our patients with PLCH. METHODS: Patients who had been diagnosed with PLCH were retrospectively evaluated. Features of medical history, onset of the complaints, date of the diagnosis, chest X-Ray and computed tomography (CT) findings, histopathology and other laboratory investigations were considered. RESULTS: There were four cases with PLCH. All of them were male, ages were between 5 months and 16 years. In three cases, major complaints were chronic respiratory problems whereas in one of them there was acute respiratory distress beginning with cough and leading to pneumothorax. In all of the cases, multisystemic involvement was prominent. The diagnosis was proven by histopathology in all of the cases. In two children with smaller age, skin involvement was detected. Time from complaint to diagnosis was minimum 3 months and maximum 3 years. CONCLUSION: PLCH is a rare disorder in children. Pulmonary involvement is generally a component of systemic involvement but in many cases it might have been detected with early respiratory complaints. So, children with chronic respiratory problems should be carefully evaluated and should be followed up for rare entities like PLCH.

A Case of Idiopathic Pulmonary Hemosiderosis Presenting With Signs and Symptoms Mimicking Hemolytic Anemia.

Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia. While he was being investigated for clinical and laboratory signs mimicking hemolytic anemia, he developed cough and dyspnea. He had infiltrates on chest x-ray and scattered patchy infiltrates in both lungs on high-resolution computed tomography. Hemosiderin laden macrophages were identified in fasting gastric juice and bronchoalveolar lavage fluid. The patient was diagnosed with idiopathic pulmonary hemosiderosis and started corticosteroid therapy.

Quality of life and related factors among chronic hepatitis B-infected patients: a multi-center study, Turkey.

BACKGROUND: The aim of this study was to assess health-related quality of life (HRQOL) among chronic hepatitis B (CHB) patients in Turkey and to study related factors. METHODS: This multicenter study was carried out between January 01 and April 15, 2015 in Turkey in 57 centers. Adults were enrolled and studied in three groups. Group 1: Inactive HBsAg carriers, Group 2: CHB patients receiving antiviral therapy, Group 3: CHB patients who were neither receiving antiviral therapy nor were inactive HBsAg carriers. Study data was collected by face-to-face interviews using a standardized questionnaire, Short Form-36 (SF-36) and Hepatitis B Quality of Life (HBQOL). Values equivalent to p < 0.05 in analyses were accepted as statistically significant. RESULTS: Four thousand two hundred fifty-seven patients with CHB were included in the study. Two thousand five hundred fifty-nine (60.1 %) of the patients were males. Groups 1, 2 and 3, consisted of 1529 (35.9 %), 1721 (40.4 %) and 1007 (23.7 %) patients, respectively. The highest value of HRQOL was found in inactive HBsAg carriers. We found that total HBQOL score increased when antiviral treatment was used. However, HRQOL of CHB patients varied according to their socio-demographic properties. Regarding total HBQOL score, a higher significant level of HRQOL was determined in inactive HBV patients when matched controls with the associated factors were provided. CONCLUSIONS: The HRQOL score of CHB patients was higher than expected and it can be worsen when the disease becomes active. Use of an antiviral therapy can contribute to increasing HRQOL of patients.

Evaluation of tetanus antibody levels in adults in Yozgat, Turkey.

BACKGROUND/AIM: To assess the immune status against tetanus in relation to self-declared vaccination status among an adult population in Yozgat, Turkey. METHODS: Questionnaires and blood specimens were collected from 267 individuals over 18 years of age in Yozgat, Turkey. Antitetanus antibodies were determined quantitatively by enzyme-linked immunosorbent assay. RESULTS: Among the 267 subjects (mean age 43.4 ± 15.6; male:female, 133:134) 168 (62.9%) had protective level of tetanus antibody titers. There was a characteristic distribution of the tetanus antibody titers in different age groups as it dropped with increasing age. Protective immunity was obtained in 74.1% of urban and 47.9% of rural participants. The lowest immunity was observed among farmers (53.3%) and housewives (55.6%) when compared to other occupations. Protective antibody titers were detected in 25% of the individuals who had not finished any school, while the rate was 83.7% in those who had graduated from university. While the protection rate in women who had been vaccinated during pregnancy was 78.3%, this rate was 39.2% in nonvaccinated women. CONCLUSION: Our findings confirm that only 62.9% of the population is immunized against tetanus, with a lack of coverage in particular for the elderly and in rural areas. It is necessary to follow the recommendations for 10-year boosters for tetanus.

Neuromyelitis optica mimics the morphology of spinal cord tumors.

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system, that predominantly affects the spinal cord and the optic nerve. Its key features include transverse myelitis, commonly associated with extensive inflammation spanning three or more consecutive vertebral segments. Longitudinal extensive spinal cord lesions can also occur in systemic autoimmune diseases, infections, vascular and metabolic disorders, subsequent to irradiation, intramedullary tumors and paraneoplastic myelopathies. We present a case study of an 8-year-old girl seropositive for antibodies against the aquaporin 4 who displayed longitudinal extensive spinal cord lesions, that was initially misdiagnosed as an intramedullary tumor.

Pediatric langerhans cell histiocytosis: single center experience over a 17-year period.

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.

Immunity of nursing students to measles, mumps, rubella, and varicella in Yozgat, Turkey.

Measles, mumps, rubella, and varicella (MMRV) are vaccine-preventable diseases. The aim of this study was to determine the vaccination status of first-year nursing students in Turkey. The sample used was 180 students and immunoglobulin G antibodies against MMRV viruses were determined quantitatively by enzyme-linked immunosorbent assay. Immunity rates to MMRV were 82.8%, 83.3%, 98.3%, and 100%, respectively. The results of this study showed that all of the students were immune to varicella and 32.8% of the students were not immune to at least 1 of the viruses covered by the measles, mumps, and rubella vaccine.