Clinical Characteristics and Outcomes of Cancer Cases Among Syrian Refugees From Southern Turkey.

Importance: Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed. Objective: To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees. Design, Setting, and Participants: This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated for cancer between January 1, 2011, and December 31, 2020, in hematology-oncology departments of 8 university hospitals in the Southern province of Turkey. Data were analyzed from May 1, 2022, to September 30, 2022. Main Outcomes and Measures: Demographic characteristics (date of birth, sex, and residence), date of first cancer-related symptom, date and place of diagnosis, disease status at first presentation, treatment modalities, date and status at last hospital visit, and date of death. The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision and International Classification of Childhood Cancers, Third Edition, were used for the classification of cancer. The Surveillance, Epidemiology, and End Results system was applied for staging. The diagnostic interval was defined as the number of days from first symptoms until the diagnosis. Treatment abandonment was documented if the patient did not attend the clinic within 4 weeks of a prescribed appointment throughout the treatment. Results: A total of 1114 Syrian adult and 421 Syrian children with cancer were included. The median age at diagnosis was 48.2 (IQR, 34.2-59.4) years for adults and 5.7 (IQR, 3.1-10.7) years for children. The median diagnostic interval was 66 (IQR, 26.5-114.3) days for adults and 28 (IQR, 14.0-69.0) days for children. Breast cancer (154 [13.8%]), leukemia and multiple myeloma (147 [13.2%]), and lymphoma (141 [12.7%]) were common among adults, and leukemias (180 [42.8%]), lymphomas (66 [15.7%]), and central nervous system neoplasms (40 [9.5%]) were common among children. The median follow-up time was 37.5 (IQR, 32.6-42.3) months for adults and 25.4 (IQR, 20.9-29.9) months for children. The 5-year survival rate was 17.5% in adults and 29.7% in children. Conclusions and Relevance: Despite universal health coverage and investment in the health care system, low survival rates were reported in this study for both adults and children with cancer. These findings suggest that cancer care in refugees requires novel planning within national cancer control programs with global cooperation.

Effects of parent- and child-related behavioral feeding problems in early childhood on malnutrition.

OBJECTIVE: Children's responses to food and their caregivers during normal developmental periods are known as feeding behavior. For the healthy development of these behaviors, parent and child relationships must also be healthy. Therefore, we aimed to investigate the effect of behavioral feeding problems on primary malnutrition (PM). METHOD: The Behavioral Pediatric Nutrition Assessment Scale (BPFAS) was administered to 300 malnourished and 300 control pediatric patients aged from 9 months to 4 years who were referred to our pediatric gastroenterology outpatient clinic. Pre- and posttreatment data were compared between the two groups. RESULTS: There was no statistically significant difference between patients with and without malnutrition in terms of gender and age (p = 0.191, p = 0.128, respectively). Total behavioral frequency (TBF) and total behavioral problem (TBP) scores were significantly higher in the malnutrition group (p < 0.001). In the logistic regression analysis of risk factors that may affect malnutrition we found that a total TBF score of ≥85 increases the risk of developing malnutrition 3.731 times, a child TBF score of ≥62 increases it 2.644 times, and a parental TBF score of ≥21 increases it 4.82 times (p < 0.001). When anthropometric measurements and BPFAS scores of 127 PM patients who received behavioral therapy with enteral products and who attended follow-up were compared with their pretreatment data, there was a significant improvement (p < 0.05). CONCLUSION: Our study showed that behavioral feeding problems may increase the risk of PM and that behavioral therapy together with enteral products has a positive effect on treatment. Therefore, in addition to nutritional support in patients with PM, offering behavioral feeding therapy to parents will positively affect both the child's physical development and the relationship between the parents and their child.

Immunodeficiency associated with a novel functionally defective variant of SLC19A1 benefits from folinic acid treatment.

Insufficient dietary folate intake, hereditary malabsorption, or defects in folate transport may lead to combined immunodeficiency (CID). Although loss of function mutations in the major intestinal folate transporter PCFT/SLC46A1 was shown to be associated with CID, the evidence for pathogenic variants of RFC/SLC19A1 resulting in immunodeficiency was lacking. We report two cousins carrying a homozygous pathogenic variant c.1042 G > A, resulting in p.G348R substitution who showed symptoms of immunodeficiency associated with defects of folate transport. SLC19A1 expression by peripheral blood mononuclear cells (PBMC) was quantified by real-time qPCR and immunostaining. T cell proliferation, methotrexate resistance, NK cell cytotoxicity, Treg cells and cytokine production by T cells were examined by flow cytometric assays. Patients were treated with and benefited from folinic acid. Studies revealed normal NK cell cytotoxicity, Treg cell counts, and naive-memory T cell percentages. Although SLC19A1 mRNA and protein expression were unaltered, remarkably, mitogen induced-T cell proliferation was significantly reduced at suboptimal folic acid and supraoptimal folinic acid concentrations. In addition, patients' PBMCs were resistant to methotrexate-induced apoptosis supporting a functionally defective SLC19A1. This study presents the second pathogenic SLC19A1 variant in the literature, providing the first experimental evidence that functionally defective variants of SLC19A1 may present with symptoms of immunodeficiency.

Pseudo-Meigs syndrome secondary to endodermal sinus tumor.

Ovarian tumors are the most common gynecological tumors seen in girls. Approximately 60-70% of them are germ cell tumors. Pseudo-Meigs syndrome is characterized by the presence of pelvic tumoral mass (benign or malign), pleural effusion, and massive acid. If the tumor is removed, acid and hydrothorax disappear. Endodermal sinus (yolk sac) tumor is a very rare cause in the diagnosis of Pseudo-Meigs syndrome, and only a few cases have been reported. This case is one of the rare cases presenting with Pseudo-Meigs syndrome and pathologically diagnosed as yolk sac tumor.

Evaluation of pediatric patients presenting with acute-onset unilateral transient acquired blepharoptosis / Avaliação de pacientes pediátricos apresentando blefaroptose transitória unilateral de início agudo

ABSTRACT Purpose: To evaluate the clinical features of pediatric patients with acute-onset, unilateral transient acquired blepharoptosis. Methods: In this retrospective study, the clinical records of patients between April 2015 and June 2020 were reviewed for evaluation of demographic features, accompanying neurological and ophthalmologic manifestations, symptom duration, etiological cause, and imaging findings. Patients with congenital and acquired blepharoptosis with chronic etiologies were excluded. Results: Sixteen pediatric patients (10 boys and 6 girls) with acquired acute-onset unilateral transient blepharoptosis were included in this study. The patients' mean age was 6.93 ± 3.16 years. The most commonly identified etiological cause was trauma in 7 patients (43.75%) and infection (para-infection) in 5 patients (31.25%). In addition, Miller Fisher syndrome, Horner syndrome secondary to neuroblastoma, acquired Brown's syndrome, and pseudotumor cerebri were identified as etiological causes in one patient each. Additional ocular findings accompanied blepharoptosis in 7 patients (58.33%). Blepharoptosis spontaneously resolved, without treatment, in all the patients, except those with Miller Fisher syndrome, neuroblastoma, and pseudotumor cerebri. None of the patients required surgical treatment and had ocular morbidities such as amblyopia. Conclusion: This study demonstrated that acute-onset unilateral transient blepharoptosis, which is rare in childhood, may regress without the need for surgical treatment in the pediatric population. However, serious pathologies that require treatment may present with blepharoptosis.

RESUMO Objetivo: Avaliar as características clínicas de pacientes pediátricos com blefaroptose adquirida unilateral, transitória e de início agudo. Métodos: Neste estudo retrospectivo, foram revisados prontuários clínicos entre abril de 2015 e junho de 2020. Os pacientes foram avaliados em termos de características demográficas, manifestações neurológicas e oftalmológicas associadas, duração dos sintomas, etiologia e achados de imagem. Foram excluídos pacientes com blefaroptose congênita e com blefaroptose adquirida de etiologia crônica. Resultados: Foram incluídos neste estudo 16 pacientes pediátricos (10 masculinos e 6 femininos) com blefaroptose adquirida transitória unilateral de início agudo. A média de idade dos pacientes foi de 6,93 ± 3,16 anos. As causas etiológicas mais comumente identificadas foram trauma em 7 pacientes (43,75%) e infecção (casos parainfecciosos) em 5 pacientes (31,25%). Além disso, a síndrome de Miller-Fisher, a síndrome de Horner secundária a neuroblastoma, a síndrome de Brown adquirida e pseudotumor cerebral foram determinados como causas etiológicas em um paciente cada uma. Achados oculares adicionais estavam associados à blefaroptose em 7 pacientes (58,33%). Foi observada a resolução espontânea da blefaroptose, sem tratamento, em todos os pacientes, exceto nos pacientes com síndrome de Miller-Fisher, neuroblastoma e pseudotumor cerebral. Nenhum paciente precisou de tratamento cirúrgico. Morbidades oculares, como ambliopia, não foram encontradas em nenhum paciente. Conclusão: Este estudo demonstrou que a blefaroptose transitória unilateral de início agudo, rara na infância, pode regredir sem a necessidade de tratamento cirúrgico na população pediátrica. No entanto, também não deve ser esquecido que patologias graves que requerem tratamento podem se apresentar com blefaroptose.

Evaluation of cases with infantile hemangioma requiring treatment.

AIM: Infantile hemangiomas are the most common benign vascular tumours in infants. In this study, we aimed to evaluate the effectiveness of propranolol therapy in patients with infantile hemangioma.

Evaluation of pediatric patients presenting with acute-onset unilateral transient acquired blepharoptosis.

PURPOSE: To evaluate the clinical features of pediatric patients with acute-onset, unilateral transient acquired blepharoptosis. METHODS: In this retrospective study, the clinical records of patients between April 2015 and June 2020 were reviewed for evaluation of demographic features, accompanying neurological and ophthalmologic manifestations, symptom duration, etiological cause, and imaging findings. Patients with congenital and acquired blepharoptosis with chronic etiologies were excluded. RESULTS: Sixteen pediatric patients (10 boys and 6 girls) with acquired acute-onset unilateral transient blepharoptosis were included in this study. The patients' mean age was 6.93 ± 3.16 years. The most commonly identified etiological cause was trauma in 7 patients (43.75%) and infection (para-infection) in 5 patients (31.25%). In addition, Miller Fisher syndrome, Horner syndrome secondary to neuroblastoma, acquired Brown's syndrome, and pseudotumor cerebri were identified as etiological causes in one patient each. Additional ocular findings accompanied blepharoptosis in 7 patients (58.33%). Blepharoptosis spontaneously resolved, without treatment, in all the patients, except those with Miller Fisher syndrome, neuroblastoma, and pseudotumor cerebri. None of the patients required surgical treatment and had ocular morbidities such as amblyopia. CONCLUSION: This study demonstrated that acute-onset unilateral transient blepharoptosis, which is rare in childhood, may regress without the need for surgical treatment in the pediatric population. However, serious pathologies that require treatment may present with blepharoptosis.

Results of multicenter registry for patients with inherited factor VII deficiency in Turkey.

INTRODUCTION: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients. METHODS: Demographic, clinical, and laboratory data of patients with FVIID extracted from the national database, constituted by the Turkish Society of Hematology, were examined. Bleeding phenotypes, general characteristics, and laboratory features were assessed in terms of FVIILs. Bleeding rates and prophylaxis during special procedures/interventions were also recorded. RESULTS: Data from 197 patients showed that 46.2% of patients had FVIIL< 10%. Most bleeds were of mucosal origin (67.7%), and severe bleeds tended to occur in younger patients (median age: 15 (IQR:6-29)). Cut-off FVIILs for all and severe bleeds were 16.5% and 7.5%, respectively. The major reason for long-term prophylaxis was observed as central nervous system bleeding (80%). CONCLUSION: Our data are consistent with most of the published literature in terms of cut-off FVIIL for bleeding, as well as reasons for prophylaxis, showing both an increased severity of bleeding and younger age at diagnosis with decreasing FVIIL. However, in order to offer a classification similar to that in Hemophilia A or B, data of a larger cohort with information about environmental and genetic factors are required.

Is N95 face mask linked to dizziness and headache?

OBJECTIVES: During the COVID-19 pandemic, healthcare professionals are recommended to use PPE to prevent the transmission of disease. Healthcare workers who use N95 FFR, which has an important place, experience complaints such as headache and dizziness. In this study, we plan to find the cause of these complaints and aim to clarify whether they are associated with the use of N95 mask. METHOD: Healthcare workers first put on a surgical mask for at least 1 h and a maximum of 4 h, this process was then repeated on another day with the same workers wearing N95 masks. After removing the mask, capillary blood gases were taken and a questionnaire was given. RESULTS: Thirty-four participants over the age of 18 were included in the study; 19 participants were female (56%) and 15 male (44%). The results of the capillary blood gas analysis after the use of surgical mask and N95 mask, respectively: pH: 7.43 ± 0.03; 7.48 ± 0.04 (p < 0.001); pCO2: 37.33 ± 8.81; 28.46 ± 7.77 mmHg (p < 0.001); HCO3: 24.92 ± 2.86; 23.73 ± 3.29 mmol/L (p = 0.131); Base excess (BE): 1.40 (- 3.90-3.10); - 2.68 (- 4.50-1.20) [median (Q1-Q3)] (p = 0.039); lactate: 1.74 ± 0.68; 1.91 ± 0.61 (p = 0314). Headache, attention deficit and difficulty in concentrating were significantly higher after using N95 mask. CONCLUSION: Respiratory alkalosis and hypocarbia were detected after the use of N95. Acute respiratory alkalosis can cause headache, anxiety, tremor, muscle cramps. In this study, it was quantitatively shown that the participants' symptoms were due to respiratory alkalosis and hypocarbia.

What chances do children have against COVID-19? Is the answer hidden within the thymus?

A new type of coronavirus named as SARS-CoV-2 pandemic has begun to threaten human health. As with other types of coronaviruses, SARS-CoV-2 affects children less frequently, and it has been observed that the disease is mild. In the pathogenesis of a standard viral infection, the pathogen's contact with the mucosa is initially followed by an innate immunity response. T cells are the primary decisive element in adaptive immunity capability. For this reason, the adaptive immune response mediated by the thymus is a process that regulates the immune response responsible for preventing invasive damage from a virus. Regulatory T cells (T-reg) are active during the early periods of life and have precise roles in immunomodulation. The thymus is highly active in the intrauterine and neonatal period; it begins to shrink after birth and continues its activity until adolescence. The loss of T-reg function by age results in difficulty with the control of the immune response, increased inflammation as shown in coronavirus disease (COVID-19) as an inflammatory storm. Also, the thymus is typically able to replace the T cells destroyed by apoptosis caused by the virus. Thymus and T cells are the key factors of pathogenesis of SARS-CoV-2 in children.Conclusion: We speculated that thymus activity and T lymphocyte function in children protect them against the virus effects. Stimulating and preventing the inhibition of the thymus can be possible treatment components against COVID-19. What is Known: • The SARS-CoV-2 infection does not often progress with an invasive clinic in children. • Thymus activity and T lymphocyte functions are highly active in children. What is New: • Effective thymus activity and T lymphocyte function in children protect them against the invasive SARS-CoV-2 infection. • Stimulating and preventing the inhibition of the thymus can be possible treatment components against COVID-19.

Effects of Oxidant-Antioxidant and Vitamin D Levels on Clinical and Laboratory Data in Children With Fatty Liver Disease.

Background and Aims Fatty liver increases oxidative stress and may trigger antioxidant mechanisms. We aimed to compare the levels of vitamin D, which has antioxidant properties, as well as total oxidant status (TOS), total antioxidant status (TAS), and catalase between patients with nonalcoholic fatty liver (NAFL) and the control group. Methods We compared vitamin D, TOS, TAS, catalase levels, and other biochemical parameters between pediatric patients with ultrasonographically detected NAFL and an age-matched healthy control group. Results NAFL patients had a significantly lower vitamin D level (p < 0.001). The patient group also had significantly greater height, weight, body mass index (BMI) Z score, parathyroid hormone, triglyceride, glucose, antioxidant (TAS and catalase), and TOS levels compared to the controls (p ≥ 0.001). There was no significant difference between the obese and non-obese NAFL patients with respect to TAS, TOS, catalase levels, and other biochemical parameters (p < 0.05). There was a positive correlation between height, weight, BMI Z score, and hepatosteatosis grade, and TAS, TOS, and catalase levels, and a negative correlation with vitamin D level. We also found a negative correlation between vitamin D level and TOS and catalase level. Conclusions Our study revealed lower levels of vitamin D and higher levels of oxidant-antioxidants including TOS, TAS, and catalase in patients with NAFL.

Assessment of Children Exposed to Maras Powder Intoxication.

INTRODUCTION: A smokeless tobacco known as Maras powder (MP) is commonly consumed in the southern region of Turkey. To the extent of our knowledge, no previous study in literature has reported acute MP intoxication in children. AIM: Our aim was to determine the clinical effects and treatment strategies of MP poisoning in children.Materials and methods: We retrospectively reviewed the medical records of children <18 years of age with MP intoxication who were followed up in the Intensive Care Unit between January 2016 and April 2018 in our center. RESULTS: Forty-one patients (M/F= 25/16) were included in this study. The mean age was 13.2±22.4 months (age range: 7 to 30 months). The patients presented with vomiting (n=23, 56%), cough (n=17, 41.5%), loss of consciousness (n=11, 26.8%), respiratory distress (n=6, 14.6%), convulsion (n=7, 17%), cyanosis (n=1, 2.4%), and abdominal pain (n=1, 2.4%) following oral ingestion of the substance. On their physical examination all patients with convulsion were in a comatose state. Thirty-two patients (78%) had tachycardia; 15 patients (36.5%) had pharyngeal hyperemia; and three (7.3%) had hiccups. Although the female patients had a lesser rate of symptoms than male patients, the difference was not statistically significant. CONCLUSIONS: Our aim was to inform the doctors about the clinical picture that develops after taking this substance and contribute to the understanding of the treatment approach.

Role of Hemodialysis in the Management of Cyanide Intoxication From Apricot Kernels in a 3-Year-Old Child.

Cyanide (CN) is one among the most potent and rapidly acting lethal poisons, and it may cause death unless immediately diagnosed and treated. We report an unusual case of pediatric CN poisoning after ingestion of apricot kernels containing amygdalin, who survived with antidotal therapy and hemodialysis. A 3-year-old girl presented with respiratory distress and coma following tonic-clonic convulsions after ingestion of 3 apricot kernels. She had severe metabolic acidosis (pH 6.91, bicarbonate [HCO3] 5.6 mEq/L, base excess -26.0 mEq/L). Her blood CN level was measured 3.15 mg/L, 3 hours after ingestion. Hydroxocobalamin could not be administered immediately because it had to be brought from a medical center 4 hours apart. Therefore, a 3-hour hemodialysis session was carried out, following which she showed some clinical improvement. In addition, when hydroxocobalamin was obtained, it was then administered. During follow-up, she was completely asymptomatic with blood pressure, and other hemodynamic parameters normalized. This case presents hemodialysis as a way to correct metabolic derangements from CN poisoning and suggests that it may have a role in select cases of pediatric CN poisoning, especially when CN-scavenging antidotes may be unavailable.

Cerebral atrophy in 21 hypotonic infants with severe vitamin B12 deficiency.

AIM: Nutritional B12 deficiency is a treatable cause of neurodevelopmental delay in infants. We report 21 infants with developmental regression and brain atrophy as revealed using cranial magnetic resonance imaging (MRI), secondary to severe vitamin B12 deficiency. METHODS: Twenty-one infants aged 4-24 months with B12 deficiencies who were admitted to our clinic between May 2013 and May 2018 were included in the study. MRI, bone marrow aspiration and the Denver-II Developmental Screening Test were performed in all infants. RESULTS: The mean age of the infants was 12.3 months, and the mean B12 level was 70.15 ± 32.15 ng/L. Hypotonia and neurodevelopmental retardation, and anaemia were present in all patients. Their bone marrow examinations were compatible with megaloblastic anaemia. Twelve patients had microcephaly, seven had tremor and one patient died of severe sepsis. Almost all patients were fed with breast milk and their mothers were also malnourished. Nine (42.9%) of the patients were Turkish and 12 (57.1%) were Syrian. All patients had abnormal Denver-II Developmental Screening Test scores. Most patients had severe cortical atrophy, cerebral effusion, thinning of the corpus callosum and delayed myelinisation in cranial MRI. Treatment with B12 resulted in dramatic improvement in general activity and appetite within 72 h. Tremors resolved in all cases. CONCLUSION: Neurological findings and developmental delay related to nutritional B12 deficiency can be prevented without sequelae if diagnosed early. Screening and treating of mothers for this deficiency will contribute to the health of both the mother and their feeding infant.

A Rare Catheter Complication in a Patient With Neuroblastoma: Horner's Syndrome.

Neuroblastoma is the most common type of extracranial solid tumor during childhood. Clinical presentation includes ipsilateral ptosis, myosis, anhydrosis and enophthalmos. The case of a 2.5-year-old boy who had a complaint of constriction of the left pupil for 3 days is presented. In the physical examination, the pupil of the OD was moderately dilated; there was myosis on the OS and ptosis on the left eyelid. Horner syndrome was considered due to these findings. History of the patient revealed that a central venous catheter insertion procedure was tried from the left side.

Disordered bone metabolism in hereditary spherocytosis patients.

Aim: This study was planned to evaluate bone health in patients with hereditary spherocytosis.Materials and methods: In this prospective study, a total of 30 hereditary spherocytosis patients which followed in the Pediatric Hematology and Oncology Department of KSU Medical Faculty and 30 patients for control group were included. Patient and control group were chosen equal in age and sex. Hemogram and biochemical tests (serum calcium, phosphorus, alkaline phosphatase, parathormone, vitamin D) and osteocalcin were studied from the patient and control groups. Also DXA examination was performed in the patient group.Results: There was a significant difference in hemogram parameters between the two groups due to hemolytic anemia in hereditary spherocytosis patients. In the patient group, osteocalcin was 6.88 ± 4.35 ng/ml, vitamin D was 17.74 ± 7.76 ng/ml and in the control group osteocalcin was 11.93 ± 8.92 ng/ml, vitamin D was 24.04 ± 11.70 ng/ml. There was a statistically significant difference between the vitamin D and osteocalcin levels of the two groups (p = 0.017 and 0.008, respectively). Bone density was assessed in the patient group. In patients DXA results showed lower Z-scores then the normal population according to age and sex.Conclusion: Hereditary spherocytosis patients should be followed closely in terms of development, puberty, bone health as they are in other hemolytic anemias. Nutritional recommendations, vitamin D supplementation, physical activity should be advised to protect bone health.

Late diagnosis of leukocyte adhesion deficiency type II and Bombay blood type in a child: a rare case report.

Leukocyte adhesion deficiency type II (LAD II) is a rare, autosomal, recessive inherited immunodeficiency disease that induces frequent and recurrent infections, persistent leukocytosis, severe mental and growth retardation, and impaired wound healing. The Bombay blood group is a rare blood group phenotype that is characterised by the deficiency of H, A, and B antigens on the surface of red cells. LAD II and the Bombay blood group are always seen together, because both of them are associated with a global defect in the common pathway of fucose metabolism. Here we report the case of an 11-year-old boy with LAD II, who presented with the Bombay blood group. Agglutination with strength of 4+ was detected in all cross-matching due to erythrocyte transfusions for our patient. Therefore, the Bombay blood group was incidentally determined due to deficient expression of the CD15 adhesion molecules on the surface of the leukocytes according to the results of flow cytometry. Upon detecting the Bombay blood type, LAD II was then diagnosed as a result of flow cytometry and the clinical findings of mental retardation and history of recurrent infections such as abscesses.