Wilms' tumor is the most common malignant renal tumor in childhood. The brain metastasis of a Wilms' tumor with anaplastic histopathology is rare. We present the case of an 8-year-old girl with Wilms' tumor, who presented with multiple brain metastases 5 years after her primary diagnosis. The brain masses were diagnosed after a generalized tonic-clonic seizure attack. The big solid mass in the cerebellum was resected, and whole-brain radiotherapy was performed, after which, she succumbed to her disease. In the case of clinical suspicion, cranial surveillance should be included in the routine clinical work-up for Wilms' tumor. Combined aggressive therapy (surgery+radiotherapy+chemotherapy) should be applied whenever possible, for both better survival and palliative aspects.
Brain* ; Cerebellum ; Child ; Diagnosis ; Drug Therapy ; Female ; Glycogen Storage Disease Type VI ; Humans ; Neoplasm Metastasis* ; Radiotherapy ; Seizures ; Wilms Tumor*

Purpose: This study aimed to evaluate the radiation-induced adverse effects on ocular structures in head and neck cancer patients and investigate the radiation dose-volume effects on the cornea, lacrimal gland, retina, optic nerve and chiasm. Materials and Methods: A total of 38 eyes of 19 patients were included in this prospective, cohort study. All patients underwent complete ophthalmological examination in addition to contrast sensitivity, visual field and visual evoked potentials (VEP) tests. Ophthalmological examinations and psychophysical tests were performed in 6th, 12th, 18th, 24th months and in the last visit. The relationship between the ophthalmologic findings, and the radiation doses below and above the cut-off values was evaluated. Results: Contrast sensitivity decrease and visual field deterioration were observed in 42% of the patients in the last visit (median 26 months) whereas a prolonged latency and decreased amplitude of P100 wave in VEP was observed in 58% and 33% of the eyes, respectively at 24th month. Totally 16 patients (84.2%) developed dry eye disease and eight of them received radiotherapy below tolerance doses and had mild to moderate dry eye findings. Radiation-induced retinopathy was observed in three of the eyes in eight patients who received radiation above tolerance dose. Conclusion Head and neck cancers treated with radiotherapy, resulted in various ophthalmic complications. All patients who are treating with radiotherapy should be evaluated by an ophthalmologist in terms of anterior and posterior segment damage, even if the radiation dose is below the tolerance limit.

Visceral leishmaniasis (VL) is a life-threatening infection caused by Leishmania species. In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena. To date, VL mimicking or exacerbating various autoimmune diseases have been described, including systemic lupus erythematosus (SLE), rheumatoid arthritis, and autoimmune hepatitis (AIH). Herein, we presented a patient with VL who had overlapping clinical features with SLE, AIH, as well as antimitochondrial antibody (AMA-M2) positive primary biliary cirrhosis.
Adult ; Autoantibodies/blood ; Bone Marrow/pathology ; Cytological Techniques ; Diagnosis, Differential ; Female ; Hepatitis, Autoimmune/complications/*pathology ; Histocytochemistry ; Humans ; Leishmaniasis, Visceral/complications/*diagnosis/*pathology ; Liver/pathology ; Liver Cirrhosis, Biliary/complications/*pathology ; Lupus Erythematosus, Systemic/complications/*pathology

BACKGROUND AND PURPOSE: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy. Although its etiology is unknown, certain conditions are commonly associated with CTS, such as obesity, arthritis, hypothyroidism, diabetes mellitus, trauma, mass lesions, amyloidosis, and sarcoidosis. We aimed to determine the association between metabolic syndrome and CTS, and we compared the severity of CTS between patients with diabetes (and no concomitant metabolic syndrome) and patients with metabolic syndrome. METHODS: Two hundred patients with a clinically and electrophysiological confirmed diagnosis of CTS were included in the study. Their demographic characteristics and severity of CTS were analyzed according to the presence or the absence of metabolic syndrome. Differences in the electrophysiological findings were evaluated between the following four groups: 1) metabolic syndrome alone (n=52), 2) diabetes alone (n=20), 3) combined metabolic syndrome and diabetes (n=44), and 4) no metabolic syndrome or diabetes (n=84). RESULTS: CTS was more severe in the patients with metabolic syndrome than those without this syndrome. The electrophysiological findings were worse in patients with metabolic syndrome alone than in those with diabetes alone and those without diabetes and metabolic syndrome. CONCLUSIONS: CTS appears to be more severe in patients with metabolic syndrome than patients with diabetes. Diabetes is one of the well-known risk factors for CTS, but other components of metabolic syndrome may have a greater effect on the severity of CTS.
Amyloidosis ; Arthritis ; Carpal Tunnel Syndrome* ; Diabetes Mellitus ; Diagnosis ; Electrophysiology ; Humans ; Hypothyroidism ; Obesity ; Risk Factors ; Sarcoidosis

PURPOSE: Food allergy (FA) affects the daily lives of children and parents in varying degrees. The Food Allergy Quality of Life Questionnaire-Parent Form (FAQLQ-PF) is a valid and reliable instrument to assess the quality of life (QoL) of children from parents' perception. The aim of this study was to validate and determine the reliability of the Turkish FAQLQ-PF and to assess QoL in food-allergic children. METHODS: Children aged between 0 and 12 years and diagnosed with immunoglobulin E (IgE)-mediated FA for at least 1 month were enrolled. The English FAQLQ-PF was translated into Turkish according to the World Health Organization guidelines. The Food Allergy Independent Measure and the Turkish Child Health Questionnaire-Parent Form 50 were used for construct validity. RESULTS: One hundred and fifty-seven patients participated. The median age of patients and FA duration were 2.4 years (1.2–5.2 years, interquartile-ranges) and 2 years (0.8–5.1), respectively. Ninety-six (61.1%) patients had anaphylaxis. The Cronbach's alpha coefficient and intra-class correlation coefficient for test-retest reliability was good for all age groups of children ( < 4, 4–6, and 7–12 years). Patients with either asthma or anaphylaxis had worse scores than others. Total scores of FAQLQ-PF tended to increase with age. Patients aged 7–12 had the highest total scores among all patients (2.2±0.1, 3.0±0.2, and 3.3±0.3 for < 4, 4–6, and 7–12 years, respectively, P < 0.001, P for trend < 0.001). Other factors causing the poor QoL were cow's milk allergy, sibling allergy, mother's age over 30 years, mother's high education level and lower number of persons in household. CONCLUSIONS: The Turkish FAQLQ-PF is a valid and reliable scale. FA-related QoL was significantly worse with age. Coexistent asthma, anaphylaxis regardless of its severity, cow's milk allergy, sibling allergy and the older and educated mothers seem to poorly affect QoL.
Anaphylaxis ; Asthma ; Child Health ; Child* ; Education ; Family Characteristics ; Food Hypersensitivity ; Humans ; Hypersensitivity ; Immunoglobulin E ; Immunoglobulins ; Milk Hypersensitivity ; Mothers ; Parents ; Quality of Life* ; Reproducibility of Results ; Siblings ; World Health Organization

OBJECTIVE: This study was performed to explore the possibility that each oocyte and its surrounding cumulus cells might have different genetic expression patterns that could affect human reproduction. METHODS: Differential gene expression analysis was performed for 10 clusters of cumulus cells obtained from 10 cumulus-oocyte complexes from 10 patients. Same procedures related to oocyte maturation, microinjection, and microarray analyses were performed for each group of cumulus cells. Two differential gene expression analyses were performed: one for the outcome of clinical pregnancy and one for the outcome of live birth. RESULTS: Significant genes resulting from these analyses were selected and the top 20 affected pathways in each group were analyzed. Circadian entrainment is determined to be the most affected pathway for clinical pregnancy, and proteoglycans in cancer pathway is the most affected pathway for live birth. Circadian entrainment is also amongst the 12 pathways that are found to be in top 20 affected pathways for both outcomes, and has both lowest p-value and highest number of times found count. CONCLUSION: Although further confirmatory studies are necessary, findings of this study suggest that these pathways, especially circadian entrainment in cumulus cells, may be essential for embryo development and pregnancy.
Circadian Clocks ; Cumulus Cells ; Embryonic Development ; Female ; Gene Expression ; Granulosa Cells ; Humans ; Infertility ; Live Birth ; Microarray Analysis ; Microinjections ; Oocytes ; Ovarian Follicle ; Pregnancy ; Proteoglycans ; Reproduction ; Reproductive Techniques, Assisted

Background: Internalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons’ illness, has not been studied previously in pediatric psoriasis patients. Objective: We aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients. Methods: This multicenter,cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study. Results: The mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores. Conclusion Internalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds.