Rare skin manifestation of juvenile dermatomyositis: peri-orbital oedema and facial swelling.

Juvenile dermatomyositis (JDM) is an auto-immune disease characterised by muscle weakness and typical skin findings. Although peri-orbital oedema and facial swelling are compatible cutaneous findings in JDM, they are extremely rare. A 7-year-old boy who presented with peri-orbital oedema and facial swelling without muscle weakness is reported. In addition, he had cholestasis and marked cytopenia, which are uncommon in JDM, and malignancy and metabolic disorders were primarily considered in the aetiology. He had no musculoskeletal complaints other than elevated muscle enzymes on presentation but developed muscle weakness during follow-up, and a muscle biopsy was compatible with inflammatory myopathy. He responded favourably to conventional treatment and there were no physical limitations or skin findings by the 14th month of follow-up. Although patients presenting with typical clinical features are easy to diagnose, atypical skin findings are challenging for the clinician. In the presence of atypical skin and clinical findings in addition to muscle enzyme elevation, JDM should be considered in the differential diagnosis.Abbreviations: AHCE: asymptomatic hyper-CKemia; AST: aspartate aminotransferase; C: complement; CK: creatine kinase; IVIG: intravenous immunoglobulin; IIM: idiopathic inflammatory myopathy; JDM: juvenile dermatomyositis; LDH: lactate dehydrogenase; MAA: myositis-associated antibodies; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council; MRI: magnetic resonance imaging; MSA: myositis-specific antibodies; MTX: methotrexate NXP2: nuclear matrix protein 2; STIR: short tau inversion recovery; US: ultrasound.

The Impact of Menstrual Disorders on Sleep Quality in Adolescents: An Observational Study.

STUDY OBJECTIVE: Premenstrual syndrome (PMS), dysmenorrhea, and abnormal uterine bleeding are frequent gynecological problems in adolescent girls. Studies show that sleep disorders and menstrual cycle irregularities are common conditions and indicate that they may occur together. Studies on the relationship between sleep quality and menstruation are mostly available for late adolescents (17 years and older) and young adult age groups. Our study aims to evaluate this relationship in adolescents aged 12-18. METHODS: A survey study was structured and consisted of 4 sections. The first section includes anthropometric measurements and medical history of the participants; the second section includes "menstruation and menstruation symptoms history"; the third section includes "The Sleep Quality Scale and Sleep Variable Questionnaire"; and the fourth section includes the "Premenstrual Syndrome Assessment Scale" (PMSAS). Our survey was applied to those who visited the adolescent medicine outpatient clinic. RESULTS: The Sleep Quality Scale score was significantly lower in those with high PMSAS scores (P < .001). The participants who had dysmenorrhea and experienced other symptoms during menstruation had significantly lower SQS scores and sleep efficiency (SE) (P < .001). There was no significant difference between heavy menstrual bleeding, defined as lasting more than 7 days, requiring more than 5-6 pads/tampons per day, and SE/quality (P > .05). CONCLUSION: According to our study, dysmenorrhea and the presence of PMS may negatively affect the sleep quality of adolescents. Health professionals dealing with young people should take into account the effects of menstrual problems on sleep quality and offer appropriate support/treatment options.

Sounds unrealistic: an adolescent girl with anorexia nervosa consumes 19 L of fluid in a few hours: what happens to the physiology?

BACKGROUND: Adolescents with eating disorders (EDs) may present not only with abnormal eating behaviors but also with abnormal drinking behaviors varying widely. These behaviors include water loading to cheat on weight measurements, to feel full and suppress appetite and/or to induce vomiting; as well as restricting fluid intake in addition to food. METHOD: We present a 16-year-old female adolescent with anorexia nervosa restrictive type and major depressive disorder who was hospitalized due to acute food refusal and developed generalized seizures due to dilutional hyponatremia in consequence of consuming excessive amount of water. Psychiatric diagnoses were made according to 'The Diagnostic and Statistical Manual of Mental Disorders' (5th ed.; DSM-5) criteria. RESULTS: After starting nutritional rehabilitation with a low calorie meal plan to avoid refeeding syndrome, a weight gain of 2 kg was noted in the second day of hospitalization. At the bedside visit, she was observed in a disoriented manner and consecutively in seconds, lost consciousness with a generalized tonic-clonic seizure lasting 2 min. Her serum sodium level was measured as 116 mEq/L, which was normal at the time of admission. It was later learned that she secretly ingested 19 L of water in a short amount of time. She regained consciousness and no further seizures were observed after intravenous sodium deficit correction and fluid restriction therapy. Her serum sodium level was normalized (137 mEq/L) within 12 h. CONCLUSION: A thorough clinical assessment of hydration and drinking behaviors as well as eating behaviors is essential for patients with EDs to avoid serious medical complications with high mortality and morbidity during follow-up. It is interesting that this amount of fluid consumption in such a short period of time did not present to the clinic with vomiting, gastric dilatation or bowel irrigation symptoms in a case with acute food refusal and restriction for a year, instead absorbed very quickly causing acute and severe symptomatic hyponatremia with generalized seizures.

Perfil de mortalidad en una Unidad de Cuidados Intensivos Pediátricos: asociación entre la mortalidad y el período fuera del horario diurno semanal / Six year mortality profile of a Pediatric Intensive Care Unit: associaton between out-of-hours and mortality

Introducción. El personal capacitado y los avances tecnológicos mejoraron las unidades de cuidados intensivos pediátricos (UCIP); por ende, mejoraron la atención, la supervivencia y el pronóstico. Pero la calidad de la atención varía según la disponibilidad de recursos humanos y técnicos. Objetivo. Determinar la correlación de las tasas de mortalidad dentro y fuera del horario diurno en días de semana. .Métodos. Se definió horario de días de semana de 8:00 a. m. a 5:00 p. m., el período fuera del horario fueron de 5:00 p. m. a 8:00 a. m., fines de semana y feriados, con un residente en la UCIP y un médico de planta de guardia pasiva. Se clasificaron las causas de mortalidad en la UCIP. Resultados. Se hospitalizaron 2187 pacientes en la UCIP. Fallecieron 394; 151 niñas (38,3 %) y 243 varones (61,7 %). Según el horario de muerte, la mayoría ocurrió fuera del horario diurno 244 (61,9 %) versus a 150 (38,1 %) durante el turno diurno, una diferencia estadísticamente significativa (p < 0,05). La edad, el sexo y la duración de la hospitalización en la UCIP no fueron significativos (p > 0,05) al comparar el período dentro y fuera del horario diurno. Se evaluaron las afecciones que acompañaron la muerte. La relación entre el horario de médicos de planta y la muerte fue significativa (p < 0,05). Hubo más afecciones asociadas a mortalidad fuera del horario diurno . Conclusión. El período fuera del horario diurno sin médicos de planta estuvo asociado con mayor mortalidad.

Introduction.Recent improvements in pediatric intensive care units (PICUs) were achieved through trained personnel and better technology, leading to an increase in patient care, survival rates and good prognosis. Nevertheless the quality of care varies according to the availability of human and technical resources. Objective. The aim was to determine the correlation of mortality rates with daytime shifts compared to other shift periods (off-hours). Methods. Work hours were defined as week days between 8:00 a.m. and 5:00 p.m., with in-house attendance of senior staff, and off-hours as week days between 5:00 p.m. and 8:00 a.m., weekends and public holidays, with one resident covering the PICU and senior staff directly available only on-call. Mortality causes in children hospitalized in the intensive care unit were classified. Results. During this period, a total of 2,187 patients were hospitalized in the PICU. A total of 394 patients died; 151 were girls (38.3 %) and 243 boys (61.7 %). Evaluating time of mortality showed that death occurred mostly at out-off hours: 244 (61.9 %) vs. 150 (38.1 %) during the daytime shift, which was statistically significant (p < 0.05). In addition, age, gender and the length of stay in the PICU were not significant (p > 0.05) when daytime was compared to outoff hours. The conditions which accompanied death were evaluated, the relationship between working hours and death was also significant (p < 0.05). More conditions related to mortality were encountered at off-hours.

Six year mortality profile of a Pediatric Intensive Care Unit: associaton between out-of-hours and mortality. / Perfil de mortalidad en una Unidad de Cuidados Intensivos Pediátricos: asociación entre la mortalidad y el período fuera del horario diurno semanal.

INTRODUCTION: Recent improvements in pediatric intensive care units (PICUs) were achieved through trained personnel and better technology, leading to an increase in patient care, survival rates and good prognosis. Nevertheless the quality of care varies according to the availability of human and technical resources. OBJECTIVE: The aim was to determine the correlation of mortality rates with daytime shifts compared to other shift periods (off-hours). METHODS: Work hours were defined as week days between 8:00 a.m. and 5:00 p.m., with in-house attendance of senior staff, and off-hours as week days between 5:00 p.m. and 8:00 a.m., weekends and public holidays, with one resident covering the PICU and senior staff directly available only on-call. Mortality causes in children hospitalized in the intensive care unit were classified. RESULTS: During this period, a total of 2,187 patients were hospitalized in the PICU. A total of 394 patients died; 151 were girls (38.3 %) and 243 boys (61.7 %). Evaluating time of mortality showed that death occurred mostly at out-off hours: 244 (61.9 %) vs. 150 (38.1 %) during the daytime shift, which was statistically significant (p < 0.05). In addition, age, gender and the length of stay in the PICU were not significant (p > 0.05) when daytime was compared to out-off hours. The conditions which accompanied death were evaluated, the relationship between working hours and death was also significant (p < 0.05). More conditions related to mortality were encountered at off-hours. CONCLUSION: Off-hours without 24 hour attendance of senior staff, was associated with higher mortality.

Introducción. El personal capacitado y los avances tecnológicos mejoraron las unidades de cuidados intensivos pediátricos (UCIP); por ende, mejoraron la atención, la supervivencia y el pronóstico. Pero la calidad de la atención varía según la disponibilidad de recursos humanos y técnicos. Objetivo. Determinar la correlación de las tasas de mortalidad dentro y fuera del horario diurno en días de semana. Métodos. Se definió horario de días de semana de 8:00 a. m. a 5:00 p. m., el período fuera del horario fueron de 5:00 p. m. a 8:00 a. m., fines de semana y feriados, con un residente en la UCIP y un médico de planta de guardia pasiva. Se clasificaron las causas de mortalidad en la UCIP. Resultados. Se hospitalizaron 2187 pacientes en la UCIP. Fallecieron 394; 151 niñas (38,3 %) y 243 varones (61,7 %). Según el horario de muerte, la mayoría ocurrió fuera del horario diurno 244 (61,9 %) versus a 150 (38,1 %) durante el turno diurno, una diferencia estadísticamente significativa (p < 0,05). La edad, el sexo y la duración de la hospitalización en la UCIP no fueron significativos (p > 0,05) al comparar el período dentro y fuera del horario diurno. Se evaluaron las afecciones que acompañaron la muerte. La relación entre el horario de médicos de planta y la muerte fue significativa (p < 0,05). Hubo más afecciones asociadas a mortalidad fuera del horario diurno. Conclusión. El período fuera del horario diurno sin médicos de planta estuvo asociado con mayor mortalidad.

Evaluation of Syrian refugees who received inpatient treatment in a tertiary pediatric hospital in Turkey between January 2016 and August 2017.

Background: Turkey is the leading country among those that accept Syrian refugees. This study aimed to determine the density of Syrian refugees who received inpatient treatment at Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital between January 2016 and August 2017. Methods: Syrian refugees' gender, age, place of birth, duration of hospitalization, admission diagnosis and services required as an inpatient were recorded from the registration system. Admission diagnoses were grouped according to the 'International Statistical Classification of Diseases and Related Health Problems'. Results: A total of 623 Syrian patients were included in this research. The median age of inpatients was 25.1 months; 58% (362/623) were male and 41% (257/623) were born in Turkey. The mean duration of hospitalization was 3 d. The highest number of admissions was observed in January. Pediatric emergency (17.7%) and pediatric surgery (12%) departments were the services with the highest number of admissions in this hospital. The most common admission reasons were found to be respiratory tract diseases, acute gastroenteritis and burns. Conclusions: Syrian refugees received inpatient treatment in services with different diagnoses. Preventive health measures targeting Syrian refugees may provide treatment at an earlier stage and reduce the rate of hospitalization.

Association of Van Wyk Grumbach and Debre Semelaigne Syndromes with Severe Hypothyroidism.

BACKGROUND: Van Wyk Grumbach syndrome (VWGS) and Kocher-Debre Semelaigne syndrome (KDSS) are rare forms of pseudo-precocious puberty and myopathy in patients with longstanding untreated hypothyroidism. We present the case of an adolescent girl who developed pseudo-precocious puberty and myopathy caused by long-term untreated hypothyroidism. CASE: A 17-year-old female patient was referred to our outpatient clinic due to menstrual irregularities. She had muscle pain and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. High levels of creatinine and creatinine kinase and accompanying muscle hypertrophy were present. After the initiation of L-thyroxine therapy, the symptoms were alleviated in a short time. CONCLUSION: Congenital and acquired hypothyroidism should be considered in the differential diagnosis of pseudo-precocious puberty and myopathy that presents with muscle pain, muscle hypertrophy, and elevated creatinine kinase levels.