Inborn errors of metabolism in palliative care.

BACKGROUND: In parallel with the increase in the number of children with life-limiting or life-threatening diseases, the need for and right to pediatric palliative care is also increasing. This study aimed to examine the metabolic diseases, evaluate the symptoms, and review the needs of children who were admitted to the pediatric palliative care service. METHODS: Following the approval of the local ethics committee (18/7/2019-107), the computer records of patients hospitalized in the pediatric palliative care service between December 1, 2018 and January 6, 2019 were reviewed retrospectively. Dr Behçet Uz Children's Hospital, Pediatric Palliative Care Center is third center in Turkey. Our pediatric palliative care center has one year of experience. RESULTS: In the study period, there were 101 patients who were hospitalized in the pediatric palliative care service. Eighteen patients (19.7%) diagnosed with inborn errors of metabolism were included in the study. The mean age was 3.2 ± 2.1 (0-8) years. Although it could not be shown statistically, the duration of hospitalization of patients with congenital malformation was long. CONCLUSION: Pediatric palliative care provides the best care with the control of various symptoms in neurodegenerative congenital metabolic diseases that do not have treatment or treatment, but progressive symptoms cannot be prevented.

Thoraco-abdominal duplication cyst of the jejunum presenting as respiratory distress in a young infant-a case report.

Thoraco-abdominal duplication cysts are rare congenital anomalies that can accompany vertebral and spinal cord pathologies and occur most frequently in the small intestines. Symptoms such as respiratory distress, dyspnea, tachypnea, cough, hemoptysis, cyanosis, vomiting and dysphagia may develop depending on the location. The cyst has several clinical and radiological dilemmas. We present a rare case of thoraco-abdominal duplication cyst in a 3-month-old male patient presenting with respiratory distress in the neonatal period. Thoraco-abdominal duplications require a high index of suspicion and meticulous clinical management. Thus, patients can be successfully managed without any unnecessary interventions, complications and loss of time.

Investigation of care burden and musculoskeletal pain of parents of paediatric palliative care patients in Turkey.

Background: Caring for a paediatric patient is associated with psychological, physical and social challenges. It may be easy to identify the emotional effects of caregiving burden on parents, but the physical effects are often not known. Aims: We aimed to examine the relationship between caregiver burden and musculoskeletal pain in caregiver parents of children accessing palliative care services. Methods: This was a cross-sectional study among caregiving parents of paediatric palliative care patients, conducted between 15 July 2020 and 15 January 2021. The care burden was examined using the Zarit Caregiver Burden Questionnaire, and the physical complaints of parents with the Extended Nordic Musculoskeletal Questionnaire. Results: A total of 69 caregiving parents were included in the study. The median Zarit Caregiver Burden score was 54.0. In parents with pain complaints, the most common pain areas were lower back (62.3%), back (60.9%) and neck (42.0%). Pain was statistically significantly associated with the duration of the disease in the child, especially shoulder pain (P = 0.023) and wrist pain (P = 0.024). Conclusion: Lower back pain was observed at a higher rate among caregiving parents. As the duration of the child's illness increased, the number of caregiver parents with shoulder and wrist pain increased. It is important to extend support to caregivers of paediatric palliative care patients to alleviate their physical difficulties.

Evaluation of Primary Caregiver Parents' Quality of Life in Pediatric Palliative Care with the WHOQOL-Bref (TR).

OBJECTIVE: Pediatric palliative care is a top-level care system that focuses on improving the quality of life of the child and family. Quality of life is an expression of individual well-being based on an individual's assessment of their own life. It includes satisfaction in all areas of life, including physical and mental health, environment, and social areas. METHODS: The study was conducted with the primary caregiver parents of children admitted to the pediatric palliative care service of the Health Science University Izmir Dr. Behçet Uz Child Disease and Surgery Training and Research Hospital. The Turkish version of the World Health Organization Quality of Life WHOQOL-Bref (TR) questionnaire was applied with a personal information form containing demographic data. RESULTS: Eighty-four patients were hospitalized in the specified period, and 67 primary caregiver parents agreed to participate in the study. Total scores evaluated by WHOQOL-Bref (TR); the physical domain was 19.95 ± 3.30, the mental domain was 19.95 ± 3.18, the social domain was 10.11 ± 2.40, and surrounding area was 16.38 ± 2.82. The physical and psychological subgroups' scores were statistically significantly higher in primary caregiver parents with good social support (P < .005). CONCLUSION: It has been determined that in order to increase the quality of life and care of children with life-limiting and/or threatening diseases, the quality of life of primary caregiver parents should be increased, and "social support" procurement, which has the most important effect on the quality of life, is an important need.

Evaluation of aortic stiffness in children with chronic renal failure.

The measurement of aortic stiffness (As) [aortic strain (S), pressure strain elastic modulus (Ep) and pressure strain normalized by diastolic pressure (Ep*)] is suggested as an excellent marker of subclinical arterial sclerosis. We aimed to investigate the presence of As and to determine the relationship between As and some risk factors in children with chronic renal failure (CRF). Twenty-six pre-dialysis (PreD) [female/male (F/M) 7/19] patients and 23 chronic peritoneal dialysis (CPD) (F/M 13/10) patients were assessed. Twenty-nine healthy children were selected as a control group (F/M 14/15). We determined anemia, abnormal calcium/phosphate metabolism, hypertension, diastolic dysfunction, increased left ventricular mass (LVM), hypertriglyceridemia, increased stiffness (Ep, Ep*), and decreased strain (S) in the CRF (PreD and CPD) group compared with the controls (P < 0.05). Presence of renal disease, LVM and usage of angiotensin-converting enzyme inhibitor (ACE-I) in all groups; female gender, duration of disease and the usage of anti-hypertensive drug therapy in CRF patients; and LVM and LVM index in healthy children were found to be independent predictors for aortic stiffness and/or strain. In conclusion, CRF is associated with significant arterial functional abnormalities in uremic children and not controlled by dialysis treatment. These results suggest that, even in young children, uremia has a profound impact on arterial function.