Preference of the patient's family for family-centred care in a paediatric hospital.

Objective: To explore the dimensions of family-centred care preferred by families of paediatric inpatients in a public healthcare setting. METHODS: The qualitative study was conducted at the National Institute of Child Health, Karachi, from October 2021 to August 2022, and comprised family members of the hospitalised children. Data was collected through three focus group discussions that were guided by a semi-structured questionnaire. Using the five-step Fereday and Muir-Cochrane guidelines, data was coded and subjected to thematic analysis. RESULTS: Of the 21 subjects, 13(62%) were males and 8(38%) were females. The overall mean age was 32.24±7.58 years (range: 18-50 years). In terms of relationship with the patient, 9(43%) were fathers and 6(28.6%) were mothers. Each focus group discussion had 7(33.3%) subjects. Thematic analysis showed that the participants perceived family-centred care positively. Eight categories emerged depicting family perception and experience of family-centred care in a tertiary-care setting. Conclusion: The participants perceived family-centred care respectful and empathic towards patients' families, making them integral care team members.

Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population.

Intraocular medulloepithelioma is a rare, congenital tumour of the non-pigmented ciliary epithelium. It most frequently arises from the ciliary body but can also have its origin from the retina, iris and optic nerve. The age when lesion first appears is typically around 2-10 years. Nearly 50-60% of patients having this lesion may also have secondary features such as cataract and neovascular glaucoma. Those with extrascleral medulloepithelioma are at risk for metastasis. Systemic correlation of the tumour with pleuropulmonary blastoma/DICER1 gene is reported in the literature. Here, we report a case of a 15 years old boy with one year history of right eye proptosis and painful red right eye along with decreased vision for one week. He was assessed and operated for cataract elsewhere three years back. The ophthalmology team managed him for endophthalmitis with intravenous antibiotics, followed by 2 sessions of cryotherapy and finally an enucleation of right eye was performed due to severe pain and no vision in the involved eye. His left eye, general physical examination and systemic evaluation were normal. Histopathology revealed the diagnosis of 'malignant teratoid medulloepithelioma'. Therefore, evaluation of systemic associations for DICER1 gene mutations was performed by the oncology team. For high risk feature of scleral invasion on histopathology, he was treated with chemotherapy. Since the tumour is of rare occurrence; an international expert team with vast research experience in PPB/DICER1 associated tumours was also contacted. He was registered in International PPB/DICER1 registry where a detailed central radiology and pathology review was performed. Genetic counseling and surveillance plan was also suggested by the international registry.

A relation between serum albumin level and prognosis of critically ill children admitted to the paediatric Intensive Care Unit.

OBJECTIVE: To determine the frequency of hypoalbuminaemia in critically ill children, and to assess the association of low serum albumin with clinical deterioration and outcome. METHODS: The prospective, descriptive study was conducted from September 1, 2020, to October 31, 2021, at the National Institute of Child Health, Karachi, and comprised critically ill children of either gender aged between 3 months and 16 years admitted to the paediatric intensive care unit. Serum albumin values were documented at 2 hours post-admission and at 24 hours. Paediatric Index of Mortality 2 score, Vasoactive Inotropic Score, and Paediatric Sequential Organ Failure Assessment scores were calculated. Hypoalbuminaemia was defined as serum albumin ≤3.3gdl. Data was analysed using SPSS 27. Results: Of the 110 patients, 70(63.6%) were boys and 40(36.4%) were girls. The overall mean age was 46.72±43.28 months. Hypoalbuminaemia at 24 hours was found in 74(67.3%) subjects compared to 60(54.5%) at 2 hours, and mean serum albumin was lower at 24 hours compared to 2 hours post-admission (p<0.05). Patients with hypoalbuminaemia had significant relation with Paediatric Index of Mortality 2 score, Vasoactive Inotropic Score, Paediatric Sequential Organ Failure Assessment score, and outcome (p<0.05). The risk of mortality was 4.1 times higher in patients with hypoalbuminaemia (p=0.001). CONCLUSIONS: The incidence of hypoalbuminaemia was found to be higher in children in intensive care settings, and hypoalbuminaemia was a significant independent predictor of mortality in a critically ill child.

Effectiveness And Safety Of Direct Acting Antiviral Agents In Thalassaemic Patients With Chronic Hepatitis C.

BACKGROUND: Pakistan has the highest prevalence of Β-Thalassemia major in children and Chronic Hepatitis C (HCV) infection is a common transfusion transmitted infection. After the emergence of new generations of Antiviral drugs labelled as Direct Acting Antivirals (DAAs), substantial eradication of HCV has been reported as 90-95% with fewer side effects as compared to older regimen of Peginterferon with or without Ribavirin. The main objective of this study was to assess the Rapid virological response (RVR) at 4th week, End of treatment response (ETR) at 12th week and sustained viral response (SVR) at 24th week achieved by using direct acting antiviral and to assess their safety. Methods: Retrospective descriptive study was conducted from July 2018 to July 2020 at National Institute of Child Health. All ß-thalassemia major paediatric patients with HCV infection and age between 3-14 years were included. Demographic data, liver function test, HCV PCR, and response of antiviral therapy was recorded and analyzed. Safety was determined by adverse effects reported in records and efficacy was documented by clearance of HCV-RNA to see ETR and SVR. RESULTS: Total 21 patients were treated. Mean age was 7.67±3yr and 12 (57%) were male. Mean weight was 19.3±3.2kg. RVR and ETR was achieved in all (100%) and SVR was achieved in 20/21 (95%) patients. Headache in 2(9.5%) and generalized body ache was found in 1 (4.25%) patient. Conclusion: Combined Sofosbuvir and Daclatasvir were found to be effective and safe for treating HCV in Thalassaemia Major Children.