Sat-316 Health Related Quality of Life of Children With Nephrotic Syndrome in Lagos Nigeria

Abstract

Accumulation of ammonia in the blood may result in an acute life-threatening event in children. Patients typically present with lethargy, poor feeding, and hypotonia; if not managed efficiently, patients can develop seizures, coma, and eventually die. Management of hyperammonemia is difficult in children given the non-specific symptoms, age specific etiologies, and lack of consensus in the treatment plan. We systematically reviewed published literature to provide expert consensus panel recommendations for medical management and renal replacement therapy (RRT) in pediatric patients with hyperammonemia. Methods: PubMed/Medline, Embase and Cochrane database search was performed to include studies about hyperammonemia and RRT in children <18 years old. Two independent reviewers reviewed each title, abstract, and relevant full text articles. An expert panel of international pediatric nephrologists discussed medical management and RRT for hyperammonemia in children at a consensus conference to provide recommendations. Results: The initial search returned a total of 477 citations and 25 studies met the inclusion criteria. A total of 132 patients were included in these 25 studies and were treated with different dialysis modalities. Twenty-three heperammonemia patients were treated with peritoneal dialysis with 65% success rate, five were treated with intermittent hemodialysis with 100% success rate, 92 were treated with continuous renal replacement therapy (CRRT) with 60% success rate and three were treated by extracorporeal membrane oxygenation (ECMO) combined with CRRT and had 100% success rate. Conclusions: Expert panel recommendations were provided with regards to non-RRT (medical management), hemodialysis, peritoneal dialysis, CRRT, high dose CRRT, and hybrid therapy. CRRT was the first line dialysis modality of choice recommended for hyperammonemia management in children. Indications for RRT were variable among studies reviewed; RRT was recommended at ammonia blood level > 400mmol/L or in hemodynamically unstable children irrespective of blood ammonia level. More studies are needed to further strengthen these expert recommendations.

Reliability of generic quality-of-life instruments in assessing health-related quality of life among children and adolescents with idiopathic nephrotic syndrome: a systematic review

“…The ten selected studies consist of case–control studies (n = 5) [ 10 , 17 , 21 – 23 ], longitudinal cohort studies (n = 2) [ 24 , 25 ], and cross-sectional studies (n = 3) [ 15 , 16 , 26 ]. The countries of study were distributed among four continents as follows: North America [ 24 , 25 ], Europe [ 16 , 21 ], Asia [ 15 , 17 , 22 , 23 ], and Africa [ 10 , 26 ]. All the studies were hospital-based.…”

“…In five studies that reported sex distribution [ 15 , 17 , 22 , 24 , 26 ], the total number of males and females was 204 and 122, respectively, with an approximate ratio of 1.7: 1 (Table 1 ). Study methodological quality, using the NOS, shows a star-rating of < 7 (low quality) for three studies [ 15 , 16 , 26 ] and ≥ 7 (high quality) for seven studies [ 17 , 21 – 25 ]. (N/B: Rating indicates the range of quality [based on the adopted criteria from NOS] and does not translate to unreliable study findings for studies rated as low quality.…”

“…The main findings were the significantly worse patient-reported outcomes measurement-information systems (PROMIS) scores in ‘prevalent’ than in ‘incident’ nephrotic syndrome for ‘pain interference’ and ‘peer relationships’ domains, and the significantly worse PedsQL scores in ‘prevalent’ than ‘incident’ nephrotic syndrome for ‘social functioning’ and ‘school functioning’ domains. On the other hand, Troost et al evaluated HRQoL profiles in children and adult cohorts with nephrotic syndrome to improve the interpretability and clinical utility of PROMIS [ 25 ]. In the PROMIS II cohort, 56 patients with ‘incident’ INS and 65 patients with ‘prevalent’ INS were involved.…”

“…In the ten reviewed studies, the Pediatric Quality of Life Inventory (PedsQL™ 4.0 Generic Core Scales) was the most frequently employed generic QoL instrument for assessing QoL in children with INS; seven studies used it either alone [ 17 , 22 , 23 , 26 ], or in combination with other instruments such as Pediatric Quality of Life Questionnaire for nephrotic syndrome [ 15 ], PROMIS instrument [ 24 ], and Generic Children's QoL Measure (GCQ) [ 10 ]. The remaining three studies used the following instruments alone in evaluating the HRQoL of the patients: Netherlands Organization for Applied Scientific Research-Academical Medical Center (TNO-AZL) Child Quality of Life Questionnaire, Child Behavior checklist, and Teacher Report form [ 21 ]; 30-item standardized questionnaire with a global score of 0–100 [ 17 ]; and PROMIS instrument [ 25 ]. The PedsQL™ 4.0 Generic Core Scales was used to evaluate child-rated HRQoL in six studies [ 10 , 16 , 17 , 22 – 24 ], and both child- and parent-rated HRQoL in one study [ 26 ].…”

Assessment of health-related quality of life in Sudanese children with nephrotic syndrome: a questionnaire-based study

“…There is currently no disease specific HRQOL survey for children with NS. PedsQL™-4 GCS has been used on NS children from diverse populations [ 5 , 17 , 23 , 24 ], however, its trustworthiness to assess QOL in NS children was lately evaluated in a systematic review involving 10 studies which concluded that the uniformity of the assumptions across the assessed studies accentuates the reliability of non-specific QOL instruments in valuing HRQOL in those children [ 25 ]. Recently a trial to create an NS-specific QOL tool was published in 2019, assessed by 11 experts, and applied to 100 NS children [ 26 ], however, this tool is not published or authorized yet.…”