Administration and Toxicity Profile of the Capizzi Interim Maintenance—Retrospective Study from a Tertiary Care Cancer Centre

Introduction The Capizzi-style methotrexate (MTX) is an integral part of acute lymphoblastic leukemia (ALL) treatment. The escalating dose of MTX originally used in the United Kingdom and Children’s Oncology Group protocols along with L-asparaginase has been modified in the Indian Childhood Collaborative Leukemia (ICiCLe) group protocol where L-asparaginase has been omitted. The data regarding the incidence of toxicities and ease of administration on the Capizzi-style interim maintenance is not robust. Objectives We have compiled our experience with administration and toxicity profile in children with intermediate-risk ALL. Materials and Methods A retrospective data collection of all children diagnosed with intermediate-risk ALL as per the ICiCLe risk stratification in the year 2019 was included in the analysis. Each cycle of MTX was started after ensuring an absolute neutrophil count of >750/mm3 and transaminases <2 upper limit of normal. As a unit protocol, pre- and post-MTX hydration was administered in all our children. No urine pH or midcycle biochemical parameter monitoring was done. Statistical analysis was done using Microsoft Excel and SPSS version 24 IBM Corp. in Armonk, New York, United States. Results Forty-six children were included in the study. The median age of children in our study was 6 years (range: 1 year 2 months–12 years). Undernutrition was associated with a significant increase in toxicity (p = 0.02). Fifty-two percent of children had evidence of toxicity, elevated transaminases being the most common. There were recurring symptoms resulting in 53 episodes of toxicities overall. Incidence of toxicity was more in the early cycles (<3). Conclusion The pre- and post-MTX hydration is an effective way to reduce toxicities with the Capizzi-style MTX and this course can be administered with ease on outpatient basis with minimal need for monitoring or admission.

A219 EARLY PREDICTORS OF UNFAVORABLE OUTCOME IN NORTH AMERICAN INDIAN CHILDHOOD CIRRHOSIS

Background North American Indian Childhood Cirrhosis (NAIC) has only been described in the Cree-Ojibway First Nations of Northern Quebec. NAIC presents with transient neonatal jaundice and progresses to biliary cirrhosis often requiring liver transplantation (LT) in childhood. Only 30 patients have been described to date and risk factors associated with an earlier progression to LT have not yet been identified. Moreover, NAIC patients seem to experience more fractures than other cholestatic patients, but this has not been confirmed. Aims With this study, we aimed to identify predictors at 6 months from presentation that might suggest progression to end-stage liver disease as well as evaluate bone health in affected patients. Methods The records of all NAIC patients diagnosed between 2000–2020 were reviewed. Subjects were split into 2 groups based on whether they had undergone LT or not (No_LT) before age 18. Recorded complications included: hepatic encephalopathy (HE), variceal bleeding (VB), ascites, spontaneous bacterial peritonitis (SBP), bacteremia, and pulmonary shunts. Laboratory data (alanine aminotransferase, ALT; total bilirubin, TB) were collected at presentation and follow-up. Bone mineral density (BMD) of the lumbar spine (Z-scores) and number of fractures were compared between groups. NAIC patients were then compared to cohorts with other chronic cholestatic diseases such as biliary atresia (BA, n=24) and Alagille syndrome (AS, n=11). Results A total of 14 patients (M=9, F=5) were diagnosed with NAIC. Average age at presentation was 2.1 months (IQR 1–16.9 months), with 3 patients older than 18 months. Overall, 6 patients were transplanted (avg 8.6±1.7 years), one was listed for LT but died waiting, and 7 remained in a state of compensated cirrhosis. All complications were only observed in the LT group except for VB which also occurred in 2 patients of the No_LT group. Between presentation and 6 months, ALT and TB levels increased more in the LT vs No_LT group (p=ns). There was a greater variation of ALT/TB levels in the LT group (p=0.0047) even once the 3 patients with late referral were excluded (p=0.0381). No patient in the No_LT group had fractures, while 3 did in the LT group. BMD was lower in the LT group vs No_LT group (-2.2±1.2 vs. -1.1±1.3, p=ns). NAIC patients had lower BMD (-1.7±1.3) than those with AS (0.7±0.9, p=0.003) or BA (-0.9±1.4, p=ns) and had a higher prevalence of fractures (21.4% vs. 12.5% for BA and 18.2% for AS patients). Conclusions In patients with NAIC, variation of ALT/TB levels at 6 months from presentation may be used as an early predictor of unfavorable outcome and progression towards end-stage liver disease. Patients who evolved to LT had more complications, higher prevalence of fractures and lower BMD values. Compared to children with BA or AS, NAIC patients had poorer bone health. Funding Agencies None

“Like Bananas with Brown Spots”

Employing an analytical autoethnographic methodology, this paper examines how the polysemic meanings and punctuated character of epilepsy produces social and corporeal vulnerabilities in an Indian childhood. The paper further establishes the importance of the family in influencing individual perceptions and constructions of chronic illness as well as in building resilience or increasing vulnerabilities. In examining the process of research, this paper also makes an argument that disabled researchers in the field can become vulnerable in multivalent ways but also argues that the act of disclosure of epileptic/disabled identities during the research process can become central to building community and resilience. This paper also complicates the often North-centric narrative of disability studies and underlines the importance of social contexts around individual categories of disability or chronic illness.

GENDER EQUALITY AND WOMEN’S POWER IN AMERICAN INDIAN TRADITIONAL CULTURE IN ZITKALA-SA'S SHORT STORIES

This article intends to reveal gender equality and women’s power in American Indian traditional culture before they were assimilated in white American society in the late nineteenth century as reflected in Zitkala-Sa’s short stories, Impressions of an Indian Childhood, The Soft Hearted Sioux and A Warrior Daughter. This study is a qualitative research that applies feminist literary approach which uses liberal feminist theory for the data analysis. This study shows that American Indian women enjoyed gender equality in traditional American Indian culture that had complementary gender relation. American Indian women had power and equal rights to be warriors and leaders in their society.Keywords: gender role, American Indian women, complimentary relation.KESETARAAN GENDER DAN KUASA PEREMPUAN DALAM BUDAYA TRADISIONAL INDIAN AMERIKA DALAM CERITA-CERITA PENDEK ZITKALA-SA AbstrakArtikel ini bertujuan untuk mengungkapkan kesetaraan gender dan kuasa perempuan Indian Amerika dalam budaya tradisional masyarakat Indian Amerika sebelum mereka mengalami program asimilasi yang bertujuan untuk menyatukan masyarakat Indian Amerika dengan masyarakat kulit putih di akhir abad ke-19 seperti yang terefleksi dalam cerita pendek Zitkala-Sa, Impressions of an Indian Childhood,The Soft Hearted Sioux dan A Warrior Daughter. Artikel ini ditulis dengan mengunakan metode kualitatif dan menerapkan pendekatan kajian feminisme sastra dengan teori feminisme liberal. Hasil dari analisa ini menunjukkan bahwa perempuan Indian Amerika telah menikmati kesetaraan gender dalam budaya tradisional Indian Amerika yang memiliki relasi gender yang saling melengkapi dan mereka memiliki kuasa dan hak yang setara dengan laki laki Indian Amerika untuk menjadi prajurit dan pemimpin dalam masyarakat Indian Amerika.Kata kunci: peran gender, Perempuan Indian Amerika, relasi setara

Differenzialdiagnose des Morbus Wilson

ZusammenfassungNach dem Stellen der Verdachtsdiagnose Morbus Wilson gilt es, diese zu bestätigen. Bis zum eindeutigen Nachweis bzw. bei Ausschluss einer autosomal-rezessiven Störung des hepatischen Kupfertransporters ATP 7B sind Differenzialdiagnosen anhand der vorliegenden Befunde zu prüfen. Laborchemische Parameter des Kupferstoffwechsels weisen außer auf nicht relevante Normabweichungen auch auf andere Störungen im Kupferstoffwechsel hin.Neben bekannten Erkrankungen wie den Morbus Menkes, das Occipital Horn Syndrom (OHS), die Indian childhood cirrhosis (ICC) und den Coeruloplasminmangel werden neu entdeckte Störungen berücksichtigt. Dazu gehören das MEDNIK-Syndrom, das Huppke-Brendel-Syndrom und der Chaperonmangel CCS. Einen weiteren Schwerpunkt stellen alterskorrelierte Differenzialdiagnosen des kindlichen Ikterus und der Anämie sowie extrapyramidalmotorischer Bewegungsstörun-gen dar. Der Kayser-Fleischer-Kornealring wird als klassische ophthalmologische Manifestation relativiert. Mit der neu beschriebenen Manganspeichererkrankung besteht eine weitere seltene Metallstoffwechselstörung, die eine dem Morbus Wilson ähnliche Symptomatik hat.Wie diese Übersicht zeigt, ordnet sich der Morbus Wilson in ein weites Spektrum internistischer und neurologischer Krankheitsbilder mit Ikterus, Anämie und EPS ein. Auch neu entdeckte Krankheitsbilder des Mangan- und Kupferstoffwechsels sind relevant.