Academic literature on the topic 'Pediatric nephrology'

Publications 1-49 represent studies that I have undertaken myself or conjointly over a 34 year period to investigate a variety of issues relating to diseases of the kidney and urinary tract in children. The studies were carried out at the Royal Alexandra Hospital for Children, Camperdown when I was Clinical Superintendent from 1968 - 1970; The Department of Paediatrics, University of Minnesota, Minneapolis, USA when I was Overseas Research Fellow of the Post Graduate Foundation in Medicine, University of Sydney, 1970 - 1972, then as Staff Physician in Nephrology at the Royal Alexandra Hospital for Children, Camperdown, 1972 - 1977, and then Head of that Department at the Hospital until 1995 and then as an Honorary Staff Specialist at that hospital. Some of the studies were done conjointly with members of the Renal Unit of Royal Prince Alfred Hospital where I hold an Honorary appointment and others conjointly with members of the Renal Unit of Prince Henry Hospital, Little Bay. I was appointed Clinical Associate Professor to the Department of Paediatrics and Child Health, University of Sydney in 1993. In 1966 paediatric nephrology was in the early phase of development as a medical subspecialty. There was no definitive textbook, the first was published in 1975 (Pediatric Nephrology, Ed. Mitchell I. Rubin. Williams and Wilkins.). In the preface to the 2nd edition of Renal Disease (Blackwell) in 1967 the editor D.A.K. Black noted that he had included a chapter on paediatric aspects which had been planned for the 1st edition in 1962 but ”it could not be arranged”. In the chapter on Renal Disease in Children the author, D.Macauly, comments that the mortality rate of acute renal failure in children was 50%. When I joined the resident staff of the Royal Alexandra Hospital for Children in 1966, children with renal disease were managed by general paediatricians. There was no active program for the treatment of children with acute or chronic renal failure. A small number of kidney biopsies had been performed by Dr Trefor Morgan who, together with Dr Denis Wade, had taught me the technique while I was a resident medical officer at the Royal Prince Alfred Hospital in the preceding year. With the guidance and support of Dr S.E.J. Robertson and Dr C. Lee, Honorary Medical Officers, and Dr R.D.K. Reye, Head of the Department of Pathology, I began performing kidney biopsies on children at the request of the paediatrician in charge. In the same year, encouraged again by Doctors Robertson and Lee, and by J.C.M. Friend and J. Brown, I introduced peritoneal dialysis for the treatment of children with acute renal failure, a technique which I had also been taught by Dr Trefor Morgan whilst I was a resident at Royal Prince Alfred Hospital. Dr Robertson encouraged me to present my experience in percutaneous renal biopsy in children at the Annual Meeting of the Australian Paediatric Association in 1968 and this study became the first paper I published in relation to disease of the urinary tract in children (1). In 1970 I was granted an Overseas Research Fellowship by the Post Graduate Foundation in Medicine, University of Sydney, to enable me to undertake a fellowship in the Department of Paediatrics at the University of Minnesota. I had the great fortune in undertaking studies in the new discipline of paediatric nephrology and related research under the guidance of Dr A. F. Michael, Dr R.L.Vernier and Dr A. Fish. I acquired the techniques of immunopathology and electron microscopy. On my return to Australia I established a Department of Nephrology at the Royal Alexandra Hospital for Children. I introduced immunofluorescent and electron microscopic studies for the kidney biopsies that I continued to perform and, with the support of Dr R.D.K. Reye, I provided the official reports of these studies until 1990. As a result these studies became part of the histopathologic service provided by the hospital. I continue to be consulted concerning the interpretation of some electron microscopic findings in renal tissue. With the assistance of Dr J.D. Harley I set up a laboratory in the Children’s Medical Research Foundation to continue and expand the studies I had commenced during my Fellowship. Establishing a dialysis and transplant program for children with end stage renal disease (ESRD) was extremely time consuming. At that time most children with ESRD died. The program was initially established jointly with the Renal Unit at Royal Prince Alfred Hospital in 1972 and eventually dialysis facilities were established at the Children’s Hospital using predominantly peritoneal dialysis. By 1978 the existence of the Unit was well known in the general community and articles appeared in the press. One prompted the late Sir Lorimer Dods, the first Professor of Paediatrics in Australia to write to me congratulating me on what I had achieved. He remarked “I have just read with special interest Shaun’s review in the SMH of some of your recent achievements in the field of renal failure in infancy and childhood and want to offer you my personal congratulations on all that you have achieved and are achieving in this area of paediatrics which, in my little world of yesterday, meant nothing more than progressive and unrelenting fatal illness”. Taking part in the development of a relatively new discipline led me to study a number of areas. I encouraged trainees to write reports concerning clinical observations and eventually I was joined by Fellows whom I encouraged and supported to study a number of different areas to ensure that children were being cared for in an environment of strong and open enquiry. This led to studies on investigations of chronic renal failure which Dr Elisabeth Hodson pursued and studies on urinary tract infection in small children for which Dr Jonathon Craig was awarded a PhD. As I had been a contributor and co-author in a number of these studies they have been included in my list of publications. As a result of this diversity I have listed the publications in 9 sections. The overall theme is to study diseases of the renal tract in children and treatments used to understand the processes and ensure the most effective treatment. Some published abstracts of papers presented at scientific meetings have been included to clarify invitations I received to prepare reviews and chapters on various subjects and my involvement in some conjoint studies. I was author or coauthor of several book chapters, reviews, editorials and certain published studies to which I was invited to contribute as a result of my primary studies and these I have included as “Derivative References”numbered 50-76.

Doctor of Medicine
Publications 1-49 represent studies that I have undertaken myself or conjointly over a 34 year period to investigate a variety of issues relating to diseases of the kidney and urinary tract in children. The studies were carried out at the Royal Alexandra Hospital for Children, Camperdown when I was Clinical Superintendent from 1968 - 1970; The Department of Paediatrics, University of Minnesota, Minneapolis, USA when I was Overseas Research Fellow of the Post Graduate Foundation in Medicine, University of Sydney, 1970 - 1972, then as Staff Physician in Nephrology at the Royal Alexandra Hospital for Children, Camperdown, 1972 - 1977, and then Head of that Department at the Hospital until 1995 and then as an Honorary Staff Specialist at that hospital. Some of the studies were done conjointly with members of the Renal Unit of Royal Prince Alfred Hospital where I hold an Honorary appointment and others conjointly with members of the Renal Unit of Prince Henry Hospital, Little Bay. I was appointed Clinical Associate Professor to the Department of Paediatrics and Child Health, University of Sydney in 1993. In 1966 paediatric nephrology was in the early phase of development as a medical subspecialty. There was no definitive textbook, the first was published in 1975 (Pediatric Nephrology, Ed. Mitchell I. Rubin. Williams and Wilkins.). In the preface to the 2nd edition of Renal Disease (Blackwell) in 1967 the editor D.A.K. Black noted that he had included a chapter on paediatric aspects which had been planned for the 1st edition in 1962 but ”it could not be arranged”. In the chapter on Renal Disease in Children the author, D.Macauly, comments that the mortality rate of acute renal failure in children was 50%. When I joined the resident staff of the Royal Alexandra Hospital for Children in 1966, children with renal disease were managed by general paediatricians. There was no active program for the treatment of children with acute or chronic renal failure. A small number of kidney biopsies had been performed by Dr Trefor Morgan who, together with Dr Denis Wade, had taught me the technique while I was a resident medical officer at the Royal Prince Alfred Hospital in the preceding year. With the guidance and support of Dr S.E.J. Robertson and Dr C. Lee, Honorary Medical Officers, and Dr R.D.K. Reye, Head of the Department of Pathology, I began performing kidney biopsies on children at the request of the paediatrician in charge. In the same year, encouraged again by Doctors Robertson and Lee, and by J.C.M. Friend and J. Brown, I introduced peritoneal dialysis for the treatment of children with acute renal failure, a technique which I had also been taught by Dr Trefor Morgan whilst I was a resident at Royal Prince Alfred Hospital. Dr Robertson encouraged me to present my experience in percutaneous renal biopsy in children at the Annual Meeting of the Australian Paediatric Association in 1968 and this study became the first paper I published in relation to disease of the urinary tract in children (1). In 1970 I was granted an Overseas Research Fellowship by the Post Graduate Foundation in Medicine, University of Sydney, to enable me to undertake a fellowship in the Department of Paediatrics at the University of Minnesota. I had the great fortune in undertaking studies in the new discipline of paediatric nephrology and related research under the guidance of Dr A. F. Michael, Dr R.L.Vernier and Dr A. Fish. I acquired the techniques of immunopathology and electron microscopy. On my return to Australia I established a Department of Nephrology at the Royal Alexandra Hospital for Children. I introduced immunofluorescent and electron microscopic studies for the kidney biopsies that I continued to perform and, with the support of Dr R.D.K. Reye, I provided the official reports of these studies until 1990. As a result these studies became part of the histopathologic service provided by the hospital. I continue to be consulted concerning the interpretation of some electron microscopic findings in renal tissue. With the assistance of Dr J.D. Harley I set up a laboratory in the Children’s Medical Research Foundation to continue and expand the studies I had commenced during my Fellowship. Establishing a dialysis and transplant program for children with end stage renal disease (ESRD) was extremely time consuming. At that time most children with ESRD died. The program was initially established jointly with the Renal Unit at Royal Prince Alfred Hospital in 1972 and eventually dialysis facilities were established at the Children’s Hospital using predominantly peritoneal dialysis. By 1978 the existence of the Unit was well known in the general community and articles appeared in the press. One prompted the late Sir Lorimer Dods, the first Professor of Paediatrics in Australia to write to me congratulating me on what I had achieved. He remarked “I have just read with special interest Shaun’s review in the SMH of some of your recent achievements in the field of renal failure in infancy and childhood and want to offer you my personal congratulations on all that you have achieved and are achieving in this area of paediatrics which, in my little world of yesterday, meant nothing more than progressive and unrelenting fatal illness”. Taking part in the development of a relatively new discipline led me to study a number of areas. I encouraged trainees to write reports concerning clinical observations and eventually I was joined by Fellows whom I encouraged and supported to study a number of different areas to ensure that children were being cared for in an environment of strong and open enquiry. This led to studies on investigations of chronic renal failure which Dr Elisabeth Hodson pursued and studies on urinary tract infection in small children for which Dr Jonathon Craig was awarded a PhD. As I had been a contributor and co-author in a number of these studies they have been included in my list of publications. As a result of this diversity I have listed the publications in 9 sections. The overall theme is to study diseases of the renal tract in children and treatments used to understand the processes and ensure the most effective treatment. Some published abstracts of papers presented at scientific meetings have been included to clarify invitations I received to prepare reviews and chapters on various subjects and my involvement in some conjoint studies. I was author or coauthor of several book chapters, reviews, editorials and certain published studies to which I was invited to contribute as a result of my primary studies and these I have included as “Derivative References”numbered 50-76.

O objetivo deste estudo é compreender como as crianças com Síndrome Nefrótica estão sendo cuidadas, buscando identificar novos elementos contextuais e horizontes para reconstruir o cuidado de enfermagem, tendo como foco a família. Trata-se de um estudo qualitativo com apoio teórico-metodológico na hermenêutica, fundamentada principalmente em Gadamer e Ricouer. Foi realizado com famílias de crianças com Síndrome Nefrótica usuárias do Ambulatório de Nefrologia Pediátrica do Hospital Universitário Júlio Müller da Universidade Federal de Mato Grosso, em Cuiabá. Para a coleta de dados foram usados o histórico de enfermagem da família, a construção do genograma e do ecomapa, a análise de documentos e a entrevista. Seis famílias e quatro membros da equipe do serviço de saúde participaram do estudo, totalizando 42 participantes. Os encontros aconteceram no hospital ou no domicílio das famílias. Os resultados foram discutidos em quatro temas: Apresentação das famílias, no qual elas são mostradas por meio das suas biografias, projetos, personagens e contexto. Elas têm em comum história de perdas, afastamento das origens, morte de pessoas significativas, separações, paternidades presumidas e outros problemas de saúde além da doença da criança em foco. Não são famílias ‘convencionais’ e têm uma grande mobilidade no espaço geográfico. As condições materiais de existência são sofríveis. Padecem pelas condições próprias da doença e pela ausência de políticas sociais que dêem suporte ao seu cotidiano. Contam fatos que aparentemente não têm nenhuma relação com a criança doente, mas que foi entendido como um ‘dar-se a conhecer’ da família para que o contexto do cuidado pudesse ser compreendido; Na trajetória da doença da criança na família, são apresentados os caminhos percorridos desde o início da doença, o esforço para superar as dificuldades financeiras, as mudanças na vida da família, o temor das recidivas, a busca por explicações para a sua ocorrência e o orgulho da família em superar o pior; no cuidado da criança com Síndrome Nefrótica, revelam-se os horizontes dos cuidadores, familiares e profissionais de saúde, e o cuidado possível a partir dos arranjos da família e limites da equipe do serviço. Estes expuseram os seus dilemas e limites no enfrentamento do cuidado à criança e na abordagem da família. Familiares e equipe expressaram a necessidade de uma mútua interação para compor o produto do amplo diálogo, o cuidado, que deve incluir o conhecimento técnico-científico, as subjetividades e os horizontes das famílias. Ao final da discussão, faz-se o exercício da avaliação do cuidado e do serviço de Nefrologia Pediátrica a partir da proposta de avaliação formativa de Ayres, compreendida como um recurso técnico e político para a reorientação da racionalidade das práticas de saúde, por meio de um conjunto de procedimentos sistemáticos para dar visibilidade ao que é feito por referência ao que se pode e/ou se quer fazer em saúde.
This study aims to understand the care of children with Nephrotic Syndrome, searching to identify new elements and horizons to reconstruct the nursing care, focalizing to the family. A qualitative study was realized, using hermeneutics according to Gadamer and Ricouer as a theoretical-methodological reference framework. Study participants were families of children and adolescents with Nephrotic Syndrome who attended the Pediatric Nephrology Service at the University Hospital Júlio Müller of Mato Grosso Federal University in Cuiabá, Brazil. Data were collected by means of: the families’ nursing records, the construction of the genogram and ecomap, documentary analysis and talks with the families. A total of 42 subjects participated, including six families and four health team members. The meetings happened at the hospital or at the families’ homes. The results were organized in three themes: Presentation of the families by means of their projects, characters and context. They share a history of losses, distance from one’s origins, death of important persons, separations, presumed fatherhood and other health problems, besides the child’s disease. These are ‘unconventional’ families which are very mobile in terms of space. Material conditions are sufferable. They suffer as a result of the disease’s characteristic conditions and the absence of social policies to support their daily lives. They talk about facts that do not seem to be related with the sick child, but were considered as the family’s ‘getting to know’ in order to understand the context of care. The trajectory of the child’s disease in the family presents the family’s experiences since the start of the disease, their efforts to overcome financial difficulties, changes in family life, the fear of relapses, the search for explanations for their occurrence and the family’s pride when they get over the worst of it. The third theme Care for the nephrotic child reveals the viewpoints of caregivers, family members and health professionals, as well as possible care on the basis of family arrangements. The health team also exposes its dilemmas and limits in facing the child’s care and approaching the family. Family and team members express the need for mutual interaction to construct the product of this broad dialogue, that is, care, which should exclude neither technical-scientific knowledge nor the families’ perspective. What care is concerned, the exercise of formative assessment proposed by Ayres is recommended.

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Terapia Comunitária Integrativa (TCI) é um ambiente comunitário que visa a partilhar experiências de vida e sabedorias de forma horizontal e circular. Cada participante se torna seu próprio terapeuta através da escuta das histórias de vida relatadas neste espaço. Todos são corresponsáveis na busca de soluções e superação dos desafios do cotidiano, em um ambiente acolhedor e caloroso. A condição crônica na infância interfere no funcionamento corporal da criança em longo prazo, demanda assistência e acompanhamento por profissionais de saúde, limita as atividades diárias, origina alterações no seu processo de crescimento e desenvolvimento, comprometendo o cotidiano de todos os membros da família. Como na maioria das doenças crônicas na infância, as doenças crônicas renais alteram as vidas das crianças em todos os níveis, além de lhes restringir atividades rotineiras. Estudo descritivo, com enfoque qualitativo. A intervenção foi feita no Ambulatório de Nefrologia Pediátrica de um Hospital Universitário, no município de Cuiabá, Mato Grosso. Os sujeitos do estudo foram crianças, adolescentes e suas famílias atendidas no ambulatório, que participaram ao menos uma vez da roda de TCI. Também participaram duas acadêmicas de enfermagem, uma funcionária do hospital e uma terapeuta, totalizando 32 pessoas. O material empírico é composto da transcrição das rodas, que foram filmadas, e das anotações da observação participante. Os resultados evidenciaram que as rodas de TCI foram um instrumento de promoção da saúde, proporcionando um espaço comunitário para que os participantes ressignifiquem suas vivências, partilhem sabedorias e experiências, favorecendo desta forma a superação de dificuldades, a busca por soluções, promovendo a autonomia dos indivíduos. É um meio de integração entre as pessoas, que busca elevar a autoestima e descobrir potenciais, construir redes de apoio e orientar quanto aos seus direitos e seu papel social. Ressalta-se a relevância da TCI como espaço de partilha de experiências com outros familiares/cuidadores em situações semelhantes, visto que os assuntos mais recorrentes nas rodas de TCI foram a sobrecarga da família no cuidado, a responsabilização exacerbada/culpabilização pelo estado de saúde/cuidado do filho e a abnegação por parte da família para uma melhor assistência e acompanhamento do tratamento da criança/adolescente. Apesar das adversidades, ficou evidente que o vínculo afetivo entre mãe/familiar e filho torna o cuidado com a criança uma experiência gratificante. A TCI proporcionou o compartilhamento de sentimentos e vivências, fornecendo segurança e auxiliando a lidar com as incertezas geradas pela condição da criança. A análise dos resultados encontrados neste estudo possibilitou a constatação de que a Terapia Comunitária Integrativa prestada às crianças, adolescentes e suas famílias, em um Ambulatório de Nefrologia Pediátrica no município de Cuiabá, foi uma tecnologia de cuidado complementar, possibilitando uma assistência mais humanizada e acolhedora.
ABSTRACT: Community Integrative Therapy (TCI) is a community environment that aims to share life experiences and wisdoms horizontally and circularly. Each participant becomes his own therapist through listening to the life stories reported in this space. All share responsibility in finding solutions and overcoming the challenges of everyday life in a warm and friendly atmosphere. Chronic conditions in childhood interferes with the functioning body of the child in long term, demand assistance and monitoring by health professionals, limits daily activities, causes changes in its growth and development, affecting the daily lives of all family members. As in most chronic childhood diseases, chronic kidney diseases alter the lives of children at all levels, and restrict their routine activities. This is a descriptive study with a qualitative approach. The action was taken at an Ambulatory of Pediatric Nephrology of a University Hospital in the city of Cuiabá, Mato Grosso. The study subjects were children, adolescents and their families attended the clinic, who participated at least once in TCI circle. Two nursing students, an employee of the hospital and a therapist also participated, totaling 32 people. The empirical material consists of the transcript of the circles, which were videotaped, and the notes of participant observation. The results showed that the circles of TCI were an instrument for health promotion, providing a community space for participants resignify their experiences, share wisdom and experiences, thus favoring the overcoming of difficulties, the search for solutions, promoting the autonomy of the individuals. It is a mean of integration between people, who seek to raise the self-esteem and discovering potential, building networks of support and guidance about their rights and their social role. We stress the importance of TCI as an space to share experiences with other families / caregivers in similar situations , as the most recurrent issues on the TCI circles were overloading the family in care , the heightened accountability / culpability for health / child care and dedication by the family to better care and follow-up treatment of children / adolescents . Despite the adversities, it became evident that the emotional bond between parent / child and family makes child care a rewarding experience. TCI provided the sharing of feelings and experiences, providing security and helping to deal with the uncertainties generated by the child's condition. The results found in this study enabled the realization that Community Integrative Therapy provided to children , adolescents and their families in a Pediatric Nephrology Unit in the city of Cuiabá, it was a technology of complementary care, enabling a more humane and friendly assistance.
RESUMEN: Terapia Integradora de la Comunidad (TCI) es un entorno de comunidad que tiene como objetivo compartir experiencias de vida y sabidurías de forma horizontal y circular. Cada participante se convierte en su propio terapeuta a través de escuchar las historias de vida reportados en este espacio. Todos comparten la responsabilidad en la búsqueda de soluciones y la superación de los retos de la vida cotidiana en un ambiente cálido y acogedor. Las condiciones crónicas en la infancia interfieren en el funcionamiento del cuerpo del niño al largo plazo, demanda la asistencia y el seguimiento por profesionales de la salud, limita las actividades diarias, causa cambios en su crecimiento y desarrollo, lo que afecta la vida cotidiana de todos los miembros de la familia. Al igual que en la mayoría de las enfermedades crónicas de la infancia, las enfermedades renales crónicas alteran la vida de los niños en todos los niveles, y restringen sus actividades de rutina. Estudio descriptivo con enfoque cualitativo. La intervención fue tomada en el Ambulatorio de Nefrología Pediátrica de un Hospital Universitario en la ciudad de Cuiabá, Mato Grosso. Los sujetos del estudio fueron niños, adolescentes y sus familias asistidas en el ambulatorio, que participaron al menos una vez de la rueda de TCI. Dos estudiantes de enfermería, una empleada del hospital y una terapeuta también participaron, de un total de 32 personas. El material empírico consiste en la transcripción de las ruedas, las cuales fueron grabadas en video, y las notas de la observación participante. Los resultados mostraron que las ruedas de la TCI eran un instrumento para la promoción de la salud, proporcionando un espacio comunitario para que los participantes resignifican sus experiencias, compartir la sabiduría y experiencias, favoreciendo la superación de las dificultades, la búsqueda de soluciones, la promoción de la autonomía de los individuos. Es un medio de integración entre las personas, que busca elevar la autoestima y descubrir el potencial, la construcción de redes de apoyo y orientación sobre sus derechos y su papel social. Hacemos hincapié en la importancia de las TCI como un espacio para compartir experiencias con otras familias / cuidadores en situaciones similares, como los temas más recurrentes en las ruedas de TCI fueron la sobrecarga de la familia en el cuidado, la responsabilidad mayor / culpabilidad por la salud / cuidado de niños y la dedicación a la familia para una mejor atención y tratamiento de seguimiento de los niños / adolescentes. A pesar de las adversidades, se hizo evidente que el vínculo emocional entre padre / hijo y su familia hace que el cuidado de niños sea una experiencia gratificante. La TCI proporciona el intercambio de sentimientos y experiencias, proporcionan seguridad y ayuda a hacer frente a las incertidumbres generadas por la condición del niño. Los resultados encontrados en este estudio permitieron a la comprensión de que la Terapia Comunitaria Integrativa proporcionada a niños, adolescentes y sus familias en una Unidad de Nefrología Pediátrica en la ciudad de Cuiabá, era una tecnología de atención complementaria, lo que permite una asistencia más humana y agradable.

Background. This study aims to establish age- and gender-specific cystatin C (CysC) reference values for healthy infants, children, and adolescents and to relate them to pubertal stage, height, weight, and body mass index (BMI). Methods. Serum CysC and creatinine levels of 6217 fasting, morning venous blood samples from 2803 healthy participants of the LIFE Child study (age 3 months to 18 years) were analyzed by an immunoassay. Recruitment started in 2011; 1636 participants provided at least one follow-up measurement. Percentiles for CysC were calculated. Age- and gender-related effects of height, weight, BMI, and puberty status were assessed through linear regression models. Results. Over the first 2 years of life, median CysC levels decrease depending on height (ß = − 0.010 mg/l/cm, p < 0.001) and weight (ß = − 0.033 mg/l/kg, p < 0.001) from 1.06 to 0.88 mg/l for males and from 1.04 to 0.87 mg/l for females. Following the second year of age, the levels remain stable for eight years. From 11 to 14 years of age, there is an increase of median CysC levels in males to 0.98 mg/l and a decrease in females to 0.86 mg/l. The change is associated with puberty (ß = 0.105 mg/l/Tanner stage, p < 0.001 in males and ß = − 0.093 mg/l/Tanner stage, p < 0.01 in females) and in males with height (ß = 0.003 mg/l/cm, p < 0.001). Conclusions. CysC levels depend on age, gender, and height, especially during infancy and puberty. We recommend the use of age- and gender-specific reference values for CysC serum levels for estimating kidney function in clinical practice.:1 Contents ........................................................................................................... 1 2 Introduction ...................................................................................................... 2 GFR measurement ............................................................................................. 2 Technically advanced methods ........................................................................ 2 Serum creatinine................................................................................................ 3 Serum Cystatin C............................................................................................... 3 Current state of research..................................................................................... 5 Reference values of Cystatin C ......................................................................... 5 Cystatin C in healthy test persons .................................................................... 6 Validity of Cystatin C for kidney diseases.......................................................... 6 Post-transplant validity of Cystatin C ............................................................... 7 Validity of Cystatin C in extra-renal diseases .................................................... 7 GFR-equations................................................................................................... 8 Confounders ...................................................................................................... 9 Relevance of the topic ......................................................................................... 11 Hypothesis ........................................................................................................... 13 3 Publication – manuscript................................................................................... 14 4 Summary and interpretation ............................................................................. 16 5 References ........................................................................................................ 20 6 Appendix ........................................................................................................... I 7 Description of the own contributions ................................................................ VII 8 Erklärung über die eigenständige Abfassung der Arbeit .................................. VIII 9 Curriculum vitae ................................................................................................ IX 10 Scientific publications and presentations ....................................................... XI 11 Acknowledgements.......................................................................................... XII

Chronic Kidney Disease (CKD) is a progressive condition that in the majority of cases leads to End Stage Renal Failure (ESRD) and the need for dialysis, with the only cure being renal transplant. CKD affects both adults and children; however the underlying causes of the disease are different. CKD in adults is most commonly secondary to diabetes and/or hypertension while CKD in children is usually caused by congenital structural abnormalities that result directly in renal dysfunction. There have been numerous reports of inflammatory and immunological disturbances in adult CKD that involve both the cellular and humoral immune systems. Consequences of these include an increased rate of cardiovascular disease (CVD), decreased response to vaccinations, as well as increased rates of infection, anaemia and malnutrition. Children with CKD display many of the clinical complications seen in adult kidney disease that are associated with inflammatory and immunological changes. In adults however, many of the primary conditions associated with CKD are inherently pro-inflammatory; therefore it is not clear whether the inflammatory changes observed in adults with CKD are due to pre-existing inflammatory conditions, renal disease per se or a combination of both. The majority of CKD in children is caused by conditions that are not inflammatory in nature. This presents a unique opportunity to study the inflammatory consequences of CKD alone, without the added complication of underlying inflammatory disorders. Despite this, there has been little investigation of the inflammatory and immunological status of children with CKD. Some very recent studies have shown that children with CKD have an increased systemic inflammatory state[1-3], however the nature of these immunological and inflammatory changes remains poorly defined. Identification of the specific inflammatory processes that occur in CKD may provide new treatment targets and the opportunity to develop urgently needed new therapies. The purpose of this thesis is to investigate the presence of immunological changes associated with inflammation in children with CKD. This is the first study to include children with very mild disease, and the significant changes that are present in the early stages of the disease are of particular note. I have shown that CKD in children is an intrinsically inflammatory condition, with increased accumulation of markers of oxidative stress and production of pro-inflammatory cytokines. The inflammatory markers identified in this study may be applied as a foundation for more sensitive diagnostic markers of disease progression as well as provide a basis for novel treatment strategies in this group of patients. Early identification of increased inflammation is a prerequisite for the application of preventive strategies. In addition, a better understanding of the level and mechanisms of systemic inflammation in children with CKD may enable a more accurate assessment of their risk of other inflammatory conditions such as CVD, anaemia, muscle wasting, and malnutrition. Future research that specifically focuses on the reasons and mechanisms for different rates of disease progression may emerge as a result of this study. Importantly, the findings of this study may have implications in the long term treatment of disease and may allow identification of new treatment strategies to achieve better patient outcomes. The outcomes of the study are: • Better definition of inflammatory profiles in paediatric CKD and correlation with disease severity and progression, which should contribute to improved management strategies. • Identification of new treatment targets to reduce the damage caused by chronic systemic inflammation. • Mechanistic understanding of the relationship of the inflammatory profile in regard to source leucocytes or other contributing cell types.
Thesis (Ph.D.) -- University of Adelaide, School of Paediatrics and Reproductive Health, 2008

Chronic Kidney Disease (CKD) is a progressive condition that in the majority of cases leads to End Stage Renal Failure (ESRD) and the need for dialysis, with the only cure being renal transplant. CKD affects both adults and children; however the underlying causes of the disease are different. CKD in adults is most commonly secondary to diabetes and/or hypertension while CKD in children is usually caused by congenital structural abnormalities that result directly in renal dysfunction. There have been numerous reports of inflammatory and immunological disturbances in adult CKD that involve both the cellular and humoral immune systems. Consequences of these include an increased rate of cardiovascular disease (CVD), decreased response to vaccinations, as well as increased rates of infection, anaemia and malnutrition. Children with CKD display many of the clinical complications seen in adult kidney disease that are associated with inflammatory and immunological changes. In adults however, many of the primary conditions associated with CKD are inherently pro-inflammatory; therefore it is not clear whether the inflammatory changes observed in adults with CKD are due to pre-existing inflammatory conditions, renal disease per se or a combination of both. The majority of CKD in children is caused by conditions that are not inflammatory in nature. This presents a unique opportunity to study the inflammatory consequences of CKD alone, without the added complication of underlying inflammatory disorders. Despite this, there has been little investigation of the inflammatory and immunological status of children with CKD. Some very recent studies have shown that children with CKD have an increased systemic inflammatory state[1-3], however the nature of these immunological and inflammatory changes remains poorly defined. Identification of the specific inflammatory processes that occur in CKD may provide new treatment targets and the opportunity to develop urgently needed new therapies. The purpose of this thesis is to investigate the presence of immunological changes associated with inflammation in children with CKD. This is the first study to include children with very mild disease, and the significant changes that are present in the early stages of the disease are of particular note. I have shown that CKD in children is an intrinsically inflammatory condition, with increased accumulation of markers of oxidative stress and production of pro-inflammatory cytokines. The inflammatory markers identified in this study may be applied as a foundation for more sensitive diagnostic markers of disease progression as well as provide a basis for novel treatment strategies in this group of patients. Early identification of increased inflammation is a prerequisite for the application of preventive strategies. In addition, a better understanding of the level and mechanisms of systemic inflammation in children with CKD may enable a more accurate assessment of their risk of other inflammatory conditions such as CVD, anaemia, muscle wasting, and malnutrition. Future research that specifically focuses on the reasons and mechanisms for different rates of disease progression may emerge as a result of this study. Importantly, the findings of this study may have implications in the long term treatment of disease and may allow identification of new treatment strategies to achieve better patient outcomes. The outcomes of the study are: • Better definition of inflammatory profiles in paediatric CKD and correlation with disease severity and progression, which should contribute to improved management strategies. • Identification of new treatment targets to reduce the damage caused by chronic systemic inflammation. • Mechanistic understanding of the relationship of the inflammatory profile in regard to source leucocytes or other contributing cell types.
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Thesis (Ph.D.) -- University of Adelaide, School of Paediatrics and Reproductive Health, 2008

It is known that operational costs of health care organizations constitute an important part of health expenditure in the world. Careful examination of all kinds affecting costs is inevitable in order to provide more effective and efficient health care services. For this reason, in the study, impact of factors affecting hospitalization period on costs is mentioned. The purpose of the study is to measure the effects of these factors on costs by identifying the factors prolonging the duration of patients’ hospital stay. It is regarded that the factors extending the duration of patients’ hospital stay increase the costs. In the scope of the study, 405 patients who are discharged from Cerrahpaşa Faculty of Medicine Hospital Pediatric Nephrology Service in 2012 are discussed. Variables of the study, the average hospitalization days, number of patients, the maximum number of patients, bed occupancy rate, bed turnover and bed units are used in the rev range. The data of the patients included in our study has been gathered with the help of retrospective study area, retrospective method, scanning archives, and financial epicrisis in the system of hospital automation. The obtained data have been studied on standard deviation, maximum and minimum value by making use of the excel programme. As a result of studies based on these reviews, it has been found out that the factors extending the period of patients’ hospital stay increase the costs.