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Article ; Online: Early bilateral nephrectomy in infantile autosomal recessive polycystic kidney disease.

Mallett, Tamara Mary / O'Hagan, Emma / McKeever, Karl Gerard

BMJ case reports

2015 Volume 2015

Abstract: The management of neonatal autosomal recessive polycystic kidney disease (ARPKD) complicated by severe pulmonary insufficiency presents complex clinical challenges. Where massive nephromegaly exists, early bilateral nephrectomy, supportive peritoneal ... ...

Abstract	The management of neonatal autosomal recessive polycystic kidney disease (ARPKD) complicated by severe pulmonary insufficiency presents complex clinical challenges. Where massive nephromegaly exists, early bilateral nephrectomy, supportive peritoneal dialysis and early aggressive nutrition can minimise infant mortality. Consensus, however, is lacking on the role and optimal timing of nephrectomy, with decision-making driven by the patient's clinical condition and the expertise of the centre. We report on our experience of an infant with ARPKD requiring neonatal renal replacement therapy and survival at 14 months following early bilateral nephrectomy.
MeSH term(s)	Echocardiography ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Nephrectomy/methods ; Peritoneal Dialysis/methods ; Polycystic Kidney, Autosomal Recessive/diagnosis ; Polycystic Kidney, Autosomal Recessive/surgery ; Pregnancy ; Treatment Outcome
Language	English
Publishing date	2015-12-15
Publishing country	England
Document type	Case Reports ; Journal Article
ISSN	1757-790X
ISSN (online)	1757-790X
DOI	10.1136/bcr-2015-211106
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Article ; Online: A multiple methods approach to determine adherence with prescribed mycophenolate in children with kidney transplant.

Almardini, Reham / Taybeh, Esra' O / Alsous, Mervat M / Hawwa, Ahmed F / McKeever, Karl / Horne, Rob / McElnay, James C

British journal of clinical pharmacology

2019 Volume 85, Issue 7, Page(s) 1434–1442

Abstract: Aims: The aim of this study was, to use a multiple methods approach, including, for the first time, dried blood spot (DBS) sampling with population pharmacokinetic interpretation, to assess adherence to mycophenolate in children with kidney transplant. ... ...

Abstract	Aims: The aim of this study was, to use a multiple methods approach, including, for the first time, dried blood spot (DBS) sampling with population pharmacokinetic interpretation, to assess adherence to mycophenolate in children with kidney transplant. A second aim was to identify patient/parental factors that influenced adherence and to link adherence behaviour to clinical outcomes. Methods: A convenience sample of 33 children with kidney transplant (age ≤ 18 years) who had been prescribed mycophenolate for at least 3 months were recruited from participating outpatient clinics in the UK and Jordan. Medication adherence was determined via self-report questionnaires, medication refill data from dispensing records, and via mycophenolic acid concentrations in plasma and DBS samples obtained from children during a clinic visit. Results: Through triangulation of results from the different methodological approaches a total of 12 children (36.4%) were deemed to be nonadherent with their prescribed mycophenolate treatment. Logistic regression analysis indicated that nonadherence was significantly associated with the presence of mycophenolate side effects. Poor adherence was positively linked to measures of poor clinical outcomes (hospitalisation and the need for kidney biopsy). Conclusions: Despite the imperative regarding medication adherence to help prevent organ rejection, a significant proportion of children are not fully adherent with their therapy. Side-effects appear to be an important factor leading to nonadherence. Measurement of mycophenolate in DBS samples, coupled with the use of population pharmacokinetics modelling, was a convenient direct approach to assessing adherence in children with kidney transplant and has the potential to be introduced into routine practice.
MeSH term(s)	Adolescent ; Child ; Dried Blood Spot Testing ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Immunosuppressive Agents/adverse effects ; Immunosuppressive Agents/pharmacokinetics ; Kidney Transplantation ; Male ; Medication Adherence ; Models, Biological ; Mycophenolic Acid/administration & dosage ; Mycophenolic Acid/adverse effects ; Mycophenolic Acid/pharmacokinetics ; Self Report ; Surveys and Questionnaires
Chemical Substances	Immunosuppressive Agents ; Mycophenolic Acid (HU9DX48N0T)
Language	English
Publishing date	2019-05-11
Publishing country	England
Document type	Journal Article
ZDB-ID	188974-6
ISSN	1365-2125 ; 0306-5251 ; 0264-3774
ISSN (online)	1365-2125
ISSN	0306-5251 ; 0264-3774
DOI	10.1111/bcp.13911
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Article: Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report.

O'Hagan, Emma / Mallett, Tamara / Convery, Mairead / McKeever, Karl

Clinical nephrology. Case studies

2015 Volume 3, Page(s) 25–30

Abstract: Antiglomerular basement membrane (anti-GBM) antibody disease is uncommon in the pediatric population. There are no cases in the literature describing the development of anti-GBM disease following XGP or nephrectomy. We report the case of a 7-year-old boy ...

Abstract	Antiglomerular basement membrane (anti-GBM) antibody disease is uncommon in the pediatric population. There are no cases in the literature describing the development of anti-GBM disease following XGP or nephrectomy. We report the case of a 7-year-old boy with no past history of urological illness, treated with antimicrobials and nephrectomy for diffuse, unilateral xanthogranulomatous pyelonephritis (XGP). Renal function and ultrasound scan of the contralateral kidney postoperatively were normal. Three months later, the child represented in acute renal failure with rapidly progressive glomerulonephritis requiring hemodialysis. Renal biopsy showed severe crescentic glomerulonephritis with 95% of glomeruli demonstrating circumferential cellular crescents. Strong linear IgG staining of the glomerular basement membranes was present, in keeping with anti-GBM disease. Circulating anti-GBM antibodies were positive. Treatment with plasma exchange, methylprednisolone, and cyclophosphamide led to normalization of anti-GBM antibody titers. Frequency of hemodialysis was reduced as renal function improved, and he is currently independent of dialysis with estimated glomerular filtration rate 20.7 mls/min/1.73 m
Language	English
Publishing date	2015-11-02
Publishing country	Germany
Document type	Case Reports
ISSN	2196-5293
ISSN	2196-5293
DOI	10.5414/CNCS108594
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Article ; Online: Economic Evaluation of Using Daily Prednisolone versus Placebo at the Time of an Upper Respiratory Tract Infection for the Management of Children with Steroid-Sensitive Nephrotic Syndrome: A Model-Based Analysis.

PharmacoEconomics - open

2022 Volume 6, Issue 4, Page(s) 605–617

Abstract: Background: Childhood steroid-sensitive nephrotic syndrome is a frequently relapsing disease with significant short- and long-term complications, leading to high healthcare costs and reduced quality of life for patients. The majority of relapses are ... ...

Abstract	Background: Childhood steroid-sensitive nephrotic syndrome is a frequently relapsing disease with significant short- and long-term complications, leading to high healthcare costs and reduced quality of life for patients. The majority of relapses are triggered by upper respiratory tract infections (URTIs) and evidence shows that daily low-dose prednisolone at the time of infection may reduce the risk of relapse. Objective: The aim of this study was to assess the cost effectiveness of a 6-day course of low-dose prednisolone at the start of a URTI when compared with placebo. Methods: A state-transition Markov model was developed to conduct a cost-utility analysis with the outcome measured in quality-adjusted life-years (QALYs). Resource use and outcome data were derived from the PREDNOS2 trial. The analysis was performed from a UK National Health Service perspective and the results were extrapolated to adulthood. Model parameter and structural uncertainty were assessed using sensitivity analyses. Results: The base-case results showed that administering low-dose prednisolone at the time of a URTI generated more QALYs and a lower mean cost at 1 year compared with placebo. In the long-term, low-dose prednisolone was associated with a cost saving (£176) and increased effectiveness (0.01 QALYs) compared with placebo and thus remained the dominant treatment option. These findings were robust to all sensitivity analyses. Conclusion: A 6-day course of low-dose prednisolone at the time of a URTI in children with steroid-sensitive nephrotic syndrome has the potential to reduce healthcare costs and improve quality of life compared with placebo.
Language	English
Publishing date	2022-06-22
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2874287-4
ISSN	2509-4254 ; 2509-4262
ISSN (online)	2509-4254
ISSN	2509-4262
DOI	10.1007/s41669-022-00334-6
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Article ; Online: Daily low-dose prednisolone to prevent relapse of steroid-sensitive nephrotic syndrome in children with an upper respiratory tract infection: PREDNOS2 RCT.

Health technology assessment (Winchester, England)

2022 Volume 26, Issue 3, Page(s) 1–94

Abstract: Background: Most children with steroid-sensitive nephrotic syndrome have relapses that are triggered by upper respiratory tract infections. Four small trials, mostly in children already taking maintenance corticosteroid in countries of different upper ... ...

Abstract	Background: Most children with steroid-sensitive nephrotic syndrome have relapses that are triggered by upper respiratory tract infections. Four small trials, mostly in children already taking maintenance corticosteroid in countries of different upper respiratory tract infection epidemiology, showed that giving daily low-dose prednisone/prednisolone for 5-7 days during an upper respiratory tract infection reduces the risk of relapse. Objectives: To determine if these findings were replicated in a large UK population of children with relapsing steroid-sensitive nephrotic syndrome on different background medication or none. Design: A randomised double-blind placebo-controlled trial, including a cost-effectiveness analysis. Setting: A total of 122 UK paediatric departments, of which 91 recruited patients. Participants: A total of 365 children with relapsing steroid-sensitive nephrotic syndrome (mean age 7.6 ± 3.5 years) were randomised (1 : 1) according to a minimisation algorithm based on background treatment. Eighty children completed 12 months of follow-up without an upper respiratory tract infection. Thirty-two children were withdrawn from the trial (14 prior to an upper respiratory tract infection), leaving a modified intention-to-treat analysis population of 271 children (134 and 137 children in the prednisolone and placebo arms, respectively). Interventions: At the start of an upper respiratory tract infection, children received 6 days of prednisolone (15 mg/m Main outcome measures: The primary outcome was the incidence of first upper respiratory tract infection-related relapse following any upper respiratory tract infection over 12 months. The secondary outcomes were the overall rate of relapse, changes in background treatment, cumulative dose of prednisolone, rates of serious adverse events, incidence of corticosteroid adverse effects, change in Achenbach Child Behaviour Checklist score and quality of life. Analysis was by intention-to-treat principle. The cost-effectiveness analysis used trial data and a decision-analytic model to estimate quality-adjusted life-years and costs at 1 year, which were then extrapolated over 16 years. Results: There were 384 upper respiratory tract infections and 82 upper respiratory tract infection-related relapses in the prednisolone arm, and 407 upper respiratory tract infections and 82 upper respiratory tract infection-related relapses in the placebo arm. The number of patients experiencing an upper respiratory tract infection-related relapse was 56 (42.7%) and 58 (44.3%) in the prednisolone and placebo arms, respectively (adjusted risk difference -0.024, 95% confidence interval -0.14 to 0.09; Limitations: A larger number of children than expected did not have an upper respiratory tract infection and the sample size attrition rate was adjusted accordingly during the trial. Conclusions: The clinical analysis indicated that giving 6 days of daily low-dose prednisolone at the time of an upper respiratory tract infection does not reduce the risk of relapse of steroid-sensitive nephrotic syndrome in UK children. However, there was an economic benefit from costs associated with background therapy and relapse, and the health-related quality-of-life impact of having a relapse. Future work: Further work is needed to investigate the clinical and health economic impact of relapses, interethnic differences in treatment response, the effect of different corticosteroid regimens in treating relapses, and the pathogenesis of individual viral infections and their effect on steroid-sensitive nephrotic syndrome. Trial registration: Current Controlled Trials ISRCTN10900733 and EudraCT 2012-003476-39. Funding: This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in
MeSH term(s)	Child ; Child, Preschool ; Cost-Benefit Analysis ; Humans ; Neoplasm Recurrence, Local ; Nephrotic Syndrome/drug therapy ; Prednisolone/adverse effects ; Prednisolone/therapeutic use ; Quality of Life ; Respiratory Tract Infections/drug therapy ; Respiratory Tract Infections/epidemiology
Chemical Substances	Prednisolone (9PHQ9Y1OLM)
Language	English
Publishing date	2022-01-21
Publishing country	England
Document type	Journal Article ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
ZDB-ID	2006765-3
ISSN	2046-4924 ; 1366-5278
ISSN (online)	2046-4924
ISSN	1366-5278
DOI	10.3310/WTFC5658
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Article: Aminoaciduria in the prediction of ifosfamide-induced tubulopathy after childhood cancer: a feasibility study.

Morgan, Jessica E / McKeever, Karl / Tyerman, Kay S / Henderson, Michael / Picton, Susan / Phillips, Robert S

Pilot and feasibility studies

2016 Volume 2, Page(s) 4

Abstract: Background: Ifosfamide, an alkylating agent used widely in the treatment of childhood malignancy, can cause many side effects including a proximal tubulopathy. Studies suggest that aminoaciduria is seen most commonly of all the biochemical abnormalities ...

Abstract	Background: Ifosfamide, an alkylating agent used widely in the treatment of childhood malignancy, can cause many side effects including a proximal tubulopathy. Studies suggest that aminoaciduria is seen most commonly of all the biochemical abnormalities of ifosfamide-induced tubulopathy. A recent systematic review has found a paucity of data regarding the value of early markers indicating clinically significant tubulopathy. We undertook a pilot study to determine the feasibility of examining whether patients can be risk-stratified on the basis of aminoaciduria for the development of future significant ifosfamide-induced tubulopathy, to allow the evolution of appropriate follow-up strategies. We also aimed to define accrual rates, costs and clinical demands for a future larger study. Methods: This observational study recruited 21 patients from the Leeds Paediatric Oncology service. The medical notes of each patient were reviewed for demographic and clinical data. Simultaneous samples of blood and urine were obtained. Results: The investigations in the feasibility study were acceptable to patients and were minimally demanding on both clinical and laboratory staff. Financially, the cost per patient was minimal. This study was not powered to detect significant associations with TmP/GFR (ratio of renal tubular maximum reabsorption rate of phosphate to glomerular filtration rate), growth and electrolyte supplementation. However, all patients with minimal aminoaciduria (≤2 elevated urinary amino acids) had normal TmP/GFR and no need for electrolyte supplementation. Conclusions: This pilot study has shown that a larger study is feasible and may provide clinically useful data to change current practice. This should aim to establish whether the number of abnormal amino acids or the degree of abnormality is most significant in predicting clinically significant proximal tubulopathy.
Language	English
Publishing date	2016-01-22
Publishing country	England
Document type	Journal Article
ZDB-ID	2809935-7
ISSN	2055-5784
ISSN	2055-5784
DOI	10.1186/s40814-015-0040-0
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Article ; Online: Evaluation of Daily Low-Dose Prednisolone During Upper Respiratory Tract Infection to Prevent Relapse in Children With Relapsing Steroid-Sensitive Nephrotic Syndrome: The PREDNOS 2 Randomized Clinical Trial.

JAMA pediatrics

2021 Volume 176, Issue 3, Page(s) 236–243

Abstract: Importance: In children with corticosteroid-sensitive nephrotic syndrome, many relapses are triggered by upper respiratory tract infections. Four small studies found that administration of daily low-dose prednisolone for 5 to 7 days at the time of an ... ...

Abstract	Importance: In children with corticosteroid-sensitive nephrotic syndrome, many relapses are triggered by upper respiratory tract infections. Four small studies found that administration of daily low-dose prednisolone for 5 to 7 days at the time of an upper respiratory tract infection reduced the risk of relapse, but the generalizability of their findings is limited by location of the studies and selection of study population. Objective: To investigate the use of daily low-dose prednisolone for the treatment of upper respiratory tract infection-related relapses. Design, setting, and participants: This double-blind, placebo-controlled randomized clinical trial (Prednisolone in Nephrotic Syndrome [PREDNOS] 2) evaluated 365 children with relapsing steroid-sensitive nephrotic syndrome with and without background immunosuppressive treatment at 122 pediatric departments in the UK from February 1, 2013, to January 31, 2020. Data from the modified intention-to-treat population were analyzed from July 1, 2020, to December 31, 2020. Interventions: At the start of an upper respiratory tract infection, children received 6 days of prednisolone, 15 mg/m2 daily, or matching placebo preparation. Those already taking alternate-day prednisolone rounded their daily dose using trial medication to the equivalent of 15 mg/m2 daily or their alternate-day dose, whichever was greater. Main outcomes and measures: The primary outcome was the incidence of first upper respiratory tract infection-related relapse. Secondary outcomes included overall rate of relapse, changes in background immunosuppressive treatment, cumulative dose of prednisolone, rates of serious adverse events, incidence of corticosteroid adverse effects, and quality of life. Results: The modified intention-to-treat analysis population comprised 271 children (mean [SD] age, 7.6 [3.5] years; 174 [64.2%] male), with 134 in the prednisolone arm and 137 in the placebo arm. The number of patients experiencing an upper respiratory tract infection-related relapse was 56 of 131 (42.7%) in the prednisolone arm and 58 of 131 (44.3%) in the placebo arm (adjusted risk difference, -0.02; 95% CI, -0.14 to 0.10; P = .70). No evidence was found that the treatment effect differed according to background immunosuppressive treatment. No significant differences were found in secondary outcomes between the treatment arms. A post hoc subgroup analysis assessing the primary outcome in 54 children of South Asian ethnicity (risk ratio, 0.66; 95% CI, 0.40-1.10) vs 208 children of other ethnicity (risk ratio, 1.11; 95% CI, 0.81-1.54) found no difference in efficacy of intervention in those of South Asian ethnicity (test for interaction P = .09). Conclusions and relevance: The results of PREDNOS 2 suggest that administering 6 days of daily low-dose prednisolone at the time of an upper respiratory tract infection does not reduce the risk of relapse of nephrotic syndrome in children in the UK. Further work is needed to investigate interethnic differences in treatment response. Trial registration: isrctn.org Identifier: ISRCTN10900733; EudraCT 2012-003476-39.
MeSH term(s)	Adrenal Cortex Hormones/therapeutic use ; Child ; Humans ; Male ; Nephrotic Syndrome/complications ; Nephrotic Syndrome/drug therapy ; Prednisolone/therapeutic use ; Quality of Life ; Recurrence ; Respiratory Tract Infections/drug therapy ; Respiratory Tract Infections/prevention & control
Chemical Substances	Adrenal Cortex Hormones ; Prednisolone (9PHQ9Y1OLM)
Language	English
Publishing date	2021-12-19
Publishing country	United States
Document type	Journal Article ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
ZDB-ID	2701223-2
ISSN	2168-6211 ; 2168-6203
ISSN (online)	2168-6211
ISSN	2168-6203
DOI	10.1001/jamapediatrics.2021.5189
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Article: An epidemiological, clinical and genetic survey of neurofibromatosis type 1 in children under sixteen years of age.

McKeever, Karl / Shepherd, Charles W / Crawford, Hilda / Morrison, Patrick J

The Ulster medical journal

2008 Volume 77, Issue 3, Page(s) 160–163

Abstract: Aim: To identify all cases of Neurofibromatosis type 1 in Northern Ireland under 16 years of age, document age, modes of presentation and any complications that occurred.: Methods: All cases of Neurofibromatosis type 1 in children less than 16 years ... ...

Abstract	Aim: To identify all cases of Neurofibromatosis type 1 in Northern Ireland under 16 years of age, document age, modes of presentation and any complications that occurred. Methods: All cases of Neurofibromatosis type 1 in children less than 16 years of age were identified from the records in the Department of Medical Genetics. From the records and by direct contact with the patient's parents the relevant clinical information was obtained. Results: Seventy-five children aged sixteen years or less were identified (prevalence of 17.6 per 100,000 (1 in 5681) of the population under 16 years of age). 45 (57%) had an affected first degree relative and 32 (43%) had no family history. 54 (72%) had at least one complication, 18 (24%) had 2; 9 (12%) had 3 and 3 (4%) had 4 complications. The most common complication was learning difficulties, which was seen in 37 (49.3%) cases. 11 (14.7%) patients had a malignancy; of whom 5 (6.7%) had an optic glioma (2 identified after diagnosis) and 3 (4%) had a CNS malignancy. Conclusion: Children with NF1 should be seen yearly by a health professional or team until after puberty and have a thorough clinical examination. The minimum prevalence is 1 in 5681 (17.6 per 100,000). We suggest a checklist is used to review nine important features; height, weight, head circumference, examination of the skin, blood pressure, ophthalmology examination (includes visual fields), examination of the spine, and for early/late puberty and consider referral to educational psychology. Educational authorities should identify all individuals with NF1 as they are at high risk of developing learning difficulties.
MeSH term(s)	Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Neurofibromatosis 1/complications ; Neurofibromatosis 1/diagnosis ; Neurofibromatosis 1/epidemiology ; Neurofibromatosis 1/genetics ; Northern Ireland/epidemiology ; Prevalence
Language	English
Publishing date	2008-09
Publishing country	Northern Ireland
Document type	Journal Article
ZDB-ID	603342-8
ISSN	2046-4207 ; 0041-6193
ISSN (online)	2046-4207
ISSN	0041-6193
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Article ; Online: Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial.

Webb, Nicholas J A / Frew, Emma / Brettell, Elizabeth A / Milford, David V / Bockenhauer, Detlef / Saleem, Moin A / Christian, Martin / Hall, Angela S / Koziell, Ania / Maxwell, Heather / Hegde, Shivram / Finlay, Eric R / Gilbert, Rodney D / Booth, Jenny / Jones, Caroline / McKeever, Karl / Cook, Wendy / Ives, Natalie J

Trials

2014 Volume 15, Page(s) 147

Abstract: Background: Relapses of childhood steroid-sensitive nephrotic syndrome (SSNS) are treated with a 4- to 8-week course of high-dose oral prednisolone, which may be associated with significant adverse effects. There is a clear association between upper ... ...

Abstract	Background: Relapses of childhood steroid-sensitive nephrotic syndrome (SSNS) are treated with a 4- to 8-week course of high-dose oral prednisolone, which may be associated with significant adverse effects. There is a clear association between upper respiratory tract infection (URTI) and relapse development. Previous studies in developing nations have suggested that introducing a 5- to 7-day course of daily prednisolone during an URTI may prevent a relapse developing and the need for a treatment course of high-dose prednisolone. The aim of PREDNOS 2 is to evaluate the effectiveness of a 6-day course of daily prednisolone therapy during an URTI in reducing the development of a subsequent relapse in a developed nation. Methods/design: The subjects will be 300 children with relapsing SSNS (≥2 relapses in preceding year), who will be randomised to receive either a 6-day course of daily prednisolone or no change to their current therapy (with the use of placebo to double blind) each time they develop an URTI over 12 months. A strict definition for URTI will be used. Subjects will be reviewed at 3, 6, 9 and 12 months to capture data regarding relapse history, ongoing therapy and adverse effect profile, including behavioural problems and quality of life. A formal health economic analysis will also be performed. The primary end point of the study will be the incidence of URTI-related relapse (3 days of Albustix +++) following the first infection during the 12-month follow-up period. DNA and RNA samples will be collected to identify a potential genetic cause for the disease. Subjects will be recruited from over 100 UK centres with the assistance of the Medicines for Children Research Network.PREDNOS 2 is funded by the National Institute for Health Research Health Technology Assessment Programme (11/129/261). Discussion: We propose that PREDNOS 2 will be a pivotal study that will inform the future standard of care for children with SSNS. If it is possible to reduce the disease relapse rate effectively and safely, this will reduce the morbidity and cost associated with drug treatment, notwithstanding hospital admission and parental absence from employment. Trial registration: Current Controlled Trials (ISRCTN10900733).
MeSH term(s)	Adolescent ; Child ; Child, Preschool ; Clinical Protocols ; Cost-Benefit Analysis ; Double-Blind Method ; Drug Administration Schedule ; Drug Costs ; Glucocorticoids/administration & dosage ; Glucocorticoids/economics ; Humans ; Nephrotic Syndrome/diagnosis ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/economics ; Prednisolone/administration & dosage ; Prednisolone/economics ; Recurrence ; Research Design ; Respiratory Tract Infections/diagnosis ; Respiratory Tract Infections/drug therapy ; Respiratory Tract Infections/economics ; Time Factors ; Treatment Outcome ; Young Adult
Chemical Substances	Glucocorticoids ; Prednisolone (9PHQ9Y1OLM)
Language	English
Publishing date	2014-04-27
Publishing country	England
Document type	Clinical Trial, Phase III ; Journal Article ; Randomized Controlled Trial ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	2040523-6
ISSN	1745-6215 ; 1468-6694 ; 1745-6215
ISSN (online)	1745-6215
ISSN	1468-6694 ; 1745-6215
DOI	10.1186/1745-6215-15-147
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Article ; Online: The clinical, biochemical and genetic features associated with

Ng, Yi Shiau / Alston, Charlotte L / Diodato, Daria / Morris, Andrew A / Ulrick, Nicole / Kmoch, Stanislav / Houštěk, Josef / Martinelli, Diego / Haghighi, Alireza / Atiq, Mehnaz / Gamero, Montserrat Anton / Garcia-Martinez, Elena / Kratochvílová, Hana / Santra, Saikat / Brown, Ruth M / Brown, Garry K / Ragge, Nicola / Monavari, Ahmad / Pysden, Karen /

Ravn, Kirstine / Casey, Jillian P / Khan, Arif / Chakrapani, Anupam / Vassallo, Grace / Simons, Cas / McKeever, Karl / O'Sullivan, Siobhan / Childs, Anne-Marie / Østergaard, Elsebet / Vanderver, Adeline / Goldstein, Amy / Vogt, Julie / Taylor, Robert W / McFarland, Robert

Journal of medical genetics

2016 Volume 53, Issue 11, Page(s) 768–775

Abstract: Background: Mutations in the : Methods: We summarised the clinical, biochemical and molecular genetic investigation of an international cohort of affected individuals with : Results: We identified 14 new cases from 11 pedigrees that harbour ... ...

Abstract	Background: Mutations in the Methods: We summarised the clinical, biochemical and molecular genetic investigation of an international cohort of affected individuals with Results: We identified 14 new cases from 11 pedigrees that harbour recessive Conclusions: The clinical phenotypes and prognosis associated with
Language	English
Publishing date	2016-07-13
Publishing country	England
Document type	Journal Article
ZDB-ID	220881-7
ISSN	1468-6244 ; 0022-2593
ISSN (online)	1468-6244
ISSN	0022-2593
DOI	10.1136/jmedgenet-2016-103910
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