Late outcomes in children with Langerhans cell histiocytosis.

Abstract:

INTRODUCTION:Langerhans cell histiocytosis (LCH) is a rare disease with diverse clinical courses. Despite improvement in survival outcomes in the recent decades, sequelae of the disease remain a concern. This study aimed to provide information on the long-term outcomes in patients with LCH, particularly on the sequelae and any associated factors. METHOD:Medical records of patients with diagnosis of LCH and being managed in our centre were retrospectively reviewed. Data on the courses of illness, mortality, intervention, types and time of late events were collected and analysed. RESULTS:70 patients were included with a mean observation time of 12 years (median 10.7 years, range 1-31.3 years). Sequelae related to LCH were present in 56% (n=39), being more common in multisystem diseases and patients with reactivations. Prevalence of sequelae is as follows: orthopaedic related 27%, diabetes insipidus 19%, growth retardation 13%, cosmetic 10%, neurological 7%, hearing 7%, anterior pituitary hormone deficiency 7%, hepatobiliary 4% and ophthalmological 3%. Neurological sequelae could manifest even 10 years after initial diagnosis of LCH. Reactivations, presence of central nervous system (CNS) risk lesions and treatment with radiotherapy were associated with a higher rate of sequelae. The cumulative incidence of reactivation was 34%. Most reactivations occurred in the first 2.5 years after diagnosis. CONCLUSION:Sequelae were common after LCH, although some were mild. Neurological sequelae could be particularly severe and debilitating. Vigilant long-term follow-up would be essential for optimising patient outcomes. Further studies on the prevention and treatment of CNS disease of LCH are warranted.

journal_name

Arch Dis Child

authors

Chow TW,Leung WK,Cheng FWT,Kumta SM,Chu WCW,Lee V,Shing MMK,Li CK

doi

10.1136/archdischild-2016-312185

subject

Has Abstract

pub_date

2017-09-01 00:00:00

pages

830-835

issue

9

eissn

0003-9888

issn

1468-2044

pii

archdischild-2016-312185

journal_volume

102

pub_type

杂志文章
  • Weekly sport practice and adolescent well-being.

    abstract:OBJECTIVE:Sport practice is widely encouraged, both in guidelines and in clinical practice, because of its broad range of positive effects on health. However, very limited evidence directly supports this statement among adolescents and the sport duration that we should recommend remains unknown. We aimed to determine s...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/archdischild-2013-303729

    authors: Merglen A,Flatz A,Bélanger RE,Michaud PA,Suris JC

    更新日期:2014-03-01 00:00:00

  • The association between birth condition and neuropsychological functioning and educational attainment at school age: a cohort study.

    abstract:OBJECTIVE:Poor condition at birth may impact on IQ, although its effect on other measures of neurodevelopment is unclear. The authors' aim was to determine whether infants receiving resuscitation after birth have reduced scores in measures of attention, memory and language skills or the need for educational support at ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,多中心研究

    doi:10.1136/adc.2009.176065

    authors: Odd DE,Whitelaw A,Gunnell D,Lewis G

    更新日期:2011-01-01 00:00:00

  • PROLONGED INFUSIONS OF KETAMINE AND IMPACT ON INFECTIONS AND WASTE.

    abstract:AIM:Ketamine is used for post-operative analgesia. There has been recent disruption in it's supply. It is usually prescribed by patient's weight (3 mg/kg in 50 ml 0.9% saline) at a rate of 1-5 ml/hr (1-5 microgram/kg/minute). To conserve ketamine supplies our policy was changed to a concentrated "standardised" concentr...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/archdischild-2016-311535.12

    authors: Wong J,Sutherland A

    更新日期:2016-09-01 00:00:00

  • Munchausen syndrome by proxy.

    abstract::Information is presented about 19 children, under age 7 years, from 17 families, whose mothers consistently gave fraudulent clinical histories and fabricated signs so causing them needless harmful medical investigations, hospital admissions, and treatment over periods of time ranging from a few months to 4 years. Epis...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.57.2.92

    authors: Meadow R

    更新日期:1982-02-01 00:00:00

  • Inappropriate oral formulations and information in paediatric trials.

    abstract::Previously, quality of formulations information provided for oral medications used in paediatric clinical trials published in 10 highly cited journals between 2002 and 2004 raised concerns. This short report explores if there was any subsequent improvement on how the formulations used in trials involving children <12 ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.2009.175661

    authors: Pandit S,Shah U,Kirby DJ,Nunn T,Tuleu C

    更新日期:2010-09-01 00:00:00

  • Maternal anxiety disorders predict excessive infant crying: a prospective longitudinal study.

    abstract:PURPOSE:To prospectively examine relations between maternal DSM-IV-TR anxiety and depressive disorders and excessive infant crying. METHODS:Based on the prospective longitudinal Maternal Anxiety in Relation to Infant Development Study, n=306 expectant mothers were enrolled during early pregnancy and repeatedly intervi...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/archdischild-2013-305562

    authors: Petzoldt J,Wittchen HU,Wittich J,Einsle F,Höfler M,Martini J

    更新日期:2014-09-01 00:00:00

  • Copper deficiency in the preterm infant of very low birthweight. Four cases and a reference range for plasma copper.

    abstract::Four preterm infants of very low birthweight (less than 1500 g) developed signs of copper deficiency between age 8 and 10 weeks. All had required prolonged ventilatory support, parenteral nutrition, and nasojejunal feeding. The clinical features, which included osteoporosis, oedema, anaemia, neutropenia, and late apno...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.60.7.644

    authors: Sutton AM,Harvie A,Cockburn F,Farquharson J,Logan RW

    更新日期:1985-07-01 00:00:00

  • Patterns of scald injuries.

    abstract:OBJECTIVES:To describe common patterns of bath water scald injuries in children, to examine differences between accidental and non-accidental bath water scalds in children, and to examine potential for prevention. DESIGN:A two year six month retrospective analysis of admissions to a specialist burns unit. SETTING:The...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.71.2.156

    authors: Yeoh C,Nixon JW,Dickson W,Kemp A,Sibert JR

    更新日期:1994-08-01 00:00:00

  • Height screening at school: ineffective without high standards and adequate resources.

    abstract:BACKGROUND:The Coventry Consensus in 1998 recommended a single height measurement of all children at school entry or around the age of 5 years and prompt referral of children with height <0.4th centile for further assessment, in order to identify undetected and treatable asymptomatic growth disorders. AIM:To determine...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.88.6.477

    authors: Banerjee S,Morgan RJ,Rees SA,Latif AH

    更新日期:2003-06-01 00:00:00

  • The effect of asthma and its treatment on growth.

    abstract::Asthma has little, if any, significant effect on attained adult height. Untreated asthma results in a delay of puberty by approximately 1.3 years, and pubertal delay is likely to explain the majority of apparent growth failure in asthmatics. All currently available inhaled corticosteroids (ICS) result in growth suppre...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,评审

    doi:10.1136/adc.2003.014365

    authors: Doull IJ

    更新日期:2004-01-01 00:00:00

  • Bile acid excretion after pull-through operation for Hirschsprung's disease.

    abstract::Four children with chronic diarrhoea and perianal excoriation after a pull-through operation for Hirschsprung's disease have been shown to have increased but not markedly raised levels of faecal bile acids. Bile acid analysis of the 'bile-rich' duodenal fluid obtained after pancreozymin stimulation in 3 of the patient...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.50.3.243

    authors: Gaze H,Murphy GM,Nelson R,Corkery JJ,Anderson CM

    更新日期:1975-03-01 00:00:00

  • Genomics for paediatricians: promises and pitfalls.

    abstract::In recent years, there have been significant advances in genetic technologies, evolving the field of genomics from genetics. This has huge diagnostic potential, as genomic testing increasingly becomes part of mainstream medicine. However, there are numerous potential pitfalls in the interpretation of genomic data. It ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,评审

    doi:10.1136/archdischild-2017-314558

    authors: Hammond CL,Willoughby JM,Parker MJ

    更新日期:2018-09-01 00:00:00

  • A retrospective observational study comparing rescue medication use in children on combined versus separate long-acting beta-agonists and corticosteroids.

    abstract:BACKGROUND:Data on the efficacy and safety of long-acting β2-agonists (LABA) in children are limited, and current guidelines recommend that LABA always be used with inhaled corticosteroids (ICS). OBJECTIVE:To compare asthma control, assessed by rescue medications use, in children prescribed LABA and ICS as a fixed-dos...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,多中心研究

    doi:10.1136/adc.2009.179069

    authors: Elkout H,McLay JS,Simpson CR,Helms PJ

    更新日期:2010-10-01 00:00:00

  • Familial partial trisomy of the long arm of chromosome 3 (3q).

    abstract::A case of partial trisomy of the long arm of chromosome 3 (3q21 leads to qter) is described. The clinical findings are compared with those in 5 previously reported cases. There is hirsutism and characteristic facial dysmorphism, the common features of which are a square-shaped face, prominent nasal bridge, everted nos...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.54.2.135

    authors: Fear C,Briggs A

    更新日期:1979-02-01 00:00:00

  • Higher rectal temperatures in co-sleeping infants.

    abstract::The effect on deep body temperature of infants co-sleeping (with either or both parents) is investigated in this case control study. Overnight continuous recordings of rectal temperature were made from 34 babies co-sleeping with one or both parents throughout the night and 34 infants matched for age, feeding regimen, ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.75.3.249

    authors: Tuffnell CS,Petersen SA,Wailoo MP

    更新日期:1996-09-01 00:00:00

  • Calcium, magnesium, and glucose levels in blood and CSF of children with febrile convulsions.

    abstract::Levels of calcium, magnesium, and glucose were measured in the blood and cerebrospinal fluid (CSF) of children with febrile convulsions. Calcium and magnesium levels were within the normal range and the blood: CSF ratios were similar to those of normal subjects. Hyperglycaemia was a frequent finding and was reflected ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.51.2.141

    authors: Rutter N,Smales OR

    更新日期:1976-02-01 00:00:00

  • Small bowel biopsy.

    abstract::We describe our technique of small bowel biopsy, which has been used on 190 occasions over a four year period. In 77%, the examination was completed within 10 minutes, and fluoroscopy times were less than 10 seconds in 75% of the cases. The technique is easily taught, and it has made the examination a minor procedure....

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.60.11.1082

    authors: Collins AL,Brookfield DS,Hyde I,Rolles CJ

    更新日期:1985-11-01 00:00:00

  • Management of foreskin problems.

    abstract::One hundred consecutive cases of boys with foreskin problems referred to local paediatric surgeons in the Edinburgh area were studied. Fifty five underwent circumcision and the remainder were managed more conservatively with or without the use of local or general anaesthetic. Although sometimes avoiding general anaest...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.66.6.696

    authors: Lafferty PM,MacGregor FB,Scobie WG

    更新日期:1991-06-01 00:00:00

  • Erythropoietin responsive progenitors in anaemia of prematurity.

    abstract::Circulating erythroid progenitors (BFU-E) in five anaemic preterm infants (haemoglobin less than 100 g/l) were about 2 and 4.4 times as abundant as in 10 preterm infants who were not anaemic and five healthy adults, respectively, and were significantly more responsive to low concentrations of recombinant human erythro...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.66.7_spec_no.810

    authors: Emmerson AJ,Westwood NB,Rackham RA,Stern CM,Pearson TC

    更新日期:1991-07-01 00:00:00

  • Management and outcome of severe head injuries in the Trent region 1985-90.

    abstract::In a five year period, 39 children (29 boys, 10 girls) aged 2 months to 13 years (mean 7.8 years) were studied who had suffered a major head injury (29 road traffic accidents, six falls, and four non-accidental injury). The injury had been assessed clinically and by cranial computed tomography or cranial ultrasound (i...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.67.12.1430

    authors: Elias-Jones AC,Punt JA,Turnbull AE,Jaspan T

    更新日期:1992-12-01 00:00:00

  • Lived experience of Silver-Russell syndrome: implications for management during childhood and into adulthood.

    abstract:OBJECTIVE:There is limited information on the psychosocial impact of growing up with Silver-Russell syndrome (SRS), characterised by slow growth in utero leading to short stature in adulthood. Such information could aid families in making difficult treatment decisions and guide management strategies for health professi...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/archdischild-2018-314952

    authors: Ballard LM,Jenkinson E,Byrne CD,Child JC,Davies JH,Inskip H,Lokulo-Sodipe O,Mackay DJG,Wakeling EL,Temple IK,Fenwick A

    更新日期:2019-01-01 00:00:00

  • Superhero-related injuries in paediatrics: a case series.

    abstract::Five cases of serious injuries to children wearing superhero costumes, involving extreme risk-taking behaviour, are presented here. Although children have always displayed behaviour seemingly unwise to the adult eye, the advent of superhero role models can give unrealistic expectations to the child, which may lead to ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.2006.109793

    authors: Davies P,Surridge J,Hole L,Munro-Davies L

    更新日期:2007-03-01 00:00:00

  • How parents and families cope with chronic renal failure.

    abstract::We studied the effects on the family of childhood chronic renal failure by contrasting children with varying degrees of severity of illness with healthy controls. Disruption of family life was most common in families with the more severely ill children (on hospital haemodialysis) and there was a tendency for more ment...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.63.7.821

    authors: Reynolds JM,Garralda ME,Jameson RA,Postlethwaite RJ

    更新日期:1988-07-01 00:00:00

  • Renal scarring and vesicoureteric reflux.

    abstract::A review of 105 children with urinary tract infection showed an increasing prevalence of grades II-III vesicoureteric reflux with diminishing age. During infancy reflux was almost always severe, and affected boys as often as girls. Radiologically scarred kidneys were drained by refluxing ureters in 98% of cases. The p...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.53.3.210

    authors: Shah KJ,Robins DG,White RH

    更新日期:1978-03-01 00:00:00

  • Improved outcome of acute myeloid leukaemia in Down's syndrome.

    abstract:OBJECTIVE:To review the clinical features, treatment, and outcome of children in the UK with Down's syndrome and acute myeloid leukaemia (AML). DESIGN:A retrospective study of 59 children with Down's syndrome and AML presenting between 1987 and 1995. Data were obtained from hospital case notes, trial records, and by q...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.81.1.32

    authors: Craze JL,Harrison G,Wheatley K,Hann IM,Chessells JM

    更新日期:1999-07-01 00:00:00

  • Is there a role for clowns in paediatric intensive care units?

    abstract::Hospital clowning is a programme in healthcare facilities involving visits from specially trained actors. In the paediatric intensive care unit (PICU), clowning may appear inappropriate and less intuitive. The patient could appear too ill and/or sedated, the environment too crowded or chaotic and the parents too stres...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,评审

    doi:10.1136/archdischild-2016-311583

    authors: Mortamet G,Roumeliotis N,Vinit F,Simonds C,Dupic L,Hubert P

    更新日期:2017-07-01 00:00:00

  • Chronic lymphoglandular enlargement and toxoplasmosis in children.

    abstract::Serum antitoxoplasma titres were determined simultaneously by the direct agglutination and the indirect immunofluorescent tests in 52 children aged 2 to 16 years having chronic lymph node enlargement, mainly cervical. Direct agglutination titres were raised (64 to 4096) in 22 children (42%), but rarely in the control ...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.52.5.403

    authors: Thomaidis T,Anastassea-Vlachou K,Mandalenaki-Lambrou C,Theodoridis C,Vrahnou E

    更新日期:1977-05-01 00:00:00

  • Therapeutic efficacy and safety of ACE inhibitors in the hypertensive paediatric population: a review.

    abstract:PURPOSE:Since 1997, strong incentives have been introduced worldwide to improve access to safe and effective medicines addressing the therapeutic needs of children. ACE inhibitors, the most prescribed antihypertensive drugs in the paediatric population, are one of the prototype drugs targeted by the legislation initiat...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章,评审

    doi:10.1136/archdischild-2016-310582

    authors: Snauwaert E,Vande Walle J,De Bruyne P

    更新日期:2017-01-01 00:00:00

  • Monitoring of cerebral function after severe asphyxia in infancy.

    abstract::Thirty nine infants with severe asphyxia (28 affected perinatally and 11 later) were studied by electrophysiological cerebral function monitoring (CFM) for periods varying from a half to 49 days. Nineteen infants died while still in intensive care and two died later from sequelae. Eighteen survived and were followed u...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.58.12.997

    authors: Bjerre I,Hellström-Westas L,Rosén I,Svenningsen N

    更新日期:1983-12-01 00:00:00

  • Respiratory complications in long-term survivors of oesophageal atresia.

    abstract::One hundred of the 192 survivors of repaired oesophageal atresia at the Royal Children's Hospital, Melbourne, aged one year and over on 1 November 1973, were reviewed to determine the frequency of respiratory complications. 78 children suffered from more than 3 attacks of bronchitis per year during the first 3 years o...

    journal_title:Archives of disease in childhood

    pub_type: 杂志文章

    doi:10.1136/adc.51.4.279

    authors: Dudley NE,Phelan PD

    更新日期:1976-04-01 00:00:00