-
Journal of Pediatric Surgery Feb 2012Minimally invasive adrenalectomy (MIA) is the criterion standard for removal of small adrenal tumors in adults. The purpose of this review was to determine the place of... (Review)
Review
PURPOSE
Minimally invasive adrenalectomy (MIA) is the criterion standard for removal of small adrenal tumors in adults. The purpose of this review was to determine the place of MIA in children.
METHODS
The authors conducted a systematic review of the pediatric and adult literature about MIA, focusing on the technique and indications.
RESULTS
Minimally invasive adrenalectomy appears superior to open adrenalectomy for small tumors. The potential advantages of MIA are appealing for postoperative pain, risk of intestinal obstruction, and quality of scars. The most common approach is the transperitoneal lateral laparoscopy, which allows for a large working space. For small tumors or for bilateral adrenalectomy, the prone retroperitoneoscopy is a promising new technique. In children, the learning curve is an issue because the indications are rare. The most common indication is neuroblastoma without image-defined surgical risk factors. The incidence of local recurrence is low, but the follow-up is short in most cases.
CONCLUSIONS
Minimally invasive adrenalectomy is promising for removal of small adrenal tumors. Long-term follow-up is required to evaluate the efficacy of MIA in neuroblastomas. Benign diseases are excellent candidates for this minimally invasive technique.
Topics: Adenoma; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adrenocortical Adenoma; Carcinoma; Child; Cicatrix; Esthetics; Ganglioneuroma; Humans; Laparoscopy; Learning Curve; Minimally Invasive Surgical Procedures; Neuroblastoma; Pheochromocytoma; Postoperative Complications; Posture; Robotics; Tumor Burden
PubMed: 22325405
DOI: 10.1016/j.jpedsurg.2011.08.003 -
The Journal of Clinical Endocrinology... Sep 2010Treatment for patients with congenital adrenal hyperplasia (CAH) may affect the final height of these patients. (Meta-Analysis)
Meta-Analysis Review
CONTEXT
Treatment for patients with congenital adrenal hyperplasia (CAH) may affect the final height of these patients.
OBJECTIVE
Our objective was to determine the distribution of achieved height in patients with classic CAH diagnosed at infancy or early childhood and treated with glucocorticoids.
DATA SOURCES
We searched MEDLINE, EMBASE, Cochrane Library, ISI Web of Science, and Scopus through September 2008; the reference sections of included studies; and expert files.
STUDY SELECTION
Eligible studies included patients diagnosed with CAH before age 5 and followed to final height.
DATA EXTRACTION
Reviewers working in duplicate independently extracted data on study characteristics and outcomes and determined each study's risk of bias.
DATA SYNTHESIS
The sd score (SDS) for final height and corrected height (defined as final height SDS - midparental height SDS) were estimated from each study and pooled using random-effects metaanalysis. The I(2) statistic was used to assess inconsistency in results across studies.
RESULTS
We found 35 eligible studies, most of which were retrospective single-cohort studies. The final height SDS achieved by CAH patients was -1.38 (-1.56 to -1.20; I(2) = 90.2%), and the corrected height SDS was -1.03 (-1.20 to -0.86; I(2) = 63.1%). This was not significantly associated with age at diagnosis, gender, type and dose of steroid, and age of onset of puberty. Mineralocorticoid users had a better height outcome in comparison with the nonusers (P = 0.02).
CONCLUSION
Evidence derived from observational studies suggests that the final height of CAH patients treated with glucocorticoids is lower than the population norm and is lower than expected given parental height.
Topics: Adrenal Hyperplasia, Congenital; Adult; Algorithms; Body Height; Glucocorticoids; Growth Disorders; Human Growth Hormone; Humans; Mineralocorticoids; Treatment Outcome
PubMed: 20823467
DOI: 10.1210/jc.2009-2616 -
Clinical Endocrinology Oct 2010Prenatal treatment with dexamethasone to prevent virilization in pregnancies at risk for classical congenital adrenal hyperplasia (CAH) remains controversial. (Meta-Analysis)
Meta-Analysis Review
Prenatal dexamethasone use for the prevention of virilization in pregnancies at risk for classical congenital adrenal hyperplasia because of 21-hydroxylase (CYP21A2) deficiency: a systematic review and meta-analyses.
CONTEXT
Prenatal treatment with dexamethasone to prevent virilization in pregnancies at risk for classical congenital adrenal hyperplasia (CAH) remains controversial.
OBJECTIVE
To conduct a systematic review and meta-analyses of studies that evaluated the effects of dexamethasone administration during pregnancies at risk for classical CAH because of 21-hydroxylase deficiency (CYP21A2).
DATA SOURCES
We searched MEDLINE, EMBASE, and Cochrane CENTRAL from inception through August 2009. Review of reference lists and contact with CAH experts further identified candidate studies.
STUDY SELECTION
Reviewers working independently and in duplicate determined trial eligibility. Eligible studies reported the effects on either foetal or maternal outcomes of dexamethasone administered during pregnancy compared to a control group that did not receive any treatment.
DATA EXTRACTION
Reviewers working independently and in duplicate determined the methodological quality of studies and collected data on patient characteristics, interventions, and outcomes.
DATA SYNTHESIS
We identified only four eligible observational studies (325 pregnancies treated with dexamethasone). The methodological quality of the included studies was overall low. Meta-analysis demonstrates a reduction in foetus virilization measured by Prader score in female foetuses treated with dexamethasone initiated early during pregnancy (weighted mean difference, -2.33, 95% CI, -3.38, -1.27). No deleterious effects of dexamethasone on stillbirths, spontaneous abortions, foetal malformations, neuropsychological or developmental outcomes were found although these data are quite sparse. There was increased oedema and striae in the mothers treated with dexamethasone. There were no data on long-term follow-up of physical and metabolic outcomes in children exposed to dexamethasone.
CONCLUSIONS
The observational nature of the available evidence and the overall small sample size of the whole body of the literature significantly weaken inferences about the benefits and harms of dexamethasone in this setting. Dexamethasone seems to be associated with reduction in foetus virilization without significant maternal or foetal adverse effects. However, this review underscores the current uncertainty and further investigation is clearly needed. The decision about initiating treatment should be based on patients' values and preferences and requires fully informed and consenting parents.
Topics: Adrenal Hyperplasia, Congenital; Dexamethasone; Female; Fetus; Humans; Pregnancy; Risk; Virilism
PubMed: 20550539
DOI: 10.1111/j.1365-2265.2010.03826.x -
Journal of Public Health Medicine Sep 1998Developments in screening technology and increased understanding of the natural history and treatment of inborn errors of metabolism (IEMs) have produced pressure to... (Review)
Review
BACKGROUND
Developments in screening technology and increased understanding of the natural history and treatment of inborn errors of metabolism (IEMs) have produced pressure to extend neonatal screening programmes. This review aims to assess the evidence for the appropriateness of such programmes.
METHODS
A formal systematic literature review was conducted. Exclusion and inclusion criteria were used to select papers for critical appraisal by pairs of reviewers. Standard criteria were used to assess the appropriateness of neonatal screening for various IEMs. Site visits were conducted to assess new technologies for newborn screening.
RESULTS
A total of 1866 papers were identified and 407 systematically selected for full critical appraisal. Published evidence confirmed that universal newborn screening for phenylketonuria (PKU) meets all of the screening criteria and justifies the expense and infrastructure necessary for the collection and testing of neonatal blood spots. There was insufficient evidence in the literature to assess the cost-effectiveness of screening for any other IEMs. There was reasonable evidence to support inclusion in extended neonatal screening of four other IEMs: biotinidase deficiency, congenital adrenal hyperplasia (CAH), medium-chain acyl CoA dehydrogenase (MCAD) deficiency and glutaric aciduria type 1 (GA1).
CONCLUSIONS
Large-scale trials of screening for biotinidase, CAH, MCAD and GA1 should be conducted, with careful evaluation to establish their clinical effectiveness and cost-effectiveness in practice. Screening for the latter two disorders would be dependent upon the use of tandem mass spectrometry (tandem MS). The application of tandem MS to newborn screening requires further evaluation. The extension of neonatal screening programmes to other IEMs is not currently justified.
Topics: Evidence-Based Medicine; Humans; Infant, Newborn; London; Metabolism, Inborn Errors; Neonatal Screening; Program Evaluation; Technology Assessment, Biomedical
PubMed: 9793900
DOI: 10.1093/oxfordjournals.pubmed.a024777