Tuesday, June 10, 2014

Systematic Review and Meta-Analysis: Parent and Family-Based Interventions for Children and Adolescents With Chronic Medical Conditions.

Law EF, Fisher E, Fales J, Noel M, Eccleston C. (2014) Systematic Review and Meta-Analysis: Parent and Family-Based Interventions for Children and Adolescents With Chronic Medical Conditions. Journal of Pediatric Psychology. 2014 May 30. pii: jsu032. [Epub ahead of print]



To quantify the effects of parent- and family-based psychological therapies for youth with common chronic medical conditions on parent and family outcomes (primary aim) and child outcomes (secondary aim).


MEDLINE, EMBASE, and PsycINFO were searched from inception to April 2013. 37 randomized controlled trials were included. Quality of the evidence was evaluated using GRADE criteria. Data were extracted on parent, family, and child outcomes.


Pooled psychological therapies had a positive effect on parent behavior at post-treatment and follow-up; no significant improvement was observed for other outcome domains. Problem-solving therapy (PST) improved parent mental health and parent behavior at post-treatment and follow-up. There was insufficient evidence to evaluate cognitive-behavioral and systems therapies for many outcome domains.


Parent- and family-based psychological therapies can improve parent outcomes, with PST emerging as particularly promising. Future research should incorporate consensus statements for outcomes assessment, multisite recruitment, and active comparator conditions.

PMID: 24881048

Spina bifida: a multidisciplinary perspective on a many-faceted condition.

Fieggen G, Fieggen K, Stewart C, Padayachy L, Lazarus J, Donald K, Dix-Peek S, Toefy Z, Figaji A. (2014) Spina bifida: a multidisciplinary perspective on a many-faceted condition.  South African Medical Journal. 2014 Mar;104(3):213-7.

Open spina bifida or myelomeningocele (SBM) is the most common birth defect involving the central nervous system, second only in incidence to congenital cardiac disease. Outcomes in this disorder were poor until the mid-20th century, when modern neurosurgical techniques (closing the lesion and treating hydrocephalus) and treatment for the neuropathic bladder addressed the major causes of mortality, although SBM may still be poorly treated in the developing world. Initial management - or mismanagement - has a profound impact on survival and long-term quality of life.

PMID: 24897828

Fetal surgery for myelomeningocele is effective: a critical look at the whys.

Meuli M, Moehrlen U. (2014) Fetal surgery for myelomeningocele is effective: a critical look at the whys Pediatric Surgery International. 2014 Jun 8. [Epub ahead of print]


Formerly, the disastrous cluster of neurologic deficits and associated neurogenic problems in patients with myelomeningocele (MMC) was generally thought to solely result from the primary malformation, i.e., failure of neurulation. Today, however, there is no doubt that a dimensional additional pathogenic mechanism exists. Most likely, it contributes much more to loss of neurologic function than non-neurulation does. Today, there is a large body of compelling experimental and clinical evidence confirming that the exposed part of the non-neurulated spinal cord is progressively destroyed during gestation, particularly so in the third trimester. These considerations gave rise to the two-hit-pathogenesis of MMC with non-neurulation being the first and consecutive in utero acquired neural tissue destruction being the second hit. This novel pathophysiologic understanding has obviously triggered the question whether the serious and irreversible functional loss caused by the second hit could not be prevented or, at least, significantly alleviated by timely protecting the exposed spinal cord segments, i.e., by early in utero repair of the MMC lesion. Based on this intriguing hypothesis and the above-mentioned data, human fetal surgery for MMC was born in the late nineties of the last century and has made its way to become a novel standard of care, particularly after the so-called "MOMS Trial". This trial, published in the New England Journal of Medicine, has indisputably shown that overall, open prenatal repair is distinctly better than postnatal care alone. Finally, a number of important other topics deserve being mentioned, including the necessity to work on the up till now immature endoscopic fetal repair technique and the need for concentration of these extremely challenging cases to a small number of really qualified fetal surgery centers worldwide. In conclusion, despite the fact that in utero repair of MMC is not a complete cure and not free of risk for both mother and fetus, current data clearly demonstrate that open fetal-maternal surgery is to be recommended as novel standard of care when pregnancy is to be continued and when respective criteria for the intervention before birth are met. Undoubtedly, it is imperative to inform expecting mothers about the option of prenatal surgery once their fetus is diagnosed with open spina bifida.

PMID: 24908159

Thursday, January 2, 2014

The baric probe: a novel long-term implantable intracranial pressure monitor with ultrasound-based interrogation

Limbrick DD Jr, Lake S, Talcott M, Alexander B, Wight S, Willie JT, Richard WD, Genin GM, Leuthardt EC. (2012)  The baric probe: a novel long-term implantable intracranial pressure monitor with ultrasound-based interrogation.  Journal of Neurosurgery: Pediatrics. 2012 Dec;10(6):518-24.


Prompt diagnosis of shunt malfunction is critical in preventing neurological morbidity and death in individuals with hydrocephalus; however, diagnostic methods for this condition remain limited. For several decades, investigators have sought a long-term, implantable intracranial pressure (ICP) monitor to assist in the diagnosis of shunt malfunction, but efforts have been impeded by device complexity, marked measurement drift, and limited instrumentation lifespan. In the current report, the authors introduce an entirely novel, simple, compressible gas design that addresses each of these problems.


The device described herein, termed the "baric probe," consists of a subdural fluid bladder and multichannel indicator that monitors the position of an air-fluid interface (AFI). A handheld ultrasound probe is used to interrogate the baric probe in vivo, permitting noninvasive ICP determination. To assess the function of device prototypes, ex vivo experiments were conducted using a water column, and short- and long-term in vivo experiments were performed using a porcine model with concurrent measurements of ICP via a fiberoptic monitor.


Following a toe region of approximately 2 cm H(2)O, the baric probe's AFI demonstrated a predictable linear relationship to ICP in both ex vivo and in vivo models. After a 2-week implantation of the device, this linear relationship remained robust and reproducible. Further, changes in ICP were observed with the baric probe, on average, 3 seconds in advance of the fiberoptic ICP monitor reading.


The authors demonstrate "proof-of-concept" and feasibility for the baric probe, a long-term implantable ICP monitor designed to facilitate the prompt and accurate diagnosis of shunt malfunction. The baric probe showed a consistent linear relationship between ICP and the device's AFI in ex vivo and short- and long-term in vivo models. With a low per-unit cost, a reduced need for radiography or CT, and an indicator that can be read with a handheld ultrasound probe that interfaces with any smart phone, the baric probe promises to simplify the care of patients with shunt-treated hydrocephalus throughout both the developed and the developing world.
doi: 10.3171/2012.8.PEDS11565

Friday, December 27, 2013

Influenza and congenital anomalies: a systematic review and meta-analysis.

Luteijn JM, Brown MJ, Dolk H. (2013) Influenza and congenital anomalies: a systematic review and meta-analysis. Human Reproduction. 2013 Dec 22. [Epub ahead of print]



Does first trimester maternal influenza infection increase the risk of non-chromosomal congenital anomalies (CA)?


First trimester maternal influenza exposure is associated with raised risk of a number of non-chromosomal CA, including neural tube defects, hydrocephaly, congenital heart defects, cleft lip, digestive system defects and limb reduction defects.


Hyperthermia is a well-established risk factor for neural tube defects. Previous studies suggest influenza may be a risk factor not only for neural tube defects, but also other CA. No systematic review has previously been undertaken.


Systematic review and meta-analysis. A search of EMBASE and PUBMED was performed for English and Dutch studies published up to July 2013. A total of 33 studies (15 case-control, 10 cohort and 8 ecological) were included in the systematic review of which 22 studies were included in the meta-analysis.


A total of 29 542 babies with congenital anomaly (1112 exposed) from case-control studies and 1608 exposed pregnancies resulting in 56 babies with congenital anomaly from cohort studies were included in the meta-analysis. Maternal influenza exposure was defined as any reported influenza, influenza-like illness or fever with flu, with or without serological or clinical confirmation during the first trimester of pregnancy. Data for 24 (sub)groups with congenital anomaly available from ≥3 studies were analysed using the DerSimonian-Laird random effects model. The hypothesis of publication bias was assessed using funnel plots and risk of bias of included studies was assessed using a slightly modified version of the Newcastle-Ottawa scale.


First trimester maternal influenza exposure was associated with an increased risk of any congenital anomaly [adjusted odds ratio (AOR) 2.00, 95% CI: 1.62-2.48], neural tube defects [odds ratio (OR) 3.33, 2.05-5.40], hydrocephaly (5.74, 1.10-30.00), congenital heart defects (1.56, 1.13-2.14), aortic valve atresia/stenosis (AOR 2.59, 1.21-5.54), ventricular septal defect (AOR 1.59, 1.24-2.14), cleft lip (3.12, 2.20-4.42), digestive system (1.72, 1.09-2.68) and limb reduction defects (2.03, 1.27-3.27). An increased risk for cleft lip (but not for cleft palate) was also reported by ecological studies not included in the meta-analysis. Study outcomes reported for 27 subgroups of congenital anomaly could not be included in the meta-analysis. Visual inspection of funnel plots did not suggest evidence for publication bias.


This study enrolled observational studies that can be subject to limitations such as confounding, retrospective maternal exposure reports and non-response of intended participants. Influenza exposed pregnancies can also have been exposed to influenza related medication.


Prevention of influenza in pregnant women may reduce congenital anomaly risk, and would be relevant to more than just neural tube defects. More research is needed to determine whether influenza and/or its related medication is teratogenic, to determine the role of hyperthermia in teratogenicity and the role of other environmental factors such as nutritional status in determining susceptibility.


Funded by the EC, under the framework of the EU Health Programme 2008-2013, Grant Agreement 2010 22 04 (Executive Agency for Health & Consumers). No competing interests.


congenital anomalies, influenza, meta-analysis, observational studies, public health

PMID: 24365800

Thursday, December 26, 2013

Longitudinal mediators of achievement in mathematics and reading in typical and atypical development.

Barnes MA, Raghubar KP, English L, Williams JM, Taylor H, Landry S. (2013) Longitudinal mediators of achievement in mathematics and reading in typical and atypical development. Journal of Expimental Child Psychology. 2014 Mar;119:1-16. 

Longitudinal studies of neurodevelopmental disorders that are diagnosed at or before birth and are associated with specific learning difficulties at school-age provide one method for investigating developmental precursors of later-emerging academic disabilities. Spina bifida myelomeningocele (SBM) is a neurodevelopmental disorder associated with particular problems in mathematics, in contrast to well-developed word reading. Children with SBM (n=30) and typically developing children (n=35) were used to determine whether cognitive abilities measured at 36 and 60months of age mediated the effect of group on mathematical and reading achievement outcomes at 8.5 and 9.5years of age. A series of multiple mediator models showed that: visual-spatial working memory at 36months and phonological awareness at 60months partially mediated the effect of group on math calculations, phonological awareness partially mediated the effect of group on small addition and subtraction problems on a test of math fluency, and visual-spatial working memory mediated the effect of group on a test of math problem solving. Groups did not differ on word reading, and phonological awareness was the only mediator for reading fluency and reading comprehension. The findings are discussed with reference to theories of mathematical development and disability and with respect to both common and differing cognitive correlates of math and reading.

doi: 10.1016/j.jecp.2013.09.006.

The prevalence of small intestinal bacterial overgrowth and methane production in patients with myelomeningocele and constipation.

Ojetti V, Bruno G, Paolucci V, Triarico S, D'aversa F, Ausili E, Gasbarrini A, Rendeli C. (2013) The prevalence of small intestinal bacterial overgrowth and methane production in patients with myelomeningocele and constipation. Spinal Cord. 2013 Nov 19.

Study design:Prospective study.Objectives:The objective of this study was to assess the prevalence of small intestinal bacterial overgrowth (SIBO), methane (CH4) production and orocecal transit time (OCTT) in children affected by myelomeningocele.Setting:This study was conducted at the Catholic University in Rome, Italy.Methods:Eighteen (6M/12F; 16.4±7.6 years) children affected by myelomeningocele were enrolled. All subjects underwent H2/CH4 lactulose breath tests to assess SIBO and OCTT. All patients performed a visual analog scale to investigate abdominal pain, bloating and flatulence, and maintained a diary of the frequency and consistency of the stool during the previous 7 days. A nephro-urological clinical evaluation of the number of urinary tract infections (UTIs) and neurogenic bowel disease score were also performed.Results:Thirty-nine percent (7/18) of the children showed SIBO and 61% (11/18) presented a delayed OCTT. Moreover 44.4% (8/18) produced high levels of CH4. Interestingly, all myelomeningocele children who produced CH4 showed a delayed OCTT and a higher incidence of UTI, with a lower frequency of evacuation, compared with those with a normal or accelerated OCTT.Conclusion:The association between CH4 and constipation suggests that CH4 has an active role in the development of constipation. One of the most interesting features of our study is to identify a correlation between myelomeningocele, CH4, delayed OCTT and UTI. The intestinal decontamination with locally acting drugs in these children may reduce the number of UTIs and improve intestinal motility.

doi: 10.1038/sc.2013.131.