Deliberate termination of life of newborns with spina bifida, a critical reappraisal

Objects: Deliberate termination of life of newborns (involuntary euthanasia) with meningomyelocele (MMC) is practiced openly only in the Netherlands. 'Unbearable and hopeless suffering' is the single most cited criterion for this termination, together with the notion that 'there are no other proper medical means to alleviate this suffering'. In this paper, both (and other) statements are questioned, also by putting them in a broader perspective. Methods: First, a historical overview of the treatment of newborns with MMC is presented, concentrating on the question of selection for treatment. Second, a thorough analysis is made of the criteria used for life termination. Third, a case of a newborn with a very severe MMC is presented as a 'reference case'. Conclusion: 'Unbearable and hopeless suffering' cannot be applied to newborns with MMC. They are not 'terminally ill' and do have 'prospects of a future'. In these end-of-life decisions, 'quality of life judgments' should not be applied. When such a newborn is not treated, modern palliative care always will suffice in eliminating possible discomfort. There is no reason whatsoever for active life-termination of these newborns

Determinants of bone mineral density, bone mineral content, and body composition in a cohort of healthy children: Influence of sex, age, puberty, and physical activity

Interventions directed to the recognition of abnormal bone mineral density, bone mineral content, and body composition in the pediatric age require the definition of factors influencing bone mass acquisition during growth. We have evaluated in a cross-sectional manner by dualenergy X-ray absorptiometry the impact of sex, age, puberty, and physical activity on total body areal bone mineral density, regional (lumbar and femoral) bone mineral densities, bone mineral content, and body composition (fat mass and lean mass) in a cohort of 359 healthy Italian children aged 3-14 years and investigated their specific contribution to bone mass accrual. Statistical multiple regression analysis was performed dividing the population in pre- and post-pubertal groups. Bone mineral density at the lumbar spine has resulted equally distributed in both sexes before puberty while has resulted higher at the femoral necks in males at whatever age. A significant effect on bone mass acquisition was exerted by male sex and lean mass. In the areas where the cortical bone is prevalent, males of the pre-pubertal group have presented the highest values; in the areas where the cancellous bone is prevalent, both sexes were equivalent until the age of 9 years, but after this age, females have presented higher increases, probably related to the inferior dimensional development of lumbar vertebrae. Conclusively, male sex and lean mass seem to represent independent predictors of bone mass accrual in the cortical bone of the examined children, while female sex and pubertal maturation are independent predictors of bone mass accrual in the trabecular bone. © Springer-Verlag 2011

Contrast enhanced gray scale and color Doppler voiding urosonography versus voiding cystourethtography in the diagnosis and grading of vesicoureteral reflux

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

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

Serum interleukin-18 in children with henoch-schönlein purpura: A promising marker of disease activity?

Henoch-Schönlein purpura (HSp) is the most common systemic vasculitis of childhood with typical skin involvement and concurrent signs involving joints, gastrointestinal tract, and kidney. HSp pathogenesis is still far from being completely understood, though a knotty cytokine complex is believed to contribute to its intimate processes. The aim of our evaluation is to establish the relationship between serum levels of interleukin (IL)-18 and disease outcome and establish its feasibility to provide a marker of disease activity or even a prognostic tool in clinical practice. We examined clinical/laboratory variables and serum IL-18 in 17 children hospitalized during a year for HSp, diagnosed by EULAR/PRINTO/PRES criteria; the same patients were re-evaluated after 6 months. All results were compared with 25 age-matched healthy controls. IL-12 and IL-6 were also evaluated in a cohort of the same patients and compared with controls. General and clinical variables (sex, edema of the extremities, gastrointestinal or renal complications, relapses and renal involvement at 6 months) had no relationship with cytokine levels. Serum IL-18 and IL-6 levels were found significantly increased at diagnosis in HSp patients when compared with healthy controls. After 6 months, serum IL-18 and IL-12 levels were significantly decreased in patients, while IL-12 and IL-6 levels were significantly increased compared to healthy controls. Though preliminary and expecting further confirmation on a larger sample, our data support the conclusion that serum IL-18 levels reflect HSp activity

Longitudinal study of microvascular involvement by nailfold capillaroscopy in children with Henoch-Schönlein purpura

The aim of this study is to describe by video-nailfold capillaroscopy the microvascular involvement and capillary changes in children with Henoch-Schönlein purpura (HSp) and to establish a possible correlation with clinical outcome. Thirty-one patients underwent capillaroscopic evaluation through a videomicroscope during the acute phase and after 6 months. Twenty sex/age-matched controls were also examined. All capillaroscopic variables were statistically examined in combination with laboratoristic/clinical data. Architectural and morphological changes recorded during the acute phase were statistically significant in comparison to the controls (p < 0.01). At the follow-up, oedema was still observed in all patients, whereas, morphological changes only in two. There was a no significant correlation between capillaroscopy changes, laboratoristic/clinical data, and outcome. Video-nailfold capillaroscopy can be a simple tool to evaluate microvascular abnormalities in the acute phase of HSp, and the persistence of oedema could suggest an incomplete disease resolution at a microvascular level

Neuromotor rehabilitation in spina bifida: the need of randomized controlled trials

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Occult spinal dysraphisms in newborns with skin markers: role of ultrasonography and magnetic resonance imaging

Purpose: The purpose of this paper is to investigate occult spinal dysraphisms (OSD) using lumbar ultrasonography (LUS) in newborns presenting with specific skin markers or sacrococcygeal dimple. Method: From 2012 to 2015, we performed LUS in newborns with cutaneous stigmata and/or sacroccygeal dimple. Magnetic resonance imaging (MRI) was performed in all patients with abnormal ultrasound or features of neurological involvement in order to detect spinal lesions. Results: We prospectively evaluated 475 newborns who presented cutaneous stigmata performing LUS during their 4 weeks of life though 439 completed the study. All patients had a follow-up of almost 12 months. Of these, 39 presented abnormal ultrasonography and underwent MRI. In this group, spinal dysraphism was confirmed in 12 patients. When considering skin markers, dermal sinus correlated with higher risk of spinal cord lesions, on the other hand the presence of simple sacral dimple alone denoted a very low risk of occult spinal dysraphism. The simultaneous presence of more skin markers and/or the presence of lumbar ultrasonography abnormality regarding the level of the conus, pulsatility, and the position of the cord, thickness of the filum terminale, or the presence of an intratecal mass, lipoma, or dermal sinus tract indicated the necessity to perform MRI in order to detect spinal cord abnormalities because of higher risk of spinal lesions. Conclusion: LUS in newborns with specific skin markers is a valid method to select patients in which MRI can be performed to detect OSD. The presence of a simple sacral dimple alone is a negligible marker for occult neural pathology while the presence of isolated dermal sinus or more than one cutaneous marker could be considered indicative of higher risk of spinal dysraphism

Dietary approach to prevent obesity risk in Spina Bifida patients

PURPOSE: The aim of this prospective, analytic study is to evaluate if dietary approach can improve the body mass index (BMI) in a total of N\u2009=\u2009152 patients with SB. METHODS: BMI levels were evaluated stratifying patients by gender and age classes. Patients with BMI\u2009 65\u200925 have been randomized (1:1) in two groups: the "diet" group that received a dietary program and the "no diet" group that did not receive any program. Patients have been observed at the beginning of the study (T0), and again at the end of the study, 1 year later (T1). The main objective of the study was to evaluate BMI score in SB patients and how it could be influenced by dietary changes. RESULTS: A total of 36.8% patients were classified as overweight or obese. Females present a mean BMI level higher than male, and patients older than 20 years old present the highest mean BMI. The "diet" group BMI decreased from 29.7 (\ub1\u20093.8) to 27.7 (\ub1\u20093.7) during the year of program. The mean BMI in the "no diet" group decreased from 30.3 (\ub1\u20094.6) to 29.2 (\ub1\u20094.7). There was a statistically significant difference in BMI level between groups (p\u2009<\u20090.0005). There was a statistically significant effect of time on BMI levels for the "diet program" group (p\u2009=\u20090.001), and there was NOT a statistically significant effect of time on BMI levels for the "no diet group" (p\u2009=\u20090.053). CONCLUSIONS: Spina Bifida population has high risk of obesity which is related to other comorbidities such as diabetes and hypertension for example. Specific dietary program, since pediatric age, correlates with an improvement of quality of life, a reduction of BMI and of risk of related diseases with obesity. This study confirms that the transition to adulthood marks the beginning of the overweight status for many SB patients, but it also demonstrates that, following a dietary program, even disabled patients with limited training capabilities can achieve a BMI reduction

Post-natal ultrasound morpho-dynamic evaluation of mild fetal hydronephrosis: a new management

BACKGROUND: Fetal hydronephrosis is the most common anomaly detected on antenatal ultrasound examination, affecting 1-5% pregnancies. AIM: A new management in mild antenatal renal pelvis dilatation (ARPD), using a technique based on both morphological and dynamical evaluation. MATERIALS AND METHODS: Prospective study conducted during a 36-months period in 180 consecutive newborns referred as having mild ARPD. Examinations consisted in a morphological ultra-sound (US) scan evaluating antero-posterior diameter, renal parenchyma, ureteral evidence and pelvis morphology and, subsequently, a dynamic evaluation to analyze any change of the urinary tract during bladder voiding. All children were evaluated both at 3rd day and 1 month after birth. They were divided among those with negative examinations and those with at least one positive scan, trying to discriminate within the latter, children suspected for transient pyelectasis from those suspected for organic pathology. RESULTS: 108 patients had normal US findings both at birth and at 1 month. The remaining 72 babies had at least one abnormal US examination: 54 were suspected for transient pyelectasis, while 18 suspected for organic pathology. At the end of the study, 61 babies (33.9%) had final diagnosis of transient pyelectasis and 11 cases (6.1%) of organic pathology. At one month the dynamic pattern of US findings had the highest negative predictive value, while renal parenchyma evaluation has the highest accuracy. CONCLUSIONS: a dynamic US approach allowed to better select among infants suspected for transient pyelectasis from those suspected for organic pathology, avoiding unnecessary and invasive examinations in healthy babies