Treatment of pressure sores in spina bifida patients with calcium alginate and foam dressings.

STUDY DESIGN: Prospective study on local treatment of pressure sores using calcium alginate and foam dressings in spina bifida patients. OBJECTIVE: Investigate if this sequential approach is valid and safe for selected patients with neurological impairments. MATERIALS AND METHODS: Using European Pressure Ulcer Grading System, after clinical evaluation of local sore, selected patients of Spina Bifida Center of Rome were treated with sequential calcium alginate and foam dressings for 12 weeks. Pressure ulcere surfaces were measured monthly by ulcer tracing. The endpoints were the mean absolute areas surface reduction during every month and number of patients achieving a 50% or more during study. RESULTS: 14 patients (7 males aged 12-24 years) with spina bifida and pressure sores were treated. Mean and standard deviation of mean surface area reduction were 12.5 ± 7.5 cm 2 at start of the study versus 3.7 ± 5.2 cm 2 after 12 weeks, p < 0.001. 75% of the patients reached mean surface area reduction of 50% during trial. Dressing tolerance was good in every patient. CONCLUSIONS: Calcium alginate and foam dressings are valid and safe approach in the treatment of pressure sores in selected patients with spina bifida. In fact, they protect the wound and create an environment favorable to healing.

Transanal irrigation in myelomeningocele children: an alternative, safe and valid approach for neurogenic constipation.

STUDY DESIGN: A total of 60 children with myelomeningocele referred to Spina Bifida Center of Rome (31 boys and 29 girls; aged 8-17 years) were treated with transanal irrigation for three months. OBJECTIVE: To investigate whether transanal irrigation is a valid and alternative approach for neurogenic constipation in children with myelomeningocele. METHODS: A questionnaire on bowel disturbances, quality of life and side effects was completed before the beginning and at the termination of the study. SETTING: Italy. RESULTS: About 60% (36/60) of patients reported relief from constipation and 75% (12/16) for fecal incontinence. Wheelchair-bound and walking patients showed same high improvement of bowel habit. Mean (s.d.) scores before and after the study were: neurogenic bowel dysfunction total score: 17.5 (5.2) versus 8.5 (4.3) (P<0.001); digital stimulation of anorectum: 4.2 (2.8) versus 1.3 (2.5) (P<0.01); frequency of fecal incontinence: 5.5 (1.2) versus 1.3 (1.7) (P<0.01) and degree of general satisfaction: 3.0 (2.4) versus 7.7 (1.5) (P<0.001).We observed a reduction of urinary tract infections during the course of treatment: 14 total urinary tract infections (9 caused by Escherichia coli) before versus 6 (3) during treatment (P<0.01). CONCLUSION: Transanal irrigation in children with myelomeningocele is an alternative and relatively safe approach for managing neurogenic constipation; in fact, it improves bowel disturbances, quality of life and seems to reduce the risk of urinary tract infections.

Neurotrophic factor expression in newborns with myelomeningocele: preliminary data.

BACKGROUND: Neurotrophic factors play a crucial role in the stimulation of sprouting, synaptic plasticity and reorganization after spinal cord damage. The aim of this study was to investigate the expression of some neurotrophic factors [brain derived neurotrophic factor (BDNF), glial derived neurotrophic factor (GDNF), and nerve growth factor (NGF)] in the cerebrospinal fluid (CSF) of newborns with myelomeningocele (MMC) and to determine their correlations with this malformation. METHODS: To measure the expression of BDNF, GDNF, and NGF, we collected CSF samples of six newborns during the neurosurgical operation to correct the open MMC and of 10 matched controls. Endogenous neurotrophic factor levels were quantified using a two-site immuno-enzymatic assay. The statistical analysis was performed using the Mann-Whitney two-tailed two-sample test. FINDINGS: In the CSF of patients analysis of neurotrophic factor expression showed a significant increase of BDNF, GDNF, and NGF compared to the mean level of the control group (445.8+/-82.3, 86.5+/-2.6, and 59.9+/-6.2 pg/mL, respectively, respect to 10.2+/-5.9, 19.9+/-11.3, and 15.3+/-2.6 pg/mL) (p<0.001). INTERPRETATION: Our study shows an over-expression of neurotrophic factors in the CSF of newborns with MMC. This neurotrophin up-regulation may stimulate axonal sprouting and synaptic reorganization of the damaged neural cells at the site of spinal cord lesion. The neurotrophic factor up-regulation may represent a particularly important biochemical markers of spinal cord damage and might be associated with the severity of spine injury in MMC patients.

Bone mineral density and body composition in a myelomeningocele children population: effects of walking ability and sport activity.

Myelomeningocele causes serious locomotor disability, osteoporosis and pathologic fractures. The aim of this study was to investigate the relationship between body composition, bone mineral density, walking ability and sport activity in myelomeningocele children. 60 patients aged between 5 and 14 yrs with myelomeningocele (22 ambulatory and 38 non-ambulatory), were studied. Fat mass and fat-free-mass were calculated by anthropometry. The bone mineral density at lumbar and femoral neck were evaluated. Bone mineral density at the lumbar and femoral neck was lower than in the normal population. In the non-ambulaty group, bone mineral density was approximately 1 SD lower than in the ambulatory one (p < 0.01). Fat mass was greater than expected but without significantly differences between walking group (mean 26%) and wheel-chair users (25%). Patients practised sport activity had a better bone mineral density and body fat compared with other patients with the same disability. Patients with myelomeningocele have decreased bone mineral density and are at higher risk of pathologic bone fractures. All subjects showed an excess of fat as percentage of body weight and are shorter than normal children. The measurement of bone mineral density may help to identify those patients at greatest risk of suffering of multiple fractures. Walk ability and sport activity, associated with the development of muscle mass, are important factors in promoting bone and body growth, to reduce the risk of obesity and of pathological fractures.

Loperamide cause of prolonged urinary retention after acute gastroenteritis.

UNLABELLED: Urinary retention is uncommon in children and only one case has been described in literature with loperamide treatment. We report the occurrence of prolonged urinary retention in a 10-years-old girl after receiving oral loperamide for an acute gastroenteritis. The first episode of urinary retention lasted for 24 hours; radiological evaluation (Magnetic Resonance, cistography and renal ultrasound) did not find abnormality; it was necessary deplete bladder with catheterism. Previous to the gastroenteritis, diuresis has always been regular and she did not suffer for any neurological or urinary problems. Patient was submitted to, without success, percutaneous posterior tibial nerve stimulation (SANS) and recovered after one year clean intermittent catheterization. CONCLUSION: In a period of increasing popularity of self-medical therapy for common children's diseases, paediatricians should be aware of the potential, rare and chronic effects of this type of treatment. Moreover our case suggests that reaction to loperamide should be added to the etiological list of prolonged urinary retention in young patients.

Prevalence of latex allergy in spina bifida: genetic and environmental risk factors.

AIM OF STUDY: To evaluate the prevalence of latex allergy in a population of children with spina bifida (SB) and to assess the role of early exposure to latex products and others risk factors. INTRODUCTION: SB is related with an higher incidence of latex allergic reactions. These patients received repeated surgical procedures, implant of latex-containing materials and catheterization. MATERIALS AND METHODS: Eighty consecutive subjects affected with SB besides answering a questionnaire, underwent a skin-prick test (SPT) to latex and the determination of the specific serum IgE (RAST CAP) to latex. 40% (32/80) of the patients showed a latex sensitization with specific IgE > 0.7 kU/I but only twelve of the 32 sensitized patients (40%) suffered from clinical reactions to latex (urticaria, conjunctivitis, angioedema, rhinitis, bronchial asthma). Number of surgical procedures, but particularly early exposure to latex and familiarity for allergy are correlated with latex allergy (p < 0.01). CONCLUSION: Latex allergy in SB children is multifactorial situation related with a disease-associated propensity for latex sensitization, early exposure and number of surgical procedures. Prophylactic measures to avoid the exposure, not only in the sanitary environment, through the institution of latex-safe routes and every day, prevent potentially serious allergic reactions.

Multidimensional study on quality of life in children with type 1 diabetes.

AIM: To study the Health Related Quality of Life (HRQoL) and metabolic assessment in 33 children affected with type 1 diabetes (18 males, 15 females; mean age 10.3 years). METHODS AND RESULTS: We used the Child Health Questionnaire-Parental Form 50 items (CHQ-PF50), measurements of metabolic control and we related them to patient management and family status. Quality of life (QoL) in diabetic children was worse than in the healthy sample. Interestingly, mean and last glycosylated hemoglobin (mean HbAlc r: -.4410 p < .01 and last HbAlc r: -.4012 p < .01), age of patients (r: -.4428; p < .009) and number of glycaemia controls (r: -.37, p < .03) were the most important parameters related to HRQoL parameters. CONCLUSION: This multidimensional study stressed that HRQoL is influenced by the metabolic assessment. Moreover, the report examined the parental perception of QoL in children with chronic diseases. Higher number of glycaemia controls/day, better metabolic control, lower age of children and earlier onset of diabetes produced better physical and psychological aspects of QoL. In comparison with adolescent patients, in children with diabetes, factors as number of insulin injections and daily snacks, and the level of education of the mother were not so important to influence QoL. Unexpectedly, in this sample, life habits, family features, and anthropometric parameters did not correlate with specific domains of QoL.

Polyethylene glycol 4000 vs. lactulose for the treatment of neurogenic constipation in myelomeningocele children: a randomized-controlled clinical trial.

AIM: To compare the therapeutic effectiveness and tolerability of low daily doses of polyethylene glycol 4000 vs. lactulose in the treatment of neurogenic constipation in children with myelomeningocele. METHODS: Sixty-seven children with chronic neurogenic constipation were randomized allocated to receive either polyethylene glycol 4000 (0.50 g/kg) or lactulose (1.5 g/kg) for 6 months. Patients or their parents reported frequency and modality of evacuation and side effects on a diary card. Primary outcome was bowel frequency > or =3/week, and the second one was side effects at the end of treatment. RESULTS: Complete remission of constipation was reported by a significantly (P < 0.01) higher number of patients treated with polyethylene glycol compared with lactulose. At the end of the study, 46% patients of polyethylene glycol group and 22% of the lactulose group were asymptomatic. Compared with lactulose, patients treated with polyethylene glycol reported higher bowel frequency (5.1 vs. 2.9 bowel movements/week, P < 0.01) and reduction of encopresis. Neither lactulose nor polyethylene glycol caused clinically-significant serious side effects and palatability was similar. CONCLUSIONS: Polyethylene glycol 4000 compared with lactulose provided a higher success rate, without significant side effects, for the treatment of constipation in myelomeningocele children.

Acute cholestasis: atypical onset of Kawasaki disease.

Atypical onset of Kawasaki disease (KD) is a frequent problem leading to diagnostic mistake. Acute cholestasis and liver involvement occur occasionally as minor manifestation of KD. We report the case of a 6-year-old boy presenting fever, jaundice, abdominal pain, and ascites who subsequently developed typical KD clinical pattern just at the same time of echocardiographic coronary arteries anomalies. Abdominal radiological evaluation was normal and seroimmunologic markers resulted negative. Shortly after intravenous immunoglobulin and acetylsalicylic acid administration the clinical features disappeared. KD should be considered in differential diagnosis in children with cholestasis, abdominal pain and fever of unknown etiology.

Urodynamic evaluation in children with lipomeningocele: timing for neurosurgery, spinal cord tethering and followup.

PURPOSE: We assessed the usefulness of urodynamic testing for determining the optimal timing of surgery and for evaluating the development of bladder function in children with lipomeningocele. MATERIALS AND METHODS: We retrospectively evaluated 64 patients (40 females) 3 to 17 years old (mean 8.5) with lipomeningocele. Patients were divided into 3 groups based on age at surgery, ie younger than 12 months (34 patients), 12 to 36 months (17) and older than 36 months (13). All patients underwent urodynamic testing preoperatively and during extended followup (mean 6.5 years, range 3 to 12). RESULTS: Bladder capacity and mean detrusor leak pressure improved in all groups but particularly in patients operated on within the first year of life. At the end of the study mean bladder capacity was 420 cc in patients younger than 12 months, 300 cc in those 12 to 36 months old and 260 cc in those older than 36 months (p <0.01), and mean detrusor leak pressure was 37, 54 and 55 cm H(2)O, respectively (p <0.01). At the latest followup 65% of patients in the youngest group had improved urodynamic parameters vs 33% of those 12 to 36 months old and 28% of those older than 36 months. CONCLUSIONS: Urodynamic evaluation and the presence of neurological impairment have crucial roles in determining the optimal timing of surgery in patients with lipomeningocele, and in diagnosing the onset of tethered cord. Our data show that early surgical repair seems to reduce the risk of neurological deterioration of the lower urinary tract, and allows a more physiological development of urinary function.