Myelomeningocele: Long-Term Neurosurgical Management.

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.

Reliability and reproducibility analysis of the AOSpine Sacral Fractures Classification System by spinal and pelvic surgeons.

STUDY DESIGN: Retrospective Cohort Study OBJECTIVES: The AOSpine Sacral Classification System was proposed as a comprehensive and universally accepted new classification for Sacral Fractures, and was recently internally validated. However, an external, independent and multidisciplinary reports on validation of this classification is lacking. Aim of the present study is to analyze the interobserver reliability and intraobserver reproducibility of the AOSpine Sacral Classification System for Sacral Fractures between orthopedic spinal and pelvic surgeons with different levels of experience. METHODS: Our institutional database was searched to retrieve patients with acute, traumatic sacral injury admitted from June 2017 to June 2020. For each patients, X-Rays and CT scans were collected. Three Orthopedic Pelvic Surgeons (Group A) and three Spine Surgeons (Group B), with different level of experience (Junior, 〈 5 years; Middle, 5-10 years; Expert 〉 10 years) independently classified all the sacral fractures included in the dataset, with two separate evaluation three weeks apart. Both intra and interobserver reliability were calculated with k-coefficient. RESULTS: Overall, 150 patients were included in the final dataset, for a total of 1800 different assessments, with all the subtypes reported. The intraobserver reproducibility for the whole group was substantial (κ=0.72). Overall, the interobserver reliability was moderate, with a κ=0.57. When only fracture type was taken in account, the κ value became substantial (κ=0.62). No significant differences were found comparing group A and group B (0.55 vs κ 0.55, p>0.05). No significant differences according to surgeon's experience were found; however, the κ value was slightly lower among the junior surgeons. CONCLUSIONS: Our findings confirmed the reliability and reproducibility of this classification in clinical practice. In the current study the surgeon's expertise (pelvic and spinal trauma) and the level of experience does not influence the reliability of the classification system.

The management of emergency spinal surgery during the COVID-19 pandemic in Italy.

AIMS: The current pandemic caused by COVID-19 is the biggest challenge for national health systems for a century. While most medical resources are allocated to treat COVID-19 patients, several non-COVID-19 medical emergencies still need to be treated, including vertebral fractures and spinal cord compression. The aim of this paper is to report the early experience and an organizational protocol for emergency spinal surgery currently being used in a large metropolitan area by an integrated team of orthopaedic surgeons and neurosurgeons. METHODS: An organizational model is presented based on case centralization in hub hospitals and early management of surgical cases to reduce hospital stay. Data from all the patients admitted for emergency spinal surgery from the beginning of the outbreak were prospectively collected and compared to data from patients admitted for the same reason in the same time span in the previous year, and treated by the same integrated team. RESULTS: A total of 19 patients (11 males and eight females, with a mean age of 49.9 years (14 to 83)) were admitted either for vertebral fracture or spinal cord compression in a 19-day period, compared to the ten admitted in the previous year. No COVID-19 patients were treated. The mean time between admission and surgery was 1.7 days, significantly lower than 6.8 days the previous year (p < 0.001). CONCLUSION: The structural organization and the management protocol we describe allowed us to reduce the time to surgery and ultimately hospital stay, thereby maximizing the already stretched medical resources available. We hope that our early experience can be of value to the medical communities that will soon be in the same emergency situation. Cite this article: Bone Joint J 2020;102-B(6):671-676.

Application of absolute qPCR as a screening method to detect illicit 17ß-oestradiol administration in male cattle.

It has been previously demonstrated that the progesterone receptor gene is up-regulated in the sex accessory glands of pre-pubertal and adult male bovines after 17ß-oestradiol treatment. In the present study, a qualitative screening method was optimised to detect 17ß-oestradiol treatment using absolute quantification by qPCR of the progesterone receptor gene to determine the amount of gene expression in bulbo-urethral glands. An external standard curve was generated and developed with TaqMan® technology. Based on two in vivo experiments, the decision limit CCα, sensitivity and specificity of this screening method were established. Trial 1 consisted of 32 Friesian veal calves divided into two groups: group A (n = 12), consisting of animals treated with four doses of 17ß-oestradiol (5 mg week(-1) per animal); and group B (n = 20), consisting of control animals. Trial 2 was performed on 26 Charolaise beef cattle that either received five doses of 17ß-oestradiol (group C; 20 mg week(-1) per animal; n = 6) or remained untreated (group D; n = 20). Further, progesterone receptor gene expression was evaluated in beef and veal calves for human consumption. A specific CCα on 20 Piedmontese control beef cattle was calculated to include these animals in a field investigation. Five out of 190 beef cattle and 26 out of 177 calves tested expressed the progesterone receptor gene above their respective CCα and they were classified as being suspected of 17ß-oestradiol treatment. Additionally, 58% of veal calves that tested suspect via qPCR exhibited histological lesions of the bulbo-urethral gland tissue, which are typical of oestrogen administration and are consistent with hyperplasia and metaplasia of the glandular epithelium.

Thymus atrophy and regeneration following dexamethasone administration to beef cattle.

Thymus atrophy and regeneration were studied in 13- to 22-month-old beef calves treated with dexamethasone (DMT), using anabolic dosages and implementing different withdrawal times. Two trials were conducted. In trial 1, group A (n=6) received 0.7 mg/day DMT orally for 40 days, group B (n=6) received 1.4 mg/day orally for 40 days and group C (n=6) was the control. In trial 2, group D (n=6) received 0.7 mg/day DMT orally for 40 days, group E (n=6) received 1.4 mg/day orally for 40 days and group K (n=6) was the control. DMT withdrawal times before slaughter were six days (groups A and B) and 26 days (groups D and E). At slaughter, thymus atrophy was severe and progressive in animals from groups A and B. In contrast, thymus weight and volume of the animals from groups D and E were almost normal. Slight atrophy was also detected in the calves in these groups. Histological changes and Ki67 immunostaining revealed a large number of positive lymphoid cells, mostly in the cortical area, associated with higher expression of apoptosis in the medulla compared with controls. This demonstrated that the thymus of beef cattle is still able to regenerate following DMT administration.

Effects of anabolic and therapeutic doses of dexamethasone on thymus morphology and apoptosis in veal calves.

Three groups of 10 veal calves were treated, respectively, with 5 mg of dexamethasone-21-isonicotinate administered intramuscularly on days 0 and 7 (group A); 0.4 mg/day of dexamethasone-21-phosphate administered orally for 20 days (group B); or left untreated as controls (group C). Two animals from each group were slaughtered on day 3, 7, 14, 32 and 52. The size and weight of the thymus decreased progressively in both treated groups until day 32. On day 14, in comparison with the controls, there was a mean reduction of 76 per cent in the thymus weight of group A and 35 per cent in group B. On day 32, the reductions were 13 per cent in group A and 50 per cent in group B, but the thymus weight of both groups had recovered completely by day 52. Dexamethasone-induced changes in thymus weight associated with lymphoid depletion and fat replacement, and there were clear correlations between these changes and apoptosis of thymocytes.

Optimized architecture for nutrition in the avascular retina of Megachiroptera.

Investigations were undertaken to evaluate the unique choroidal vascular system of the Megachiroptera (fruit bats) and its possible significance for retinal nutrition. Fluorescein angiography was performed and documented on Pteropus poliocephalus. Vascular casts were made of the eye of Pteropus scapulatus. Histologic evaluation was performed on P. scapulatus. Results confirmed that each papilla has a vascular core, and a unique vascular system emanating from the optic disc. The histological appearance of the choroid and retina of P. scapulatus confirmed a vascular core to the papillae with a thin, but definite Bruch's membrane. Megachiroptera have a unique vascular system to supply nutrition to the retina consisting of capillary loops within a dense, uniform mosaic of choroidal projections or papillae, which permit the diffusion of metabolites to the retina and a heretofore undescribed vascular tuft emanating from the optic disc. We suggest that this vascular system provides nutrition by diffusion to a thick avascular retina, without any shadowing by vessels, and allows for nocturnal visual acuity and light-gathering capabilities.

Italian multicentre open trial on DDAVP spray in nocturnal enuresis.

OBJECTIVE: The aim of the study was to compare the efficacy and safety of different doses of DDAVP spray treatment (20 to 40 mcg/day) in patients with primary monosymptomatic nocturnal enuresis (defined as three or more wet nights per week). MATERIAL AND METHODS: 237 patients (152 males, 75 females; age range 5-17 years), with no infections or organic abnormalities of the urinary apparatus and no neurological disorders, were admitted into the trial. The experimental design was planned as an "open study" with five different treatments schedules (5 groups). The daily doses of DDAVP at bedtime in groups 1 and 2 were 20 and 30 mcg, respectively, for 6 weeks. In groups 3 and 4 the daily doses for the first 2 weeks were 20 and 30 mcg, respectively, and then, after a washout period of 2 weeks, the daily doses for the two groups were 30 and 20 mcg, respectively. A dose-response study (20 to 40 mcg/day) was carried out in group 5. RESULTS: DDAVP spray therapy in primary monosymptomatic nocturnal enuresis was found to be resolutive in 70-75% of treated patients. No difference in response was found between the patients treated with the daily dose of 20 and those on 30 mcg. No important reactions were observed in patients treated with DDAVP spray at the different daily dose (20 to 40 mcg) or for different periods of time (up to 6 weeks). CONCLUSIONS: DDAVP spray therapy at a dose of 20 mcg/day was effective in 70-75% of primary monosymptomatic nocturnal enuretics. In non-responders the daily dose of DDAVP should be increased to 30 to 40 mcg.

A possible correlation between the blood leukocyte formula and the use of glucocorticoids as growth promoters in beef cattle.

Clinical analyses designed to set welfare parameters were performed on blood drawn from the caudal vein of 14 groups of cattle (young bulls and heifers) (n = 10) from 480 to 550 kg b.w., each group representative of a different farm. The leukocyte formula exhibited a lymphocytopenia in four groups compared with the values from a control group (n = 50). This finding was related to the possible illicit use of corticoids as growth promoters in meat production. The individual plasmas tested negative by two different ELISA kits for corticosteroids, but chemical analyses by LC-MS/APCI (detection limit 0.5 ng/ml) on the pooled plasma of each of the 14 groups revealed the presence of beclomethasone and fluocinolone acetonide in 3 of the 4 suspect farms. These corticosteroids are not always efficiently screened by commercially available immunoassays. The epidemiological reliability of blood analysis as a screening test for such drugs is discussed in the light of the need for quality certification of the whole meat production processes 'from farm to fork', and for enhanced animal welfare.

An Italian epidemiological multicentre study of nocturnal enuresis.

OBJECTIVE: To estimate the prevalence of enuresis in schoolchildren in Italy. SUBJECTS AND METHODS: The Italian Club of Nocturnal Enuresis promoted a prevalence study of nocturnal enuresis using a self-administered questionnaire in seven cities in Northern, Central and Southern Italy. The association between enuresis and potential risk factors, e.g. a family history of enuresis, stress, socio-economic status and abnormal diurnal voiding habits, was investigated. The perceived impact on the child and on the family was also evaluated. A random-cluster sampling scheme was used to obtain a sample of primary and secondary schoolchildren from each city. One primary school and one secondary school for each socio-economic level was sampled in each city, giving a total of 42 schools surveyed; 9086 children were covered by the survey. In a cluster sampling method, the variance of prevalence is divided into two components, binomial and extra-binomial variability. Both the DSM III and DSM IV definitions of enuresis were used because at present, there is no consensus on the diagnostic criteria. RESULTS: Completed questionnaires were received from 7012 children, an overall response rate of 77.2%. Those aged 6-14 years were analysed, restricting the sample to 6892 children. There were 250 enuretic children using the DSM III definition of enuresis and 112 using the DSM IV definition. The overall prevalence was 3.88% and showed a decreasing trend with increasing age. Bedwetting was more frequent in boys than in girls. The prevalence of enuresis was higher when the child was from a family of low socio-economic status despite the child's age group. The logistic analysis showed that familiality, stress, birthweight, age of attaining diurnal continence, soiling and, for girls, menstruation, were statistically significant variables and thus contributed to predicting the probability of bedwetting, confirming the findings of previous studies. There was a large difference in prevalence using the two DSM definitions; a high percentage of DSM III enuretic children had more than two wet nights per week. CONCLUSION: It is important that a consensus about the 'working definitions' of enuresis is reached to avoid bias in the recruitment step, to carry out comparable epidemiological studies and to obtain adequate therapeutic responses.

[Protective effect of oxatomide in infantile bronchial asthma. Double-blind study vs placebo]. / Effetto protettivo dell'oxatomide sull'asma bronchiale infantile. Studio in doppio cieco verso placebo.

BACKGROUND: This study has been designed to assess the protective effect of oxatomide in allergic bronchial asthma of the seasonal type in young children. METHODS: The study was carried out in a paediatric clinic; sixteen children divided into two balanced groups took oxatomide in an oral suspension at the dosage of 1 mg/kg/day, or placebo for a period of 2 months. Eight patients (7 males, 1 female), aged 22 months +/- 2.83 (mean +/- SD) took oxatomide in an oral suspension at the dosage of 1 mg/kg/day, while the other eight (3 males, 5 females; 22.13 months +/- 3.48) took placebo. Efficacy was assessed by monitoring cough, dyspnea at rest, dyspnea following exercise, wheezing, sleep disorders at baseline and after 15, 30 and 60 days of treatment, on the basis of a semiquantitative scale. All side effects were recorded. RESULTS: Persistent coughing was significantly reduced (p < 0.05) after two weeks' treatment with oxatomide. Sleep disorders and other symptoms remarkably improved. Dyspnea at rest and following exercise disappeared after 15 days' therapy, while the intensity of wheezing decreased after 30 days' active treatment. In all parameters examined, oxatomide was significantly more active than placebo at the first examination (p < 0.05 and p < 0.01). Oxatomide was well tolerated and only 2 patients complained of drowsiness which required a reduction in dosage. CONCLUSIONS: Oxatomide, at the dose of 1 mg/kg/day, obtained a good control of respiratory symptoms.

Is the sugar intestinal permeability test a reliable investigation for coeliac disease screening?

BACKGROUND: The lactulose/mannitol (L/M) intestinal permeability test is a simple, non-invasive screening test for coeliac disease. The reliability of the L/M test has so far only been tested in selected groups of patients with coeliac disease. AIM: To evaluate the reliability of the L/M test in a group of patients with coeliac disease who had been diagnosed during mass serological screening of the general population. PATIENTS AND METHODS: Twenty nine patients with coeliac disease detected by screening and 54 age matched coeliac disease free controls aged 11-15 years underwent an L/M test with 5 g lactulose and 2 g mannitol in isotonic aqueous solution. Urinary sugars were measured by high performance liquid chromatography. RESULTS: The median % urinary recovery of lactulose (lactulose UR) was significantly higher in patients with coeliac disease than in controls (0.63 v 0.18, p < 0.001). The mean mannitol % UR was lower in patients with coeliac disease than in controls (17.6 v 18.5) but the difference was not significant. The median urinary L%/M% ratio was significantly higher in patients with coeliac disease than in controls (0.038 v 0.014, p < 0.001). However, 16 of the 29 patients with coeliac disease showed an L%/M% ratio within normal limits (< 0.044). CONCLUSIONS: The L/M intestinal permeability test is not a valuable tool for screening of coeliac disease in the general population. The pattern of the urinary probe recovery suggests that many patients with coeliac disease could remain symptomless because the extent of their intestinal mucosal damage is small ("short" coeliac disease).

Lactose, oligosaccharide and monosaccharide content of milk from mothers delivering preterm newborns over the first month of lactation.

BACKGROUND: Monosaccharide, lactose and oligosaccharide content of milk from mothers delivering prematurely (PT milk) was studied to evaluate whether changes occur during lactation, as observed in milk from mothers delivering at term (T milk). METHODS: To study a homogeneous population, women having the most common phenotype (secretory both Lewis and A, B, or H phenotype) were selected. Milk samples from 26 mothers who delivered between the 27th and 35th week of gestation were collected at the 4th, 10th, and 30th post-partum days. Monosaccharides, lactose and oligosaccharides were measured by high-pressure liquid chromatography. RESULTS: Lactose concentration increased significantly (p < 0.05) from 52.81 +/- 8.2 g/L on day 4 to 69.24 +/- 9.36 g/L on day 30. During the same period of time oligosaccharide content decreased significantly (p < 0.05) from 25.61 +/- 5.19 g/L to 15.83 +/- 6.05 g/L. Monosaccharides did not show statistically significant variations. CONCLUSIONS: Our results indicate that PT milk contains in addition to lactose, a substantial amount of oligosaccharides and a lower percentage of monosaccharides. Compared to T milk, in the colostral phase PT milk presents significantly lower lactose concentration (p < 0.0001); on the contrary its oligosaccharide content is significantly higher (p < 0.0001). The physiological role of human milk carbohydrates in view of the peculiar needs of the preterm newborn is also discussed.

[Percutaneous renal biopsy in children: use of a fine needle]. / Biopsia renale percutanea in età pediatrica: uso di un ago sottile.

Percutaneous renal biopsies were performed on native kidneys in 40 children and adolescent, aged 1.7-13 (mean 6.7) years. Bleeding diatheses were excluded by the determination of Hb, blood platelets, PT, PTI and fibrinogen. Biopsies were performed under ultrasound imaging, using a semiautomated and thin needle (20 gauge in children with age under 5 years and 18 gauge for those over 5 years). All the patients were lightly sedated, except for 3 ones who received a general anesthesia. Diagnostically adequate tissue was retrieved in 38 of 40 biopsy procedures (95%). A macro-haematuria was observed with elimination of haematic coagula in 3 children (7.5%) and 24-h post-biopsy ultrasonography disciosed a small haematoma of the biopsied kidney in 2 one (5%). No mayor complications occurred. We conclude that the use of ultrasound imaging and a semiautomated needle is a safe and efficient method for performing renal biopsies in paediatric patients. The use of smaller (18 or 20-gauge) cutting needles would reduce the complications rate while allowing retrieval of sufficient tissue for histologic diagnosis.

The coeliac iceberg in Italy. A multicentre antigliadin antibodies screening for coeliac disease in school-age subjects.

BACKGROUND: Recent studies suggest that coeliac disease (CD) is one of the commonest, life-long disorders in Italy. The aims of this multicentre work were: (a) to establish the prevalence of CD on a nationwide basis; and (b) to characterize the CD clinical spectrum in Italy. PATIENTS AND METHODS: Fifteen centres screened 17,201 students aged 6-15 years (68.6% of the eligible population) by the combined determination of serum IgG- and IgA-antigliadin antibody (AGA) test; 1289 (7.5%) were IgG and/or IgA-AGA positive and were recalled for the second-level investigation; 111 of them met the criteria for the intestinal biopsy: IgA-AGA positivity and/or AEA positivity or IgG-AGA positivity plus serum IgA deficiency. RESULTS: Intestinal biopsy was performed on 98 of the 111 subjects. CD was diagnosed in 82 subjects (75 biopsy proven, 7 not biopsied but with associated AGA and AEA positivity). Most of the screening-detected coeliac patients showed low-grade intensity illness often associated with decreased psychophysical well-being. There were two AEA negative cases with associated CD and IgA deficiency. The prevalence of undiagnosed CD was 4.77 x 1000 (95% CI 3.79-5.91), 1 in 210 subjects. The overall prevalence of CD, including known CD cases, was 5.44 x 1000 (95% CI 4.57-6.44), 1 in 184 subjects. The ratio of known to undiagnosed CD cases was 1 in 7. CONCLUSIONS: These findings confirm that, in Italy, CD is one of the most common chronic disorders showing a wide and heterogeneous clinical spectrum. Most CD cases remain undiagnosed unless actively searched.