Improvement and standardization of disinfection in hospital theatre with ultraviolet-C technology.

BACKGROUND: The level of disinfection between operating sessions is important to prevent cross-contamination risk in operating theatres. AIM: To assess the difference in microbial contamination between different disinfection levels, before (T0) and after (T1) application of a UVC device (UVC-D). METHODS: A cross-sectional study was conducted between December 2019 and August 2020 in a clinic. Three disinfection levels - no disinfection after surgery, after in-between cleaning, and after terminal cleaning - were compared to assess the reduction of microbial presence at T0 and T1 according to the use of UVC-D for 3-5 min per bedside. A total of 260 Petri dishes, divided into a preliminary phase followed by a probabilistic model-driven experiment, were used in three operating theatres, and colony-forming units (cfu) were counted. The Mann-Whitney test was performed in the preliminary phase to establish UVC exposure time. Using the probabilistic model, descriptive statistics and percentage and log10 reduction were calculated. The multivariate analysis of variance (MANOVA) for repeated measures was performed to verify the 95% statistical difference between T0 and T1, combined with the disinfection levels and different operating theatres. FINDINGS: The Mann-Whitney test showed no cfu difference between 3 and 5 min of UVC exposure time; the MANOVA test showed no significant difference between disinfection levels in T0 - T1 cfu reduction with a mean cfu reduction of 72% (95% confidence interval: 61.7-84.9) regardless of the disinfection level applied previously. CONCLUSION: UVC-D has improved environmental disinfection in all initial conditions. Together with the classic sanitizing procedures already present, it improves and standardizes the level of environmental hygiene.

[An integrated approach for better contamination control in hospital settings]. / Un approccio integrato per un miglior controllo della contaminazione in contesti ospedalieri.

Disinfection of hospital environments is a cornerstone of intervention strategies to reduce the risk of hospital-associated infections. Many studies show that standard cleaning procedures are not sufficient for proper disinfection of hospital environments and that the addition of no-touch technologies, such us ultraviolet light, can provide deeper sanitisation. This study aims to test whether the application of ultraviolet light after standard procedures improves hygiene levels in the shortest possible time and shows the degree of contamination before and after irradiation. A cross-sectional study was conducted in a real clinical setting in rehabilitation rooms of a contracted clinic "Rugani Hospital" in Monteriggioni (SI), Italy, between December 2019 and August 2020.the study was carried out according to the following protocol: i) quantization of contamination of 12 selected target points in room; ii) attribution to the points of a probability of contamination risk; iii) sampling of a subset of 6 points with probabilistic assignment; iv) evaluation of the pre-post disinfection environmental hygiene using a UV-C system. For the pre-post statistical analysis the non-parametric Wilcoxon test was used; the multivariate MANOVA was used to verify the role of different confounders, with post hoc Bonferroni test. Probabilistic calculations minimised the samplings required to conclude that the application of the ultraviolet light device reduced the level of contamination in a statistically significant manner (p < 0.01) when comparing pre- and post-exposure, with less irradiation time than indicated by the manufacturer.

Peripheral neuropathy in late-onset Krabbe disease: report of three cases.

Late-onset Krabbe disease may have variable misleading clinical manifestations and be a puzzling problem for physicians. We report clinical and peripheral nerve studies of three patients with adult-onset Krabbe disease. Two cases had a predominantly spastic paraparesis; in one case, the symptoms mimicked a cerebrovascular disorder. Predominantly, demyelinating neuropathy was observed in one case and axonal neuropathy in two cases. In all cases, no typical intracytoplasmic inclusions were found. These observations suggest that peripheral neuropathy in adult-onset Krabbe disease has variable clinical and pathological characteristics, different from those described in the classic form.

Leukoencephalopathy as a rare complication of hepatitis C infection.

We report the case of a 64-year-old female patient with hepatitis C infection (HCV), who developed Sjögren's disease and sensory peripheral neuropathy. Clinical conditions worsened over three years with central nervous system involvement characterised by transient third cranial nerve paresis and mild selective impairment of attention and memory. Brain magnetic resonance imaging showed diffuse periventricular and lobar white matter hyperintensity. Laboratory findings included mixed cryoglobulinaemia (type II), cryocrit 1.47%, low serum levels of complement C4 and high levels of rheumatoid factor, HCV 1b genotype, high HCV mRNA levels in serum and cerebrospinal fluid. Skin biopsy showed evidence of vasculitis. After one year of plasmapheresis, immunosuppressant therapy and occasional corticosteroid treatment, neurological symptoms improved, skin biopsy changed and inflammation parameters normalised, suggesting that neurological symptoms might be related to the high levels of mixed cryoglobulins.

A syndrome of bilateral hemorrhage of the thalamus and myocarditis with fatal course.

We report the case of a 41-year-old patient with bilateral hemorrhage of the thalamus, leading to death. Post-mortem examination showed acute myocarditis. Neuropathological study showed perivascular infiltrates in affected thalamic regions. Laboratory investigation failed to find any causal agent. We hypothesize an infective agent, affecting the heart and thalamus, as the cause of this syndrome. Diaschisis due to the strategic anatomical position of the thalamus may have been responsible for coma state and death.

Mitochondrial alterations in muscle biopsies of patients on statin therapy.

Clinical and biopsy study of nine patients on statin therapy suffering from various myopathic syndromes is reported. Biopsy findings showed non specific myopathic signs and mitochondrial changes, such as subsarcolemmal accumulation, morphological alterations, lipid increase and Cox-negative fibers. These findings confirm that statins may cause muscle damage and impair oxidative metabolism.

DNA end labelling (TUNEL) in a 3 year old girl with Leigh syndrome and prevalent cortical involvement.

Neuropathological study of a 3 1/2 year old girl with familial Leigh syndrome who also harboured a rare ATPase gene mutation disclosed extensive and unusual lesions in the cerebral cortex, despite a typical histological pattern. Early lesions in the periacqueductal grey matter of the brainstem, characterised by capillary congestion and initial regressive neuronal changes, were also observed, along with TUNEL reactive neuronal cells showing morphological signs typical of apoptosis in cortical areas with neuronal cell loss. The finding of lesions in atypical brain areas and for the first time, very early regressive neuronal phenomena, suggest that early changes in crucial brain areas may have been a cause of death. The abundance of TUNEL positive nuclei in cortical areas in the present case suggests that the apoptosis may be involved in the mechanism of neuronal death in Leigh syndrome.

Mitochondrial abnormalities in genetically assessed oculopharyngeal muscular dystrophy.

We report a family with a clinical diagnosis of oculopharyngeal muscular dystrophy in which muscle biopsy showed mitochondrial changes such as cytochrome-c-oxidase-negative fibers and aggregates of mitochondria containing paracrystalline inclusions. Molecular analysis demonstrated a GCG expansion in the poly(A)-binding protein 2 (PABP2) gene and failed to demonstrate multiple deletions of mtDNA. We hypothesize that mitochondrial abnormalities may be a secondary phenomenon. This observation may suggest that the PABP2 gene could interfere in the posttranscriptional regulation of genes involved in mitochondrial function.

Novel SACS mutations in autosomal recessive spastic ataxia of Charlevoix-Saguenay type.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset familial disease with prominent myelinated fibers in the optic fundus. ARSACS is frequent in the Charlevoix-Saguenay region of Quebec but rare elsewhere. Mutations in SACS, encoding sacsin, a protein of unknown function, are associated with ARSACS. The authors identified three new SACS mutations in two Italian patients whose phenotype closely matches that of Quebec cases, but without retinal striation.

Asymptomatic cores and paracrystalline mitochondrial inclusions in CADASIL.

Three siblings with genetically assessed cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with core-like lesions and mitochondrial abnormalities in muscles are described. Involvement of the Ryanodine receptor 1 gene was excluded. In the current cases, the relation between molecular genetic lesion and muscle fiber abnormalities remains to be determined, but the Notch3 gene may influence mitochondrial metabolism.

Nemaline myopathy: description of an adult onset case.

Nemaline myopathy is a rare congenital muscle disease, with neonatal or adult onset. We report clinical and ultrastructural study of a 73-year-old woman whose symptoms manifested at age 40 years with proximal muscle weakness, nocturnal cramps, muscle pain and walking impairment. Muscle biopsy showed rods and other typical findings suggesting nemaline myopathy. This myopathy should be taken into account in the differential diagnosis of adult onset myopathies. Only ultrastructural examination allows an exact diagnosis.

Ultrastructural findings in the peripheral nerve in a family with the intermediate form of Charcot-Marie-Tooth disease.

The authors describe here the ultrastructural findings in peripheral nerve biopsies from two affected members of a family with a previously undescribed intermediate form of Charcot-Marie-Tooth (CMT) disease. We found prevalent demyelinating features such as onion bulbs and myelin splits with uncompacted and irregularly enlarged lamellae, mostly at the Schmidt-Lantermann incisures and in paranodal region. Signs of a chronic axonopathy such as regeneration clusters, large fiber loss, Büngner's bands and unmyelinated fiber involvement were also seen. The presence of both demyelinating and axonal findings, not found in other genetically determined types of CMT disease, confirms the hypothesis of a new nosographic entity of intermediate type.

PAX6 mutation in a family with aniridia, congenital ptosis, and mental retardation.

Congenital aniridia is due to deletions and point mutations in the PAX6 gene. We describe here a case of a mother and her two sons with a syndrome comprising congenital aniridia, ptosis, and slight mental retardation. The sons also show behavioral changes. The possibility of deletion around the PAX6 locus was excluded by polymorphism studies and fluorescence in situ hybridization analysis. Mutation screening of the PAX6 gene revealed the presence of a transversion C719A, resulting in the substitution of arginine for serine at residue 119. We suggest that this missense mutation is responsible both for aniridia and ptosis, and possibly also for the observed cognitive dysfunction in this family.

Neuropathological findings associated with retained lead shot pellets in a man surviving two months after a suicide attempt.

We describe the neuropathological findings in a 30-year-old man who died two months after attempting suicide with a shotgun. We focused our study on lesions associated with retained lead shot pellets and distant therefrom, as well as lesions distant from the principal site of injury. At the sites of the retained lead shot pellets, we found macrophage proliferation and astrocyte activation, together with axonal spheroids and signs of neuronal damage. In the remaining white matter we observed axonal swellings, astrocyte activation and rarefaction of the neuropil; regressive phenomena of the neurons were also present. All axonal spheroids immunoreacted with antibodies against APP, alphaB-crystallin, NF subunits and ubiquitin. Most reactive astrocytes were positive for GFAP and alphaB-crystallin immunostaining. Some neurons immunoreacting with alphaB-crystallin were also found. These data indicated that an important local reaction developed at the sites of lead shot retention, and mild signs of diffuse axonal damage were found throughout the brain.

Autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.

OBJECTIVE: To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc). METHODS: We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms. RESULTS: We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found. CONCLUSION: Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.