Hybrid cooperative complexes to decrease VAS score and enhance sexual function in women with vulvar lichen sclerosus.

OBJECTIVE: Lichen sclerosus is a chronic, inflammatory, progressive skin disease predominantly affecting anogenital areas. Vulvar lichen sclerosus (VLS) is one of the most common conditions treated in vulvar clinics; most patients report distressing symptoms of itching, burning, stinging, and pain (particularly during or after sexual intercourse). A preliminary, prospective, single-center study was performed to investigate the efficacy of hyaluronan hybrid cooperative complex (HCC) comprising high and low molecular weight hyaluronic acid to treat menopausal women with VLS. PATIENTS AND METHODS: Patients (N = 30) received two HCC injections at 32 mg/ml (one month apart). At baseline and one and six months after treatment, patients completed validated psychometric questionnaires to assess their self-reported pain, itching, and dryness using the Visual Analogue Scale (VAS) and sexual function by the Female Sexual Function Index (FSFI). RESULTS: After treatment with HCC, no side effects or complications were reported. VAS scores showed a trend towards reduced pain and itching intensity, and there was a statistically significant reduction in median VAS score for dryness at follow-up vs. baseline (p=0.038). For sexual function, there was a statistically significant improvement in lubrication (p=0.001) and orgasm (p=0.001) FSFI domains. CONCLUSIONS: Overall, this preliminary study demonstrated the promising efficacy of HCC in menopausal women with VLS without side effects.

Pyoderma Gangrenosum and inflammatory bowel disease: a combined medical and surgical approach - case report and literature review.

OBJECTIVE: Pyoderma Gangrenosum (PG) is an immune-mediated neutrophilic dermatosis, characterized by large painful ulcers occurring in various body segments. It can be associated to Inflammatory Bowel Disease (IBD) including both Ulcerative Colitis and Crohn Disease. Prompt and effective management is fundamental, due to its high morbidity and mortality rates. By presenting our clinical experience, we aimed at showing the efficacy of a combined therapeutic approach, in which the best of every specialty cooperates managing this hazardous disease. PATIENTS AND METHODS: We report on two patients attending our outpatient clinic with ulcerative skin lesions at the level of the back. Patient 1 suffered from Crohn disease and Patient 2 presented a positive history of abdominal pain, diarrhea with mucus and blood in the stool. Histological exam was performed with final diagnosis of PG associated with IBD. A Literature review was carried out in order to highlight the role of combined clinical-surgical management of PG in adult patients with IBD. RESULTS: Complete resolution of the lesions was achieved in 4 months and 3 months for each patient respectively without relapse. PubMed was searched from 2000 to 2020 with the following keywords: (Pyoderma) AND/OR (Pyoderma Gangrenosum) AND (Inflammatory Bowel Disease) AND/OR (Ulcerative Colitis) AND/OR (Crohn Disease) AND (Management). Seven papers were included (4 case reports, 2 case series, 1 comprehensive review) and reviewed using a descriptive checklist. CONCLUSIONS: PG should be treated by dedicated multidisciplinary teams, in which every specialist plays a crucial role from the diagnosis to the treatment and up to the long-term follow-up.

Systemic corticosteroids could be used as bridge treatment in children with obstructive sleep apnea syndrome waiting for surgery.

PURPOSE: Local and systemic inflammatory markers and pro-inflammatory cytokines are increased in children with obstructive sleep apnea syndrome (OSAS). Therefore, systemic or topical anti-inflammatory agents are used to treat this syndrome. We evaluated the treatment with systemic corticosteroids in children with severe OSAS and adenotonsillar hypertrophy before surgery. METHODS: This was an unblinded open label study. Children with severe OSAS (diagnosed through polysomnography, obstructive apnea-hypopnea index [AHI] > 10 eV/h) were recruited. Exclusion criteria included age < 3 years, history of acute or chronic cardiorespiratory or neuromuscular or metabolic disease; major craniofacial abnormalities; and chromosomal syndromes and epilepsy. Computer-generated random numbers were used for simple randomization of subjects. All children were treated with intranasal beclomethasone spray, and 15 children additionally received oral betamethasone and 0.1 mg/kg per day for 7 days. Sleep clinical record (SCR) and pulsoximetry were performed before and after 7 days in all children. RESULTS: Among 28 children with severe OSAS mean age was 4.5 ± 1.8 years, AHI 20.4 ± 1.8 eV/h). In children treated with intranasal and oral corticosteroids, mean (95.3 ± 1.1 vs 97.0 ± 0.8%, p = 0.0001) and minimum oxygen saturation (78.8 ± 6.3 vs 89.2 ± 4.2, p = 0.001) improved, and the SCR score (12.6 ± 1.2 vs 8.3 ± 1.1, p = 0.0001) was reduced. Children treated only with intranasal beclomethasone spray showed no differences in outcome measures before and after treatments. When we considered the oximetry measures, after corticosteroid treatment, we obtained statistical differences between the 2 groups (p < 0.01). CONCLUSIONS: These results seem to suggest that a short course of oral betamethasone could be useful to treat children with severe OSAS and adenotonsillar hypertrophy waiting for surgery.

A Retrospective Comparative Analysis of Latissimus Dorsi (LD) Flap Versus Thoracodorsal Artery Perforator (TDAP) Flap in Total Breast Reconstruction with Implants: A Pilot Study.

BACKGROUND: In breast surgery, an autologous flap combined with implant may reduce the risk or repair the soft-tissue defects in several cases. Traditionally, the preferred flap is the myocutaneous latissimus dorsi (LD) flap. In the perforator flap era, the evolution of LD flap is the thoracodorsal artery perforator (TDAP) flap. The aim of this study is the comparison between LD flap and TDAP flap with implants in terms of early complications and shoulder function. METHODS: We performed a retrospective cohort study in accordance with the STROBE guidelines. Between January 1 2015 and January 1 2020, 27 women underwent a unilateral total breast reconstruction with LD or TDAP flap combined with an implant at our institution. 15 women were operated with LD flap and 12 with TDAP flap. The most frequent indications for intervention were results of mastectomy and radiation-induced contracture. We evaluated several data in terms of clinical and demographical characteristics, operative and perioperative factors, and follow-up variables. We assessed shoulder function through the Disability of the Arm, Shoulder and Hand Questionnaire (DASH). RESULTS: The rate of complications was significantly lower in the TDAP group compared with the LD group (16.7% vs 60.0%, p = 0.047. Table 3). Although the small sample size limited further detailed statistical analyses, we particularly noticed no cases of donor site seroma in the TDAP group, as compared with four in the LD group. Patients in the TDAP group had an ∼11-point lower mean DASH score compared with the LD group (9.8 vs 20.5). This difference was statistically significant (p = 0.049). CONCLUSIONS: TDAP flap seems to be a reliable technique for soft-tissue coverage in total breast reconstruction with implants. In comparison with the traditional LD flap, it could be a more favorable option in terms of less complications and better quality of life.

Patient's proprioceptive remnants in nipple reconstruction planning.

OBJECTIVE: The aim of this study is to demonstrate that for patients undergoing mastectomy the use of the proprioceptive memory represents a valid method to identify the perfect position of the nipple, which will be reconstructed on an operated breast. PATIENTS AND METHODS: Fifty-one patients undergoing breast reconstruction after unilateral Modified Radical Mastectomy or unilateral Skin Sparing Mastectomy were included in the study. All patients were asked to identify, while keeping their eyes closed, the mammary segment where they perceived their nipples, both on the reconstructed breast mound and on the contralateral breast. Sternal Notch-to-nipple distance (SN), Nipple-to-inframammary Fold distance (NF), Midclavicular line-to-nipple distance (CN), the distance from the nipple to the chest Midline (NM), Anterior Axillary line-to-nipple distance (ZN) were measured on both breasts. The ideal position of the nipple to be reconstructed was evaluated using a geometric method based on the Pythagorean Theorem. RESULTS: A statistically significant correlation emerges between the distances measured from the anatomical landmarks of the chest to the point coinciding with the patient's perception of the nipple on the reconstructed breast, and the distances measured from the same chest landmarks to the nipple on the contralateral native breast and to the nipple placed in the ideal position assessed with the geometric method. CONCLUSIONS: The patient's proprioceptive memory of the nipple position can be useful to identify the exact place to reconstruct the nipple in breast reconstruction.

Effectiveness and tolerability of perampanel in children and adolescents with refractory epilepsies-An Italian observational multicenter study.

PURPOSE: To evaluate the efficacy and tolerability of Perampanel (PER) in children and adolescents with refractory epilepsies in daily clinical practice conditions. PATIENTS AND METHODS: This Italian multicenter retrospective observational study was performed in 16 paediatric epilepsy centres. Inclusion criteria were: (i) ≤18 years of age, (ii) history of refractory epilepsy, (iii) a follow-up ≥5 months of PER add-on therapy. Exclusion criteria were: (i) a diagnosis of primary idiopathic generalized epilepsy, (ii) variation of concomitant AEDs during the previous 4 weeks. Response was defined as a ≥50% reduction in monthly seizure frequency compared with the baseline. RESULTS: 62 patients suffering from various refractory epilepsies were included in this study: 53% were males, the mean age was 14.2 years (range 6-18 years), 8 patients aged <12 years. Mean age at epilepsy onset was 3.4 years and the mean duration of epilepsy was 10.8 years (range 1-16), which ranged from 2 seizures per-month up to several seizures per-day (mean number=96.5). Symptomatic focal epilepsy was reported in 62.9% of cases. Mean number of AEDs used in the past was 7.1; mean number of concomitant AEDs was 2.48, with carbamazepine used in 43.5% of patients. Mean PER daily dose was 7.1mg (2-12mg). After an average of 6.6 months of follow-up (5-13 months), the retention rate was 77.4% (48/62). The response rate was 50%; 16% of patients achieved ≥75% seizure frequency reduction and 5% became completely seizure free. Seizure aggravation was observed in 9.7% of patients. Adverse events were reported in 19 patients (30.6%) and led to PER discontinuation in 4 patients (6.5%). The most common adverse events were behaviour disturbance (irritability and aggressiveness), dizziness, sedation and fatigue. CONCLUSION: PER was found to be a safe and effective treatment when used as adjunctive therapy in paediatric patients with uncontrolled epilepsy.

Headache, migraine and obesity: an overview on plausible links.

Headache can represent different disorders with different etiologies; including cardiac, cerebral, vascular, psychiatric, metabolic, neurologic diseases. Recent studies have highlighted that obesity is significantly associated with headache and disability in adults. This rule also applies to children. This review focuses on literature data studying any eventual relationship between headache, migraine and obesity [shown in Body Mass Index (BMI)] in children. Research data have highlighted that there is a relationship between headache physiopathology and central and peripheral mechanisms responsible for food assumption. In this regard, neurotransmitters such as serotonin, and peptides such as orexin and adipocytokines (adiponectin and leptin) seem to play a key role both in food assumption and in headache pathogenesis. These data further emphasize the potential association between headache and BMI. Therefore, those therapeutic strategies aiming to decrease BMI may represent a model of useful treatment to understand whether weight loss reduces the incidence and the severity of headache in obese children. In conclusion, considering the effects of obesity and weight loss on the natural history of headache, important changes are expected in therapeutic management of paediatric headaches.

Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication.

BACKGROUND AND PURPOSE: Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes. METHODS: This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.1-23.0 years. The following clinical characteristics were evaluated: gender, patient age at seizure onset, history of febrile seizures and migraine, family history of epilepsy, duration and seizure manifestations, circadian distribution and frequency of seizures, history of medications including the number of drugs, therapeutic response and final outcome. RESULTS: Visual hallucinations were the first symptom in 62% and the only manifestation in 38.8% of patients. Patients were subdivided into two groups: group A with isolated COE-G; group B with other epileptic syndromes associated with COE-G. The most significant (P < 0.05) difference concerned antiepileptic therapy: in group A, 45 children responded to monotherapy; in group B only 15 children responded to monotherapy. At the end of follow-up, the percentage of seizure-free patients was significantly higher in group A than in group B. CONCLUSIONS: Childhood occipital epilepsy of Gastaut has an overall favourable prognosis and a good response to antiepileptic therapy with resolution of seizures and of electroencephalogram abnormalities. The association of typical COE-G symptoms with other types of seizure could be related to a poor epilepsy outcome.

Iginio Tansini revisited.

The origin of the muscolocutaneous latissimus dorsi flap dates back to 1906 when Igino Tansini, an Italian surgeon, described a procedure to reconstruct the mastectomy defect. After a detailed study of Tansini's original description and drawings, new insights about the pedicle of its compound flap have been found, showing that it has the same pedicle of the scapular flap. In the end, Tansini's flap should be more correctly considered as a compound musculocutaneous scapular flap.

Role of the gluten-free diet on neurological-EEG findings and sleep disordered breathing in children with celiac disease.

PURPOSE: To determine whether celiac children are at risk for EEG-neurological features and sleep disordered breathing (SDB), and whether an appropriate gluten-free diet (GFD) influences these disorders. METHODS: We consecutively enrolled 19 children with a new biopsy-proven celiac disease (CD) diagnosis. At CD diagnosis and after 6 months of GFD, each patient underwent a general and neurological examination, an electroencephalogram, a questionnaire about neurological features, and a validated questionnaire about SDB: OSA (obstructive sleep apnea) scores<0 predict normality; values>0 predict OSA. RESULTS: At CD diagnosis, 37% of patients complained headache that affected daily activities and 32% showed positive OSA score. The EEG examinations revealed abnormal finding in 48% of children. After 6 months of GFD headache disappeared in 72% of children and EEG abnormalities in 78%; all children showed negative OSA score. CONCLUSION: According to our preliminary data, in the presence of unexplained EEG abnormalities and/or other neurological disorders/SDB an atypical or silent CD should also be taken into account.

Migraine and obesity: metabolic parameters and response to a weight loss programme.

BACKGROUND AND OBJECTIVE: Weight loss can determine significant improvement of migraine in obese patients. Herein, we evaluated a clinical sample of adolescent migraineurs with obesity who participated in an interdisciplinary programme for weight loss, in order to identify possible metabolic parameters associated with good migraine control. SUBJECTS AND METHODS: Using a cross-sectional design, we evaluated 112 out of 135 adolescents who previously underwent our intervention programme. Based on persistence of headache, subjects for comparison were 40 migraine-free and 72 not migraine-free adolescents. Participants underwent anthropometric evaluations and biochemical tests. RESULTS: Patients with persistence of migraine had significantly higher weight (P < 0.01), body mass index (P < 0.01), waist circumference (P < 0.01), homeostasis model assessment of insulin resistance (P < 0.001), triglyceride (P < 0.05), total cholesterol (P < 0.05) and low-density lipoprotein cholesterol (P < 0.05) values when compared with those who became migraine-free. Between potential predictors, only insulin resistance (odds ratio = 3.5, 95% confidence interval = 1.4-6.1; P < 0.001) was significantly associated with persistence of migraine after intervention programme. CONCLUSIONS: Among obese adolescents with migraine who underwent an intervention programme for weight loss, patients who did not become migraine-free showed higher adiposity values than those who became migraine-free. Patients with insulin resistance had 3.5 times the odds of having persistence of migraine compared with those without.

Management of microanastomosis in patients affected by vessel diseases.

OBJECTIVE: In the last 10 years with the advances in microsurgery of techniques and materials the indications for free tissue transfer have considerably been increased. But, there are still some limitations and drawbacks. Among risk factors associated with flap failure, atherosclerosis can affect both the flap and the recipient vessels of free microvascular tissue transfers. The purpose of this paper is to discuss about the pathogenesis of Monckeberg's sclerosis, and the topics that must be taken into consideration when performing microsurgery in these patients. METHODS: PubMed database was searched using Mesh. The following terms was added to the search builder: Monckeberg's sclerosis, free flap. The Boolean operator "AND" was selected. All the selectable Mesh headings for "Monckeberg's sclerosis" and "free flap" were included. RESULTS: Almost all the literature works about microsurgery in Monckeberg's sclerosis patient show the importance of an accurate preoperative and postoperative evaluation and of a proper surgical technique. CONCLUSIONS: When adequate preoperative evaluation, surgical technique and postoperative monitoring are performed, even severe atherosclerosis should not be considered an absolute contraindication for microvascular surgery.

Efficacy and tolerability of add-on lacosamide in children with Lennox-Gastaut syndrome.

OBJECTIVE: Available data on the efficacy of lacosamide in children with Lennox-Gastaut syndrome (LGS) are scarce and controversial. We present our experience with lacosamide therapy in children affected by LGS. MATERIAL AND METHODS: Medical charts of all children affected by LGS receiving oral lacosamide adjunctive therapy in six paediatric neurology centres were retrospectively evaluated. Efficacy was determined according to the frequency of countable seizures during the 4 weeks prior to treatment and the frequency in the last 4 weeks of observation. Patients whose seizure frequency was reduced by at least 50% were defined as responders. RESULTS: Eighteen children (mean age 12.3 years) were identified. After a mean follow-up period of 9 months, 33% of patients were responders. None of them was seizure-free during the study period. The overall seizure reduction rate was 29%. The percentage reductions from baseline in tonic seizures and drop-attacks rates were 31% and 20%, respectively. Adverse reactions occurred in 44% of patients. The drug was discontinued in four (22%) patients because of increased seizure frequency (three cases) and walking instability (another patient). CONCLUSIONS: A third of children with LGS were responders after lacosamide adjunctive therapy. Although caution is still necessary when the drug is used in children with LGS, our preliminary observations suggest that lacosamide might be effective and represent a possible therapeutic option in children affected by LGS.

Superficial leiomyosarcoma of the glans: report of a case and literature review.

Primary leiomyosarcomas of the penis are rare. Clinically and pathologically, these tumors fall into two groups: superficial and deep. Superficial lesions usually are low grade and show a limited tendency toward distant metastasis. In contrast, deep-seated tumors usually show a more aggressive behavior associated with a poor prognosis. A 62-year-old man with a superficial leiomyosarcoma of the glans penis is reported.

Exercise-induced oxidative stress in elderly subjects: the effect of red orange supplementation on the biochemical and cellular response to a single bout of intense physical activity.

Aging is characterized by an impaired capacity to maintain the redox balance both in physiological and pathological situations associated with an increased production of reactive oxygen species. Since the extent of this phenomenon may be influenced by an antioxidants-rich diet, we investigated the effect of supplementation with fresh red orange juice (ROJ) on biochemical and cellular biomarkers of oxidative stress in healthy, trained elderly women after a single bout of exhaustive exercise (EE). To this purpose, a sample of 22 females, 15 (69.0 ± 5.1 years) taking the ROJ supplementation and 7 (68.1 ± 2.7 years) as Control group, was constituted. Blood samples were collected immediately before, 30 minutes, and 24 hr after a single bout of EE, at baseline and after 4 weeks. Our results demonstrate that markers of DNA damage or apoptosis were not affected by EE both in Control and ROJ group, and by ROJ, whereas, exercise temporarily affected the redox balance in both groups. Controls didn't change their response to EE after the experimental period, but experimental group after ROJ supplementation had lower EE-induced MDA, consumed less ascorbic acid, and had less activation of the hypoxanthine/xanthine system, i.e., they seemed to be protected from hypoxia/reoxygenation mechanisms.

Impact of a weight loss program on migraine in obese adolescents.

BACKGROUND AND PURPOSE: Increased headache frequency and severity have been observed in obese populations, but the real impact of a weight loss treatment on headache has not been studied. We investigated this issue in a sample of obese adolescents. METHODS: In all, 135 migraineurs, aged 14-18 years, with body mass index (BMI) ≥ 97 th percentile, participating in a 12-month-long program, were studied before and after treatment. The program included dietary education, specific physical training, and behavioral treatment. RESULTS: Decreases in weight (P < 0.01), BMI (P < 0.01), waist circumference (P < 0.01), headache frequency (P < 0.01) and intensity (P < 0.01), use of acute medications (P < 0.05), and disability (P < 0.05) were observed at the end of the first 6-month period and were maintained through the second 6 months. Both lower baseline BMI and excess change in BMI were significantly associated with better migraine outcomes 12 months after the intervention program. CONCLUSIONS: Significant improvements in both adiposity and headache data were observed in obese adolescents with migraine who participated in a 12-month-long interdisciplinary intervention program for weight loss. Initial body weight and amount of weight loss may be useful for clinicians to predict migraine outcomes.

Early-onset absence epilepsy: SLC2A1 gene analysis and treatment evolution.

BACKGROUND AND PURPOSES: To determine the prevalence of SLC2A1 mutations in children with early-onset absence epilepsy (EOAE) and to investigate whether there were differences in demographic and electroclinical data between patients who became seizure-free with anti-epileptic drug (AED) monotherapy (group I) and those who needed add-on treatment of a second AED (group II). METHODS: We reviewed children with EOAE attending different Italian epilepsy centers. All participants had onset of absence seizures within the first 3 years of life but otherwise conformed to a strict definition of childhood absence epilepsy. Mutation analysis of SLC2A1 was performed in each patient. RESULTS: Eighty-four children (57 in group I, 27 in group II) fulfilled the inclusion criteria. No mutation in SLC2A1 was found. There were no statistical differences between the two groups with regard to F/M ratio, age at onset of EOAE, early history of febrile seizures, first-degree family history for genetic generalized epilepsy, duration of AED therapy at 3 years after enrollment, use of AEDs at 3 years, failed withdrawals at 3 years, terminal remission of EOAE at 3 years, and 6-month follow-up EEG data. Mean duration of seizures/active epilepsy was significantly shorter in group I than in group II (P = 0.008). CONCLUSIONS: We demonstrate that in a large series of children with rigorous diagnosis of EOAE, no mutations in SLC2A1 gene are detected. Except for duration of seizures/active epilepsy, no significant differences in demographic and electroclinical aspects are observed between children with EOAE who responded well to AED monotherapy and those who became seizure-free with add-on treatment of a second AED.