Reconstruction of the Axillary Region after Excision of Hidradenitis Suppurativa: A Systematic Review.

Introduction Hidradenitis suppurativa (HS) is a chronic, debilitating, recurrent, auto-inflammatory disease of the pilosebaceous units of the skin. The axillary region is the most affected anatomical site and its reconstructive options include skin grafts, local random plasties, regional axial flaps, and regional perforator flaps. The main aim of this systematic review is to identify the best surgical technique for axillary reconstruction in the context of HS, in terms of efficacy and safety. Methods We adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) throughout the whole review protocol build-up. The literature search was performed using MEDLINE, Embase, and Cochrane library databases, updated to March 2021. Quality was assessed for each study, through the National Institutes of Health Quality Assessment Tool. Results A total of 23 studies were included in the final analysis. We reviewed a total of 394 axillary reconstructions in 313 patients affected by HS Hurley Stage II or III. Skin grafts were associated with the highest overall complication rate (37%), and highest rate of reconstruction failure (22%). Between thoraco-dorsal artery perforator flap, posterior arm flap, and parascapular flap, the latter showed fewer total complications, recurrences, and failures. Conclusion Regional axial flaps should be considered as the best surgical approach in the management of advanced HS. The parascapular flap emerges as the most effective and safest option for axillary reconstruction. Local random flaps might be considered only for selected minor excisions, due to the higher risk of recurrence. The use of skin grafts for axillary reconstruction is discouraged.

Single access upper blepharoplasty and lower lid lateral fat pad removal: A proven technique with low recurrence rate.

BACKGROUND: The transconjunctival approach is commonly performed in lower lid blepharoplasty so as not to violate the orbicularis oculi muscle integrity and avoid additional scars. This technique does not, however, permit easy access to the lateral fat pad (LFP). As upper and lower blepharoplasty are frequently combined in our clinical practice, the removal of LFP via upper blepharoplasty is a surgical approach that we found most applicable with good outcomes. METHODS: A prospective review of 47 consecutive bilateral combined upper and lower blepharoplasty cases using a single access technique was conducted; patient outcomes and the occurrence of any complications were recorded, in addition to preoperative and postoperative measurements between standardized anatomical landmarks. RESULTS: Forty-seven patients were included in our study. Postoperative measurements showed a decrease of both lateral cantus to inferior orbital border distance and horizontal width of LFP. Good esthetic outcomes and patient satisfaction with surgical results were achieved in the majority of patients. CONCLUSION: The single access upper blepharoplasty and lower lid LFP removal technique can improve esthetic outcomes in lower blepharoplasty. The advantages are many; a low risk of missing the lateral pad, ease of achieving a uniform contour of lower eyelid; short lower transconjunctival incision, minimizing complications associated with an intra-conjunctival scar. This technique, based on cadaveric anatomical studies, has been found to be safe and is of great utility to those patients requiring upper and lower lid blepharoplasties.

Enteral versus intravenous approach for the sedation of critically ill patients: a randomized and controlled trial.

BACKGROUND: ICU patients must be kept conscious, calm, and cooperative even during the critical phases of illness. Enteral administration of sedative drugs might avoid over sedation, and would be as adequate as intravenous administration in patients who are awake, with fewer side effects and lower costs. This study compares two sedation strategies, for early achievement and maintenance of the target light sedation. METHODS: This was a multicenter, single-blind, randomized and controlled trial carried out in 12 Italian ICUs, involving patients with expected mechanical ventilation duration > 72 h at ICU admission and predicted mortality > 12% (Simplified Acute Physiology Score II > 32 points) during the first 24 h on ICU. Patients were randomly assigned to receive intravenous (midazolam, propofol) or enteral (hydroxyzine, lorazepam, and melatonin) sedation. The primary outcome was percentage of work shifts with the patient having an observed Richmond Agitation-Sedation Scale (RASS) = target RASS ±1. Secondary outcomes were feasibility, delirium-free and coma-free days, costs of drugs, length of ICU and hospital stay, and ICU, hospital, and one-year mortality. RESULTS: There were 348 patients enrolled. There were no differences in the primary outcome: enteral 89.8% (74.1-100), intravenous 94.4% (78-100), p = 0.20. Enteral-treated patients had more protocol violations: n = 81 (46.6%) vs 7 (4.2%), p < 0.01; more self-extubations: n = 14 (8.1%) vs 4 (2.4%), p = 0.03; a lighter sedative target (RASS = 0): 93% (71-100) vs 83% (61-100), p < 0.01; and lower total drug costs: 2.39 (0.75-9.78) vs 4.15 (1.20-20.19) €/day with mechanical ventilation (p = 0.01). CONCLUSIONS: Although enteral sedation of critically ill patients is cheaper and permits a lighter sedation target, it is not superior to intravenous sedation for reaching the RASS target. TRIAL REGISTRATION: ClinicalTrials.gov, NCT01360346 . Registered on 25 March 2011.

Hemophilia Care in the Pediatric Age.

Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be considered in the neonatal period in the case of unusual bleeding or in the case of positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bruising/bleeding or unusual bleeding following invasive procedures-for example, tonsillectomy or circumcision. Prophylactic treatment that is started early with clotting-factor concentrates has been shown to prevent hemophilic arthropathy and is, therefore, the gold standard of care for hemophilia A and B in most countries with adequate resources. Central venous access catheters and arterovenous fistulas play an important role in the management of hemophilia children requiring repeated and/or urgent administration of coagulation factor concentrates. During childhood and adolescence, personalized treatment strategies that suit the patient and his lifestyle are essential to ensure optimal outcomes. Physical activity is important and can contribute to better coordination, endurance, flexibility and strength. The present article focuses also on questions frequently posed to pediatric hematologists like vaccinations, day-care/school access and dental care.

Neurological assessment with validated tools in general ICU: multicenter, randomized, before and after, pragmatic study to evaluate the effectiveness of an e-learning platform for continuous medical education.

BACKGROUND: International guidelines recommend systematic assessment of pain, agitation/sedation and delirium with validated scales for all ICU patients. However, these evaluations are often not done. We have created an e-learning training platform for the continuous medical education, and assessed its efficacy in increasing the use of validated tools by all medical and nursing staff of the participating ICUs during their daily practice. METHODS: Multicenter, randomized, before and after study. The eight participating centers were randomized in two groups, and received training at different times. The use of validated tools (Verbal Numeric Rating or Behavioral Pain Scale for pain; Richmond Agitation-Sedation Scale for agitation; Confusion Assessment Method for the ICU for delirium) was evaluated from clinical data recorded in medical charts during a week, with follow-up up to six months after the training. All the operators were invited to complete a questionnaire, at baseline and after the training. RESULTS: Among the 374 nurses and physicians involved, 140 (37.4%) completed at least one of the three courses. The assessment of pain (38.1 vs. 92.9%, P<0.01) and delirium (0 vs. 78.6%, P<0.01) using validated tools significantly increased after training. Observation in the follow-up showed further improvement in delirium monitoring, with no signs of extinction for pain and sedation/agitation measurements. CONCLUSIONS: This e-learning program shows encouraging effectiveness, and the increase in the use of validated tools for neurological monitoring in critically ill patients lasts over time.

Psychological well-being and social support in chronic myeloid leukemia patients receiving lifelong targeted therapies.

PURPOSE: The main objective of this study was to investigate whether social support is independently associated with psychological well-being in chronic myeloid leukemia (CML) patients. Secondary objectives were to compare the psychological well-being profile of CML patients with that of their peers in general population and to examine possible age- and sex-related differences. PATIENTS AND METHODS: Analysis was performed on 417 patients in treatment with lifelong molecularly targeted therapies. Mean age of patients analyzed was 56 years (range 19-87 years) and 247 (59 %) were male and 170 (41 %) were female. Social support was assessed with the Multidimensional Scale of Perceived Social Support and psychological well-being was evaluated with the short version of the Psychological General Well-Being Index. Descriptive statistics and multivariate logistic regression analyses were used. RESULTS: Multivariate logistic regression analysis revealed that a greater social support was independently associated with lower anxiety and depression, as well as with higher positive well-being, self-control, and vitality (p < 0.001). Female patients reported statistically significant worse outcomes in all dimensions of psychological well-being. Age- and sex-adjusted comparisons with population norms revealed that depression (ES = -0.42, p < 0.001) and self-control (ES = -0.48, p < 0.001) were the two main impaired psychological dimensions. CONCLUSION: This study indicates that social support is a critical factor associated with psychological well-being of CML patients treated with modern lifelong targeted therapies.

Somatic, hematologic phenotype, long-term outcome, and effect of hematopoietic stem cell transplantation. An analysis of 97 Fanconi anemia patients from the Italian national database on behalf of the Marrow Failure Study Group of the AIEOP (Italian Association of Pediatric Hematology-Oncology).

We analyzed 97 Fanconi anemia patients from a clinic/biological database for genotype, somatic, and hematologic phenotype, adverse hematological events, solid tumors, and treatment. Seventy-two patients belonged to complementation group A. Eighty percent of patients presented with mild/moderate somatic phenotype and most with cytopenia. No correlation was seen between somatic/hematologic phenotype and number of missense mutations of FANCA alleles. Over follow-up, 33% of patients improved or maintained mild/moderate cytopenia or normal blood count, whereas remaining worsened cytopenia. Eleven patients developed a hematological adverse event (MDS, AML, pathological cytogenetics) and three developed solid tumors. 10 years cumulative risk of death of the whole cohort was 25.6% with median follow-up 5.8 years. In patients eligible to hematopoietic stem cell transplantation because of moderate cytopenia, mortality was significantly higher in subjects transplanted from matched unrelated donor over nontransplanted subjects, whereas there was no significant difference between matched sibling donor transplants and nontransplanted patients. In patients eligible to transplant because of severe cytopenia and clonal disease, mortality risk was not significantly different in transplanted from matched unrelated versus matched sibling donor versus nontransplanted subjects. The decision to transplant should rely on various elements including, type of donor, HLA matching, patient comorbidities, impairment, and clonal evolution of hematopoiesis. Am. J. Hematol. 91:666-671, 2016. © 2016 Wiley Periodicals, Inc.

Diagnostic potential of hepcidin testing in pediatrics.

OBJECTIVES: Hepcidin, a peptide hormone released by hepatocytes into circulation is the main regulator of dietary iron absorption and cellular iron release. Although commercial tests are available, assay harmonization for hepcidin has not been yet reached, making reference intervals and consequent clinical decisions still elusive for each assay and specific population. The aim of this study is to set up hepcidin measurement in pediatric age and to investigate its potential usefulness in the diagnosis and management of iron disorders in children. METHODS: Serum hepcidin was measured by using an automated commercial immunoassay. Reference values were obtained from 86 healthy children. Hepcidin was then evaluated in 52 children with diseases where this hormone was expected to be differently regulated. RESULTS: Hepcidin values were 43.6 ng/mL median; 32-52.7 1-3 q: in males and 36.4 ng/mL median; 28.5-45.7 1-3 q: in females (P = 0.039). Hepcidin was significantly higher in postpubertal normal females than in normal males. Hepcidin resulted up-regulated in anemia of chronic disease of children affected by systemic Juvenile Idiopathic Arthritis and decreased after treatment with anakinra, an anti-interleukin-1 receptor antagonist. In iron deficiency anemia patients on oral iron supplementation and in ß-thalassemia subjects, hepcidin levels were similar to those found in healthy subjects. CONCLUSIONS: This study sets up reference values for pediatric population and shows that in normal controls serum hepcidin react differently to puberty in females vs. males. In addition, it suggests that serum hepcidin may discriminate microcytic inflammatory anemia of Juvenile Idiopathic Arthritis from iron deficiency anemia. Overall these findings may represent a helpful tool for future studies tailored to understand the role of hepcidin in management of iron disorders in children.

Infectious complications in children with severe congenital, autoimmune or idiopathic neutropenia: a retrospective study from the Italian Neutropenia Registry.

We describe the incidence and characteristics of infections in children with severe congenital neutropenia (SCN), autoimmune neutropenia (AN) and idiopathic neutropenia (IN). Data extracted from the Italian Neutropenia Registry on 73 patients with 108 episodes of infections were collected from 2000 to 2009. All SCN patients with SCN and one third of AN and IN experienced at least 1 infectious episode, equating to 5.7 infections/patient in SCN and approximately 0.6 in AN and IN. The rate of infections before diagnosis of neutropenia was 6.35/1000 patient-days at risk in SCN, 0.48 in AN and 0.71 in IN (P < 0.001) and significantly decreased after diagnosis. Skin and subcutaneous abscesses and cellulitis were the most frequent types of infection encountered, followed by pneumonia. Infections are an important clinical issue in the management of neutropenic patients, even in those considered at lower risk.

Flow cytometric analysis of peripheral blood dendritic cells in patients with severe sepsis.

BACKGROUND: Dendritic cells (DC) play a key role in cell-mediated immunity. We aimed to analyse the number and function of peripheral blood (PB) myeloid and plasmacytoid DC (mDC/pDC) in patients with severe sepsis. METHODS: Twenty-six septic patients, 20 surgical patients (abdominal aortic aneurysm) and 20 healthy controls were enrolled in this prospective study. RESULTS: At day 1 (enrollment in the study), septic patients showed in comparison with healthy controls, decreased mDC (P < 0.001) and increased pDC (P = 0.03), resulting in a reduction of the mDC/pDC ratio (P < 0.001). Surgery induced a decrease in both mDCs and pDCs level, without modification of mDC/pDC ratio. Septic patients included 15 survivors and 11 nonsurvivors. At day 1 no significant difference was found in mDC between the two groups, while pDCs were significantly higher in nonsurvivors (P = 0.03). At the outcome, mDC were selectively increased with respect to day 1 in survivors (P = 0.001), while no significant differences were observed as far as pDC count is concerned in both groups. Sorted DC from septic patients showed in comparison with healthy controls, reduced levels of HLA DR (P < 0.001), CD11c (P < 0.001), CD83 (P = 0.006) and of costimulatory molecule CD86 (P = 0.003); an up-regulation of chemokine receptor CXCR4 (P = 0.031) and increased apoptosis (P < 0.001). CONCLUSIONS: Sepsis has a profound effect on PB DC compartment. These alterations appear to be sepsis-specific. Increased apoptosis and alteration of migration and trafficking mechanism may be involved in DC compartment modification. DC alterations could contribute to sepsis-mediated immunoparalysis.

Cerebral perfusion pressure and cerebral tissue oxygen tension in a patient during cardiopulmonary resuscitation.

OBJECTIVE: To report on the effects of cardiopulmonary resuscitation (CPR) instituted immediately after a cardiac arrest on cerebral perfusion pressure (CPP) and cerebral tissue oxygen tension (PbrO(2)). DESIGN: Case report. SETTING: ICU of a university hospital. PATIENT: A head-injured 17-year-old man submitted to multimodal neurological monitoring underwent sudden cardiac arrest and successful CPR. INTERVENTIONS: External chest compression, 100% oxygen ventilation, volume expansion and standard ACLS protocols. MEASUREMENTS AND RESULTS: Heart rate, ECG, mean arterial blood pressure (MABP), ETCO(2), PaO(2), intracranial pressure (ICP), CPP and PbrO(2) were continuously monitored during CPR and data recorded at 15-s intervals by a dedicated personal computer. At the onset of the cardiac arrest, PbrO(2) decreased to zero. The institution of CPR resulted in a progressive increase of MABP, CPP and PbrO(2). Assuming, on the basis of previous experimental and clinical reports, 8 mmHg PbrO(2) as a possible ischaemic/hypoxic threshold value, during the first 6.5 min of CPR, PbrO(2) values were below this threshold (range 0-7 mmHg) and CPP values were <25 mmHg for 81.5% of the time. In the following 5.5 min, more efficient CPR generated CPP values >25 mmHg for 77.3% of the time. These values were associated with a PbrO(2) >8 mmHg (range 8-28 mmHg) at all times. CONCLUSIONS: In the clinical setting of a witnessed cardiac arrest, immediate institution of CPR can be effective in generating PbrO(2) values above a supposed ischaemic/hypoxic threshold when CPP is >25 mmHg. PbrO(2) monitoring by the Licox system is sensitive and reliable, even at low values, and can be suitable for evaluating cerebral oxygenation during experimental CPR.