Incubadora neonatal con ventilación mecánica embebida / Mechanical Ventilation embedded in Neonatal Incubator

RESUMEN Se presenta un equipo de soporte de vida neonatal (ESVIN) que emplea la terapia térmica y terapia ventilatoria (neumática) en un solo equipo para proveer ventilación pulmonar con aire caliente, humedecido y enriquecido con oxígeno, en un ambiente caliente, humidificado y estéril. El equipo es capaz de simultáneamente dar ventilación pulmonar e incubar, siendo una de sus características principales la minimización de la condensación del agua en el corrugado y ofrece la característica adicional de evitar la movilización y/o desconexión del neonato para realizar ciertos procedimientos tales como: cirugías e intubaciones, entre otras. Los principales resultados son el tiempo de acceso al neonato menor a 2 s y minimización de la condensación de agua. Asimismo, los resultados del control térmico son de tiempo de estabilización en el habitáculo de 75 minutos para la temperatura de 36 °C y tiempo de estabilización de la temperatura de la piel del neonato de 58 minutos.

ABSTRACT A neonatal life support equipment (ESVIN) employing simultaneously thermal therapy and ventilatory (pneumatic) therapy is presented in a single kit to provide pulmonary ventilation with warm, moistened and oxygen enriched air in a warm, humidified and sterile environment. The invention behind ESVIN provided simultaneously pulmonary ventilation and incubation having optimized the minimization of water condensation in the corrugated pipe and offered the additional feature of avoiding the mobilization and / or disconnection of the neonate to perform certain procedures such as: surgeries and intubations, among others. ESVIN has an access to newborns of less than 2 s and non-visible water condensation. The main results in thermal control were a stabilization time in the newborn compartment of 75 minutes for the temperature of 36 °C and a stabilization of the temperature of the skin of the neonate of 58 minutes.

Analysis of Italian regulations on pathways of care for patients in a vegetative or minimally conscious state.

Different rehabilitation models for persons diagnosed with disorders of consciousness have been proposed in Europe during the last decade. In Italy, the Ministry of Health has defined a national healthcare model, although, to date, there is a lack of information on how this has been implemented at regional level. The INCARICO project collected information on different regional regulations, analysing ethical aspects and mapping care facilities (numbers of beds and medical units) in eleven regional territories. The researchers found a total of 106 laws; differences emerged both between regions and versus the national model, showing that patients with the same diagnosis may follow different pathways of care. An ongoing cultural shift from a treatment-oriented medical approach towards a care-oriented integrated biopsychosocial approach was found in all the welfare and healthcare systems analysed. Future studies are needed to explore the relationship between healthcare systems and the quality of services provided.

Effectiveness of surgical treatment of severe macrocheilia in a patient with orofacial granulomatosis.

Orofacial granulomatosis (OFG) is the term given to a group of diseases characterized by the presence of non-necrotizing granulomatous inflammation affecting the soft tissues of the orofacial region. Treatment of OFG is often challenging and unsatisfactory. We report on a 32-year-old man with a 2-year history of oedema and swelling of the upper lip without systemic symptoms. The history, clinical features and histopathological findings led to the diagnosis of cheilitis granulomatosa (CG), a disease included in the spectrum of OFG. The patient was treated with oral diaminodiphenyl sulfone (DDS) and clofazimine without success. Oral doxycycline led to a slight improvement of the disease. Because the volume of the upper lip was twice normal size, surgical reduction was performed, followed by administration of oral doxycycline for 3 months. This therapeutic approach led to complete remission, with no recurrence after 3 years.

Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement.

Granuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brown-purple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinico-pathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented.

Acute generalized exanthematous pustulosis: report of five cases and systematic review of clinical and histopathological findings.

Acute generalized exanthematous pustulosis (AGEP) is a rare, drug-related pustular eruption usually starting from folds with edema and erythema and with subsequent spreading. Clinically AGEP is characterized by the sudden appearance of dozen of sterile, non follicular, small pustules on erythematous and edematous skin. Mild non erosive mucosal involvement, mostly oral, may sometimes occur. Fever, neutrophilia and peripheral blood eosinophilia (in a third of patients) are present. Other skin signs such as facial edema, purpura, target-like lesions and blisters have been described but are not typical for AGEP. Diagnostic criteria for AGEP were established by an international committee of experts, the European Study of Severe Cutaneous Adverse Reactions (EuroSCAR). The most relevant histopathological feature is represented by the detection of non-follicular subcorneal and/or intracorneal spongiform pustules that are usually large, contiguous and tend to coalesce. After elimination of the causative drug, pustules usually spontaneously disappear in a few days with desquamation and the reaction fully resolves within 15 days. Internal organs are not usually involved and no systemic treatment is required. Withdrawal of the culprit drug is mandatory. Although AGEP is a self-limiting disease with a favourable prognosis, secondary infections are a not infrequent complication in patients in poor general medical conditions. The reported mortality is about 5%. The most severe cases are associated with drug rechallenge.

Histopathologic spectrum of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): a diagnosis that needs clinico-pathological correlation.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by an heterogeneous group of severe dermatologic manifestations and systemic involvement, due to several groups of medicaments. A series of 9 consecutive cases, observed from 2008 to 2013 in the Department of Dermatology, University of Pavia, is reported, all satisfying the clinical, hematological and systemic diagnostic criteria of DRESS. Clinically, 4 out of 9 patients had an urticarial and papular eruption, 2 an erythema-multiforme-like (EM-like) pattern, 2 erythroderma and 1 had an erythematous and macular reaction. Aim of the study was to describe the histopathologic features of DRESS and to trace a possible correlation between the four clinical recognized types of the syndrome and the histopathological patterns. Predominantly, a superficial perivascular lymphocytic infiltrate, extravasation of erythrocytes, and focal interface changes characterized DRESS cases. Less frequently, histopathology revealed the presence of necrotic keratinocytes; surprisingly, only in 2 cases the presence of rare dermal eosinophils was detected, even if all the patients had significant peripheral eosinophilia. A histopathological diagnosis of DRESS seems per se, according to our data, not feasible, since the main histopathological changes (interface changes, superficial perivascular dermatitis, focal spongiosis, lichenoid infiltrate, rare presence of necrotic keratinocytes) can be interpreted generically as a drug induced dermatitis. The above mentioned histopathological changes, however, when associated with clinical information on cutaneous and systemic involvement of the patient, allow the pathologist or the dermatopathologist to make a diagnosis of DRESS with a reliable margin of certainty.

Tyrosine kinase inhibitors: muco-cutaneous side effects at the microscope.

In the past recent years, treatments that target receptors with kinase activity, involved in the transmission of neoplastic proliferation signals, had revolutionized cancer therapy. Imatinib mesylate has been the first of this novel family of drugs approved for the treatment of hematologic malignancies. Afterwards, other second-generation kinase inhibitors, such as dasatinib and nilotinib, have been introduced to circumvent resistance to imatinib. These target therapies have a better tolerability profile than standard chemotherapy, but their range of activity is not simply directed at tumor cells, and a wide spectrum of systemic side effects is now recognized. In particular, muco-cutaneous side effects represent the most frequent non-hematological adverse events. Due to the need of a prompt recognition of these toxicities, diagnosis is usually made on clinical grounds, and an accurate histological characterization is generally lacking. The aim of this paper was to focus on the histopathological findings of cutaneous reactions related to tyrosine kinase inhibitors use. We propose a differentiation between specific and non-specific cutaneous side effects, through an analysis of the possible etiopathogenetic mechanisms of actions of the drug, clinical aspects and major histological features. A review of the literature has been integrated by our personal experience, highlighting the importance of clinico-histological correlation, necessary to make a proper diagnosis.

Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune mucocutaneous bullous disease caused by autoantibodies against type VII collagen, a component of anchoring fibrils that stabilizes dermoepidermal adherence. Type VII collagen is composed of a collagenous domain linked by the noncollagenous (NC)1 and NC2 domains. OBJECTIVES: To assess the repeatability, sensitivity and specificity of a recently developed enzyme-linked immunosorbent assay (ELISA) for detection of anti-type VII collagen autoantibodies, and to ascertain whether they may be a marker of disease activity in EBA. METHODS: Using this ELISA, which was able to recognize autoantibodies against the NC1 and NC2 epitopes of type VII collagen, we tested 14 EBA sera, 30 healthy control sera and 113 disease control sera. RESULTS: In the EBA sera group, 12 out of the 14 samples were positive in ELISA, with autoantibody titres varying from 7·2 to 127·9UmL(-1) (cutoff value <6), the sensitivity of the method being 86%. Among the controls, only two bullous pemphigoid sera tested positive, the specificity being 98·6%. A good correlation was found between EBA disease severity, expressed as autoimmune bullous skin disorder intensity score, and the serum levels of anti-collagen VII autoantibodies, measured by ELISA (n =14; r=0·965; P=0·0001). The intra- and interassay coefficients of variation of the ELISA method ranged from 6·3% to 18·3%. CONCLUSIONS: This NC1+NC2 ELISA can be a practical assay for the diagnosis of EBA. The correlation between autoantibody titres and disease severity suggests its usefulness as a marker of disease activity in EBA However, this should be confirmed by studies on larger series of patients.

IgA anti-epidermal transglutaminase autoantibodies: a sensible and sensitive marker for diagnosis of dermatitis herpetiformis in adult patients.

BACKGROUND: Dermatitis herpetiformis (DH) is a rare gluten-sensitive blistering itchy skin disease, strictly related to coeliac disease (CD). Direct immunofluorescence, demonstrating IgA granular deposits localized either in the dermal papillae or along the dermo-epidermal junction, is currently the gold standard for diagnosis of DH. It has been shown that DH immunocomplexes contain epidermal transglutaminase (eTG) and that sera from patients with DH contain antibodies specifically directed against eTG. OBJECTIVES: We studied the usefulness of serum eTG antibodies in discriminating between DH, CD and other gastrointestinal and dermatologic diseases. METHODS: eTG antibodies were tested in 308 adult patients' sera: 44 patients with untreated dermatitis herpetiformis (UDH), 99 patients with untreated coeliac disease (UCD), 70 dermatological controls and 95 gastrointestinal controls. RESULTS: In UDH eTG antibody levels were significantly higher than in DH patients on gluten-free diet, UCD, gastrointestinal controls and dermatological controls. In UCD eTG antibodies strongly correlated with tissue transglutaminase (tTG) antibodies, whereas in UDH no significant correlation was observed. CONCLUSION: Serum IgA eTG antibody determination can efficiently distinguish UDH from other dermatological itchy diseases and is highly sensitive to gluten-free diet.

Erroneously low continuous ScvO2 reading due to red blood cells transfusion.

Hemoglobin oxygen saturation in superior vena cava (ScvO(2)) is used as a parameter to guide hemodynamic management in shock patients and it can be continuously read through a central venous catheter equipped with a fiberoptic spectrophotometric probe (Edwards PreSep catheter) connected to a specific monitor (Edwards Vigileo). We report of an episode of erroneous ScvO(2) reading by this technology in a patient with septic shock who was receiving an erythrocytes transfusion through the PreSep catheter main lumen. We think this artifact should be known by intensivists since it can lead to ScvO(2) misinterpretation and subsequent erroneous therapeutic decisions.

Isomorphic cutaneous graft-versus-host disease reaction after ultraviolet exposure: clinical, histological and direct immunofluorescence studies of four allo-transplanted patients.

BACKGROUND: Acute and chronic graft-versus-host disease (GVHD) continues to be a major limitation to successful haematopoietic stem cell transplantation. If experimental studies and clinical observations could partially elucidate the pathophysiology of acute GVHD, the biology of chronic GVHD is still much less well understood. OBJECTIVES: The aim of this study is to describe a peculiar photoinduced rash which triggered acute and then chronic lesions of GVHD in four allogenic haematopoietic-transplanted patients and discuss the possible aetiology and treatment. PATIENTS/METHODS: Four patients, two children and two adults affected by either mild or severe chronic GVHD, developed an erythematous rash on sun- or narrow-band ultraviolet B-exposed area, which triggered the onset of acute lesions of GVHD. Any of the patients presented neither a history of photosensitivity nor circulating autoantibodies nor urinary/fecal porphyrine. RESULTS: The histopathologic findings were characterized by an interface dermatitis with sparse perivascular infiltrate of lymphocytes and scattered necrotic keratinocytes, especially in the upper part of epidermis. Direct immunofluorescence studies excluded lupus-like pattern, revealing nests of fluorescent bodies at the dermal-epidermal junction and in papillary dermis. CONCLUSIONS: This peculiar isomorphic reaction of cutaneous GVHD after sun or narrow-band ultraviolet B exposures is described, and the possible mechanism involved is discussed. It may represent an interesting model of progression of chronic GVHD, starting with an acute stage and ending up with chronic clinical and histological findings, especially considering that there is no animal model that fully replicates all of the features of chronic GVHD in humans.

The Super-Magenstrasse and Mill operation with pyloroplasty: preliminary results.

BACKGROUND: Our strategy for performing various types of bariatric operations in order to make them suitable for the individual morbidly obese patient, has led us to take into account the original Magenstrasse and Mill (M&M) operation and modify it. This resulted in the so-called Super-Magenstrasse and Mill with pyloroplasty (SM&M-P). METHODS: In the past 3 years, 34 patients with mean BMI 48 and mean age 43 years underwent the SM&MP operation for morbid obesity. A digitoclasic pyloroplasty was performed and a 36-Fr bougie was used to calibrate the Magenstrasse. A 21-mm circular stapler was used to create a gastric window 10 cm proximal to the pylorus. The stapled division of the stomach to 3-4 cm from the pylorus and to the angle of His was performed first distally via the gastric window and then proximally from the window. RESULTS: 2 patients have reached 3 years since the operation: one could not be assessed due to the development of rectal cancer 1 year after surgery, and the other one has had an excellent outcome with 3-year BMI 26 kg/m2. 8 patients who underwent the surgery 2 years ago have a mean percent excess BMI loss (%EBL) of 69, and 19 patients who have reached 1 year have %EBL 64. All patients have experienced a clear reduction of appetite, with vomiting absent or rare. CONCLUSIONS: The SM&M-P procedure confers to this restrictive intervention some characteristics similar to gastric bypass, including rapid transit of the alimentary contents in the prepyloric "mill", scarse reflux into the gastric fundus, possible entero-endocrine effects and loss of interest in food.