Nailfold videocapillaroscopy findings in bradykinin-mediated angioedema

Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitors (ACEI-AAE) are types of bradykinin-mediated angioedema without wheals characterized by recurrent swelling episodes. Recent evidence suggests that a state of “vascular preconditioning” predisposes individuals to attacks, although no data are available on possible structural alterations of the vessels. Objective: This study aims to compare the features of nailfold capillaries to highlight possible structural anomalies between patients affected by C1-INH-HAE and controls and between patients with ACEI-AAE and hypertensive controls. Methods: We used nailfold videocapillaroscopy (NVC) to assess the following: apical, internal, and external diameter; loop length; intercapillary distance; and capillary density, distribution, and morphology. Plasma levels of vascular endothelial growth factor (VEGF) A, VEGF-C, angiopoietin (Ang) 1, and Ang2 were also measured. Results: Compared with healthy controls (n=28), C1-INH-HAE patients (n = 34) were characterized by significant structural alterations of the capillaries, such as greater intercapillary distance (216 vs 190 µm), increased apical, internal, and external diameter (28 vs 22 µm; 22 vs 20 µm; and 81 vs 65 µm, respectively), decreased density (4 vs 5 capillaries/mm2), more irregular capillary distribution, and more tortuous morphology. Apical diameter was enlarged in patients with ≥12 attacks per year. In ACEI-AAE patients, NVC showed no alterations with respect to hypertensive controls. NVC performed in 2 C1-INH-HAE patients during attacks showed no changes compared with the remission phase. Conclusions: We detected major structural capillary alterations in C1-INH-HAE patients, thus confirming the involvement of microcirculation in the pathogenesis of angioedema

Serum resistin levels in children with primary snoring.

Primary Snoring (PS) has been positioned at the milder end of the Sleep-Disordered Breathing severity continuum characterized by snoring and it is usually underestimated. PS is defined as snoring without apnea, frequent arousals, or gas exchange abnormalities and recent studies demonstrated that children with PS have increased blood pressure and reduced arterial distensibility. The association between adipokines and SDB has been recently investigated, though most of the studies were focused on OSAS where intermittent hypoxia characterizing the disease may lead to an inflammatory cascade and to the release of several adipokines, contributing to oxidative stress. Resistin, initially described as an adipokine increasing insulin resistance, has been recently identified as a novel important member of the cytokine family involved in the regulation of inflammation. The aim of our study was to investigate circulating resistin levels in normal weight children with PS. Sixty-five children of normal weight aged between 4 and 14 years of age were selected for habitual snoring. Children with positive polysomnography were excluded from the study. Serum resistin levels were detected in all children with PS. Thirty-three healthy non-snorer children with similar age, sex and BMI were selected as a control group. A significantly higher level of resistin was observed in patients with PS compared to the control group (4.67±1.91 ng/ml vs 3.98±1.58 ng/ml; p<0.01). Patients with inconclusive pulse oximetry showed significantly higher resistin levels than those with negative recordings (5.29±1.91 ng/ml vs 4.20±1.93 ng/ ml; p<0.008). Moreover, there was a significant increasing trend between sieric adipokine level and the frequency of snoring (p<0.006). Our results suggest that systemic inflammation and oxidative stress may also play a significant role in the pathophysiology of PS

Un caso di sbagliata diagnosi di allergia alla penicillina

Vi presentiamo il caso clinico di una bambini di 8 anni con diagnosi di malattia reumatica. Nell'anamnesi familiare si segnalano madre e sorella con deficit del citocromo p450 con conseguente allergia ritardata a diverse classi di farmaci

Allergia alimentare e sviluppo di asma

Le allergie sono patologie multifattoriali in cui sviluppo dipende dall'associazione di fattori ambientali e genetici. La loro prevalenza soprattutto in età pediatrica è in continuo aumento

Studio pediatrico sui fenotipi della rinite allergica in Italia

La rinite allergica (RA) è una patologia causata da iperproduzione di IgE specifiche verso allergeni ambientali innocui e ubiquitari con infiammazione della mucosa nasale che può interessare organi contigui e distanti anche attraverso riflessi neuronali e processi infiammatori sistemici

Long-term power spectral analysis in angioedema: proposal of a translational approach

C1 inhibitor hereditary angioedema (C1-INH-HAE) is a rare disease characterized by self-limiting edema associated with localized vasodilation due to increased levels of bradykinin. Although an involvement of the autonomic nervous system has been demonstrated by means of the analysis of the heart period (HP) variability during remission, no evaluation of the cardiac autonomic profile in the prodromal phase and during an attack has been performed. We positioned a multiday electrocardiogram recorder in a C1-INH-HAE 46-years old female patient until an attack occurrence. The HP variability indices were computed in the day of the attack and in the day before, in the 4 hours preceding (PRE) and following the attack. We found that the index related to the vagal modulation directed to the sinus node increased in the PRE period during the day of the attack. This is explainable by the expected vasodilation linked to the release of bradykinin that is the main mediator of the attack. We conclude that the HP parameters linked to the vagal cardiac modulation could be exploited and tested in future studies as early markers of the angioedema attack. The possibility to predict the attack could be of interest in the development of drugs to prevent the attack itself

Livelli sierici di resistina in bambini con Rinite Allergica

La resistina è una proteina sierica prodotta dagli adipociti e dai macrofagi circolanti con dimostrata attività proinfiammatoria. Molti autori hanno descritto un aumento dei livelli di questa adipochina sierica negli atopici con malattie respiratorie come la rinite allergica (RA)

WHEN BRONCHIAL OBSTRUCTION IS NOT ONLY ASTHMA: A CASE OF CONGENITAL CYSTIC ADENOMATOID MALFORMATION TYPE 0

Congenital cystic adenomatoid malformation (CCAM) is one of the most common congenital lung anomalies. It is a rare pulmonary alteration, characterized by lung tissue dysplastic or hamartomatous, mixed with normal tissue. The injury is likely related to an insult during embryological development with altered terminal bronchiolar structures. We describe an unusual case of a boy came to our observation at the age of 12 years, for mild persistent bronchial asthma with exercise induced asthma, allergic to dust mites, pollens of Grasses, Cypress and to epithelium of the cat. At birth, respiratory distress treated with oxygen therapy, which resolved on the second day of life. For the first two years of life, he had recurrent episodes of wheezing requiring bronchodilator therapy and oral corticosteroids; subsequently, he presented rare episodes of bronchospasm, and asthma induced from intensive exercise. He practiced martial arts at a competitive level. The routine spirometry showed FEV1 58.5% and MEF50 24.7%, values that were not reversible after salbutamol, despite therapy with CSI + LABA. Chest x-ray showed thickening of the right parietal pleura and of the apical one and evidence of fibrotic striae. The chest HRCT revealed mosaic bilateral parenchymal destruction, bronchiectasis and multiple formations nodules like, poly-lobed with blurred margins, the larger of a diameter of 12 mm. The bronchioloalveolar lavage was normal. Inflammatory, autoimmune, infectious and other congenital diseases were excluded. The histology of the lung biopsy obtained by thoracotomy surgery, suggested the diagnosis of CCAM type 0 without malignancy