Aldo Naselli

Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: Case report and review of literature

Abstract Objectives: To review the data available in literature about nebulized amphotericin B (AMB) in the treatment of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) and to report our experience in the use of this drug, with a particular therapeutic scheme. Case Report: We used nebulized liposomal amphotericin B (L-AMB) in a patient affected by CF, complicated by ABPA. The previous combined treatment with oral steroids and azoles had no respiratory benefit and caused relevant side effects. Amphotericin B has always been well tolerated and permitted a slight steroid tapering. We also observed benefits in pulmonary function and laboratory tests. Conclusions: Few data are available in literature about the use of nebulized AMB in CF and there are no RCTs evaluating antifungals in CF-ABPA. In our opinion, the reported case suggests that nebulized L-AMB could represent a possible strategy in ABPA management in CF patients.

Persistence of high-level (1,3)-β-D-glucan after candidemia following autologous peripheral SCT in a pediatric patient.

Persistence of high-level (1,3)-β- D -glucan after candidemia following autologous peripheral SCT in a pediatric patient

Acute post-infectious cerebellar ataxia due to co-infection of human herpesvirus-6 and adenovirus mimicking myositis

Acute cerebellar ataxia (ACA) is a relatively common neurological disease in children. Most common types of ACA are acute post-infectious (APCA) and acute disseminated encephalomyelitis (ADEM). Less common but important causes include opsoclonus-myoclonus syndrome (OMS) and acute cerebellitis. Cerebellar neoplasms and acute hydrocephalus are additional causes of paediatric ataxia. APCA is the most common cause of ACA in children, comprising about 30-50% of total cases. This is a report about an immunocompetent 4-yrs-old male affected by APCA, due to co-infection by human herpesvirus-6 (HHV-6) and adenovirus, with symptoms mimicking myositis.

Management of nontuberculous mycobacterial lymphadenitis in a tertiary care children's hospital: A 20 year experience

PURPOSE Nontuberculous mycobacteria are uncommon cause of chronic cervicofacial lymphadenitis in healthy children. We describe clinical features and management strategies of cervicofacial nontuberculous mycobacterium lymphadenitis in a tertiary pediatric hospital. METHODS Retrospective analysis of medical records of children discharged from 1992 to 2014 with a diagnosis of cervicofacial nontuberculous mycobacterium was made. Diagnosis certainty was based on microhistological investigations. Clinical stage was evaluated according to lymph node size and presence of fistulas. Successful therapy was defined by the regression of the lymph node enlargement (>75%) or complete surgical excision without relapse. RESULTS Cervicofacial nontuberculous mycobacterium was diagnosed in 33 patients. Complete excision was performed in 73% of cases primarily observed in our hospital, while 83% of those referred from other hospitals required further surgical treatment. No case of relapse was observed after one year of follow-up. CONCLUSIONS We recommend surgical approach as the first therapeutic option in the management of cervicofacial nontuberculous mycobacterium lymphadenitis. LEVELS OF EVIDENCE Prognosis and Retrospective Study - Level II.

Inhaled hypertonic saline+hyaluronic acid in cystic fibrosis with asthma-like symptoms: a new therapeutic chance

The aim of the paper is to report the case of a boy affected by cystic fibrosis, with non-ABPA-related recurrent wheezing and frequent pulmonary exacerbation during childhood, who had been inhaling 7% NaCl+0.1% hyaluronic acid (HA) as a maintenance therapy. We reviewed patient database and, analysing a 7-year follow-up, considered pulmonary exacerbation, antibiotic and steroid courses, pulmonary function (forced expiratory volume in one second; FEV1) and microbiological data. After starting 7% NaCl+0.1% HA treatment, we observed a dramatic decrease of oral antibiotic need (0.55 courses/month during the pretreatment period against 0.10 courses/month in the treatment period), associated with a good initial recovery and a stability of FEV1. In our opinion this case could suggest an extended indication for inhaled 7% NaCl+0.1% HA use in CF, not only in patients who did not tolerate hypertonic saline, but also in patients with coexistent asthma-like symptoms.

A long-term follow-up of residual mass neuroblastoma in a patient with cystic fibrosis

Purpose To report a long-term follow-up of a young woman affected by cystic fibrosis (CF) with a residual retroperitoneal mass of neuroblastoma (NBL) after treatment. Case report We reviewed the patients database and analysed a 20-year follow-up by considering pulmonary exacerbation, nutritional condition, pulmonary function (forced expiratory volume in 1 s), microbiological data and residual retroperitoneal mass volume. We observed stable pulmonary and nutritional conditions. No variation was found in the residual retroperitoneal mass volume. Discussion We report this case of a patient with CF with previous NBL because such a long time of follow-up of a NBL with a stable retroperitoneal remaining tumour is uncommon and needs to be reported. Multidisciplinary management has been crucial in this case because of the presence of concomitant diseases and consequently, differential diagnosis challenges.

Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

Bacterial respiratory infections have an important impact on the development and progression of pulmonary disease in cystic fibrosis (CF). Viral infections are possible triggers of acute deterioration in the clinical status of CF patients. Macrophage activation syndrome (MAS) is a life‐threatening complication of rheumatic disease characterized by pancytopenia, hepatitis, hyperferritinemia, coagulopathy, and neurologic symptoms. This syndrome is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and well‐differentiated macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Here, we report the case of a boy affected by CF who developed MAS triggered by pandemic H1N1 influenza; good clinical response was obtained through high dose prednisone treatment. Pediatr Pulmonol. 2014; 49:E10–E12.

Comment to Letter to the Editor regarding the article by R. Casciaro et al. titled ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: case report and review of literature’

Comment to Letter to the Editor regarding the article by R. Casciaro et al. titled ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: case report and review of literature’ Rosaria Casciaro, Federico Cresta, Aldo Naselli, Elio Castagnola Cystic Fibrosis Center, IRCCS Istituto Giannina Gaslini, Genova, Italy, Infectious Diseases Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy

Successful management of cutaneous BCG dissemination in a child affected by SCID and receiving allogeneic hematopoietic stem cell transplant

Successful management of cutaneous BCG dissemination in a child affected by SCID and receiving allogeneic hematopoietic stem cell transplant

Tuberculosis diagnosed after chemotherapy for presumed mediastinal malignant neoplasia.

To the Editor: Mediastinum represent a kind of Pandora’s box in young children since the many structures here contained may present a lot of diseases such as congenital cysts, infections, benign tumours, and primary and metastatic malignant neoplasms [1]. Many of these conditions may be asymptomatic and the lesions discovered incidentally on chest X-rays, but more than 80% of mediastinal masses in children aremalignant, mostly lymphomas or neurogenic tumours [2]. A 5-months-old female was referred for a suspect mediastinal neuroblastoma treated unsuccessfully with two doses of cyclophosphamide in another centre. Diagnosis was based on the presence of a large mediastinal mass but not confirmed by biopsy or bone marrow evaluation. At time of admission, chest X-ray and CT scan (Fig. 1A–C) confirmed the presence of an expansive process of the middle and posterior mediastinum with slight dislocation and deformation of the trachea. The main right bronchus appeared to be compressed and right lung appeared to be extremely diaphanous and with two focal lesions at the level of the advanced lobe. The child presented severe respiratory distress that contraindicated general anaesthesia for biopsy and therefore chemotherapy according to AIEOP NB 99 protocol (carboplatin 6.6mg/kg, etoposide 5mg/kg days 1–3 for each cycle) were started with the aim of reducing the volume of the mass, allowing a biopsy. Near 10 days after the end of this treatment a chest CT scan showed a worsening of the pulmonary lesions, with increase of the dimensions of the mediastinal mass, while the secondary lesions were unchanged. Tuberculin skin test (TST) was performed and resulted positive with a diameter of 15mm. Interferon Gamma Release Assay (IGRA) was not available at that time. Gastric aspirate samples resulted positive for acid-fast bacilli and culture yielded a strain of Mycobacterium tuberculosis. A diagnosis of primary tuberculosis (TB) was made and the patient received therapy with isoniazid, rifampin, pyrazinamide, ethambutol, and steroids with gradual improvement of respiratory symptoms and mediastinal lesions. We described a 5 old month female with a large madiastinal mass dislocating and compressing the respiratory structures that was initially misinterpreted as neuroblastoma and treated accordingly with chemotherapy, but finally diagnosed as primary TB. In patients with cancer, TST may result negative or slightly positive, especially after administration of chemotherapy [3]. In our patient, on the contrary, TSTwas highly positive in spite of three cycles of antineoplastic drugs. Themost recent edition of the Red Book states that “an initial TST or IGRA should be performed before initiation of immunosuppressive therapy, including prolonged corticosteroid administration, use of TNF-alpha antagonists, or other immunosuppressive therapy in any child requiring these treatments” [4]. Despite this recommendation, many patients are not routinely tested for latent TB prior of chemotherapy. This case clearly shows that TST can be still highly positive after chemotherapy, and