DPO: Diffuse Pulmonary Ossification - A Diagnostic Challenge.

Diffuse pulmonary ossification (DPO) is a rare condition of DLD (diffuse lung disease) characterized by the presence of metaplastic ectopic bone in the lungs and is less frequent in patients without a clear background of lung diseases. DPO is characterized by very small calcific nodules, often with bone mature located in both lungs and often in peripheral areas of the lungs. Two patterns of DPO have been recognized dendriform and nodular. The dendriform type is less common and is characterized by a coral-like network of bone spiculae along the alveolar septa and is often related to interstitial fibrosis or chronic obstructive lung disease [1]. Recent literature papers indicate that DPO may be a predictor of pulmonary fibrosis, is related to Usual Interstitial Pneumonia (UIP) pattern, and has a higher correlation with Idiopathic Pulmonary Fibrosis (IPF). We present a case of a 41-years-old male with persistent bronchitis who underwent a chest X-ray (CXR) that showed multiple pulmonary small calcified nodules in both lungs. These findings were then defined with a high-resolution computed tomography of the chest (HRCT) that showed multiple small nodules spread in both lungs with a "tree-like pattern". A lung biopsy was performed to confirm the radiological diagnostic hypothesis of DPO, and further pathological examination showed multifocal areas of mature bone tissue within the lung parenchyma.

The Many Faces of Covid-19: Organizing Pneumonia (OP) Pattern HRCT Features.

Covid-19 (coronavirus disease 2019) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). On 30 January 2020 the World Health Organization (WHO) declared that the outbreak of Covid-19 realizes a public health emergency of international concern. Because of the primary involvement of the respiratory system, chest CT is strongly recommended in suspected Covid-19 cases, for both initial and follow-up. We present the case of a Covid-19 patient, a 57-year-old man, with a typical HRCT course of OP reaction.

Renal pathological changes in Fabry disease.

Fabry disease is a rare X-linked disorder, characterized by deficient activity of the lysosomal enzyme alpha-galactosidase A. This leads to systemic accumulation of the glycosphingolipid globotriaosylceramide (Gb3) in all body tissues and organs, including the kidney. Renal manifestations are less evident in female heterozygotes than in male hemizygotes, according to the Lyon hypothesis. Accumulation of Gb3 occurs mainly in the epithelial cells of Henle's loop and distal tubule, inducing early impairment in renal concentrating ability; involvement of the proximal tubule induces Fanconi syndrome. All types of glomerular cells are involved, especially podocytes, and glomerular proteinuria may occur at a young age. The evolution of renal Fabry disease is characterized by progressive deterioration of renal function to end-stage renal failure (ESRF). Ultrastructural study of kidney biopsies reveals typical bodies in the cytoplasm of all types of renal cells, characterized by concentric lamellation of clear and dark layers with a periodicity of 35-50 A. Management of progressive renal disease requires dietetic and therapeutic strategies, usually indicated in developing chronic renal failure, with dialysis and renal transplantation required for patients with ESRF. The recent development of enzyme replacement therapy, however, should make it possible to prevent or reverse the progressive renal dysfunction associated with Fabry disease.

[Problems of patients with schizophrenic disorders and of their families]. / I problemi dei pazienti con disturbi schizofrenici e delle loro famiglie.

OBJECTIVE: To evaluate psychopathological symptoms, disabilities and family burden in schizophrenic patients and to analyse predictors of family burden and relatives' satisfaction. DESIGN: Descriptive study of 203 patients with an ICD 10--F2 diagnosis (schizophrenia and related disorders) in contact with the Desio Department of Mental Health on 31st December 1994. SETTING: The Desio Department of Mental Health. MAIN OUTCOME MEASURES: The patients have been evaluated in three areas: disability (by ADC-DAS), psychiatric symptoms (by 24 items BPRS) and family burden (by Family Problems questionnaire). The outpatient, hospital and residential care contacts of the patients have been collected for six months by our service information system. For each area (DAS, BPRS and FP) a principal component analysis and a rotation of the significant components have been performed. Eleven factors, derived from three scales, have been retained as explanatory variables. Finally, a multiple regression analysis has been performed to assess the influence of explanatory variables on the set of response variables regarding family burden and relatives' satisfaction. RESULTS: One third of patients suffer of moderate-severe positive symptoms, while negative symptoms are less frequent. Manic symptoms are rare while depressive ones more frequent. Disability, related to work and sexual problems, is frequent; social withdrawn, underactivity, lack of participation in household duties and lack of self care are less frequent. Family burden is severe in one third of relatives, mainly in social relationships. Disability is the main predictor of family burden; manic and positive symptoms, time spent by the carer with the patient and carer's social support are less important. Satisfaction with services is predicted by family burden. CONCLUSIONS: To be more responsive to the needs of patients and relatives we should increase activities in rehabilitation and family support areas. Further analysis of severity of psychosocial and psychiatric problems, based on an epidemiological based sample, could give interesting results on the case-mix of different services.

Dupuytren's disease: recurrence and extension treated by selective aponeurectomy. A clinical review of 124 cases.

The treatment of Dupuytren's disease involves two problems: the amount of aponeurectomy and choice of the incision. It is the purpose of this study to critically review 124 cases of Dupuytren's disease treated by selective aponeurectomy, using the Skoog and/or Dieckman/Iselin routes of access. The results obtained appeared to be favorable as compared to other series, and this is attributed to the method used and to patient selection.

Bilateral aseptic necrosis of the humeral head following combined therapy for Hodgkin's lymphoma.

A young woman treated with combined therapy (chemo- and radiotherapy) for Hodgkin's disease later developed avascular necrosis of the humeral heads. The unusual location of the aseptic necrosis, and particularly the fact that it occurred bilaterally, made the case exceptional. The hypothesis is that the radiotherapy was a contributory factor.

Alternaria allergy: a three-year controlled study in children treated with immunotherapy.

In this prospective controlled study, 39 children with Alternaria-induced asthma and/or rhinitis were followed-up over a 3-year period. Skin tests and RAST were positive to Alternaria tenuis only. All children were treated with specific Alternaria immunotherapy. The long-term results have shown that immunotherapy was successful in 80% of the children given more than 80.000 PNU. By contrast, the outcome of 40 selected controls also followed during the same period who did not receive immunotherapy was almost exactly the opposite. In addition to demonstrating the clinical effectivity of specific Alternaria immunotherapy, the authors stress the relationship between successful results, highest tolerated doses, and larger cumulative dosage, which is irrespective of the duration of the therapy.