Transition from Childhood to Adulthood in Patients with Duchenne Muscular Dystrophy.

Recently, progress has been observed in the knowledge about Duchenne Muscular Dystrophy (DMD), which is a severe and commonly diagnosed genetic myopathy in childhood, historically resulting in early death. Currently, there are a lot of methods available to improve the clinical course of DMD and extend patients' life expectancy to more than 30 years of age. The key issue for DMD patients is the period between 16-18 years of age, which is described as a transition from pediatric- to adult-oriented healthcare. Adolescents and adults with DMD have highly complex healthcare needs associated with long-term steroid usage, orthopedic, ventilation, cardiac, and gastrointestinal problems. The current paper provides a comprehensive overview of special healthcare needs related to the transfer of a patient with DMD from child-oriented to adult-oriented care. Additionally, the need to organize effective care for adults with DMD is presented.

Oral premedication in patients with a history suggesting hypersensitivity to iodinated contrast media.

INTRODUCTION: Iodinated contrast media (ICM) are pharmaceuticals widely used in diagnostic procedures. Adverse effects associated with their administration are quite frequent and mostly mild. However, they raise concerns in patients and doctors in the context of their future use. AIM: To determine efficacy of premedication before medical procedures with the use of iodinated contrast media in patients with a history suggesting a hypersensitivity reaction after their past use. MATERIAL AND METHODS: Out of 152 patients consulted due to adverse reactions after ICM (85 women and 67 men, aged 43-90), 101 were selected with the history suggesting a mild hypersensitivity reaction (urticaria, itching, skin redness, malaise etc.). All the patients had health problems requiring a procedure with ICMadministration in the near future. The premedication was given with cetirizine (10 mg) and prednisone (20 mg or 50 mg, randomly assigned) 13, 7 and 1 h before the ICM administration. Presence of adverse events was compared between the subgroups with χ 2 test and efficacy of premedication - with Wilcoxon test. RESULTS: Seventy-six patients underwent the radiologic procedure with premedication with antihistamine and a lower (40 patients) or higher dose (36 patients) of prednisone. Four of them reported a cutaneous hypersensitivity reaction (urticaria, itching, redness) and one - dyspnoea. There was no statistically significant difference in relation to the premedication protocol (p = 0.1306). CONCLUSIONS: Premedication with cetirizine and prednisone before radiologic procedures proved to be efficient in patients with a history suggesting hypersensitivity to iodinated contrast media. There was no significant difference in efficacy related to the dose of prednisone (20 mg vs. 50 mg).

Histoplasmosis in an elderly polish tourist - a case report.

BACKGROUND: Histoplasmosis is a mycosis caused by soil-based fungus Histoplasma capsulatum endemic in the USA, Latin America, Africa and South-East Asia. The disease is usually self-resolving, but exposure to a large inoculum or accompanying immune deficiencies may result in severe illness. Symptoms are unspecific with fever, cough and malaise as the most common. Thus, this is a case of disease which is difficult to diagnose and very rare in Europe. As a result, it is usually not suspected in elderly patients with cough and dyspnea. CASE PRESENTATION: This is a case of a 78-year-old patient, admitted to our department due to respiratory failure, cough, shortness of breath, fever and weight loss with no response to antibiotics administered before the admission. Chest CT revealed numerous reticular and nodular infiltrations with distribution in all lobes. The cytopathology of BAL showed small parts of mycelium and numerous oval spores. Considering clinical presentation and history of travel to Mexico before onset of disease, pulmonary histoplasmosis was diagnosed. After introduction of antifungal treatment rapid improvement was achieved in terms of both clinical picture and respiratory function. CONCLUSIONS: Since the risk of Histoplasma exposure in Europe is minimal, patients, who present with dyspnea, fever and malaise are not primarily considered for diagnosis of histoplasmosis. However, taking into account increasing popularity of travelling, also by elderly or patients with impaired immunity, histoplasmosis should be included into differential diagnosis.

Venom Immunotherapy Does Not Affect Survival of Patients with Malignant Tumor in Poland.

Background: Allergen immunotherapy (AIT) is a well-established and efficient method of causative treatment for allergic rhinitis, asthma and insect venom allergy. Traditionally, a recent history of malignant neoplasm is regarded as a contraindication to AIT due to concerns that AIT might stimulate tumor growth. However, there are no data confirming that the silencing of the Th2 response affects prognosis in cancer. Objectives: The aim of this study was to investigate frequency of malignant tumors in patients undergoing AIT and the association between AIT and cancer-related mortality. Patients and Methods: A group of 2577 patients with insect venom allergy undergoing AIT in 10 Polish allergology centers was screened in the Polish National Cancer Registry. Data on cancer type, diagnosis time and patients' survival were collected and compared with the general population. Results: In the study group, 86 cases of malignancies were found in 85 patients (3.3% of the group). The most common were breast (19 cases), lung (9 cases), skin (8 cases), colon and prostate cancers (5 cases each). There were 21 cases diagnosed before AIT, 38 during and 27 after completing AIT. Laplace's crude incidence rate was 159.5/100,000/year (general population rate: 260/100,000/year). During follow-up, 13 deaths related to cancer were revealed (15% of patients with cancer). Laplace's cancer mortality rate was 37.3/100,000/year (general population rate: 136.8/100,000/year). Conclusions: Malignancy was found in patients undergoing immunotherapy less often than in the general population. Patients with cancer diagnosed during or after AIT did not show a lower survival rate, which suggests that AIT does not affect the prognosis.

[Coexistence of sarcoidosis with seminoma--a case report]. / Wspólistnienie sarkoidozy i nasieniaka jadra ­ opis przypadku.

A 30-year-old patient, with diagnosis of seminoma (T1 Nx Mx) was treated radically with orchidectomy. In chest CT performed postoperatively numerous diffuse nodules were revealed in both lungs. Lesions were situated particularly in the upper and middle pulmonary zones. In order to verify the nature of pulmonary abnormalities videothoracoscopy of the right pleural cavity was performed with specimen collection. Histopathological examination excluded the possibility of cancer metastases to pulmonary parenchyma and revealed the presence of sarcoid-like granulomas. Coexistence of seminoma and diffuse sarcoid-like abnormalities is only sporadically described. Up till now it has not been unequivocally explained whether the pulmonary abnormalities develop in the course of idiopathic sarcoidosis or only reflect a sarcoid-like reaction to cancer antigens.

A portable automatic cough analyser in the ambulatory assessment of cough.

BACKGROUND: Cough is one of the main symptoms of advanced lung disease. However, the efficacy of currently available treatment remains unsatisfactory. Research into the new antitussives requires an objective assessment of cough. METHODS: The aim of the study was to test the feasibility of a new automatic portable cough analyser and assess the correlation between subjective and objective evaluations of cough in 13 patients with chronic cough. The patients' individual histories, a cough symptom score and a numeric cough scale (1-10) were used as a subjective evaluation of cough and a computerized audio-timed recorder was used to measure the frequency of coughing. RESULTS: The pre-clinical validation has shown that an automated cough analyser is an accurate and reliable tool for the ambulatory assessment of chronic cough. In the clinical part of the experiment for the daytime, subjective cough scoring correlated with the number of all cough incidents recorded by the cough analyser (r = 0.63; p = 0.022) and the number of cough incidents per hour (r = 0.60; p = 0.03). However, there was no relation between cough score and the time spent coughing per hour (r = 0.48; p = 0.1). As assessed for the night-time period, no correlation was found between subjective cough scoring and the number of incidents per hour (r = 0.29; p = 0.34) or time spent coughing (r = 0.26; p = 0.4). CONCLUSION: An automated cough analyser seems to be a feasible tool for the ambulatory monitoring of cough. There is a moderate correlation between subjective and objective assessments of cough during the daytime, whereas the discrepancy in the evaluation of night-time coughing might suggest that subjective analysis is unreliable.

[Lung microangiopathy in diabetes]. / Mikroangiopatia plucna w przebiegu cukrzycy.

Diabetes mellitus (DM) is the metabolic disorder, which is characterised by persistent hyperglycaemia and abnormal metabolism of carbohydrates, proteins and lipids. These metabolic disorders result from impaired insulin secretion, altered tissue sensitivity to insulin or the coexistence of both these mechanisms. Chronic DM usually results in micro- and macroangiopathy, which in turn may have a negative impact on the function of internal organs. Microangiopathy specifically affects eyes (retinopathy), kidney (nephropathy) and peripheral nervous system (neuropathy). Little is known about the influence of diabetic microangiopathy on lung function. A few available papers describe lung function and lung diffusing capacity for carbon monoxide (DLCO) impairment in patients with both DM type 1 and type 2. Reduction of DLCO can indicate, however, that DM leads to alveolar-capillary barrier damage in the lung. In this paper authors review available literature on microangiopathy and its influence on the lung function.

Analysis of safety, risk factors and pretreatment methods during rush hymenoptera venom immunotherapy.

BACKGROUND: The safety profile of venom immunotherapy is a relevant issue. We evaluated the frequency of severe adverse events (SAE), associated risk factors, retrospective comparison of pretreatment protocols including solely H1 receptor blockers and a combination of H1 and H2 receptor blockers during rush Hymenoptera venom immunotherapy. METHODS: The study group comprised 118 patients. The treatment was initiated according to a 5-day rush protocol with the use of standardized venom allergens of either wasp or honeybee. RESULTS: During the rush induction, side effects occurred in 18 patients (15.2%), whereas SAE were present in 7 patients (5.9%). Twelve out of 18 (66.6%) developed anaphylactic reactions on the fourth day of the rush protocol, with the majority of cases at a dose of 40 or 60 microg of the venom extract (p = 0.001). The frequency of SAE was also significantly higher on the fourth day than thereafter (p = 0.0001) as well as in patients allergic to bee venom (p = 0.049). All systemic side effects were more frequent in women (p = 0.0065). However, this relation was not true when SAE were consider (p = 0.11). A higher percentage of SAE was observed in the subjects pretreated with both H1 and H2 receptor antagonists than in those pretreated with H1 blocker only (8.8 vs. 4.1%); however, the difference was not significant. CONCLUSIONS: Considerable severity of allergic adverse events requires particular attention to patients allergic to bee venom and during rush phase, especially when rapidly increasing doses are administered. Pretreatment with H2 blockers is debatable and warrants further investigation.

[Hyperlucent lung syndrom caused by pulmonary artery hypoplasia in patient with diagnosed asthma--case report]. / Zespól jednostronnie jasnego pluca w przebiegu hipoplazji tetnicy plucnej u chorej z objawami astmy oskrzelowej--opis przypadku.

We present case of 67-years-old, non-smoking woman with unilateral hyperlucent lung syndrome. She has diagnosed asthma and since 1997 she has been treated with inhaled corticosteroids and long-acting beta-agonists without improvement. She complained of a cough, shortness of breath, pulmonary function test reveal irrvesibility airflow obstruction. The routine X-ray chest showed unilateral hyperlucent left lung. Ct-angiography has shown unilateral hypoplasia of pulmonary artery. It indicates that in all cases of uncontrolled asthma should be considered another or coexisting diagnosis.

[Latent tetany masked by syndroms of bronchial asthma and depressive syndrome. Case report]. / Astma oskrzelowa i zespól depresyjno-lekowy maskujace utajona tezyczke. Opis przypadku.

Tetany, which occurs in young women, poses common diagnostic problem. Two types of tetany are distinguished: one which is characterized by hypocalcemia and which rarely occurs (in person after strumectomy in the course of post-operational hypoparathyroidism) and latent one, which occurs more often. In the literature there is a lack of precise data concerned witch most probably results from its underestimation. Clinical symptoms which appear in latent tetany (normocalcemic) are related to the intracellular magnesium deficiency and increased respiratory drive. A noncharacteristic clinical picture and the lack of a pathognomonic symptom, cause that despite of the fearly common occurrence, the latent tetany is rarely recognized. In this paper the case of a 53 year old women has been described. The women had the symptoms of gastroesophageal reflux of asthma and depressive syndrome, witch masked the symptoms of the latent tetany.