2014, Vol 10, No 4
Clinical pictures of phenotypes of intolerance to aspirin and other nonsteroidal anti-inflammatory drugs. Part I
Pediatr Med rodz Vol 10 Numer 4, p. 346–359
DOI: 10.15557/PiMR.2014.0036

Clinically, three phenotypes of intolerance to aspirin and other nonsteroidal anti-inflammatory drugs are distinguished: bronchospastic phenotype, urticaria/oedema and chronic hyperplastic eosinophilic sinusitis. Recently, the term aspirinexacerbated respiratory disease has been proposed for an aspirin-intolerant respiratory disease. The bronchospastic phenotype of aspirin sensitivity, called aspirin-intolerant asthma, occurs only in patients with asthma. In these individuals, the symptoms of aspirin sensitivity include dyspnoea and extrabronchial symptoms, such as: watery rhinorrhoea, conjunctivitis and lacrimation, flushing of the face and neck, oedema of the larynx, fall in blood pressure and even death. Aspirin-intolerant urticaria/angioedema occurs mainly in patients with chronic or recurrent urticaria and angioedema. The typical features of aspirin-intolerant asthma are nasal and paranasal polyps. They occur almost in 80% of patients with aspirin-intolerant asthma, and in only 3% of those with aspirin-intolerant urticaria. Bronchial and nasal mucosae are inflamed mainly with eosinophils. Aspirin-intolerant asthma and urticaria/angioedema can occur at any age, but they especially affect women between 30 and 50 years of age. In about 50% of aspirin-intolerant asthmatics, atopic features were found. The clinical course of aspirin-intolerant asthma is usually severe, but total or partial control can be achieved with the use of inhaled corticosteroids and long-acting β2-agonists. The authors assume that anamnesis plays the major role in the detection of intolerance to aspirin. Oral challenge tests should be applied only with the use of acetylsalicylic acid, administered at low, increasing doses at intervals not shorter than 24 hours. The majority of nonsteroidal anti-inflammatory drugs elicit dyspnoea in patients with aspirin-intolerant asthma and skin eruptions in those with aspirin-intolerant urticaria. Sometimes nasal and inhalation tests with lysine aspirin are performed. These tests are safer, but less sensitive and for that reason, oral challenge with acetylsalicylic acid is treated as “the gold standard.” In patients with aspirin-intolerant asthma and aspirin-intolerant urticaria, tolerance to acetylsalicylic acid is achieved by using increasing doses of aspirin.

Keywords: sensitivity to aspirin, aspirin-intolerant asthma, aspirin-intolerant urticaria, aspirin-intolerant angioedema, nonsteroidal anti-inflammatory drugs, tolerance to aspirin
Selected problems that may be the cause of cardiogenic syncope in children and adolescents. Useful information for family doctor and paediatrician
Pediatr Med rodz Vol 10 Numer 4, p. 360–375
DOI: 10.15557/PiMR.2014.0037

Children with syncope are common patients in paediatric and family doctor clinics as well as emergency departments. The current guidelines of the European Society of Cardiology (2009) distinguish between three types of syncope: reflex, orthostatic hypotension and cardiac syncope. The essence of any syncope is the reduction of cerebral blood flow, loss of consciousness and a subsequent fall. The most common and the mildest form of syncope is reflex syncope, usually preceded by prodromal symptoms, while the rarest but also the most dangerous form is cardiac syncope. Cardiac syncopes are generally organic in origin and it is usually possible to determine their cause. In some cases it is possible to treat the cause of cardiac syncopes. They differ from reflex syncopes in the mechanism of origin: they usually do not have prodromal symptoms, and sometimes the first symptom of an ongoing disease can be syncope and even sudden cardiac death. Cardiac syncope affects patients with cardiac arrhythmias – tachy- or bradyarrhythmias, patients who suffer from malfunction of a previously implanted pacemaker, patients with defects that cause disturbed outflow from the right or the left ventricle, patients with defects and diseases of the heart muscle and/or coronary vessels as well as abnormalities causing impediment to the flow of blood within the heart or vessels (e.g. tumours). Some of these diseases such as, for example, hypertrophic cardiomyopathy or long QT syndrome are genetic abnormalities. In cardiac syncope, as in other types of syncope it is important to obtain a detailed family history and perform specialised tests. In recent years tilt tests have been increasingly less used and emphasis has been put on a very detailed diagnosis of cardiac arrhythmias as a potential cause of syncope and sudden cardiac death. Every patient with a suspected cardiac syncope should be referred to a specialist centre for detailed diagnosis.

Keywords: syncope, cardiac arrhythmias, prolonged QT interval, sudden cardiac death
Long QT interval in resting electrocardiogram – and what next? Sports cardiologist’s point of view Part 2. From provocative tests to prevention of cardiac events
Pediatr Med rodz Vol 10 Numer 4, p. 376–384
DOI: 10.15557/PiMR.2014.0038

Resting electrocardiogram is a basic diagnostic tool used to identify myocardial diseases caused by a genetic defect of ion channels that results in bioelectric instability of cardiomyocytes and the risk of a complex induction of life-threatening ventricular arrhythmia. Long QT syndrome is the most common genetic ion channel disease affecting the general population. Congenital long QT syndrome is a heterogeneous group of ion channel genetic diseases, which manifest as a prolongation of QT interval on resting electrocardiogram. Some of the patients with long QT syndrome show QTc interval within the normal range, which is associated with the risk of false negative diagnosis. The use of function or pharmacological tests in patients with nondiagnostic QTc interval, but with a justified suspicion of long QT syndrome may provoke manifestation of phenotypic features of channelopathies. Prevention of cardiac events in patients with long QT syndrome involves, among others, the use of beta blockers. Implantation of a cardioverter-defibrillator is recommended in patients who developed cardiac arrest, and left cervicothoracic sympathectomy is also recommended in some cases. In terms of everyday functioning, patients with long QT syndrome should avoid drugs that prolong QT interval and have their electrolyte abnormalities corrected. Participation of long QT syndrome patients in competitive sports is still a matter of debate among the experts. The second part of the study, which is devoted to the prolonged QT interval on the electrocardiogram, focuses on provocation tests (both function and pharmacological tests) used in the diagnostics of long QT syndrome. Methods of risk stratification for sudden cardiac events in patients diagnosed with long QT syndrome, current possibilities associated with prevention and treatment as well as recommendations related to sports participation are also discussed.

Keywords: prolonged QT interval, provocation testing, risk stratification, sports participation
Role of lung ultrasound in paediatric patients
Pediatr Med rodz Vol 10 Numer 4, p. 386–396
DOI: 10.15557/PiMR.2014.0039

Transthoracic lung ultrasound is ultrasonographic assessment of the lungs, parietal and visceral pleura and the space between these two membranes. It is a part of chest ultrasound examination which also includes assessment of the ribs, intercostal spaces, diaphragm, heart and mediastinum. Lung ultrasound as a diagnostic tool which has been known for 30 years, but its advantages and disadvantages are not commonly known and therefore it is not routinely used by clinicians. In paediatrics, lung ultrasound has become more popular since the first decade of the 21st century. The main advantage of this method is no exposure to ionizing radiation. Transthoracic lung ultrasound can be performed in practically any conditions and repeated several times. What is more, its accessibility, reliability, accuracy and low cost compared to other methods, makes lung ultrasound the great tool for lung assessment. It can be used both for initial diagnosis and monitoring of treatment (bedside examinations are possible). In this paper, we describe the technique of lung ultrasound examination, equipment requirements, normal lung ultrasound appearance, disadvantages of the examination and lung ultrasound findings in the most common paediatric lung diseases, such as: pneumonia, pleural fluid, atelectasis, pneumothorax, bronchiolitis, pulmonary oedema and pulmonary embolism.

Keywords: ultrasonography, transthoracic lung ultrasound, lung diseases, children, paediatrics
Value of ultrasound in the diagnostics of abdominal wall hernias
Pediatr Med rodz Vol 10 Numer 4, p. 398–404
DOI: 10.15557/PiMR.2014.0040

The aim of this paper was to present current state of knowledge and clinical practice in the scope of imaging of anterior abdominal wall hernias. At the moment, diagnostic imaging of abdominal wall hernias utilizes such modalities as ultrasound, computed tomography and magnetic resonance. The two latter methods are not easily available, expensive and usually require filling the intestine with contrast medium or, at times, additional administration of intravenous contrast. Moreover, computed tomography exposes the patient to negative effects of ionizing radiation, and both modalities are contraindicated in claustrophobic patients or patients with renal failure when examination requires intravenous administration of contrast. Under such circumstances, ultrasound examination constitutes the basis of imaging in cases of suspected anterior abdominal wall hernias and in patients with palpable masses in such a location. It ensues from high availability, low cost, noninvasiveness and high diagnostic value of this modality as well as its applicability in all life periods – from foetal life up to old age. Other advantages of ultrasonography include: possibility to conduct the examination dynamically at patient’s bedside, including application of various tests facilitating diagnosis of small, spontaneously reducing and immovable inguinal, femoral, or umbilical hernias, hernia of the linea alba, Spigelian hernia, or various other incisional hernias that pose diagnostic challenges. Ultrasound examination allows for assessment of the size and content of the hernia sac with great precision. Some diagnosticians use the ultrasound probe to exert pressure in an attempt to place the hernia back into the abdominal cavity. Moreover, the above-mentioned method effectively visualizes any complications related to surgical reconstruction of the abdominal wall, such as pathological fluid-filled spaces, recurrent hernias, or tissue reactions to surgical material. All other pathologies of the abdominal wall that might imitate hernias are within the scope of ultrasound examination. All of the mentioned advantages of ultrasound lose significance if the doctor performing this examination lacks proper theoretical and practical background.

Keywords: abdominal wall, anterior abdominal wall hernias, incisional hernias, Spigelian hernias, ultrasound
Fresh approach to Henoch–Schönlein purpura. Comparison of its course and complications in children and adults in the light of the latest reports
Pediatr Med rodz Vol 10 Numer 4, p. 405–410
DOI: 10.15557/PiMR.2014.0041

Henoch–Schönlein purpura is a disease that belongs to the group of vasculitides. The disease in children is usually mild, but if it occurs in adulthood it can bring many adverse consequences. In mild forms, the major manifestation is skin lesions. The disease may also affect the joints, kidneys, gastrointestinal tract and lungs. The aim of the study is to present the differences in the course and prognosis of Henoch–Schönlein purpura in children and adults, the diversity of aetiological factors of IgA-associated vasculitis as well as clinical implications and diseases concomitant with purpura. Typically, it is believed that viral and bacterial agents induce Henoch–Schönlein purpura. Vaccination is one of the stimuli which can cause IgA-associated vasculitides. The coexistence of Henoch–Schönlein purpura with neoplasms is another interesting issue. The appearance of purpura may indicate a metastasis or recurrence. In adults, the course of Henoch–Schönlein purpura is much more severe – up to 75% of adults who have developed it have gastrointestinal complications. Renal complications occur in up to 87% of adult patients. Pain in the hip joints in the course of Henoch–Schönlein purpura may be a manifestation of necrosis of the femoral head. There are also described cases of complete myocardial infarcts or only its clinical signs. Epididymitis and scrotal oedema are complications of boys and men who have developed purpura. The occurrence of Henoch–Schönlein purpura in males older than 50 is associated with a worse prognosis. Patients with recurrent vasculitis should be carefully evaluated for the presence of a neoplasm.

Keywords: Henoch–Schönlein purpura, complications, paraneoplastic syndrome
Inhalation therapy in children
Pediatr Med rodz Vol 10 Numer 4, p. 411–425
DOI: 10.15557/PiMR.2014.0042

Inhalation is a form of treatment used in respiratory diseases that allows medications to be administered directly to
the affected site. Thanks to this, the administration of lower doses helps obtain a higher concentration in the target organ and
eliminate or effectively reduce systemic adverse effects. For drug deposition in the respiratory system, we use inhalers
(pressurised, dry powder, soft mist) or nebulisers (jet, ultrasonic, vibrating mesh). In pressurised inhalers, spacers or holding
chambers can be used to effectively synchronise inspiration with dose release. Inhalers are indicated in lower respiratory tract
diseases, and the only contraindication is the inability to use the device. Nebulisers can be used in upper and lower respiratory
tract diseases, but there are certain contraindications (among others: acute inflammation, flares of chronic diseases, circulatory
insufficiency). The selection of an inhaler (or a shift to another device) must be justified. It should be based on the knowledge
of the properties and principles of individual inhalers, assessment of the child’s ability to use the device properly (including
obtaining optimal inspiratory flows) as well as on the preferences of patients and their guardians. It is significant to
individually instruct the patient how to use an inhaler when this treatment is being implemented and to review the inhalation
technique during each follow-up visit. Treatment benefits can be achieved when age, clinical condition, proper inhalation
technique correlated with the abilities of patients are considered.

Keywords: inhalation therapy, inhaler, nebulisation, respiratory diseases, children
The use of natural substances in the treatment of rhinosinusitis in the light of the European Position Paper on Rhinosinusitis and Nasal Polyps 2012
Pediatr Med rodz Vol 10 Numer 4, p. 427–439
DOI: 10.15557/PiMR.2014.0043

Rhinosinusitis is a very common condition of the upper respiratory tract. The disease may be caused by viral, bacterial or fungal infections as well as by allergens or air pollution (e.g. tobacco smoke), with viral infections being the most common cause. The first phase of viral rhinosinusitis therapy involves the use of antipyretics, analgesics, anti-oedematous agents as well as nasal decongestants, occasionally, topical glucocorticosteroids, nasal irrigation and adjunct phytotherapy. Viral or allergic rhinosinusitis does not require antibiotic therapy. Antibiotics should be used if symptoms persist after 7–10 days or bacterial superinfection develops. More intense treatment (antibiotic, metronidazole, intranasal glucocorticosteroid as well as formulations that dilute mucous secretions and have anti-oedematous effects) for up to several days is necessary in cases of acute rhinosinusitis in children, which is quite often a very serious disease bordering on sepsis (pansinusitis). According to the 2012 European Position Paper on Rhinosinusitis and Nasal Polyps 2012 (EPOS 2012), natural compounds may be used as an adjunct treatment in some cases and in certain periods of the disease. These include nasal irrigation with saline or an appropriate seawater solution as well as the use of herbal medicinal products. The paper discusses in detail the use of different herbal medicinal products and presents literature data related to the efficacy and tolerance of these products.

Keywords: rhinosinusitis, diagnostic management guidelines, treatment, phytotherapy (as adjunct treatment)
Neurological complications of Lyme disease – clinical observations
Pediat r Med rodz Vol 10 Numer 4, p. 441–450
DOI: 10.15557/PiMR.2014.0044

Lyme disease is a chronic, multiorgan disease caused by the spirochete Borrelia burgdorferi, which is transmitted by Ixodes ticks. Poland has medium to high rate of tick infection. Lyme disease incidence has been increasing in recent years, with the peak incidence recorded in the summer, especially in endemic areas. The risk of infection depends on the type of spirochete and the time it is present in the human skin. It is crucial to remove the parasite as soon as possible, not later than 24 hours after the spirochete enters the body. The infection usually occurs in three stages, although not all of them have to be present. A characteristic erythema migrans or, less common, lymphocytic lymphoma, may be observed in the first stage of the disease. General symptoms, such as myocarditis, arthritis or nervous system involvement, are developed in the second stage. In the late stage of the disease, serious irreversible complications of the nervous system, musculoskeletal system or the skin occur. The diagnosis of Lyme disease is based on a history of tick bite as well as on the presence of clinical symptoms confirmed by serological findings. The presence of erythema migrans is sufficient for diagnosis and treatment initiation, therefore laboratory diagnostics is not necessary in this case. Serological diagnostics is primarily based on ELISA testing, while the second step uses a Western blot test. Positive serology test in the absence of clinical symptoms or a positive medical history is insufficient for diagnosis and treatment initiation. The type of the antibiotic used as well as the route and duration of its administration depend on the stage of the disease and on the affected organ. The most common antimicrobials used in the treatment of Lyme disease include amoxicillin, doxycycline (over the age of 12 years) and ceftriaxone.

Keywords: Lyme disease, erythema migrans, neuroborreliosis, diagnosis, treatment