2013, Vol 9, No 4
Thrombotic thrombocytopenic purpura – diagnosis and treatment
Pediatr Med rodz Vol 9 Numer 4, p. 323–330
ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) was once diagnosed with five manifestation: thrombocytopenia, microangiopathic haemolytic anaemia, nervous system malfunctioning, renal failure and fever. Yet 35% of patients in whom TTP is diagnosed do not develop all manifestations, i.e. neurologic manifestations, renal dysfunction and fever. At present the basis for TTP diagnosis is thrombocytopenia and microangiopathic haemolytic anaemia (MAHA). The diagnosis includes blood count, blood film, renal function, LDH level. A helpful parameter in diagnosing TTP is ADAMTS13 metalloproteinase and anti-ADAMTS13 antibodies. The treatment starts with therapeutic plasmapheresis. Plasmapheresis should be undertaken even when the diagnosis is not confirmed. It removes ULvWF multimers and acquired anti-ADAMTS13. Plasma transfusion is not as effective as plasmapheresis but it can be used provisionally. The effectiveness of the treatment is assessed by lack of thrombocytopenia normal LDH level, lack of anaemia and neurological disorders. Another therapeutic option is to use glicocorticosteroids and immunoglobulins. In some patients a beneficial procedure may be splenectomy. TTP therapy has recently employed
rituximab. It reduces ADAMTS13 inhibitor ratio and increases proteinase activity. Rituximab treatment is reported to have resulted in clinical remissions in the cases where other treatment modalities failed.

Keywords: thrombotic thrombocytopenia purpura, plasmapheresis, ADAMTS13, haemolytic uremic syndrome
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Chronic kidney disease and the aging population
Pediatr Med rodz Vol 9 Numer 4, p. 331–337
ABSTRACT

The proportion of older people in the general population is steadily increasing worldwide, with the most rapid growth in low- and middle-income countries(1). This demographic change is to be celebrated, because it is the consequence of socioeconomic development and better life expectancy. However, population aging also has important implications for society – in diverse areas including health systems, labor markets, public policy, social programs, and family dynamics( 2). A successful response to the aging population will require capitalizing on the opportunities that this transition offers, as well as effectively addressing its challenges. Chronic kidney disease (CKD) is an important public health problem that is characterized by poor health outcomes and very high health care costs. CKD is a major risk multiplier in patients with diabetes, hypertension, heart disease and stroke – all of which are key causes of death and disability in older people(3). Since the prevalence of CKD is higher in older people, the health impact of population aging will depend in part on how the kidney community responds. March 13, 2014 will mark the celebration of the 9th World Kidney Day (WKD), an annual event jointly sponsored by the International Society of Nephrology and the International Federation of Kidney Foundations. Since its inception in 2006, WKD has become the most successful effort to raise awareness among policymakers and the general public about the importance of kidney disease. The topic for WKD 2014 is “CKD in older people”. This article reviews the key links between kidney function, age, health and illness – and discusses the implications of the aging population for the care of people with CKD.

Keywords:
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Relationships between fat and bone during growth
Pediatr Med rodz Vol 9 Numer 4, p. 338–349
ABSTRACT

An increasing worldwide obesity epidemics and a number of obesity-related morbidities has brought on the largescale research into associations between adipose tissue and the skeleton and bone metabolism. The results of relevant published studies, conducted in adults and children, are inconsistent: some reports suggest protective role of body fat on skeletal health, whereas others emphasize a detrimental effect of adiposity on bone mineral density (BMD) and bone turnover. The discrepancies may, at least partly, result from differing methodological approaches across studies. During the last decade, a growing body of evidence has emerged, supporting the view of a negative role of obesity in bone mass accrual during growth. Childhood obesity may lead to fragility fractures and may, therefore, predict early development of osteoporosis in adulthood. The adipokines and hormones secreted by adipocytes are substantially responsible for fat-bone interactions. The effects of adipokines, such as leptin, adiponectin, resistin or visfatin, on BMD during growth, bone modelling and remodelling processes appear multidirectional. Although low BMI has become a commonly accepted risk factor of postmenopausal osteoporosis, and high BMI has been perceived to prevent bone loss, several well-designed paediatric studies have clearly shown a paradoxical deleterious impact of obesity and excessive weight gain on bone tissue. Finally, there is significant evidence that chronic low-grade inflammation induced by obesity is the key mechanism of this negative effect of adiposity on the growing skeleton. The aim of this review is to demonstrate updated knowledge concerning complexity of fat-andbone interactions, and to highlight important pathogenic and clinical implications of childhood obesity on bone structure, BMD, bone strength and metabolism.

Keywords: adipose tissue, skeleton, childhood obesity, bone mass, fractures
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Dietary prevention of osteoporosis – recommendations and controversies
Pediatr Med rodz Vol 9 Numer 4, p. 350–356
ABSTRACT

Osteoporosis is a systemic metabolic disease characterized by loss of bone mass and its impaired microarchitecture, resulting in an increased risk of fragility fracture. Epidemiological data indicate an increase in the incidence of osteoporotic fractures worldwide. The high cost of health, social and economic treatment of osteoporosis requires seeking effective methods of prevention. Considering the multifactorial aetiology of osteoporosis, to the well documented risk factors belong: age, gender, ethnogenetic factors, current or history of chronic disease, steroid therapy and lifestyle. The modifying lifestyle factors as nutrition and physical activity are the potential tools for effective primary prevention of osteoporosis addressed to the whole of society, with particular emphasis on the paediatric population. The results of previous studies evaluating the effect of diet on the reduction of osteoporotic fractures are ambiguous, difficult to interpret and translate into specific dietary recommendations. Although there is a lot of evidence of beneficial effects on skeletal metabolism due to intake of various nutrients (macro- and micronutrients, vitamins D, K and C, isoflavones, plant, polyunsaturated fatty acids omega-3), but both the intake assessment of these nutrients and the monitoring their impact on bone remains extremely difficult in practice. Beneficial role of calcium and vitamin D in mineral homeostasis and bone metabolism has been thoroughly proven, however, further studies are needed, particularly prospective and randomized, in order to determine the optimal calcium intake, dose, bioavailability and nutrient sources, and indications for preventive supplementation. Due to the wide‑spread deficit and insufficient dietary intake of vitamin D, in the light of current guidelines for osteoporosis prevention, the entire population should receive supplementation of vitamin D in the doses strictly dependent on age, sex and season.

Keywords: diet, prophylaxis, osteoporosis, recommendations, calcium, supplementation of vitamin D, controversies
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Assessment of physical development and nutrition status in children beginning education in sports primary school
Pediatr Med rodz Vol 9 Numer 4, p. 357–362
ABSTRACT

Introduction: Sports schools play crucial role in promotion of physical activity by organized sports participation. Aim of the study: The aim of this study was to evaluate physical development and nutrition status in children beginning education in sports primary school (athletic profile). Material and methods: The research was carried out in the group of 41 children beginning education in sports elementary school and in group of 46 children beginning education in common primary school. The anthropometric measurements (height, weight) with body mass index (BMI) and skinfolds measurements were performed in the examined groups. Results: No significant differences between groups occurred for basic anthropometric measurements (height, weight, BMI). Extreme values some of these parameters were more common in children from sports school, e.g. tall stature (height above the 95th centile) and obesity (BMI above the 95th centile). Underweight (weight below the 5th centile), short stature (height below the 5th centile) and malnutrition (BMI below the 5th centile) were observed only in children from sports school. Thickness of skinfolds (subscapular, suprailiac, crural) were statistically lower in children from sports school. Conclusions: Basic anthropometric measurements (height, weight, BMI) were not significantly different between groups, but extreme values of these parameters were more common in children from sports school. Thickness of subscapular, suprailiac and crural skinfolds were statistically lower in children from sports school.

Keywords: physical development, nutrition status, physical activity, children, sports school
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Obstruction as a complication of treatment with intragastric balloon for obesity
Pediatr Med rodz Vol 9 Numer 4, p. 363–366
ABSTRACT

Background: Obesity is a disease of civilization, which may result in and aggravate already existing diseases. One method of weight loss is a gastric balloon – BioEnterics Intragastric Balloon (BIB). Treatment with the BIB is safe. Complications are rare. Aim: Consult doctors about the possibility of this rare and serious complication that can be treated conservatively. Description of case: Patient, 38 years old, hospitalized due to continuing strong 3 days intra-abdominal pain, nausea and vomiting. In an interview founded for over 9 months ago slimming balloon into the stomach. Imaging studies, such as abdominal X-ray and computed tomography (CT) of the abdomen and pelvis, in the left lower abdomen visualized an irregular change in the corresponding slimming balloon. In the absence of indications for emergency laparotomy, conservative treatment was used. Finally, there was a spontaneous expulsion of the balloon. An improvement of the general condition, relief of symptoms. Discuss the case: Treatment of obesity by setting the gastric balloon is a minimally invasive method of treatment. However, the effectiveness and the lack of complications are dependent on the patient’s knowledge about the possible complications of obesity and the chosen therapeutic method, a sense of the disease and the patient’s motivation to achieve the therapeutic success. Symptoms of spontaneous expulsion of the balloon are symptoms of a foreign factor in the gastrointestinal tract. In the case described, these symptoms have evolved along with the movement of the balloon for the next segments of the gastrointestinal tract. There were signs of high obstruction and intestinal colic. The exact position of the balloon in the gastrointestinal tract was possible to determine after taking CT scan of the abdomen. In the case described, a conservative method was used. This was possible thanks to the close cooperation between doctors of conservative and surgical specializations, and a careful observation of the dynamics of disease symptoms and movement of the balloon for the gastrointestinal tract. Conclusions: Because of the potential complications of intervention methods for weight reduction should be preceded by conservative methods. Selection of invasive methods of treatment should be preceded by a detailed analysis of the causes of failure of conservative treatment. Maintenance of balloon slimming timeout therapy may result in dangerous to life and health complications. If symptoms of obstruction or intestinal colic, patient with founded into the stomach slimming balloon should be observed in a hospital. In the absence of clinical signs of gastrointestinal perforation, esophagoduodenoscopy should be performed to locate the balloon in the stomach in the first instance, further radiological imaging studies locating the position of the balloon in the gut and documenting progress in its elimination. Surgical treatment should be considered if there is no progress spontaneous expulsion of the balloon.

Keywords: obesity, endoscopy, balloon slimming, complications, obstruction
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Frontometaphyseal dysplasia (Gorlin-Cohen syndrome) as an example of rare X-link skeletal disorder – clinical description of two cases with late diagnosis
Pediatr Med rodz Vol 9 Numer 4, p. 367–373
ABSTRACT

Frontometaphyseal dysplasia, known as Gorlin-Cohen syndrome, is a rare entity inherited as X-link dominant trait caused by FLNA gene (Xq28) mutations. FLNA mutations are responsible for several known clinical entities with heterogeneous symptoms (pleiotropism). Gorlin-Cohen syndrome is characterized by: skeletal defects of cranium and metaphysis of long bones, osteoporosis, thorax and sternum deformations, platyspondyly, limited movement and flexion deformity of elbows and wrists, scoliosis, typical craniofacial, hands and feet dysmorphism as well as intellectual disability in part of cases, deafness, ophthalmological problems, malocclusion of teeth and following oligodontia. An obstructive uropathy and cardiological disturbances are reported to occur in adults with frontometaphyseal dysplasia. We report cases of very late diagnosed two patients with frontometaphyseal dysplasia. Performed molecular tests confirm the clinical diagnosis. Additionally, we present a clinical follow-up of frontometaphyseal dysplasia and a review of literature. There was diagnosed a pathogenic point mutation in exon 45 of gene FLNA – 7267C>T – causing a change of amino acids: proline to serine in position 2423 of amino acid chain in patient 1 as well as a point mutation in position c.745G->T, causing a change of amino acid sequence of FLNA protein (p.Asp249Tyr) in patient 2. In both  cases a carriership of mutation in patient’s mother were found (heterozygous status). There were no clinical symptoms typical for frontometaphyseal dysplasia in both mothers of presented patients.

Keywords: frontometaphyseal dysplasia, Gorlin-Cohen syndrome, skeletal defects, craniofacial dysmorphism
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Variety of clinical manifestations of Mycoplasma pneumoniae infection. Case report
Pediatr Med rodz Vol 9 Numer 4, p. 374–378
ABSTRACT

Mycoplasma pneumoniae infection is a complex clinical problem. Atypical pneumonia with mild course, moderately elevated body temperature and presence of small auscultatory changes of the lung fields are its most common manifestation. Moreover, the severity of the symptoms is related to the age of patient. In the group of older children, disease may occur as interstitial or lobar pneumonia, less often as a pleurisy. Additionally fever can show higher values. Dry cough that lasts for 4 to 6 weeks is common and the most characteristic symptom of pneumonia caused by M. pneumoniae. Beyond the respiratory system, M. pneumoniae infection can manifest symptoms on almost every organ of the human body. For this reason neurological, cardiovascular, digestive, osteoarticular and skin complications are observed. The bacteria’s feature which results multitude of clinical manifestation is that despite of direct damaging of epithetical cell, has ability to stimulate autoimmune and prothrombotic processes. Diagnosis of infection is mainly based on serological methods. Treatment options include macrolides and therapy lasts at least 2 weeks. This article presents two cases of M. pneumoniae infection which are greatly different in their clinical course. In the first case the main manifestation of infection was pneumonia, which proceeded with a high fever, pleurisy, and the presence of many auscultatory changes of the lung fields. Additionally in this case were observed the symptoms of the gastrointestinal system in the form of vomiting and intense abdominal pain. On the other hand, the second case showed development of massive of deep vein thrombosis next to fresh M. pneumoniae infection and other risk factors.

Keywords: Mycoplasma pneumoniae, pneumonia, pleurisy, thrombosis, vascular anomaly
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A newborn with atrial flutter
Pediatr Med rodz Vol 9 Numer 4, p. 379–385
ABSTRACT

Atrial flutter is a supraventricular tachyarrhythmia seldom, in the paediatric population occurs mainly in children with congenital heart disease. It may also exist in children with normal heart, especially in newborns. It can be diagnosed in the prenatal period. Symptoms are dependent on the heart rate, the rapid progressive heart failure is possible. The paper contains main data of epidemiology, diagnosis and treatment of atrial flutter in foetus and newborns. In restoring the sinus rhythm an electrical cardioversion is recommended. In the prevention of recurrence of arrhythmia antiarrhythmic drugs should be use, multidrug therapy may be required. This paper presents a newborn with normal heart and recurrent atrial flutter. During the arrhythmia symptoms of the heart failure appeared, due to slow atrioventricular conduction and decreasing heart rate. In the neonatal period four recurrences of the arrhythmia were observed, the child required three electrical cardioversions and she needed three antiarrhythmic drugs.

Keywords: neonatal arrhythmia, atrial flutter, supraventricular arrhythmia, foetal arrhythmia, cardioversion
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Practical spirometry – how to use it in diagnosis and treatment of respiratory diseases?
Pediatr Med rodz Vol 9 Numer 4, p. 386–396
ABSTRACT

Spirometry is one of the basic additional examinations which should be performed by the family doctor and other physicians of primary care. It is used to assess ventilation of the respiratory system. It measures the amount of air in the lungs and the velocity of its flow through the airways during inspiration and expiration. Reciprocal relationship between flow and volume determine whether lung function is normal or whether there are certain anomalies. Spirometry is the essential tool in diagnosis, monitoring and treatment assessment as well as establishing prognosis of numerous respiratory disorders including common lung diseases i.e. chronic obstructive pulmonary disease and bronchial asthma. Absolute contraindications to spirometry include: aneurysms of the aorta and cerebral arteries, history of retinal detachment or recent ophthalmological surgery, haemoptysis with unknown aetiology, pneumothorax (one month before) and recent myocardial infarction or recent stroke. There are no age limitations. However, the condition necessary for performing spirometry is the ability to coordinate inspiration and expiration and thus, cooperation of the patient with the health care professional who conducts the examination. A spirometer is a measuring device used to examine lung capacity and volume. It registers the amount, flow and pressure of inhaled and exhaled air in a given time. The measured air flow velocity is changed into volume. The programme, which is built in the spirometer, calculates spirometric variables and compares them with standard values. Medical practitioners use so-called basic spirometry and spirometry performed after administration of a bronchodilator, a post BD test (referred to as “a spirometry to assess the reversibility of obturation”). A correctly performed test should include at least 3 acceptable FVC measurements. Apart from this, spirometry also includes a forced expiratory manoeuvre. It consists of three subsequent phases: maximal fast inspiration, abrupt beginning of expiration and subsequent smooth and long expiration. The basic types of ventilation disorders in respiratory diseases are: obstructive and restrictive ones.

Keywords: spirometry, spirometer, lung capacity and volume, ventilation disorder types
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Nutrition education for families in the prevalence of obesity
Pediatr Med rodz Vol 9 Numer 4, p. 399–403
ABSTRACT

Nutritional education is an important component of the treatment of overweight and obesity, being often supplement for weight loss diet or medication. Properly selected and implemented the method of education should serve as a mobilizing and amplifying factor, so that you will be willing to modify your lifestyle. The main objective is to provide nutrition education to and preserve the right attitudes habits. Considering the problem of nutrition education for families obese, remember that form of communication of knowledge about nutrition, aimed at children and their parents/guardians cannot be the same. This is due to differences in the understanding and assimilation of information between adults and children. Even so, keep in mind that the family is a community whose members are linked to each other and are mutually influence each other. Therefore, you can not ignore an education that will encourage joint activities the whole family. Changing eating habits must be for the whole family, as the parents through their views, preferences and behaviours shape the beliefs, preferences, and behaviour of children. Even if one person in the family is slim, should adapt to the new rules for the sake of their loved ones. Their mutual motivation may be the key to success. Changing lifestyle is also associated with an increase in physical activity, which is an essential component of the treatment of obese patients. The increase in physical activity can effectively shorten the duration of treatment and contribute to better his effects.

Keywords: nutritional education, methods of education, educational program, obesity, family, nutrition
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Use of the CANDIVAC cps. yeast plant lyophilisate in females with recurrent vulvovaginal candidiasis: clinical study results
Pediatr Med rodz Vol 9 Numer 4, p. 404–407
ABSTRACT

The problem of the relapsing vulvovaginal candidiasis is not due to the infection with a new yeast plant, it lies in susceptibility to being infected by one’s own yeast plant. Help can be provided through immunomodulation by CANDIVAC cps. yeast lysates, which in a dose of 10 tablets per month for a period of six months brought a statistically significant reduction in the relapse occurrence as well as intensity of patients’ subjective complaints. There is no direct relation between the amount of yeast in the vagina, the quality of lactobacillary grading and the degree of seriousness of clinical symptoms.

Keywords: recurrent vulvovaginal candidiasis, immunomodulation therapy, lyofilisates, relapse of vulvovaginal candidiasis
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