2019, Vol 15, No 1
Croup – the most important questions and answers
Pediatr Med Rodz 2019, 15 (1), p. 6–11
DOI: 10.15557/PiMR.2019.0001

Until the end of the first half of the 20th century croup was associated mainly with diphtheria caused by Corynebacterium diphtheriae. However, now the term “croup” covers a whole group of disorders of the larynx, trachea and bronchi. The term refers to a number of disorders included in ICD-10 and may be used as a synonym for acute laryngitis, laryngotracheitis and laryngotracheobronchitis. The group of diseases included under the term “croup” is particularly dangerous for children: due to specific anatomical and physiological features stridor may only occur when a child is crying or moving. The most common aetiological factors for acute laryngotracheitis are viruses (e.g. parainfluenza). Croup may also be caused by bacteria. Typical symptoms include stridor, cough (barking, seal-like), hoarseness and activation of accessory respiratory muscles. As the disease progresses, upper airway obstruction and laryngeal dyspnoea increase and the patient develops tachypnoea with a prolonged inspiratory phase. The child’s anxiety increases. The course of the disease and the ultimate level of airway obstruction may vary. The diagnosis of croup symptoms is based on physical examination and interview. Initially, the severity of symptoms should be assessed using the Westley score. Pharmacotherapy includes glucocorticoids (nebulised, oral, parenteral) and adrenaline (nebulised). In Poland, the first line therapy for croup is nebulised glucocorticoids. The oral and inhaled route of administration of glucocorticoids are equally recommended. In severe forms of croup oxygen may be administered.

Keywords: croup, acute laryngotracheitis, glucocorticoids, respiratory obstruction, children
New trends in the search for alternative antifungal therapies
Pediatr Med Rodz 2019, 15 (1), p. 12–16
DOI: 10.15557/PiMR.2019.0002

The paper addresses the issue of fungal infections in the context of growing resistance to currently available antifungal agents and the development of new antimycotics. Fungal pathogens belonging to the genuses Candida, Aspergillus, Pneumocystis and Cryptococcus account for about 90% of all fungal infections. Candida albicans infections are a global clinical problem, and systemic candidiasis is considered one of the most severe fungal infections, with mortality rates of about 40% despite treatment. Currently, there are five classes of antimycotics available, of which only three (azoles, echinocandins and polyenes) are used for systemic infections. The limited variety of available therapies as well as their overuse in both therapy and prevention have contributed to the growing resistance among fungal pathogens. Many mechanisms of resistance to antimycotics have been identified. These include in particular: mutations in genes encoding target proteins, increase or decrease in target protein, protein pump activity, biofilm formation or activation of stress response. The growing incidence of fungal infections and the difficulty of their treatment have forced the search for alternative therapeutic agents with new mechanisms of action. Due to the eukaryotic nature of fungal cells, recent trends in literature imply that novel agents should specifically target virulence factors or stress response of the pathogen.

Keywords: Candida albicans, antifungal agents, virulence
Sleep bruxism risk factors in children: a literature review
Pediatr Med Rodz 2019, 15 (1), p. 17–21
DOI: 10.15557/PiMR.2019.0003

Introduction: Bruxism is defined as a repetitive jaw and muscle activity involving teeth clenching and/or grinding which may occur during everyday activities or during sleep. The aetiology of bruxism includes three groups of causes: psychosocial, personal and pathophysiological factors. Currently, there is no effective method of treatment to completely eliminate the problem of bruxism. Teeth protection and pain reduction methods are used. Bruxism-associated pain is found in approximately 47.6% of children aged 3–6 years. The aim of the present study was to establish sleep bruxism risk factors in children based on a literature review. Material and methods: The material for review included articles from the PubMed, ResearchGate and Google Scholar databases. In order to identify suitable publications the search was conducted using combinations of the following keywords: “bruxism,” “child,” “teeth grinding” (according to Medical Subject Headings, MeSH). This review includes 12 publication regarding sleep bruxism risk factors in children. Results: The association between bruxism and respiratory disorders and that between bruxism and sleep problems were described by three studies each. Stress as a predisposing factor was noted in four articles and parental divorce in two papers. Conclusions: 1) Respiratory disorders during sleep, sleep disorders and stressful situations, particularly parents’ divorce, contribute to sleep bruxism in children. 2) The impact of the remaining factors discussed in this paper on the development of sleep bruxism requires further research.

Keywords: risk factors, bruxism, children, literature review
Epidemiology and types of urolithiasis
Pediatr Med Rodz 2019, 15 (1), p. 22–25
DOI: 10.15557/PiMR.2019.0004

Urolithiasis is a lifestyle disease. Sex, age, race, place of residence (geographical region), chronic diseases, lifestyle (including diet) and various genetic factors contribute to the risk of calculus formation within the urinary tract. The prevalence of urolithiasis in adults ranges from 1 to 20%, and children account for 2–10% of patients. The annual incidence of paediatric urolithiasis is estimated at several cases per 100,000 children worldwide; it is the reason for approximately 1 per 1,000 hospitalisations among paediatric patients. The prevalence of urolithiasis has been increasing in both adults and children. In young children, the disease is usually a result of a genetically determined metabolic defect (such as hypercalciuria, distal tubular acidosis, familial hypomagnesaemia with hypercalciuria and nephrocalcinosis, or Lesch–Nyhan syndrome). Another significant risk factor in children is a urinary tract defect with urinary retention. Urinary tract infections, in turn, may be either a cause or a complication of urolithiasis. In older children and adults, malnutrition, obesity and metabolic syndrome play a significant role. Other factors conductive to urolithiasis are: certain drugs, low level of physical activity, long immobilisation, low fluid intake, warm climate, inadequate diet and improper vitamin D3 supplementation. The disease recurs within 5 years in 50% of patients. Urolithiasis and its complications may lead to end-stage renal failure. The disease can be divided into different types based on the chemical composition of a calculus, aetiology of the disease and site of calculus formation (upper or lower urinary tract). The composition of calculi is quite often mixed, and they form due to various causes.

Keywords: urolithiasis, hypercalciuria, children, epidemiology
Hypocitraturia: its importance as a factor in the development of urolithiasis
Pediatr Med Rodz 2019, 15 (1), p. 26–32
DOI: 10.15557/PiMR.2019.0005

The citrate anion is an endogenous inhibitor of calcium deposits in the urinary tract. Moreover, by urine alkalisation, citrates enhance uric acid and cystine solubility. Hypocitraturia in adults is defined as excretion of citrates with urine below 320 mg/24 h. The most common definitions in children involve citrate excretion in 24 hour urine collection at a level <365 mg/1.73 m2/24 h in boys and <310 mg/1.73 m2/24 h in girls. Moreover, the urine citrate/creatinine ratio is also considered: for children aged 0–5 years <0.42 mg/mg and for children older than 5 years of age <0.25 mg/mg. Hypocitraturia is a common metabolic disturbance; such a diagnosis is made in approximately 20–60% of adults and 10–68% of children with urolithiasis. Various factors may affect citrate excretion with urine, the most significant of which is the pH value (of blood, urine or intracellular fluid). Most patients are diagnosed with so-called idiopathic hypocitraturia, although it also accompanies acidosis, including distal tubular acidosis, hypokalaemia, high-protein diet and treatment with certain medications (carbonic anhydrase inhibitors, angiotensin-converting enzyme inhibitors, thiazide diuretics). Patients with hypocitraturia should first and of foremost have dietary modifications introduced. This involves an increase in the dietary amount of fluids and citrus fruit as well as salt and protein restriction. Patients with recurring urolithiasis may also be recommended citrate products, the most common of which is potassium citrate. Citrus juices may be an alternative, but their effect is not as potent and unambiguous as in the case of commercially available citrate products.

Keywords: hypocitraturia, citrates, urolithiasis, children
Infectious urolithiasis
Pediatr Med Rodz 2019, 15 (1), p. 33–37
DOI: 10.15557/PiMR.2019.0006

Infectious urolithiasis is a form of urolithiasis associated with the presence of bacteria in the urinary tract (Proteus spp., Klebsiella spp., Providencia spp., Pseudomonas, Morganella morganii and Staphylococcus aureus). These bacteria produce urease, i.e. an enzyme which decomposes urea. Deposits made of magnesium ammonium phosphate (struvite), sometimes combined with carbonate apatite, can quickly expand, fill the entire renal pelvis and lead to renal failure. In children, struvite urolithiasis has a variable frequency – in 1–29% of all patients with urinary tract deposits. The predisposing factors for the development of this urolithiasis in children are urinary tract defects and neurogenic bladder. Its symptoms are a combination of the symptoms of urolithiasis and urinary tract infection (fever, dysuria, lumbar pain, urinary urgency). In imaging studies, a large branched deposit is often visible, sometimes filling the entire renal pelvis and calyces. Treatment consists of surgical removal of deposits (most often by percutaneous nephrolithotomy) in combination with targeted antibiotic therapy. In Poland, combined treatment (percutaneous nephrolithotomy + ureterorenoscopy) proved to be a very effective and safe method, allowing the removal of large urinary deposits from the urinary tract in children. Antimicrobial treatment without surgical treatment is rarely effective. Other options for non-surgical management include reducing dietary phosphates, acidifying urine, administration of urease inhibitors (in adults) or citrates, and rinsing the renal pelvis with a solution of citrates or hemiacidrin. The prognosis in this group of patients depends mainly on the completeness of the surgical procedure and the lack of relapses.

Keywords: infectious urolithiasis, struvite urolithiasis, urinary tract infection, children
Rare forms of nephrolithiasis
Pediatr Med Rodz 2019, 15 (1), p. 38–41
DOI: 10.15557/PiMR.2019.0007

Inborn errors of metabolism that may lead to calculus formation within the urinary tract include: adenine phosphoribosyltransferase deficiency, xanthine oxidase deficiency, orotic aciduria (acidosis) type I and alkaptonuria. All of them are autosomal recessive diseases. Adenine phosphoribosyltransferase deficiency and xanthine oxidase deficiency (congenital xanthinuria) are defects of purine metabolism, which lead to the formation of calculi composed of 2,8-dihydroxyadenine and xanthine. Xanthinuria is accompanied by hypouricaemia and hypouricosuria. Treatment involves a low-purine diet, abundant fluid administration and allopurinol (in adenine phosphoribosyltransferase deficiency) and alkalisation of urine (in xanthinuria). Orotic aciduria type I is a pyrimidine metabolism defect that manifests with failure to thrive, developmental delay, megaloblastic anaemia, immunodeficiency, skin appendage disorders and excessive excretion of orotic acid with urine. Nephrolithiasis is a rare manifestation of this disease. Treatment involves uridine and haematopoietic drugs. Alkaptonuria is a defect of tyrosine metabolism whereby homogentisate is deposited in tissues. It manifests with connective tissue pigmentation (ochronosis), osteoarthritis, calcifications in the coronary arteries, heart valve damage, pigmentation of the sclera and urolithiasis. Treatment involves nitisinone, vitamin C and dietary restriction of tyrosine and phenylalanine. Melamine urolithiasis is a consequence of crystallisation of melamine (cyanuramide), which was illegally added to infant formulas in China. Melamine stones were spontaneously evacuated from the urinary tract, but some patients developed irreversible renal changes. Moreover, certain drugs may also crystallise in the kidneys. These include aciclovir, indinavir, atanavir, sulphadiazine, triamterene, methotrexate, orlistat, ciprofloxacin and ceftriaxone. Prevention and treatment of this form of nephrolithiasis mostly consist in the administration of a large quantity of fluids.

Keywords: inborn errors of metabolism, melamine, drug-induced urolithiasis, children
Minimally invasive techniques used for treatment of urolithiasis in children
Pediatr Med Rodz 2019, 15 (1), p. 42–46
DOI: 10.15557/PiMR.2019.0008

The paediatric population is a special group among patients with urolithiasis. Within the past 25 years, the prevalence of this disease in the paediatric population has increased from 6% to 10%. The causes of this higher prevalence are not entirely clear but may be associated with various factors, such as: inappropriate eating habits, inappropriate diet, particularly rich in salt and animal protein, insufficient fluid intake, obesity, hypertension, pollution, faster pace of living as well as uncontrolled intake of multivitamin preparations and dietary supplements. The mean age of a paediatric patient with urolithiasis is 7–8 years. The aim of this publication is to present minimally invasive techniques used for treatment of urolithiasis in the paediatric population. Due to a high risk of disease recurrence, the selection of a treatment method that enables removal of calculi in the least invasive and the most effective way is vital in this group of patients. The choice of management depends on various factors, such as: location, size and composition of calculi, patient’s age, anatomical conditions, the degree of urinary outflow obstruction and recurring urinary tract infections. Surgical methods of urolithiasis treatment are identical in adults and in children. Minimally invasive techniques include extracorporeal shock wave lithotripsy (ESWL), percutaneous nephrolithotomy (PCNL), ureterorenoscopic lithotripsy (URSL; ureterolithotripsy) and retrograde intrarenal surgery (RIRS). Owing to the technical advancement, minimally invasive techniques are effective in children and help evacuate calculi fully, even with a single procedure. It must be remembered, however, that they should be performed by experienced urologists in highly specialised and well-equipped centres.

Keywords: urolithiasis, children, ESWL, PCNL, URSL, RIRS
Cardiovascular response to exercise in hypertension – clinical characteristics of ASSECURE study participants
Pediatr Med Rodz 2019, 15 (1), p. 47–56
DOI: 10.15557/PiMR.2019.0009

Aim: Arterial hypertension leads to progressive cardiovascular dysfunction and heart failure. The aim of the study was to assess exercise capacity in hypertensives with the use of a cardiopulmonary exercise test, impedance cardiography and 6-minute walk test with special emphasis on haemodynamic response to exercise workload. Methods: 114 patients (53.5% women, 55.7 ± 9.1 years) were evaluated for reported symptoms, N-terminal pro-B-type natriuretic peptide (NTproBNP) levels, echocardiographic parameters and exercise capacity with 6-minute walk test, cardiopulmonary exercise test and haemodynamic parameters (by means of impedance cardiography). Results: 50% of patients had reported symptoms of impaired exercise tolerance, mostly dyspnoea on exertion (37.7%). NTproBNP levels exceeded 125 pg/mL in 19.3% of patients. Left ventricular diastolic dysfunction was found in 8.8% and heart failure was diagnosed in 6.2% of patients. A wide range of peak oxygen uptake (peak VO2) and 6-minute walk test distance was observed: 19.4 ± 5.2 mL/min/kg and 526.7 ± 112.0 m, respectively. A major proportion of patients demonstrated decreased exercise capacity parameters: 56.1% achieving peak VO2 < 22 mL/min/m2; 45.9% achieving peak VO2 < 80% of the predictive value; 37.3% achieving 6-minute walk test distance shorter than the predicted values. The impedance cardiography recorded at peak exercise: heart rate 147.2 ± 22.4 bpm, stroke volume 110.2 ± 21.8 mL, cardiac output 15.9 ± 4.2 L/min, peak systemic vascular resistance 587.4 ± 168.0 dyn.s/cm5. Conclusions: Although a very small proportion of patients with uncomplicated arterial hypertension meet the criteria for being diagnosed with heart failure, the symptoms of impaired exercise tolerance as well as abnormal results of objective exercise capacity assessments are quite common in these patients.

Keywords: arterial hypertension, heart failure, cardiopulmonary exercise test, exercise capacity, impedance cardiography
Atherosclerosis risk factors in prepubertal children in relation to their birth weight and gestational age
Pediatr Med Rodz 2019, 15 (1), p. 57–66
DOI: 10.15557/PiMR.2019.0010

According to Barker’s hypothesis, intrauterine malnutrition results in abnormal metabolism of a foetus and a tendency to develop atherosclerotic disease in the future life. Aim of the research: Assessment of atherosclerosis risk factors in prepubertal children, depending on their birth weight and duration of pregnancy. Material and methods: 113 children aged 7–9 were divided into groups: born prematurely, with low body weight (group I – 37 people), born on time, with low body weight (group II – 36 people) and born on time, with normal body weight (group K – 40 people) and groups of children born with body weight <10th or ≥10th percentile for gestational age, regardless of the length of pregnancy. Demographic and social data on health behaviours and health status of the children and their parents were collected. Anthropometric measurements and blood pressure was taken. Body composition was estimated with bioelectrical impedance method. Glucose, cholesterol and triglyceride levels in the blood were determined with the strip test method. Results: Compared to the other groups, in group K, significantly higher mean values and significantly higher occurrence of high centile values of body mass index and waist circumference as well as insignificantly higher rate of adipose tissue and abnormal glucose and cholesterol concentrations were found. In children born prematurely or on time, with birth weight <10th percentile, the centiles of waist circumference were significantly less frequent. A positive correlation was found in the body mass index of children born at the time with low body mass and body mass index of their mothers. Among the independent variables which influence the number of atherosclerosis risk factors, the importance of parental obesity was confirmed. Conclusions: 1) Premature birth or birth with low birth weight does not increase the risk of obesity or elevated cholesterol and glucose levels in the prepubertal age. 2) The occurrence of atherosclerosis risk factors in prepubertal children (regardless of duration of gestation and birth weight) is associated with obesity/overnutrition of parents.

Keywords: birth weight, duration of pregnancy, risk factors for atherosclerosis, prepubertal age
Significance of inflammatory markers in children’s infectious diseases
Pediatr Med Rodz 2019, 15 (1), p. 67–72
DOI: 10.15557/PiMR.2019.0011

Aim of the study: The aim of the study was to assess the clinical utility of inflammatory markers for differentiating the aetiology of diseases associated with fever in children. Material and methods: A retrospective analysis was performed on the medical records of 1,454 children (658 girls and 796 boys) hospitalised at a paediatric department in 2016–2017 for diseases associated with fever. The analysis was performed on the whole study group, which was divided into the following age groups: children in their 1st year of life (n = 422; 29.1%), children between their 2nd and 6th year of life (n = 870; 59.8%) and children from their 7th year onwards (n = 162; 11.1%). The following inflammatory markers were analysed: white blood cell count, C-reactive protein level, procalcitonin level, erythrocyte sedimentation rate and neutrophil-to-lymphocyte ratio. Results: C-reactive protein, erythrocyte sedimentation rate and neutrophil-to-lymphocyte ratio turned out to be useful in the identification of aetiology of upper and lower respiratory tract infections in all children (p < 0.05). Higher inflammatory marker values were observed in compound infections as opposed to single conditions (p < 0.05). C-reactive protein and erythrocyte sedimentation rate were proven to be useful in identifying the aetiology of acute gastroenteritis. A detailed analysis was performed on the group of children in their 1st year of life. It revealed that inflammatory markers were higher for urinary tract infections than for lower respiratory tract infections and gastroenteritis (p < 0.05). Conclusions: Inflammatory markers may be helpful for determining the aetiology of infectious diseases associated with fever in children. However, therapeutic decisions should always be taken based on the complete clinical picture.

Keywords: inflammatory markers, pneumonia, acute gastroenteritis, urinary tract infection, children
Iatrogenic diaphragmatic hernia in a child as a complication of ventricular assist device implantation. A case report
Pediatr Med Rodz 2019, 15 (1), p. 73–76
DOI: 10.15557/PiMR.2019.0012

The aim of the paper is to present a case of an anterior diaphragmatic hernia in a patient after multiple cardiosurgical interventions and heart transplantation. Case presentation: A child with dilated cardiomyopathy diagnosed in the neonatal period underwent heart transplantation at the age of 10 years. When she was 12 years old, she was referred to the Department of Paediatric Surgery and Organ Transplantation of the Children’s Memorial Health Institute for surgical treatment of diaphragmatic hernia detected on echocardiography. Prior to heart transplantation, the patient had required the use of biventricular assist device (EXCOR type) due to progressive cardiac insufficiency. The patient was deemed eligible for surgical treatment and underwent diaphragmatic hernia repair with a polytetrafluoroethylene (PTFE) patch.

Keywords: diaphragmatic hernia, heart transplantation, ventricular assist device
Huge posterior triangle lymphadenopathy as a rare sole presentation of papillary thyroid carcinoma
Pediatr Med Rodz 2019, 15 (1), p. 77–80
DOI: 10.15557/PiMR.2019.0013

Papillary thyroid carcinoma is well-known for its low malignant potential with good prognostic outcome. It usually presents as a palpable thyroid mass but scarcely manifests as an isolated cervical lymphadenopathy. A 50-year-old man presented with a huge posterior cervical lymphadenopathy which was suspicious for metastatic papillary thyroid carcinoma on fine needle aspiration cytology. The thyroid gland was clinically not visible or palpable. After computed tomography of the neck, the patient was preceded with total thyroidectomy and right selective lymph node dissection. Histopathological assessment confirmed metastases in the lymph node and papillary thyroid carcinoma in the resected gland. The paper revisits this unfortunate case of huge metastatic posterior triangle papillary thyroid carcinoma, illustrating the surgical outlines and discussing literature review.

Keywords: papillary thyroid carcinoma, lymphadenopathy, neck dissection
Laryngeal cleft type IV: a rare entity
Pediatr Med Rodz 2019, 15 (1), p. 81–84
DOI: 10.15557/PiMR.2019.0014

Laryngeal cleft is a rare disease. The manifestation of the disease and its management vary depending on the severity of the cleft. Laryngeal cleft should be considered in the differential diagnosis, especially in neonates presenting with stridor and recurrent aspiration. The outcomes in patients with laryngeal cleft have improved significantly over the years due to increased awareness of the disease, early detection, good intensive care and advancement in surgical techniques. We present a unique case of laryngeal cleft type IV. To the best of our knowledge, there is no reported case in the author’s country, Malaysia.

Keywords: laryngeal cleft, congenital malformation, children
Facial nerve palsy as a complication of an acute otitis media
Pediatr Med Rodz 2019, 15 (1), p. 85–87
DOI: 10.15557/PiMR.2019.0015

Acute otitis media is an infection of abrupt onset that occurs in the middle ear. It usually presents with ear pain. In young children, common presentations include fever, reduced oral intake, ear discharge, irritability and poor sleep. We describe a rare case of acute otitis media presenting with facial nerve palsy without any ear symptoms that resolved after myringotomy and grommet insertion.

Keywords: acute otitis media, facial nerve palsy, myringotomy, grommet insertion
Intranasal button battery: a case series
Pediatr Med Rodz 2019, 15 (1), p. 88–92
DOI: 10.15557/PiMR.2019.0016

Intranasal foreign bodies are a common occurrence in children that may be either accidental or self-induced. The most vulnerable age is between 6 months and 4 years, when children tend to explore new objects. Button batteries as foreign bodies in the nasal cavity are rarely seen but common in the paediatric age group; and therefore they represent a growing danger. They are hazardous as they can cause liquefaction necrosis with subsequent severe local tissue destruction. In the case of diagnostic uncertainty regarding the precise nature of a metallic looking foreign body in the nose, a high index of suspicion should be retained for the possibility of a button battery and urgent removal should be undertaken. We present three cases of intranasal button battery to emphasise their hazards of impaction and the value of early diagnosis and treatment, which can prevent severe local tissue damage resulting in late sequelae, such as septal perforation.

Keywords: foreign body, nose, button battery, nasal septal perforation
Munchausen syndrome by proxy – a case report
Pediatr Med Rodz 2019, 15 (1), p. 93–96
DOI: 10.15557/PiMR.2019.0017

Child abuse may take different forms. Munchausen syndrome by proxy is a factitious disorder in which disease symptoms are induced in a dependent person so that the perpetrator could play the role of a caregiver. Parent (mother in most cases) is usually the perpetrator, and a child is usually the victim. The desire to draw the attention of medical personnel, which can be a psychological reward, is the main motivation for a person affected by Munchausen syndrome by proxy. The scale of the problem remains unknown due to diagnostic difficulties. We present a case of a 10-year-old girl who was initially hospitalised for more than 2 months due to unexplained bleeding from the lower gastrointestinal tract, and was then admitted to another paediatric unit due to consciousness disorders. Accurate diagnosis and prevention of further child maltreatment was possible due to collecting thorough medical history, diagnostic vigilance and cooperation between medical centres where the girl was hospitalised.

Keywords: Munchausen syndrome by proxy, diagnostic difficulties, child abuse syndrome, haematuria, factitious disorders