Infectious complications are a major cause of morbidity and mortality in patients with primary and secondary immunodeficiency. Present recommendation
based on the last PID /IEI (Inborn Errors of Immunity) classification, of the International Union of Immunological Societies (IUIS). The aim of this document
is the propose of vaccinations standard based on published literature and the experience of the Polish Working Group on Primary Immunodeficiency
and the Committee for Immunology and Aetiology of Human Infections, Polish Academy of Science, about vaccines safety and efficacy, which can be used
in PID. This guideline is providing also recommendations for vaccines used in patients receiving immunosuppressive, biological or antimetabolite drugs.
Standardy Medyczne/Pediatria  2021  T. 18  403-413

The authors discussed selected reports on hepatological problems, presented during the 6th World Congress of the of Pediatric Gastroenterology,
Hepatology and Nutrition Society for Children (WCPGHAN), which took place in Vienna from 2-5.06.2021 and was also conducted on-line.
Standardy Medyczne/Pediatria  2021  T. 18  421-430

This article discusses the current epidemiological situation for influenza and COVID-19 in Poland. Many clinicians underline less frequent diagnosis of influenza
in the current epidemiological season, which is reflected in the statistics. Comparisons are also made between the two disease entities in order to
help with the diagnosis.
Standardy Medyczne/Pediatria  2021  T. 18  431-435

A balanced gut microbiota is crucial for health. Conversely, an altered gut microbiota composition and/or activity (dysbiosis) contributes to diseases. There
is an interest in the microbiota modifications. The article summarizes major consensus papers developed by the International Scientific Association for
Probiotics and Prebiotics (ISAPP) on biotics, i.e., probiotics, prebiotics, synbiotics, and postbiotics. The aim is to help making rational decisions regarding
their use. Apart from microbiota, many other factors affect health and contribute to diseases. Thus, one should have realistic expectations regarding
biotics. None of them will act as a panacea. While deciding on a specific “biotic” it is best to recommend the use of only those with documented efficacy
and safety and to use a regimen (dose and formulation, duration of treatment) proven to be effective in well-designed trials for the same indication, informing
about the expected effect size. This will allow patients (or their caregivers) to decide whether the anticipated benefits are in line with their expectations
and worth the costs incurred.
Standardy Medyczne/Pediatria  2021  T. 18  437-446

Polycystic ovary syndrome (PCOS) is one of the most common endocrine diseases and, according to some sources, it may affect 6-15% of women of reproductive
age. It is a disease that affects many processes in the human body and often has long-term health consequences, such as an increased risk of metabolic
disorders and cardiovascular diseases. The etiopathogenesis of the disease is complex and not fully understood, although it is emphasized that it may
be autoimmune. While the diagnosis of PCOS among adult women is widespread and established, the subject should be approached with some caution
among adolescents. Making a diagnosis in adolescents is difficult and often requires long-term follow-up because what is considered pathology in adult
women may not always be pathology in adolescent girls.
Standardy Medyczne/Pediatria  2021  T. 18  448-457

It should be emphasized that most of the flat valgus feet spontaneously resolve by the age of 10 and should not be treated until then, which does not exclude
incentives for an active lifestyle, cycling, swimming, etc. performed by a physiotherapist, participation in corrective classes against the patient’s will,
may cause more psychological and sociological harm. Similarly, the use of footwear with the so-called with hard heels, additionally with a hard sole or orthopedic insoles, is unnecessary in feet, which we consider physiological at this age.
After the age of 10, when the deformity of the squamous-valgus feet is permanent, but does not limit motor activity, does not generate pain, it can also be
accepted as a normal variant and untreated.
Flat-valgus feet, the so-called symptomatological, with pain symptoms, should be treated with implants inserted into the tarsal sinus, which leads to the
complete normalization of the function.
Standardy Medyczne/Pediatria  2021  T. 18  459-464

Alpha 1-antitrypsin deficiency is an inherited disease characterized by a low concentration or a non-functional form of the serum alpha-1 antitrypsin (AAT)
proteinase inhibitor. AAT deficiency (AATD) renders high susceptibility of lung tissue to noxious substance both endogenous and inhaled, and its damage,
which ultimately leads to variety of respiratory disorders, for example premature emphysema. Although the disease is relatively frequent, awareness and
knowledge about it in the general population and in the medical community is still insufficient, also due to the non-specific symptomatology of AATD. The
solution to the problem is creation of national registries of patients with alpha-1 antitrypsin deficiency, that have been established for several years. Their
goal is to collect the most comprehensive clinical information on the course of AATD, risk factors, and treatment, which provides the opportunity to perform
clinical, statistical analyses of a relatively large population. However, a coordinated effort is needed, which has been the rationale for the implementation
of the European Alpha-1 Clinical Research Collaboration (EARCO) project, linking together national European AATD registries, offering a much comprehensive
AAT research effort. The aim of this paper is to present the European AATD registries and their relevance to research on alpha-1 antitrypsin deficiency.
Standardy Medyczne/Pediatria  2021  T. 18  465-472

Cerebral palsy (CP) is a clinical diagnosis of motor and posture symptoms related to the early brain injury. The incidence of CP is 1,5 to 3 per 1000 live births.
In this paper we present the reasons and consequences of malnutrition in CP. The indications and methods of nutritional treatment are also listed.
Standardy Medyczne/Pediatria  2021  T. 18  475-482

In the group of children with disorders of sex development (DSD) feminizing genitoplasty is mainly performed in patients with congenital adrenal hyperplasia
(CAH). Feminizing genitoplasty is rarely performed in other forms of DSD like ovotesticular DSD or DSD with mosaic kariotype. In patiens with CAH virilization
of external genitalia is caused by enzymatic block in steroidogenesis resulting in androgen overproduction. After birth it is crucial to stabilize the
patient and to start hormonal treatment. Feminizing genitoplasty is performed between 6-12 month of life. Reconstruction procedure consists of: urethroplasty,
vaginoplasty, clitoroplasty and labioplasty. Cosmetic and functional results are good but some patients may require reoperation of a narrow vaginal
outlet at pubertal age.
Standardy Medyczne/Pediatria  2021  T. 18  484-489

Article explains step by step technique of preparing and performing bone marrow aspiration and bone marrow trephine biopsy with possible complications.
Standardy Medyczne/Pediatria  2021  T. 18  491-494

Meningococcal meningitis (Neisseria meningitidis), commonly known as meningococcus, is a pathogenic microorganism that causes rapid, life-threatening,
generalized infections – invasive meningococcal disease (IMD) –- septicemia, and meningitis. Of all serogroups, meningococci belonging to serogroups
A, B, C, W, and Y are the most important, accounting for more than 90% of all meningococcal infections worldwide. We present a case of a 7-year-old boy
whose illness began insidiously, in a manner typical for many trivial viral infections of childhood, quickly leading to severe general condition and hospitalization.
Rapid suspicion of meningococcal infection and prompt initiation of appropriate treatment saved the child’s life. This paper also highlights the role
of immunization as an effective way to prevent invasive meningococcal disease.
Standardy Medyczne/Pediatria  2021  T. 18  DOI:10.17444/SMP2021.18.09  502-506

According to Articles 32 and 34 of the Act on the doctor and dental surgeon professions and Article 17 of the Act on patient’s rights and the Patient
Ombudsman, the law authorizes a statutory representative (in the most of cases – parent/s) of a minor (anyone under the age of 18) to give informed consent
for most medical decisions on behalf of the child. However, in the case of minors at the age of 16 or older, the so-called cumulative consent (expressed
by the patients themselves and responsible for them adults) is obligatory. Exceptionally, it is possible to conduct treatment with the actual guardian’s permission
when it comes to uncomplicated surgeries. Generally, treatment activities undertaken in relation to a minor require the presence of a statutory
representative.
In general, the unaccompanied minor patient can be treated by medical staff provided that the guardian accepted a consent form prior to the treatment.
The doctor may then only provide such health services about which the statutory representative was previously properly informed and for which he consented.
However, all afore-mentioned solutions are applicable only to simple medical procedures, certainly not those that threaten patient’s life. In the case
of surgeries or high-risk procedures, their performance without the presence of an authorized guardian should be limited to a minimum.
Standardy Medyczne/Pediatria  2021  T. 18  508-513

The aim of study was to assess the impact of obesity on the incidence of pancreatitis in children. Materials and methods: The study included 303 patients
with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP), aged 1.5-18 years, hospitalized in the years 2001-2021. The analysis of the nutritional
status of patients included: body mass percentiles and z-scores for sex and age, height percentiles and z-scores for sex and age, BMI (body mass index) –
value in kg/m2, percentiles and z-scores for gender and age. The data necessary to establish the etiological factor and the severity of the course of ARP and
CP were analyzed. Results: 74 (24.4%) children with ARP and 229 (75.6%) with CP were analyzed. Hypertriglyceridemia was more common in overweight
or obese children (p < 0.05). Overweight and obesity were associated with a lower incidence of CP (p < 0.05), and the first episode of pancreatitis was diagnosed at a later age compared to patients with normal weight or underweight (p < 0.05). The incidence of diabetes was higher in the obese group (p <
0.05). Children with obesity received pancreatic enzyme supplementation less frequently, as in this group pancreatic exocrine insufficiency was observed
less frequently, but the difference was not statistically significant (NS). Conclusions: Overweight and obesity are associated with a later age of onset of pancreatitis and a later diagnosis of CP compared to an underweight or normal weight population. Chronic pancreatitis and pancreatic exocrine insufficiency
are less common in overweight or obese children.
Standardy Medyczne/Pediatria  2021  T. 18  496-500