List of Abbreviations
DIC – disseminated intravascular coagulation
BV – blood volume
РКИ – randomized controlled trial
Ht – hematocrit
Hb – hemoglobin
Terms and Definitions
Neonatal polycythemia – diagnosed in newborns at a venous hematocrit (Ht) level of 65% or higher.
"True" polycythemia – polycythemia detected in newborns with normal or increased circulating blood volume.
"False" polycythemia – polycythemia occurring secondary to neonatal dehydration and a relative increase in blood cell count relative to circulating plasma volume.
Partial exchange transfusion – a procedure of staged phlebotomy with equivalent replacement of the removed blood volume.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Neonatal polycythemia – diagnosed in newborns at a venous hematocrit (Ht) level of 65% or higher [1–5].
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
Hematocrit increases progressively with advancing gestational age; consequently, the probability of polycythemia is higher in post-term infants than in term infants [3,6]. Factors increasing the risk of increased placental transfusion and neonatal polycythemia include delayed umbilical cord clamping time and positioning the newborn below the level of the placenta [7–15]. Neonatal polycythemia is accompanied by an increase in blood viscosity [16–19], which can lead to tissue hypoxia, acidosis, hypoglycemia, microthrombi formation in microvascular beds, hemodynamic disturbances, and increased pulmonary vascular resistance [19–24]. Hematocrit in a healthy term newborn reaches its maximum 6–12 hours after birth, decreases by the end of the first day of life (typically by 18 hours of life), reaching cord blood values [3,25].
1.3 Epidemiology of the Disease or Condition
The incidence of neonatal polycythemia worldwide is estimated to range from 1 to 4–5% in appropriate-for-gestational-age term newborns [2,18].
1.4 Features of Coding the Disease or Condition
P61.1 – Neonatal polycythemia
1.5 Classification of the disease or condition
Depending on the presence or absence of clinical manifestations, neonatal polycythemia is divided into:
Symptomatic;
Asymptomatic.
Depending on circulating blood volume, it can be classified as normovolemic, hypervolemic, and hypovolemic [25]:
- Normovolemic polycythemia ("true" polycythemia) – a condition characterized by normal intravascular blood volume despite an increased red blood cell count.
- Hypervolemic polycythemia ("true" polycythemia) is characterized by an increased circulating blood volume (CBV) with a simultaneous increase in red blood cell count.
- Hypovolemic polycythemia occurs secondarily as a relative increase in red blood cell count relative to the circulating plasma volume. This condition typically develops > 24–48 hours after birth due to dehydration ("false" polycythemia) and can be compensated by adequate fluid administration.
1.6 Clinical presentation of the disease or condition
Up to 40% of newborns with polycythemia present with various clinical manifestations [26]. However, most of the discussed symptoms are non-specific and may also be observed in numerous other neonatal conditions (e.g., sepsis, asphyxia, hypocalcemia, respiratory and cardiovascular disorders).
Clinical and laboratory characteristics of polycythemia:
The following symptoms may be detected in neonatal polycythemia with varying frequency and in various combinations:
1. Skin discoloration:
- Crimson or bright red skin color, pronounced perioral cyanosis and acrocyanosis.
2. Central nervous system:
- Lethargy;
- Muscular hypotonia;
- Poor sucking;
- Increased excitability;
- Tremor;
- Seizures;
- Apnea;
- Cerebral venous thrombosis;
- Multiple cerebral infarctions;
- Intraventricular hemorrhage.
3. Respiratory system:
- Respiratory distress;
- Persistent pulmonary hypertension.
4. Cardiovascular disorders:
- Tachycardia;
- Congestive heart failure with low cardiac output;
- Cardiomegaly;
- Peripheral gangrene.
5. Gastrointestinal tract:
- Poor sucking;
- Vomiting;
- Abdominal distension;
- Hepatomegaly;
- Necrotizing enterocolitis.
6. Genitourinary system:
- Proteinuria;
- Hematuria;
- Renal vein thrombosis;
- Acute kidney injury;
- Priapism;
- Testicular infarction.
7. Metabolic disorders:
- Hypoglycemia;
- Hypocalcemia;
- Hypomagnesemia.
8. Hyperbilirubinemia.
9. Hematological disorders:
- Thrombocytopenia;
- Reticulocytosis;
- Thrombosis.
2. Diagnostics, medical indications and contraindications for diagnostic methods
Criteria for establishing the diagnosis/condition: based on:
1) physical examination: crimson or bright red skin color, pronounced perioral cyanosis and acrocyanosis, pastosity of subcutaneous tissue;
2) laboratory tests: venous hematocrit of 65% or higher.
2.1 Complaints and Anamnesis
In a newborn with polycythemia, it is recommended to review the maternal medical history to identify risk factors for polycythemia [2,27–32].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
Comments: Normovolemic neonatal polycythemia may develop due to placental insufficiency and/or chronic intrauterine fetal hypoxia, the history indicators of which include: fetal growth restriction; pregnancy-induced hypertension; maternal diabetes mellitus [29,30]; maternal tobacco smoking, both active [27,32] and passive [28]; post-term pregnancy [31]. Other conditions predisposing to normovolemic polycythemia include fetal endocrine and genetic disorders: congenital hypothyroidism; neonatal thyrotoxicosis; Beckwith-Wiedemann syndrome; congenital adrenal hyperplasia, chromosomal diseases (trisomies 13, 18, 21). In hypervolemic polycythemia, anamnestic risk factors may include: fetomaternal transfusion, including delayed cord clamping [4,10]; twin-twin transfusion syndrome (about 10% of monochorionic twins) [13]; placental transfusion.
2.2 Physical Examination
To identify symptoms of polycythemia and rule out "false" polycythemia, it is recommended that the newborn undergo a physical examination by a pediatrician [23,33].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
Comments: The physical examination by a pediatrician includes: examination of the skin and mucous membranes (color, moisture), assessment of tissue turgor, presence of respiratory disorders, and assessment of the newborn's neurological status. A characteristic clinical sign of neonatal polycythemia is a crimson or bright red skin color, pronounced perioral cyanosis and acrocyanosis. If dehydration develops in the newborn, the following signs appear: dry mucous membranes, decreased tissue turgor, and a decrease in urine output rate. Dehydration may be accompanied by the development of polycythemia, which is considered "false" polycythemia.
In a newborn with polycythemia, determination of daily and hourly urine output is recommended to detect renal function impairment [34].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
2.3 Laboratory Diagnostics
To determine the management tactics in a newborn with suspected polycythemia, a complete blood count is recommended [11].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
In a newborn with symptoms of polycythemia, a complete blood count is recommended no later than 6–12 hours after their onset to determine further management tactics [11].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
In a newborn with suspected polycythemia and a peripheral hematocrit > 65%, assessment of (venous) hematocrit is recommended no later than 6–12 hours after the detection of polycythemia to clarify the true hematocrit level [3].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
Comments: As a rule, capillary blood hematocrit is 5–15% higher than venous hematocrit [17,27]; treatment of polycythemia is carried out solely based on venous hematocrit test results.
In a newborn who has undergone staged bloodletting with equivalent replacement of the removed blood volume (partial exchange transfusion), a complete blood count and (venous) hematocrit assessment are recommended 4–6 hours after the procedure to monitor treatment efficacy [35,36].
GPP ALOE 1Grade of Recommendation A (Level of Evidence – 1).
2.4 Instrumental Diagnostics
If intraventricular hemorrhage, cerebral venous thrombosis, cerebral infarction, or renal vessel thrombosis is suspected in a newborn with polycythemia, ultrasound examinations are recommended: neurosonography (NSG), transcranial color duplex scanning of arteries and veins, ultrasound of the kidneys and adrenal glands, color duplex scanning of renal arteries, and color duplex scanning of the inferior vena cava and renal veins [37–42].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
2.5 Other Procedures
In a newborn older than 24 hours of life with suspected polycythemia, body weight measurement and assessment of the percentage of weight loss from birth weight are recommended to rule out "false" polycythemia [2].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
Comments: It is necessary to perform a differential diagnosis between "true" neonatal polycythemia and "false" polycythemia caused by dehydration in the infant [2]. Dehydration or hypovolemic polycythemia occurs secondarily as a relative increase in red blood cell count relative to circulating plasma volume, which is characterized by hemoconcentration and an elevated hematocrit level [25]. Dehydration should be suspected if body weight loss in the first days of life exceeds 8-10%. Six hours after adequate rehydration, the hematocrit level will decrease.
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
In a newborn with asymptomatic polycythemia and a hematocrit level of 70%–75%, to reduce blood viscosity and prevent complications associated with hyperviscosity, it is recommended to prescribe infusion therapy with other irrigation solutions (ATC code B05CX) – 10% dextrose** solution for at least 24 hours until the hematocrit level drops below 70% [43].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
Comments: The administered fluid volume is up to 100 ml/kg/day, monitored by urine output and body weight dynamics.
3.2 Surgical Treatment
In a newborn presenting with two or more symptoms of polycythemia and a venous hematocrit level of 65% or higher in the absence of signs of dehydration, as well as in an asymptomatic newborn with a hematocrit level of 76% or higher, it is recommended to perform step-by-step bloodletting with equivalent replacement of the removed blood volume (partial exchange transfusion) [17, 22, 43].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
Comments: The surgical method of treatment for true polycythemia is partial exchange transfusion [17,22]. Informed consent for the partial exchange transfusion must be obtained from the child's legal representative. Strict adherence to aseptic and antiseptic rules is mandatory. The volume of blood replacement depends on the desired target venous hematocrit level. The target venous hematocrit level is 50-60%. The total replacement volume is calculated using the formula: Replacement volume = BCC (80-90 ml/kg) x Body weight in kg x (Child's Ht - Target Ht)/Child's Ht, where BCC is blood circulating volume; Weight is body mass in kg, Ht is hematocrit [22]. The volume of a single replacement (single blood removal) and a single infusion must not exceed 5 ml/kg [44]. During and after the procedure, monitoring of vital functions (heart rate, respiratory rate, blood pressure, blood oxygen saturation) is required. Vascular access for partial exchange transfusion is typically established via the umbilical vein. In the absence of technical feasibility or in the presence of contraindications to umbilical vein catheterization, alternative venous access may be established to effectively perform the exchange of the required blood volume with an equivalent volume of sodium chloride** (ATC code B05XA) 0.9% solution.
When partial exchange transfusion (step-by-step bloodletting with equivalent replacement of the removed blood volume) is indicated in a newborn, it is recommended to use electrolyte solutions (ATC code B05XA) – 0.9% sodium chloride** solution as the replacement medium [36, 45–47].
GPP ALOE 1Grade of Recommendation A (Level of Evidence – 1).
Comments: Sodium chloride** (ATC code B05XA) 0.9% solution during partial exchange transfusion provides an optimal combination of quality and efficacy (low cost combined with the lowest potential side effects). Blood substitutes and plasma protein fractions (ATC code B05AA) are not superior in efficacy to sodium chloride** (ATC code B05XA) 0.9% solution [24,36,46,48,49]. The use of blood substitutes and plasma protein fractions (ATC code B05AA) is associated with a higher incidence of necrotizing enterocolitis [21,47,50].
3.3 Other Treatment
Not applicable.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
Not applicable.
5. Prevention and Dispensary Observation
To prevent the development of polycythemia in a newborn, it is not recommended to place the infant below the level of the placenta after birth before the umbilical cord is clamped [10, 14].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
Comments: Placing a term newborn below the level of the placenta increases the risk of polycythemia. The placental transfusion phenomenon is enhanced if the newborn is positioned 40 cm below the level of the placenta [14].
To prevent the development of polycythemia in a newborn, delayed cord clamping (more than 180 seconds after birth) is not recommended [15, 51].
GPP BLOE 3Grade of Recommendation B (Level of Evidence – 3).
Comments: Delayed cord clamping increases the risk of polycythemia in infants during the first 48 hours of life [51]. Blood redistribution between the infant and the placenta at birth corresponds to a ratio of approximately 67% / 33% (infant/placenta); after 3 minutes (180 seconds), 13% of the blood remains in the placenta and 87% in the infant [15].
Eligible for standard outpatient follow-up.
6. Organization of Medical Care
Indications for hospital admission to a medical organization:
1) Dark red or bright red skin color, pronounced perioral and acrocyanosis;
2) Venous hematocrit of 65% or higher.
Indications for discharging a patient from a medical organization:
1) Satisfactory condition;
2) Venous hematocrit less than 65%.
7. Additional Information
Long-term neurodevelopmental outcomes of polycythemia remain a subject of debate. Several studies have shown that polycythemia may be considered a risk factor for delayed psychomotor development and speech disorders [21, 48, 49, 52–54].
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | Performance assessment (yes/no) |
|---|---|---|
1. | Visual therapeutic examination performed | Yes/no |
2. | Daily and hourly urine output determination performed | Yes/no |
3. | Body weight measurement and assessment of the percentage of weight loss from birth weight performed in a newborn older than 24 hours of life | Yes/no |
4. | Complete blood count (CBC) performed when polycythemia is suspected or symptoms of polycythemia are present, no later than 6–12 hours after their onset | Yes/no |
5. | Venous hematocrit assessment performed when peripheral hematocrit exceeds 65%, no later than 6–12 hours after detection of polycythemia | Yes/no |
6. | Neurosonography and transcranial color duplex scanning of arteries and veins performed when intraventricular hemorrhage or cerebral venous thrombosis is suspected | Yes/no |
7. | Ultrasound of the kidneys and adrenal glands, color duplex scanning of renal arteries, and color duplex scanning of the inferior vena cava and renal veins performed when renal vessel thrombosis is suspected | Yes/no |
8. | Step-by-step bloodletting with equivalent replacement of the removed blood volume with 0.9% sodium chloride** solution (partial exchange transfusion) performed in the presence of two or more symptoms of polycythemia with a venous hematocrit of 65% or higher in the absence of signs of dehydration, or in asymptomatic polycythemia with a hematocrit of 76% or higher | Yes/no |
9. | Infusion therapy with 10% dextrose** solution (ATC code B05CX) administered for asymptomatic polycythemia with a venous hematocrit of 70–75% | Yes/no |
10. | Complete blood count (CBC) and venous hematocrit assessment performed 4–6 hours after partial exchange transfusion | Yes/no |
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Appendix A1. Working Group Composition
Ekaterina N. Balashova – Candidate of Medical Sciences, Leading Researcher at the Prof. A.G. Antonov Department of Anesthesiology and Intensive Care, Institute of Neonatology and Pediatrics, National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov of the Russian Ministry of Health, Associate Professor at the Department of Neonatology, Institute of Professional Education, National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov of the Russian Ministry of Health, Associate Professor at the Department of Neonatology, Pediatric Faculty, I.M. Sechenov First Moscow State Medical University of the Ministry of Health of Russia (Sechenov University), Member of the Council of the Russian Society of Neonatologists
Dmitry N. Degtyarev – Doctor of Medical Sciences, Professor, Deputy Director for Research, National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov of the Russian Ministry of Health, Head of the Department of Neonatology, Pediatric Faculty, I.M. Sechenov First Moscow State Medical University of the Ministry of Health of Russia (Sechenov University), Member of the Council of the Russian Society of Neonatologists
Anna L. Karpova – Candidate of Medical Sciences, Deputy Chief Physician for Pediatric Care at the Kaluga Regional Clinical Hospital (State Budgetary Healthcare Institution of the Kaluga Region), Chief freelance Neonatologist of the Ministry of Health of the Kaluga Region, Assistant at the Department of Outpatient Therapy, Clinical Laboratory Diagnostics and Medical Biochemistry of the Institute of Postgraduate Professional Education at the Yaroslavl State Medical University (Ministry of Health of the Russian Federation), Member of the Council of the Russian Society of Neonatologists.
Aleksey V. Mostovoy – Candidate of Medical Sciences, Head of the Neonatal Intensive Care Unit at the Kaluga Regional Clinical Hospital (State Budgetary Healthcare Institution of the Kaluga Region), Assistant at the Department of Outpatient Therapy, Clinical Laboratory Diagnostics and Medical Biochemistry of the Institute of Postgraduate Professional Education at the Yaroslavl State Medical University (Ministry of Health of the Russian Federation), Chief freelance Neonatologist of the Ministry of Health of the Russian Federation in the North Caucasian Federal District, Member of the Council of the Russian Society of Neonatologists, Member of the European Society for Paediatric Research (ESPR).
Marina V. Narogan – Doctor of Medical Sciences, Professor at the Department of Neonatology of the N.F. Filatov Clinical Institute of Child Health at the I.M. Sechenov First Moscow State Medical University of the Ministry of Health of Russia (Sechenov University), Member of the Council of the Russian Society of Neonatologists.
Olga I. Sapun – Head of Neonatal ICU No. 2 at the Children's Regional Clinical Hospital, Krasnodar, Chief freelance Neonatologist of the Ministry of Health of the Krasnodar Territory, Member of the Council of the Russian Society of Neonatologists.
No conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
Methods used for collecting/selecting evidence: electronic database search.
Description of methods used for evidence collection/selection: the evidence base for the guidelines included publications from the Cochrane Library, PubMed, EMBASE, MEDLINE, Scopus, Web of Science, eLIBRARY, clinicaltrials.gov, electronic libraries, and clinical guidelines published by the National Institute for Health and Care Excellence, the European Association of Perinatal Medicine, the European Society for Pediatric Research, the European Foundation for the Care of Newborn Infants, and the European Society for Neonatology. The search depth was 59 years.
Methods used for assessing the quality and strength of evidence:
expert consensus;
assessment of significance in accordance with the rating scheme.
Grading scales for evidence levels (Tables 1, 2) for diagnostic, preventive, therapeutic, and rehabilitation methods (interventions) with definitions, and the grading scale for recommendation strength levels (Table 3) for preventive, diagnostic, therapeutic, and rehabilitation methods (interventions).
Methods used for analyzing evidence:
reviews of published meta-analyses;
systematic reviews with evidence tables.
Description of methods used for analysis of evidence.
When selecting publications as potential sources of evidence, the methodology used in each study was reviewed to ensure its validity. Methodological evaluation was based on questions focused on study design features that significantly affect the validity of the results and conclusions.
To minimize potential subjective errors, each study was evaluated independently by at least two members of the working group. Any discrepancies in assessments were discussed by the full working group. If consensus could not be reached, an independent expert was involved.
Economic analysis:
Cost analysis was not performed, and pharmacoeconomic publications were not evaluated.
Method of guidelines validation:
external expert review;
internal expert review.
Description of the guidelines validation method:
The preliminary version of these guidelines was reviewed by independent experts who were asked to comment primarily on whether the interpretation of the evidence underlying the recommendations was accessible and understandable.
Comments were received from primary care physicians and district pediatricians regarding the clarity of the presentation of the guidelines and their assessment of the importance of the guidelines as a working tool in daily practice.
The draft version was also sent to a non-medically trained reviewer to obtain comments from a patient perspective.
Comments received from experts were carefully systematized and discussed by the chair and members of the working group. Each point was discussed, and resulting modifications to the guidelines were recorded. If no changes were made, the reasons for declining the changes were recorded.
Consultation and peer review:
The latest amendments to these guidelines are presented for discussion as a draft version. The updated version for broad discussion was posted on the websites of the Russian Society of Neonatologists (RSN) www.neonatology.pro and the Russian Association of Perinatal Medicine (RAPM) www.raspm.ru to allow all interested parties to participate in the discussion and improvement of the guidelines.
The draft guidelines were reviewed by independent experts who were asked to comment primarily on the clarity and accuracy of the interpretation of the evidence base underlying the recommendations.
For the final revision and quality control, the recommendations were re-evaluated by the members of the working group, who concluded that all comments and feedback from the experts had been taken into account and the risk of bias in the development of the recommendations was minimized.
Target audience for these clinical guidelines:
1. Neonatologists
2. Anesthesiologists-intensivists
3. Pediatricians
4. Obstetricians-gynecologists
Table 1. Levels of Evidence (LoE) grading scale for diagnostic methods (diagnostic interventions)
LoE | Definition |
|---|---|
1 | Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | Only justification of mechanism of action or expert opinion is available |
Table 2. Levels of Evidence (LoE) grading scale for prevention, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, therapeutic, and rehabilitative interventions)
LoE | Definition |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available |
Table 3. Scale for assessing the grades of recommendation (GR) for prevention, diagnostic, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, diagnostic, therapeutic, and rehabilitative interventions)
GoR | Definition |
|---|---|
A | Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
B | Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent) |
C | Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines.
The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.
Appendix A3. Reference Materials
Order of the Ministry of Health of the Russian Federation No. 921n dated November 15, 2012, "On Approval of the Procedure for Providing Medical Care in the Profile 'Neonatology'."
Federal Law No. 323-FZ "On the Fundamentals of Protecting the Health of Citizens in the Russian Federation" dated November 21, 2011.
Order of the Russian Ministry of Health No. 203n of May 10, 2017, "On Approval of Criteria for Assessing the Quality of Medical Care."
International Statistical Classification of Diseases and Related Health Problems, 10th Revision (ICD-10) (World Health Organization), 2019 version.
Nomenclature of Medical Services (Ministry of Health and Social Development of the Russian Federation), 2020.
Methodological Letter of the Ministry of Health of the Russian Federation No. 15-4/I/2-2570 dated March 4, 2020, "Resuscitation and Stabilization of Newborn Infants in the Delivery Room." Edited by E.N. Baybarina. Moscow: Ministry of Health of the Russian Federation; 2020.
Order of the Ministry of Health of the Russian Federation No. 1130n dated October 20, 2020, "On Approval of the Procedure for Providing Medical Care in the Profile 'Obstetrics and Gynecology (Excluding Assisted Reproductive Technologies)'."
State Register of Medicines: https://grls.rosminzdrav.ru
List of Vital and Essential Medicines for Medical Use and Minimum Assortment of Medicinal Products Required for Medical Care (Decree of the Government of the Russian Federation No. 2406-r dated October 12, 2019).
Appendix B. Physician Action Algorithms
Diagram. Physician's action algorithm for polycythemia in a newborn infant.
Appendix C. Patient Information
Polycythemia is diagnosed in newborn infants with a venous hematocrit (Ht) of 65% or higher. Hematocrit reflects the ratio of red blood cells to blood plasma. The probability of polycythemia is higher in post-term infants.
Neonatal polycythemia may occur due to placental insufficiency and/or chronic intrauterine fetal hypoxia caused by: arterial hypertension; maternal diabetes mellitus; maternal tobacco smoking (both active and passive). Polycythemia can develop in newborn infants with intrauterine growth restriction. Other conditions predisposing to the development of polycythemia include rare endocrine and genetic disorders in the fetus: congenital hypothyroidism; neonatal thyrotoxicosis; Beckwith-Wiedemann syndrome; congenital adrenal hyperplasia; chromosomal disorders (trisomies 13, 18, 21).
Polycythemia in newborns is accompanied by increased blood viscosity, which can lead to reduced tissue oxygen levels, decreased blood glucose content, and microthrombus formation in blood vessels. Neonatal polycythemia may also develop in cases of increased blood transfusion to the fetus from the mother or from a co-twin in multiple pregnancies.
Polycythemia is characterized by changes in skin color, which becomes dark purple-cyanotic or cherry-colored. The infant may develop symptoms such as lethargy, tachypnea, decreased urine output, and jaundice due to elevated blood bilirubin levels resulting from the breakdown of an excessive number of red blood cells. A complete blood count reveals elevated hematocrit, hemoglobin, and red blood cell counts, and frequently a decreased platelet count.
The active treatment method for polycythemia is partial exchange blood transfusion, which leads to the normalization of hematocrit and hemoglobin. Indications for the procedure are determined by the physician. To perform the procedure, umbilical (or other) vein catheterization is carried out. The goal of partial exchange blood transfusion is to reduce blood viscosity and minimize the risk of complications from hyperviscosity. The procedure consists of sequentially withdrawing a portion of the infant's blood and replacing it with a 0.9% sodium chloride solution** (ATX code B05XA). As a result of this procedure, excessive levels of red blood cells, hemoglobin, and hematocrit are normalized, and the clinical manifestations of polycythemia resolve over time.
Following the procedure, a blood count is monitored, and the infant is observed for 3 days. The infant is discharged home only in a satisfactory condition.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Not applicable.