Newborns with polycythemia neonatorum have nonspecific findings, related most often to the central nervous and respiratory systems: irritability or lethargy, hypotonia, seizures, tremor or jitters [1], motor delay and respiratory distress, tachypnea, apnea, and cyanosis. However, some remain asymptomatic [2]. Neurologic suffering is caused by low cerebral blood flow and impaired tissue metabolism due to hypoglycemia and hypocalcemia [3] [4]. Respiratory abnormalities are due to reduced pulmonary blood flow and increased pulmonary resistance, which can lead to hypoxia [5]. Poor feeding may be a consequence of the increased respiratory effort, recurrent vomiting or heart failure. Abdominal distension can sometimes be observed. Debate exists on whether necrotizing enterocolitis, seen in some newborn polycythemia cases, is caused by the blood abnormality itself or by its treatment.

Clinical examination may also reveal plethora, signs of heart failure, transient arterial hypertension and genitourinary abnormalities: oliguria, hematuria, and priapism. Peripheral gangrene and testicular infarctions are seldom noticed.

Neonatal polycythemia often occurs in children with ABO or Rh incompatibility. In this case, jaundice is observed and whether it is due to the polycythemia (and breakdown of a high number of red blood cells) or the blood type incompatibility is hard to determine.

• Trisomy 21

  Genetics disorders Genetic conditions that increase fetal erythropoiesis include the following: Trisomy 13 Trisomy 18 Trisomy 21 Beckwith-Wiedemann syndrome Hypertransfusion Polycythemia-hyperviscosity could also be secondary to increased blood volume [emedicine.medscape.com]

  Chromosomal abnormalities (including Turner’s syndrome, trisomy 18, trisomy 21): Certain chromosomal abnormalities are associated with a paucity of intrahepatic bile ducts, resulting in cholestasis. [pathophys.org]

• Gangrene

  Peripheral gangrene and testicular infarctions are seldom noticed. Neonatal polycythemia often occurs in children with ABO or Rh incompatibility. [symptoma.com]

  E08.52 Diabetes mellitus due to underlying condition with diabetic peripheral angiopathy with gangrene Includes: Diabetes mellitus due to underlying condition with diabetic gangrene E08.59 Diabetes mellitus due to underlying condition with other circulatory [app.drchrono.com]

• Failure to Thrive

  Brief resolved unexplained event -- BRUE (Medical Encyclopedia) Crying - excessive (0-6 months) (Medical Encyclopedia) Failure to thrive (Medical Encyclopedia) Hemorrhagic disease of the newborn (Medical Encyclopedia) Hyperglycemia - infants (Medical [icdlist.com]

  […] to thrive, urosepsis, poor urinary stream, and straining or grunting while voiding Appearance of erythema toxicum Multiple erythematous macules and papules (1 to 3 mm in diameter) that rapidly progress to pustules on an erythematous base Marijuana use [brainscape.com]

  […] to thrive) 缺乏預期正常生理發育，生長遲緩 Ill-defined conditions 其他診斷欠明之病態 Accidental fall 意外墜落 Vaccine against viral hepatitis B b型肝炎 預防注射 Routine infant or child health check 嬰幼兒例行之健康檢查 Observation for suspected condition, newborn 新生兒可疑病態之觀察 Counseling (Health education [money511.com]

  […] to thrive Multifactorial causes Tachycardia, fever Infection causing cholestasis Diagnosis Am Fam Physician. 2002 Feb 15; 65 ( 4 ):599-606. [pathophys.org]

  […] to thrive in newborn 新生兒生長遲滯 P928 Other feeding problems of newborn 新生兒其他餵食問題 P929 Feeding problem of newborn, unspecified 新生兒餵食問題，未特定 P93 Reactions and intoxications due to drugs adminiserred to newborn 新生兒用藥所致之反應及中毒 P930 Grey baby syndrome 灰嬰症候群 P938 [csh.org.tw]

• Recurrent Vomiting

  Poor feeding may be a consequence of the increased respiratory effort, recurrent vomiting or heart failure. Abdominal distension can sometimes be observed. [symptoma.com]

• Cyanosis

  […] with polycythemia neonatorum have nonspecific findings, related most often to the central nervous and respiratory systems: irritability or lethargy, hypotonia, seizures, tremor or jitters, motor delay and respiratory distress, tachypnea, apnea, and cyanosis [symptoma.com]

  […] pol·y·cy·the·mi·a ve·ra [MIM*263300] a chronic form of polycythemia of unknown cause; characterized by bone marrow hyperplasia, an increase in blood volume as well as in the number of red blood cells, redness or cyanosis of the skin, and splenomegaly. [medical-dictionary.thefreedictionary.com]

  Tachypnea, mild cyanosis, plethoric skin color, and neurological depression persisted on average for 30 h after birth. Chest X-rays showed mild cardiomegaly, pulmonary congestion and edema, and pleural effusions. [karger.com]

  Frequent signs and symptoms included "feeding problems" (21.8%), plethora (20.0%), lethargy (14.5%), cyanosis (14.5%), respiratory distress (9.1%), jitteriness (7.3%), and hypotonia (7.3%). [pediatrics.aappublications.org]

  Why would giving oxygen not improve cyanosis if the infant has a right-to-left shunt? Blood flow to the lungs is decreased, and unoxygenated blood is mixed with oxygenated blood in the systemic circulation. [studystack.com]

• Tachycardia

  […] newborn P283Primary sleep apnea of newborn P284Other apnea of newborn P2881Respiratory arrest of newborn P2889Other specified respiratory conditions of newborn P289Respiratory condition of newborn, unspecified P290Neonatal cardiac failure P2911Neonatal tachycardia [cms.gov]

  […] disease Cholestasis (Impaired excretion) Jaundice Accumulation of conjugated bilirubin Hepatomegaly Obstruction of hepatic outflow ducts Pale stool Lack of stercobilin (which normally gives feces its dark colour) Failure to thrive Multifactorial causes Tachycardia [pathophys.org]

  Shock presents with tachycardia, a low blood pressure and delayed capillary filling time. Within 30 minutes of the haemorrhage the haemoglobin and packed cell volume start to fall rapidly. [intranet.tdmu.edu.ua]

  […] specified conditions originating in the perinatal period 779.81 Neonatal bradycardia Excludes: abnormality in fetal heart rate or rhythm complicating labor and delivery (763.81-763.83) bradycardia due to birth asphyxia (768.5-768.9) 779.82 Neonatal tachycardia [theodora.com]

  […] newborn 新生兒其他特定的呼吸病況 P289 Respiratory condition of newborn, unspecified 新生兒呼吸病況，未特定 P29 Cardiovascular disorder originating in the perinatal period 周產期的心臟血管疾患 P290 Neonatal cardiac failure 新生兒心臟衰竭 P291 Neonatal cardiac dysrhythmia 新生兒心律不整 P2911 Neonatal tachycardia [csh.org.tw]

• Jaundice

  Yea... 762 x 529 jpeg 133kB pic source Neonatal Jaundice 1 638 x 479 jpeg 75kB pic source Jaundice charts - Neon... 1242 x 864 jpeg 252kB pic source Neonatal Jaundice 1 638 x 479 jpeg 87kB pic source Candidiasis symptoms m... 236 x 177 jpeg 10kB pic [mnogolok.info]

  […] from other and unspecified hepatocellular damage P59.20 Neonatal jaundice from unspecified hepatocellular damage P59.29 Neonatal jaundice from other hepatocellular damage P59.3 Neonatal jaundice from breast milk inhibitor P59.8 Neonatal jaundice from [icd10data.com]

  In this case, jaundice is observed and whether it is due to the polycythemia (and breakdown of a high number of red blood cells) or the blood type incompatibility is hard to determine. [symptoma.com]

  […] chlamydial conjunctivitis Ophthalmia neonatorum NOS excludes: gonococcal conjunctivitis (A54.31) P39.4 Neonatal skin infection Inclusion: Neonatal pyoderma excludes: pemphigus neonatorum (L00) staphylococcal scalded skin syndrome (L00) P58.3 Neonatal jaundice [codelay.com]

  […] for signs of worsening Pathological jaundice · Clinical jaundice detected 24 hrs of age · Rise in TSB by 5 mg/ dl/ day ( 86 umol/l/day) · TSB 15 mg/ dl ( 257 umol/l) · Clinical jaundice persisting 14 Ds · Clay/white colored stool and/or dark urine staining [pt.slideshare.net]

• Neonatal Jaundice

  Gallery images and information: Diagnosis Polycythemia Neonatorum pic source Neonatal Jaundice 1 638 x 479 jpeg 79kB pic source Neonatal Jaundice 1 638 x 479 jpeg 54kB pic source Hyperbilirubinemia Car... 252 x 182 png 63kB pic source Kern Medical Third [mnogolok.info]

  jaundice from other and unspecified hepatocellular damage P59.20 Neonatal jaundice from unspecified hepatocellular damage P59.29 Neonatal jaundice from other hepatocellular damage P59.3 Neonatal jaundice from breast milk inhibitor P59.8 Neonatal jaundice [icd10data.com]

  jaundice due to polycythemia P61.1 Polycythemia neonatorum P83.0 Sclerema neonatorum P83.8 Other specified conditions of integument specific to newborn Inclusion: Bronze baby syndrome Neonatal scleroderma Urticaria neonatorum R71 Abnormality of red blood [codelay.com]

  jaundice due to other excessive hemolysis 其他過量溶血所致之新生兒黃疸 P580 Neonatal jaundice due to bruising 瘀傷所致之新生兒黃疸 P581 Neonatal jaundice due to bleeding 出血所致之新生兒黃疸 P582 Neonatal jaundice due to infection 感染所致之新生兒黃疸 P583 Neonatal jaundice due to polycythemia [csh.org.tw]

  […] delivery Hyperbilirubinemia of prematurity Jaundice due to delayed conjugation associated with preterm delivery 774.3 Neonatal jaundice due to delayed conjugation from other causes 774.30 Neonatal jaundice due to delayed conjugation, cause unspecified [theodora.com]

• Scleral Icterus

  The most notable signs of hyperbilirubinemia are jaundice and scleral icterus. Jaundice refers to yellowing of the skin, which can be seen by blanching the skin with digital pressure. [pathophys.org]

• Muscle Weakness

  Facial palsy is muscle weakness of one side of the face due to trauma to the facial nerve. This is almost always caused by pressure from a forceps blade on the facial nerve just in front of the ear. [intranet.tdmu.edu.ua]

• Skin Discoloration

  Discoloration of skin (finding) Discoloration, NOS Discolored Discolouration Major Diagnostic Categories M.D.C MDC Category : 15 MDC Type : Medical Description : Newborn And Other Neonates (Perinatal Period) Medicare Severity-Diagnosis Related Groups [medicbind.com]

• Seizure

  Newborns with polycythemia neonatorum have nonspecific findings, related most often to the central nervous and respiratory systems: irritability or lethargy, hypotonia, seizures, tremor or jitters, motor delay and respiratory distress, tachypnea, apnea [symptoma.com]

  Differential Diagnosis section highlights 50 new topics, including: alcohol-related seizures, dysentery and inflammatory enterocolitis, hair loss, cystic and solid pancreatic lesions, and COPD decompensation. [books.google.com]

  Symptoms may include: Extreme sleepiness Feeding problems Seizures There may be signs of breathing problems, kidney failure, low blood sugar, or newborn jaundice. [medlineplus.gov]

  · Often apnea and secondary asphyxia · Bradycardia · Hypotonia of muscles, hyporephlexia · Hyperesthesia · Seizures · Fixed glance · Strained large fontanel Intracranial hemorrhages · Coma · Severe breathing and cardiac disturbances · Tonic seizures, [intranet.tdmu.edu.ua]

  […] prenatal drug exposure. appear hungry, but suck & swallow poorly coordinated; hyperactive, increased muscle tone; regurgitation, vomiting, & diarrhea; signs typical of hypoglycemia but normal glucose level; restlessness; & irritability, fail to gain wt, & seizures [studystack.com]

• Lethargy

  Newborns with polycythemia neonatorum have nonspecific findings, related most often to the central nervous and respiratory systems: irritability or lethargy, hypotonia, seizures, tremor or jitters, motor delay and respiratory distress, tachypnea, apnea [symptoma.com]

  Frequent signs and symptoms included "feeding problems" (21.8%), plethora (20.0%), lethargy (14.5%), cyanosis (14.5%), respiratory distress (9.1%), jitteriness (7.3%), and hypotonia (7.3%). [pediatrics.aappublications.org]

  […] amount of bilirubin Nonhemolytic State Jaundice Accumulation of unconjugated bilirubin Ecchymosis/hemorrhage Birthing trauma Ruddy complexion, hepatomegaly Polycythemia Metabolic (Impaired conjugation) Jaundice Accumulation of unconjugated bilirubin Lethargy [pathophys.org]

  This period provides an ideal opportunity to look out for hypothermia, respiratory distress, abnormal neurological features such as jitteriness, convulsions, excessive lethargy, etc. [intranet.tdmu.edu.ua]

• Neuralgia

  Diseases and Disorders section features more than 300 new figures and tables, as well as 20 new topics including: cyclic vomiting syndrome, traveler’s diarrhea, chronic pruritus, post-herpetic neuralgia, enteropathic arthritis, and hoarding disorder. [books.google.com]

  […] neuropathy, unspecified E08.41 Diabetes mellitus due to underlying condition with diabetic mononeuropathy E08.42 Diabetes mellitus due to underlying condition with diabetic polyneuropathy Includes: Diabetes mellitus due to underlying condition with diabetic neuralgia [app.drchrono.com]

The clinical suspicion of polycythemia neonatorum is confirmed by venous hematocrit (>65%) or blood viscosity ( >12 centipoise) measurement. Additional tests should include complete cell blood count (could reveal thrombocytopenia [6] [7] as a consequence of localized thrombosis or disseminated intravascular coagulation), hypoglycemia, hypocalcemia [8], bilirubin level (high due to the high amount of bilirubin precursors). In addition, hypoxia stimulates peripheral release of young, nucleated red blood cells and reticulocytosis. Cyanotic infants may have decreased oxygenation demonstrated by arterial blood gas measurement. Maternal blood glucose should also be measured because the disease is more frequent in infants of diabetic mothers.

Urine analysis may reveal hematuria and proteinuria if renal vein thrombosis has occurred. A low glomerular filtration rate has also been described.

Postero-anterior thoracic radiography shows pulmonary congestion and cardiomegaly, therefore echocardiography is indicated in affected babies. This test will highlight the presence of decreased cardiac output and increased pulmonary resistance.

Symptomatic infants, as well as those whose hematocrit level is above 65% two hours after birth, should be evaluated in a dynamic manner, at 12 and 24 hours. Screening should be performed in children of diabetic mothers, small and large for gestational age infants and monochorionic twins [9]. In these cases, the disease should be suspected in utero, based on middle cerebral artery peak systolic velocity [10].

1. Rosenkrantz TS. Polycythemia and hyperviscosity in the newborn. Semin Thromb Hemost. 2003;29:515–27.
2. Wiswell TE, Cornish JD, Northam RS. Neonatal polycythemia: Frequency of clinical manifestations and other associated findings. Pediatrics. 1986;78:26–30.
3. Sarkar S, Rosenkrantz TS. Neonatal polycythemia and hyperviscosity. Semin Fetal Neonatal Med. 2008;13:248–255.
4. Dempsey EM, Barrington K. Short and long term outcomes following partial exchange transfusion in the polycythaemic newborn: A systematic review. Arch Dis Child Fetal Neonatal Ed. 2006;91:F2–F6.
5. Herson VC, Raye JR, Rowe JC, et al. Acute renal failure associated with polycythemia in a neonate. J Pediatr. 1982;100:137–139.
6. Vlug RD, Lopriore E, Janssen M, et al. Thrombocytopenia in neonates with polycythemia: incidence, risk factors and clinical outcome. Expert Rev Hematol. 2015;8(1):123-129.
7. Acunas B, Celtik C, Vatansever U, et al. Thrombocytopenia: An important indicator for the application of partial exchange transfusion in polycythemic newborn infants? Pediatr Int. 2000;42:343–347.
8. Saggese G, Bertelloni S, Baroncelli GI, et al. Elevated calcitonin gene-related peptide in polycythemic newborn infants. Acta Paediatr. 1992;81:966–968.
9. Oh W. Neonatal polycythemia and hyperviscosity. Pediatr Clin North Am 1986;33:523-532.
10. Veujoz M, Sananès N, Severac F, et al. Evaluation of prenatal and postnatal diagnostic criteria for twin anemia-polycythemia sequence. Prenat Diagn. 2015;35(3):281-288.