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Polycythemia Neonatorum

Neonatal Polycythemia Syndrome

Polycythemia neonatorum represents a clinical condition due to a high hematocrit (above 65% or 2 standard deviations above the normal value for age, measured in a venous, not capillary blood sample) and characterized by signs of hyperviscosity, poor tissue oxygenation and perfusion and a tendency for thrombosis. The test should be performed using venous blood because capillary blood normally has a higher hematocrit value.


Presentation

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.

Down Syndrome
  • Pediatrics 38 , 419 (1966) PubMed Google Scholar Weinberger, M.M., Oleinick, A.: Congenital marrow dysfunction in Down's syndroms. J.[link.springer.com]
  • Chronic sinusitis 慢性鼻竇炎 Congenital anomaly,unspecified 先天畸形未明示者 Conjunctivitis 結膜炎 Constipation 便秘 Contact dermatitis and other eczema 接觸性皮膚炎及其他濕疹 Convulsions 痙攣 Croup 嘶哮 Cystitis 膀胱炎 Dermatophytosis of the body 體部之髮癬菌病 Disorder of metabolism 新陳代謝失調症 Down's[money511.com]
  • More children suffer from brachial plexus injuries sustained at birth than Down's Syndrome or Muscular Dystrophy - yet information on this disability is not so readily obtained.[intranet.tdmu.edu.ua]
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]
Jaundice
  • Car... 252 x 182 png 63kB pic source Neonatal Jaundice 1 638 x 479 jpeg 75kB pic source Neonatal juindice 728 x 546 jpeg 66kB pic source i 's a L bou m3dIcS 198 x 162 jpeg 15kB pic source Neonatal Jaundice 1 638 x 479 jpeg 79kB pic source Neonatal Jaundice[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
  • Car... 252 x 182 png 63kB pic source Neonatal Jaundice 1 638 x 479 jpeg 75kB pic source Neonatal juindice 728 x 546 jpeg 66kB pic source i 's a L bou m3dIcS 198 x 162 jpeg 15kB pic source Neonatal Jaundice 1 638 x 479 jpeg 79kB pic source Neonatal Jaundice[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]
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]
  • […] and residuals- no bottle or breast feeding Neonatal Abstinence Syndrome and Fetal Alcohol Syndrome- Treatment - Only can keep them comfortable when they are withdrawing - Pharmacological medications such as Dilantin, Valium, Clonidine, Morphine for seizure[quizlet.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]
  • Epidemiology : 1–5% of newborns Risk factors SGA LGA Infants of diabetic mothers Maternal tobacco use Delayed umbilical cord clamping Clinical features Respiratory distress , cyanosis , apnea Poor feeding , vomiting Hypoglycemia Ruddiness ( plethora ) Lethargy[amboss.com]
  • […] 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]
  • Hypothermia* - Hyperthermia* - Temperature instability Sepsis Neonatorum- S/S of sepsis- Cardiovascular - Bradycardia - Tachycardia* - Arrhythmias - Hypotension - HTN - Decreased perfusion* Sepsis Neonatorum- S/S of sepsis- Neurological - Tremors - Lethargy[quizlet.com]
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]

Workup

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].

Treatment

  • Ankylosing spondylitis - Current protocols for diagnosis and treatment options. Wegener granulomatosis - Autoimmune etiology and clinical course.[netdoc.com]
  • Debate exists on whether necrotizing enterocolitis, seen in some newborn polycythemia cases, is caused by the blood abnormality itself or by its treatment.[symptoma.com]
  • Ferri’s Clinical Advisor 2018 is the most efficient, intuitive, and thorough resource of its kind, trusted by physicians to provide current diagnosis and treatment recommendations for hundreds of common medical conditions.[books.google.com]
  • Access up-to-date diagnostic and treatment information on more than 700 common medical conditions with Ferri’s Clinical Advisor 2017 , which boasts the popular "5 books in 1" format![books.google.com]
  • Treatments depend on the cause. They may include medicines and transfusions of blood, platelets, or clotting factor.[icdlist.com]

Prognosis

  • Usually good prognosis Spontaneous resolution is common (70% by age 7) Visual impairment in untreated periorbital hemangioma Others Milia neonatorum : tiny epidermal papules on the face caused by the buildup of keratin and sebaceous secretions .[amboss.com]
  • , Prevention, and Complications Prognosis good to very good in most individuals if untreated, adrenal crisis can lead to death within 1-6 weeks males usually have normal fertility; females may be abnormal fertility Prevention genetic counseling may help[medbullets.com]
  • Prognosis Curing or removing the underlying cause of this disorder generally eliminates the symptoms. Resources OTHER "Secondary Erythrocytosis." The Merck Page . June 3, 1998. 〈 〉 .[encyclopedia.com]
  • HIE - Grading and prognosis The staging system proposed by Sarnat and Sarnat in 1976 is often useful.[intranet.tdmu.edu.ua]
  • Prognosis and treatment of polycythemia vera. Last reviewed June 2013. UpToDate Inc., Waltham, MA. Wang WC, Dwan K. Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease.[aetna.com]

Etiology

  • Wegener granulomatosis - Autoimmune etiology and clinical course. Diabetes - disease and management information, including diagnosis, typical treatment plans and diabetes supplies.[netdoc.com]
  • FOLLOW-UP RECOMMENDATIONS Periodic laboratory follow-up depending on the etiology of polycythemia. Monitor for the following: Headache, dizziness, or syncope Blurred visio... Cario H, McMullin MF, Pahl HL.[5minuteconsult.com]
  • (Phototherapy) Treatment depends on the severity of hyperbilirubinemia, its etiology, and the risk of developing serious neurological complications. Phototherapy The mainstay of treatment for hyperbilirubinemia is phototherapy .[pathophys.org]
  • Increased blood viscosity can cause hypoperfusion directly by decreasing blood flow (as is seen in the lungs and the renal system), whereas other organs can become hypoperfused due to changes in arterial oxygen content (as noted in the brain). [ 6 ] Etiology[emedicine.medscape.com]
  • Etiology : unknown (probable contributing factors: immature sebaceous glands and/or hair follicles) Clinical features Small, red macules and papules that progress to pustules with surrounding erythema Located on trunk and proximal extremities ; spares[amboss.com]

Epidemiology

  • Pagina 340 - The Framingham Study: an epidemiological investigation of cardiovascular disease. National Institutes of Health publication, 1974:74-599. ‎[books.google.it]
  • Capillary malformations : ( naevus flammeus , port-wine stain , firemark ) Definition: congenital, benign vascular malformations of the small vessels in the dermis Epidemiology : may occur in association with a neurocutaneous disorder such as S turge–Weber[amboss.com]
  • Monochorionic diamniotic (MCDA) twin pregnancies with amniotic fluid discordance appear to increase the risk of development of twin anemia-polycythemia sequence (TAPS), a form of TTTS, by nearly two-fold. [12] Epidemiology Polycythemia is a relatively[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • The acquired form is more common, and pathophysiology has not been fully elucidated.[pathophys.org]
  • […] and size of lesion) Prognosis : Large nevi are at risk of degeneration frequent follow-up Infantile hemangioma ( strawberry hemangioma ) Definition : benign vascular tumors of infancy Epidemiology Occurs in up to 10% of infants Mostly affects girls Pathophysiology[amboss.com]
  • Pathophysiology Most of the clinical significance of polycythemia is related to the associated increase in blood viscosity. [3] Blood viscosity increases linearly with increased hematocrit (Hct), but it can increase exponentially when Hct is greater than[emedicine.medscape.com]
  • Hereditary hemochromatosis: Pathophysiology, diagnosis, and management. Crit Care Nurs Clin North Am. 2008;20(2):191-201, vi. Finazzi G, Barbui T. Evidence and expertise in the management of polycythemia vera and essential thrombocythemia.[aetna.com]
  • Advances in science and technology have allowed researchers to gain a better understanding of the pathophysiology leading to long-term neurologic damage in newborns.[intranet.tdmu.edu.ua]

Prevention

  • - 0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis[medicbind.com]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Neonatal Intensive Care Unit(NICU) Pediatrics Intensive Care Unit(PICU) Child Psychiatric Day Care Center Emergency Service (24 hours) Outpatient Service Pediatrics SPECIALTY/PRACTICE AREAS: • Pediatric pulmonology medicne • General pediatrics • Preventive[english.tmuh.org.tw]
  • Treating polycythaemia Treatment for polycythaemia aims to prevent symptoms and complications (such as blood clots), and treat any underlying causes.[nhs.uk]

References

Article

  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.

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Last updated: 2018-06-21 23:11