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Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series

Standard

Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series. / Lehmberg, K; Hassenpflug, W A; Klaassen, I; Hillebrand, G; Oyen, F; Budde, U; Singer, D; Schneppenheim, R.

In: Z GEBURTSH NEONATOL, Vol. 221, No. 1, 02.2017, p. 39-42.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Lehmberg, K, Hassenpflug, WA, Klaassen, I, Hillebrand, G, Oyen, F, Budde, U, Singer, D & Schneppenheim, R 2017, 'Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series', Z GEBURTSH NEONATOL, vol. 221, no. 1, pp. 39-42. https://doi.org/10.1055/s-0042-109404

APA

Lehmberg, K., Hassenpflug, W. A., Klaassen, I., Hillebrand, G., Oyen, F., Budde, U., Singer, D., & Schneppenheim, R. (2017). Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series. Z GEBURTSH NEONATOL, 221(1), 39-42. https://doi.org/10.1055/s-0042-109404

Vancouver

Lehmberg K, Hassenpflug WA, Klaassen I, Hillebrand G, Oyen F, Budde U et al. Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series. Z GEBURTSH NEONATOL. 2017 Feb;221(1):39-42. https://doi.org/10.1055/s-0042-109404

Bibtex

@article{6745a0b3e27842728d62e7bf3f155e63,
title = "Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series",
abstract = "We report on 3 male neonates with hereditary ADAMTS13 deficiency (Upshaw Schulman syndrome, USS), the inherited form of thrombotic thrombocytopenic purpura (TTP). 2 presented shortly after birth with thrombocytopenia followed by microangiopathic Coombs-negative haemolytic anaemia. Both initially received antibiotic treatment for suspected infection-associated thrombocytopenia. In one patient's brother, the first bout of incipient TTP did not occur before 6 months of age, despite the same genetic defect. ADAMTS13 activity was<5%, compound heterozygous mutations were found in all patients. USS constitutes a differential diagnosis to thrombocytopenia caused by disseminated intravascular coagulation in neonatal septicaemia. Administration of fresh frozen plasma usually resolves acute bouts of the disease. In some cases of thrombocytopenia of unknown origin in infancy, the resolution of signs and symptoms after infusion of plasma may point towards the diagnosis.",
author = "K Lehmberg and Hassenpflug, {W A} and I Klaassen and G Hillebrand and F Oyen and U Budde and D Singer and R Schneppenheim",
note = "{\textcopyright} Georg Thieme Verlag KG Stuttgart · New York.",
year = "2017",
month = feb,
doi = "10.1055/s-0042-109404",
language = "English",
volume = "221",
pages = "39--42",
journal = "Z GEBURTSH NEONATOL",
issn = "0948-2393",
publisher = "Georg Thieme Verlag KG",
number = "1",

}

RIS

TY - JOUR

T1 - Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series

AU - Lehmberg, K

AU - Hassenpflug, W A

AU - Klaassen, I

AU - Hillebrand, G

AU - Oyen, F

AU - Budde, U

AU - Singer, D

AU - Schneppenheim, R

N1 - © Georg Thieme Verlag KG Stuttgart · New York.

PY - 2017/2

Y1 - 2017/2

N2 - We report on 3 male neonates with hereditary ADAMTS13 deficiency (Upshaw Schulman syndrome, USS), the inherited form of thrombotic thrombocytopenic purpura (TTP). 2 presented shortly after birth with thrombocytopenia followed by microangiopathic Coombs-negative haemolytic anaemia. Both initially received antibiotic treatment for suspected infection-associated thrombocytopenia. In one patient's brother, the first bout of incipient TTP did not occur before 6 months of age, despite the same genetic defect. ADAMTS13 activity was<5%, compound heterozygous mutations were found in all patients. USS constitutes a differential diagnosis to thrombocytopenia caused by disseminated intravascular coagulation in neonatal septicaemia. Administration of fresh frozen plasma usually resolves acute bouts of the disease. In some cases of thrombocytopenia of unknown origin in infancy, the resolution of signs and symptoms after infusion of plasma may point towards the diagnosis.

AB - We report on 3 male neonates with hereditary ADAMTS13 deficiency (Upshaw Schulman syndrome, USS), the inherited form of thrombotic thrombocytopenic purpura (TTP). 2 presented shortly after birth with thrombocytopenia followed by microangiopathic Coombs-negative haemolytic anaemia. Both initially received antibiotic treatment for suspected infection-associated thrombocytopenia. In one patient's brother, the first bout of incipient TTP did not occur before 6 months of age, despite the same genetic defect. ADAMTS13 activity was<5%, compound heterozygous mutations were found in all patients. USS constitutes a differential diagnosis to thrombocytopenia caused by disseminated intravascular coagulation in neonatal septicaemia. Administration of fresh frozen plasma usually resolves acute bouts of the disease. In some cases of thrombocytopenia of unknown origin in infancy, the resolution of signs and symptoms after infusion of plasma may point towards the diagnosis.

U2 - 10.1055/s-0042-109404

DO - 10.1055/s-0042-109404

M3 - SCORING: Journal article

C2 - 27383044

VL - 221

SP - 39

EP - 42

JO - Z GEBURTSH NEONATOL

JF - Z GEBURTSH NEONATOL

SN - 0948-2393

IS - 1

ER -