Transient Myeloproliferative Disorder Associated with Trisomy 21

2007 ◽  
Vol 26 (1) ◽  
pp. 7-19 ◽  
Author(s):  
Branda Kruger
Keyword(s):  
Trisomy 21 ◽  
Case Reports ◽  
Myeloproliferative Disorder ◽  
Neonatal Nurses ◽  
Transient Myeloproliferative Disorder ◽  
Congenital Leukemia ◽  
Life Threatening ◽  
Dermatologic Manifestations

Transient myeloproliferative disorder (TMD) is a spontaneously resolving condition affecting infants born with trisomy 21 syndrome. Although TMD is rather rare among infants with trisomy 21, its ramifications can become severe enough that neonatal nurses should be aware of the condition, its manifestations, and its management. The spectrum of TMD presentation ranges from subtle blastemia in an otherwise healthy infant to severe, life-threatening expression of the disease. TMD may be a precursor to congenital leukemia—thus, the importance of nurses’ becoming aware of this condition. This article addresses the pathology of TMD, case reports in the literature, potential complications of the disorder, and nursing implications. A case study of an infant with dermatologic manifestations of TMD is presented, including history, differential diagnoses, treatment, and follow-up.

scholarly journals Natural history of transient myeloproliferative disorder clinically diagnosed in Down syndrome neonates: a report from the Children's Oncology Group Study A2971

Blood ◽  
2011 ◽  
Vol 118 (26) ◽  
pp. 6752-6759 ◽  
Author(s):  
Alan S. Gamis ◽  
Todd A. Alonzo ◽  
Robert B. Gerbing ◽  
Joanne M. Hilden ◽  
April D. Sorrell ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Natural History ◽  
Trisomy 21 ◽  
Myeloid Leukemia ◽  
Clinical Findings ◽  
Myeloproliferative Disorder ◽  
Spontaneous Resolution ◽  
Transient Myeloproliferative Disorder ◽  
Life Threatening ◽  
History Of

Abstract Transient myeloproliferative disorder (TMD), restricted to newborns with trisomy 21, is a megakaryocytic leukemia that although lethal in some is distinguished by its spontaneous resolution. Later development of acute myeloid leukemia (AML) occurs in some. Prospective enrollment (n = 135) elucidated the natural history in Down syndrome (DS) patients diagnosed with TMD via the use of uniform monitoring and intervention guidelines. Prevalent at diagnosis were leukocytosis, peripheral blast exceeding marrow blast percentage, and hepatomegaly. Among those with life-threatening symptoms, most (n = 29/38; 76%) received intervention therapy until symptoms abated and then were monitored similarly. Organomegaly with cardiopulmonary compromise most frequently led to intervention (43%). Death occurred in 21% but only 10% were attributable to TMD (intervention vs observation patients: 13/14 vs 1/15 because of TMD). Among those solely observed, peripheral blasts and all other TMD symptoms cleared at a median of 36 and 49 days from diagnosis, respectively. On the basis of the diagnostic clinical findings of hepatomegaly with or without life-threatening symptoms, 3 groups were identified with differing survival: low risk with neither finding (38%), intermediate risk with hepatomegaly alone (40%), and high risk with both (21%; overall survival: 92% ± 8%, 77% ± 12%, and 51% ± 19%, respectively; P ≤ .001). Among all, AML subsequently occurred in 16% at a median of 441 days (range, 118-1085 days). The trial is registered at http://www.clinicaltrials.gov as NCT00003593.

scholarly journals TRANSIENT MYELOPROLIFERATIVE DISORDER: A POINTER TO UNDERLYING TRISOMY 21

2019 ◽  
Vol 6 (6) ◽  
pp. 335-337
Author(s):  
Sharan Subramanian ◽  
Sushma Malik ◽  
Purvi Kadakia Kutty ◽  
Poonam Abhay Wade
Keyword(s):  
Trisomy 21 ◽  
Myeloproliferative Disorder ◽  
Transient Myeloproliferative Disorder

scholarly journals Amoxicillin Induced Steven Johnson’s Syndrome: A Case Report

2020 ◽  
Vol 10 (4-s) ◽  
pp. 220-222
Author(s):  
R Mahendra Kumar ◽  
Sanatkumar Nyamagoud ◽  
Krishna Deshpande ◽  
Ankitha Kotian
Keyword(s):  
Adverse Drug Reaction ◽  
Drug Reaction ◽  
Case Reports ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Hypersensitivity Reactions ◽  
Johnson Syndrome ◽  
Oral Consumption ◽  
Life Threatening

Stevens-Johnson syndrome (SJS) is a very rare, potentially fatal skin reaction that is typically the result of reaction to the drug. In particular, SJS is characterized by extensive skin and mucous membrane lesions (i.e. mouth, nose, esophagus, anus, and genitalia), epidermis detachment, and acute skin blisters. In 95 % of case reports, drugs were found to be an important cause for the development of SJS. This story is a case of A 42 year old male hospitalized with rashes all over the body and fever, after oral consumption of Amoxicillin drug for sore throat. This case study discusses the possibility that serious hypersensitivity reactions with Amoxicillin can rarely occur and can be extremely harmful and life-threatening Menacing. Keywords: Toxic Epidermal Necrolysis, Stevens Johnson Syndrome, Adverse drug reaction, Nikolsky’s sign

Transient myeloproliferative disorder with partial trisomy 21

2015 ◽  
Vol 62 (11) ◽  
pp. 2021-2024 ◽  
Author(s):  
Takahide Takahashi ◽  
Akira Inoue ◽  
Junko Yoshimoto ◽  
Kiichiro Kanamitsu ◽  
Tomohiko Taki ◽  
...  
Keyword(s):  
Trisomy 21 ◽  
Partial Trisomy ◽  
Myeloproliferative Disorder ◽  
Transient Myeloproliferative Disorder

Vesiculopustular Eruption in Neonate with Trisomy 21 and Transient Myeloproliferative Disorder

2013 ◽  
Vol 162 (3) ◽  
pp. 643-644 ◽  
Author(s):  
Noriyuki Iwashita ◽  
Chieko Sadahira ◽  
Yuki Yuza ◽  
Hiroshi Yoshihashi ◽  
Masatoshi Kondou
Keyword(s):  
Trisomy 21 ◽  
Myeloproliferative Disorder ◽  
Transient Myeloproliferative Disorder

482 PRENATAL HEPATOSPLENOMEGALY WITH TRANSIENT MYELOPROLIFERATIVE DISORDER OF TRISOMY 21.

2004 ◽  
Vol 52 (Suppl 1) ◽  
pp. S163.5-S163
Author(s):  
M. Steinraths ◽  
D. Pugash ◽  
K. Lim ◽  
M. I. Van Allen
Keyword(s):  
Trisomy 21 ◽  
Myeloproliferative Disorder ◽  
Transient Myeloproliferative Disorder

Sonographer’s Role in the Screening, Diagnosis, and Treatment of Dialysis Fistula Pseudoaneurysms

2020 ◽  
Vol 36 (4) ◽  
pp. 357-363
Author(s):  
Ashley Gaspard
Keyword(s):  
Diagnosis And Treatment ◽  
Dialysis Access ◽  
Dialysis Patients ◽  
Access Site ◽  
Spectral Doppler ◽  
Life Threatening ◽  
Dialysis Fistula ◽  
Dependent Patient

Pseudoaneurysm (PSA) formation, in an arteriovenous fistula (AVF) undergoing hemodialysis, can occur as a result of frequent venipunctures and can lead to a weakened vessel wall. Since many dialysis patients’ lives depend on their weekly hemodialysis sessions, complications that result from dialysis require treatment. A PSA can be a life-threatening complication, especially for these patients. Sonography has been demonstrated to be a useful modality of choice in the screening, diagnosis, and treatment of a PSA. The sonographic appearance of a PSA using gray-scale, color, and spectral Doppler can play a significant role in ruling out other diagnoses. A case study is presented of a dialysis-dependent patient who developed a PSA in his dialysis access site. Sonography played a key role in the diagnosis and follow-up for this specific case. Due to the risks involved with these patients, there is an essential need for sonographers to be competent in performing these examinations. Evaluation of the AVF to check for patency and a thorough assessment of the PSA are required.

Fifteen-minute consultation: The review of a child with trisomy 21 (Down’s syndrome)

2021 ◽  
pp. edpract-2020-319814 ◽  
Author(s):  
Rebecca Amy Dalrymple ◽  
Laura Helen Somerville ◽  
Sherin Hamza ◽  
Nashwa Matta
Keyword(s):  
Intellectual Disability ◽  
Chromosomal Abnormality ◽  
Trisomy 21 ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Many Body ◽  
Life Threatening ◽  
Many Body Systems

Down’s syndrome (DS) is the most common chromosomal abnormality seen in live born children and it is the most common genetic cause of intellectual disability. It is associated with abnormalities in many body systems, some of which can cause life threatening complications. This article aims to cover the important aspects to cover when seeing children with DS for their routine follow-up in the neurodevelopmental or general paediatric clinic.

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