PMID- 20211026
OWN - NLM
STAT- MEDLINE
DCOM- 20101028
LR  - 20231105
IS  - 1824-7288 (Electronic)
IS  - 1720-8424 (Linking)
VI  - 36
DP  - 2010 Mar 8
TI  - Neonatal arterial iliac thrombosis in type-I protein C deficiency: a case report.
PG  - 23
LID - 10.1186/1824-7288-36-23 [doi]
AB  - A male infant born by caesarean section at 38 weeks of gestational age (B.W. 4055 
      g; Apgar 9-10), in the first two hours of life his right leg became 
      hypo-vascularized. Normal values of leukocytes, red cells, haematocrit, 
      hemoglobin, platelets. C-Reactive Protein negative. Electrolytes and coagulation 
      tests were normal. Normal vitamin K coagulation proteins levels. Serological 
      tests for TORCH (IgM) and Parvovirus (IgG and IgM) were negative. Sonography 
      showed a reduced blood flow in the iliac artery and reported a 1 cm long vessel 
      thrombosis. From 8 hours of life we administered an intravenous infusion of 
      unfractionated heparin (UFH) 75 UI/Kg for the first 10 minutes then 28 UI/Kg/h.On 
      the 2nd day tests were performed to assess absence of inhibiting-clot factors. 
      The dosage of homocysteine, protein S and antithrombin was normal. FV Leiden and 
      antiphospholipid antibodies were negative. The mapping of G20210A prothrombin's 
      gene resulted normal, whereas the concentration of Protein C was lower than 
      normal: activity 46% (68-150%), antigen 35% (70-150%).The same deficiency was 
      also found in the father. The mother showed normal concentrations. No episodes of 
      thrombosis events were documented in the family. The intravenous unfractionated 
      heparin (UFH) therapy was replaced after 64 hours by subcutaneous nadroparin 600 
      UI twice/day, which was stopped 5 days later when the vessel sonographic images 
      were completely normal. During the hospitalization the infant didn't show 
      bleeding. The child was followed-up yearly until 4 years of age: he was well and 
      had a normal body and mental development.The final diagnosis is likely to be of a 
      permanent protein C deficiency in heterozygous form. Our case is interesting 
      because the first manifestation was an important thrombosis of large vessel that 
      occurred within a few hours of life in absence of perinatal risk factors, as if 
      it was a homozygous disease, but the patient had a heterozygotic form. In 
      literature few cases are reported of heterozygous forms that became symptomatic, 
      but only in old age.After a severe first manifestation, a normal and asymptomatic 
      development is uncommon without new thrombotic episodes. In our patient the 
      neonatal thrombosis was the sole event in his life.
FAU - Tridapalli, Elisabetta
AU  - Tridapalli E
AD  - Department of Preventive Paediatrics and Neonatology-University of Bologna, 
      Policlinico S Orsola-Malpighi, Via Massarenti 11, 40138 Bologna, Italy. 
      trida@med.unibo.it
FAU - Stella, Marcello
AU  - Stella M
FAU - Capretti, Maria G
AU  - Capretti MG
FAU - Faldella, Giacomo
AU  - Faldella G
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20100308
PL  - England
TA  - Ital J Pediatr
JT  - Italian journal of pediatrics
JID - 101510759
RN  - 0 (Anticoagulants)
RN  - 0 (Protein C)
RN  - 9005-49-6 (Heparin)
SB  - IM
MH  - Anticoagulants/administration & dosage
MH  - Heparin/administration & dosage
MH  - Humans
MH  - *Iliac Artery
MH  - Infant, Newborn
MH  - Infusions, Intravenous
MH  - Male
MH  - Protein C/*metabolism
MH  - Protein C Deficiency/blood/*complications
MH  - Thrombosis/diagnostic imaging/drug therapy/*etiology
MH  - Ultrasonography
PMC - PMC2848059
EDAT- 2010/03/10 06:00
MHDA- 2010/03/10 06:01
PMCR- 2010/03/08
CRDT- 2010/03/10 06:00
PHST- 2009/09/23 00:00 [received]
PHST- 2010/03/08 00:00 [accepted]
PHST- 2010/03/10 06:00 [entrez]
PHST- 2010/03/10 06:00 [pubmed]
PHST- 2010/03/10 06:01 [medline]
PHST- 2010/03/08 00:00 [pmc-release]
AID - 1824-7288-36-23 [pii]
AID - 10.1186/1824-7288-36-23 [doi]
PST - epublish
SO  - Ital J Pediatr. 2010 Mar 8;36:23. doi: 10.1186/1824-7288-36-23.