We have recently encountered a case of Kasabach-Merritt syndrome which was characterized by hemangioma, thrombocytopenia, and Disseminated Intravascular Coagulation. The baby was delivered at 35 weeks gestation. A large hemangioma covered right forearm. He received interferon alfa-2a for 120 days after 20 day steroid treatment failure. Blood platelet count increased to acceptable range (>20,000/mm3) after 8weeks of interferon alfa-2alpha treatment and the size of hemangioma decreased.
Disseminated Intravascular Coagulation ; Forearm ; Hemangioma ; Interferons* ; Kasabach-Merritt Syndrome* ; Platelet Count ; Pregnancy ; Thrombocytopenia ; Treatment Failure

Small patella syndrome (SPS) is characterized by aplasia or hypoplasia of the patella and pelvic girdle abnormalities, including bilateral absence or delayed ossification of the ischiopubic junction and infra-acetabular axe-cut notches. Here, we report a case of SPS in a 26-year-old female. Magnetic resonance image (MRI) showed a small patella with thick eccentric non-ossified patellar cartilage and femoral trochlear dysplasia with hypoplastic patellar undersurface. To our knowledge, this is the first report of MRI findings in SPS. MRI findings could be clinically relevant because elongation of the medial patellofemoral ligament and trochlear dysplasia with eccentric non-ossified patellar cartilage might lead to patellofemoral maltracking with an osteochondral lesion or acute dislocation or an extensor mechanism injury. Though the patient presented in this case report only had a gastrocnemius injury at the origin site, physicians should carefully examine abnormalities with MRI when an SPS patient has a trauma to the knee.
Adult ; Cartilage ; Dislocations ; Female ; Humans ; Knee ; Ligaments ; Magnetic Resonance Imaging ; Patella

OBJECTIVE: To determine whether ACE insertion/deletion (I/D) polymorphism is associated with the ossification of the posterior longitudinal ligament (OPLL) of the spine in the Korean population. METHODS: A case-control study was conducted to investigate the association between I/D polymorphism of the angiotensin I converting enzyme (peptidyl-dipeptidase A) 1 (ACE) gene and OPLL. The 95 OPLL patients and 274 control subjects were recruited. Polymerase chain reaction for the genotyping of ACE I/D polymorphism was performed. The difference between the OPLL patients and the control subjects was compared using the contingency chi2 test and the logistic regression analysis. For statistical analysis, SPSS, SNPStats, SNPAnalyzer, and Helixtree programs were used. RESULTS: The genotype and allele frequencies of ACE I/D polymorphism showed significant differences between the OPLL patients and the control subjects (genotype, p<0.001; allele, p=0.009). The frequencies of D/D genotype and D allele in the OPLL group were higher than those in the control group. In logistic regression analysis, ACE I/D polymorphism was associated with OPLL (dominant model; p=0.002; odd ratio, 2.20; 95% confidence interval, 1.33-3.65). CONCLUSION: These results suggest that the deletion polymorphism of the ACE gene may be a risk factor for the development of OPLL in the Korean population.
Alleles ; Angiotensin I* ; Angiotensins* ; Case-Control Studies ; Gene Frequency ; Genotype ; Humans ; Logistic Models ; Longitudinal Ligaments* ; Peptidyl-Dipeptidase A* ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Risk Factors ; Spine