Negative pressure dressing for promoting wound healing of purpura fulminans in a newborn with homozygous protein C deficiency.

A negative pressure dressing to promote wound healing of purpura fulminans in a girl aged 35 days with homozygous protein C deficiency is reported. Two wounds of 11 x 11 cm2 at the abdominal wall and 14 x 14 cm2 at the left trunk were covered with sterile sponges embedded with a multiple-hole drain tube and transparent plastic film. The exposed end of the drain was then connected to the wall suction apparatus to create negative pressure at -120 mmHg. The dressing was changed every 2 days. Within 4-6 weeks, the wounds were completely healed and skin grafting was not required.     

Antitubercular cassane furanoditerpenoids from the roots of Caesalpinia pulcherrima

Activity-guided fractionation of a root extract of Caesalpinia pulcherrima led to the isolation of two cassane-furanoditerpenoids, 6 beta-benzoyl-7 beta-hydroxyvouacapen-5 alpha-ol (1) and 6 beta-cinnamoyl-7beta-hydroxyvouacapen-5 alpha-ol (2). Compound 2 showed strong antitubercular activity with a minimum inhibitory concentration (MIC) of 6.25 microg/mL, whereas the benzoyl analogue (1) was less active (MIC 25 microg/mL). Both compounds expressed moderate cytotoxic activity towards KB (human oral carcinonoid cancer), BC (human breast cancer) and NCl-H187 (small cell lung cancer) cell lines.     

Lipoprotein(a) and the risk of thromboembolism in Thai children

High lipoprotein(a) [Lp(a)] level was identified as a risk factor of both venous and arterial thromboembolism (TE), especially in Caucasian children. The Lp(a) level is affected by apo(a) gene. The genetic polymorphisms that associated with Lp(a) level are the size of apo(a) gene, pentanucleotide repeat (TTTTA )_n and + 93 C/T at promoter region. The increasing size of apo(a) gene, more than 8 pentanucleotide repeats and + 93 C > T polymorphisms are associated with low level of Lp(a) in African and Caucasian populations. This cross - sectional, case control study, aims to identify the association of Lp(a) level and the risk for TE in Thai children. Forty-nine patients and 116 healthy children were enrolled. Mean ± SD for age of patients and controls were 7.6 ± 4.7 and 11.2 ± 1.7 years, with female:male ratios of 1:1.2 and 1.8:1, respectively. The median Lp(a) levels in patients was 8.2 (0-87.3) mg/dL and 7.9 (0-74.9) mg/dL in controls, which were not statistically different, P = 0.65. The frequencies of 8 pentanucleotide repeats and + 93 C/T were different compared to Caucasian and African populations but similar to Chinese population. However, both polymorphisms did not affect the level of Lp(a).     

The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate.

We describe a 10-year-old severe hemophilia B boy with a stop codon mutation of exon 2 in the factor IX gene who developed high inhibitor of 70 Bethesda units (BU) from 12 months of age after exposure to prothrombin complex concentrate for 14 days. The inhibitor spontaneously disappeared within 3 months. The patient, however, exhibited anaphylactic reaction to the administration of prothrombin complex concentrate and factor IX concentrate at ages 15 and 23 months, respectively. Although recombinant activated factor VII was alternatively given, he suffered from progressive hemophilic arthropathy. At the age of 10 years, the boy underwent desensitization to factor IX concentrate and could tolerate factor IX concentrate of 40 U/kg administered on day 9 of desensitization. Unfortunately, the inhibitor of 16 BU was detected on day 6 and rapidly increased to 180 BU on day 9 of desensitization. Rituximab 375 mg/m2 per week was therefore immediately initiated on day 10 and a total of four doses were given. The inhibitor gradually decreased to 21.5 BU after the fourth dose of rituximab. The daily factor IX concentrate administration of 40 U/kg was continued for 1 month and decreased to three times per week for another month, and then to once to twice per week for the remaining 14 months of desensitization. The patient was able to attend regular school and the most recent inhibitor ranged from 4.4 to 10 BU. No proteinuria or alteration of renal function was found. In conclusion, rituximab is a helpful adjuvant to immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate.     

Intramuscular anti‐D in chronic immune thrombocytopenia children with severe thrombocytopenia

Nine patients with chronic immune thrombocytopenia and platelet counts <20 × 10⁹/L, with a median age of 7.8 (3.8–15.5) years, received three phases of 10 mcg/kg/dose of intramuscular anti‐D. Phase 1 was anti‐D daily for 5 days, followed by phase 2, anti‐D weekly for 12 weeks and withheld when platelet counts ≥20 × 10⁹/L, and then phase 3 was anti‐D once every 2 weeks for 24 weeks. According to the International Working Group criteria, in phase 1, 66.7% of patients responded to the treatment. In phases 2 and 3, 11.1% (0–41.7%) and 7.7% (0–33.3%) of total episodes of follow up, respectively, responded to the treatment. Therefore, intramuscular anti‐D given at a dose of 10 mcg/kg for 5 days is an alternative method to raise platelet counts in chronic immune thrombocytopenia children with severe thrombocytopenia where the intravenous form of anti‐D is not available.