A Chinese Boy With Lupus Anticoagulant-hypoprothrombinemia Syndrome: A Case Report and Review of the Literature.

We herein report an unusual case of lupus with bleeding diathesis in a Chinese adolescent boy. In the presence of lupus anticoagulant and hypoprothrombinemia, the diagnosis of lupus anticoagulant-hypoprothrombinemia syndrome was made. He responded promptly to immunosuppressive agents and achieved disease remission.

Measuring Erythrocyte Thiopurine Methyltransferase Activity in Children-Is It Helpful?

OBJECTIVE: To assess the profile of thiopurine transmethyltransferase (TPMT) enzyme activities in children and discuss the utility of measuring TPMT levels before commencing azathioprine therapy. STUDY DESIGN: Retrospective study in a single pediatric center of all patients who had TPMT enzyme assay measured during a 3-year period before the start of azathioprine therapy. Patients' TPMT enzyme activities were classified as normal (26-50 pmol/h/mgHb), intermediate (10-25 pmol/h/mgHb), and deficient (<10 pmol/h/mgHb). Medical and electronic prescribing records were studied, with records of patients' demographic data, diagnoses, dosages, and adverse drug reactions monitored. RESULTS: A total of 228 (45% female) patients ages 1-18 years (median 10 years) were tested for TPMT activity. They were all subsequently commenced on azathioprine. Erythrocyte TPMT activities were 14-76 (median 33.7) pmol/h/mgHb with 88% and 12% of patient having normal and intermediate activity respectively (none were deficient). The initial prescribed dosage of azathioprine was 0.7-3.5 (median 2.0) mg/kg/day. Only 2 patients developed mild neutropenia and required reduction of azathioprine dosage. CONCLUSION: This large cohort study demonstrates that the majority of pediatric patients had normal TMPT activities. We would recommend close monitoring of full blood counts after instituting azathioprine therapy but would question the cost-effectiveness of pretreatment TPMT activity in pediatric practice.

Risk factors for post-operative complications in Chinese children with tonsillectomy and adenoidectomy for obstructive sleep apnea syndrome.

PURPOSE: Tonsillectomy and adenoidectomy (T&A) is commonly performed in children with obstructive apnea syndrome (OSAS). It was our hospital practice to observe all patients post T&A in the pediatric intensive care unit. We aim to describe the post-operative complications after tonsillectomy and adenoidectomy in children with OSAS and to identify risk factors for these complications. METHOD: Medical records of patients from 1 to 16 years old with OSAS and T&A done in this department were retrieved for analysis from April 1999 to July 2006. Information of the individual patients including the demographic data, polysomnography data, and presence of post-operative complications were recorded and analyzed. RESULTS: A total of 86 patient records were analyzed (M/F = 69:17). The mean BMI z score was 1.13 ± 1.53, and 36% of patients were classified as obese with z > 1.96. The median apnea-hypopnea index (AHI) before T&A was 9.8 episodes/h. Only six patients had post-operative desaturation. No bleeding complications were reported in our cohort. It was found that patients with desaturation after T&A had significantly higher mean BMI z score than children without desaturation (p = 0.014). There was otherwise no significant difference between the age, sex, AHI score, and the history of allergic rhinitis or asthma between the two groups. CONCLUSION: Our results showed that most children with OSAS underwent T&A without complications. The respiratory complication rate was 7%, and desaturation was the most common post-operative complication. Children with higher BMI z score were more likely to have desaturation after T&A (p = 0.014). Hence, careful monitoring with pulse oximeter after T&A should be offered to those who are obese.