SummaryOBJECTIVE Desplte regular transfuslon and desferrioxamine treatment, growth fallure Is commonly seen In adolescent children wlth Pthalassaemla malor. The growth failure has been thought to be due to GH reslstance rather than GH deflclency. We Investigated the effect of GH on short non-GH deflclent chlldren wlth P thalassaemla. DESIGN Recomblnant human GH was given In a dose of 0.14 IUlkglday subcutaneously in an open study.PATIENTS Flfteen prepubertal Chlnese children with p thalassaemla malor (ranglng from 7.16 to 147 years In age) wlth height -1.5 SD or more below the population mean for age and a growth velocity of less than 5 cmlyear were treated with growth hormone for one year. All chlldren had peak GH response >lSmlU/I to lnsulln induced hypoglycaemla and normal thyroid functlon and adrenal reserve. MEASUREMENTS Anthropometric measurements were performed every 3 months. Morning urine was tested twlce weekly for glycosurla. Blood count, renal and liver function tests, fastlng blood glucose, IGF-l and fructosamine levels were assessed at entry and every 3 months during treatment. Fasting lnsulln was measured before and after 3 and 12 months of GH treatment. Skeletal maturlty was assessed before and after one year of treatment. RESULTS Treatment was stopped in two chlldren after 6 months because of poor growth response and noncompliance wlth treatment and In one child at 9 months because o f bone marrow transplantation. In the 13 chlldren, the growth velocity Increased from 3.6 * 0.7 cmlyear to
PurposeTo investigate the clinical effects of a single high dose intravenous immunoglobulin (IVIG) combined with initial dexamethasone as a primary treatment on Kawasaki disease (KD).Materials and MethodsBetween January 2008 and December 2010, we reviewed the medical records of 216 patients with complete KD patients that were admitted to a single medical center. 106 patients were treated with a single high dose of IVIG (2 g/kg) alone and 110 patients received IVIG and dexamethasone (0.3 mg/kg per day for three days).ResultsThe combined IVIG plus dexamethasone patient group had a significantly shorter febrile period and duration of hospital stay (1.4±0.7 days vs. 2.0±1.2 days, p<0.001; 5.8±1.7 days vs. 6.9±2.5 days, p<0.001, respectively) than the IVIG alone group. The combined IVIG plus dexamethasone group required IVIG retreatment significantly less than the IVIG only group (12.7% vs. 32%, p=0.003). After completion of the initial IVIG, C-reactive protein levels in the combined IVIG plus dexamethasone group were significantly lower than those in the IVIG only group (2.7±4.0 mg/dL vs. 4.6±8.7 mg/dL, p=0.03). In the combined IVIG plus dexamethasone group, the incidence of coronary artery lesions tended to be lower without worse outcomes at admission after initial infusion of IVIG and in follow-up at two months; however, the differences were not significant (8.2% vs. 11.3%, p=0.22; 0.9% vs. 2.8%, p=0.29).ConclusionInitial combined therapy with dexamethasone and a single high-dose of IVIG resulted in an improved clinical course, in particular a shorter febrile period, less IVIG retreatment, and shorter hospital stay without worse coronary outcomes.
We report two cases of 'familial testotoxicosis' in a family of Southern Chinese descent. The proband, an 8-year 4-month-old boy and his 35-year-old father both presented with early sexual development. In both cases the testicular volume was only 6 ml despite fully developed secondary sexual characteristics. Both patients had adult testosterone concentrations but a suppressed gonadotrophin response to gonadotrophin-releasing hormone. The suppressed gonadotrophin response to gonadotrophin-releasing hormone in the father suggests that autonomous gonadal production of sex steroid by the testes can persist well into adult life in some patients with familial testotoxicosis.
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