OBJECTIVETo study the value of the determination of serum and urine haptoglobin (HP) and alpha 1-antitrypsin (AAT) in predicting the response to glucocorticoid therapy in children with primary nephrotic syndrome (PNS).

METHODSA total of 84 children with PNS were classified to steroid-sensitive nephrotic syndrome (SSNS) (n=58) and steroid-resistant nephrotic syndrome (SRNS) groups (n=26). Forty healthy children were randomly selected for the control group. HP and AAT levels in blood and urinary samples were determined using ELISA. The efficiency of HP and AAT in predicting the response to glucocorticoid treatment of PNS was evaluated by the receiver operating characteristic (ROC) curve.

RESULTSCompared with the control group, both the SSNS and SRNS groups had significantly higher serum HP concentrations and urine AAT/Cr ratio before treatment (P<0.05); compared with the SSNS group, the SRNS group had significantly higher serum HP concentrations and urine AAT/Cr ratio before treatment and after one week and four weeks of treatment (P<0.05). Serum HP had the highest efficiency in predicting the response to glucocorticoid treatment of PNS at the concentration of 37.935 mg/mL, with the sensitivity and specificity being 92.3% and 86.2% respectively. Urine AAT/Cr ratio had the highest prediction efficiency at 0.0696, with the sensitivity and specificity being 100% and 79.3% respectively. ROC curve analysis of serum HP combined with urine AAT/Cr ratio showed a better prediction efficiency, with the sensitivity and specificity being 92.3% and 96.6% respectively.

CONCLUSIONSThe increase in serum HP level or urine AAT/Cr ratio may indicate glucocorticoid resistance in the early stage of PNS. A combination of the two can achieve better efficiency in the prediction of SRNS.

Child ; Child, Preschool ; Creatinine ; urine ; Female ; Glucocorticoids ; therapeutic use ; Haptoglobins ; analysis ; urine ; Humans ; Male ; Nephrotic Syndrome ; blood ; drug therapy ; urine ; alpha 1-Antitrypsin ; analysis ; blood ; urine

Glucocorticoids/urine* ; Humans ; Hydrocortisone ; Marathon Running ; Saliva/chemistry* ; alpha-Amylases/analysis*

OBJECTIVETo study the effect of Shenbing Mistura (SM) combined with glucocorticoid on recurrent nephrotic syndrome (RNS) in children and levels of interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-alpha) in blood and urine.

METHODSThe treatment group was treated with SM plus glucocorticoid, the control group with glucocorticoid alone, and a healthy control group was adopted, 30 cases in each group. The clinical effect and recurrence rate were observed, and levels of IL-6 and TNF-alpha in blood and urine were determined before treatment and at the 4th, 8th, 12th week after treatment.

RESULTSSignificant difference of IL-6 and TNF-alpha levels in blood and urine was found in either the pre- and post- treatment auto-control of both the treatment group and control group, or in the inter-group comparison of them (P < 0.01); clinical effect also showed remarkable difference between the two groups (P < 0.01). Furthermore, the recurrence rate of the treatment group was lower than that of the control group showed by a 18-month follow-up (P < 0.01).

CONCLUSIONSM combined with glucocorticoid could significantly reduce the recurrence rate and elevate the clinical effect in children with RNS, it could also lower the levels of IL-6 and TNF-alpha in patients' blood and urine.

Adult ; Child ; Child, Preschool ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Follow-Up Studies ; Glucocorticoids ; therapeutic use ; Humans ; Interleukin-6 ; analysis ; blood ; urine ; Male ; Nephrotic Syndrome ; drug therapy ; pathology ; Phytotherapy ; Prednisone ; therapeutic use ; Recurrence ; Treatment Outcome ; Tumor Necrosis Factor-alpha ; analysis ; blood ; urine

Congenital adrenal hyperplasia (CAH) is characterized by decreased adrenal hormone production due to enzymatic defects and subsequent rise of adrenocorticotrophic hormone that stimulates the adrenal cortex to become hyperplastic, and sometimes tumorous. As the pathophysiology is basically a defect in the biosynthesis of cortisol, one may not consider CAH in patients with hypercortisolism. We report a case of a 41-yr-old man with a 4 cm-sized left adrenal tumorous lesion mimicking Cushing's syndrome who was diagnosed with CAH. He had central obesity and acanthosis nigricans involving the axillae together with elevated 24-hr urine cortisol level, supporting the diagnosis of Cushing's syndrome. However, the 24-hr urine cortisol was suppressed by 95% with the low dose dexamethasone suppression test. CAH was suspected based on the history of precocious puberty, short stature and a profound suppression of cortisol production by dexamethasone. CAH was confirmed by a remarkably increased level of serum 17-hydroxyprogesterone level. Gene mutation analysis revealed a compound heterozygote mutation of CYP21A2 (I173N and R357W).
17-alpha-Hydroxyprogesterone/blood ; Acanthosis Nigricans/complications ; Adrenal Hyperplasia, Congenital/complications/*diagnosis/drug therapy ; Adult ; Cushing Syndrome/diagnosis ; DNA Mutational Analysis ; Dexamethasone/therapeutic use ; Glucocorticoids/therapeutic use ; Heterozygote ; Humans ; Hydrocortisone/urine ; Male ; Mutation ; Obesity/complications ; Steroid 21-Hydroxylase/genetics ; Tomography, X-Ray Computed