Percutaneous ultrasound-guided renal biopsy in children: a single centre experience.

BACKGROUND AND AIM: The contribution of renal biopsy is of major importance in many renal diseases in children. In our study we aimed to evaluate retrospectively the indications, safety, efficacy and the spectrum of histopathological findings of percutaneous ultrasound-guided renal (PRB) biopsy during a 7 year period as well as to analyze specific groups of renal patients. PATIENTS AND METHODS: A total of 84 renal biopsies were performed in 81 children. Demographic data, clinical symptoms at presentation, indications for renal biopsy, laboratory findings, complications of the procedure and histological diagnosis were obtained from all patients who underwent PRB. RESULTS: The commonest indication for biopsy accounted was steroid resistant, steroid dependent or frequent relapsing idiopathic nephrotic syndrome (INS). Subcapsular hematoma presented 11% of the patients, but none of them needed blood transfusion. Adequate renal tissue sample was obtained in 97.7% of the renal biopsies. In 80% the histopathology revealed glomerular diseases. The most frequent types of biopsy-proven renal diseases were: focal segmental glomerulosclerosis (FSGS) (15%), IgA nephropathy (13.5%), minimal change disease (10%), various stages of lupus nephritis (8.5%), Henoch-Schonlein nephritis (7.5%), membranous glomerulonephritis (7.5%), mesangioproliferative glomerulonephritis (6%), post-infectious glomerulonephritis (6%), hemolytic uremic syndrome (5%), tubulointerstitial nephropathies (3.5%), acute tubular necrosis 2.5%. Among the 28 cases of INS, FSGS accounted for 43%. The leading histopathological pattern found in patients with recurrent episodes of gross haematuria was IgAN (84.5%). Among 7 cases of lupus nephritis, the observed histological types were: IV+V in 3/7, IIIA in 3/7 (43%) and IIB in 1/7. CONCLUSIONS: Our study shows that percutaneous ultrasound-guided renal biopsy is a safe, reliable and effective technique in children. It also provides updated information for childhood renal disease pattern.

Safety and efficacy of adalimumab treatment in Greek children with juvenile idiopathic arthritis.

OBJECTIVES: To evaluate the safety and efficacy of adalimumab (AD) administration in patients with juvenile idiopathic arthritis (JIA). METHODS: Twenty-six patients were enrolled from January 2004 to January 2008 in this prospective observational study. Inclusion criteria were either unresponsiveness to disease-modifying anti-rheumatic drugs (DMARDs; n = 17) or to other anti-tumour necrosis factor (anti-TNF) agents (n = 9) or development of uveitis under other anti-TNFs (n = 2 of the 9). Efficacy was estimated using the American College of Rheumatology Pediatric (ACR Pedi) criteria. RESULTS: After 1-5 years of AD exposure, nine different adverse events (AEs) were recorded (12.6 AEs/100 patient-years), mainly mild respiratory tract infections and injection site-related reactions. Serious AEs (SAEs, 2.8/100 patient-years) were the development of abscess at the site of injection (n = 1) and lethal sepsis (n = 1). The ACR Pedi ≥ 30 responses for the first to the fifth year of treatment were 88.5, 57.7, 50.0, 34.6, and 11.5%, respectively. In total, 17 of the 26 (65.4%) patients responded to AD. Five of the 11 patients under steroids discontinued them 6 months post-treatment. Seven patients required weekly AD treatment to maintain remission and four of them benefited from this policy. Recurrent uveitis was hindered in three of the six patients, no new cases were recorded, and radiological regression was observed in two of the four patients with lesions. CONCLUSIONS: AD was safe and efficacious during the study period in the majority of patients. However, vigilance is required for the early detection of severe and potentially fatal infections. AD may control recurrent uveitis and radiological progression.

A patient with Lemierre syndrome.

Lemierre syndrome, also known as postanginal sepsis, is a severe complication of an acute oropharyngeal infection that results in septic thrombophlebitis of the ipsilateral internal jugular vein with subsequent septicemia, often complicated by metastatic infections (Syed et al., Laryngoscope 117:1605-1610, 2007). We present the case of a previously healthy 12-year-old boy with Lemierre syndrome, caused by streptococci (Abiotrophia defectiva), complicating a subcutaneous neck abscess. The patient had metastatic sequelae, was treated with antibiotics (clindamycin and vancomycin) and low molecular weight heparin, and had an uneventful outcome.

Evaluation of pelvic ultrasonography in the diagnosis and differentiation of various forms of sexual precocity in girls.

OBJECTIVES: This study was conducted to assess the role of pelvic ultrasound variables in discriminating between normal girls and girls with different forms of sexual precocity, and to establish reliable cut-off limits of pelvic ultrasound measurements for differentiating between these conditions. METHODS: Eighty-eight girls with different forms of sexual precocity (23 with premature thelarche, 15 with premature pubarche and 50 with central precocious puberty) were enrolled. All diagnoses were based on clinical examination with confirmation using the gonadotropin-releasing hormone-stimulation test. Additionally, 81 prepubertal girls, aged 0-10 years, were included as a control group. For statistical analysis and to facilitate comparisons, the groups were subdivided by age intervals (0-6, > 6-8 and > 8-10 years). All subjects underwent pelvic ultrasound examination for the measurement of uterine length, uterine volume, ovarian volume and the anteroposterior diameter at the fundus divided by the anteroposterior diameter at the cervix (fundal/cervical ratio, (F/C)). Finally, the morphological appearance of the ovaries was assessed. RESULTS: Patients with central precocious puberty had significantly higher values for all the ultrasound variables (with the exception of uterine length in the 1-6-year age group) in comparison to normal girls. Patients with premature thelarche and patients with premature pubarche showed similar pelvic ultrasound parameters to those of normal girls. Ovarian volume was the best parameter for identifying patients with central precocious puberty (a cut-off of 3.04 cm(3) had a sensitivity of 100% and a specificity of 97.1% for age interval 0-6 years, a cut-off of 3.35 cm(3) had a sensitivity of 100% and a specificity of 89.5% for age interval > 6-8 years, and a cut-off of 4.46 cm(3) had a sensitivity of 80.8% and a specificity of 88.5% for age interval > 8-10 years). Uterine length was the best parameter for distinguishing between patients with central precocious puberty and patients with premature thelarche (a cut-off of 3.185 cm had a sensitivity of 85.7% and a specificity of 91.7% for age interval 0-6 years, and a cut-off of 3.83 cm had a sensitivity of 82.4% and a specificity of 90.9% for age interval > 6-8 years). CONCLUSIONS: Ultrasound examination of the uterus and ovaries could serve as a complementary tool for the diagnosis of central precocious puberty and, consequently, for the early initiation of appropriate treatment.

Serial magnetic resonance imaging findings in mucopolysaccharidosis IIIB (Sanfilippo's syndrome B).

Sanfillippo B syndrome (mucopolysaccharidosis (MPS) III, type B) is characterized by mild expression of the characteristic 'Hurler' phenotype and a severe central nervous system involvement. We report three patients with Sanfilippo B syndrome, referred to our clinic because of peculiar facies, delay in language development and behavioral problems, at the ages of 4, 3 and 5 years, respectively. At presentation they manifested clinical features of MPS, severe developmental retardation, radiological features of dysostosis mutiplex, as well as neurophysiological findings suggestive of carpal tunnel syndrome and sensorineural hearing impairment. Due to marked urinary excretion of heparan sulfate, as well as deficiency of alpha-N-acetylglucosaminidase in leukocytes, the diagnosis of Sanfilippo B syndrome was made. Serial brain magnetic resonance imaging (MRI) at different ages demonstrated white matter abnormalities, cortical atrophy and ventricular enlargement in all three patients, while other findings included thickening of the diploe in two patients and callosal atrophy, basal ganglia involvement, cerebellar changes and dilatation of venous sinuses in one patient. Although the combination of the above MRI findings is highly suggestive of a MPS, they carry a little predictive value in the different clinical stages of MPS IIIB.

Incidence of ceftriaxone-associated gallbladder pseudolithiasis.

We prospectively evaluated the incidence of gallbladder pseudolithiasis in children treated with high doses of ceftriaxone for a variety of serious infections. We also monitored the time interval needed for this phenomenon to develop and resolve completely after initiation and cessation of treatment, respectively. Included in this study are 44 children treated with ceftriaxone 100 mg/kg/d divided into 2 equal intravenous doses and followed by serial abdominal sonography. Eleven children developed pseudolithiasis of gallbladder 2-9 d after initiation of ceftriaxone therapy. Six children (54.5%) developed this complication within the first 3 d. Lithiasis completely resolved 8-23 d after the end of treatment. In conclusion, pseudolithiasis of the gallbladder developed in 25% of sick children and completely resolved in all patients. Early development of this complication was not exceptional. It occurred in more than half of these children.

Conservative treatment of hemangiomas in infancy and childhood with interferon-alpha 2a.

Hemangioma is the most common tumor of infancy. Most hemangiomas proliferate for 8 to 18 months and then slowly regress over the next 5 to 8 years. So far, steroids have been the established means of treatment, but there have been some reports of the use of interferon-alpha 2a (IFN-A2a) in such tumors. To evaluate the efficacy of IFN-A2a in treating giant hemangiomas in infancy and childhood, we administered it to ten patients aged 4 h to 6.5 years with life-threatening hemangiomas in various parts of the body. The dose used was 3 MU/m2 body surface area subcutaneously every second day. All children had previously received corticosteroids for 2 to 5 weeks without obvious success. The results were satisfactory: five patients had total or almost total regression; the rest have had partial regression while treatment is still in progress. A flu-like syndrome with fever, rhinitis, etc. accompanied the injections in some patients. The symptoms were controlled with paracetamol as necessary. Mild, transient leukopenia occurred in some children as well, but subsided after cessation of therapy for 1 week. IFN-A2a appears to be a promising and well-tolerated element in the treatment of life-threatening hemangiomas in infancy and childhood.

Late postoperative results in males treated for testicular torsion during childhood.

Successful preservation of a testis that has undergone torsion is directly related to the duration and degree of torsion. From 1979 to 1991, 75 cases of testicular torsion were treated. Eleven orchiectomies were performed; operative detorsion was done in the other 64 cases, with concurrent orchiopexy of both testicles. Twenty-five patients were reexamined 1 to 12 years after the surgery. Age of torsion, duration of symptoms, and operative findings were reevaluated. Testicular volume was determined using Prader's orchidometer, a pachymeter, and ultrasonography. In four postpubertal patients, semen analysis was performed. According to clinical data, results of testicular atrophy correlated with duration of symptoms and operative findings. In all cases of surgical detorsion in which torsion lasted more than 24 hours and viability of the testis was questionable, subsequent atrophy was the rule.