Doppler sonographic indices in diagnosing the nutcracker phenomenon in a hematuric adolescent

Compression of the left renal vein between the aorta and the superior mesenteric artery, known as the nutcracker phenomenon, may cause gross or microscopic hematuria, pain in the flank, proteinuria, or a combination of these symptoms. We report the case of a hematuric adolescent diagnosed with a high index of suspicion by noninvasive Doppler sonography using the diagnostic indices of Doppler sonography established for adults with hematuria. Compression of the patient's left renal vein at the aortomesenteric portion and dilatation at the hilar portion were visualized by magnetic resonance angiography, which verified the diagnosis. (C) 2003 Wiley Periodicals, Inc

A partial response to abatacept in a patient with steroid resistant focal segmental glomerulosclerosis

Background. Herein we present our experience with abatacept in a patient diagnosed with primary focal segmental glomerulosclerosis (FSGS) and resistant to steroid and other immunosuppressives. Case. A 17-year-old girl was diagnosed with idiopathic nephrotic syndrome (NS) at the age of 8 years. Kidney biopsy was performed when she did not respond to 6-weeks of steroid (2mg/kg) therapy followed by three doses of pulse methylprednisolone (PMP) and considered as steroid resistant NS. The biopsy revealed focal segmental glomerulosclerosis (FSGS) and cyclophosphamide was added to the steroid treatment but the patient had no response. The genetic analysis revealed G34G/A318A compound homozygous synonym aminoacid variation in NPHS2 gene, thus all immunosuppressive regimes were stopped and she was put on supportive treatment. Throughout this period, she had nephrotic range of proteinuria, however serum albumin levels were >3g/dl. At the end of two years, the patient had NS with severe edema and hypoalbuminemia. When the genetic analysis was interpreted again, it was found to be consistent with a polymorphism rather than a mutation. Following 3 doses of PMP, oral steroid treatment was resumed and cyclosporine (CsA) was added to the treatment at the fifth year of follow up. However, she was unresponsive to CsA at the end of the first year as well as mycophenolate mofetil used for 12 months and rituximab used for 6 months, respectively. Then abatacept was instituted and proteinuria decreased below 1 gr/day and serum albumin levels increased to 3 g/dl at the end of 6 doses. Serum albumin levels remained stable in the following 7 months. Conclusion. Partial remission including the decrease in proteinuria and increase in albumin levels achieved in our patient encourages the usage of abatacept in patients who do not respond to multiple immunosuppressive therapies

Megacalycosis: report of two cases

Congenital megacalycosis is a rare renal disorder consisting of caliceal dilatation without evidence of renal pelvic or ureteral obstruction. Since its first description in 1963, only a few cases have been reported. The defect is mostly unilateral, shows male predominance and does not disrupt the renal functions. We report on two male patients with bilateral megacalycosis and different clinical presentations. The first case was diagnosed in adolescence during examination for a urinary tract infection and was followed-up uneventfully for nearly ten years. The second case was found to have megacalycosis during the assessment of antenatal hydronephrosis. We aimed to emphasize the importance of considering megacalycosis in the differential diagnosis of both antenatal hydronephrosis and other causes of caliceal dilatation to prevent unnecessary diagnostic and therapeutic interventions

A new recommendation for febrile urinary tract infection in children aged 2-24 months: Tepecik UTI Guideline-2

AIM: Urinary tract infections (UTIs) represent a common febrile illness in infancy. The study compared two UTI guidelines in terms of number of imaging studies, presence of parenchymal damage and radiation exposure in patients with the first febrile UTI between 2 and 24 months of age. METHOD: The results of Tepecik UTI Guideline-1 used until 2012 (Group 1, n = 105) were retrospectively compared with Tepecik UTI Guideline-2 (Group 2) used after 2013. In Group 1, urinary tract ultrasonography (US), dimercaptosuccinic acid (DMSA) and voiding cystourethrography (VCUG) were made in all patients. In Group 2, if the US result was abnormal, patients were evaluated with VCUG and DMSA. If the US was normal, only DMSA was performed. If the DMSA was abnormal, the VCUG was undergone (n: 43, 40.9%). RESULTS: The abnormal VCUG detection rate was 69.2% in Group 1 and 30.8% in Group 2 (p = 0.09). Sensitivity and specificity of US in the diagnosis of vesicoureteral reflux (VUR) was 15.9% and 96.7% in Group 1 and 61.5% and 70.5% in Group 2, respectively. Abnormal DMSA findings were observed among 33.3% (Groups 1) and 66.7% (Groups 2) subjects, respectively (p > 0.05). The median radiation exposure (500 mrem) of patients in Group 1 was statistically significantly higher than those in Group 2 (200 mrem) (p < 0.001). CONCLUSION: The VCUG should not be the first examination to be considered in such patients. We think that Tepecik UTI Guideline-2 reduces unnecessary invasive procedure and radiation exposure and not missed VUR in the management of children with UTI at 2-24 months. Needs prospective follow-up studies before considering this recommendation