Activated Hedgehog-GLI Signaling Causes Congenital Ureteropelvic Junction Obstruction

Renal osteodystrophy secondary to congenital bilateral ureteropelvic junction obstruction.

A 4-year-old girl presented with progressive distension of the abdomen and bowing of the legs (genu valgum) since birth. She was anemic, with a blood pressure of 180/110 mmHg. The serum level of creatinine was 1.7 mg/dL. She had hypocalcemia, and elevated serum level of phosphorous and parathyroid hormones. Ultrasonography revealed bilateral grossly hydronephrotic kidneys with thinned out paren...

Congenital Ureteropelvic Junction Stenosis

Introduction Congenital ureteropelvic junction (UPJ) stenosis is a disturbance in the flow of urine at the crossover point from the kidney pelvis to the ureter, with a dilatated and obstructed renal-pelvic calyceal system (Figure 89.1). UPJ obstruction is the most frequent congenital malformation of the genitourinary system. Its incidence is around 1 out of 1,000 newborns. The first sonographic...

Elevated Hedgehog/Gli signaling causes β-cell dedifferentiation in mice

Although Hedgehog (Hh) signaling regulates cell differentiation during pancreas organogenesis, the consequences of pathway upregulation in adult β-cells in vivo have not been investigated. Here, we elevate Hh signaling in β-cells by expressing an active version of the GLI2 transcription factor, a mediator of the Hh pathway, in β-cells that are also devoid of primary cilia, a critical regulator ...

Editorial: Ureteropelvic Junction Obstruction (UPJO) in Infants

A Rare Cause of Ureteropelvic Junction Obstruction

An 8-year-old boy was referred to Pusan National University Yangsan Hospital with left ureteropelvic junction (UPJ) obstruction. He had experienced episodes of intermittent gross hematuria that had first presented at the age of 5 years. He was otherwise healthy and had no previous medical problems. He had no history of urinary tract infections or stones. A physical examination showed no left fl...