11/120. A new syndrome with craniofacial and skeletal dysmorphisms and developmental delay.We report a 16-year-old boy with multiple craniofacial and skeletal dysmorphic features including brachycephaly, acrocephaly, hypertelorism, wide palpebral fissures, broad nose, anteverted nares, broad columella, long and smooth philtrum, thin upper lip, macrostomia, carp-like mouth, micrognathia, low-set and posteriorly angulated ears with small and abnormal pinnae, a low posterior hairline, a short neck, hypoplastic and widely-spaced nipples, multiple severe pterygia, an umbilical hernia, metatarsus varus, low implantation of the halluces, and delayed motor and language development. An MRI of the head showed bilateral frontal pachygyria but no sign of heterotopia. The unique features of our patient suggest that he represents a new syndrome.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
12/120. Posterior urethral valves presented at birth despite normal prenatal ultrasound scans.Most patients with significant posterior urethral valves are identified antenatally. However, even with a normal antenatal ultrasound scan, posterior urethral valves may be detected after birth. We present a patient with significant upper urinary tract involvement identified 2 days after birth, despite a normal third-trimester antenatal ultrasound scan.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
13/120. erdheim-chester disease.erdheim-chester disease (ECD) is a rare histiocytic infiltrative disease, characterized by long bone sclerosis.We describe two confirmed cases of ECD, both of which demonstrate non-malignant retroperitoneal and peri-renal infiltration causing dilatation of the upper renal tracts. The cases are illustrated with contrast studies, computed tomography (CT) and magnetic resonance imaging (MRI). Typical sclerosis of the long bones was apparent on radiography. Both cases have been treated conservatively to date.A brief review of the literature regarding the manifestations of ECD is included. In cases of non-malignant retroperitoneal infiltration, ECD should be considered as a diagnosis and radiographs of the long bones performed.- - - - - - - - - - ranking = 1.0476191907623keywords = upper, chest (Clic here for more details about this article) |
14/120. Pyeloureteral junction stenosis and ureteral valve causing hydronephrosis.A young female patient underwent ureteral resection and Hynes-Anderson pyeloureteroplasty because of pyeloureteral junction stenosis and a ureteral valve (UV), which caused hydronephrosis. Despite using ultrasonography and intravenous urography the ureteral valve was observed only on exploration. UV should be considered as a possible cause of upper urinary tract obstruction; retrograde urography can be essential.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
15/120. retroperitoneal fibrosis: unusual cause of low back pain.retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease with a male predominance. Back pain with no specific radiation pattern is common, and bilateral obstructive uropathy, potentially reversible, is frequently associated with RPF. We report a case of RPF and review its diagnosis and management.- - - - - - - - - - ranking = 3.9900844287731keywords = back pain, back (Clic here for more details about this article) |
16/120. The silent neurogenic bladder.Children with occult or silent neurogenic bladders may present with urinary reflux and severe upper urinary tract deterioration. Commonly, the neuro-deficit eludes detection. Reimplantation of these ureteral units will likely result in failure.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
17/120. Lower pole ureteral obstruction secondary to fecal impaction in an 8-year-old girl.An 8-year-old girl presented with frequent, nonfebrile, urinary tract infections and a lifelong history of severe constipation. Ultrasound revealed bilateral duplicated renal systems, severe right upper pole hydronephrosis, and moderate left lower pole hydronephrosis. Computerized tomographic scan revealed a large, fecal-impacted colon. Diuretic renogram showed high-grade obstruction (T1/2 >30 minutes) of the right upper pole and left lower pole moieties. After administering an aggressive bowel preparation, a repeat diuretic renogram was performed, which revealed resolution of the left lower pole obstruction. constipation has long been established as a contributing factor to urinary tract infections in children. However, fecal impaction causing ureteral obstruction in a child has not been reported. We report the case of an 8-year-old girl who had left lower pole ureteral obstruction secondary to fecal impaction.- - - - - - - - - - ranking = 2keywords = upper (Clic here for more details about this article) |
18/120. Protein-losing enteropathy in a young African-American woman with abdominal pain, diarrhea and hydronephrosis.The case of a 21-year-old African-American woman who presented with abdominal pain, diarrhea and hydronephrosis and who proved to have protein-losing enteropathy secondary to systemic lupus erythematosus is discussed. This is an unusual complication of lupus.- - - - - - - - - - ranking = 26.164139928216keywords = abdominal pain (Clic here for more details about this article) |
19/120. Bilateral complete renal duplication with total obstruction of both lower pole collecting systems.sepsis and a flank mass developed in a twenty-two-year-old primagravida two days after a normal delivery. urography showed normal upper pole collecting structures bilaterally. A spherical mass containing curvilinear calcification occupied the left lower pole, and a large inflammatory mass filled the right inferior renal fossa. angiography and retrograde pyelography demonstrated marked bilateral lower pole hydronephrosis with complete obstruction of the water to each inferior duplicated collecting system.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
20/120. Pulmonary hyalinizing granuloma with hydronephrosis.A 49-year-old man was admitted for the evaluation of a bilateral mass shadow in his chest x-ray film. No definitive diagnosis was established either by brushing cytology or biopsy through bronchoscopy. No malignancies were suggested by general work-up. Both masses were surgically removed, and were diagnosed as pulmonary hyalinizing granuloma (PHG). Fifteen months later, low grade fever continued and the renal function decreased. Laboratory examinations revealed bilateral hydronephrosis with polyclonal hypergammaglobulinemia. The findings of abdominal CT and urography were compatible with retroperitoneal fibrosis. Steroid treatment completely reversed the initial abnormality in laboratory data and the symptoms disappeared.- - - - - - - - - - ranking = 0.011904797690583keywords = chest (Clic here for more details about this article) |
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