Cases reported "Kidney Diseases, Cystic"

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1/14. Prenatal collapse of cysts in a dysplastic kidney.

    Postnatal regression of prenatally or neonatally detected multicystic dysplastic kidney disease has been widely documented. However, renal cysts can regress during gestation, although they usually become larger in utero. We present a case of prenatally detected multicystic dysplastic kidney with an atypical course. During the third trimester, unilateral multicystic renal lesions in the fetus first enlarged and later involuted; by the second postpartum year, the kidney had become hypoplastic and nonfunctional. Our case also shows that before birth, blood flow in the affected kidney, measured with Doppler imaging, was normal until the cysts involuted.
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2/14. prenatal diagnosis of a partial trisomy 19q.

    Phenotypic anomalies due to a genetic imbalance of chromosome 19 have been reported in very rare postnatal cases. Here a case of partial trisomy 19 diagnosed prenatally by ultrasonography and cytogenetic analysis is presented. Detailed evaluation by sonography showed major anomalies which could be correlated to the typical appearance of this chromosomal anomaly. Termination of pregnancy at 21 weeks of gestation was performed, and the prenatal diagnosis was confirmed postnatally by autopsy. The syndrome in this case was caused by a duplication of the long arm of chromosome 19 (46,XY, dup(19) (q13.1-->qter).
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ranking = 1.9362521714657
keywords = gestation, pregnancy
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3/14. Interplay of pregnancy, lactation, and hyperthyroidism leading to severe osteoporosis in a young woman.

    OBJECTIVE: To describe the case of a young woman who had severe osteoporosis due to the compounding effects of pregnancy, lactation, and hyperthyroidism and who had a presumed metastatic lesion in the lumbar spine. methods: We present the clinical, pathologic, radiologic, and laboratory findings and describe the clinical course of our patient. RESULTS: A 31-year-old Arabic woman was referred to the M. D. Anderson Cancer Center because of a lytic lesion in her lumbar spine, presumed to be metastatic deposits. She had a history of two consecutive pregnancies and intermittently treated hyperthyroidism. Our initial evaluation revealed that the patient had clinical and biochemical thyrotoxicosis, and we treated her with thionamides, corticosteroids, and radioiodine ablation. Radiologic studies disclosed a complex renal cyst that had increased uptake on a bone scan, which was highly suggestive of a primary malignant lesion. Ultimately, however, it proved benign on pathologic analysis after a left nephrectomy. Bone mineral density measurements identified severe osteoporosis (T-scores: lumbar spine, -3.3; right hip, -2.2; and left hip, -2.0), which had led to vertebral collapse and was misinterpreted as malignant metastatic disease. The bone mineral densities improved ( 5 to 11% at the various sites) within 4 months after definitive treatment and cure of the hyperthyroidism. CONCLUSION: The effect of pregnancies and prolonged lactation, in the milieu of other risk factors for bone depletion such as hyperthyroidism, may cause severe osteoporosis in a young patient. The resulting osteoporosis may manifest as a lesion suggestive of malignant metastatic involvement.
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ranking = 4.6812608573286
keywords = pregnancy
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4/14. Meckel Gruber syndrome: a first trimester diagnosis of a recurrent case.

    We report of a case of Meckel Gruber syndrome (MGS) in a woman, who suffered previously from a pregnancy with the same disorder. MGS, consisting of an occipital encephalocele, bilateral cystic kidneys and postaxial polydactyly, is a rare autosomal recessive disorder, with a recurrence risk of 25%. With the present technology, a targeted ultrasound in the late embryonic or early fetal stages of pregnancy has the potential to diagnose this syndrome. Clinical screening in further pregnancies is of utmost importance and the management of such cases is presented.
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ranking = 1.8725043429315
keywords = pregnancy
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5/14. survival in second trimester oligohydramnios secondary to bilateral pelviureteral junction obstruction.

    The extent and onset of obstruction in hydronephrosis determine the varying degrees of renal impairment. Bilateral hydronephrosis, especially in combination with oligohydramnios, is considered a negative predictor for pregnancy outcome. We describe a case of bilateral pelviureteral junction obstruction causing severe oligohydramnios between 25 and 29 weeks of gestation. The prenatal and postnatal findings and treatment are demonstrated. In the presence of bilateral renal impairment and oligohydramnios, our patient had an unfavorable prognosis. The respiratory and renal function, however, were better than expected. We show how urinary tract reconstruction and neonatal intensive therapy can result in an acceptable outcome.
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ranking = 1.9362521714657
keywords = gestation, pregnancy
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6/14. Meckel Gruber syndrome--a case report.

    Meckel Gruber syndrome is a rare syndrome inherited as Mendelian autosomal recessive condition. The affected infant usually has a large occipital encephalocoele associated with renal cysts and sometimes polydactyly. The prognosis is poor. The affected child is still born or dies early in infancy. If diagnosis is done by prenatal ultrasound examination termination of pregnancy can be done.
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ranking = 0.93625217146573
keywords = pregnancy
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7/14. Bilateral cystic dysplasia of the rete testis with renal adysplasia.

    Cystic dyplasia of the rete testis (CDRT) is an uncommon, generally unilateral lesion characterized by anastomosing cystic spaces lined by a flattened simple cuboidal epithelium in the rete testis. In the literature this lesion often is associated with an ipsilateral urogenital lesion such as renal agenesia or multicystic dysplasia of the kidney, in order of frequency. The pathogenesis is explained by some authors by their common embryologic origin. We are reporting the finding of bilateral CDRT associated with ultrasound-diagnosed renal adysplasia in a 20-week gestational age fetus with oligohydramnios. Although CDRT has been referred to as being associated with multicystic renal dysplasia or renal agenesis, the present case appears to be unique in combining all the malformations together.
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8/14. Reversal of polyhydramnios by drainage of a fetal renal cyst. A case report.

    At 28 weeks' gestation a patient developed polyhydramnios associated with a large fetal renal cyst demonstrated by ultrasound. drainage of the cyst was performed, resulting in the disappearance of polyhydramnios. The pregnancy progressed normally to term and resulted in the delivery of a healthy infant.
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ranking = 1.9362521714657
keywords = gestation, pregnancy
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9/14. Acute renal failure in pregnancy associated with solitary kidney, congenital ureteropelvic junction obstruction, and renal cortical cyst.

    We have described a patient with acute renal insufficiency during pregnancy related to unilateral renal agenesis and congenital obstruction of the ureteropelvic junction. Optimism for a good outcome in both the mother and fetus seems justified if therapeutic measures in such cases can maintain renal homeostasis.
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ranking = 4.6812608573286
keywords = pregnancy
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10/14. prenatal diagnosis of glomerulocystic kidney disease in short-rib polydactyly syndrome type II, Majewski type.

    An 18-year-old gravida 1 para 0 female was presented at 35 weeks' gestation with severe oligohydramnios. Sonography revealed a fetus with hydrops, bilaterally enlarged echogenic kidneys, and short extremities. The infant died at birth and autopsy was compatible with short-rib polydactyly syndrome type II, Majewski type. Renal histology was consistent with glomerulocystic disease.
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