Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Phacomatosis pigmentokeratotica is characterized by the coexistence of an organoid epidermal naevus, follow-ing Blaschko's lines, and a large speckled lentiginous naevus, typically arranged in a chequerboard pattern. This entity has been isolated from the group of epidermal naevus syndromes and is frequently associated with extracutaneous anomalies. We report here the first observation of phacomatosis pigmentokeratotica associated with nephroblastoma. In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect. The link between the extracutaneous manifestations and cutaneous twin spot phenotype is discussed.
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PMID:Phacomatosis pigmentokeratotica with nephroblastoma and juvenile hypertension. 2052 46

The combination of hyponatremia and renovascular hypertension is known as hyponatremic-hypertensive syndrome (HHS) and so rarely described in children but associated with various kinds of occlusions of the renal artery. We describe two children who presented HHS with severe hypokalemia, polyuria, and polydipsia associated with Wilms tumor, which required treatment with an angiotensin-converting enzyme inhibitor before nephrectomy. All HHS signs and symptoms resolved only following surgical resection of the tumor, allowing chemotherapy to be given.
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PMID:Severe polyuria and polydipsia in hyponatremic-hypertensive syndrome associated with Wilms tumor. 2065 33

Wilms' tumour comprises 95% of all renal cancers among children less than 15 years of age. The purpose of this review is to examine the existing literature on perinatal and environmental risk factors for Wilms' tumour. A search for epidemiological studies that examined risk factors for Wilms' tumour was undertaken in Medline, LILACS, ISI Web of Science and Dissertation Abstracts. A total of 37 studies, including 14 cohort, 21 case-control and 2 case-cohort studies, were identified that examined environmental and perinatal risk factors. Most studies were from Western Europe and North America, and among case-control studies, 16 used randomly selected population-based controls. We observed a significantly increased risk of Wilms' tumour with maternal exposure to pesticides prior to the child's birth (OR = 1.37 [95% CI 1.09, 1.73]), high birthweight (OR = 1.36 [95% CI 1.12, 1.64]) and preterm birth (OR = 1.44 [95% CI 1.14, 1.81]), although the results regarding pesticide exposure may be subject to publication bias (Egger's test, P = 0.09). Further analyses to adjust for the heterogeneity in the results for high birthweight and preterm birth did not statistically change the significance of the results. Additionally, an increased though not statistically significant risk of Wilms' tumour was associated with maternal hypertension (OR = 1.30 [95% CI 0.99, 1.72]), and, compared with the first born, being a second or later birth was associated with a significantly decreased risk (OR = 0.82 [95% CI 0.71, 0.95]). This review suggests a role for several perinatal and environmental risk factors in the aetiology of Wilms' tumour.
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PMID:Wilms' tumour: a systematic review of risk factors and meta-analysis. 2067 Feb 26

Primary tumors of the renal pelvis and ureter account for about 8% of all urinary tract tumors. More than 90% of them are urothelial carcinomas. On the other hand, unilateral multicystic renal disease is an uncommon pathologic condition that may be mistaken for unilateral autosomal dominant polycystic kidney disease, multilocular cystic nephroma or cystic neoplasm. We present the case of a 54-year-old male known with arterial hypertension, admitted in the Second Surgery Department of Emergency County Hospital, Constanta, with intense right flank and right lumbar pain. This symptom started one month before hospital admission. Based on clinical features and imaging evaluations we established a presumptive diagnosis of unilateral autosomal dominant polycystic kidney disease. For these reasons, total right nephrectomy was performed. Pathologic examination of the nephrectomy specimen revealed high-grade urothelial carcinoma of the renal pelvis associated with unilateral multicystic renal disease. The particularity of this case lies in the uncommon association between two rare renal pathological conditions diagnosed by pathological examination.
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PMID:Urothelial carcinoma of the renal pelvis associated with cystic disease of the kidney. 2142 1

Cystic neoplasms of kidney in pediatric age group include a wide variety of tumors, both of benign and malignant pathology. In a child, renal neoplasms with associated hypertension are rare. Here, we present a 2-year-old child who had a multicystic renal tumor with hypertension. She underwent radical nephrectomy subsequent to which hypertension disappeared. The postoperative pathology revealed multicystic nephroma.
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PMID:Cystic renal neoplasm causing hypertension in a 2-year-old child. 2174 28

Neuroblastoma is the most common extracranial solid tumor to occur during infancy and early childhood. However, primary renal neuroblastoma is rare, and only scattered case reports exist in the English medical literature. We report 8 cases that accumulated at our institution over the past 15 years and summarize their clinicopathologic features. The composite picture of a patient with renal neuroblastoma is that of a boy of 17 months of age, who presented with a large renal mass, about 9 cm in size, accompanied by hypertension. The mass was typically hemorrhagic, either encapsulated or unencapsuated, and infiltrating. A renal neuroblastoma can be undifferentiated, poorly differentiated, or differentiating; it falls into either the favorable or the unfavorable histology category, and presentation at higher stages is the rule. The N-myc is usually unamplified, and the bone marrow is usually not involved at presentation. Unless the tumor is undifferentiated or very poorly differentiated, patients with renal neuroblastoma fare well, although not without new and improved modalities of treatment. Primary renal neuroblastoma is perhaps more common than people realize; a higher level of awareness and early recognition are important for its prognosis and management, as they are very different from Wilms tumor.
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PMID:Primary renal neuroblastoma--a clinical pathologic study of 8 cases. 2302 38

Mutations in the WT1 gene can lead to Denys-Drash syndrome or Frasier syndrome and can also cause isolated nephrotic syndrome (NS). Most patients with isolated NS caused by WT1 mutations present as 46, XX phenotypic females. There have been two cases with an onset age younger than 3 years with isolated NS caused by WT1 mutations presenting as 46, XY phenotypic males. We present a 46, XY phenotypic male patient with isolated NS and end-stage renal disease (ESRD) at the age of 6.3 years. He had normal male external genitalia with normal penis length and soft and normal volume of both testes. A mutation, 1051A>G (K351E), in exon 8 of WT1 was identified in the patient. After starting hemodialysis, manifestations of hypertension and renal failure improved, but he died at 6.8 years of age as a result of respiratory failure and heart failure. Our study supports the necessity of searching for mutations in WT1 in 46, XY phenotypic male patients with isolated NS and ESRD.
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PMID:A child with isolated nephrotic syndrome and WT1 mutation presenting as a 46, XY phenotypic male. 2383 59

Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt.
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PMID:Prenatal intrarenal neuroblastoma mimicking a mesoblastic nephroma: a case report. 2290 38

The identification of the different cell types involved in human nephrogenesis, when solely based on morphology, may lead to errors in its interpretation, given the complexity of the histological picture of the fetal and of the newborn kidney. In this study, the most recent works utilizing immunohistochemistry for the identification of the multiple cell types involved in human nephrogenesis are reviewed. The role of WT1, MUC1, Thymosin beta 10, Thymosin beta 4, CD10 and CD44 in the different phases of glomerulogenesis and of tubulogenesis is here described, with particular emphasis on their expression in the early phases of nephrogenesis. On the basis of our data, immunohistochemistry appears to be a useful tool in the study of human nephrogenesis, giving new data on the different steps of the differentiation of metanephric mesenchyme towards the multiple cell types characterizing the mature human kidney. Moreover, allowing a better knowledge of the protein products involved in the generation of new nephrons, immunohistochemistry could open new perspectives in the field of renal regenerating medicine, evidencing the factors able to prolong nephrogenesis after birth, helping us to reach our goal: allowing newborn kidneys to restore their nephron endowment, escaping susceptibility to hypertension and renal disease in adulthood.
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PMID:The role of immunohistochemistry in the study of the newborn kidney. 2295 45

Congenital mesoblastic nephroma (CMN) is a rare renal tumor. It can be detected antenatally especially with judicious use of ultrasonography.A premature female neonate 28 weeks' gestation, complicated by polyhydramnios, was born to a 28-year-old woman. An abdominal mass was detected antenatally. At the end of the first week of life, the newborn had hypertension that was controlled by hydralazine. Ultrasonography and CT scan showed a right-sided renal heterogeneous solid mass. Right nephrectomy was performed and the histology showed CMN.
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PMID:Congenital mesoblastic nephroma: a case report. 2301 73


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