Neurofibromatosis with bilateral renal artery stenosis and end stage renal disease: case report

Neurofibromatosis type 1 [NF-1] is an autosomal dominant, hereditary, neurocutaneous syndrome that may, primarily or secondarily, affect different organs or systems of the body including the cardiovascular system. The most common vascular abnormality in patients with NF-1 is renal artery stenosis. Here we report the case of a middle-aged gentleman who presented at Sultan Qaboos University Hospital, Oman, with end stage renal disease and severe hypertension and was diagnosed to have NF-1 with bilateral renal artery stenosis. He was started on renal replacement therapy

Persistent hypertension in Thai children: etiologies and outcome.

A retrospective study was done in 66 children (0.21% of all admitted children) below the age of 18 years with persistent hypertension diagnosed at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital from Jan 1999 to Dec 2003. Male to female ratio was 1.4:1 with 54.5% aged between 6-12 years old and 9.1% aged less than 1 year. Hypertension was found to be severe (BP more than the 99th percentile for age, sex and height) in 79.1% but most (78.6%) of the patients did not have symptoms related to hypertension. Chronic headaches were found in 10%, hypertensive encephalopathy in 8.6%, epistaxis in 1.4% and visual disturbance in 1.4%. The most common cause of hypertension was renal parenchymal diseases (62.7%) mainly lupus nephritis (26.9%), idiopathic nephrotic syndrome (16.4%) and chronic renal failure (16.4%). Other causes of hypertension included renovascular diseases (7.5%), drug-induced (7.5%), essential (7.5%), tumors (4.5%), coarctation of aorta (3.0%), bronchopulmonary dysplasia (3.0%), and pheochromocytoma (1.5%). Obesity and overweight (body mass index, BMI more than 25) was found in only 10 patients (15.1%).The proportion of children with BMI more than 25 was not different between essential and secondary hypertension (p = 0.15). Left ventricular hypertrophy was noted in 7.5%, hypertensive retinopathy in 3.0%, and hypertensive encephalopathy in 9.0%. One-third of the patients had normal BP within 1 month and another 47.0% had normal BP within 6 months of diagnosis. One-fifth of the patients also needed surgical intervention for specific underlying diseases. The authors suggest that since a large number of children with hypertension have secondary hypertension, intensive investigation and prompt management should be done in all. Obesity and overweight is not reliable in the differentiation between primary and secondary hypertension. Short term outcome of hypertension is good with medications and surgery in selected cases but long term outcome is still unknown.

Riñón e hipertensión / Kidney and hypertension

En este artículo se describen las interrelaciones entre el riñón e hipertensión arterial: el riñón como causa de hipertensión y la hipertensión como causa de nefropatía crónica e insuficiencia renal. Se destaca a la insuficiencia renal como problema de salud pública y a la nefropatía diabética con hipertensión y a la enfermedad renal hipertensiva como las causas más relevantes. Se discuten mecanismos fisiopatológicos y medidas farmacológicas destinadas a disminuir la incidencia y la progresión de la insuficiencia renal crónica.

Juxtaglomerular cell tumor as a rare cause of hypertension in adults

The juxtaglomerular cell tumor is a cause of secondary hypertension in adults. A 35-year-old female patient suffering from hypertension and low serum potassium had a 3 x 3 cm solid mass at the lower pole of left kidney diagnosed by abdominal sonography. Partial nephrectomy was performed and the postoperatory was uneventful. Normalization of blood pressure was observed within the first month.

Juxtaglomerular cell tumor of the kidney: a case report

We report a case of renin-secreting juxtaglomerular cell tumor which developed in a hypertensive 47-yr-old Korean man. Presumptive clinical diagnosis was made before surgery based on the high level of plasma renin and the radiologic evidence of renal mass. Grossly, a round, bulging, well-encapsulated mass of 3x3 cm was located in the mid-portion of the right kidney. On microscopic examination, the tumor was composed of ovoid to polyhedral cells with bland nuclei, indistinct nucleoli and light eosinophilic cytoplasm. The immunostaining for renin showed strong positivity in the cytoplasm of tumor cells. The characteristic rhomboid shaped renin protogranules were observed in ultrastructural analysis.

Association of the ACE gene polymorphism with the progression of autosomal dominant polycystic kidney disease

Renin-angiotensin system is considered important in the genesis of hypertension and development of end-stage renal disease (ESRD) in autosomal dominant polycystic kidney disease (ADPKD). The angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism has been associated with susceptibility to the development of some renal diseases. We investigated the association of ACE gene polymorphism with the progression to hypertension and ESRD in 108 patients with ADPKD. The ACE I/D polymorphism was amplified with the flanking primers by polymerase chain reaction. In patients genotyped for ACE gene polymorphism, the frequencies of DD (15+ACU-), ID (51+ACU-) and II (34+ACU-) genotypes were similar to those of the general population. Of the 108 patients, 64 (59+ACU-) developed hypertension and 24 (22+ACU-) reached ESRD at the time of study. The prevalence of hypertension was not significantly different among the three genotypes. The mean renal survival time was 53-6 yr in II genotype, 5510 yr in ID genotype and 529 yr in DD genotype which was not significantly different among them. Cumulative renal survival was not significantly different either. There was no association of ACE gene polymorphism with the prevalence of hypertension and renal survival in ADPKD. We suggest that ACE I/D polymorphism is not an important modifying gene in the progression of ADPKD.

Association of the ACE gene polymorphism with the progression of autosomal dominant polycystic kidney disease

Renin-angiotensin system is considered important in the genesis of hypertension and development of end-stage renal disease (ESRD) in autosomal dominant polycystic kidney disease (ADPKD). The angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism has been associated with susceptibility to the development of some renal diseases. We investigated the association of ACE gene polymorphism with the progression to hypertension and ESRD in 108 patients with ADPKD. The ACE I/D polymorphism was amplified with the flanking primers by polymerase chain reaction. In patients genotyped for ACE gene polymorphism, the frequencies of DD (15+ACU-), ID (51+ACU-) and II (34+ACU-) genotypes were similar to those of the general population. Of the 108 patients, 64 (59+ACU-) developed hypertension and 24 (22+ACU-) reached ESRD at the time of study. The prevalence of hypertension was not significantly different among the three genotypes. The mean renal survival time was 53-6 yr in II genotype, 5510 yr in ID genotype and 529 yr in DD genotype which was not significantly different among them. Cumulative renal survival was not significantly different either. There was no association of ACE gene polymorphism with the prevalence of hypertension and renal survival in ADPKD. We suggest that ACE I/D polymorphism is not an important modifying gene in the progression of ADPKD.

Hipertensäo e doenças primárias renais / Hypertension and nephropathy

A hipertensão nas doenças renais primárias possui múltiplas causas. Num extremo, quando a perda de função é mais presente, estão as causas relacionadas ao volume e sódio, caracterizando hipertensão sal-sensível. No outro extremo, onde a função está ainda mantida próxima ao normal, estão mais presentes as causas relacionadas à vasoconstrição periférica. No texto descrevemos as várias causas aventadas e sua inter-relação nos pacientes com doença renal.

A angiografia por subtraçäo digital no diagnóstico da isquemia visceral / Digital subtraction angiography in the diagnosis of visceral eschemia

Oitenta e cinco pacientes hipertensos foram submetidos a angiografia por subtraçäo digital, para exploraçäo de artérias renais e identificaçäo da causa da hipertensäo no período de fevereiro de 1984 a agosto de 1991, nas Clínicas Péan e Bizet, Paris, França. A aortografia abdominal, em várias incidências, permitiu também a observaçäo das artérias digestivas, tronco celíaco e artéria mesentérica superior. As incidências preferencialmente estudadas foram as de perfil, oblíqua anterior esquerda e oblíqua anterior direita. A análise do tronco celíaco e da artéria mesentérica superior se faz na incidência de perfil, e das artérias renais esquerda e direita, nas incidências oblíquas anterior esquerda e direita, respectivamente. Esses pacientes foram submetidos a análise retrospectiva para identificaçäo de lesöes incidentes nas artérias digestivas concomitantes a lesöes arteriais renais. Observou-se alto porcentual de concomitância de lesöes ateroscleróticas em artérias renais e digestivas, variando de 44,7 por cento a 52,9 por cento, para lesöes do tronco celíaco, e de 14,1 por cento a 28,2 por cento, para lesöes da artéria mesentérica superior, o que justifica o estudo minuncioso da aorta abdominal e de seus ramos quando da pesquisa de lesöes arteriais renais ou digestivas

Valoración del sodio plasmático en resección transuretral de próstata / Plasmatic sodium meassurement in prostatic transurethral resection

Las complicaciones que se presentan durante la resección transuretral de próstata se deben a la utilización de líquidos de irrigación, los cuales se absorben y pueden ocasionar una hiponatremia dilucional. por tal motivo, se estudiaron dos grupos de pacientes sometidos a resección transuretral. El primer grupo, integrado por 34 pacientes, no presentaron manifestaciones clínicas de hiponatremia durante la resección transuretral, al medir el sodio plasmático durante el pre y postoperatorio. En el segundo se estudiaron 33 pacientes, los cuales presentaron datos de hiponatremia al medir el sodio pre, trans y postoperatorio. Se encontró que hay hiponatremia dilucional cuando los pacientes presentan las manifestaciones clínicas (p< 0.01) y ésta se corrige al administrar bicarbonato de sodio (p < 0.04). Sin embargo, una hora después de aplicar el medicamento, los niveles de sodio no fueron los óptimos, por lo que en algunos casos es necesaria la administración de una segunda dosis de bicarbonato de sodio. También se encontró hiperkalemia con diferencias estadísticamente significativas (p < 0.005)

Fístulas arteriovenosas renales / Renal arteriovenosas fistulas

Em 5 pacientes con fístulas arteriovenosas renales, 4 de origem traumático y 1 por perforación de un aneurisma arteriosclerótico en una vena adyacente, se operaron 4 entre los 20 días y 3 años de producida la lesión. La indicación se basó en la presencia de hematuria persistente, compromiso hemodinámico general, hipertensión arterial, posibilidades de deterioro de la función renal y/o riesgo de rotura del aneurisma. La cirugía consistió en el 1r caso, en la ligadura de los vasos comprometidos y resección parcial del riñon; en el 3§ en la ligadura de la arteria comprometida; en el 4§ cierre del orificio fistular, utilizando hipotyermia profunda y paro cardíaco; en el 5§ en la resección del aneurisma y reparación "ex-situ" de los vasos comprometidos. El 2§ no fue operado porque su fístula era pequeña. Todos los pacientes quírurgicos evolucionaron favorablemente, menos 1 que falleció aol año, por agravamiento de su insuficiencia renal crónica