Hipertensión arterial en Pediatría. Factores de riesgo inflamatorios y sensibilidad gustativa salina / Arterial hypertension in Pediatrics. Inflammatory risk factors and salt taste sensitivity

Detectar un caso de hipertensión arterial en un paciente pediátrico obliga las más de las veces a descartar una enfermedad orgánica asociada, generalmente, una nefropatía. Entre los últimos años, sin embargo, se diagnostican cada vez más casos de hipertensión arterial esencial o primaria, debido fundamentalmente al aumento de la prevalencia de obesidad y sobrepeso en los niños y adolescentes. En la presente revisión se analizan las relaciones existente entre la hipertensión arterial y la obesidad con otros factores de riesgo cardiovascular tanto clásicos como con los nuevos factores de tipo inflamatorio de reciente aparición. El conocimiento del fenómeno de canalización o tracking y el estudio de estos factores permitirán una menor caracterización del riesgo de nuestros pacientes pediátricos (AU)

In order to detect a case of arterial hypertension in a pediatric patient often requires ruling out an associated organic disease, generally kidney disease. In recent years, however, more and more cases are being diagnosed of essential or primary hypertension, fundamentally due to the increase of the prevalence of obesity and overweight-ness in children and adolescents. In the present review, the relationships existing between arterial hypertension and obesity with other cardiovascular risk factors, both classical and the new inflammatory type factors of recent appearance are analyzed. The knowledge of the canalization phenomenon or tracking and the study of these factors will allow for a better characterization of the risk of our pediatric patients (AU)

[Risk of urolithiasis in patients with cystic fibrosis]. / Riesgo de urolitiasis en pacientes con fibrosis quística.

INTRODUCTION: It has been generally admitted that kidneys are not affected in Cystic fibrosis (CF) patients. Anyway, there are several studies demonstrating a higher prevalence of calcium oxalate nephrolithiasis prevalence in CF patients compared to normal population. The aim of our study was to evaluate renal disease regional prevalence in CF patients METHODS: Cross-sectional regional controlled study. Index group = 20 CF subjects 4- 30 years old controlled in a CF outpatient clinic in a tertiary hospital. Control group = 73 healthy subjects randomly selected among the 100 subjects who completed the follow-up visits of the RICARDIN study. Physical examination and renal function analysis were conducted in both groups. CF patients had their functional measurements repeated in a 20 month interval. Renal ultrasonography was performed only in CF patients. RESULTS: Height estimated creatinine clearance in CF patients were not statistically different from controls. Oxaluria (0.77 mg/kg/24 h, p = 0.001) and phosphate excretion (18.98 mg/kg/24 h, p = 0.04) were significantly higher while citrate (7.76 mg/kg/24 h, p = 0.04) and magnesium (1.60 mg/kg/24 h, p = 0.04) excretion were significantly lower in CF patients than normal population reference values. No signs of nephrolithiasis or nephrocalcinosis were found. CONCLUSIONS: CF patients showed a conserved renal function , without ecographic abnormalities. CF patients showed increased urinary elimination of phosphate and oxalate and lower citrate and magnesium elimination, findings that predispose these patients to suffer from urolithiasis in the future.

Risk of urolithiasis in patients with Cystic Fibrosis

Introduction: It has been generally admitted that kidneys are not affected in Cystic fibrosis (CF) patients. Anyway, there are several studies demonstrating a higher prevalence of calcium oxalate nephrolithiasis prevalence in CF patients compared to normal population. The aim of our study was to evaluate renal disease regional prevalence in CF patients Methods: Cross-sectional regional controlled study. Index group = 20 CF subjects4- 30 years old controlled in a CF outpatient clinic in atertiary hospital. Control group = 73 healthy subjects randomly selected among the 100 subjects who completed the follow-up visits of the RICARDIN study. Physical examination and renal function analysis were conducted in both groups. CF patients had their functional measurements repeated in a 20 month interval. Renal ultrasonography was performed only in CF patients. Results: Height estimated creatinine clearance in CF patients were not statistically different from controls. Oxaluria (0.77mg/kg/24 h, p = 0.001) and phosphate excretion (18.98 mg/kg/24 h, p = 0.04) were significantly higher while citrate (7.76mg/kg/24 h, p = 0.04) and magnesium (1.60mg/kg/24 h, p = 0.04) excretion were significantly lower in CF patients than normal population reference values. No signs of nephrolithiasis ornephrocalcinosis were found. Conclusions: CF patients showed a conserved renal function , without ecographic abnormalities. CF patients showed increased urinary elimination of phosphate and oxalate and lower citrate and magnesium elimination, findings that predispose these patients to suffer from urolithiasis in the future (AU)

Introducción: generalmente, se ha aceptado que el riñón no sea un órgano afectado en la Fibrosis Quística (FQ). Diferentes estudios han demostrado que los pacientes con FQ tienen una mayor predisposición a padecer nefrolitiasis por cálculos de oxalato cálcico que la población general. Objetivo: estudiar la prevalencia autonómica de enfermedad renal en pacientes con FQ. Pacientes y métodos: estudio transversal analítico de ámbito autonómico, con grupo control. Grupo índice: 20 pacientes con FQ de 4-30 años de edad. Grupo control: 73 sujetos sanos, seleccionados al azar entre los 100 individuos de 13-21años de edad que completaron el seguimiento longitudinal del estudio RICARDIN. Realización en ambos grupos de estudio de examen físico y pruebas analíticas de función renal, repitiendo en el grupo FQ la recogida de datos 20 meses después. Ecografía renal sólo en el grupo índice. Resultados: el filtrado glomerular estimado por la talla no alcanzó diferencias significativas en el grupo FQ respecto al control. Los pacientes del grupo FQ presentaron valores significativamente más altos de oxaluria (0,77mg/kg/24 h, p = 0,001) y fosfaturia (18,98 mg/kg/24 h, p =0,04), y valores más bajos de citraturia (7,76 mg/kg/24 h,p = 0,04) y magnesuria (1,60 mg/kg/24 h, p = 0,04) que los valores normales de referencia. La ecografía renal no demostró en ningún caso hallazgos sugerentes de nefrocalcinosis ni nefrolitiasis. Conclusiones: los pacientes con FQ presentan una función renal conservada, sin diferencias significativas con los controles. Estos pacientes presentan alteraciones en la excreción urinaria de solutos que pueden favorecer la formación futura de cálculos renales (AU)

[High blood pressure due to aortic coarctation and renal artery stenosis in a teenager with type 1 neurofibromatosis]. / Hipertensión secundaria a coartación de aorta y estenosis de arteria renal en adolescente con neurofibromatosis tipo 1.

Hypertension affect about 1% of patients with neurofibromatosis type 1 (NF1). Major causes are concomitant pheocromocytoma in adults and renovascular hypertension in children. In most cases, NF1 is associated with renal artery stenosis, smooth cell proliferation and advential fibrosis. We describe a 16 year old girl with hypertension complicating NF1 secondary to severe coarctation of abdominal aorta and tight stenosis of right renal artery, a very uncommon case. She was first diagnosed when she was 3-years-old and managed with antihypertensive drugs (atenolol, hidralazine and nifedipine); she experienced progressive uncontrollable hypertension but no symptoms, thus she was admitted to repeat studies. Laboratory evaluation (including creatinine, serum electrolytes, urinalysis, urine catecholamines and creatinine clearance) was normal Percutaneous transfemoral magnetic resonance angiography disclosed severe coarctation of abdominal aorta, functional occlusion of superior mesenteric artery and tight stenosis of right renal artery with poststenotic dilatation. Patient underwent surgery with aorto-aortic by-pass and right kidney artery reimplantation. Periodical controls confirmed no hypertension, even four years after surgery and normal flow patterns in Doppler ultrasonography. Patients with NF1 must be screened for pheochromoctyoma and renovascular hypertension. If hypertension appears, careful management is mandatory, as periodical follow-up even after surgery, since the long-term recurrence rate of renovascular lesions is not well established.

Hipertensión secundaria a coartación de aorta y estenosis de arteria renal en adolescente con neurofibromatosis tipo 1 / High blood pressure due to aortic coarctation and renal artery stenosis in a teenager with type 1 neurofibromatosis

La frecuencia estimada de la hipertensión arterial en pacientes con neurofibromatosis tipo 1 (NF1) es de aproximadamente un 1%, habitualmente secundaria a feocromocitomas y estenosis de las arterias renales. La coartación de aorta asociada en estos pacientes es una causa infrecuente de hipertensión. Presentamos el caso de una paciente conNF1 que padece hipertensión desde los 3 años de vida, mal controlada con tratamiento farmacológico. Antecedentes familiares de NF1 (madre). A la exploración física presentaba múltiples manchas café con leche, efélides axilares y presión arterial por encima de percentil 95 para edad y altura. Las pruebas de laboratorio (creatinina sérica, electrolitos, catecolaminas urinarias y aclaramiento de creatinina)fueron normales. La monitorización ambulatoria de presión arterial reveló hipertensión diurna con valores nocturnos normales. La ecografía Doppler renal mostró un patrón anormal de flujo en arteria renal derecha compatible con estenosis y la angiorresonancia magnética: coartación severa de aorta abdominal, con oclusión funcional de arteria mesentérica superior y estenosis moderada de arteria renal derecha con dilatación postestenótica. Ante estos hallazgos, la paciente fue intervenida quirúrgicamente, con realización de by-pass aorto-aórtico y reimplantación de arteria renal derecha. Durante los cuatro meses siguientes a la intervención recibió tratamiento hipotensor con nifedipino oral que se suspendió ante la buena evolución clínica. Los controles periódicos en la consulta durante los cuatro años siguientes a la cirugía fueron satisfactorios, con presión arterial normal y función renal conservada. Comentarios: El control de la presión arterial en pacientes con NF1 es aconsejable para detectar casos de hipertensión arterial que, en muchas ocasiones, va a requerir tratamiento quirúrgico (AU)

Hypertension affect about 1% of patients with neurofibromatosis type 1 (NF1). Major causes are concomitant pheocromocytoma in adults and renovascular hypertension in children. In most cases, NF1 is associated with renal artery stenosis, smooth cell proliferation and advential fibrosis. We describe a 16 year old girl with hypertension complicating NF1 secondary to severe coarctation of abdominal aorta and tight stenosis of right renal artery, a very uncommon case. She was first diagnosed when she was 3-years-old and managed with antihypertensive drugs (atenolol, hidralazine and nifedipine); she expirienced progressive uncontrollable hypertension but no symptoms, thus she was admitted to repeat studies. Laboratory evaluation (including creatinine, serum electrolites, urinalysis, urine catecholamines and creatinine clearance) was normal Percutanaous transfemoral magnetic resonance angiography disclosed severe coarctation of abdomina aorta, functional oclussion of superior mesenteric artery and tight stenosis of right renal artery with postestenotic dilatation. Patient underwent surgery with aorto-aortic by-pass and right kidney artery reimplantation. Periodical controls confirmed no hypertension, even four years after surgery and normal flow patterns in Doppler ultrasonography. Patients with NF1must be screened for pheochromoctyoma and renovascular hypertension. If hypertension appears, careful management ismandatory, as periodical follow-up even after surgey, since the long-term recurrence rate of renovascular lessions is not well established (AU)

[Hemolytic uremic syndrome by Streptococcus pneumoniae]. / Síndrome hemolítico urémico asociado a neumonía neumocócica.

Hemolytic uremic síndrome (HUS) is an acute disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, usually,but not always, associated with the prodrome of diarrhea. A 2-year-6-month old boy with pneumonía caused by Streptococcus pneumoniae developed the third day after admission oliguric acute renal failure, anemia, and thrombocytopenia. The patient needed veno-venous hemodiafiltration/ hemofiltration during ten days. He received five packed red blood cell, and one platelet transfusions, and was treated with plasma infusions during six days. Renal function was normal at discharge. HUS caused by Streptococcus pneumoniae has more morbility and mortality that classic HUS, although they usually recovery renal function. In those cases of atypical HUS, different from others, blood transfusions that contains IgM should be avoided,because they can increase antigen-antibody reaction and cellular damage. The low incidence of HUS by Streptococcus pneumoniae and the systemic infectious component difficult the early diagnosis. Taking into account the different transfusion approach in these cases, it is necessary to be aware for diagnosing HUS by Streptococcus pneumoniae in all patients with systemic infection and sudden renal function deterioration.

Síndrome hemolítico urémico asociado a neumonía neumocócica / Hemolytic uremic syndrome by streptococcus pneumoniae

El síndrome hemolítico urémico (SHU) se caracteriza por anemia hemolítica microangiopática, trombocitopenia e insuficiencia renal aguda, habitualmente, aunque no siempre, en el contexto de procesos digestivos. Se presenta el caso de un varón de 2 años y 6 meses de edad con neumonía neumocócica que, el tercer día de ingreso hospitalario, inició fracaso renal oligoanúrico, anemización y trombopenia. El paciente precisó tratamiento sustitutivo mediante hemodiafiltración/hemofiltración durante 10 días y recibió transfusiones de hematíes, plaquetas y plasma fresco congelado, con buena respuesta. La función renal al alta fue normal. Aunque los SHU debidos a infección por neumococo presentan mayor morbimortalidad que los asociados a diarrea (típicos) y que otros de los considerados atípicos, suelen evolucionar hacia la recuperación de la función renal. En estos casos, a diferencia del resto, deben evitarse las transfusiones de derivados sanguíneos que contengan IgM, ya que pueden aumentar la reacción antígeno-anticuerpo y el daño celular. La baja frecuencia de SHU atípico y el compromiso sistémico de estos enfermos en el contexto de su enfermedad puede dificultar su reconocimiento. Por ello y dadas las implicaciones terapéuticas que conlleva, es preciso considerar la posibilidad de SHU por neumococo en todo paciente con infección neumocócica demostrada y deterioro agudo de la función renal o presencia de discrasias sanguíneas

Hemolytic uremic síndrome (HUS) is an acute disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, usually, but not always, associated with the prodrome of diarrhea. A 2-year-6-month old boy with pneumonía caused by Streptococcus pneumoniae developed the third day after admission oliguric acute renal failure, anemia, and thrombocytopenia. The patient needed veno-venous hemodiafiltration/ hemofiltration during ten days. He received five packed red blood cell, and one platelet transfusions, and was treated with plasma infusions during six days. Renal function was normal at discharge. HUS caused by Streptococcus pneumoniae has more morbility and mortality that classic HUS, although they usually recovery renal function. In those cases of atypical HUS, different from others, blood transfusions that contains IgM should be avoided, because they can increase antigen-antibody reaction and cellular damage. The low incidence of HUS by Streptococcus pneumoniae and the systemic infectious component difficult the early diagnosis. Taking into account the different transfusion approach in these cases, it is necessary to be aware for diagnosing HUS by Streptococcus pneumoniae in all patients with systemic infection and sudden renal function deterioration

Risk factors in the development of early technetium-99m dimercaptosuccinic acid renal scintigraphy lesions during first urinary tract infection in children.

AIM: To establish the variables that correlate with uptake defects in dimercaptosuccinic acid (DMSA) scintigraphy performed in the acute phase of a first episode of urinary tract infection (UTI). METHODS: A prospective observational study was conducted in a cohort of 158 consecutive children with a first episode of symptomatic UTI. The therapeutic delay time (TDT) was recorded. DMSA scintigraphy was performed in all children and voiding cystourethrography in 150 of them. RESULTS: 85% of the patients were younger than 2 y. Mean TDT was 33.5 h. The aetiological agent was Escherichia coli in 140 children. DMSA scintigraphy was normal in 81. Vesicoureteric reflux was detected in 33. After a multivariate logistic regression analysis the following variables were retained in the final model: TDT > or = 48 h, growth of bacteria other than E. coli, percentage of polymorphonuclear cells > or = 60% and C-reactive protein > or = 30 mg l(-1). CONCLUSION: TDT > or = 48 h, bacteria other than E. coli, percentage of polymorphonuclear cells > or = 60% and CRP > or = 30 mg l(-1) influence the findings detected in the DMSA scintigraphy performed in the acute phase of a first episode of UTI.

Caracterização clínica e histopatológica da adenite sebácea em diferentes fases

O artigo não apresenta resumo.

Displasia microvascular hepática. Relato de um caso

O artigo não apresenta resumo.

Tumor endócrino de pâncreas associado a síndrome hipoglicêmica. Relato de caso e caracterização imuno-histoquímica

O artigo não apresenta resumo.

Tratamiento conservador de la displasia renal multiquística durante la infancia / Conservation treatment of multicystic renal dysplasia during chilhood

La displasia renal multiquística (DRM) continúa generando interrogantes por su relación potencial con complicaciones evolutivas, tales como hipertensión arterial, aumento en la incidencia de infecciones urinarias y potencial degeneración maligna. Nos planteamos este estudio con el fin de conocer la evolución natural de la DRM tratada de forma conservadora en una Unidad de Nefrología Pediátrica. Métodos: Se diagnosticaron 35 pacientes (13 mujeres) de DRM. De ellos, 26 fueron tratados conservadoramente durante un período medio de 9 años y 7 meses (rango: 1 mes-14 años). El protocolo de trabajo consistió en controles clínico-analíticos y realización de ecografía renal semestral durante los dos primeros años de vida y posteriormente anual, hasta la completa involución del riñón displásico. Resultados: El 69 por ciento de las DRM fueron detectadas precozmente en ecografías prenatales (24/35 pacientes). Doce pacientes (34 por ciento) presentaron anomalías urológicas asociadas, siendo la ureterohidrofenosis y el reflujo vesicoureteral las malformaciones más frecuentes. Un total de 13 nefrectomías se efectuaron durante el período de seguimiento, nueve de ellas durante la época neonatal y lactancia. En el resto de pacientes, un 82 por ciento (14/17) presentaban involución renal a los 6 años de seguimiento. En 5 enfermos no pudo completarse el seguimiento. En 5 enfermos no pudo completarse el seguimiento. Exceptuando a los pacientes con uropatías asociadas que presentaron infecciones urinarias (5 casos) o insuficiencia renal crónica (3 pacientes), no se registraron otros casos de hipertensión arterial o malignización atribuibles a la DMR. Conclusiones: El seguimiento de la DMR indica que una considerable proporción de los pacientes presentan una involución espontánea, por lo que el manejo conservador debe ser, en nuestra opinión, la modalidad terapéutica de elección inicial en estos pacientes. En nuestra serie, el riesgo de complicaciones de la DMR es bajo, en especial si no se asocia a otras malformaciones urológicas (AU)

Targeted reduction in expression of Trypanosoma cruzi surface glycoprotein gp90 increases parasite infectivity.

A previous study had shown that the expression of gp90, a stage-specific surface glycoprotein of Trypanosoma cruzi metacyclic trypomastigotes, is inversely correlated with the parasite's ability to invade mammalian cells. By using antisense oligonucleotides complementary to a region of the gp90 gene implicated in host cell adhesion, we investigated whether the selective inhibition of gp90 synthesis affected the capacity of metacyclic forms to enter target cells. Parasites were incubated for 24 h with 20 microM PS1, a phosphorothioate oligonucleotide based on a sequence of the gp90 coding strand; PS2, the antisense counterpart of PS1; or PO2, the unmodified version of PS2 containing phosphodiester linkages, and the expression of surface molecules was analyzed by flow cytometry and immunoblotting using specific monoclonal antibodies. PS2 but not PS1 or PO2 inhibited the expression of gp90. Inhibition by PS2 was dose dependent. Northern blot analysis revealed that steady-state gp90 mRNA levels were diminished in PS2-treated parasites compared to untreated controls. Treatment with PS2 did not affect the expression of other metacyclic stage surface glycoproteins involved in parasite-host cell interaction, such as gp82 and the mucin-like gp35/50. Expression of gp90 was also inhibited by other phosphorothioate oligonucleotides targeted to the gp90 gene (PS4, PS5, PS6, and PS7) but not by PS3, with the same base composition as PS2 but a mismatched sequence. Parasites treated with PS2, PS4, or PS5 entered HeLa cells in significantly higher numbers than untreated controls, whereas the invasive capacity of PS1- and PS3-treated parasites was unchanged, confirming the inverse association between infectivity and gp90 expression.

Towards guidelines for dialysis in children with end-stage renal disease.

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary.

Predictors of final adult height after renal transplantation during childhood: a single-center study.

AIM: Assessment of final adult height and its predictive factors in children transplanted (RTx) and followed up in a single center. METHODS: A cohort of 32 patients (17 boys, 15 girls) who received RTx before the age of 15 years and had reached a final adult height was selected. Twenty patients received a single RTx, 9 patients received two RTx, and 3 patients received three RTx. Seven children were transplanted preemptively, while the remaining 25 children received peritoneal dialysis for relatively short periods of time. In 11 patients, recombinant human growth hormone (rhGH) was administered either before (n = 8) or after (n = 3) RTx. RESULTS: In 13 patiens (41%), the final height standard deviation score for chronological age (hSDS) was -2.3+/-0.5, below the 95% confidence limits for target height (group A), while in 19 patients (59%), it was -0.7+/-0.8, within the 95% confidence limits for target height (group B). The hSDS values at the start of dialysis and at the time of first RTx were significantly lower in group A than in group B. A higher hSDS at the start of dialysis and at the time of first RTx had a significant positive influence on the final height (FH), whereas a longer duration of dialysis had a significant negative effect on the FH. Administration of rhGH after RTx played an important role in the achievement of a normal FH in 3 girls. No differences were observed between group A and B with respect to age at start of dialysis, chronological or bone age at first RTx, number of rejection episodes, duration of the study period from last RTx to FH, glomerular filtration rate during this study period, or percentage of time on prednisone therapy. CONCLUSIONS: The FH is almost exclusively predetermined by the height achieved at the start of dialysis and at the time of first RTx. Therefore, to reach target adult height after RTx, the best strategy is to shorten the time of dialysis and to start rhGH administration at a young age and as early as possible during the course of chronic renal failure. Administration of rhGH after RTx is also highly effective, but, given its potential danger, still remains a matter of investigation.

Analysis of chromosome 6p in Spanish families with recessive polycystic kidney disease.

Several previous reports have suggested that autosomal recessive polycystic kidney disease (ARPKD) is caused by mutations in a single gene (the PKDH1 gene). Linkage analysis showed a positive linkage for polymorphic markers at the short arm of chromosome 6 (6p) in all families. PKHD1 has not been cloned. Recombinants in the critical region would permit the narrowing of the 6p interval containing the PKHD1 gene, thus facilitating the final identification (cloning) of this gene. Our study included 30 Spanish families. Each family consisted of both parents and at least two children, with at least one diagnosed with ARPKD by clinical and pathological parameters. DNA was obtained and 6p microsatellite markers were used to establish haplotypes for each family. A positive linkage to chromosome 6p was found for all families. In 2 cases, recombinants in the region containing the PKHD1 gene were found. These families will help narrow the size of the 6p region, facilitating the efforts to position and clone the PKHD1 gene. In conclusion, our analysis of Spanish ARPKD families confirms the lack of linkage heterogeneity. This suggests that mutations at a single locus on chromosome 6p21.1-p12 are responsible for the broad clinical spectrum of variable phenotypes.