Association of adrenal medullar and cortical nodular hyperplasia: a report of two cases with clinical and morpho-functional considerations.

Arterial hypertension of adrenal etiology is mainly attributed to primary hyperaldosteronism. However, subtle expressions of hyperadrenergic or glucocorticoid excess can also generate arterial hypertension. The present report describes two hypertensive patients cataloged as resistant essential hypertensives, in whom adrenal masses were found incidentally, who highlight the need to recognize these tenuous clinical or laboratory presentations. Case 1 was a 50-yr-old female with hyperadrenergic hypertension associated to a left adrenal node, normal cortisol and aldosterone:renin ratio, marginally increased urinary normetanephrine, and a positive 131I MIBG radioisotope scan. Adrenalectomy normalized blood pressure and urinary metanephrines. Pathology showed a hyperplastic adrenal medulla associated to a multinodular cortical hyperplasia. Case 2 was a 62- yr-old female with progressive hypertension, a slight Cushing phenotype, non-suppressible hypercortisolism, normal urinary metanephrines, and bilateral adrenal nodes. Bilateral adrenalectomy and subsequent replacement normalized blood pressure and phenotypic stigmata. Pathology demonstrated bilateral cortical multinodular hyperplasia and medullary hyperplasia. The clinical study in both patients was negative for MEN. The apparently rare association of cortical and medullary lesions presented by both patients is probably overlooked in routine pathology exams, but should be meticulously searched since the crosstalk between the adrenal cortex and medulla may prompt dual abnormalities.

[Acute interstitial nephritis associated to lamotrigine use. Report of one case]. / Nefritis intersticial aguda y lamotrigina.

Acute interstitial nephritis is a mononuclear and sterile inflammation of the renal interstice caused by drugs, infections or immune phenomena. The clinical presentation is characterized by the triad of rash, fever and eosinophilia. We report a 32 years old man, in treatment with lamotrigine for depression, admitted to the hospital due to fever, abdominal pain, jaundice, cutaneus rash and malaise. Due to an oliguric renal failure of acute onset, a renal biopsy was done. The pathological study showed a granulomatous acute interstitial nephritis. He was started on hemodialysis and treated with cessation of the drug and corticosteroids, with complete recovery of the renal function.

Outcome of chilean patients with lupus nephritis and response to intravenous cyclophosphamide.

Several recent open studies suggest that the response rates of lupus nephritis to intravenous (IV) cyclophosphamide are lower than those observed in clinical trials. One explanation could be ethnic differences; for example, black patients more frequently have treatment-resistant lupus nephritis. Another could be the inclusion of patients who are noncompliant with therapy. From our register of 268 systemic lupus erythematosus (SLE) patients examined between 1973 and 1996, 61 patients were treated for proliferative lupus nephritis (17 had World Health Organization [WHO] type III and 43 had WHO type IV) and were followed through to 2001. Exclusion criteria included a serum creatinine level >3 mg/dL. In this retrospective study, we assessed renal outcome and survival with an endpoint of end-stage renal disease (ESRD) or death (Kaplan-Meier). In the univariate analysis, worse prognostic factors for survival were serum creatinine >1.3 mg/dL (p < 0.001), age <30 years (p < 0.001), class 2 renal function stage (p < 0.03), and renal biopsy activity index >7 (p < 0.02). In the subgroup of 26 patients treated with IV cyclophosphamide, survival at 5 and 10 years was 82% and 73%, respectively. The dosage of IV cyclophosphamide was slightly lower than usual and used for a shorter period (median = 23 months) than what is usually recommended because of the high frequency of complications. Renal outcome of the IV cyclophosphamide-treated patients was poorer than that reported in the National Institutes of Health series (ESRD: 15% versus 3%). This low survival rate could reflect the short course and lower doses of IV cyclophosphamide used or ethnic differences. These data emphasize the need for continuous research for better-tolerated drug schemes for treatment of our lupus nephritis patients.

Clinicopathologic implications of hMSH2 gene expression and microsatellite instability in prostate cancer.

Human mismatch repair (MMR) genes encode highly conserved interacting proteins that correct replication errors predisposing to hereditary gastrointestinal and genitourinary malignancies. We previously investigated expression of the prototype MMR gene hMSH2 in normal, benign prostatic hyperplasia and malignant prostate tissues (Velasco et al., Cancer 94:690-699, 2002). An association was detected between reduced hMSH2 staining and favorable outcome as determined by undetectable serum prostate specific antigen (PSA) after radical prostatectomy. We now investigate the association between clinicopathological variables and hMSH2 expression or microsatellite instability (MSI). A statistically significant association was found between tumors exhibiting MSI (MSI+ tumors) and lower preoperative serum PSA values, smaller tumor volumes and lower frequency of surgical specimens with extracapsular extension of tumor. No statistically significant association (P value less than or equal to 0.05) was found between hMSH2 staining and these clinicopathologic variables. Based on our analysis, we conclude that MMR deficiency has important clinicopathologic implications in prostate cancer. Furthermore, specific MMR genes may have different effects on prostate cancer biology.

[Eosinophilic fasciitis: report of 3 cases]. / Fascitis eosinofílica: experiencia en tres casos.

Eosinophilic fasciitis (EF) is a scleroderma-like disease of unknown etiology characterized by cutaneous swelling and induration that affects predominantly the extremities, elevated immune globulins, and peripheral eosinophilia. We report three patients with clinical, laboratory and pathologic characteristics of EF. The main symptoms were cutaneous and included "peau d' orange", groove signs and induration of the affected extremities. Two patients had skin changes after exercising and one had a hypothyroidism. None had extra-cutaneous manifestations. All patients had peripheral eosinophilia. Pathologic findings were thickening and inflammation of the fascia and normal epidermis. Only one patient was treated with prednisone 30 mg daily and showed only moderate improvement. There is no follow up information about the other two cases. EF is a rare disorder classified at times as scleroderma variants. However, its clinical picture, capillary microscopy findings, pathology and prognosis differentiate it from systemic sclerosis.

Differential expression of the mismatch repair gene hMSH2 in malignant prostate tissue is associated with cancer recurrence.

BACKGROUND: Mismatch repair (MMR) genes are responsible for coordinated correction of misincorporated nucleotides formed during DNA replication. Inactivating mutations in MMR genes have been described in sporadic cancers and a hereditary cancer predisposition syndrome. Mismatch repair deficiency causes instability at microsatellites and increased mutation rates. Although microsatellite instability (MSI) has been described in high-grade and lymph node positive prostate carcinoma specimens, an analysis comparing hMSH2 expression, MSI, and outcome in clinically organ confined prostate carcinoma has not been reported. METHODS: Immunohistochemical analysis of benign and malignant prostate tissue from 101 patients was performed using a monoclonal antibody specific for the hMSH2 protein. Expression was correlated with MSI using dinucleotide repeat markers and laser-captured microdissected DNA from normal and tumor cells. hMSH2 protein expression and MSI were assessed with respect to pathologic stage, Gleason score, and time to detectable serum prostate specific antigen (PSA) after prostatectomy in patients with clinically localized prostate carcinoma. RESULTS: In normal glands, hMSH2 staining was minimal to low and confined to the basal cell layer. In 32% of benign prostatic hyperplasia cases, hMSH2 staining was increased in the basal and luminal cell layers whereas 71% of cancer specimens had uniform moderate to high staining. Microsatellite instability was detected in 60% of absent to low staining and 26% of moderate to high staining prostate carcinoma specimens. Differential staining in benign versus malignant prostate tissues was statistically significant (P < 0.001) as was the correlation between absent to low hMSH2 staining and presence of MSI (P = 0.028). Decreased risk for PSA recurrence after radical prostatectomy correlated with absent to low hMSH2 staining in malignant prostate tissue but was only marginally significant (P = 0.05 for 24 month recurrence and P = 0.08 for overall time to PSA recurrence). CONCLUSIONS: The results of the current study demonstrate differential hMSH2 expression in benign and malignant prostate tissue. Moreover, hMSH2 expression is altered in a subset of clinically localized prostate carcinoma specimens independent of pathologic stage and Gleason pattern. A statistically significant correlation between hMSH2 immunohistochemical staining intensity and MSI also was identified in prostate carcinoma specimens. Furthermore, the time to cancer recurrence as determined by detectable serum PSA after prostatectomy was associated with hMSH2 staining intensity. Taken together, our results suggest that hMSH2 gene expression in prostate carcinoma may be a useful prognostic marker for outcome in men with clinically organ confined prostate carcinoma.

Cintigrafía renal en la evolución postoperatoria del trasplante renal / Renal radionuclide imaging in the postoperative evaluation of the renal transplant

Para evaluar la utilidad de la Cintigrafía Renal Dinámica (CRD) en trasplante renal, se analizó retrospectivamente 142 estudios efectuados en 61 trasplantes en el postoperatorio reciente. La sensibilidad y especificidad del procedimiento en la detección de alteraciones de perfusión y/o función renal fue de 100%. Se diagnosticó correctamente el 87,2% de las complicaciones parenquimatosas. Cinco de los seis episodios, en los cuales no fue posible hacer una clasificación etiológica no se dispuso de estudio basal. La visualización de un área fría o la ausencia de perfusión asociada a la "no visualización" del riñón trasplantado son signos cintigráficos de mal pronóstico. Concluimos que el estudio cintigráfico es un excelente método para pesquizar precozmente la existencia de complicaciones en la evolución del trasplante renal

La glomérulonefritis aguda en el niño / Acute glomerulonephritis in children

Se analiza una serie de 45 niños de 2 a 15 años, promedio 7,7 años con cuadro clínico e histológico de GNA, que fueron seguidos hasta por treinta meses. Se realizó en ellos dos estudios histológicos (ML, ME, IN) en biopsias practicadas al comienzo de la enfermedad (promedio 20 días) y la segunda a los 194 ó 590 días en promedio. En el estudio clínico se destaca la normalización del edema a los 8,8 días y la hipertensión a los 4,3 días. Sólo se obtuvo estreptococo betahemolítico en cuatro enfermos, lo que puede relacionarse con el tratamiento antibiótico recibido previamente por estos niños. En el examen de orina un alto porcentaje no presentó hematuria (44%) o proteinuria (49%) en el examen de ingreso y sólo 29,9% tuvo cilindruria. La albuminemia tuvo valores bajo lo normal en el 35,6% de los niños, alfa 2 globulina y gamaglobulina con valores normales en todos. El complemento C3 fue en promedio 41,3m% y sólo 3 enfermos tenían valores sobre 100mg%. Se describen las curvas de antiestreptolisina O (ASO) y STZ en estos niños y se destaca la necesidad de determinaciones seriadas tanto de C3 como ASO y STZ. En el análisis de 194 contactos se obtuvo sólo 5 frotis faríngeos y 10 cutáneos positivos a estreptococo betahemolítico e estafilococo. En el estudio histológico se encontró buena correlación entre la intensidad de las lesiones en microscopía de luz (ML) y microscopía electrónica (ME). En esta última se destaca en la primera biopsia que todos tenían depósitos inmunes intramembranosos segmentarios y 84% con depósitos en joroba. La segunda biopsia mostró una disminución apreciable de la intensidad de las lesiones a los 6 meses y en las realizadas en promedio a los 590 días se encontró ya tres casos que pueden considerarse normales desde el punto de vista histológico. Otros tres con lesiones...

Hematuria esencial de origen glomerular / Essential hematuria of glomerular origin

Se describen 32 niños con hematuria recurrente idiopática, de quienes se tomaron biopsias renales que fueron estudiadas morfológicamente mediante microscopio óptico, electrónico e imunoflorescencia en un hospital de Santiago entre los años 1972 a 1982. En 21,5% de los casos se encontró enfermedad de Berger, en 66% alteraciones mínimas glomerulares y en 12,5% membrana basal delgada glomerular. Se propone una clasificación en sub-grupos para las alteraciones mínimas glomerulares. Se concluye que esta es en general una afección benigna en la infancia, que habitualmente no requiere de biopsia renal, con excepción de la enfermedad de Berger y la membrana basal delgada en los que se ha descrito excepcionalmente progresión a insuficiencia renal