Medullary Sponge Kidney on Retrograde Pyelography / 전남의대학술지

A woman aged 31 had recurrent urinary tract infection with bloody urine. A series image of medullary sponge kidney presented by intravenous urography (IVU) was detected dynamically by retrograde pyelography (RP). Other than ultrasonography and IVU, RP is also a reliable method to detect medullary sponge kidney.

Medullary Sponge Kidney on Retrograde Pyelography / 전남의대학술지

A woman aged 31 had recurrent urinary tract infection with bloody urine. A series image of medullary sponge kidney presented by intravenous urography (IVU) was detected dynamically by retrograde pyelography (RP). Other than ultrasonography and IVU, RP is also a reliable method to detect medullary sponge kidney.

Medullary Sponge Kidney Detected in the Pediatric Age

PURPOSE: Medullary sponge kidney (MSK) is a rare congenital disease characterized by diffuse ectasia or dilatation of precalyceal collecting tubules. MSK incidence and prevalence in the general population is uncertain and only a few patients are reported especially in the pediatric age. There has been increasing reports of patients with MSK who have other malformative disorders. Also several case reports concerning about etiological association of some genes. METHODS: Collaborative study through nation-wide survey was done to investigate the incidence and etiological association of some genes such as GDNF gene, ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children. RESULTS: Four cases of MSK who have various other malformative disorders were collected. There are no mutations of GDNF gene, ATP6V1B1, ATP6V0A4 gene in all patients. CONCLUSION: MSK is one of the very rare diseases in pediatric age. The etiological association of GDNF gene , ATP6V1B1, ATP6V0A4 gene in developing MSK in Korean children is not proved.

Metabolic Risk Factors and Treatment Effects of Extracorporeal Shock Wave Lithotripsy in the Medullary Sponge Kidney with Nephrocalcinosis / 대한비뇨기과학회지

PURPOSE: The medullary sponge kidney(MSK) is a congenital anomaly of the renal medulla characterized by fusiform and cystic dilatation of the collecting tubules confined to the renal pyramids. The aim of this study is to evaluate the metabolic risk factors and effect of extracorporeal shock wave lithotripsy(ESWL) and complications in an MSK with nephrocalcinosis. MATERIALS AND METHODS: We compared stone metabolic studies between 45 patients with MSK and 279 non-MSK patients. We evaluated the effect of ESWL for MSK. Group A patients received ESWL and follow-up within 3 months and Group B patients had a period of cessation for more than 6 months. The frequency of complications between group A and group B patients was compared. RESULTS: There were no statistically differences between the MSK group and non-MSK group of patients in the stone metabolic studies. Among the 45 patients with MSK, 26(58%) had one or several of the following metabolic disorders: hypocitraturia(36.7%), hypercalciuria(18.3%), hyperoxaluria(16.3%), hyperuricosuria(16.3%), low urine volume(12.4%). The percentage of patients that experienced more than 75% of a reduction of stone burden on plain films was 48.8%, 25?75% of the reduction was 35.6%, and less than 25% of the reduction was 15.6%. The most common complication was renal colic(17.7%). Renal colic and stone re-growth was shown to have a more significant association with group B patients as compared to group A patients. CONCLUSIONS: ESWL reduced the stone burden more than 75% in 48.8% of MSK patients with nephrocalcinosis, and prevented flank pain, and obstructive uropathy. Therefore, we plan to develop treatments that are more effective.

Diversity of initial manifestations in renal tubular acidosis / 대한내과학회지

BACKGROUND: Diagnosis of RTA (renal tubular acidosis) is not easy due to its nonspecific and various manifestations. To find out the clues to diagnosis, we investigated initial manifestations, laboratory features and clinical course of RTA patients. METHODS: Thirty-seven patients with RTA type I or II, whose follow-up period was over 6 months were included in the study. We reviewed their medical records retrospectively. RESULTS: Male to female ratio was 5:32 and the average age at the time of diagnosis was 38.7 (15~60). Twenty-five patients had RTA type I, nine had type II, and three had both. The average follow-up period was 6.4 years. Initial manifestations were asthenia (54%), nausea (46%), urinary stone (24%), paresthesia (24%), lower extremity weakness (22%), and paralysis (11%). Underlying diseases at the time of diagnosis include Sjogren's syndrome (14%), SLE (8%), drug-induced nephropathy (11%), diabetic nephropathy (5.4%), Sjogren's syndrome combined with SLE (2.7%), and medullary sponge kidney (2.7%). Laboratory tests revealed acidosis with hypokalemia (59%), acidosis without hypokalemia (14%), and hypokalemia without acidosis (24%). The level of total CO2 was 22 mmol/L or lower in 27 patients. The Na:Cl ratio on the average was 1:1.26 and for 33 patients below 1:1.35. Renal function deteriorated in 8 patients and 7 of them had underlying diseases. Urinary stone developed in 2 patients with RTA type I. CONCLUSION: When patients with nonspecific symptoms show decreased levels of serum total CO2, potassium, or Na:Cl ratio, RTA should always be considered.

Medullary sponge kidney.

Medullary sponge kidney is a benign asymptomatic developmental anomaly of the kidney mostly seen in adult females. Presentation in childhood is uncommon. Urinary tract infection, nephrolithiasis, hematuria and hypercalciuria are the common complications. We report a eleven-year-old female child who presented with recurrent urinary tract infection and nephrolithiasis and was found to have bilateral medullary sponge kidney.

A Case of Distal Renal Tubular Acidosis Associated with Medullary Sponge Kidney

Renal tubular acidosis is a clinical state of systemic hyperchloremic acidosis resulting from impaired urine acidification. Medullary sponge kidney is a renal parenchymal malformation characterized by cystic dilatation of the collecting ducts. Although medullary sponge kidney is a congenital disease, it is rarely identified in childhood and is usually discovered in adulthood. Medullary sponge kidney patients may have defects in urinary acidification and concentration mechanism. We experienced a case of distal renal tubular acidosis associated with medullary sponge kidney. So, we report a case of distal renal tubular acidosis associated with medullary sponge kidney with a brief review of the related literature.

Rim esponja medular, litíase, hipercalciúria e hiperuricosúria em criança / Medullary sponge kidney, nephrolithiasis, hypercalciuria and hyperuricosuria in child. Report of a case and follow-up

O rim esponja medular é uma patologia rara em crianças, que pode se manifestar com hematúria e litíase. Relatamos o caso de uma criança com rim esponja medular, litíase, hipercalciúria e hiperuricosúria, cujo tratamento prolongado evitou a recidiva da litíase.

Hipercalciuria: Presentación clínica, laboratorio y tratamiento / Hypercalciuria: clinic presentation, laboratory and treatment

El propósito del presente trabajo es el de reportar la experiencia de la consulto del servicio de nefrología del Hospital "J.M delos Ríos" en el diagnóstico y manejo de esta entidad, desde enero de 1991 hasta diciembre de 1991, de un total de 4.800 pacientes evaluados, se obtuvieron 653 pacientes con una relación calcio/creatinina en orina mayor de 0,11. El antecedente familiar más importante que se encontró fue litiasis renal (39,5 por ciento). Las formas de presentación clínica más frecuentes fueron: Infección Urinaria (56,6por ciento), dolor abdominal (56,8por ciento), hematuria microscopica (43 por ciento), hematuria macroscopica (31,8 por ciento) y retardo del crecimiento (12,9 por ciento). Dentro de las patologías renales asociadas predominaron las siguientes: Litiasis Renal (39,8 por ciento) y riñon de esponja (29,5 por ciento). El manejo médico incluyó: Regulación dietética, tiazidas, citrato de potasio o bicarbonato de sodio, segun las necesidades de cada caso

Urolitiasis en la edad pediátrica: presentacion de 72 casos / Urolithiasis in the pediatric age: report of 72 cases

La litiasis urinaria en el niño ha demostrado, en nuestro medio, una incidencia mayor a la reportada en la mayoría de los estudios publicados hasta ahora. En el presente trabajo reportamos nuestra experiencia en 71 niños con diagnóstico de urolitiasis. Las manifestaciones clínicas más frecuentes fueron la hematuria y el dolor abdominal. Encontramos un predominio significativo de la litiasis del tracto urinario superior siendo sólo dos, los cálculos de localización vesical. En la totalidad de los casos se logró evidenciar un factor etiológico para la litiasis, siendo más importantes las causas de tipo metabólico acidosis tubular distal e hipercalcinuria). Las anomalías estructurales del tracto urinario también constituyen una causa importante de litiasis en nuestros pacientes, siendo la más frecuente de ellas el Riñón de Esponja. En la exploración diagnóstica del niño con urolitiasis debe incluirse la evaluación de la función renal, especialmente en lo que refiere a la excreción urinaria de calcio y la capacidad de acidificación distal. Es importante la ultrasonografía renal como método diagnóstico debido a su mayor sensibilidad para evidenciar calcificaciones renales incipientes. Se hace énfasis en la prevención de la urolitiasis en el niño mediante la educación, en cuanto a evitar las dietas ricas en lacticíneos y mediante la estimulación de una ingesta adecuada de líquidos, especialmente en las regiones más cálidas de nuestra geografía

Nefrocalcinosis / Nephrocalcinosis

Se define como nefrocalcinosis a los depósitos de calcio en el parénquima renal pesquisados por ecografía, radiografía o ambos, reservándose calcificaciones para el depósito microscópico. Esta afección es extremadamente rara. Se presentan 6 pacientes con nefrocalcinosis demostrada por eco y radiología en 4 y 2 por ecografía, coincidiendo estos últimos con los asociados a hipercalcemia. Se presentan 3 pacientes con ATR tipo I, uno por ectasia canalicular precaliciaria o enfermedad de Cacchi-Ricci y dos intoxicaciones por vitamina D. El tratamiento debe estar dirigido a la etiología ya se ésta ATR o hipercalcemia. La enfermedad de Cacchi-Ricci no tiene tratamiento. El pronóstico estaría condicionado por la enfermedad base. Aparentemente la nefrocalcinosis radiológica es irreversible y puede progresar a la insuficiencia renal terminal, mientras que el hallazgo ecográfico puede remitir en los pacientes con intoxicación por vitamina D

Estudio metabólico en 100 pacientes con urolitiasis con especial énfasis en la diferenciación de las hipercalciurias / Metabolic study in 100 with urolithiasis patients specially in differentiating hypercalciuria

Se reportan los hallazgos de 100 pacientes con urolitiasis estudiados con un protocolo simple factible de realizarse en laboratorios y que permite con un mínimo margen de error diagnóstico las causas más frecuentes de formación de cálculos. La edad promedio de los pacientes fue 39 años predominante el sexo masculino 2 a 1. El 40 por ciento presentaron antecedentes familiares de litiasis. Las determinaciones en sangre mostraron: calcio 9,1 mas menos 0,1 mg por ciento; fósforo 3,6 mas menos 0,1 mg por ciento; ácido úrico en varones 5,0 mas menos 0,2 y en mujeres 3,8 mas menos 0,2 mg por ciento. La diferencia fue estadísticamente significativa (p menor que 0,01). La creatinina sérica fue de 0,9 mas menos 0,03 mg por ciento. En 41 pacientes se detectó hipercalciuria idiopática como único defecto. En 20 pacientes la hipercalciuria se encontró asociada a hiperuricosuria. En 26 pacientes no se detectó defecto alguno y en 8 sólo se encontró hiperuricosuria aislada. La prueba de tolerancia aguda se realizó 50 pacientes con hipercalciuria, encontrándose que el 50 por ciento eran renales, el 28 por ciento absurtivos y el 22 por ciento con ambos defectos; el 49 por ciento de cálculos analizados fueron oxalato de calcio puro, el 20 por ciento oxalato de calcio y fosfato de calcio y el 14 por ciento oxalato de calcio y uratos. El 9 por ciento de cálculos se encontró solamente ácido úrico y en 8 por ciento estruvita. Se detectaron 5 pacientes con hipercalciuria secundaria; dos con hiperparatiroidismo, uno con acidosis tubular distal y 2 con riñones esponjosos. De 78 urocultivos realizados fueron positivos 25 (32 por ciento). La escherichia coli fue el patógeno más comunmente aislado en 80 casos. En la radiografía los cálculos se encontraron con mayor frecuencia en los riñones y/o uréteres en el 80 por ciento de los casos; y solamente en 2,5 por ciento la litiasis se encontró en vejiga. El 12 por ciento se detectó estrechez úteropiélica uni o bilateral, duplicación total o parcial del sistema colector en 5 por ciento, deformaciones calicilares en 10 por ciento, cifra muy superior a la encontrada en la población sana. En 3 por ciento riñón esponjoso medular y en 5 por ciento litiasis coraliforme. Se concluye que en nuestro medio es factible diagnosticar y tipificar el defecto metabólico causal en formadores de cálculo sin tener que recurrir a estudios sofisticados y costosos

Rim esponja medular / Medullary sponge kidney

Rim esponja medular (REM) é uma anormalidade congênita, rara, da medula renal caracterizada por dilatações e malformações císticas dos túbulos coletores. REM é assintomático, de curso benigno, na fase inicial, e o diagnóstico essencialmente radiológico. em fases tardias, tem alta morbidade quando complicado com cálculos ou infecção, que acabam clinicamente por gerar cólicas renais de repetição, hematúria ou mesmo pielonefrite crônica. Não há relato na literatura médica brasileira sobre REM. Apresentamos dois casos diagnosticados na Clínica de Nefrologia do Hospital do Servidor Público Municipal - HSPM - São Paulo, salientando as manifestações radiológicas na ausência de complicações, uma vez que os casos publicados na literatura médica mundial sempre se apresentaram associados à litíase ou outra condição patológica formadora de cálculos.

Medullary sponge kidney (REM) is a congenital abnormality, rare, renal medulla and malformations characterized by cystic dilatation of collecting tubules. REM is asymptomatic, a benign course, in the initial phase, and essentially radiological diagnosis. in late stages, when they have high morbidity or infection complicated calculations, you end up generating clinically recurrent renal colic, haematuria or chronic pyelonephritis. There is no report in the Brazilian medical literature about REM. We present two cases diagnosed in the Clinic of Nephrology, Hospital do Servidor Publico Municipal - HSPM - São Paulo, highlighting the radiographic manifestations in the absence of complications, since the cases published in medical literature had always been associated with gallstones or other pathological condition forming calculations.

Medullary Sponge Kidney

Medullary sponge kidney is uncommon disease and rare in children. It is characterized by localized dilatation of collecting ducts in renal pyramids. The diagnosis of medullary sponge ki-dney depends mainly upon intravenous and retrograde pyelography. This paper presented a case of medullary sponge kidney found in a 9-year-old boy during the study about enuresis, and a brief review of literature was made.