Síndrome de McKittrick-Wheelock: una causa infrecuente de hipokalemia e injuria renal aguda. Caso clínico / McKrittick-Wheelock syndrome: report of one case

McKittrick-Wheelock syndrome is caused by chronic water and electrolyte hypersecretion from an intestinal tumor, usually a villous adenoma, located in the rectum or sigmoid. Patients often have dehydration, hypovolemic shock and kidney failure associated with hypokalemia, hyponatremia, hypochloremia and metabolic acidosis. We report a 62-year-old male, suffering chronic diarrhea for eight years who was admitted after a syncope. He had severe hypokalemia, hyponatremia, metabolic acidosis, hypovolemia and acute renal failure. After his metabolic disorders were corrected, a colonoscopy showed a large rectosigmoid tumor with the characteristics of a villous adenoma. During the follow up after the complete tumor resection, the patient has remained asymptomatic.

Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management

ABSTRACT Main findings: A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinary team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management.

Hidrometrocolpos: presentación de un caso / Hydrometrocolpos: presentation of a Case

El hidrometrocolpos es una obstrucción a nivel del tracto genital femenino, que aparece en la etapa neonatal como una masa en hemiabdomen inferior, por encima de la sínfisis del pubis. Presentamos recién nacida pretérmino, con edad gestacional de 33,4 semanas por método de Capurro, producto de parto eutócico, apgar 2/5, peso 1954 gr, que presenta masa abdominal constatada durante la reanimación cardiopulmonar realizada inmediatamente después del nacimiento. Al examen físico posterior se observa la presencia de gran aumento de volumen en región perineal que afecta los genitales externos, borramiento del pliegue interglúteo, e himen imperforado. Durante el estudio se constata riñón poliquístico del lado derecho e hidronefrosis severa del lado izquierdo, así como gran masa intrabdominal de contenido líquido. Se plantea el diagnóstico de hidrometrocolpos y se procede a la realización de intervención quirúrgica que consistió en perforación del himen, obteniéndose la salida de aproximadamente 200ml de líquido procedente de la vagina. Después de lo cual se produce la regresión de las estructuras anatómicas paulatinamente.

The hydrometrocolpos is an obstruction at the level of female genital tract that appears during the neonatal stage as a mass in the lower hemi abdomen, over the synphisis of the pubis. It is presented a 33, 4-weeks-gestational-newborn girl, by means of Bud method, due to a euthocic birth, apgar 5/2, and weight 1954gr, who has an abdominal mass noted during the Cardiopulmonar reanimation made immediately after her birth. In the later physical exam it is observed the presence of a great increasement of volume in the perineal area that affects the external genitals, erasing of the intergluteal fold, imperforated hymen. During this study it is also noted polycystic kidney disease in the right side and a sever hydronephrohydrosis in the left side, as well as a great intra-abdominal mass with a liquid content. The diagnosis of hydrometrocolpus is posed and then a surgery is made to pierce the hymen, obtaining the outlet of 200ml of liquid approximately, coming from the vagina. After this, the regression of the anatomical structures is gradually produced.

Hidrocolpo por ectopia ureteral em hemivagina associado a displasia renal: relato de caso e revisão da literatura / Congenital hydrocolpos due to ectopic ureter in hemivagina associated with renal dysplasia: a case report and review of the literature

Os autores apresentam um caso de displasia renal associada a ectopia ureteral vaginal homolateral, com uretero-hidronefrose contralateral devida a compressão por hidrocolpo causado pela ectopia e por um septo vaginal oblíquo. Os exames de imagem utilizados foram urografia excretora, cistografia, ultra-sonografia, tomografia computadorizada e cintilografia renal com Tc-99m-DMSA. Foi realizada revisão da literatura sobre casos de hidrocolpo associado a malformações urinárias.

The authors report a case of congenital hydrocolpos due to vaginal malformation (vaginal septae) associated with ectopic ureter and renal displasia. Several imaging diagnostic procedures (ultrasonography, nuclear medicine, intravenous urography and computed tomography) were performed. A review of the literature on hydrocolpos and its association with urinary malformations is also presented