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Corresponding Author Edwin M. Keywords: Autosomal dominant polycystic kidney disease Nephrectomy Transplantation. It is characterized by progressive cyst formation in both kidneys, often leading to end-stage kidney disease. Indications for surgical removal of an ADPKD kidney include intractable pain, hematuria, infection, or exceptional enlargement and small abdominal cavity hampering implantation of a donor kidney. Autosomal dominant polycystic kidney disease ADPKD is characterized by progressive cyst formation in the kidneys leading to massive kidney enlargement.
Over time, the kidney function deteriorates as functional parenchyma is replaced by growing cysts. Renal cysts may rupture leading to hematuria and pain.
A pyogenic infection may also be located in these cysts. ADPKD patients with chronic pain related to the enlarged kidneys, recurrent hematuria or therapy-resistant cyst infection may benefit from nephrectomy. In addition, in some patients, the cystic kidneys are exceptionally enlarged leaving too little abdominal space to allow for kidney transplantation. Here, we report nephrectomy of an extremely enlarged ADPKD kidney prior to transplantation in a year-old man. A year-old male patient with ADPKD visited our outpatient clinic while preparing for renal replacement therapy.
His medical history included hypertension and myocardial infarction, complicated with aorta dissection, classified as a Stanford type A. He had experienced multiple periods with hematuria and kidney pain, most likely caused by recurrent cyst bleedings. He inherited ADPKD from his paternal side; his father started renal replacement therapy at 42 years of age and died at 46 years due to complications during a second kidney transplantation.
Physical examination of the patient revealed a man weighing 99 kg lb and measuring cm 6 feet 2. His waist and hip circumferences were and cm, respectively. Since a potential living unrelated donor allograft was found for the patient, he was evaluated to undergo kidney transplantation. Magnetic resonance imaging showed exceptionally enlarged kidneys in a small abdominal space total kidney volume is 10, ml, left kidney 5, ml and right kidney 4, ml fig.
Of note, the total kidney volume was measured on T2-weighted coronal images using the commercially available software Analyze Direct 8.
Intra- and interreviewer coefficients of variation were 2. In addition, several small cysts were found in the liver.Magnificent Passes
A left-sided nephrectomy was performed to allow for kidney transplantation. Postoperatively, no complications were reported and he was discharged from the hospital in a good condition 7 days later. The removed kidney weighed 8. Subsequent dissection of the removed kidney online suppl. Some of these cysts were filled with clear pre-urine, others with hemorrhagic fluid and again others with a chocolate-like substance. No pathological s of pre- malignancy were Exceptional Dominant.
After the nephrectomy, the patient was on temporary hemodialysis until he received a living unrelated renal allograft 3 months later. One week after the transplantation, he experienced an episode of vascular allograft rejection type IIA, which was successfully treated with antithymocyte globulin and plasmapheresis.
Several cysts are present in the liver. ADPKD is the most common hereditary kidney disease. Most affected subjects have progressive renal function decline and need renal replacement therapy between their 40th and 70th year of age [ 2 ]. However, this case is extraordinary because of the massive kidney enlargement. The maximal length of the removed kidney was 48 cm 19 inch. The weight of the removed kidney was 8. This indicates that the volumetric mass density mass per unit volume of the removed kidney was much higher than water. This may be due to the fibrotic deposition, multiple hemorrhages and cloths.
To our knowledge, only one case reported a patient with ADPKD that had kidneys with higher total weight than the present case [ 3 ]. Transection of the kidney revealed the presence of clear and cloudy fluid, to chocolate-brown semisolid contents in the cysts, consistent with the variable aspects of the cysts in the magnetic resonance image fig.
The dark contents of some cysts probably reflect passed bleeding events that may have been associated with a sudden increase of tension on the kidney capsule, a cause for pain and discomfort reported by the patient, although symptomatic episodes probably underestimate the true frequency of cyst hemorrhage.
In the removed kidney, most renal parenchyma in both the cortex and the medulla had been replaced completely by cysts and extensive scar tissue. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions.
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