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Year : 2019  |  Volume : 5  |  Issue : 4  |  Page : 79-82

A missed cause of abdominal pain and malnutrition in autosomal dominant polycystic kidney disease

1 Department of Nephrology and Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission13-Mar-2020
Date of Acceptance06-Apr-2020
Date of Web Publication09-Jun-2020

Correspondence Address:
Dr. Smita Divyaveer
Department of Nephrology, Post Graduate Institute of Medical Education and Research, Sector 12. Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jrnm.jrnm_4_20

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A 62-year-old lady having autosomal dominant polycystic kidney disease (ADPKD) with gradual progression of chronic kidney disease (CKD) to end-stage renal disease presented with anorexia and dull-aching continuous abdominal discomfort. She had no response to conservative management and was started on maintenance hemodialysis. She followed up 3 months later. Her symptoms had only marginally improved initially despite continuing dialysis, and she continued to be malnourished. She presented to the emergency department with worsening abdominal pain and obstipation. Contrast-enhanced computed tomography abdomen was done which showed signs of abdominal tuberculosis with bowel obstruction. She was taken up for surgery. On postoperative day 2, she had refractory septic shock and cardiac arrest and could not be revived. Dull-aching chronic abdominal pain in ADPKD is common and is attributed to increased kidney sizes. With progression of CKD to uremia, anorexia is common. However, other causes of abdominal pain and malnutrition may be overlooked. In our case, the patient had abdominal tuberculosis which was the cause of persistent anorexia and malnutrition. Close follow-up of nutritional status and evaluation of malnutrition in patients with CKD is of paramount importance.

Keywords: Autosomal dominant polycystic kidney disease, malnutrition, pain, tuberculosis

How to cite this article:
Prabakaran R, Sethi J, Divyaveer S, Thirunavukkarasu B, Bal A, Kohli H S. A missed cause of abdominal pain and malnutrition in autosomal dominant polycystic kidney disease. J Renal Nutr Metab 2019;5:79-82

How to cite this URL:
Prabakaran R, Sethi J, Divyaveer S, Thirunavukkarasu B, Bal A, Kohli H S. A missed cause of abdominal pain and malnutrition in autosomal dominant polycystic kidney disease. J Renal Nutr Metab [serial online] 2019 [cited 2022 May 26];5:79-82. Available from: http://www.jrnm.in/text.asp?2019/5/4/79/286279

  Introduction Top

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited multisystem disorder characterized by multiple cysts in both the kidneys often accompanied by cysts in the liver and other organs such as the pancreas and spleen in decreasing order of frequency. It is known to be caused by mutation in PKD1 and PKD2 gene which code for polycystin-1 and polycystin-2, respectively. It is the fourth most common cause of end-stage renal failure worldwide.[1] The most common clinical manifestation of ADPKD is pain.[2] Acute pain in ADPKD is usually attributed to cyst rupture, hemorrhage, infection, and nephrolithiasis.[3] Chronic pain although less defined has been attributed to mechanical low back pain due to increased lumbar lordosis, cyst enlargement, pressure effects and neurological causes.[2] An ADPKD patient presenting with chronic pain is usually attributed to the disease process per se, and pain due to other intra-abdominal pathologies can be overlooked.

  Case Report Top

A 62-year-old lady was diagnosed with ADPKD and chronic kidney disease (CKD) Stage 4 in September 2013 with a positive family history (her 36-year-old daughter and 29-year-old son were affected, and her elder brother developed end-stage renal disease (ESRD) due to ADPKD and expired at 60 years of age due to coronary artery disease). She had dull-aching abdominal pain for 8–9 months. She had a gradual rise in serum creatinine to the value of 5 mg/dl in September 2019. She presented with anorexia and ongoing dull-aching continuous abdominal discomfort that relieved partially with analgesics. She had no response to conservative management and continued to have anorexia and weight loss of about 2 kg in 3 months. The serum albumin at this point was 3.1 g/dl. In view of possibility of uremia, she was started on maintenance hemodialysis. She presented 3 months later to the emergency department with a history of progressively increasing dull-aching, diffuse abdominal pain for 2 weeks along with abdominal distension and obstipation for 2 days. On admission, the patient had pallor, pulse rate of 116/min, blood pressure of 130/70 mmHg, and diffuse tender abdomen with absent bowel sounds. Blood analysis revealed hemoglobin of 10.6 g/dl, total leukocyte count – 6700 cells per cumm, platelets – 35000, prothrombin time 18.8 s, activated partial thromboplastin time – 39 s, serum sodium – 136 meq/L, serum potassium – 3.6 meq/L, serum urea – 72 meq/L, creatinine – 4.2 meq/L, serum albumin – 2.6 gm/dl, and amylase – 52 IU/L. Urine analysis revealed 1+ albumin, 4–5 pus cells, and no red blood cells. Blood culture and urine culture were sterile. X-ray chest was normal, and X-ray abdomen revealed dilated bowel loops.

Contrast-enhanced computed tomography (CT) of the abdomen and pelvis revealed enlarged liver of size 22 cm with multiple cysts, enlarged kidneys of 13 cm size with parenchyma replaced by multiple cysts with few showing hyperdense content and tiny calcifications [Figure 1] and dilated small bowel loops with hypodense mild diffuse thickening and adherence of mid- and distal ileal loops, mesenteric thickening, and collapsed large bowel suggestive of small intestinal obstruction [Figure 2] and [Figure 3]. Intra-abdominal and mediastinal lymphadenopathy was absent. She was started on empirical intravenous antibiotics and hemodialysis as per institutional protocol. In view of subacute intestinal obstruction and suspected bowel ischemia, the patient was taken up for surgery. She underwent adhesiolysis and loop ileostomy. Ileal biopsy taken at the time of surgery revealed multiple necrotizing epithelioid cell granulomas in the serosal aspect [Figure 4], with Ziehl–Neelsen stain being positive suggestive of Mycobacterium tuberculosis infection [Figure 5]. She was started on category one antituberculous therapy. However, on postoperative day 2, the patient went into refractory septic shock requiring multiple inotropic supports, and serum procalcitonin in the postoperative period was found to be 100 ng/ml. She expired on postoperative day 4.
Figure 1: Sagittal section of contrast-enhanced computerized tomography of the abdomen showing thickened mesentery with matted and dilated small bowel loops

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Figure 2: Cross-sectional of contrast-enhanced computerized tomography of the abdomen showing thickened mesentery with matted and dilated small bowel loops

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Figure 3: Contrast-enhanced computerized tomography of the abdomen showing multiple cysts replacing renal parenchyma, some with hyperdense contents, and multiple cysts in the liver

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Figure 4: Low-magnification image showing ileal mucosa with intact lining and presence of multiple necrotizing epithelioid cell granulomas in the serosal aspect. Arrow indicating a granuloma (H and E; ×20)

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Figure 5: Ziehl–Neelsen stain highlights slender acid-fast bacilli adjacent to a giant cell (arrow pointed toward the bacilli) (Ziehl–Neelsen; ×1000)

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  Discussion Top

Pain is one of the most disabling symptoms of ADPKD and affects 60% of those with the diagnosis.[4] Massively enlarged kidneys are associated with early satiety, chronic pain, and dyspnea. Chronic pain has been attributed to distension of renal and liver capsule, traction on the renal pedicle, and compression of nearby structures[5] and is hypothesized to be maintained by the abnormal activity of sensory and autonomic neurons innervating the kidney. Chronic pain has been associated with poor quality of life reflected by low physical component summary, sleep deprivation, fatigue, and anxiety[6] and can be associated with unexpected exacerbation which can cause limitation in day-to-day activities.[7] In addition, acute episodes of pain due to cyst hemorrhage, rupture, and nephrolithiasis can complicate the picture. The most sites of pain in ADPKD include lower back, abdomen, head, chest, and legs in decreasing order.

Therapeutic modalities for management of pain in ADPKD include systemic analgesics, acupuncture, ice massage, heating pads, transcutaneous electrical nerve stimulation, autonomic plexus blockade, acupuncture, psychobehavioral measures, neuromodulation by spinal cord stimulation, neuraxial opioids, and local anesthetics. Surgical modalities for pain control include the least invasive methods such as cyst aspiration with or without sclerosis,[8] fenestration of kidney cysts which is reported to significantly relieve pain in 85%–90% of the cases immediately but resurgence of pain may occur later. In refractory severe pain[9] laparoscopic renal denervation,[10] nephrectomy,[11] and transcatheter arterial embolization have been tried as a last resort in ESRD patients who are not suitable for nephrectomy.[12]

In addition to the above-described causes of pain in ADPKD, some rare causes of pain which have been reported so far include recurrent acute pancreatitis due to pancreas divisum,[13] intestinal obstruction usually as a result of renal cysts per se,[14] or diverticulitis.[15]

Intestinal obstruction in ADPKD is a rare phenomenon. To the best of our knowledge, about 14 cases have been reported so far, the most common cause has been a compression of intestines by renal cysts, and approximately 5 of those cases required nephrectomy to relieve the obstruction.[16]

Our patient had chronic abdominal pain in a frequency two to three times per month ever since the time of diagnosis of ADPKD which was conservatively managed. Two weeks before the date of emergency visit, she had recurrence of abdominal pain which was not in a higher intensity to start with but gradually increased in nature along with the occurrence of constipation and obstipation which developed later. She did not report any fever, any respiratory symptoms, or contact history of tuberculosis. Contrast CT showed thickening and dilatation of ileal loops, which is one of the most common sites of intra-abdominal tuberculosis.

Our patient had a mixed type of peritoneal tuberculosis. The most common CT findings seen in 70%–90% of cases in peritoneal tuberculosis include lymph node enlargement and densification of the mesenteric root flat planes, smooth peritoneal thickening, and ascites, out of which the latter three were present in our patient.[17]

Despite surgical measures, the patient expired on postoperative day 4 due to refractory septic shock which could be explained by bacteremia and endotoxemia that occurred due to the breach of the gut mucosal barrier due to extensive adhesion-related obstruction and bowel ischemia.

A close follow-up of nutrition status and persistent anorexia in a patient with CKD may indicate causes other than uremia. A high index of suspicion may clinch the diagnosis early and prevent complications.

To conclude, the nutrition status of patients with CKD whether on dialysis or not, should be closely monitored and reviewed timely. If patients have persistent malnutrition despite appropriate therapy for chronic kidney disease and are on adequate diet alternative causes beyond CKD itself should be evaluated to avoid missing other concurrent diseases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, et al. Autosomal-dominant polycystic kidney disease (ADPKD): Executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 2015;88:17-27.  Back to cited text no. 1
Bajwa ZH, Gupta S, Warfield CA, Steinman TI. Pain management in polycystic kidney disease. Kidney Int. 2001;60:1631-44.  Back to cited text no. 2
Thomas M Coffman Ronald J. Molitoris, Bruce A, RJ Falk, Eric G Nelson, Robert W Schrier Schrier's diseases of the kidney 2015. Schrier's Diseases Of The Kidney. 9th ed. Philadelphia: Wolters Kluwer, p. p. 519-56.  Back to cited text no. 3
Gabow PA. Autosomal dominant polycystic kidney disease, more than a renal disease. Am J Kidney Dis 1990;16:403-13.  Back to cited text no. 4
Hogan MC, Norby SM. Evaluation and management of pain in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis 2010;17:e1-16.  Back to cited text no. 5
Rizk D, Jurkovitz C, Veledar E, Bagby S, Baumgarten DA, Rahbari-Oskoui F, et al. Quality of life in autosomal dominant polycystic kidney disease patients not yet on dialysis. Clin J Am Soc Nephrol 2009;4:560-6.  Back to cited text no. 6
Heiwe S, Bjuke M. An evil heritage: Interview study of pain and autosomal dominant polycystic kidney disease. Pain Manag Nurs 2009;10:134-41.  Back to cited text no. 7
Bennett WM, Elzinga L, Golper TA, Barry JM. Reduction of cyst volume for symptomatic management of autosomal dominant polycystic kidney disease. J Urol 1987;137:620-2.  Back to cited text no. 8
Lee DI, Andreoni CR, Rehman J, Landman J, Ragab M, Yan Y, et al. Laparoscopic cyst decortication in autosomal dominant polycystic kidney disease: Impact on pain, hypertension, and renal function. J Endourol 2003;17:345-54.  Back to cited text no. 9
Resnick M, Chang AY, Casale P. Laparoscopic renal denervation and nephropexy for autosomal dominant polycystic kidney disease related pain in adolescents. J Urol 2006;175:2274-6.  Back to cited text no. 10
Binsaleh S, Luke PP, Nguan C, Kapoor A. Comparison of laparoscopic and open nephrectomy for adult polycystic kidney disease: Operative challenges and technique. Can J Urol 2006;13:3340-5.  Back to cited text no. 11
Ubara Y, Tagami T, Sawa N, Katori H, Yokota M, Takemoto F, et al. Renal contraction therapy for enlarged polycystic kidneys by transcatheter arterial embolization in hemodialysis patients. Am J Kidney Dis 2002;39:571-9.  Back to cited text no. 12
Başar O, Ibiş M, Uçar E, Ertuǧrul I, Yolcu OF, Köklü S, el al. Recurrent Pancreatitis in a Patient with Autosomal-Dominant Polycystic Kidney Disease Pancreatology 2006;6:160-2.  Back to cited text no. 13
Bevers EC. A renal cyst causing chronic intestinal obstruction. Br Med J 1914;1:702-3.  Back to cited text no. 14
Kumar S, Adeva M, King BF, Kamath PS, Torres VE. Duodenal diverticulosis in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant 2006;21:3576-8.  Back to cited text no. 15
João Gonçalves, Rui Filipe, Catarina Santos, José Montalban, António Ramalheiro, Ernesto Rocha Partial intestinal obstruction: a rare complication of autosomal dominant polycystic kidney disease. Case report and review of the literature. Port J Nephrol Hypert 2012; 26(3): 207-22.  Back to cited text no. 16
da Rocha EL, Pedrassa BC, Bormann RL, Kierszenbaum ML, Torres LR, D'Ippolito G. Abdominal tuberculosis: A radiological review with emphasis on computed tomography and magnetic resonance imaging findings. Radiol Bras 2015;48:181-91.  Back to cited text no. 17


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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