Case Report: Irreversible Watery Diarrhea, Severe Metabolic Acidosis, Hypokalemia and Achloridria Syndrome Related to Vasoactive Intestinal Peptide Secreting Malignant Pheochromocytoma

BackgroundPheochromocytoma (PHEO) clinical manifestations generally mirror excessive catecholamines secretion; rarely the clinical picture may reflect secretion of other hormones. Watery diarrhea, hypokalemia and achlorhydria (WDHA) is a rare syndrome related to excessive secretion of vasoactive intestinal peptide (VIP).Clinical CaseA 73-year-old hypotensive man affected by adrenal PHEO presented with weight loss and watery diarrhea associated with hypokalemia, hyperchloremic metabolic acidosis (anion gap 15 mmol/l) and a negative urinary anion gap. Abdominal computed tomography scan showed a right adrenal PHEO, 8.1 cm in maximum diameter, with tracer uptake on 68GaDOTA-octreotate positron emission tomography. Metastasis in lumbar region and lung were present. Both chromogranin A and VIP levels were high (more than10 times the normal value) with slightly elevated urine normetanephrine and metanephrine excretion. Right adrenalectomy was performed and a somatostatin analogue therapy with lanreotide started. Immunostaining showed chromogranin A and VIP co-expression, with weak somatostatin-receptor-2A positivity. In two months, patient clinical conditions deteriorated with severe WDHA and multiple liver and lung metastasis. Metabolic acidosis and hypokalemia worsened, leading to hemodynamic shock and exitus.ConclusionsA rare case of WDHA syndrome caused by malignant VIP-secreting PHEO was diagnosed. High levels of circulating VIP were responsible of the rapidly evolving clinical picture with massive dehydration and weight loss along with severe hyperchloremic metabolic acidosis and hypokalemia due to the profuse untreatable diarrhea. The rescue treatment with lanreotide was unsuccessful because of the paucity of somatostatin-receptor-2A on VIP-secreting PHEO chromaffin cells.

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Case Report: Uroenteric Fistula in a Pediatric-en-bloc Kidney Transplant Manifests as Deceptive Watery Diarrhea and Normal Anion Gap Acidosis

Frontiers in Pediatrics ◽

10.3389/fped.2021.687396 ◽

2021 ◽

Vol 9 ◽

Author(s):

Malek Al Barbandi ◽

Marissa J. Defreitas ◽

Juan C. Infante ◽

Mahmoud Morsi ◽

Patricia A. Arroyo Parejo Drayer ◽

...

Keyword(s):

Metabolic Acidosis ◽

Small Bowel ◽

Kidney Transplant ◽

Renal Allograft ◽

Anion Gap ◽

Watery Diarrhea ◽

Diagnostic Tools ◽

Kidney Transplant Patient ◽

En Bloc ◽

Hyperchloremic Metabolic Acidosis

Introduction: The diagnosis of a post–surgical uroenteric fistula can be challenging and may be delayed for months after symptoms begin. A normal anion gap metabolic acidosis has been reported in up to 100% of patients after ureterosigmoidostomy, and bladder substitution using small bowel and/or colonic segments. Here, we describe a rare case of a pediatric patient who developed a uroenteric fistula from the transplant ureters into the small bowel, after an en-bloc kidney transplantation resulting in profound acidosis and deceptive watery diarrhea.Case Presentation: The patient is an 8-year-old girl with end stage kidney disease (ESKD) secondary to focal segmental glomerulosclerosis. Through a right retroperitoneal approach, she underwent a right native nephrectomy and a pediatric deceased donor en-bloc kidney transplant including two separate ureters. One month later, she had a renal allograft biopsy for suspected rejection. During the week after the biopsy, she experienced abdominal pain followed by watery diarrhea and metabolic acidosis requiring continuous bicarbonate/acetate infusions. An extensive gastro-intestinal evaluation for the cause of the diarrhea including endoscopy was inconclusive. The urine output decreased to <500 ml daily; although, the kidney function remained normal. After 2 weeks of unexplained watery diarrhea a magnetic resonance urogram with contrast was performed which demonstrated extravasation of urine from both ureters with fistulization into the small bowel. She underwent corrective surgery which identified the fistulous tract, which was resected and both ureters were re-implanted. The diarrhea and acidosis resolved, and she has maintained normal renal allograft function for over 1 year.Conclusion: An important aspect in the early diagnosis of a uroenteric fistula is the sudden onset of severe hyperchloremic metabolic acidosis that results when urine is diverted into the intestinal tract. The mechanism is similar to that described in cases of urinary diversions and/or bladder augmentation using the intestine. Important diagnostic tools are the measurements of solute excretion and pH in the urine as compared to the “watery diarrhea” or bowel output.Summary: We describe a case of a uroenteric fistula in a pediatric-en-bloc kidney transplant patient that went undiagnosed for almost 3 weeks due to the deceptive nature of the watery diarrhea which was actually urine. A uroenteric fistula should be considered in the differential diagnosis of diarrhea and hyperchloremic metabolic acidosis as a complication of kidney transplant. The simultaneous comparison of stool and urine pH and solute excretions may lead to the diagnosis, appropriate imaging and surgical intervention.

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A newborn girl with watery diarrhea, weight loss, and severe metabolic acidosis

Current Opinion in Pediatrics ◽

10.1097/00008480-199404000-00007 ◽

1994 ◽

Vol 6 (2) ◽

pp. 163-168 ◽

Cited By ~ 1

Author(s):

Victor Nizet ◽

Cedric j. Priebe

Keyword(s):

Weight Loss ◽

Metabolic Acidosis ◽

Watery Diarrhea ◽

Severe Metabolic Acidosis ◽

Newborn Girl

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The Use of the Urinary Anion Gap in the Diagnosis of Hyperchloremic Metabolic Acidosis

New England Journal of Medicine ◽

10.1056/nejm198803103181002 ◽

1988 ◽

Vol 318 (10) ◽

pp. 594-599 ◽

Cited By ~ 146

Author(s):

Daniel C. Batlle ◽

Miguel Hizon ◽

Eric Cohen ◽

Cory Gutterman ◽

Roop Gupta

Keyword(s):

Metabolic Acidosis ◽

Anion Gap ◽

Hyperchloremic Metabolic Acidosis

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Starvation ketoacidosis and refeeding syndrome

BMJ Case Reports ◽

10.1136/bcr-2021-245065 ◽

2021 ◽

Vol 14 (12) ◽

pp. e245065

Author(s):

Angela Heulwen Boal ◽

Maurizio Panarelli ◽

Caroline Millar

Keyword(s):

Body Mass Index ◽

Weight Loss ◽

Metabolic Acidosis ◽

Body Mass ◽

Anion Gap ◽

Electrolyte Imbalance ◽

Intravenous Fluids ◽

Severe Metabolic Acidosis ◽

Refeeding Syndrome ◽

Electrolyte Replacement

Starvation ketoacidosis (SKA) is a rarer cause of ketoacidosis. Most patients will only have a mild acidosis, but if exacerbated by stress can result in a severe acidosis. We describe a 66-year-old man admitted with reduced consciousness and found to have a severe metabolic acidosis with raised anion gap. His body mass index (BMI) was noted to be within the healthy range at 23 kg/m2; however, it was last documented 1 year previously at 28 kg/m2 with no clear timeframe of weight loss. While his acidosis improved with intravenous fluids, he subsequently developed severe electrolyte imbalance consistent with refeeding during his admission. Awareness of SKA as a cause for high anion gap metabolic acidosis is important and knowledge of management including intravenous fluids, thiamine, dietetic input and electrolyte replacement is vital.

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Diet-induced Ketoacidosis in a Non-diabetic: A Case Report

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2020.2.44736 ◽

2020 ◽

Vol 4 (2) ◽

pp. 259-262

Author(s):

Sam Slade ◽

John Ashurst

Keyword(s):

Weight Loss ◽

Case Report ◽

Metabolic Acidosis ◽

Ketogenic Diet ◽

Emergency Physician ◽

Anion Gap ◽

Low Carbohydrate Diet ◽

Low Carbohydrate ◽

Sliding Scale ◽

Developed Nations

Introduction: Anion gap metabolic acidosis is a common disorder seen in the emergency department. The differential can include toxicological, renal, endocrine, infectious, and cardiogenic disorders. Ketosis, however, is one of the rarer causes of metabolic acidosis seen by the emergency physician in developed nations. Case Report: A 53-year-old female presented after starting a low-carbohydrate ketogenic diet for weight loss. She reported xerostomia, nausea with abdominal pain and a 17-pound weight loss over the previous 22 days. Labs revealed an anion-gap metabolic acidosis with ketosis. She was treated with 5% dextrose in normal saline and a sliding scale insulin coverage. Her anion gap corrected during her hospital course and was discharged on hospital day three. Discussion: The ketogenic diet typically consists of a high-fat, adequate protein and low carbohydrate diet that has previously been thought to be relatively safe for weight loss. However, when carbohydrates are completely removed from the diet an overproduction of ketones bodies results in ketoacidosis. Treatment should be aimed at halting the ketogenic process and patient education. Conclusion: Although rarely included in the differential for metabolic acidosis, diet-induced ketosis should be included by the emergency physician when faced with a patient who recently changed their eating patterns.

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Changes in the plasma anion gap during chronic metabolic acid-base disturbances

AJP Renal Physiology ◽

10.1152/ajprenal.1978.235.4.f291 ◽

1978 ◽

Vol 235 (4) ◽

pp. F291-F297 ◽

Cited By ~ 2

Author(s):

H. J. Adrogue ◽

J. Brensilver ◽

N. E. Madias

Keyword(s):

Metabolic Acidosis ◽

Metabolic Alkalosis ◽

Protein Concentration ◽

Anion Gap ◽

Acid Base ◽

Plasma Protein Concentration ◽

Basic Premise ◽

Hyperchloremic Metabolic Acidosis ◽

Large Groups

A basic premise in the utilization of the plasma anion gap in the assessment of acid-base disorders is that this parameter remains constant during hyperchloremic metabolic acidosis and metabolic alkalosis. Experimental data under in vitro conditions, however, cast serious doubt on this premise. The purpose of the present study was to characterize the plasma anion gap, estimated as (Na + K) - Cl + HCO3), in two large groups of dogs with graded degrees of chronic, HCl-induced metabolic acidosis or chronic, diuretic-induced metabolic alkalosis. The data indicate that the plasma anion gap decreases significantly in HCl acidosis and increases significantly in metabolic alkalosis; the predicted mean anion gap in animals with a plasma bicarbonate concentration of 10, 21 (normal), and 40 meq/liter approximated 13, 18, and 26 meq/liter, respectively. The observed variation in the plasma anion gap is interpreted as originating mainly from directional changes in the net negative charge of plasma proteins; these changes result from the titration process secondary to the altered plasma acidity and, in the case of metabolic alkalosis, from the additional effect of an increased plasma protein concentration.

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Normal "anion gap" (hyperchloremic) acidosis.

Clinical Chemistry ◽

10.1093/clinchem/31.2.309 ◽

1985 ◽

Vol 31 (2) ◽

pp. 309-313 ◽

Cited By ~ 4

Author(s):

R N Walmsley ◽

G H White

Keyword(s):

Metabolic Acidosis ◽

Anion Gap ◽

Common Condition ◽

Diagnostic Problem ◽

Hyperchloremic Acidosis ◽

Logical Approach ◽

Normal Limits ◽

Hyperchloremic Metabolic Acidosis ◽

Hospital Population

Abstract Hyperchloremic metabolic acidosis in which the anion gap is within normal limits is a common condition in the hospital population, and often presents a difficult diagnostic problem. We describe nine typical cases of this disorder and suggest a logical approach to its evaluation.

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