Prolonged watery diarrhea and malnutrition caused by Aliarcobacter butzleri (formerly Arcobacter butzleri): the first pediatric case in Croatia and a literature review.

Aliarcobacter butzleri (formerly Arcobacter butzleri), is a newly recognized Campylobacter-like emerging foodborne pathogen worldwide, usually causing gastrointestinal symptoms in young children. A 4-year-old boy was admitted to the Department of Pediatrics, University Hospital of Split, Croatia, because of malnutrition, lost appetite and prolonged watery diarrhea. A comprehensive diagnostics, including biochemistry, haematology, allergology, microbiology and radiology, were performed. The only positive microbiology result was unexpected isolation of Aliarcobacter butzleri on selective media for Campylobacter, after 48 hours of incubation on 42°C, among microaerophilic atmosphere. Clinical course was favorable and after symptomatic therapy child was discharged in good clinical condition and normal peristalsis to home care, with the recommendation of taking high-protein preparations to improve nutritional status. In addition, we performed a literature review of clinical cases caused by Aliarcobacter butzleri infection. The first report of Aliarcobacter butzleri isolated from stool sample in a 4-year old boy in Croatia, along with other clinical reports in literature, highlights the importance of standardisation and improvement of microbiological analysis, especially implementation of new methods for the identification of emerging pathogens.

Factors associated with acute watery diarrhea among children aged 0-59 months in Obongi District, Uganda, April 2022: A case-control study.

Background: Diarrheal diseases, are major concerns for Ugandan children; persistent in Obongi District despite high rotavirus vaccination rates (2019-2021). The district recorded the country's highest annual acute watery diarrhea (AWD) incidence from 2017 to 2021. Our study, conducted in April 2022, assessed AWD risk factors among 0-59-month-old children in Obongi. Methods: We conducted a 1:2 (193:386) unmatched case-control study. A case was a child (0-59 months) with ≥ 3 loose/liquid stools/day, negative malaria/pneumonia tests, residing in Itula/Parolinya subcounty from 1 to 30 April 2022. Medical records from 10 facilities were reviewed. Simple random sampling identified cases, who were interviewed, and controls were randomly chosen from non-AWD neighboring households. Child health cards provided vaccination details. We used logistic regression to identify factors associated with AWD. Results: Among 193 cases and 386 controls, 104 (54 %) cases and 183 (47 %) controls were male, 58 (30 %) cases and 127 (33 %) controls were aged 12-23 months, 187 (97 %) cases and 369 (96 %) controls had received at least one dose of rotavirus vaccine, 58 (30 %) cases and 120 (34 %) controls treated drinking water. Comorbidity presence (undernutrition, diabetes, HIV) (AOR = 12; CI: 2.5-53), caregiver's unwashed hands post-toilet (AOR = 3.9; CI: 1.2-13), and borehole vs. piped water (AOR = 4.0; CI: 1.7-9.6) linked to AWD. Conclusion: Modifiable factors, including failure of caregivers to wash their hands with soap after visiting toilets and use of borehole water were associated with AWD, suggesting that community sensitization on handwashing at critical times, using clean water and soap, and expanded use of piped water could reduce AWD incidence in this area.

Abdominal migraine with acute watery diarrhea and dehydration: Successful treatment with Valproic acid in a pediatric case.

Abdominal migraine (AM) is a prevalent pediatric condition that rarely affects adults. Multiple diagnostic criteria have been established, but in general, AM is characterized by unprovoked episodes of acute central abdominal pain with migrainous characteristics and periods of respite. Recurrent stomach pain is a prevalent symptom globally, with a significant portion of cases falling under the category of functional gastrointestinal disorders (FGIDs) due to the absence of identified biological causes. There is a notable prevalence of migraines among individuals with a family history of the condition, indicating a genetic predisposition. A descriptive report has been prepared on the participant who had AM associated with acute watery diarrhea (AWD) on January 2023. The patient's parents had given written informed consent for publishing this case report. In this case report, we present the clinical scenario of a 12-year-old male child who experienced AM symptoms alongside a history of absence seizures. The child presented with episodes of abdominal pain and AWD. Despite extensive investigation and treatment, there was no improvement in abdominal pain. However, after 1 week of oral valproic acid administration, the patient remained symptom-free during the follow-up period. Dehydration, along with other factors, has been identified as a triggering factor for AM. Acute watery diarrhea has the potential to disrupt the normal functioning of the gastrointestinal system, and dehydration may lead to subsequent abdominal symptoms.

Concomitant Lymphocytic Colitis With Recurrent Clostridium difficile Infection.

Microscopic colitis is a clinicopathological diagnosis that is characterized by chronic microscopic inflammation of the colon and presents with chronic watery diarrhea. There are following two subtypes of microscopic colitis: lymphocytic colitis and collagenous colitis. This is a case of a 70-year-old female with a history of Clostridium difficile infections who presented with persistent watery diarrhea and was diagnosed with lymphocytic colitis in the setting of a concomitant C. difficile infection. Given her clinical presentation, the patient was initiated on empiric treatment for C. difficile infection and showed a lack of clinical improvement with persistent watery diarrhea and elevated white blood cell count. The patient's symptoms resolved upon the confirmatory diagnosis and treatment of lymphocytic colitis. This study illustrates the importance of assessing for, diagnosing, and treating lymphocytic colitis in patients with chronic non-resolving watery diarrhea, especially in the setting of concomitant or recurrent C. difficile infections. Additionally, it emphasizes the need for further characterization of the relationship between C. difficile infection and microscopic colitis.

Watery Diarrhea Is Not Always Clostridioides difficile: A Case Report of Aeromonas hydrophila Gastroenteritis.

Aeromonas species can cause acute gastroenteritis but are much less commonly observed in the hospital setting than other bacteria. Most cases of Aeromonas hydrophila gastroenteritis reported in the literature have occurred in pediatric, elderly, and/or immunocompromised patients. We present a case of subacute watery diarrhea due to A. hydrophila infection in an otherwise healthy 48-year-old female patient with prior abdominal surgeries and recent hospitalization for a catheter-associated urinary tract infection (CAUTI) for which she received antibiotics. The patient presented with 10 days of increasingly frequent non-bloody, watery, foul-smelling diarrhea as well as decreased oral intake, cramping bilateral upper abdominal pain, chills, and malaise. Initial diagnoses considered included Clostridioides difficile in the setting of CAUTI and antibiotic use, small intestinal bacterial overgrowth, dumping syndrome related to bariatric surgery, and malabsorption. A computed tomography scan of her abdomen/pelvis, admission labs, and flexible sigmoidoscopy showed no relevant findings. Stool cultures eventually returned positive for A. hydrophila. The case is an uncommon presentation of Aeromonas infection that could be easily missed while other diagnoses are pursued. Early treatment of Aeromonas infection can be crucial in preventing advanced forms of disease such as septicemia and necrotizing fasciitis.

Approach to a Child with Chronic Diarrhea.

Chronic diarrhea in children is challenging both with regards to etiological diagnosis and for management. Etiology and pathophysiological mechanisms vary widely from neonates to adolescents. Congenital or genetic causes are more frequent in neonates, while infections, allergy and immune-mediated mechanisms are more frequent in childhood. A thorough history and proper physical examination are required to decide for further diagnostic evaluation. The approach to a child with chronic diarrhea should be age specific and based predominantly on the pathophysiological mechanism involved. The nature of the stool like watery, bloody or fatty (steatorrhea) can suggest the probable etiology and organ system involved. After routine tests, evaluation with specific serological tests, imaging, endoscopy (gastroscopy/colonoscopy), histopathology of intestinal mucosa, breath tests or radionuclide imaging may be required to make a definitive diagnosis. Genetic evaluation is important in congenital diarrheas, monogenic inflammatory bowel disease (IBD) and immunodeficiency disorders. Management is aimed at stabilization, nutritional support and etiology specific treatment. Specific therapy can be as simple as exclusion of specific nutrient or as complicated as small bowel transplant. Evaluation and management require expertise and thus patients need to be referred in a timely fashion. This will minimise morbidity including nutritional consequences and improve outcome.

A Rare Case of Community-Acquired Pneumonia Only Presenting With Diarrhea, Abdominal Pain, and Fever: A Case Report.

Legionnaires' disease is an atypical pneumonia caused by Legionella pneumophila (L. pneumophila) pneumonia that features slow onset, nonproductive cough, fatigue, headache, sore throat, myalgias, and malaise. It can be difficult to diagnose, as it presents with extrapulmonary symptoms, and delay in treatment can be fatal. Here, we present the case of a previously healthy 32-year-old Caucasian male with Legionnaires disease who only presented to the clinic with abdominal pain and diarrhea. The patient did not have any pulmonary symptoms at the initial presentation. This presentation did not fit the diagnostic tools available for Legionnaires' disease, including a validated clinical prediction rule, which ruled out L. pneumophila infection with a sensitivity of 97% and a negative predictive value of 99.4%. Due to the complaint of abdominal pain, a flat/upright abdominal X-ray was ordered, which includes a chest X-ray. Upon analyzing the chest X-ray, a right lower lobe consolidation was identified, prompting an L. pneumophila urinary test to be added to the lab orders. This case represents the difficulties in diagnosing Legionnaires' disease due to the diverse clinical complexities of presentations, which may solely involve abdominal complaints.

Celiac Disease in Uzbek Children: Insights into Disease Prevalence and Clinical Characteristics in Symptomatic Pediatric Patients.

BACKGROUND: A few studies on pediatric Celiac Disease (CD) are available from Central Asia. Recent immunogenetic research has highlighted that the HLA-DQ2/8 genetic predisposition to CD as well as the dietary intake of gluten in this geographical area, are comparable to other regions of the world where CD prevalence is known to be 1% or higher. METHODS: This is a prospective and cross-sectional study investigating the prevalence and clinical characteristics of CD in symptomatic children referred to the pediatric gastroenterology department of a tertiary hospital in Uzbekistan from 1 September 2021, until 31 July 2022. In addition to collecting the relevant information related to clinical manifestations and laboratory analyses from the clinical files, a specific survey was also administered to patients' guardians. Serological, histopathological, and immunogenetic parameters specific to CD, fecal zonulin, and pancreatic elastases were assessed in CD patients. RESULTS: The study population consisted of 206 children. Overall, almost all of them (n = 192; 93.2%) were referred because of gastrointestinal manifestations, which were associated with extra-gastrointestinal manifestations in most cases (n = 153; 74.3%); a minority (n = 14; 6.8%) was mainly referred due short stature and/or growth failure only. Among all of these study participants, CD was diagnosed in 11 children (5.3%). Notably, although diarrhea was similarly reported in CD and non-CD patients, watery diarrhea (type 7 according to the Bristol stool scale) was much more frequently and significantly observed in the former group. All of these CD patients showed anti-tTG IgA 10 times higher than the upper normal limit, except one child with lower serum levels of total IgA; however, all of them received a diagnostic confirmation by histopathological analysis due to the lack of EMA testing in the country. Notably, most CD children (82%) showed a Marsh III histological grading. Around half patients (54.5%) showed zonulin values above the reference range, whereas none showed insufficient levels of pancreatic elastase. However, no correlation or association between zonulin and clinical, laboratory, histopathological, and immunogenetic parameters was found. CONCLUSIONS: This study may further suggest a relevant prevalence of CD in Uzbek children, based on this partial picture emerging from symptomatic patients only. Additionally, we highlighted the prevalence of typical CD forms with watery diarrhea, which should strongly support a full diagnostic work-up for CD in the local clinical setting. The high levels of anti-tTG IgA and high Marsh grade might also lead us to speculate a significant diagnostic delay despite the classical clinical expression of CD.

Multidisciplinary strategies for managing acute watery diarrhea in children with congenital anorectal malformation and colostomy: A case study.

Congenital anorectal malformation (ARM) is a diverse group of anomalies affecting the development of the anal and rectal regions, with an estimated incidence of one in every 5000 live births. The colostomy is commonly performed as part of the staged management of children with ARM to prevent complications. However, the presence of acute watery diarrhea in children with ARM and colostomy poses significant management challenges due to the altered anatomy and physiology affecting stool regulation and absorption, exacerbated by various factors including infections, dietary issues, medication side effects, and underlying gastrointestinal complications.This case study explores the complexities involved in managing acute watery diarrhea in children with congenital ARM and colostomy. A comprehensive literature review was conducted to examine the existing evidence on the subject. The study highlights the multidisciplinary approach required, involving pediatricians, surgeons, and other specialists, to provide comprehensive care and support for these children. Effective management of acute watery diarrhea in children with congenital ARM and colostomy necessitates collaboration between pediatricians and surgeons. Pediatricians play a crucial role in assessing hydration status, monitoring electrolyte balance, and providing appropriate fluid and nutritional management. Surgeons address the surgical aspects of care and coordinate interventions with the management of acute diarrhea. The study underscores the importance of a multidisciplinary approach to deliver comprehensive care, optimize outcomes, and improve the quality of life for affected children. The management of acute watery diarrhea in children with congenital ARM and colostomy presents significant challenges due to the complex interplay of anatomical, physiological, and clinical factors. A multidisciplinary approach involving pediatricians, surgeons, and other specialists is vital for providing comprehensive care and support. This case study emphasizes the need for further research, guidelines, and collaborative efforts to enhance the management strategies for this vulnerable population.

Intestinal Spirochetosis and Chronic Diarrhea: A Case Report and Literature Review.

Spirochetosis is a rare condition characterized by the presence of spirochetes in the gastrointestinal tract. It is typically associated with immunodeficiency. We present a case of chronic watery diarrhea in a 48-year-old housewife who had a 12-week history of variable-volume bowel movements without blood or mucus, accompanied by a sense of urgency. Chronic diarrhea led to weight loss and fatigue, significantly impacting her quality of life. Despite the absence of known risk factors, a comprehensive clinical evaluation and exclusion of other potential causes prompted a rectosigmoid biopsy, which revealed distinctive histological findings of spirochetosis. This case underscores the significance of considering spirochetosis as a differential diagnosis in cases of chronic watery diarrhea, even in the absence of immunodeficiency. The utilization of rectosigmoid biopsy and careful histopathological examination played a pivotal role in establishing an accurate diagnosis.

McKittrick Wheelock syndrome secondary to adenocarcinoma of the rectum: case report and diagnostic considerations

Introduction: McKittrick-Wheelock syndrome is a rare entity characterized by chronic diarrhea, acute kidney injury, and hydroelectrolytic imbalance associated with a large rectal tumor, frequently a villous adenoma. Case report: A 69-year-old male with chronic diarrhea with mucus. He underwent a colonoscopy with biopsies, reporting adenocarcinoma of the rectum in situ, and underwent a robot assisted intersphincteric resection with colo-anal anastomosis and a protecitive ileostomy. Discussion: Described in 1954, this syndrome is manifested by electrolyte imbalance and acute renal injury secondary to diarrhea associated with a rectal villous adenoma, often with long lasting symptoms. The most frequent symptom being watery diarrhea with mucus. The definitive treatment consists of surgical resection. Conclusion: Although this is a rare pathology, it should be considered as a differential diagnosis in cases of chronic diarrhea associated with water and electrolyte disorders. (AU)

Hypericum japonicum extract inhibited porcine epidemic diarrhea virus in vitro and in vivo.

Porcine epidemic diarrhea virus (PEDV) infection causes lethal watery diarrhea and high mortality in neonatal piglets, leading to huge economic losses in the global swine industry. Currently, the existing commercial vaccines cannot fully control PEDV, so it is urgent to develop effective antiviral agents to complement vaccine therapy. In the present study, we investigated the antiviral effect of Hypericum japonicum extract (HJ) against PEDV in vivo and in vitro. In in vitro assays, HJ could directly inactivate PEDV strains; moreover, it inhibited the proliferation of PEDV strains in Vero or IPI-FX cells at its non-cytotoxic concentrations. Time of addition assays revealed that HJ mainly inhibited PEDV at the later stages of the viral life cycle. In in vivo, compared with the model group, HJ could reduce the viral titers in the intestines of infected piglets, and improve their intestinal pathological, indicating that HJ could protect the newborn piglets from highly pathogenic PEDV variant infection. Furthermore, this effect may be related to the fact that HJ can not only directly inhibit viruses, but also regulate the structure of intestinal microbiota. In conclusion, our results indicate that Hypericum japonicum could inhibit PEDV replication in vitro and in vivo and might possess the potential to develop as the anti-PEDV drug.

Collagenous sprue: a rare cause of watery diarrhea and villous atrophy - case report.

Collagenous sprue is a rare and unrecognized cause of diarrhea and weight loss, mainly affecting the duodenum and small bowel. The clinical picture often resembles that of coeliac sprue, the main differential diagnosis, albeit, being refractory to a gluten-free diet. The histological features are fundamentally characterized by the deposition of collagen beneath the basement membrane of gut mucosa. Treatment should be initiated as soon as the diagnosis is established, so as to prevent the progression of fibrosis. We will describe the case of a 76-year-old woman with collagenous sprue, her diagnostic workup, histopathological examination, and response to treatment.

A Case of Immediate Anastomotic Leakage After Low Anterior Resection for Rectal Cancer.

A man in his seventies was referred to our hospital for radical therapy for advanced rectal cancer with multiple liver metastases. A colonic stent had already been placed in his rectum at the previous hospital because of malignant colorectal obstruction, so our therapeutic strategy was to perform systematic chemotherapy after resection of the primary tumor. Laparoscopic low anterior resection with a covering stoma was performed under general anesthesia. At about one hour after the surgery, the patient had sudden abdominal pain with watery diarrhea, and a similar discharge from his drainage tube. We suspected peritonitis caused by bowel perforation and emergency surgery was performed. The operative findings showed that his peritonitis was caused by anastomotic leakage from the rectum. Radical lavage of the abdominal space and reconstruction of colostomy was performed. The patient gradually recovered and we were able to start systematic chemotherapy at one month after the surgery. Anastomotic leakage immediately after anterior resection caused by watery diarrhea is rare, and it may be concerned with several issues. The covering stoma is intended to stop anastomotic leakage but it cannot prevent all cases of leakage especially when obstruction is present. We recommend that preventive measures be taken against anastomotic leakage, including intraoperative leakage tests or anal decompression tube placement.

Vasoactive Intestinal Peptide-Secreting Pheochromocytoma: A Case Report and Review of Literature.

Objective: To describe a case of composite vasoactive intestinal peptide (VIP)-secreting pheochromocytoma and review literature to provide insight into the various presentations and potential management of these rare tumors. Case Report: A 64-year-old male patient presented with hypertensive emergency and coronary demand ischemia with development of watery diarrhea, hypokalemia, and achlorhydria syndrome. Serum and urine studies demonstrated elevated metanephrine and VIP levels. Definitive surgical resection resolved symptoms and normalized laboratory values. Pathologic examination of the specimen revealed pheochromocytoma with a Pheochromocytoma of the Adrenal gland Scaled Score of 4 and patchy expression of VIP. Discussion: Given the different actions of hormones that can be secreted by these composite tumors, we suggest that pheochromocytomas with diversified secretory capabilities may be an underrecognized clinical entity. Localized disease is often amenable to surgical resection, although management of metastatic disease is not well established due to the rarity of these tumors and lack of randomized trials. Conclusion: In patients presenting with diarrhea of unclear etiology or the suggestion of secondary hypertension, assessment for a possible neuroendocrine tumor may be prudent. If an adrenal mass is discovered but the patient exhibits atypical symptoms of catecholamine excess, a diagnosis of composite pheochromocytoma with multisecretory properties should be considered.

A clinical analysis on functioning pancreatic neuroendocrine tumors (focusing on VIPomas): a single-center experience.

VIPomas are generally rare functioning pancreatic neuroendocrine tumors (PanNETs) that cause watery diarrhea, hypokalemia, and achlorhydria. Due to their extreme rarity, the clinicopathological features and outcomes of VIPomas have not been well reported. This study aimed to determine the diagnostic and therapeutic characteristics and prognosis of VIPomas and to compare them with other PanNETs at a Japanese reference hospital. Medical records of 293 patients with PanNETs were collected. Patient and tumor characteristics and outcomes were retrospectively reviewed. This cohort had only 1.4% (four patients) of patients with VIPomas, and three of these patients changed from non-functioning (NF-) PanNETs during their disease course. Recurrences of hormonal symptoms were observed in all patients despite the initial controls, and all of them died from their disease, more specifically mainly from hormonal symptoms. Compared to the other PanNETs, VIPomas were all located at the pancreatic tail, were larger, and had a higher Ki-67 index and more metastasis. The median survival time was significantly shorter for patients with VIPoma than for those with NF-PanNET (5.9 vs. 26.7 years, p < 0.0001), insulinoma (21.8 years, p < 0.0001), and gastrinoma (12.3 years, p = 0.0325). This study presents the possibility of shifting from non-symptomatic to symptomatic VIPomas as they grow or of transforming from NF-PanNETs to VIPomas. VIPomas should be considered in patients with relatively large NF-PanNETs, especially those located in the pancreatic tail, when diarrhea is continuously observed. As hormonal symptoms are an important cause of death in VIPomas, long-term symptomatic control, which is relatively difficult, is of great significance.

Inadequate Hand Washing, Lack of Clean Drinking Water and Latrines as Major Determinants of Cholera Outbreak in Somali Region, Ethiopia in 2019.

Introduction: Cholera remains a serious public health problem characterized by a large disease burden, frequent outbreaks, persistent endemicity, and high mortality, particularly in tropical and subtropical low-income countries including Ethiopia. The recent cholera outbreak in the Somali region began on 4 September to 1 November 2019. Cholera may spread rapidly through a population so that an early detection and reporting of the cases is mandatory. This study aimed to identify determinants of cholera infection among >5 years of age population in Somali region, Ethiopia. Methods: A community-based unmatched case-control study was conducted among 228 (76 cases and 152 controls, 1:2 ratio) systematically selected population. Data were collected using a structured questionnaire administered by an interviewer and a record review. Descriptive statistics and multivariable logistic regression analysis was used to identify the determinants of the risk factors of cholera infection with a 95% confidence interval and statistical significance was declared a tap-value < 0.05. Results: A total of 228 participants (33.3% cases and 66.7% controls) were enrolled in this study. The majority of the cases were in the range of 20-49 years of age (69.7%). The odds of acquiring cholera infection increased significantly by drinking unsafe pipe water (AOR 4.3, 95% CI 1.65-11.2), not having a household level toilet/latrine (AOR 3.25, 95% CI 1.57-6.76), hand washing only sometimes after the toilet (AOR 3.04, 95% CI 1.58-5.86) and not using water purification methods (AOR 2.3, 95% CI 1.13-4.54). Conclusion: Major risk factors for cholera infection were related to drinking water and latrine hygiene. Improvement in awareness creation about cholera prevention and control methods, including water treatment, hygiene and sanitation were crucial in combating this cholera outbreak. Primary public health actions are ensuring clean drinking water, delivery of water purification tablets, soap and hand sanitizers and provision of health care and outbreak response. Long term goals in cholera affected areas include comprehensive water and sanitation strategies. Overall, the strategic role of a multi-sectoral approach in the design and implementation of public health interventions aimed at preventing and controlling cholera are essential to avert cholera outbreaks. Preparedness should be highlighted in cholera prone areas like Somali region especially after drought periods.

Cryptosporidium and waterborne outbreaks - A mini review.

Cryptosporidium spp. is one of the prime agents of infectious diarrhea. Cryptosporidium spp. has been gaining awareness as a pathogen of public health importance in India and other developing countries. Owing to the nature of multiple transmission routes such as person-to-person, animal-to-person, waterborne and foodborne, the epidemiology of cryptosporidiosis in humans is not well known. A deeper understanding of the pathogenesis may lead to better diagnosis and better treatment of the condition. Asymptomatic human and animal transmission illustrates that the spread of infection through the environment is a more plausible explanation, waterborne transmission in particular. The disease burden is underestimated and its global impact is yet to be quantified due to the lack of country-specific estimates. Assessment of the disease itself has been crucial since the morphological indistinguishability, differences in distribution and transmission, and variations in the genotypes.

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

Background: Pheochromocytoma (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 Case: A 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. Conclusions: A 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.