Mckittrick-wheelock syndrome: a rare cause of serious secretory diarrhoea.

Resumen El síndrome de McKittrick-Wheelock (SMW) es una entidad rara caracterizada por un cuadro de diarrea secretora que puede llegar a ser grave y conducir a trastornos hidroelectrolíticos y disfunción renal, secundario a la presencia de un adenoma velloso colorrectal hipersecretor. El tratamiento definitivo es la resección quirúrgica del adenoma responsable, pudiendo administrarse inhibidores de la prostaglandina E2 como terapia puente.

Retrospective Study on Acute Kidney Injury among Cholera Patients in an Outbreak in Whitefield, Bengaluru

Introduction. Cholera is gastroenteritis caused by Vibrio cholerae. It presents with vomiting, severe secretory diarrhoea, and dehydration. It can cause severe complications with severe electrolyte imbalances and oligoanuric acute kidney injury due to acute tubular necrosis secondary to dehydration or infection itself. However, cholera presenting with significant proteinuria and acute kidney injury has not been reported. Hence, this study was conducted. Aims and Objectives. This aim of this study was to assess clinical features, treatment, and prognosis of AKI in cholera patients; to correlate proteinuria with AKI in cholera patients; and to compare cholera patients with normal kidney function and those with AKI. Material and Methods. It was a retrospective observational study involving patients with cholera. Information regarding cholera patients with acute kidney injury, proteinuria, and prognosis were collected. Results. Most of the patients had significant vomiting, moderate-to-severe diarrhoea, dehydration, and hypovolaemic shock. Cholera caused severe complications such as severe electrolyte imbalances including hyponatraemia and hypokalaemia, acute kidney injury, and proteinuria secondary to dehydration or infection. A surprising finding noted was the lack of significant association between the onset of acute kidney injury and usual risk factors such as hypovolaemic shock and dehydration. It was found that proteinuria had influenced the onset of acute kidney injury, but it did not influence recovery. As there was complete recovery in kidney function, none of the cases required kidney biopsy. There was no mortality noted. Conclusions. This study points towards the rare occurrence of proteinuria and acute kidney injury in Vibrio cholerae infection with spontaneous remission of kidney disease with appropriate therapy.

Case Series of Metastatic VIPoma With Partial Response to Somatostatin Receptor Ligand and Review of Therapeutic Options

Background: Vasoactive intestinal polypeptide secreting islet cell tumors (VIPoma) are rare neuroendocrine tumors of pancreatic origin, characterized by secretory diarrhoea and electrolyte imbalances. Metastasis, commonly hepatic, frequently occur at diagnosis in up to 80% patients. Surgery of the primary tumour remains the gold standard management and in metastatic tumors, somatostatin receptor ligands (SRL) are the drugs of choice for symptom relief and disease control. Some patients will require other approaches to control symptoms, such as liver-targeted therapy, peptide receptor radionuclide therapy (PRRT) or chemotherapy. We describe a case series of metastatic VIPoma which partially-responded to SRL and review of other treatment modalities to achieve disease and symptom control. Case: We report 3 cases, ages 37 to 58 years, with metastatic VIPoma whom presented with secretory diarrhoea, hypokalaemia, achlorhydria and elevated serum VIP levels. CT and 68Gallium PET-CT imaging identified metastases in liver, bone, lungs, spleen and lymph nodes. One case underwent distal pancreatectomy and splenectomy at diagnosis and two cases were advanced and non-resectable. All received long-acting SRL initially and achieved partial control of the gastrointestinal symptoms. Two cases underwent Peptide Receptor Radionuclide Therapy (PRRT) and one case received cytotoxic chemotherapy. Two patients have progressive disease and one succumbed to advanced disease. For inoperable disease, PRRT is useful adjuvant therapy for symptom relief and may improve progression free survival (PFS). Cytotoxic chemotherapy is an option for patients with progressive or symptomatic unresectable tumor. Chemoembolization or liver-targeted therapy are used in liver metastasis for loco-regional control. Alfa-interferon and targeted therapies such as everolimus and sunitinib are other treatment options that can be considered. Conclusion: Metastatic VIPoma remains a challenging disease to manage, requiring multimodal treatment approaches. With timely diagnosis and advancing therapeutic options, more patients can achieve improved symptom and disease control as well as increased survival.

A Rare Presentation of Ogilvie's Syndrome

Ogilvie’s syndrome or acute colonic pseudo-obstruction is characterized by massive colon dilation in the absence of mechanical obstruction or toxic megacolon. The phenotype associated with secretory diarrhoea is rare and is related to increased potassium channel activity in the colon, inducing excessive potassium loss, with increased sensitivity to normal serum aldosterone levels. The recommended therapy is potassium-sparing agents. We present the case of an 85-year-old patient who was admitted at the emergency department with prostration, abdominal distension and diarrhoea, corresponding to functional colonic dilation precipitated by severe hypokalaemia. Resolution of the condition only occurred after spironolactone was administered for suspected primary hyperaldosteronism, which was not proved as the patient showed normal aldosterone serum levels. The pathophysiological mechanism of abnormal potassium secretion in this scenario corresponds to ‘relative hyperaldosteronism’ caused by increased sensitivity of colonocytes to aldosterone.

Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and MEN1-Gene Variant

Background: Vasoactive intestinal peptide-secreting tumours (VIPomas) lead to high-volume secretory diarrhoea with hypokalaemia, as well as hyperglycaemia and hypercalcaemia. Diagnosis is often delayed. Case Description: We present a 13-year-old girl with a distal pancreatic VIPoma diagnosed on her second hospital presentation who became severely hypotensive on anaesthetic induction prior to tumour removal, likely due to the vasodilatory effect of supraphysiological VIP levels. Prior to the second surgical attempt, an octreotide infusion was started preoperatively to suppress systemic VIP levels and counter the potential for VIP-induced hypotension upon tumour manipulation, and the tumour was successfully resected. Hyperparathyroidism and history of GI tumour resection were subsequently identified in the father, and the two members were found to have a heterozygous variant of uncertain significance in the multiple endocrine neoplasia type 1 (MEN1) gene. However, as this family meets the diagnostic criteria for MEN1 clinically, ongoing surveillance for MEN1 tumours and genetic counseling for at-risk family members are required despite the non-pathogenic genetic result. Conclusion: This case highlights the importance of screening for a VIPoma in patients with high-volume secretory diarrhoea and preventing cardiovascular complications with perioperative VIP suppression. Furthermore, careful interpretation of genetic results within the clinical context is required.

Calcium-sensing receptor in nutrient sensing: an insight into the modulation of intestinal homoeostasis

The animal gut effectively prevents the entry of hazardous substances and microbes while permitting the transfer of nutrients, such as water, electrolytes, vitamins, proteins, lipids, carbohydrates, minerals and microbial metabolites, which are intimately associated with intestinal homoeostasis. The gut maintains biological functions through its nutrient-sensing receptors, including the Ca-sensing receptor (CaSR), which activates a variety of signalling pathways, depending on cellular context. CaSR coordinates food digestion and nutrient absorption, promotes cell proliferation and differentiation, regulates energy metabolism and immune response, stimulates hormone secretion, mitigates secretory diarrhoea and enhances intestinal barrier function. Thus, CaSR is crucial to the maintenance of gut homoeostasis and protection of intestinal health. In this review, we focused on the emerging roles of CaSR in the modulation of intestinal homoeostasis including related underlying mechanisms. By elucidating the relationship between CaSR and animal gut homoeostasis, effective and inexpensive methods for treating intestinal health imbalance through nutritional manipulation can be developed. This article is expected to provide experimental data of the effects of CaSR on animal or human health.