vasoactive-intestinal-peptide and Diarrhea--Infantile

A literature review was conducted in relation to a case of chronic diarrhea associated with a VIP (vasoactive intestinal polypeptide) producing ganglioneuroblastoma (GNB), in an 18-month old female baby. This is a rare entity characterized by premonitory, persisting diarrhea, causing fluid and electrolyte changes typical of the WDHA syndrome, associating watery diarrhea, hypokalemia, and achlorhydia. Elevated VIP plasma levels are an indication for an echographic and/or CT-scan search for the causal secreting tumor. Although the prognosis of this condition seems favorable, the recommended treatment is surgery. The VIP substance represents an excellent biological monitoring marker. Ganglioneuroblastomas are tumors of the sympathetic nervous system, which, according to Pearse's cell and embryologic theory (1966), have to be linked to the APUD system tumors (paraneuromas). VIP-producing forms are rare in children, and only 29 case studies have been compiled in the literature since 1970, when the VIP substance was discovered. The case reported in this study illustrates the diagnostic problems raised by such lesions, and allows us to confirm VIP's imputability for the occurrence of the chronic diarrhea condition in this child.

Topics: Chronic Disease; Diarrhea, Infantile; Female; Ganglioneuroma; Humans; Infant; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

Topics: Bile Acids and Salts; Colonic Diseases; Diarrhea, Infantile; Exocrine Pancreatic Insufficiency; Gastrointestinal Neoplasms; Humans; Infant; Infant, Newborn; Intestinal Absorption; Intestinal Diseases; Intestinal Mucosa; Intestinal Secretions; Intestine, Small; Intestines; Protein-Energy Malnutrition; Rotavirus Infections; Vasoactive Intestinal Peptide

In this case report we describe a case of mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide (VIP), causing secretory diarrhoea in an 18-month-old child. An 18-month-old girl presented with a 2-month history of diarrhoea, abdominal distension and weight loss. Investigations revealed secretory diarrhoea with hypokalaemia, hyponatraemia and hypochloraemia and metabolic acidosis. Her stool output was 2.5-3.lday(-1) with increased stool sodium. VIP levels were strikingly high with normal glucagon and gastrin levels. X-ray of the chest revealed a well-defined mass in the right upper zone with tracheal shift, which was confirmed with computed tomography (CT) of the chest. The mass was resected and the patient became asymptomatic. This case shows that secretory diarrhoea caused by VIP and produced by ganglioneuroblastoma indicates a favourable prognosis, provided it is resectable.

Topics: Diarrhea, Infantile; Female; Ganglioneuroblastoma; Humans; Infant; Mediastinal Neoplasms; Radiography; Vasoactive Intestinal Peptide

Topics: Calcinosis; Catecholamines; Chronic Disease; Colon; Diagnosis, Differential; Diarrhea, Infantile; Failure to Thrive; Feces; Female; Ganglioneuroma; Humans; Infant; Radiography; Spinal Cord Neoplasms; Spine; Vasoactive Intestinal Peptide

Topics: Adrenal Gland Neoplasms; Adrenal Glands; Diarrhea, Infantile; Female; Ganglioneuroblastoma; Humans; Infant; Vasoactive Intestinal Peptide

A 18-month-old boy with stage 4 neuroblastoma needed intensive care because of prerenal acute renal failure related to an intractable watery diarrhoea syndrome occurring 10 months after the diagnosis of the primary tumor. This diarrhoea was in relation with a late hyperproduction of vasoactive intestinal peptide by the relapsing neuroblastoma itself and stopped with intravenous somatostatin administration.

Topics: Diarrhea, Infantile; Humans; Infant; Male; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neuroblastoma; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

Neural crest tumors can be complicated by secretory diarrhea mediated by vasoactive intestinal peptide (VIP). An eight-month-old male with a several-month history of secretory diarrhea is described. Elevated urine vanillylmandelic acid (VMA), total urine catecholamines, and plasma VIP indicated that a neural crest tumor was responsible for his protracted diarrhea. An extensive search for the tumor including CT scans of his head, neck, thorax, abdomen, and pelvis was unrevealing. A selective vena caval catheterization showed elevated catecholamines in a sample obtained above the renal veins. Subsequent laparotomy disclosed a benign ganglioneuroma arising from the left adrenal; the diarrhea resolved after its removal. Selective venous sampling proved useful in establishing the tumor's location where other techniques had been unsuccessful.

Topics: Adrenal Gland Neoplasms; Catecholamines; Diarrhea, Infantile; Ganglioneuroma; Humans; Infant; Male; Vasoactive Intestinal Peptide; Vena Cava, Inferior

A 23-month-old girl with intractable diarrhea that had persisted for the past 6 months showed typical evidence of water-diarrhea-hypokalemia-achlorhydria (WDHA) syndrome. Serum vasoactive intestinal peptide (VIP)-like immunoactivity was very high, and urine homovanillic acid and noradrenaline were also abnormally increased. A calcified tumor in the right paravertebral region was noted radiologically. Soon after resection of the tumor, the diarrhea dramatically improved. A ganglioneuroblastoma was histologically confirmed. Serum VIP decreased to normal level at 1 h after the removal of the tumor. VIP activity in the tumor was extraordinarily high, and the cells were stained by the indirect immunofluorescence technique with anti-VIP serum. This is to report the rapid turnover of serum level of VIP after resection of ganglioneuroblastoma with WDHA syndrome in a child.

Topics: Achlorhydria; Child; Child, Preschool; Diarrhea, Infantile; Female; Ganglioneuroma; Gastrointestinal Hormones; Humans; Hypokalemia; Infant; Male; Retroperitoneal Neoplasms; Syndrome; Time Factors; Vasoactive Intestinal Peptide

An 8-month-old boy with persistent watery diarrhoea and failure to thrive developed abdominal distension, hypokalaemia, and flushing of the face and trunk. A high concentration of vasoactive intestinal peptide-like immunoreactivity was found in the serum. Soon after resection of a suprarenal mass, the serum level of vasoactive intestinal peptide became normal and the diarrhoea stopped. Histologically the tumour was a ganglioneuroblastoma: the cells showed fluorescence by the indirect immunofluorescence technique with anti-vasoactive intestinal peptide serum. Electron microscopical examination showed abundant secretory granules in the tumour cells. Reports of chronic watery diarrhoea in children due to neural crest tumours are reviewed, with particular respect to the clinical features of the syndrome.

Topics: Abdominal Neoplasms; Diarrhea, Infantile; Fluorescent Antibody Technique; Ganglioneuroma; Gastrointestinal Hormones; Humans; Infant; Male; Microscopy, Electron; Vasoactive Intestinal Peptide

The case of an infant who developed refractory watery diarrhea at the age of 2 weeks is described. Diarrhea was secretory in type, stool weight on no oral intake was 400 to 600 gm daily. A vasoactive intestinal peptide (VIP)-producing tumor was suspected. At the age of 7 1/2 months an exploratory laparotomy revealed nonbeta islet cell hyperplasia of the pancreas. VIP levels were elevated in plasma and pancreatic tissue. After 95% pancreatectomy, plasma VIP level dropped to normal. Hypokalemia, described in adult patients with VIP-producing pancreatic tumors and refractory watery diarrhea, was not a significant problem in this infant. This is the first report on the association of refractory watery diarrhea with elevated levels of plasma VIP and pancreatic islet nonbeta cell hyperplasia in the pediatric age group.

Topics: Adenoma, Islet Cell; Chronic Disease; Diagnosis, Differential; Diarrhea, Infantile; Humans; Hyperplasia; Infant, Newborn; Infant, Newborn, Diseases; Islets of Langerhans; Male; Pancreatic Neoplasms; Vasoactive Intestinal Peptide

Topics: Adrenal Gland Neoplasms; Child; Diarrhea, Infantile; Ganglioneuroma; Gastrointestinal Hormones; Humans; Male; Vasoactive Intestinal Peptide

The authors report a case of cervical ganglioneuroblastoma associated with intractable watery diarrhea and hypokalemia. The probable physiopathologic mechanism of the diarrhea and its relation to the vasoactive intestinal peptide secretion (VIP) by the tumor are discussed.

Topics: Diarrhea, Infantile; Ganglioneuroma; Gastrointestinal Hormones; Head and Neck Neoplasms; Humans; Infant; Male; Vasoactive Intestinal Peptide

A 1-year-old boy had intractable diarrhea and symptoms of the watery-diarrhea-hypokalemia-achlorhydria (WDHA) syndrome, a well-known entity in adults. Resection of a ganglioneuroblastoma situated in the neck caused prompt relief of symptoms. The ganglioneuroblastoma in this instance contained the enterohormone vasoactive intestinal peptide (VIP); blood levels of this peptide were elevated preoperatively. After tumor resection, the VIP level returned to normal, and the diarrhea ceased on the day of the operation. The genesis of the diarrhea in relation to the production of polypeptides from neuroendocrine origin is discussed (APUD-cell concept). VIP may be the mediator of the WHDA syndrome in ganglioneuroblastoma.

Topics: Colon; Diagnosis, Differential; Diarrhea, Infantile; Ganglioneuroma; Gastrointestinal Hormones; Head and Neck Neoplasms; Humans; Infant; Male; Peptides; Vasoactive Intestinal Peptide

Topics: Child, Preschool; Chronic Disease; Diarrhea, Infantile; Ganglioneuroma; Gastrointestinal Hormones; Humans; Male; Thoracic Neoplasms; Vasoactive Intestinal Peptide