. PAS III, considered a subtype of PAS II, presents with AITD and one or more other organ-specific autoimmune disease(s), most commonly involving the pancreas, stomach, intestine or skin; the adrenal and parathyroid glands are uninvolved ( 5 , 6
M Horsey, P Hogan, and T Oliver
Shweta Birla, Viveka P Jyotsna, Rajiv Singla, Madhavi Tripathi, and Arundhati Sharma
except for hypercalcemia (tested twice 10.8 and 9.2 mg/dl (normal range=8.1–10.4 mg/dl) and hypophosphatemia (2 and 2.1 mg/dl (normal range=2.5–4.5 mg/dl). Gastrointestinal endoscopy revealed thickened fundus and body of the stomach with edematous folds
Andrea Pucci, Wui Hang Cheung, Jenny Jones, Sean Manning, Helen Kingett, Marco Adamo, Mohamed Elkalaawy, Andrew Jenkinson, Nicholas Finer, Jacqueline Doyle, Majid Hashemi, and Rachel L Batterham
Sleeve gastrectomy (SG) is the second most commonly performed bariatric procedure worldwide. Altered circulating gut hormones have been suggested to contribute post-operatively to appetite suppression, decreased caloric intake and weight reduction. In the present study, we report a 22-year-old woman who underwent laparoscopic SG for obesity (BMI 46 kg/m2). Post-operatively, she reported marked appetite reduction, which resulted in excessive weight loss (1-year post-SG: BMI 22 kg/m2, weight loss 52%, >99th centile of 1-year percentage of weight loss from 453 SG patients). Gastrointestinal (GI) imaging, GI physiology/motility studies and endoscopy revealed no anatomical cause for her symptoms, and psychological assessments excluded an eating disorder. Despite nutritional supplements and anti-emetics, her weight loss continued (BMI 19 kg/m2), and she required nasogastric feeding. A random gut hormone assessment revealed high plasma peptide YY (PYY) levels. She underwent a 3 h meal study following an overnight fast to assess her subjective appetite and circulating gut hormone levels. Her fasted nausea scores were high, with low hunger, and these worsened with nutrient ingestion. Compared to ten other post-SG female patients, her fasted circulating PYY and nutrient-stimulated PYY and active glucagon-like peptide 1 (GLP1) levels were markedly elevated. Octreotide treatment was associated with suppressed circulating PYY and GLP1 levels, increased appetite, increased caloric intake and weight gain (BMI 22 kg/m2 after 6 months). The present case highlights the value of measuring gut hormones in patients following bariatric surgery who present with anorexia and excessive weight loss and suggests that octreotide treatment can produce symptomatic relief and weight regain in this setting.
- Roux-en-Y gastric bypass and SG produce marked sustained weight reduction. However, there is a marked individual variability in this reduction, and post-operative weight loss follows a normal distribution with extremes of ‘good’ and ‘poor’ response.
- Profound anorexia and excessive weight loss post-SG may be associated with markedly elevated circulating fasted PYY and post-meal PYY and GLP1 levels.
- Octreotide treatment can produce symptomatic relief and weight regain for post-SG patients that have an extreme anorectic and weight loss response.
- The present case highlights the value of measuring circulating gut hormone levels in patients with post-operative anorexia and extreme weight loss.
G K Dimitriadis, K Gopalakrishnan, R Rao, D K Grammatopoulos, H S Randeva, M O Weickert, and N Murthy
We report the case of a 70-year-old previously healthy female who presented acutely to the Accident and Emergency department with left-sided vasomotor symptoms including reduced muscle tone, weakness upon walking and slurred speech. Physical examination confirmed hemiparesis with VIIth nerve palsy and profound hepatomegaly. A random glucose was low at 1.7 mmol/l, which upon correction resolved her symptoms. In hindsight, the patient recalled having had similar episodes periodically over the past 3 months to which she did not give much attention. While hospitalized, she continued having episodes of symptomatic hypoglycaemia during most nights, requiring treatment with i.v. dextrose and/or glucagon. Blood tests including insulin and C-peptide were invariably suppressed, in correlation with low glucose. A Synacthen stimulation test was normal (Cort (0′) 390 nmol/l, Cort (30′) 773 nmol/l). A computed tomography scan showed multiple lobulated masses in the abdomen, liver and pelvis. An ultrasound guided biopsy of one of the pelvic masses was performed. Immunohistochemistry supported the diagnosis of a gastrointestinal stromal tumour (GIST) positive for CD34 and CD117. A diagnosis of a non islet cell tumour hypoglycaemia (NICTH) secondary to an IGF2 secreting GIST was confirmed with further biochemical investigations (IGF2=96.5 nmol/l; IGF2:IGF1 ratio 18.9, ULN <10). Treatment with growth hormone resolved the patient's hypoglycaemic symptoms and subsequent targeted therapy with Imatinib was successful in controlling disease progression over an 8-year observation period.
- NICTH can be a rare complication of GISTs that may manifest with severe hypoglycaemia and neuroglucopenic symptoms.
- NICTH can masquerade as other pathologies thus causing diagnostic confusion.
- Histological confirmation of GIST induced NICTH and exclusion of other conditions causing hypoglycaemia is essential.
- Mutational analysis of GISTs should be carried out in all cases as it guides treatment decision.
- Tailored management of hypoglycaemia, in this case using growth hormone and targeted cyto-reductive therapy, minimizes the risk of possible life-threatening complications.
Jerena Manoharan, Caroline L Lopez, Karl Hackmann, Max B Albers, Anika Pehl, Peter H Kann, Emily P Slater, Evelin Schröck, and Detlef K Bartsch
We report about a young female who developed an unusual and an aggressive phenotype of the MEN1 syndrome characterized by the development of a pHPT, malignant non-functioning pancreatic and duodenal neuroendocrine neoplasias, a pituitary adenoma, a non-functioning adrenal adenoma and also a malignant jejunal NET at the age of 37 years. Initial Sanger sequencing could not detect a germline mutation of the MEN1 gene, but next generation sequencing and MPLA revealed a deletion of the MEN1 gene ranging between 7.6 and 25.9 kb. Small intestine neuroendocrine neoplasias (SI-NENs) are currently not considered to be a part of the phenotype of the MEN1-syndrome. In our patient the SI-NENs were detected during follow-up imaging on Ga68-Dotatoc PET/CT and could be completely resected. Although SI-NENs are extremely rare, these tumors should also be considered in MEN1 patients. Whether an aggressive phenotype or the occurrence of SI-NENs in MEN1 are more likely associated with large deletions of the gene warrants further investigation.
- Our patient presents an extraordinary course of disease.
- Although SI-NENs are extremely rare, these tumors should also be considered in MEN1 patients, besides the typical MEN1 associated tumors.
- This case reports indicate that in some cases conventional mutation analysis of MEN1 patients should be supplemented by the search for larger gene deletions with modern techniques, if no germline mutation could be identified by Sanger sequencing.
Marcela Rodríguez Flores, Ruth Carmina Cruz Soto, Verónica Vázquez Velázquez, Reina Ruth Soriano Cortés, Carlos Aguilar Salinas, and Eduardo García García
In patients with gastric bypass (GB), high glucose variability (GV) and hypoglycemia have been demonstrated, which could impact the metabolic status and eating behavior. We describe the glucose patterns determined through continuous glucose monitoring (CGM) in two patients with >5 years follow-up after GB and significant weight recovery, who reported hypoglycemic symptoms that interfered with daily activities, and their response to a nutritional and psycho-educative prescription. Case 1: A 40-year-old woman without pre-surgical type 2 diabetes (T2DM) and normal HbA1c, in whom CGM showed high GV and hypoglycemic episodes that did not correlate with the time of hypoglycemic symptoms. Her GV reduced after prescription of a diet with low glycemic index and modification of meal patterns. Case 2: A 48-year-old male with pre-surgical diagnosis of T2DM and current normal HbA1c, reported skipping meals. The CGM showed high GV, 15% of time in hypoglycemia and hyperglycemic spikes. After prescription of a low glycemic index diet, his GV increased and time in hypoglycemia decreased. Through the detailed self-monitoring needed for CGM, we discovered severe anxiety symptoms, consumption of simple carbohydrates and lack of meal structure. He was referred for more intensive psychological counseling. In conclusion, CGM can detect disorders in glucose homeostasis derived both from the mechanisms of bariatric surgery, as well as the patient’s behaviors and mental health, improving decision-making during follow-up.
- High glycemic variability is frequent in patients operated with gastric bypass.
- Diverse eating patterns, such as prolonged fasting and simple carbohydrate ingestion, and mental health disorders, including anxiety, can promote and be confused with worsened hypoglycemia.
- CGM requires a detailed record of food ingested that can be accompanied by associated factors (circumstances, eating patterns, emotional symptoms). This allows the detection of particular behaviors and amount of dietary simple carbohydrates to guide recommendations provided within clinical care of these patients.
Mohammed Al-Sofiani, Dhimitri Nikolla, and V V S Ramesh Metta
. The patient was started on furosemide 40 mg intravenously daily for 2 days and the l -T 4 dose was increased from 175 to 225 μg. The patient was advised to take l -T 4 first in the morning on an empty stomach. Outcome and follow-up Over the
Tiago Nunes da Silva, (Loes) M L F van Velthuysen, Casper H J van Eijck, Jaap J Teunissen, (Hans) J Hofland, and Wouter W de Herder
(RV) < 94 μg/L). Abdominal CT showed a 12.8 cm mass in the pancreatic body with compression of the stomach and the superior mesenteric vein a 37 mm lymph node metastasis in the gastrohepatic ligament and several multilobar liver metastases ( Fig. 1A
Bernardo Marques, Raquel G Martins, Guilherme Tralhão, Joana Couto, Sandra Saraiva, Henrique Ferrão, João Ribeiro, Jacinta Santos, Teresa Martins, Ana Teresa Cadime, and Fernando Rodrigues
and the stomach (4.3 cm). Liver biopsy revealed LM of a grade 2 NET (Ki67 = 10%). She was then sent to our department for further investigation and treatment. Investigation Anamnesis revealed that she had a gastric lesion excised 16 years
Mohd Shazli Draman, Aoife Brennan, Michael Cullen, and John Nolan
Thomas Addison in 1849 and was later linked to the stomach by Austin Flint. The underlying pathological lesion is the autoimmune destruction of the gastric parietal cells with antibody formation to the parietal cell itself and to its secretory product