Addison's disease in type 1 diabetes presenting with recurrent hypoglycaemia

Abstract

A 42 year old man presented with glutenresponsive coeliac disease and secondary pancreatic insuYciency. Subsequently his symptoms relapsed and repeat small intestinal biopsy showed villous atrophy and infiltration by leukaemic cells, despite continuation of a gluten-free diet. Serious causes of relapse and non-responsiveness in coeliac disease include enteropathyassociated T-cell lymphoma, ulcerative jejunitis and an end-stage hypoplastic mucosa. This is the first report of nonresponsiveness due to infiltration by leukaemia. (Postgrad Med J 2000;76:227-229) Keywords: coeliac disease; villous atrophy; gluten sensitivity; leukaemia Most patients with coeliac disease respond satisfactorily to a gluten-free diet. A small number fail to respond, either initially or after a period of treatment. There are many causes for this. [1][2][3] In such a situation the diagnosis should be carefully reviewed and a search made for serious causes of the non-responsiveness. Case reportA 42 year old man presented with a 6-month history of severe watery diarrhoea, dehydration and weight loss of 6.4 kg. He had previously been well, although had always had loose stools since childhood. Investigation revealed a macrocytic anaemia with a low serum folate, and subtotal villous atrophy on duodenal biopsy (figure, A). Coeliac disease was diagnosed and a gluten-free diet (GFD) was started.Seven months later a repeat duodenal biopsy showed significant histological improvement (figure, B), suggesting mucosal glutenresponsiveness and supporting the diagnosis of coeliac disease. However, the diarrhoea was still present and failed to improve, despite the subsequent addition of a lactose-free diet, prednisolone and metronidazole. The macrocytic anaemia had progressed, despite folic acid replacement, and splenomegaly had developed. Bone marrow examination revealed chronic myelomonocytic leukaemia (CMML). He required monthly blood transfusions.At that stage, 18 months after his initial symptoms and 11 months after starting a GFD, he was referred to us for further assessment of his diarrhoea. He still had watery diarrhoea up to 10 times per day. He was lethargic and had lost a further 6.4 kg in weight. There was no rectal bleeding or abdominal pain. On examination he was thin and wasted, with splenomegaly and mild peripheral oedema.Investigations directed towards finding the cause of his diarrhoea included: haemoglobin 10.6 g/dl, white cell count 34 × 10 9 /l, platelets 46 × 10 9 /l, mean corpuscular volume 108 fl, monocytosis and leucoerythroblastic blood film; prothrombin time 23.8 s, albumin 22 g/l, alkaline phosphatase 679 IU/l, vitamins A and E decreased, ferritin 2310 µg/l, IgA antigliadin positive. Enteroscopy showed ulceration in the second and third parts of the duodenum. Colonoscopy and small bowel enema were normal. Abdominal computed tomography (CT) scan revealed hepatosplenomegaly and low volume inguinal lymphadenopathy. Pancreolauryl test was low at 18.5% (normal >30%). A SeHCAT scan showed severe bile salt malabsorption.The ...

ISPAD Clinical Practice Consensus Guidelines 2018: Assessment and management of hypoglycemia in children and adolescents with diabetes

“…The majority of children with type 1 diabetes who experience severe hypoglycemia have isolated events, however, a few experience recurrent episodes. When hypoglycemia is recurrent, it is important to exclude IAH and rule out coexisting autoimmune disorders such as subclinical hypothyroidism, coeliac disease, and Addison's disease . The introduction of a gluten‐free diet and appropriate treatment of Addison's disease and hypothyroidism may reduce the frequency of hypoglycemia.…”

Screening for Addison's disease in patients with type 1 diabetes mellitus and recurrent hypoglycaemia

“…A ddison's disease may present as recurrent hypoglycaemia in patients with type 1 diabetes mellitus [1][2][3][4][5][6][7] and it is recommended that diabetic patients with unexplained recurrent hypoglycaemia be screened for Addison's disease. 8 There are, however, no data on the prevalence of Addison's disease presenting with recurrent hypoglycaemia in patients with diabetes.…”

Autoimmune polyglandular syndromes in the adults: the genetic and immunological diagnostic criteria

“…При этом по мере ком-пенсации глюкокортикоидной недостаточности по-требность в инсулине увеличивается [71][72][73][74].…”