Mauriac Syndrome in a Nigerian child with Type 1 Diabetes Mellitus: A Case Report

A 14-year-old boy with Type 1 Diabetes mellitus (diagnosed at eight years of age) presented with complaints of fever, weight loss, growth failure, pubertal delay, abdominal swelling and discomfort. He was on Premixed insulin (70/30) with inadequate follow-up and poor diabetic control. Examination revealed cachexia, generalised lymphadenopathy, a protuberant abdomen and hepatosplenomegaly. Anthropometry showed a bodyweight of 19.6kg, a height of 116cm and a BMI of 14.1kg/m2, all markedly below the 3rd centile. He had no secondary sexual characteristics: axillary hair stage 1, pubic hair stage 1, penile length of 4.9cm and prepubertal testicular volumes of 3mls bilaterally. At presentation, his random blood glucose was 400mg/dl, and glycosylated haemoglobin was 11.6%. Screening for tuberculosis, human immunodeficiency virus, hepatitis and lymphoproliferative disorders were negative. Other blood investigation findings included leucocytosis, erythrocyte sedimentation rate of 30mm/hr, normal liver function tests, normal serum electrolytes, urea and creatinine. His haemoglobin genotype was AS. Chest radiograph showed features of bronchopneumonia. A presumptive diagnosis of Mauriac Syndrome was made. With the optimisation of glycaemic control, he improved clinically with a weight gain of 5.7kg over four months and resolution of hepatosplenomegaly.

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Severe Growth Failure and Poorly Controlled Type 1 Diabetes Mellitus in a 7-Year-Old Girl: Mauriac Syndrome

Journal of College of Physicians And Surgeons Pakistan ◽

10.29271/jcpsp.2019.06.590 ◽

2019 ◽

Vol 29 (6) ◽

pp. 590-591

Author(s):

Saima Gillani ◽

Attia Iqbal ◽

Nasir Kazmi

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Type 1 Diabetes Mellitus ◽

Growth Failure ◽

Mauriac Syndrome ◽

Severe Growth

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MAURIAC SYNDROME WITH CELIAC DISEASE: RARE ASSOCIATION

International Journal of Innovative Research in Medical Science ◽

10.23958/ijirms/vol01-i03/01 ◽

2016 ◽

Vol 1 (03) ◽

Author(s):

Gutch Manish ◽

Saransh Singh ◽

Annesh Bhatacharya ◽

Harendra Singh ◽

Kumar Sukriti ◽

...

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Celiac Disease ◽

Type 1 Diabetes Mellitus ◽

Growth Failure ◽

Rare Association ◽

Mauriac Syndrome ◽

Severe Growth

Growth failure in type 1 Diabetes Mellitus can occur for several reasons. Mauriac syndrome is a rare cause of severe growth failure in type 1 Diabetes Mellitus.The classical severe presentation of Celiac Disease rarely occurs in type 1 Diabetes Mellitus patients, but more often patients have few/mild symptoms of Celiac Disease or are completely asymptomatic (silent CD). In fact diagnosis of Celiac Disease is regularly performed by means of the screening in type 1 Diabetes Mellitus patients.There may be different forms and etiologies involved in Mauriac syndrome. We hereby report a case of Mauriac syndrome in type 1 Diabetes Mellitus with simultaneous celiac disease which is very rarely reported.

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Persistent lactic acidosis in the Mauriac syndrome in type 1 diabetes mellitus

Baylor University Medical Center Proceedings ◽

10.1080/08998280.2020.1866936 ◽

2021 ◽

pp. 1-2

Author(s):

Abinash Subedi ◽

Vishnu Charan Suresh Kumar ◽

Anuj Sharma ◽

Gilles Hoilat ◽

Savio John

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Lactic Acidosis ◽

Type 1 Diabetes Mellitus ◽

Mauriac Syndrome

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High Prevalence of the Polycystic Ovary Syndrome and Hirsutism in Women with Type 1 Diabetes Mellitus1

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jcem.85.11.6931 ◽

2000 ◽

Vol 85 (11) ◽

pp. 4182-4187 ◽

Cited By ~ 3

Author(s):

Héctor F. Escobar-Morreale ◽

Belén Roldán ◽

Raquel Barrio ◽

Milagros Alonso ◽

José Sancho ◽

...

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Polycystic Ovary Syndrome ◽

Type 1 Diabetes Mellitus ◽

Polycystic Ovary ◽

Normal Serum ◽

Ovary Syndrome ◽

Menstrual Dysfunction ◽

High Prevalence

The current recommendation for strict metabolic control of type 1 diabetes mellitus requires the administration of supraphysiological doses of insulin, which might result in insulin-mediated stimulation of androgen synthesis, as occurs in insulin-resistant states. At present, the prevalence of hyperandrogenic disorders in women with type 1 diabetes mellitus is unknown. Eighty-five women with type 1 diabetes mellitus were evaluated for symptoms and signs of hyperandrogenism. In 68 of the patients, several serum androgen and hormone concentrations were measured. The polycystic ovary syndrome (PCOS) was defined by the presence of menstrual dysfunction, together with clinical and/or biochemical evidence of hyperandrogenism, and exclusion of other etiologies. Eighteen healthy women, menstruating regularly, served as controls for the androgenic profiles. Thirty-three patients (38.8%) presented hyperandrogenic disorders (16 had PCOS, and 17 had hirsutism without menstrual dysfunction). Type 1 diabetic patients with PCOS presented increased serum total and free testosterone concentrations, and serum androstenedione levels, but had normal serum sex hormone-binding globulin and dehydroepiandrosterone-sulfate levels. Hirsute type 1 diabetic women without menstrual dysfunction presented normal serum androgen levels. There were no significant differences between hyperandrogenic and nonhyperandrogenic type 1 diabetes mellitus women in clinical variables such as the duration of diabetes, age at diagnosis of diabetes, conventional or intensive insulin therapy, mean daily insulin dosage, or metabolic control. In conclusion, women with type 1 diabetes mellitus have a high prevalence of hyperandrogenic disorders, including PCOS and hirsutism.

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