Introduction Severe malnutrition in childhood is considered a global burden. Micronutrient and protein-calorie deficiencies encompass a clinical spectrum from a minor nutritional problem to a myriad of life-threatening disorders. Despite worldwide awareness, 45% of the children deaths under five years old in the year 2012 were because of undernutrition.

Case-reports A 16-month-old girl (case 1) and a 19-month-old girl (case 2) were admitted to our hospital for generalised oedema of unknown cause. Physical examination revealed both children with severe stunting (Z-score length −4.2SD case 1,–3.3.3SD case 2, according to WHO), with pallor, anasarca, angular stomatitis, thin, sparse hair and ridged nails. They presented dysphonia, loose stools, irritability and severe neurodevelopment defects. Examination of the skin described diaper dermatitis in case 1, while bullae, erosion and desquamation were encountered in case 2. Case 1 had hepatomegaly of 2 cm below the right lower costal margin. Laboratory investigations showed severe carential anaemia, hypoglycemia, low cholesterol and triglycerides, low alpha 1-antitrypsin, hypoalbuminemia, altered coagulation and metabolic acidosis. Serology tests resulted negative for infections with human immunodeficiency virus. Stool analysis revealed malabsorption syndrome. Anti-transglutaminase antibody was negative. Moreover, chronic liver disease was found in Case 1, associated with a hyperechogenic granular image; initial results were leading to an ambiguous diagnosis including a possible cystic fibrosis, metabolic disorders or hipoceruloplasminemia. In Case 2 we considered infectious and genetic causes. After excluding the aforementioned conditions, findings regarding physical and laboratory evaluations were attributed to severe malnutrition considering the socioeconomic status. After receiving a high-calorie and high-protein diet, intravenous albumin, antibiotic, vitamin K, blood transfusion and correction of micronutrient deficiencies, the growth-chart was positive for case 1, while no further contact was obtained with case 2.

Conclusions Although firstly described 80 years ago, research is still carried out to establish the correlation of malabsorption, recurrent infections, chronic underlying diseases and impaired immune response with severe malnutrition. Even if the prevalence of malnutrition has reduced, the socioeconomic status continues to play a significant role in the developing countries.