Delayed Presentation of Berardinelli-Siep Lipodystrophy in an Adolescent Female.

Berardinelli-Siep syndrome (BSS) is a form of congenital generalized lipodystrophy that disrupts the pathways of lipid metabolism. It presents with physical exam findings, including muscular hypertrophy and lipoatrophy as well as serious metabolic consequences such as diabetes mellitus, hypertriglyceridemia, acute pancreatitis, hepatomegaly, and hepatic steatosis. Diagnosis generally occurs soon after birth or in childhood. The case presented is significant for a delayed diagnosis of suspected BSS Type 1 which is rather uncommon in a developed country. Due to the detrimental complications of BSS, such as hypertrophic cardiomyopathy, pancreatitis, and liver disease, early diagnosis and intervention are crucial. Pediatric providers must be knowledgeable about physical features of BSS and common presentations such as new onset diabetes, hypertriglyceridemia, or pancreatitis throughout early childhood and adolescence in order to avoid delayed diagnoses.

Sensory Characteristics of Reduced Nitrite Bacon Manufactured by the Wisconsin Process.

Bacon with a culture of lactic acid-forming bacteria, Pediococcus acidilactici , plus 0.7% sucrose and 40 or 80 mg sodium nitrite/kg (Wisconsin Process), and control bacon with 120 mg sodium nitrite/kg but no lactic acid bacteria and sucrose, were produced at three commercial bacon plants under production conditions. The bacon was stored under refrigeration for 5 to 8 wk, then subjected to sensory analyses by an experienced sensory panel. Quantitative descriptive visual analysis was performed on uncooked as well as cooked samples, and the cooked samples were served for quantitative descriptive sensory analysis. Results indicated that the test bacon with reduced amounts of sodium nitrite was as acceptable as the control bacon with no sugar and lactics, with the 80 mg/kg nitrite-bacon being the most preferred of all. These results and the results of botulinal challenge and nitrosamine tests indicate that the test process can be a satisfactory alternative to processing bacon by the conventional procedure with 120 mg sodium nitrite/kg.