Brief clinical and laboratory observation

Chronic abdominal pain caused by sorbitol malabsorption

Xylitol and sorbitol are high-added-value chemicals with important applications in the food and pharmaceutical industries, since they present a sweetness similar to that of sucrose with lower caloric content and glycemic index, insulin-independent metabolism, and microbiological and chemical stability. The market focused on these polyols has been continuously growing due to their relevant properties and to the likelihood of using them as platform chemicals. Nowadays, they are produced at the commercial scale based on chemical routes that still present significant efficiency and sustainability issues, a fact that has encouraged research focusing on the development of biotechnological routes based on using productive chain by-products, mainly lignocellulosic and starchy biomasses, as raw material. It has been suggested that integrating xylitol and sorbitol production to biorefineries would contribute to the technical and economic feasibility of these productive units. The aim of the current chapter is to explore chemical and biotechnological routes for xylitol and sorbitol production, their applications and market trends, as well as challenges and opportunities for the incorporation of these important polyols into biorefineries.

Sorbitol is a natural sugar alcohol with an increasing range of applications from the food industry (as a sweetener) to pharmaceutical applications (mainly as a carrier) to the cosmetics industry (as an emulsion stabilizer). Sorbitol is considered to be one of the top 12 high value-added building block intermediate chemicals that can be produced from renewable biomass resources, mainly from corn starch–based glucose and corn steep liquor. It can be also produced by catalytic hydrogenation or electrolytic reduction of glucose. The largest production and consumption worldwide are concentrated in China, where the main vitamin C industry is located.

To evaluate the most frequent diagnostic errors in patients with Fabry disease and the types of specialists most often consulted before diagnosis.

We evaluated 45 consecutive symptomatic patients with Fabry disease confirmed by enzymatic tests in males and genetic studies in females. We interviewed the patients, their mothers, or both regarding symptoms, age at onset, medical consultations, and recommended treatments.

Neuropathic pain was the most frequent initial complaint, and rheumatic fever was the most common diagnosis. Seven patients were treated with penicillin for many years. Ten patients sought medical consultation because of abdominal pain and were diagnosed with food intoxication or nonspecific pain. Six patients sought consultation because of anhidrosis, considered of unclear cause, and angiokeratomas diagnosed as petechiae. Internists and pediatricians were the most frequently consulted specialists. The correct diagnosis was obtained after a mean of 19.7 years.

Pediatricians as well as internists commonly misdiagnose Fabry disease. Neuropathic pain, hypohidrosis, and recurrent abdominal pain in childhood or adolescence should include Fabry disease in the differential diagnosis to facilitate earlier diagnosis and treatment of these patients.