Early Atherosclerosis in Children in Familial Hypercholesterolemia : A 2 Case Report

Familial hypercholesterolemia (FH) is inherited as an autosomal dominant. It is an important clinical condition leading to hypertension, atherosclerosis, and premature coronary artery disease. Atherosclerosis is rarely reported in children. We report here 2 children with familial hypercholesterolemia and early atherosclerosis. The first case is a 4 year-old Thai boy who presented with yellowish papules in the skin creases which had been present for 2 years. His grandfather and father had been diagnosed with diabetes mellitus and hypercholesterolemia, respectively. Physical examination revealed his weight for height to be 125% and planar xanthomas. The remaining examination was unremarkable. Plasma lipid profiles showed a total cholesterol level of 674 mg/dl, a triglyceride level of 261 mg/dl, a high-density lipoprotein cholesterol (HDL-C) level of 39 mg/dl, and a low-density lipoprotein cholesterol (LDL-C) level of 583 mg/dl. The results of a carotid duplex scan showed atherosclerotic plaques in both carotid arteries. The second case is a 10 year-old Thai boy who had no symptoms, but his mother and uncle had been diagnosed with coronary heart disease and diabetes mellitus, respectively. Physical examination revealed a weight for height of 112%, but the remaining examination was unremarkable. Plasma lipid profiles showed a total cholesterol level of 328 mg/dl, a triglyceride level of 59 mg/dl, and HDL-C level of 54 mg/dl, and an LDL-C level of 262 mg/dl. A carotid duplex scan showed atherosclerotic plaques in both carotid arteries. Atherosclerosis is rarely reported in children and it usually correlates with ischemic heart disease. The finding of atherosclerotic plaques in the large arteries may reflect deposition of atheroma in their coronary arteries. We suggest that this investigation is an important procedure in the screening of early atherosclerosis in order to primarily and secondarily prevent premature coronary artery disease

Vitamin D Deficiency, Pancreatic and Small Intestinal Enzyme Development in Rats

Maternal vitamin D deficiency has been shown to lead to reduced body weights in developing rat pups. To evaluate the effects of vitamin D deficiency alone both in dams and pups during the perinatal age on the ontogeny of gastrointestinal enzymes, female weanling rats (3 weeks of age) were divided into three groups. Groups I and III were fed a control (vitamin-D-replete) diet. Group II were fed a vitamin-D-deficient diet. Six weeks afterward they were mated with normal male rats while continuing on their respective diets until sacrifice. Only rats that delivered their pups on the same day from each group were brought into the study. Litter sizes of groups I and II were adjusted to 10, while group III was adjusted to 13 such that the rate of growth paralleled that of group II. At 19 days after birth, all dams and pups were sacrificed. There were no differences in the calcium and phosphorus contents in breast milk obtained from dams of each group. The serum calcium concentration of pups from group II (vitamin-D-deficient) was lower than the other groups. Body weights of pups from groups II and III were significantly lower than those of group I. The mucosal weight, total mucosal protein, mucosal DNA, sucrase, and maltase activities from groups II and III were similar, but lower than group I. Pancreatic weight, total pancreatic protein, DNA, amylase, and lipase activities from groups II and III were also similar, but lower than group I. Vitamin D deficiency was confirmed in both dams and pups from group II. This deficiency during gestation and lactation apparently affected the development of gastrointestinal enzymes. The effect was, however, similar to that due to an increase in litter size (group III). The results suggest that vitamin D deficiency in dams probably caused lower milk volume which resulted in a partial dietary restriction to the pups, thus affecting the development of their pancreatic and mucosal enzymes. The effect of vitamin D deficiency is, therefore, secondary.</jats:p

Global standard for the composition of infant formula : recommendations of an ESPGHAN coordinated international expert group.

The Codex Alimentarius Commission of the Food and Agriculture Organization of the United Nations (FAO) and the World Health Organization (WHO) develops food standards, guidelines and related texts for protecting consumer health and ensuring fair trade practices globally. The major part of the world's population lives in more than 160 countries that are members of the Codex Alimentarius. The Codex Standard on Infant Formula was adopted in 1981 based on scientific knowledge available in the 1970s and is currently being revised. As part of this process, the Codex Committee on Nutrition and Foods for Special Dietary Uses asked the ESPGHAN Committee on Nutrition to initiate a consultation process with the international scientific community to provide a proposal on nutrient levels in infant formulae, based on scientific analysis and taking into account existing scientific reports on the subject. ESPGHAN accepted the request and, in collaboration with its sister societies in the Federation of International Societies on Pediatric Gastroenterology, Hepatology and Nutrition, invited highly qualified experts in the area of infant nutrition to form an International Expert Group (IEG) to review the issues raised. The group arrived at recommendations on the compositional requirements for a global infant formula standard which are reported here

Thermophilic Amylase-Digested Rice-Electrolyte Solution in the Treatment of Acute Diarrhea in Children

Objective. To compare the efficacy of an oral rehydration solution (ORS) containing short polymers of glucose derived from rice (Amylyte-ORS) and five times the caloric density of current ORS to the standard glucose-ORS (World Health Organization [WHO] = ORS) in the treatment of acute diarrhea in children. Methods. The rice ORS (Amylyte-ORS) was obtained by adding thermophilic amylase (252 500 MW units) and salts (1.5 g NaCl, 600 mg KCl, and 150 mg CaCl2) to 100 g rice and boiling for 10 minutes in 500 mL water. This yields 250 mL Amylyte-ORS, which contains 92% to 96% short-chain glucose polymers, three to nine molecules in length, and provides 425 kcal/L, compared to 80 kcal/L for the WHO-ORS. One hundred forty-four male children, 4 months to 3 years of age, presenting with acute diarrhea and mild, moderate, or severe dehydration, were assigned by random allocation to receive either WHO-ORS or Amylyte-ORS. Data from 127 children were analyzed (57 received the WHO-ORS and 70 the Amylyte-ORS). Two children given Amylyte-ORS and 15 given the WHO-ORS were not included in the analysis because of improperly collected data or lost urine or fecal specimens. None were given antibiotics during the study. Free water and feeding were allowed after the children were rehydrated. Results. The clinical characteristics of the children in the two treatment groups were comparable. Five children who received the WHO-ORS and three children given Amylyte-ORS were treatment failures. Amylyte-ORS reduced diarrhea duration by 15% (41.4 ± 2.5 vs 34.7 ± 1.8 hours; P &amp;lt; .03) compared to the WHO-ORS, regardless of the severity of dehydration. In the Amylyte-treated group, ORS requirements were significantly less (234 ± 15.2 vs 295 ± 17.6 mL/kg P &amp;lt; .01) and weight gain was significantly more (367.7 ± 45.1 vs 199.2 ± 38.2 g; P &amp;lt; .01) than in those given the WHO-ORS. The net intestinal fluid balance and total body fluid balance were similar in the two groups. Conclusions. Amylyte-ORS efffectively rehydrates children with acute diarrhea, reduces diarrhea duration, decreases ORS requirements, and improves weight gain compared to the WHO-ORS.</jats:p