MELAS, Myoclonus, Ataxia and Deficiencies of Complexes I and IV in Muscle Mitochondria

A 13-year-old Japanese girl developed convulsions, periodic attacks of coma with hyperpyrexia, visual disturbance, aphasia, myoclonus, cerebellar symptoms and dementia. A muscle biopsy four years previously had shown ragged-red fibers. Biochemical studies showed NADH CoQ reductase and cytochrome c oxidase deficiencies in biopsied muscle at 13 years of age, although normal activity of cytochrome c oxidase had been histochemically detected at 9 years of age. A therapeutic trial of citrate administration produced transient improvement in the clinical features and in the hyperlactic and pyruvic acidemia.     

Stunting and Nutrient Deficiences in Children on Alternative Diets

Studies in children fed alternative diets showed that anthropometric parameters and the intake of energy and nutrients by macrobiotic children deviated most from current norms. Therefore weaning practice, growth and haematological status were investigated in 3 age-cohorts of macrobiotic fed infants between 4 and 18 months of age and a control group. A mixed longitudinal design was used for this study. Main findings were a growth retardation which was strongest in the second cohort (8–14 months of age) and related to a diet low in energy density, fat and protein. Haematological data revealed very low vitamin B12 concentrations with consequently low values of haematocrit and red blood cell count and higher values of mean corpuscular volume and mean corpuscular haemaglobin mass in the macrobiotic group. High folate concentrations also seemed to be a consequence of Vitamin B12 deficiency. Iron deficiency was found in 15% of the macrobiotic group vs. no infants in the control group. Nutritional recommendations acceptable within the macrobiotic philosophy are discussed.     

Assessment of Nutritional Status in Children With Cancer and Effectiveness of Oral Nutritional Supplements

Malnutrition is a common consequence of cancer in children, but the most effective methods of nutrition intervention are under debate. We aimed to evaluate the nutritional status of children diagnosed with cancer, and to investigate the effect of oral nutritional supplements on anthropometric measurements, biochemical parameters, and outcome. A randomized clinical study of 45 newly diagnosed cancer patients was performed. Anthropometric and biochemical data and related factors were assessed at 0, 3, and 6 months after diagnosis. On initial anthropometric assessment, prevalence of malnutrition by weight or height was found to be lower as compared with body mass index (BMI), or weight for height (WFH), or arm anthropometry. Twenty-six of the patients (55%) received oral nutritional supplement. During the second 3 months after diagnosis, there was a statistically significant decrease in number of the patients with WFH <90th percentile and BMI <5th percentile (P = .003 and P = .04, respectively). Infectious complications occurred more frequently in malnourished patients during first 3 months, and survival of children who were malnourished at the 6th month was significantly lower than that of well-nourished children (P = .003). On laboratory assessment, serum prealbumin levels of the all subjects were below normal ranges, but no relation was found for serum prealbumin or albumin levels in patients who were malnourished or not at diagnosis. Nutritional intervention is necessary to promote normal development and increase functional status as a child receives intensive treatment. Protein- and energy-dense oral nutritional supplements are effective for preventing weight loss in malnourished children.     

Appropriate Diets for the Rehabilitation of Malnourished Children in the Community Setting

The treatment of severe PEM includes–as appropriate in individual patients–fluid and electrolyte therapy, antimicrobial drugs, dietary management, and social and behavioral counseling. Except in the case of severe anorexia or life-threatening infections or dehydration, nutritional rehabilitation can be completed in the community setting, using centrally-processed or locally-prepared food mixtures and, when necessary, supplemental vitamins, minerals, and trace elements. Energy intakes should be maximized to promote rapid recovery, and protein: energy ratios must be increased according to the expected rate of weight gain. Nutrient: energy densities should meet or exceed the RDAs. Cultural and economic factors must be considered in the formulation of mixed diets, and organoleptic characteristics of the enhanced recipe(s) must be appropriate for young children. Selected issues concerning implementation of a community-based rehabilitation program are discussed.     

幽门螺杆菌感染对儿童铁营养的影响

目的探讨幽门螺杆菌(Hp)感染对儿童铁营养状况影响。方法对127例6~14岁儿童根据酶联免疫吸附法检测血清Hp抗体及Hp抗原(HpSA)测定,分为Hp感染组和非Hp感染组。Hp感染组和非Hp感染组儿童同时测定血红蛋白(Hb)、血清铁蛋白(SF)、血清铁(SI)、红细胞平均体积(MCV)。结果Hp感染组61例,非Hp感染66例,总感染率48.4%。其中6~9岁感染率38.5%,10~14岁感染率58.1%;两组感染率比较差异有显著性意义(χ2=4.89 P<0.05)。Hp感染组Hb、SF、SI、MCV分别为(111.20±3.84)g/L、(12.40±5.81)μg/L、(7.60±3.21)μmol/L、(75.38±3.78)fL,非Hp感染组Hb、SF、SI、MCV分别为(126.20±6.25)g/L(、24.20±6.92)μg/L(、11.30±4.93)μmol/L(、82.80±3.24)fL,感染组与非感染组比较有显著性差异(P均<0.01)。结论儿童Hp感染率随年龄增长而递增;Hp感染儿童与铁缺乏及缺铁性贫血有密切关系。     

Beneficial Effects of Palatable Guar and Guar Plus Fructose Diets in Diabetic Children

This randomized cross-over study evaluates the effects of extended, guar and guar + fructose diets on the metabolic balance of children with insulin-dependent diabetes mellitus (IDDM). We studied 22 children; mean age 12.2 years, mean duration of diabetes 4.4 years. The diet was supplemented for three weeks with guar in palatable form (5% of daily carbohydrate intake) and with guar + fructose (1 g of fructose/kg body weight, max 30 g/d) for another three weeks. A control group (8 children, mean age 12.3, duration of diabetes 4.3 years) followed the same experimental protocol without guar supplementation. The metabolic balance was assessed by glucosuria index (per cent of tests with less than 1% glucosuria from all urine tests) and measurements of red cell glycohaemoglobin A_1c (HbA_1c). Serum total and HDL-choiesterol, C-peptide, pancreatic and enteroglucagon were also measured. HbA_1c decreased during guar ( p <0.001) and guar + fructose diet ( p <0.001). The glucosuria index improved ( p <0.02) and the serum total cholesterol concentration decreased ( p <0.02) during the experimental guar diets. Guar in acceptable form and quantity in the diet appears to improve metabolic control of diabetic children.     

腹膜透析充分性对腹膜透析儿童营养状况的影响

目的前瞻性观察慢性腹膜透析(腹透)儿童的营养状况,探讨透析充分性对腹透儿童营养状况的影响。方法选择腹透时间超过3个月儿童,每3个月留取腹透液、尿液,抽血测BUN、Scr、清蛋白(Alb)、前清蛋白(PA)、转铁蛋白(Tf),计算尿素清除指数(Kt/V)、肌酐清除率(Ccr)、残余肾功能(RRF)、蛋白分解率(PCR),分析透析充分性与营养状况的关系。结果腹膜透析儿童10例(男6例,女4例)。平均年龄11.90岁(8.3～16.1岁);腹膜透析4～36个月,平均12.50个月。10例共进行32次检测,Scr为(830.31±156.48)μmol/L,BUN为(21.44±7.99)mmol/L,血浆Alb为(42.71±5.21)g/L,PA(419.68±118.0)mg/L,Tf(1.94±0.58)g/L,每周总Kt/V2.15±0.29,残余肾Kt/V0.57±0.45,每周总Ccr(49.13±15.25)L/1.73m2,残余肾Ccr(19.48±13.11)L/1.73m2,RRF(1.70±1.56)mL/min,PCR(1.17±0.18)g/(kg.d)。PCR与总Kt/V和总Ccr呈正相关(r=0.775,0.503Pa<0.01),与残肾Kt/V、残肾Ccr呈正相关(r=0.532,0.393Pa<0.05)。总Kt/V分别与Alb和PA呈正相关(r=0.496,0.541Pa<0.01),与Tf无关(r=0.226P>0.05)。RRF与总Ccr呈正相关(r=0.389P<0.05)。结论提高透析充分性有利于改善腹透儿童营养状况,制定合适透析处方以确保充分透析是预防腹透儿童营养不良的根本措施。     

Growth and Nutritional Status of Rural Preschool Children in Saudi Arabia

ABSTRACT. The attained growth was assessed in a cross-sectional study of Saudi children 0–71 months of age, from 3 different geographical areas of rural Saudi Arabia. individual data on weight, height, weight for height and head circumference were expressed in SD scores of international standards, and data on upper arm muscle and fat areas in percent of the reference median values. The mean (SEM) weight, height, weight for height and head circumference of the entire material were -1.7 (0.05), -1.6 (0.06), -0.9 (0.05) and -1.6 (0.06), respectively. The median upper arm muscle area was 79 percent, and fat area 57 percent of the reference medians. The differencies in mean weight, height and weight for height were trivial between the 3 geograhical areas. All mean values, except that of weight for height were clearly below the means of the reference in children 0–5 months. All mean values declined significantly, however, during the next 6 months. The growth deficits thus obtained remained unchanged or increased slightly up to 6 years. According to WHO criteria, 41 percent were classified as chronically, and 12 percent as acutely undernourished. The growth pattern of rural Saudi children was similar to that of children in most developing countries and distinctly different from that of privileged Saudi children, whose growth pattern resembled that of Western reference populations.     

Growth Impairment and Nutritional Status in Children with Chronic Kidney Disease

Objective

Malnutrition is closely linked to chronic kidney disease (CKD) in adult patients with poor outcome. But data on pediatric patients is inadequate. The aim of this study was to describe the prevalence of growth failure and malnutrition in pediatric CKD patients and explore the relationship of these parameters to each other and to other clinical parameters.

Methods

This study included 42 patients and 29 healthy children matched for age and gender. Patients were classified firstly in age group and secondly in therapy modalities. Nutritional evaluations were performed according to the Kidney Disease Outcomes Quality Initiative guidelines, and we performed adjustments using values from children with the same chronological age as reference.

Findings

In pubertal group, the mean height SDS was lower than in pre-pubertal period while it was higher than in early childhood (P=0.4 and P=0.03 respectively). In all groups, 45% of patients had malnutrition: 20 patients on predialysis, 22 patients with end stage renal disease (14 on hemodialysis, and 8 on peritoneal dialysis). The mean weight SDS was lower in end stage renal disease groups (P<0.001). The height SDS was lower in end stage renal disease groups (P<0.001).

Conclusion

Growth failure and malnutrition remain a significant clinical problem as age and therapy modalities are dependent in children with CKD.     

Growth and Nutritional Status of Less Privileged Urban Children in Saudi Arabia

ABSTRACT. The attained growth was assessed in a cross-sectional study of Saudi children 1–71 months of age, growing up in a less privileged area of Riyadh. Individual data on weight, height and weight for height were expressed in SD scores of the NCHS reference and those of triceps skinfold thickness in percent of the reference median. The mean (SD) weight, height and weight for height of the entire material were -0.9 (1.0), -0.8 (1.0), and -0.5 (0.9), respectively and the median tricpes skinfold was 90 percent of the reference median. When stratified by age groups the means were nearly identical with those of the reference populations in the 1–5 month age group. The mean weight and height were significantly below the reference means at 6–11 months, however. There was a similar decline in the mean weight for height and the median tricpes skinfold during the second year of life. The growth deficits of early life remained unchanged or increased slightly up to 6 years. According to WHO citeria, 14 percent of the children were classified as chronically and 3 percent as acutely undernourished. The faltering growth was different from that of privileged Saudi children whose growth pattern was found to resemble that of Western reference populations.     

INH and Streptomycin in Ethiopian Children with Tuberculosis and Different Nutritional Status

ABSTRACT. The plasma concentration of INH and streptomycin was followed in 45 Ethiopian children with tuberculosis. The children were grouped according to their nutritional status as normal, underweight, marasm and kwashiorkor. INH was well absorbed in all nutritional groups to give therapeutically active plasma levels. When terminal half life ( t 1/2) of INH was calculated for individual patients there were more children in all nutritional groups with t 1/2 ≥2 hours than <2 hours, indicating a slow acetylation of INH. Streptomycin was well absorbed in all nutritional groups and therapeutic levels were obtained with 20 mg/kg i.m. After 30mg/kg i.m. of streptomycin kwashiorkor children had an increased t 1/2 of streptomycin in-dictaing a decreased renal excretion of the drug in kwashiorkor. The clinical follow-up of the children indicated that serious tuberculosis could be successfully treated with INH and strpetomycin in the doses used.     

小安素干预治疗前后畏食症患儿营养状况比较

目的观察肠内营养剂小安素对畏食症患儿营养状况的临床疗效。方法30例畏食症患儿口服小安素2次/d,疗程2个月,服用前后测量身高、体质量、臂围、三头肌皮褶厚度、肩胛下皮褶厚度、腹部皮褶厚度,RBC、Hb,并记录患儿食欲和胃肠道反应等。结果小安素营养干预后患儿平均身高、体质量、皮褶厚度、上臂围均有明显增加,干预治疗前后比较差异有显著性(P<0.05);干预前后平均RBC与Hb无显著性差异(P均>0.05);所有患儿均能长期坚持服用,无明显胃肠道反应;22例(73.33%)患儿自觉食欲好转,进食量增加。结论小安素对畏食症患儿营养状况的改善有良好疗效。     

Enteral Nutritional Support by Percutaneous Endoscopic Gastrostomy in Children with Congenital Heart Disease

One of the major problems of children with severe congenital heart disease (CHD) is their poor nutritional status. Among other consequences, it influences the surgical outcome. Retrospectively we present our experience with percutaneous endoscopic gastrostomy (PEG) in 15 children with CHD. This technique allows enteral nutritional support without the disadvantages related to long-term nasogastric tube feeding. Major complications were absent, and minor complications were rare both at PEG insertion, which was performed under deep sedation, and during feeding via PEG tube. In 4 of the 8 children who were followed for at least 6 months the age-matched body weight increased more than one standard deviation. In 2 other patients it increased more than 0.5 standard deviations. In 7 children the tube was removed after 2.5 to 42 months since enteral support was no longer necessary. Apart from initial reservations the parental acceptance of PEG was good. We conclude that the PEG is a safe and reliable technique to support enteral nutrition in children with severe CHD.