Hyperferritinaemia following intrauterine transfusions for Rh isoimmunisation.

Intrauterine transfusion is one of the mainstays of treatment in isoimmunised pregnancies guided by the changes in middle cerebral artery Doppler of the fetus. The common postnatal complications associated with Rh isoimmunisation are high unconjugated bilirubin requiring blood exchange transfusions, cholestasis due to bile inspissation, thrombocytopenia and anaemia. Hyperferritinaemia is an uncommon adverse effect observed in Rh isoimmunised pregnancies. In this case report, we describe the clinical course of a Rh isoimmunised neonate with hyperferritinaemia and transfusion acquired cytomegalovirus disease which resolved. Iron chelation therapy was not necessary.

Effects of oral zinc supplementation on zinc status and catch-up growth during the first 2 years of life in children with non-organic failure to thrive born preterm and at term.

BACKGROUND: We aimed to analyze the effect of oral zinc supplementation on serum insulin-like growth factor-1 (IGF-1) levels and catch-up growth in infants with non-organic failure to thrive (NOFTT) who were born preterm as compared to those born at term. METHODS: Totally, 105 NOFTT infants aged 2 years or less were enrolled and divided into two groups according to gestational age at birth. Oral zinc sulfate was administered for 6 months to 49/66 children born at term, and 21/39 children born preterm. Serum zinc, IGF-1, weight, and height were measured at baseline and at 6 months. RESULTS: There were no differences in baseline serum zinc levels between the two groups. In preterm NOFTT infants, zinc supplementation significantly increased serum zinc levels compared to those in the non-supplementation group (Δ zinc 0-6 month 10.3 ± 26.4 µg/dL vs. -8.8 ± 23.7 µg/dL, p = 0.018), but it did not significantly change serum IGF-1 levels or weight- and height for age Z-scores. In NOFTT infants born at term who received zinc supplementation, serum zinc levels, IGF-1, weight for age Z-score, and height for age Z-score increased at 6 months (p = 0.001, p = 0.014, p = 0.049, and p = 0.029, respectively), but this increase was not significantly greater than in the non-supplementation group. Only the increase in serum zinc levels was significant after 6 months (Δ zinc 0-6 month 16.8 ± 32.0 µg/dL vs. -10.0 ± 22.6 µg/dL, p = 0.002). CONCLUSION: Zinc supplementation in NOFTT infants improves serum zinc status, regardless of gestational age at birth. Zinc supplementation in NOFTT infants born at term may improve serum IGF-1 levels and growth, but it does not in NOFTT infants born preterm. Overall nutritional support rather than supplementation of a single nutrient may be more effective for catch-up growth in NOFTT infants born preterm.

Vitamin Supplementation in the Elderly.

Vitamin supplementation is fairly common among the elderly. Supplements are often used to prevent disease and improve health. In the United States, the use of dietary supplements has continued to increase over the last 30 years, and more than half of adults report using one or more dietary supplements. Epidemiologic evidence suggests that a diet rich in fruits and vegetables does have a protective effect on health. However, clinical trials on the use of vitamin supplements for promotion of health and prevention of disease have failed to demonstrate the strong associations seen in observational studies.

[Eat a citrus fruit, stay healthy--a case report of scurvy].

Scurvy is a disease that results from a vitamin C deficient diet. Since vitamin C is available in many food products, and especially in citrus fruits, the disease is rare in developed countries. Clinical manifestations of scurvy include general weakness, cutaneous and gum bleeding, pain in the lower limbs and inability to stand and walk (pseudo paralysis). The diagnosis of scurvy requires a high level of clinical suspicion, typical radiographic features and low Levels of vitamin C in the plasma. We report a case of a 7-year-old patient with a medical history of hydrocephalus, failure to thrive and severe psychomotor retardation due to complications of prematurity. On admission she had gum bleeding, severe anemia, pain in the lower limbs and refused to stand and walk. According to her parents, her diet was restricted, without vegetables or fruit consumption. Our investigation ruled out coagulopathy, malignancy and infection. Serum vitamin C levels were low and radiographic findings were consistent with the diagnosis of scurvy. The patient improved rapidly after the initiation of vitamin C supplements. Despite being rare, scurvy should be considered in the differential diagnosis of bleeding and pain in the lower limbs, especially in a malnourished patient.

Bovine colostrum in the management of nonorganic failure to thrive: a randomized clinical trial.

OBJECTIVE: The objective of the study was to evaluate the clinical efficacy of oral bovine colostrum in the management of nonorganic failure to thrive (FTT). MATERIALS AND METHODS: In a randomized clinical trial, 120 children (1-10 years of age) of either sex with mild or moderate nonorganic FTT were divided into 2 groups. Both groups were matched with regard to age, sex, weight, and height. One group (control) received routine treatments for FTT and the other group (case), besides routine treatments, received supplementary bovine colostrum at the dose of 40 mg . kg . day for a 3-month period. Following the initial visit, subsequent visits were completed following 1, 2, and 3 months of supplementation. For quantitative measurements, Waterlow I (height for age) and Gomez (weight for age) indices were used. RESULTS: The mean value of Gomez index in the case group (81.72) was significantly higher than the control group (77.12) at the end of the third month of supplementation (P = 0.003). Such a difference was not reported based on Waterlow I index between the case and control groups (92.91 vs 91.71; P = 0.094). According to Gomez index 12 patients (20%) who received colostrum became healthy at the end of the third month, which was significantly higher than the control group (2 cases, 3.3%); P = 0.006. CONCLUSIONS: Bovine colostrum supplementation for a 3-month period is a useful method without any side effects, in addition to known medical and psychological treatments, to increase the weight of children with nonorganic FTT.

Simultaneous absence of dopamine D1 and D2 receptor-mediated signaling is lethal in mice.

Dopamine (DA) controls a wide variety of physiological functions in the central nervous system as well as in the neuroendocrine and gastrointestinal systems. DA signaling is mediated by five cloned receptors named D1-D5. Knockout mouse models for the five receptors have been generated, and, albeit impaired for some important DA-mediated functions, they are viable and can reproduce. D1 and D2 receptors are the most abundant and widely expressed DA receptors. Cooperative/synergistic effects mediated by these receptors have been suggested, in particular, in the control of motor behaviors. To analyze the extent of such interrelationship, we have generated double D1/D2 receptor mutants. Interestingly, in contrast to single knockouts, we found that concurrent ablation of the D1 and D2 receptors is lethal during the second or third week after birth. This dramatic phenotype is likely to be related to altered feeding behavior and dysfunction of the gastrointestinal system, especially because major anatomical changes were not identified in the brain. Similarly, in the absence of functional D1, heterozygous D2 mutants (D1r(-/-);D2r(+/-)) showed severe growth retardation and did not survive their postweaning period. The analysis of motor behavior in D1r/D2r compound mutants showed that loss of D2-mediated functions reduces motor abilities, whereas the effect of D1r ablation on locomotion strongly depends on the experimental paradigms used. These studies highlight the interrelationship between D1 and D2 receptor-mediated control of motor activity, food intake, and gastrointestinal functions, which has been elusive in the single-gene ablation studies.

Energy compensation in young children who fail to thrive.

BACKGROUND: Failure to thrive is generally attributed to undernutrition, but little is known about the appetite or eating behaviour of children with the condition. The hypothesis that young children who fail to thrive lack a normal sensitivity to internal hunger or satiation cues was tested in this study using an energy compensation procedure. METHOD: Twenty-seven children under assessment by a community-based service for failure to thrive, with weight gain in the lowest 5% for their age, were studied at one year of age with 26 controls of the same age and sex with normal weight gain, resident in the same local geographical area. Test meals were given in the child's own home on two separate days. The test meals were preceded by either a high energy (402 kJ) drink, or by a low energy (1 kJ) drink on a control day. The order was randomised, and the study conducted double blind, without the experimenter or the mother knowing which drink was which. Energy intake at the test meal was measured. RESULTS: There was no significant difference in the birth weight of the children in the two groups but by the time of the test the cases weighed significantly less than controls, with mean (SD) weight 9.06 (1.05) kg and 11.59 (1.59) kg respectively. In relation to the British Growth Reference for weight this is a difference of 2.2 SD. Mean (SD) energy intake at the meal on the control day was significantly lower in the case children than the controls (FTT 687.5 (334.3) kJ; controls 1065.9 (431.8) kJ; p < .001). After the high energy drink, controls reduced their energy intake at the meal by a mean (SD) -257.3 (383.3) kJ while the cases showed a slight average increase of +78.1 (365.9) kJ; t = 3.26, df 51, p < .001. Per kJ of the pre-load, the average change was -1.18 kJ in controls and +0.80 kJ in cases. CONCLUSIONS: The controls compensated as expected for their high energy load at the subsequent meal, but the case children did not, showing that they lack the normal responses to internal hunger/satiation cues. High energy snacks may improve the nutritional status of children who fail to thrive.

Zinc supplementation increases the level of serum insulin-like growth factor-I but does not promote growth in infants with nonorganic failure to thrive.

We investigated in a randomized double-blind placebo-controlled study the effects of zinc supplementation (2 mg/kg/day) for 12 weeks on growth, serum insulin-like growth factor-I (IGF-I) and insulin-like factor binding protein-3 (IGFBP-3) on 3- to 9-month-old infants with nonorganic failure to thrive (NOFTT). 25 infants completed the study, 14 received zinc supplementation (group A), and 11 received placebo (group B). The control group for baseline measurements was composed of 10 age-matched normal growing infants. There were no significant changes in weight for age, length for age, or weight for length during the entire study period in either group A or B. Serum IGF-I levels at baseline were similar in all the groups. After 12 weeks of therapy, serum IFG-I levels increased significantly only in the zinc-supplemented group, from 40.3 +/- 7 ng/ml at baseline to 65 +/- 8 ng/ml (p < 0.05). There was a marked difference in serum IGF-I levels between the zinc-supplemented group and the placebo group after 12 weeks: 65 +/- 8 vs. 49.4 +/- 5 ng/ml (p = 0.058, 95% CI of difference 9.88-21.31). No change was demonstrated in serum IGFBP-3 levels in either study group. We conclude that although zinc supplementation increased serum IGF-I levels, it did not improve the growth parameters of infants with NOFTT.

Neuropeptide Y mRNA and serotonin innervation in the arcuate nucleus of anorexia mutant mice.

The anorexia (anx) mutation causes reduced food intake in preweanling mice, resulting in death from starvation within 3-4 weeks. In wild-type rodents, starvation induces increased neuropeptide Y (NPY) mRNA levels in the arcuate nucleus that promotes compensatory hyperphagia. Despite severely decreased body weight and food intake at 3-weeks age, anx/anx mice do not show elevated NPY mRNA levels in the hypothalamic arcuate nucleus compared to wild-type/heterozygous littermates. The NPY mRNA levels can be upregulated in normal mice at this chronological age, because 24-h food deprivation increased arcuate NPY mRNA in wild-type littermates. The unresponsiveness of NPY expression in the arcuate of anx/anx mice was paralleled by serotonergic hyperinnervation of the arcuate nucleus, comparable to the serotonergic hyperinnervation previously reported in the rest of the anx/anx brain. This result is consistent with the hypothesis that wasting disorders are accompanied by disregulation of NPY mRNA expression in the arcuate nucleus, and suggests that reduced food intake, the primary behavioral phenotype of the anx/anx mouse, may be the result of altered hypothalamic mechanisms that normally regulate feeding.

Pseudohypoaldosteronism: report of a case presenting as failure to thrive.

We report a 2 month-old infant referred for failure to thrive. At birth, weight was 3820 g and length 52 cm. After physiologic weight loss, the patient showed no further weight gain for the next two months. On admittance (age 2 mo), weight was 3340 g and length 53 cm; the infant had severe dystrophy, generalized hypotonia and dehydration; blood chemistry showed hyponatremia, hyperkalemia and hypochloremia. A salt losing syndrome of adrenal origin was hypothesized. However, rehydration and hydrocortisone administration failed to correct hyponatremia and hyperkalemia. Endocrine assessment showed high levels of aldosterone and plasma renin activity, suggesting pseudohypoaldosteronism. Oral sodium chloride supplementation normalized electrolyte balance and the patient showed progressive weight gain and catch-up growth, confirming the diagnosis.

[Chronic metabolic alkalosis in a newborn infant caused by congenital chloride diarrhea]. / Chronische, metabolische Alkalose bei einem Neugeborenen durch eine kongenitale Chloriddiarrhö.

The case of a female preterm infant (gestational age 36 weeks) is described, who presented with abdominal distension, diarrhoea, dehydration and metabolic alkalosis at the fifth day of life. After different diagnostic tests had been performed, congenital chloride diarrhoea was suspected and chloride supplementation was started. However, this diagnosis could not be confirmed, until the measurement of electrolytes in faeces had been improved. Then, we found the typically elevated fecal chloride concentration (130-153 mmol/l) which exceeded the sum of the fecal concentration of sodium (64-90 mmol/l) and potassium (28-35 mmol/l). The chloride supplementation was increased to 6 mmol/kg/d NaCl and 2 mmol/kg/d KCl. The most recent examination at the age of 1 year revealed the girl to be in good clinical condition, with normal growth and psychomotor-development and with no evidence of renal impairment.

Current thinking on the role of surgery in gastroesophageal reflux.

Gastroesophageal reflux is common in infants and children. In most cases it causes little more than inconvenience and remits spontaneously with time and maturation. A small and select group of refluxing children, however, will develop complications of reflux severe enough to justify operative control when medical treatment fails. Recurrent pulmonary infections, obstructive apnea, nutritional wasting, and progressive inflammatory injury to the esophagus all qualify as surgical indications, provided a reasonable cause-effect relationship with reflux can be established. The procedure of choice depends very much upon the skill and experience of the surgeon. The complete fundoplication seems to offer more complete control of reflux, but it has the potential for more frequent and more complicated side-effects.