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Biomedical subjects

B Paes

Publications and source records attributed to B Paes.

At least 19 recordsLinked to original sources

Carnitine supplementation for preterm infants with recurrent apnea.

BACKGROUND: Apnea of prematurity is a common problem in preterm infants in the neonatal intensive care setting (NICU), often delaying their discharge home or transfer to a step down unit. Premature infants are at increased risk of carnitine deficiency. Carnitine supplementation has been used for both prevention and treatment of apnea. OBJECTIVES: To determine whether treatment with carnitine will reduce the frequency of apnea, the duration of ventilation and the duration of hospital stay in preterm infants with recurrent apnea. SEARCH STRATEGY: Computerised searches were carried out by two reviewers independently. Searches were made of MEDLINE (1966 to May 2004), EMBASE (1980 to May 2004), CINAHL (1982-2004 June 2004,1st week), the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 2, 2004), abstracts of annual meetings of the Society for Pediatric Research (1995-2004), and contacts were made with the subject experts. SELECTION CRITERIA: Only randomized or quasi-randomized treatment trials of preterm infants with a diagnosis of recurrent apnea of prematurity were considered. Trials were included if they involved treatment with carnitine compared to placebo or no treatment, and measured at least one of the following outcomes: failure of resolution of apneas, the duration of ventilation and the duration of hospital stay. DATA COLLECTION AND ANALYSIS: Two reviewers evaluated the papers for inclusion criteria and quality. Corresponding authors were contacted for further information where needed. MAIN RESULTS: No eligible trials were identified. REVIEWERS' CONCLUSIONS: Despite the plausible rationale for the treatment of apnea of prematurity with carnitine, there are insufficient data to support its use for this indication. Further studies are needed to determine the role of this treatment in clinical practice.

Apnea↗

Orbital lymphangioma with non-contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach-Merritt syndrome) and intracerebral hemorrhage.

UNLABELLED: This study describes the first reported case in a preterm infant of an orbital lymphangioma with non-contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach-Merritt syndrome) and intracerebral hemorrhage. CONCLUSION: Neonates presenting with orbital lymphangiomas should undergo radiological investigations of the lesion and a detailed cerebral evaluation for associated arteriovenous developmental anomalies.

Abnormalities, Multiple↗

Spontaneous rupture of the liver presenting as scrotal bruising.

We describe a case of spontaneous rupture of the liver in a neonate, presenting with features consistent with testicular torsion. Ruptured liver has been well described in neonates following birth trauma, coagulation defects, and Group B Streptococcal infection. The etiology in our case remains undefined.

Contusions↗

Wolf-Hirschorn syndrome resulting from partial monosomy 4p/trisomy 9p.

An infant girl was referred for a genetic consultation because of facial appearance suggestive of Wolf-Hirschorn syndrome (WHS), growth retardation and generalized hypotonia. She had an unbalanced karyotype 46,XX,der(4)t(4;9)(p15.2;p22)mat resulting in the deletion of the critical region for WHS and duplication of the critical region for the 9p duplication syndrome. The mother and the grandmother of proposita were the carriers of an apparently balanced translocation 46,XX,t(4;9)(p15.2;p22). The infant's phenotype was characteristic of WHS syndrome rather than that of duplication 9p phenotype. This is probably the first description of WHS phenotype resulting from a familial 4;9 translocation.

Abnormalities, Multiple↗

A randomized trial comparing peripherally inserted central venous catheters and peripheral intravenous catheters in infants with very low birth weight.

BACKGROUND/PURPOSE: To determine whether percutaneously inserted central venous catheters (PICC) and peripheral intravenous catheters (PIV) in infants with very low birth weight (VLBW) differ with respect to (1) incidence of sepsis, (2) number of insertion attempts and catheters required for total intravenous therapy, (3) courses of antibiotics, and (4) total duration of intravenous (IV) use. METHODS: A randomized comparative trial was conducted involving 63 VLBW infants (<1,251 g) who required IV therapy. Infants were assigned randomly at 1 week of age to either a PIV or a PICC catheter and followed up prospectively until an IV was no longer required or the infant was transferred out of the neonatal intensive care unit. RESULTS: Data were analyzed on an intention-to-treat basis. There was no difference in the incidence of sepsis (P = .64), number of courses of antibiotics (P = .16), or total duration of IV use (P= .34) between the 2 groups. The number of insertion attempts required for total IV therapy was significantly lower in the PICC group than in the PIV group (P = .008). There also was a significantly lower number of total catheters utilized in the PICC group (P = .002). When data were controlled for birth weight strata the results were similar. CONCLUSION: PICC lines reduced the number of painful IV procedures in VLBW infants without additional morbidity.

Catheterization, Central Venous↗

Congenital varicella syndrome: a rare case of central nervous system involvement without dermatological features.

An unusual case of congenital varicella syndrome with significant central nervous system involvement, but without dermatological features at birth is described. The mother contracted chicken pox at 15 weeks' gestation. Congenital varicella syndrome involves multiple systems, but rarely without skin lesions identifiable at birth. Although varicella infection in pregnant women is an uncommon complication, the fetal embryopathy that may result can be devastating. Antenatal diagnosis of fetal embryopathy during the first 20 weeks of pregnancy should be established by amniocentesis or cordocentesis when a mother presents in the first trimester with chicken pox, and appropriate risk counselling provided.

Central Nervous System Viral Diseases↗

Recurrent familial neonatal deaths: hereditary surfactant protein B deficiency.

Hereditary surfactant protein B (SP-B) deficiency is an uncommon autosomal recessive lung disorder that causes hypoxemic respiratory failure in mature, morphologically normal infants. Recognition and diagnosis of this condition is of paramount importance, as it has significant implications for future pregnancies with a recurrence risk of 25%. In a family with three neonatal deaths over 20 years, SP-B deficiency was diagnosed following the death of the fourth affected infant. Previous deaths were mistakenly attributed to hyaline membrane disease (HMD), congenital Mycoplasma hominis infection, and pulmonary hypertension, however, following the diagnosis in the proposita, SP-B deficiency was also confirmed in her deceased siblings by immunohistochemical staining of autopsy specimens. This case highlights the presentation, postnatal course, diagnosis, and therapeutic options of SP-B deficiency in addition to the mode of inheritance and the possibility of antenatal diagnosis. Genetic consultation is imperative in the investigations of recurrent neonatal deaths, especially in cases of remote events. The recent enormous advances in human genetics have shown that many conditions previously ascribed to environmental agents have a genetic basis.

Fatal Outcome↗

Influence of exogenous factor VIIa on thrombin generation in cord plasma of full-term and pre-term newborns.

Factor (F) VIIa has been used to treat adults and children with a variety of bleeding disorders. The results from these studies cannot be extrapolated to newborns because their hemostatic system differs significantly from adults, which may influence the effects of FVIIa on thrombin (IIa) generation. We compared the effects of FVIIa concentrates on IIa generation in plasmas from adults, full-term newborns and pre-term newborns. Defibrinated plasma (using arvin) from adults, or umbilical cords from full-term or pre-term deliveries was supplemented with FVIIa (Novo Nordisk, Bagsvaerd, Denmark), mixed with dilute thromboplastin reagent, and the resultant reaction mixture subsampled periodically into ethylenediamine tetraacetic acid, followed by measurement of total IIa activity (S-2238). Thrombin-alpha2 macroglobulin complexes, determined as residual activity after neutralization with heparin and antithrombin, were subtracted from total IIa to give free IIa. Prothrombin (FII) and inhibitor complexes were measured by enzyme-linked immunosorbent assays. Addition of FVIIa caused a reduction in the lag phase for the appearance of free IIa and consumption of FII, which was more pronounced in newborn plasma. There was no increase in peak IIa levels regardless of the amount of FVIIa added. Final inhibitor complex concentrations were increased in plasmas from adults compared with newborns, likely reflecting higher plasma concentrations of FII in adults. Generation of IIa was more rapid in pre-term plasma compared with that in adult and full-term cord plasmas due to increased endogenous tissue factor (TF). In summary, FVIIa enhanced IIa generation in plasma from different age groups, with the effect being more pronounced in plasma from pre-term newborns, possibly due to increased levels of plasma TF.

Adult↗

De novo deletion 12q: report of a patient with 12q24.31q24.33 deletion.

We report on a patient with de novo interstitial deletion of the long arm of chromosome 12: 46,XY,del(12)(q24.31q24.33). To our knowledge this is the first patient with this chromosomal abnormality reported. He was born with minor anomalies, ambiguous genitalia, tracheomalacia, and he was developmentally delayed at age 9 months. The phenotype associated with this deletion may be characteristic. However, because of the absence of reported cases of other patients with loss of this chromosomal region, we cannot delineate the specific phenotype further. Ambiguous genitalia or hypogonadism has been reported in other patients with chromosomal rearrangements involving 12q24.

Chromosome Banding↗

Bone metabolism and circulating IGF-I and IGFBPs in dexamethasone-treated preterm infants.

AIM: To characterize the ontogeny of circulating IGF-I, the IGF binding proteins (IGFBPs) and biochemical markers of bone turnover in dexamethasone (DEX)-treated preterm infants with chronic lung disease. METHODS: Plasma and urine samples from 17 infants were obtained prior to DEX, after 9-12 days of DEX and 10 days after the completion of DEX to assess plasma IGF-I, IGFBPs, osteocalcin and urinary N-telopeptide. Nutrient intakes and growth were monitored from birth until term corrected age at which time body composition was evaluated by dual energy X-ray absorptiometry. RESULTS: Although nutrient intakes did not differ during or after DEX, weight gain (115 vs. 174 g/week) and length gain (0.7 vs. 1.0 cm/week) were higher after DEX treatment. Plasma IGF-I, IGFBP-3 and osteocalcin increased over time. N-telopeptide was the only biochemical parameter which appeared to be suppressed during DEX (1342 nM bone collagen equivalents/mM creatinine vs. 2486 (pre-DEX) and 2292 (post-DEX)). At term corrected age, bone mineral content was lower in dexamethasone-treated infants compared to preterm and term reference infants. CONCLUSION: Changes in circulating IGFBP-2 and IGFBP-3 paralleled the changes reported in non-steroid-treated infants; however, it remains uncertain whether the natural rise in IGF-I was suppressed by DEX treatment. Assessment of these circulating components provided limited insight into the mechanisms by which DEX alters growth and bone turnover.

Absorptiometry, Photon↗

Cisapride: the problem of the heart.

Cisapride has been used in the neonatal population as a first-line gastrointestinal prokinetic agent for managing feeding intolerance secondary to decreased gastrointestinal motility. Cisapride acts specifically at many levels of the gastrointestinal tract and has no central nervous system side effects. Recently, prolonged QT interval has been reported secondary to high-dose cisapride, and concerns regarding its use in premature infants have been raised. In response, Janssen-Ortho Inc. and Janssen Pharmaceutica, the manufacturers of cisapride in Canada and the U.S., have released safety information prohibiting the use of cisapride in premature infants. In light of the present evidence, it is imperative that NICUs that continue to prescribe cisapride for the management of feeding intolerance exercise vigilance in cardiac monitoring and recognize the importance of drug interactions that lead to elevated cisapride levels.

Canada↗

Growth and body composition of preterm infants: influence of nutrient fortification of mother's milk in hospital and breastfeeding post-hospital discharge.

We examined the influence of multi-nutrient fortification of mother's milk (MM + MNF) compared to supplementation with calcium and phosphorus (MM + CaGP) alone in hospital (in a randomized trial), and of breastfeeding (post-MM) compared to formula feeding (post-FF ) after hospital discharge with a descriptive analysis of growth and body composition to 1 y corrected age in preterm infants. Anthropometry, nutrient intakes and whole body bone mineral content, lean and fat mass were determined at four time points in the first year after term corrected age. Body composition was determined with dual energy X-ray absorptiometry. MM + MNF compared to MM + CaGP for preterm infants in the early neonatal period did not appear to influence growth or body composition in the first year. Growth in post-MM and post-FF groups was within the normal range of growth references derived from term infants fed mother's milk. Post-MM infants had lower whole body bone mineral content (132.3 +/- 10.4 g) at 6 months corrected age when compared to post-FF infants (159.4 +/- 14.1 g) and greater percent fat mass to 12 months corrected age. These differences may result from the lower calcium, phosphorus and protein intakes in post-MM compared to post-FF infants. Our findings demonstrate that dietary practices after hospital discharge likely have a greater impact on body composition in prematurely born infants than dietary practices in hospital. Whether the observed differences in body composition between breastfed and formula-fed preterm infants have any long-term consequences requires further investigation.

Body Composition↗

Moderate nutrient supplementation of mother's milk for preterm infants supports adequate bone mass and short-term growth: a randomized, controlled trial.

Our objectives were 1) to determine whether moderate nutrient supplementation of mother's milk (MM) for preterm infants, in the form of a new multinutrient fortifier (MNF), would improve short-term growth and bone mineral content (BMC) when compared with supplementation with calcium and phosphorus alone; and 2) to investigate whether moderate calcium and phosphorus intakes, in the form of calcium glycerophosphate (CaGP), resulted in a BMC similar to that of term corrected infants. Twenty-five preterm infants fed MM were randomly assigned to receive either MM+MNF or MM+CaGP. A third group of infants fed preterm formula (PTF) served as a comparison group. Whole-body BMC and lean and fat mass were determined by dual-energy X-ray absorptiometry (DXA) at full-term age. Nitrogen retention and calcium, phosphorus, and zinc intakes were determined by using mass balance techniques. Nitrogen retention was significantly lower in the MM+CaGP group than in the PTF group as were both weight and length gain (weight gain: 16.6 +/- 1.6, 14.2 +/- 2.0, and 16.1 +/- 2.9 g x kg(-1) x d(-1); length gain: 1.1 +/- 0.2, 0.9 +/- 0.2, and 1.1 +/- 0.3 cm/wk for the MM+MNF, MM+CaGP, and PTF groups, respectively). Biochemical indexes of mineral status and bone turnover were normal. Conservative amounts of calcium and phosphorus, as CaGP, resulted in adequate BMC. Moderate amounts of protein, calcium, and phosphorus plus trace elements added to MM in the form of an MNF resulted in improved linear growth but did not provide any advantages to BMC when compared with supplementation with calcium and phosphorus alone.

Anthropometry↗

Longitudinal assessment of growth and bone mineral accretion in prematurely born infants treated for chronic lung disease with dexamethasone.

The objective of this study in premature infants was to assess the relationship between dexamethasone, growth and bone mineral accretion. Nine appropriate size for gestational age premature infants treated for chronic lung disease with tapering doses of dexamethasone (0.5-0.1 mg/kg/day over 37 +/- 7 days) were individually matched to a comparison infant by sex, gestational age, birth-weight, and type of feed. Infant growth and bone mineral accretion were measured at equivalent gestational ages from recruitment until 6 months corrected age. During hospitalization, mean rate of weight, length and head circumference growth and bone mineral accretion in the distal radius were significantly lower in the dexamethasone-treated infants in spite of similar nutrient intakes. Dexamethasone infants had significantly lower plasma phosphorus, and urinary calcium, pyridinoline and N-telopeptide excretion. Dexamethasone affected absolute length, but not weight, throughout the study. No significant differences were observed in body composition or absolute radial and whole body bone mineral content. The results indicate that dexamethasone therapy compromises growth and bone mineral accretion in small premature infants. 'Catch-up' linear growth was not evident at 6 months of age and reflects the importance of early nutrition interventions.

Amino Acids↗

Does vitamin C cause hemolysis in premature newborn infants? Results of a multicenter double-blind, randomized, controlled trial.

OBJECTIVE: We tested the hypothesis that vitamin C supplementation of premature neonates is associated with hemolysis. STUDY DESIGN: A double-blind, randomized, controlled trial of vitamin C supplementation (50 mg/day) was undertaken in premature neonates (birth weight, 1000 to 1500 gm). Infants were randomly assigned to receive vitamin C (Ce-Vi-Sol) (n = 32) or placebo (n = 24) for 14 days. Twenty-three subjects per group were required to detect a difference of 1 SD in corrected carboxyhemoglobin values (alpha = 0.05, beta = 0.10). RESULTS: Day 14 vitamin C levels were lower in control subjects than in supplemented neonates (62 +/- 24 vs 125 +/- 62 micromol/L, p = 0.005). There was no difference in corrected blood carboxyhemoglogin concentrations (0.72 +/- 0.44 vs 0.72 +/- 0.23%; p = 0.95), other parameters of hemolysis, weight gain, blood sampled, presumed septic episodes, necrotizing enterocolitis, feeding intolerance, or transfusion. On day 14, bilirubin values were higher in control subjects than in the supplemented group (77 +/- 37 vs 55 +/- 33 micromol/L; p = 0.04). When a distant outlier in the nonsupplemented group was excluded (163 micromol/L), statistical significance was lost (73 +/- 32 vs 55 +/- 33 micromol/L; p = 0.09). CONCLUSION: Oral supplementation of premature infants with vitamin C is not associated with evidence of increased erythrocyte destruction, hyperbilirubinemia, or other morbidity.

Anemia, Hemolytic↗

Cisapride: a review of the evidence supporting its use in premature infants with feeding intolerance.

A systematic computerized search of all databases was performed to review the scientific evidence in support of the efficacy of cisapride in reducing feeding intolerance in premature infants. Reference lists from these articles were used to identify relevant scientific literature to address important aspects of the use of cisapride. Three open prospective, uncontrolled studies were found. All studies reported improved clinical outcomes as evidenced by decreased gastric residuals, decreased incidence of vomiting, increased feeding volume, decrease in all reflux parameters measured, and increased weight gain. These observational studies reflect the current state of knowledge and have important research and clinical implications because of the profound effects of feeding intolerance on infant growth and development and on length of stay within NICUs.

Cisapride↗