Improved Growth Velocity Research Articles

SAT-096 Response to RHGH Therapy in Children with Isolated Short Stature with or Without an Identified Genetic Cause

Introduction: Children with isolated (former known as idiopathic) short stature (ISS) have been treated with rhGH with a variable response. Objectives: To evaluate the short-term response to rhGH therapy in children with ISS with or without a genetic diagnosis. Methods: We analyzed retrospectively the growth rate and height SDS change in the first year of rhGH treatment according to the presence or absence of defects in genes that regulate growth plate. The decision to start rhGH treatment was based on clinical features and the genetic results were obtained during the follow-up. Patients were enrolled in several previous genetic studies using gene candidate approach or multigene sequencing analysis. Results: A total of 51 prepubertal children (36 boys) with ISS were treated with rhGH. Thirteen of these children start puberty during the treatment and three of them were concomitant treated with GnRH analog. Basal characteristics of these children were 7.7 ± 3.2 years of age, height SDS -2.5 ± 0.8; sitting height/height (SH/H) SDS 1.2 ± 1.4; BMI SDS 0 ± 1.0 and mild delay of bone age (-1.6 ± 1.3 y). The mean target height SDS was -1.2 ± 0.9y, 18 (35%) of these children have at least one parent with height SDS < -2 and 3 (6%) both parents are short. Consanguinity was present in 3 (6%) cases. Among this cohort, fifteen children had pathogenic or likely pathogenic allele variants in genes that regulate growth plate: IHH (n = 4), SHOX (n = 9) and NPR2 (n = 2). Seven (47%) of these variants were inherited from a short stature parent. Children with or without an identified genetic cause have similar age and height SDS at the start of the treatment. A higher BMI and SH/H SDS were observed in children with genetic defects than in those without (BMI SDS 0.5 ± 1.1 vs. -0.15 ± 0.9, p = 0.02; SH/H SDS 2.0 ± 1.4 vs. 0.9 ± 1.3, p = 0.006). Additionally, children with genetic defects had a less marked bone age delay (-1.0 ± 1.3 vs. -1.9 ± 1.2; p = 0.02). Both groups were treated with similar rhGH dose (50 μg/kg/day). Patients with and without an identified genetic cause had similar improvement in growth velocity during the first year of therapy: 4.8 ± 1.6 to 8.9 ± 1.7 cm/y for patients with molecular diagnosis vs. 4.6 ± 1.2 to 8.5 ± 2.3 cm/y for those without. This resulted in similar height SDS change during this period for both groups (0.6 ± 0.3 vs. 0.6 ± 0.5 SDS for children with or without a genetic cause, respectively). Age at the start of treatment was the main variable that explains growth response variability during this first year (r2 = 0.17, p = 0.009). Conclusion: The presence or absence of an identified genetic cause, involving genes that regulate growth plate, did not significantly influence the short-term growth response to rhGH therapy of children with ISS. Long-term follow-up is still needed to assess the final height of these children and possibly to assess whether there is a different growth rate related to each known affected gene.

SAT-101 The Booster Effect of Aromatase Inhibitor to Overcome Waning Effect of Recombinant Human Growth Hormone

Aromatase inhibitor (AI) is a drug that blocks the conversion of androgens to estrogens, originally approved by FDA as a treatment of breast cancer in postmenopausal women. In several studies, it has been proposed that AI appears effective to improve the final adult height especially combined with rhGH therapy, by delaying growth plate closure. Patients treated with rhGH showed highest growth rate during the first year of treatment, with an average increase of 8–10 cm per year (often called “catch-up” growth), however, followed by a progressive decrease in growth rate over the next several years, which is called “waning effect” of rhGH. In this report, we would like to introduce 2 cases of short pubertal boys showing booster effect of AI when co-treated with rhGH, to overcome waning effect of rhGH and improve the final adult height. Case 1. A boy at the age of 8 years and 1 month visited out clinic with short stature. His height was 118cm (4%), weight was 19.2kg (0%). His MPH was calculated as 173cm. Bone age was 6.9 years by the TW3 method. Initial laboratory finding showed no other abnormal findings including normal IGF-1 levels, so we diagnosed the patient as idiopathic short stature (ISS), and started rhGH treatment. During the rhGH treatment of 4 years and 9 months, the patient obtained additional 30.7cm growth to 148.7cm (23%). However, the patient showed decreased growth velocity of 2.2 cm in the last 6 months, which was thought to be “waning effect” of rhGH. The patient and his parents wanted to improve his height using AI, and after taking agreement about the drug, we added AI. During the combined treatment of 1 year and 10 months, his height was improved to 164.9cm (32%) with a gain of 16.2cm, and no adverse reaction was observed. Case 2. A 8 years and 2 months-old boy visited out clinic with short stature. His height was 116.3cm (0%) and weight was 22.6kg (14%). There have been no unusual findings in past history and family history. His MPH was calculated as 165.9cm. Bone age was 8.2 years by the TW3 method. He was also diagnosed as ISS, and started rhGH treatment. During 5 years and 1 month of rhGH treatment, the patient obtained additional 33.3cm growth to 149.6cm (17%). However, the patient showed progressive decreased growth velocity of 3.3 cm in the last 6 months, so we added AI treatment, and after the combined treatment of 1 year and 5 months, his height was 161.6cm (23%). After that, his parents wanted to quit AI, so we maintained only rhGH treatment for 7 months, and his final height was checked as 167cm. In our cases, we suggest that the additional use of AI to patients with progressive decreased growth rate during previous years of GH treatment can improve growth velocity again, and increase the final adult height with successful bone age suppression, which has not been reported before. Therefore, AI could be expected as effective booster drug to overcome waning effect of rhGH.

SAT-108 Growth Hormone Deficiency in a Patient with Ectodermal Dysplasia

Background information:Ectodermal dysplasia (ED) is a rare heterogeneous group of genetic disorders of ectodermal derived tissues, characterized by abnormalities in skin, teeth, hair and eccrine glands. Growth failure in these children varies depending on the genetic mutation and has not been well characterized. This clinical case report presents a 11-year-old male with a heterozygous mutation in WNT 10 A, a variant of the hypohydrotic ED gene, who was found to have growth hormone (GH) deficiency and treated with GH.Case report:He was born at 35 weeks gestation by C-section with a birth weight of 5 lbs. 12 oz. to a mother who had invitro fertilization with donor eggs from the maternal aunt with ocular myasthenia gravis and sperm from the father. Pregnancy was complicated by twin gestation and polyhydraminos. He had transient myasthenia gravis and treated with pyridostigmine for 3 months for feeding problems and swallowing difficulty. He also had arthrogryposis of the distal upper extremities attributed to placental transfer of the maternal aunt’s myasthenia gravis antibodies.He was referred to the endocrine clinic for evaluation of his growth failure around the age of 8 years. His growth chart indicated that he grew along the 5thpercentile until age 5 year with a gradual decline to the 3rd percentile by age 7 year and close to 2nd percentile by age 8 year. His BMI was at 7th percentile. Mid parental height was 5’9”. There was no history of delayed adolescence in the family. His twin sister had very mild form of arthrogryposis with dental delay but steady linear growth. He also had decreased exercise tolerance. His body tended to become hot during sports activities and had to wrap his face and neck with cold soaked towels. His other problems included delayed dental development with conical incisor, thin nail, missing teeth and hearing defects that raised suspicion for ectodermal dysplasia. Genetic testing at the age of 4 years had demonstrated a heterozygous mutation in the WNT 10A gene, an important gene for tooth development. Physical examination revealed a mild facial dysmorphism with conical incisor, missing teeth and high arched palate. He had contracture of the proximal inter phalangeal joints of the hands. Investigations revealed a normal thyroid function test, IGF-1 and IGFBP-3 level, CBC, sedimentation rate, chemistry panel and celiac titer. The bone age was concordant with his chronological age of 8 years. A GH stimulation study demonstrated a peak GH level of 4.94 ng/ml. An MRI of the brain revealed a normal pituitary gland. He was started on GH therapy with 0.3 mg/kg/week at age 9 year. His height improved from 2nd percentile at age 9 year to 20th percentile by age 11 year on growth hormone therapy. His exercise capacity and stamina also improved.Conclusion:Growth failure and GH axis should be evaluated in children with ED. GH therapy improves growth velocity and exercise capacity in patients with ED.

Effect of Vitamin D level on growth velocity of RHGH treated children

Abstract Objective Recent studies have shown a relationship between vitamin D status, growth hormone and insulin-like growth factor 1 axis. Aims of the study are: 1- To evaluate the vitamin-D blood level in children on substitution treatment with rhGH. 2. To find out correlation between the vitamin D level and growth velocity with rhGH therapy, height, height SDS, height/height SDS after 1 year of treatment Methods The study included 173 prepubertal children, with short stature on treatment with rhGH (90 girls and 81 boys), aged between 2 and 10.3 years. To determine vitamin D status we measured serum 1,25-OH vitamin D by taking a single blood sample in the morning, in a fasting stage. Growth velocity over the year of observation, starting height, height SDS, height at the end of the year and its SDS all were calculated and correlated to vitamin D level. Results In the present study: 56 patients had vitamin D deficiency (&amp;lt;10 ng/ml); 54 had vitamin D insufficiency (10–20 ng/ml) and 63 had vitamin D sufficiency (&amp;gt;20 ng/ml). No statistical significance correlation was found between vitamin D level and height, height SDS, follow up height, and follow up height SDS (P.09, .066, .455, .927 respectively) Significant positive correlation was found between vitamin D and growth velocity (r .445 and P.0001). Conclusions Optimizing vitamin D level in patients with short stature under growth hormone therapy may improve growth velocity and response to growth hormone.

Recombinant GH treatment in a case of Costello syndrome with a 5-year follow-up

.Costello syndrome (CS) is a rare member of the group of neuro-cardio-facio-cutaneous diseases known as RASopathies. CS involves characteristic dysmorphic craniofacial features, cardiac defects, and increased cancer susceptibility, depending on the heterozygous activating germline mutations in HRAS. Polyhydramnios and high birth weight are the most common presentations in the perinatal and neonatal periods; while poor postnatal growth, short stature, and failure to thrive are significant issues in infancy. Possible mechanisms of short stature in CS include GH deficiency and feeding difficulties. Only a few reported cases of CS with GH deficiency exist in literature. Here, we describe the 5-yr follow-up of a CS patient with complete GH deficiency treated with recombinant human GH (rhGH) from the age of four years. No significant adverse events regarding progression of hypertrophic cardiomyopathy and tumor development were observed. She has been responsive to treatment with improved growth velocity and height standard deviation scores. She is still under continuous monitoring for concerns on the possible development of cardiac events and malignancies. This case indicated that rhGH therapy is effective for improving the height and growth velocity of CS patients with GH deficiency under close cardiac and oncological monitoring.

Acquired neuro-secretory defect in growth hormone secretion due to Imatinib mesylate and the efficacy of growth hormone therapy in children with chronic myeloid leukemia

Imatinib results in growth retardation in children with chronic myeloid leukemia (CML). The study was planned to assess the GHRH-GH-IGF1 axis in children with CML, receiving Imatinib and to evaluate the efficacy of human growth hormone (hGH) therapy. Twenty children with CML, receiving Imatinib for a period exceeding 6 months, with resultant growth retardation were included. The GHRH-GH-IGF1 axis was assessed using growth hormone stimulation tests. IGF-1 generation test was performed for the evaluation of GH insensitivity. The mean age at inclusion was 15.2 years. The mean duration of treatment with Imatinib was 5.7 years. The mean decrease in height SDS since the start of Imatinib was −0.95 (p = 0.008). IGF-1 SDS was <-2 in all the patients. 71.4% of patients had a suboptimal GH response following stimulation with GHRH-Arginine. All patients had stimulable, although a delayed GH response with glucagon stimulation. 20% of patients had GH insensitivity. Four patients were treated with hGH for a mean duration of 5.75 months, achieved normalization of IGF-1 levels and improvement in growth velocity improved from 0.21 to 0.86 cm/month. Imatinib results in an acquired neurosecretory defect in GH secretion. Treatment with growth hormone leads to an improvement in growth velocity and normalization of IGF-1.

Long-Acting Growth Hormone Preparations - Current Status and Future Considerations.

Long-acting GH (LAGH) preparations are currently being developed in an attempt to improve adherence. The profile of GH action following administration of LAGH raises practical questions about clinical monitoring and long-term safety and efficacy of these new therapeutic agents. Recent literature and meeting proceedings regarding LAGH preparations are reviewed. Multiple LAGH preparations are currently at various stages of development, allowing for decreased GH injection frequency from daily to weekly, biweekly, or monthly. Following administration of LAGH, the serum peak and trough GH and IGF-I levels vary depending upon the mechanism used to prolong GH action. Randomized, controlled clinical trials of some LAGH preparations have reported non-inferiority compared with daily recombinant human GH (rhGH) for improved growth velocity and body composition in children and adults with GH deficiency (GHD), respectively. No significant LAGH-related adverse events have been reported during short-term therapy. Multiple LAGH preparations are proceeding through clinical development with some showing promising evidence of short-term clinical efficacy and safety in children and adults with GHD. The relationship of transient elevations of GH and IGF-I following administration of LAGH to efficacy and safety remain to be elucidated. For LAGH to replace daily rhGH in the treatment of individuals with GHD, a number of practical questions need to be addressed including methods of dose adjustment, timing of monitoring of IGF-I, safety, efficacy, and cost-effectiveness. Long-term surveillance of efficacy and safety of LAGH preparations will be needed to answer these clinically relevant questions.

Improving Growth Velocity in Very Low Birth Weight Infants: a Quality Improvement Project

Background: Inadequate nutritional support in very low birth weight (VLBW) infants results in growth failure and poor neurodevelopmental outcomes. Early optimization of nutrition, especially protein intake, is an important strategy to achieve normal postnatal growth. Most infants postnatally demonstrate weight loss, do not sustain normal intrauterine growth rates once growth resumes, and do not achieve normal growth by term gestation. Identified reasons include: delays in achieving adequate nutrition, inadequate protein intake, late initiation of enteral feedings, use of unfortified human milk, and variability in practices between providers. To improve growth …

Improving Growth Velocity in Very Low Birth Weight Infants: a Quality Improvement Project

Improving Growth Velocity in Very Low Birth Weight Infants: a Quality Improvement Project

SUN-290 Responsiveness of Growth Hormone Therapy in a Patient with Complex Chromosome 4 q Interstitial Deletion

Background Information: Chromosome 4 q interstitial deletion is a rare chromosomal disorder characterized by post natal growth retardation, developmental delay, craniofacial dysmorphism and behavioral problems. Not only are the characteristics of growth failure in these patients ill defined, but their responsiveness to growth hormone (GH) treatment is also lacking in the literature. An index case of a 7 year old male with unique interstitial deletion of chromosome 4 q deletion at q13.3 and q22.12 with a normal segment in between presented with significant growth failure in early infancy and was treated with growth hormone as described below. Case Report: He was born at 36 weeks gestation with a birth weight of 4 pounds 6 ounces (3rd percentile) and a birth length of 19 inches (50th percentile). Pregnancy was complicated by oligohydraminos. Post natal course was complicated by feeding difficulties and jaundice. He grew along the 5th percentile till age 6 months and then started faltering off below the 3rd percentile in both height and weight. He was started on dense caloric formula but his growth parameters continued to decline. His intake was reported good and had no diarrhea or vomiting. A TFT obtained demonstrated a slightly elevated TSH of 9.4 mcIU/ml (reference range 0.4-9.2) and marginally low Free T4 of 0.76 ng/dl (reference range 0.89-1.48). He was started on a small dose of 12.50 mcg thyroxin daily which did not improve his growth (thyroxin was discontinued at age 4 ½ year). His chemistry panel, IGF-1 and IGFBP-3 obtained were all normal. A bone age was concordant with his chronological age. Karyotype was 46 XY. Comparative genomic hybridization (CGH) array revealed a de novo 2.2 Mb deletion at 4q 13.3 and 2.2 Mb deletion at 4q22.1-q22.3. Deletions were separated by 18.3 Mb of normal copy number sequence. Other tests obtained demonstrated a normal pituitary gland on brain MRI and no tethered cord on the spinal MRI. He was started on GH for small for gestational age indication at age 13 month. His height and weight percentile improved significantly from <1% at age 13 month to 5th% by age 2 year and has continued to grow along 10-12 % since age 4 year. His other associated medical problems include improving developmental delay (attends special class with IEP and speech therapy), sleep disturbances (on melatonin and clonidine), mild hypotonia (received OT and PT, now resolved), poor dental enamel (has needed 8 crowns), obstructive sleep apnea with high arched palate (status post turbinate reduction and CPAP), clinodactyly, hyperopia with astigmatism (wears glasses) and sacral dimple. His behavior problems include high anxiety and obsessive/perseveration behavior. Conclusions: Growth failure is a characteristic feature in patients with chromosome 4 q deletion. These patients with growth retardation when treated with GH may respond with improved growth velocity and weight gain and hence should be considered.

Mitotane (op'DDD) restores growth and puberty in nine children with Cushing's disease.

To investigate whether low-dose mitotane (up to 2 g/day) could be a temporary therapeutic alternative to transsphenoidal surgery (TSS) in pediatric Cushing’s disease (CD). Twenty-eight patients with CD aged 12.2 years (± 2.2) were referred to our center. We compared nine patients treated with mitotane alone for at least 6 months to 13 patients cured after surgery. Primary outcomes were changes in growth velocity, BMI and pubertal development. The following results were obtained: (1) Mitotane improved growth velocity z-scores (−3.8 (±0.3) vs −0.2 (±0.6)), BMI z-scores (2.1 (±0.5) vs 1.2 (±0.5) s.d.) and pubertal development. After 1 year on mitotane, the mean BMI z-score was not significantly different in both groups of patients. (2) Control of cortisol secretion was delayed and inconsistent with mitotane used as monotherapy. (3) Side effects were similar to those previously reported, reversible and dose dependent: unspecific digestive symptoms, concentration or memory problems, physical exhaustion, adrenal insufficiency and hepatitis. (4) In one patient, progressive growth of a pituitary adenoma was observed over 40 months of mitotane treatment, allowing selective adenomectomy by TSS. In conclusions, low-dose mitotane can restore growth velocity and pubertal development and decrease BMI in children with CD, even without optimal control of cortisol secretion. It may promote pituitary tumor growth thus facilitating second-line TSS. However, given its possibly life-threatening side effects (transient adrenal insufficiency and hepatitis), and in the absence of any reliable follow-up procedures, this therapy may be difficult to manage and should always be initiated and monitored by specialized teams.

Phase-field modelling on effect of pressure on growth kinetics of Mg–Al–Sn alloy

ABSTRACTThe effects of pressure on dendritic growth kinetics of Mg–Al–Sn alloy were investigated using a ternary phase-field model coupled with thermodynamics with pressure effects and experiments. The results showed that the improved growth velocity and nucleation rate caused by pressure had the opposite effects on grain size. For one-grain growth, the dendrite was larger and more developed under pressure of 85 MPa than that under ambient pressure owing to larger thermodynamic driving force, and thus higher growth velocity. However, when nucleation was considered in multigrain growth case, the average grain size under pressure was smaller owing to less growth space. Growth velocity decreased with the increase in Sn content, on which pressure had no great influence.

The Safety and Efficacy of Growth Hormone Secretagogues.

Growth hormone (GH) increases lean body mass, decreases fat mass, increases exercise tolerance and maximum oxygen uptake, enhances muscle strength, and improves linear growth. Long-term studies of GH administration offer conflicting results on its safety, which has led to strict Food and Drug Administration criteria for GH use. The potential drawbacks of exogenous GH use are believed to be due in part to impaired regulatory feedback. To review the literature on GH secretagogues (GHSs), which include GH-releasing peptides and the orally available small-molecule drug ibutamoren mesylate. Review of clinical studies on the safety and efficacy of GHSs in human subjects. Report on the physiologic changes from GHS use in human subjects including its safety profile. GHSs promote pulsatile release of GH that is subject to negative feedback and can prevent supra-therapeutic levels of GH and their sequelae. To date, few long-term, rigorously controlled studies have examined the efficacy and safety of GHSs, although GHSs might improve growth velocity in children, stimulate appetite, improve lean mass in wasting states and in obese individuals, decrease bone turnover, increase fat-free mass, and improve sleep. Available studies indicate that GHSs are well tolerated, with some concern for increases in blood glucose because of decreases in insulin sensitivity. Further work is needed to better understand the long-term impact of GHSs on human anatomy and physiology and more specifically in the context of a diversity of clinical scenarios. Furthermore, the safety of these compounds with long-term use, including evaluation of cancer incidence and mortality, is needed. Sigalos JT, Pastuszak AW. The Safety and Efficacy of Growth Hormone Secretagogues. Sex Med Rev 2018;6:45-53.

Implementation of Feeding Guidelines Hastens the Time to Initiation of Enteral Feeds and Improves Growth Velocity in Very Low Birth-Weight Infants

In Brief The CE Test for this issue is available online on the journal Web site and can be taken at http://www.nursingcenter.com/ce/anc

Implementation of Feeding Guidelines Hastens the Time to Initiation of Enteral Feeds and Improves Growth Velocity in Very Low Birth-Weight Infants.

Growth and nutrition are critical in neonatal care. Whether feeding guidelines improve growth and nutrition and reduce morbidity is unknown. Feeding guidelines for very low birth-weight (VLBW) infants were implemented in our neonatal intensive care unit (NICU) to start and achieve full enteral feeds sooner, and increase weight gain over the first month. Feeding guidelines for VLBW infants were implemented in January 2014, stratified by birth weight (<750, 750-1000, and 1000-1500 g). After trophic feedings, enteral feedings were advanced by 20 to 30 mL/kg/d.Data were analyzed for 2 years prior (baseline) and 6 months after (guideline) guidelines were implemented and included days to initiation of enteral feeds, days on total parenteral nutrition (TPN), and weight gain over the first month. Potential concomitant factors that could affect feeding tolerance were examined including indomethacin or dopamine treatment, delivery room cardiopulmonary resuscitation, and growth restriction. A total of 95 infants with a birth weight of less than 1500 g were included (59 baseline and 36 guideline). Days to start enteral feeds decreased by 47% (P < .01) and days on TPN decreased by 25% (16 days vs 11 days; P < .01). Weight gain over the first month of life increased by 15% (p < .05). Dopamine and indomethacin use decreased during the study period, and small for gestational age infants were overrepresented in the guideline group. Establishment of feeding guidelines for VLBW infants in our NICU reduced the days to start feeds and days on TPN while increasing weight gain over the first month. Improving growth and nutrition and reducing need for TPN in this vulnerable population may ultimately prevent infection and improve neurodevelopmental outcomes.

Enhanced nutrient supply to very low birth weight infants is associated with higher blood amino acid concentrations and improved growth

Customized nutrient supply is vital to ensure optimal growth among very low birth weight infants (birth weight<1500g). The supply of amino acids is especially important due to their impact on protein synthesis and growth. The objectives of this study were to evaluate the impact of enhanced nutrition on growth, blood concentrations of amino acids, and explore possible associations between amino acid concentrations and common neonatal morbidities. We hypothesized higher amino acids levels and growth velocity among infants on enhanced nutrient supply. This randomized controlled trial was performed in three university neonatal intensive care units in Oslo, Norway. Fifty very low birth weight infants were randomized to a control or intervention group. Within 24h after birth, infants in the intervention group received enhanced supply of energy, amino acids, lipids, long-chain polyunsaturated fatty acids and vitamin A, whereas the control group received a standard nutrient supply. The intervention continued until 52 weeks postmenstrual age or until a body weight of 5.5kg was reached. Amino acid analyses were performed at birth, day 3, 5 weeks of age and 5 months corrected age. Detailed information about nutrient intake, morbidities, blood amino acid concentrations and growth velocity were collected from 44 infants (6 infants excluded). High-performance liquid chromatography was used for amino acid analysis. The intervention group (n=23) received higher supply of proteins, with higher blood concentrations of amino acids measured at 5 weeks of age, and improved growth velocity (mean 17.4 vs 14.3g/kg/day, p<0.001) at 36 weeks postmenstrual age, compared to the control group (n=21). The correlation between concentrations of branched chain amino acids (leucine, isoleucine and valine) and growth was stronger and more positive among infants: a) in the control group (correlation coefficient≥0.68, p≤0.004); b) born with birth weight appropriate for gestational age (correlation coefficient≥0.53, p≤0.009) and c) not diagnosed with septicemia (correlation coefficient≥0.63, p≤0.005). Enhanced nutrient supply to very low birth weight infants led to higher blood amino acid concentrations and improved growth. The correlations between amino acid concentrations and growth velocity were weaker in the intervention group as compared to the control group. This could reflect an upper threshold for protein synthesis and growth with our intervention, whereas a potential for further growth with increasing amino acid supply was possible for the control group. NCT01103219.

CAN THE '4 P'S OF PN' IMPROVE NEONATAL NUTRIENT INTAKE?

AimsELBW infants on NICU were found to have poor growth compared to network data. Previous audit work1 showed that the time from birth to PN did not meet unit guideline...

P549. Adalimumab improves growth velocity in children with Crohn’s disease naïve to anti-TNF treatment

P549. Adalimumab improves growth velocity in children with Crohn’s disease naïve to anti-TNF treatment

Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients

Hypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Endocrine testing revealed growth hormone (GH) deficiency, secondary adrenal insufficiency, and tertiary hypothyroidism. An improvement in growth velocity with hormone replacement (GH, thyroxine, and corticosteroid) was seen; however, final adult height remained compromised. Patient 2, a 13-year-old male with Hunter syndrome, was evaluated for growth failure. He had a large empty sella turcica with posteriorly displaced pituitary. Functional endocrine testing was normal and a trial of GH-treatment yielded no significant effect. Panhypopituitarism associated with pituitary anomalies has not been previously reported in Hunter syndrome and was an incidental finding of significant clinical importance. In the setting of documented anterior hypopituitarism, while hormone replacement improved growth velocity, final height remained impaired. In patient 2 with equivocal GH-testing results, treatment had no effect on linear growth. These cases highlight the importance of careful clinical assessment in Hunter syndrome and that judicious hormone replacement may be indicated in individual cases.

Enhanced Nutrient Supply to Very Low Birth Weight Infants is Associated with Improved White Matter Maturation and Head Growth

Background: Extrauterine growth restriction is common among very low birth weight infants (VLBW, BW <1,500 g). Optimal postnatal nutrient supply is essential to limit growth restriction and ensure adequate growth and neurodevelopment. Objectives: We compared an enhanced postnatal nutrient supply to a standard supply and evaluated the effects on growth velocity, head circumference growth and cerebral maturation - the latter by magnetic resonance diffusion tensor imaging (DTI). We hypothesized increased growth velocity, head circumference growth and decreased mean diffusivity (MD) in cerebral white matter (WM) areas, suggesting improved cerebral maturation among infants on the enhanced nutrient supply. Methods: In this randomized controlled trial, infants on the enhanced nutrient supply received increased amounts of energy, protein, fat, essential fatty acids and vitamin A until discharge. DTI was performed close to term equivalent age. Outcomes were growth velocity, head circumference growth and WM mean diffusivity. Results: Among the 50 included infants, 14 in the intervention group and 11 controls underwent a successful DTI. Infants on the enhanced diet achieved improved growth velocity (16.5 vs. 13.8 g/kg/day, p = 0.01) and increased head circumference (Δz score: 0.24 vs. -0.12, p = 0.15). A significantly lower MD was seen in a large WM area such as the superior longitudinal fasciculi (1.19 × 10^-3 vs. 1.24 × 10^-3 mm²/s, p = 0.04, adjusted for age when scanned). Conclusions: Enhanced nutrient supply to VLBW infants is associated with improved growth velocity, increased head circumference growth and decreased regional WM mean diffusivity, suggesting improved maturation of cerebral connective tracts.