Defining priorities in the transition from paediatric to adult healthcare for rare bone disease patients: a dialogic approach.

The Italian patient association for Multiple Osteochondromas, Ollier Disease, and Maffucci Syndrome, Associazione Conto Alla Rovescia-ACAR Aps, conducted a mixed-methods study at its 2023 annual conference. The study included the Open Dialogue Approach and a feedback survey to identify the main priorities in the transitioning process from paediatric to adult healthcare for patients with Multiple Osteochondromas, Ollier Disease, and Maffucci Syndrome. The common needs identified by patients, families, caregivers, and healthcare professionals were coordination and continuity of care, patient empowerment and communication, social and practical support, and transition planning and support. This experience fostered a sense of collaboration and cooperation among stakeholders, helping to build trust and create a shared vision for improving the quality of care for these patients. Furthermore, it could be considered a starting point for other patient associations interested in using different approaches to identify the needs of their members and actively involve all stakeholders.

Humanization of care in pediatric wards: differences between perceptions of users and staff according to department type.

BACKGROUND: As the quality and quantity of patient-centered care may be perceived differently by recipients and independent observers, assessment of humanization of pediatric care remains an elusive issue. Herein we aim to analyze differences between the degrees of verified existing vs. perceived humanization issues of a pediatric ward. Furthermore, we examine whether there is concurrence between the degrees of humanization perceived by users (parents/visitors) vs. staff members. METHODS: The study was conducted in the pediatric wards of seven medical centers of the Campania region (Italy) categorized as general (n = 4), children's (n = 1), and university (n = 2) hospitals. The degree of existing humanization was assessed by a multidisciplinary focus group for each hospital through a pediatric care-oriented checklist specifically developed to individuate the most critical areas (i.e., those with scores < 2.5). The degree of perceived humanization was assessed through four indicators: well-being, social aspects, safety and security, and health promotion. RESULTS: The focus groups showed that critical areas common to all centers were mainly concerned with welfare, mediation, translation, and interpretation services. Specific critical issues were care and organizational processes oriented to the respect and specificity of the person and care of the relationship with the patient. Perceived humanization questionnaires revealed a lack of recreational facilities and mediation and translation services. As for specific features investigated by both tools, it was found that mediation and interpretation services were lacking in all facilities while patient perceptions and observer ratings for space, comfort, and orientation concurred only in the general hospital evaluations. CONCLUSIONS: Future humanization interventions to ensure child- and family-friendly hospital care call for careful preliminary assessments, tailored to each pediatric ward category, which should consider possible differences between perceived and verified characteristics.

A strategic approach for investigating light recipes for 'Outredgeous' red romaine lettuce using white and monochromatic LEDs.

To optimize crop production/quality in space, we studied various "light recipes" that could be used in the Advanced Plant Habitat currently aboard the International Space Station (ISS). Lettuce (Lactuca sativa cv. 'Outredgeous') plants were grown for 28 days under seven treatments of white (W) LEDs (control), red (635 nm) and blue (460 nm) (RB) LEDs, W + blue (B) LEDs, W + green (520 nm) (G) LEDs, W + red (R) LEDs, W + far red (745 nm) (FR) LEDs, and RGB + FR LEDs with ratios similar to natural sunlight. Total PAR was maintained near 180 µmol m-2 s-1 with an 18 h photoperiod. Lettuce grown under RGB + FR produced the greatest leaf expansion and overall shoot biomass, while leaves from WB and RB showed the highest levels of pigmentation, secondary metabolites, and elemental nutrients. All other supplemental treatments had varying impacts on morphology that were dependent on crop age. The WG treatment increased fresh mass early in the cycle, while WR increased biomass later in the cycle. The plants grown under WFR exhibited elongation of petioles, lower nutrient content, and similar shoot biomass to the W control. The findings suggest that supplementing a broad spectrum, white light background with discrete wavelengths can be used to manipulate total yield, morphology, and levels of phytonutrients in lettuce at various times during the crop cycle.

Adult Height after Growth Hormone Treatment at Pubertal Onset in Short Adolescents Born Small for Gestational Age: Results from a Belgian Registry-Based Study.

OBJECTIVES: Information on the efficacy of GH treatment in short SGA children starting their treatment in adolescence is limited. Therefore, adult height (AH), total height gain, and pubertal height gain were evaluated in short SGA children who started GH treatment at pubertal onset. PATIENT AND METHODS: Growth data of 47 short SGA adolescents (22 boys) who started GH treatment at pubertal onset (PUB group) were compared with results from 27 short SGA patients (11 boys) who started GH therapy at least 1 year before pubertal onset (PrePUB group). RESULTS: The PUB group achieved a mean (±SD) total height gain of 0.8 ± 0.7 SDS and an AH of -2.5 ± 0.7 SDS after 4.1 ± 1.1 years of GH treatment with a dosage of 41.8 ± 8.4 µg/kg/day. These results were comparable with those in the PrePUB group, which was treated for a longer duration (5.8 ± 2.1 years), resulting in a total height gain of 1.1 ± 0.7 SDS and an AH of -2.1 ± 1.0 SDS. Multiple regression analysis showed a significantly lower height gain in pubertal patients, females, and patients weighing less at start of GH treatment. An AH above -2 SDS and above the parent-specific lower limit of height was, respectively, reached in 28% and 70% of PUB and 44% and 67% of PrePUB patients (NS). AH SDS was positively correlated with the height SDS at start of GH. CONCLUSIONS: Short SGA adolescents starting GH therapy at an early pubertal stage have a modest and variable height gain. A normal AH can be expected in one third of the patients, especially in those with a smaller height deficit at onset of GH treatment.

Multiple gut-liver axis abnormalities in children with obesity with and without hepatic involvement.

BACKGROUND: Gut-liver axis (GLA) dysfunction appears to play a role in obesity and obesity-related hepatic complications. OBJECTIVES: This study sought to concurrently explore several GLA components in a paediatric obese population with/without liver disease. METHODS: Thirty-two children (mean age 11.2 years) were enrolled: nine controls with normal weight and 23 patients with obesity (OB+). Of the 23 patients OB(+), 12 had not steatosis (ST-), and 11 had steatosis (ST+) (associated [n = 8] or not [n = 3] with hypertransaminasaemia [ALT +/-]). Subjects were characterized by using auxologic, ultrasonographic and laboratory parameters. A glucose hydrogen breath test was performed to test for small intestinal bacterial overgrowth, a urinary lactulose/mannitol ratio (LMR) was obtained to assess intestinal permeability, and tests for transaminases, blood endogenous ethanol, endotoxin and faecal calprotectin were also conducted. RESULTS: Eleven out of 23 patients OB(+) (p < 0.05) exhibited pathological (>90th percentile of the control group values) LMR, with values paralleling the grade of liver involvement (normal weight < OB[+] < OB[+]ST[+]ALT[-] < OB[+)]ST[+]ALT[+] [p < 0.05]). LMR significantly correlated with ethanolaemia (r = 0.38, p = 0.05) and endotoxaemia (r = 0.48, p = 0.015) concentrations. Increased permeability was a risk factor for the development of steatosis (p < 0.002). SIBO was present only in patients with obesity. Faecal calprotectin concentrations were within normal limits in all subjects. CONCLUSIONS: Increased permeability, endogenous ethanol and systemic endotoxin concentrations reflect some GLA dysfunction in obesity and its hepatic complications. Pending further results to establish their potential causative roles, the modulation of the GLA appears to represent a possible target for the prevention and treatment of these conditions.

The shape of the plasma glucose curve during an oral glucose tolerance test as an indicator of Beta cell function and insulin sensitivity in end-pubertal obese girls.

It is hypothesized that the shape of the glucose curve during an oral glucose tolerance test is an early indicator of the risk for developing type 2 diabetes mellitus. In this study, we aimed to examine the shape of plasma glucose response curves and study their relationship with insulin sensitivity, insulin secretion and components of the metabolic syndrome in end-pubertal obese girls. Eighty-one end-pubertal obese girls [median (range) age: 14.4 (11.2-18.0) years; BMI: 34.6 (25.4-50.8) kg/m(²)] who underwent a 2-h oral glucose tolerance test were classified according to the shape of the glucose curve. Four shape types of the plasma glucose response curve were observed: 28 (34.6%) monophasic, 30 (37.0%) biphasic, 14 (17.3%) triphasic, and 9 (11.1%) unclassified. Patients with a monophasic shape had a higher area under the curve for glucose (p = 0.008), a lower early-phase insulin secretion (p = 0.005), and a poorer beta cell function relative to insulin sensitivity as reflected by the oral disposition index (p = 0.022) compared to the bi- and triphasic shape types. In addition, the triglyceride level and TG/HDL-C ratio was higher in patients with a monophasic shape compared to those with a biphasic shape (p = 0.040 and p = 0.048, respectively). In conclusion, end-pubertal obese girls with a monophasic plasma glucose curve are at increased risk for insulin resistance, which can contribute to the development of type 2 diabetes mellitus and cardiovascular diseases.

Exercise tolerance in obese vs. lean adolescents: a systematic review and meta-analysis.

To prescribe feasible and medically safe exercise interventions for obese adolescents, it remains to be determined whether exercise tolerance is altered and whether anomalous cardiopulmonary responses during maximal exercise testing are present. Studies that examined cardiopulmonary responses to maximal exercise testing in obese adolescents were searched: cardiopulmonary exercise tests with respiratory gas exchange measurements of peak oxygen uptake (VO2peak) were performed and comparisons between obese and lean adolescents were made. Study quality was assessed using a standardized item list. By meta-analyses VO2peak, peak cycling power output (Wpeak) and peak heart rate (HRpeak) were compared between groups. Nine articles were selected (333 obese vs. 145 lean adolescents). VO2peak (L min(-1)), HRpeak and Wpeak were not different between groups (P ≥ 0.10), while a trend was found for a reduced VO2peak (mL min(-1) kg(-1) lean tissue mass) (P=0.07) in obese vs. lean adolescents. It remained uncertain whether anomalous cardiopulmonary responses occur during maximal exercise testing in obese adolescents. In conclusion, a trend was found for lowered VO2peak (mL min(-1)kg(-1) lean tissue mass) in obese vs. lean adolescents. Whether cardiopulmonary anomalies during maximal exercise testing would occur in obese adolescents remains uncertain. Studies are therefore warranted to examine the cardiopulmonary response during maximal exercise testing in obese adolescents.

Defining morbid obesity in children based on BMI 40 at age 18 using the extended international (IOTF) cut-offs.

BACKGROUND: Studies have reported that children who are obese are becoming more severely obese. OBJECTIVE: We aimed to classify obese children based on age- and gender-specific centile curves passing through body mass index (BMI) 30, 35 and 40 at age 18 as 'class I', 'class II' or severe, and 'class III' or morbid obesity. METHODS: In addition to the International Obesity Task Force BMI cut-offs corresponding to BMI 30 and 35, we calculated the BMI cut-offs corresponding to BMI 40 using the LMS method proposed by Cole and Lobstein. We classified 217 obese children according to these criteria. RESULTS: Fifty-six (25.8%) children had class III obesity, 73 (33.6%) class II obesity and 88 (40.6%) class I obesity. Class III obese children had a higher waist circumference, systolic blood pressure and fasting insulinaemia compared with less obese children. CONCLUSION: It is clinically important to classify obese children in different classes of obesity severity.

Screening for genetic variants in BDNF that contribute to childhood obesity.

UNLABELLED: What is already known about this subject BDNF is involved in the regulation of food intake and body weight. BDNF deficient animal models are obese. Chromosomal abnormalities cause obesity in humans. What this study adds Evaluation of point mutations in BDNF. Identification of BDNF mutations in obese children. Point mutations in BDNF are not a common cause of childhood obesity. INTRODUCTION: There is ample evidence that BDNF has a role in the regulation of food intake and body weight. Study of various mouse models gave a clear indication that BDNF deficiency leads to the development of obesity. Functional loss of one copy of the BDNF gene, due to chromosomal rearrangements or microdeletions, can cause an obesity phenotype in humans. Therefore, we wanted to investigate whether point mutations in the gene also result in a comparable phenotype. METHODS: We screened 554 severely overweight and obese children and adolescents and 565 lean adults for mutations in the coding region of BDNF. Mutation screening was performed by high-resolution melting curve analysis and direct sequencing. RESULTS: Screening of obese patients led to the identification of two synonymous variations (V37V and H65H) and two non-synonymous coding mutations (T2I and V46M) in the BDNF gene. When we subsequently screened our control population, we found T2I with comparable frequency and confirmed that this is a rare and non-pathogenic variant. In addition, we found another non-synonymous mutation (N187S) in the control population. CONCLUSIONS: In silico analysis of the V46M variant did not support a clear disease-causing effect and no family data were available in order to determine whether the mutation segregates with obesity. However, we cannot rule out a possible pathogenic effect for this variant. In general, we tend to conclude that mutations in the coding region of BDNF are uncommon in obese patients and are therefore not likely to play an essential role in the pathogenesis of childhood obesity.

Mutation screen of the SIM1 gene in pediatric patients with early-onset obesity.

BACKGROUND: The transcription factor SIM1 (Single-minded 1) is involved in the control of food intake and in the pathogenesis of obesity. In mice, Sim1 is involved in the development of the paraventricular nucleus, and Sim1 deficiency leads to severe obesity and hyperphagia. In humans, chromosomal abnormalities in the SIM1 gene region have been reported in obese individuals. Furthermore, recent data also suggest that loss-of-function point mutations in SIM1 are responsible for SIM1 haplo-insufficiency that is involved in causing human obesity. In this study, we therefore wanted to expand the evidence regarding the involvement of SIM1 mutations in the pathogenesis of severe early-onset obesity. METHODS: We screened 561 severely overweight and obese children and adolescents and 453 lean adults for mutations in the coding region of the SIM1 gene. Mutation screening in all patients and lean individuals was performed by high-resolution melting curve analysis combined with direct sequencing. To evaluate the effect of the mutations on SIM1 transcriptional activity, luciferase reporter assays were performed. RESULTS: Mutation analysis identified four novel nonsynonymous coding variants in SIM1 in four unrelated obese individuals: p.L242V, p.T481K, p.A517V and p.D590E. Five synonymous variants, p.P57P, p.F93F, p.I183I, p.V208V and p.T653T, were also identified. Screening of the lean control population revealed the occurrence of four other rare SIM1 variants: p.G408R, p.R471P, p.S492P and p.S622F. For variants p.T481K and p.A517V, which were found in obese individuals, a decrease in SIM1 transcriptional activity was observed, whereas the transcriptional activity of all variants found in lean individuals resembled wild type. CONCLUSIONS: In this study, we have demonstrated the presence of rare SIM1 variants in both an obese pediatric population and a population of lean adult controls. Further, we have shown that functional in vitro analysis of SIM1 variants may help in distinguishing benign variants of no pathogenic significance from variants which contribute to the obesity phenotype.

First characterisation of natural radioactivity in building materials manufactured in Albania.

This study focuses on the radiological characterisation of building materials manufactured in Albania by using a high-resolution gamma-ray spectrometer. The average activity concentrations of (40)K, (226)Ra and (232)Th were, respectively, 644.1±64.2, 33.4 ± 6.4 and 42.2 ± 7.6 Bq kg(-1) in the clay brick samples and 179.7 ± 48.9, 55.0 ± 5.8 and 17.0 ± 3.3 Bq kg(-1) in the cement samples. The calculated activity concentration index (ACI), varied from 0.48±0.02 to 0.63±0.04 in the clay brick samples and from 0.29±0.03 to 0.37±0.02 in the cement samples. Based on the ACI, all of the clay brick and cement samples were categorised as A1 materials. The authors can exclude (at 3σ level) any restriction of their use as bulk materials.

Care delivery and outcomes among Belgian children and adolescents with type 1 diabetes.

UNLABELLED: We aimed to investigate care processes and outcomes among children and adolescents with type 1 diabetes treated in hospital-based multidisciplinary paediatric diabetes centres. Our retrospective cross-sectional study among 12 Belgian centres included data from 974 patients with type 1 diabetes, aged 0-18 years. Questionnaires were used to collect data on demographic and clinical characteristics, as well as process of care completion and outcomes of care in 2008. Most patients lived with both biological or adoption parents (77 %) and had at least one parent of Belgian origin (78 %). Nearly all patients (≥95 %) underwent determination of HbA(1c) and BMI. Screening for retinopathy (55 %) and microalbuminuria (73 %) was less frequent, but rates increased with age and diabetes duration. Median HbA(1c) was 61 mmol/mol (7.7 %) [interquartile range 54-68 mmol/mol (7.1-8.4 %)] and increased with age and insulin dose. HbA(1c) was higher among patients on insulin pump therapy. Median HbA(1c) significantly differed between centres [from 56 mmol/mol (7.3 %) to 66 mmol/mol (8.2 %)]. Incidence of severe hypoglycaemia was 30 per 100 patient-years. Admissions for ketoacidosis had a rate of 3.2 per 100 patient-years. Patients not living with both biological or adoption parents had higher HbA(1c) and more admissions for ketoacidosis. Parents' country of origin was not associated with processes and outcomes of care. CONCLUSION: Outcomes of care ranked well compared to other European countries, while complication screening rates were intermediate. The observed centre variation in HbA(1c) remained unexplained. Outcomes were associated with family structure, highlighting the continuing need for strategies to cope with this emerging challenge.

A new FSA approach for in situ γ ray spectroscopy.

An increasing demand of environmental radioactivity monitoring comes both from the scientific community and from the society. This requires accurate, reliable and fast response preferably from portable radiation detectors. Thanks to recent improvements in the technology, γ spectroscopy with sodium iodide scintillators has been proved to be an excellent tool for in-situ measurements for the identification and quantitative determination of γ ray emitting radioisotopes, reducing time and costs. Both for geological and civil purposes not only (40)K, (238)U, and (232)Th have to be measured, but there is also a growing interest to determine the abundances of anthropic elements, like (137)Cs and (131)I, which are used to monitor the effect of nuclear accidents or other human activities. The Full Spectrum Analysis (FSA) approach has been chosen to analyze the γ spectra. The Non Negative Least Square (NNLS) and the energy calibration adjustment have been implemented in this method for the first time in order to correct the intrinsic problem related with the χ(2) minimization which could lead to artifacts and non physical results in the analysis. A new calibration procedure has been developed for the FSA method by using in situ γ spectra instead of calibration pad spectra. Finally, the new method has been validated by acquiring γ spectra with a 10.16 cm × 10.16 cm sodium iodide detector in 80 different sites in the Ombrone basin, in Tuscany. The results from the FSA method have been compared with the laboratory measurements by using HPGe detectors on soil samples collected particular, the (137)Cs isotopes has been implemented in the analysis since it has been found not negligible during the in-situ measurements.

Influence of pupil dilation on retinal nerve fibre layer measurements with spectral domain OCT.

PURPOSE: To evaluate the influence of pupil dilation on retinal nerve fibre layer (RNFL) measurements with spectral domain optical coherence tomography (OCT). PATIENTS AND METHODS: In total, 29 healthy individuals and 26 glaucoma patients underwent RNFL measurements with the Cirrus HD-OCT Model 4000 (Carl Zeiss Meditec Inc.) before and 30-40 min after pupil dilation with one drop of tropicamide 1%. Average thickness, quadrant thickness, and clock-hour thickness measurements were compared with the paired Student's t-test. We also compared the quality scores of the images obtained pre- and post-pupil dilation. RESULTS: Mean ages in the glaucoma and control groups were 58.3+/-13.4 and 41.6+/-16.4 years respectively (P<0.001). Mean deviation values were -6.96+6.31 dB in the glaucoma group and -1.26+0.79 dB in the control group (P<0.001). Mean RNFL measurements obtained in the glaucoma group were significantly lower than those obtained in the control group (P<0.001). There were no statistically significant differences between mean quality scores obtained before and after dilation neither in the glaucoma group (7.73+/-0.92 vs 7.54+/-1.10, P=0.232) nor in the control group (8.14+/-0.88 vs 8.00+/-0.71, P=0.380). There was no statistically significant difference between mean RNFL measurements obtained pre- and post-pupil dilation neither among normal individuals (P>0.05), nor among glaucoma patients (P>0.05). CONCLUSION: The results of this study indicate that RNFL measurements obtained with spectral domain OCT are not influenced by pupil size.

Central corneal thickness in children with congenital cataract and children with surgical aphakia: a case-control study.

AIM: To measure the central corneal thickness (CCT) of children with congenital cataract and surgical aphakia. METHODS: Children with congenital cataract or surgical aphakia were prospectively recruited and divided into four groups: unilateral cataract (group 1, n = 14), bilateral cataract (group 2, n = 17), unilateral aphakia (group 3, n = 32) and bilateral aphakia (group 4, n = 44). An age-, sex-, and race-matched control group of normal individuals was selected. Ultrasonic pachymetry was performed by the same observer. RESULTS: The mean CCT of the control group was not significantly different from the normal (p = 0.747) and cataractous eyes of group 1 (p = 0.252). The mean CCTs of both eyes of group 2 were significantly higher than the control group (p<0.01). The mean CCT of the aphakic eyes in group 3 was significantly higher than the contralateral healthy eyes and control eyes (p<0.001). The mean CCTs of both eyes of group 4 were significantly higher than the control group (p<0.001). The mean CCT was significantly higher in aphakic eyes of groups 3 and 4 than in cataractous eyes of groups 1 and 2 (p<0.001). CONCLUSIONS: Aphakic eyes due to congenital cataract show thicker corneas than normal phakic eyes. Aphakic eyes after congenital cataract extraction show thicker corneas than eyes with congenital cataracts, suggesting that the increase in CCT occurs postoperatively.

Concerns, expectations and perception regarding stature, physical appearance and psychosocial functioning before and during high-dose growth hormone treatment of short pre-pubertal children born small for gestational age.

BACKGROUND/AIMS: Few data are available about parental concerns and psychosocial functioning of young children born small for gestational age (SGA) treated with growth hormone (GH). The present study focused on the perception of short stature and the concerns and expectations of the parents regarding GH treatment. METHODS: Forty prepubertal short SGA children, randomized into a GH-treated and a GH-untreated group, and their parents were evaluated by a questionnaire and a semi-structured interview at start and after 2 years of follow-up. RESULTS: Before start, 85% of the parents were concerned about short stature, 76% expected an increase in adult height of > or =10 cm and 81% expected a positive impact on well-being. Half of the parents expressed fears regarding GH treatment. After 2 years, more parents of treated children reported obvious growth and physical changes, and fewer parents reported teasing because of short stature. An improvement of well-being was reported by half of the parents of treated and untreated children. Fears about GH treatment disappeared almost completely. CONCLUSION: The perspective of GH treatment induced major adult height expectations. In treated children, the physical effects of GH treatment became obvious, teasing because of short stature decreased and initial concerns about short stature and GH therapy decreased.

[Childhood obesity as a risk factor for diabetes mellitus type I: the accelerator hypothesis]. / Overgewicht bij kinderen als risicofactor voor diabetes mellitus type I: de acceleratorhypothese.

Diabetes mellitus (DM) type I was diagnosed in two children, a girl aged 10.9 years and a boy aged 10.3 years, who suffered from overweight. Both were treated with subcutaneous insulin injections and dietary adjustments. Some of the data in the literature suggest that overweight or obesity during childhood increases the risk of type-I DM. Important in this connection is the so-called 'accelerator hypothesis', which postulates a common basis for both type-I and type-2 DM with genetic predisposition, insulin resistance (caused by rapid weight gain) and autoimmunity, leading to beta-cell insufficiency, as 'accelerators'. It is important to consider a diagnosis of type-I DM in children with overweight or obesity, especially in case of abnormal weight loss associated with polydipsia and polyuria.

Effect of 2 years of high-dose growth hormone therapy on cognitive and psychosocial development in short children born small for gestational age.

OBJECTIVE AND DESIGN: Children born small for gestational age (SGA) are not only at risk for short stature, but also for neurodevelopmental and behavioral problems. In this study, we analyzed the effects of high-dose GH therapy on cognitive development and psychosocial functioning in 34 prepubertal (3-8 years) short SGA children, equally randomized into a GH-treated group (TRG) and an untreated group (UTRG). METHODS: At start and after 2 years, children underwent standardized tests measuring the intellectual abilities (Wechsler Preschool and Primary Scale of Intelligence-Revised, or Wechsler Intelligence Scale for Children-Revised); their parents completed a standardized questionnaire evaluating psychosocial functioning (Child Behavior Checklist; CBCL). RESULTS: At start, total IQ scores were significantly (P < 0.05) lower in the SGA group than in the general population: 32% of the SGA patients had scores below 85. After 2 years, IQ scores remained unchanged in the TRG, but increased significantly (P < 0.05) in the UTRG. After exclusion of children with developmental problems, however, no significant changes in IQ scores occurred in the UTRG as well as the TRG. At baseline, 24% (8/34) children had problematic CBCL total problems scores, equally distributed among the two groups; no significant changes in the different subscale scores occurred after 2 years. CONCLUSION: No beneficial effect of 2 years of GH therapy on cognitive and behavioral profile could be observed in a cohort of rather young short SGA children presenting a variable degree of developmental delay and behavioral problems. Subsequent follow-up could reveal potential long-term effects of GH therapy on development and behavior.