Pearl Lilos

Body composition in untreated adult patients with Laron syndrome (primary GH insensitivity)

Objective  To quantify body adiposity and its distribution in untreated adult patients with Laron syndrome (LS; primary GH insensitivity) caused by molecular defects of the GH receptor gene or postreceptor pathways and characterized by dwarfism, obesity, insulin resistance and hyperlipidaemia.

Foot Length Before and During Insulin-like Growth Factor-I Treatment of Children with Laron Syndrome Compared to Human Growth Hormone Treatment of Children with Isolated Growth Hormone Deficiency

OBJECTIVE To compare foot length deficits between patients with Laron syndrome (LS) (primary growth hormone [GH] insensitivity) and congenital isolated GH deficiency (IGHD) and their response to replacement therapy with insulin-like growth factor-I (IGF-I) and hGH, respectively. DESIGN Data for the study were collected from the records of nine children with LS (3 M, 6 F) 7.8 +/- 4.8 years old (mean +/- SD), and nine children with IGHD (3 M, 6 F), 3.8 +/- 3.3 years old. Fifteen non-treated adult patients with LS were also included in the study. METHODS Measurements of foot length were recorded without treatment and monitored during 9 years of treatment in the children and in the untreated adult patients. For statistical analysis the non-parametric Mann-Whitney U test was used. RESULTS With almost similar basal values in growth deficit and pre-treatment growth velocities, the achievements towards norms after 9 years of treatment were greater in the patients with IGHD than in the patients with LS: foot length reached -1.4 +/- 0.8 vs. -3.3 +/- 1.0 SDS (mean +/- SD), and body height -2.2 +/- 1.0 vs. -3.9 +/- 0.5 SDS. The difference between the two groups could be due to the initiation of replacement therapy in the patients with IGHD at a younger age. Adult foot size of untreated patients with LS is small but less retarded than the height deficit. CONCLUSIONS Both IGF-I and hGH are potent growth stimulating hormones of linear growth and acrae as exemplified by foot growth.

Serum leptin in obese patients with Laron syndrome before and during IGF-I treatment.

Fifteen patients with primary GH resistance (Laron syndrome, LS) were studied before and during 6 months of daily replacement treatment with IGF-I. The main findings were that patients with LS and normal or high serum GH binding protein (GHBP) were less obese than those with a negative GHBP, and that serum leptin levels varied with body mass as in other types of obesity.

Triglyceride levels and risk of type 2 diabetes mellitus: a longitudinal large study

The relationship between triglyceridemia and diabetes mellitus remains unclear. This study evaluated the risk of diabetes and impaired fasting glucose associated with a wide range of triglyceride levels. A longitudinal retrospective study was carried out employing data from a screening center between the years 2000 and 2012. Inclusion criteria were absence of diabetes at baseline and attendance at the center at least twice over a 5-year period. Participants were divided by fasting blood glucose level (normal/impaired) at the first visit. A total of 5085 participants were eligible for the study. Of the 4164 normoglycemic participants at baseline, 40 (0.96%) had diabetes and 998 (24%) had impaired fasting glucose by the end of the study. On stepwise logistic regression analysis, every 10 mg/dL increase in triglyceride level significantly increased the risk of diabetes by 4% and of impaired fasting glucose by 2% (p<0.001). This association held true even when rising triglyceride levels remained within the accepted normal range (<150 mg/dL, p<0.001). Sustained increments in serum triglyceride level, even within the accepted normal range, are an independent risk factor for diabetes mellitus and impaired fasting glucose in normoglycemic participants.

Growth, development, puberty and adult height before and during treatment in children with congenital isolated growth hormone deficiency

OBJECTIVE To describe the growth, development and puberty in children with congenital IGHD before and during hGH treatment. SUBJECTS Patients with cIGHD treated by hGH between the years 1958-1992. SETTING All patients were diagnosed, treated and followed in our clinic. PARTICIPANTS Data were found in 37/41 patients (21 m, 16 f). 34 had hGH-1A deletions, 7 GHRH-R mutations. Patients, referred after age 25, were excluded. RESULTS The birth length of 10/37 neonates was 48.29±2.26 (44-50) cm. Birth weight of 28/37 neonates was 3380±370 g (m), 3230±409 g (f). Neuromotor milestones were variable. Age at referral was 5.7±4.2 y (m) and 5.6±3.8 y (f). Initiation of hGH treatment (35μg/kg/d) was 7.5±4.8, (0.8-15.08) y (m) and 6.8±4.36 (0.8-16.5) y (f). Height SDS increased from -4.3 to -1.8 (m) and from -4.5 to -2.6 (f). Head circumference increased from -2.6 to -1.3 (m) and from -2.7 to -2.3 (f). BMI increased from 15.8 to 20.6 (m) and from 15.5 to 20.4 (f). There was a negative correlation between age of hGH initiation and change in height SDS (r=-0.66; ρ<0.01), same for bone age (r=-0.69; ρ<0.01). Upper/lower body ratio decreased from 2.5±2.1 (m±SD) to 1.08±0.1 (ρ<0.0005). Puberty was delayed in boys, less so in girls. Mean age of 1st ejaculation (14 m) was 17.6±2.2 y and of menarche (14 f. was 13.7±1.2 y. In both genders there was a positive correlation between age at start of hGH and age at onset of puberty (r=0.57; ρ<0.01). All reached full sexual development but the penile and testicular sizes were below normal. There was a positive correlation between length of hGH treatment and final testicular volume (r=0.597, ρ=0.05) and a negative correlation between the age at initiation of hGH treatment and final testicular volume(r=-0.523, ρ=0.018). All were obese and hGH treatment increased the adiposity progressively (r=0.418, ρ=0.013). CONCLUSION Early diagnosis and treatment of cIGHD enables normal or near normal growth, development and puberty.

Hand size and growth in untreated and IGF-I treated patients with Laron syndrome.

UNLABELLED We have previously reported on the linear growth, growth of the head circumference and foot length in untreated and IGF-I treated patients with Laron syndrome (LS) (primary GH insensitivity). AIM To assess the size and growth of the hands in patients with LS from early childhood to adult age. PATIENTS Ten IGF-I treated children with LS (4 M, 6 F) and 24 untreated patients (10 M, 14 F) were studied. METHODS Measurements of palm length were made on available standardized hand X-rays from infancy to adult age. The measurements were compared to normal references and SD values were calculated for each measurement. The growth of the hand was compared to the concomitant height of the body. RESULTS Hand SDS in untreated patients with LS decreased with age, from a mean of -2.8 +/- 0.7 (age 1-3 years) to -7.3 +/- 0.8 (age 13-15 years) and to -9.0 +/- 3.9 (age 40-50 years). During 9 years of IGF-I treatment the hand size deficit SDS did not improve in contradistinction to the height SDS which decreased from -6.2 +/- 1.2 to -3.9 +/- 0.5. CONCLUSION Congenital IGF-I deficiency, as in Laron syndrome, profoundly affects the size and growth of the hand as part of its growth retardation characteristics, resulting in acromicria.

TI-59 programmable calculator program for calculating predicted operative mortality in general surgery

A program for the TI-59 programmable calculator for calculating predicted postoperative mortality is presented. Input data are based on handy, clinical, non-invasive pre-operative and operative parameters retrieved mostly significant in this respect by former multivariate logistic regression analysis of a broad data-base; their relative weights are incorporated into the program data base as basic coefficients. Considerations employed in its usage are discussed, as well as possible future technical and/or environmental modifications.

The relationship between serum levels of prolactin and growth hormone in the early postnatal period

BackgroundIn the neonatal period, the pituitary hormones including prolactin (PRL) and human growth hormone (hGH) are secreted in high amounts due to immature feedback mechanisms. As both hormones are secreted in part by the same somatomammotrophic cells, we investigated their relationship in newborns with respect to sex, gestational week, method of delivery, and anthropometric data.MethodsThe serum levels of PRL and hGH were measured in blood drawn from 225 newborns. The newborn data were extracted from medical records.ResultsA positive correlation was found between log-transformations of PRL and hGH (r=0.17; P=0.01; n=225), with a stronger correlation in newborns whose blood samples were taken more than 2 days after birth (r=0.42; P<0.001; n=130). Log-transformations of the PRL/hGH ratio demonstrated a positive correlation with the gestational week (r=0.39; P<0.001; n=200). Multiple regression analysis showed that 15% of the variance in the logarithm of this ratio is attributed to the gestational week.ConclusionIn newborns, serum PRL and hGH levels show a positive correlation that can be explained by common regulatory factors or a drift phenomenon. A higher gestational week is associated with a higher PRL/hGH ratio. Further studies are needed to look for possible confounders and to determine the PRL–hGH relationship in different conditions.

Growth, development, puberty and adult height of patients with congenital multiple pituitary hormone deficiencies

OBJECTIVE Congenital MPHD is a rare condition caused by mutations in pituitary transcription factors genes: PROP1, POU1F1 (PIT1), HESX1, LHX3, LHX4. DESIGN We evaluated in a retrospective study the effects on growth and development in 29 patients with congenital MPHD (cMPHD), during hGH replacement therapy alone and combined with sex hormones. Twenty nine patients with cMPHD were included and diagnosed, treated and followed in our clinic from diagnosis to adult age. Measurements on growth and development were taken by the same medical team. RESULTS Mean birth weight of 21/29 neonates was 3126 ± 536 g. Mean birth length of 7/29 neonates was 48.7 ± 2 cm. Neuromotor development was normal or slightly delayed. Mean age at referral was 9.5 ± 7 years (m), 6.7 ± 3.5 years (f) (p=0.17). Height (SDS) before treatment was -2.8 ± 1.0 (m), -2.8 ± 1.0 (f) (p=0.99). Mean age at initiation of hGH treatment was 9.9 ± 6.7 years (m), 10.3 ± 4.2 years (f) (p=0.85). Mean age at initiation of sex hormone treatment was 17.0 ± 3.5 years (m), 17.1 ± 2.3 years (f) (p=0.88). Penile and testicular sizes were below normal before and after treatment. Head circumference (SD) was -1.9 ± 0.9 before and -0.6 ± 1.8 at end of treatment (p<0.001). Adult height (SDS) reached -1.1 ± 0.6 (p<0.001) for both males and females. CONCLUSION Despite the multiple pituitary hormone deficiencies including hGH, children with congenital MPHD present with a better auxological development than children with congenital IGHD or congenital IGF-1 deficiency. These findings may be due to irregular and incomplete hormone deficiencies increasing with progressive age and late initiation of puberty.