Sexual Maturity Rating Research Articles

Does age influence self-perception of the soft-tissue profile in children?

Appreciation of the soft-tissue profile is important in orthodontic diagnosis and treatment. However, are the patients themselves aware of their profile appearance? We aimed to evaluate if age influences self-perception of the soft-tissue profile in children. The study population for this prospective cross-sectional investigation consisted of 3 groups of 60 patients, classified according to age (<12years; 12-15years;>15years). Each subject's right-sided facial profile was photographed to obtain a silhouette. Facial profile silhouette templates were created to represent the local population. Each subject's photograph was inserted into the corresponding template, and the subjects were asked to identify themselves. Facial profile self-recognition was recorded as a binary variable (yes or no). Other recorded variables included age, sex, and sexual maturity rating (using Tanner staging). Chi-square tests were used to analyze facial profile self-recognition between different subgroups, and stepwise multiple regression was used to predict the probabilities of facial profile self-recognition, with age, sexual maturity rating, and other recorded variables as independent variables. Eighty percent of subjects aged>15years recognized their own profile, compared with only 55% and 50% of subjects aged 12-15years and<12years, respectively. Subjects aged>15years were significantly more likely to recognize their profile than younger subjects (P=0.001). Similarly, subjects with the most advanced sexual maturity rating (stage V) were significantly more likely to recognize their profile (85% self-recognition) than those in groups I-IV (P<0.001). Girls were more likely to recognize their profiles than boys (P=0.028). When using multiple regression analysis, sexual maturity rating appears to be the only significant predictor for facial profile self-recognition (R2=0.25; P<0.001). Facial profile self-recognition seems to improve with age and sexual maturity (sexual maturity rating stage V). Because orthodontic treatment planning takes possible soft-tissue changes into account, it is important to evaluate the degree of self-perception of the patients to adapt our goals and treatment discussions.

Long-term outcomes after gonadotropin-releasing hormone agonist treatment in boys with central precocious puberty.

ObjectiveGonadotropin-releasing hormone agonist (GnRHa) treatment improves the potential for gaining height in patients with central precocious puberty (CPP). However, most studies have focused on girls because CPP in boys is relatively rare. Therefore, we aimed to determine the effect of GnRHa treatment on auxological outcomes in boys with CPP.MethodsEighty-five boys with CPP were treated with leuprolide or triptorelin acetate 3.75 mg over 2 years. Anthropometry, bone age, sexual maturity rating, and predicted adult height (PAH) were assessed every 6 months. Furthermore, 20 boys were followed up after treatment discontinuation until achievement of the final adult height (FAH).ResultsThe mean chronological age (CA) and bone age (BA) of the patients with CPP at treatment initiation were 9.5 ± 0.5 years and 11.7 ± 0.9 years, respectively. The mean duration of treatment was 2.87 ± 0.63 years. The PAH at treatment initiation was 172.1 cm (-0.23 ± 1.05 PAH standard deviation score). The PAH at treatment discontinuation (176.2 ± 6.6 cm) was significantly higher than the pretreatment PAH. In addition, the mean final adult height in the 20 boys who were followed up after discontinuation of treatment was 173.4 ± 5.8 cm, which was significantly higher than the initial PAH (170.1 ± 4.5 cm; p = 0.006). In multivariate analysis, the height gain (the difference between the FAH and PAH at treatment initiation) significantly correlated with the target height.ConclusionLong-term GnRHa treatment significantly improved the growth potential and FAH in boys with CPP.

Age, growth and survival rate in two populations of Darevskia derjugini (Nikolsky, 1898) from different altitudes (Squamata: Sauria: Lacertidae)

Abstract We investigated age structure, age at sexual maturity, lifespan, growth and survival rate and adult life expectancy (as demographic life-history traits) as well as body size of two Darevskia derjugini (Artvin lizard) populations from different altitudes, using skeletochronology. Our findings indicated that age upon attaining sexual maturity was two or three years in the low-altitude population (Fındıklı) while it was three years in the high-altitude population (Murgul) for both sexes. The maximum longevity was seven years in the high-elevation site while it was six years in the low-elevation site. As reported for many lizards, we found a significant positive relationship between age and body size within each sex of Artvin lizard at both altitudes. High- and low-altitude populations did not differ in age structure, survival rates, adult life expectancy and body size. Rather than the effect of altitude, which is hard to compare without replication of other low and high altitude populations, the fact that these two populations have similar growth rates and the similarity of local conditions (food availability and predator density) may indicate similarity between the two regions.

Stuart Egginton joins the JEB editorial team

![Graphic][1] When Stuart Egginton picked up the phone in early June 2020 and heard the voice of his old friend Hans Hoppeler, he had no idea why his colleague of four decades was calling. ‘Hans was very chatty and said how he was retiring from Journal of Experimental Biology’, recalls

Rate of sexual maturation and fecundity of the roach Rutilus rutilus (L.) of the Volga reach of the Rybinsk reservoir

Climate warming and the introduction of a new mass species of clam Dreissena bugensis can potentially lead to changes in the reproductive capacity of benthic fish species living in the upper Volga reservoirs. The age of puberty, the structure of the spawning stock, and fecundity are the main indicators of reproduction that determine, together with indicators such as the survival of young fish and the degree of fishing load, the relative number of species in the ichthyofauna. The purpose of this work was to study the size and age of the onset of puberty and the dynamics of fecundity of roach Rutilus rutilus in the Volga ples of the Rybinsk reservoir. Determination of individual absolute fecundity was performed by counting eggs in fresh gonads, without the use of fixation. The age of fish was determined by scales, and vertebrae and operculum were used as additional recording structures. As a result, it is shown that the beginning of sexual maturation of female roach occurs when reaching the age of 3+ and a length of more than 9 cm. Late maturing females were 4+ years old and over 18 cm long. The beginning of sexual maturation of males could not be determined. The smallest males in the catches, 7–8 cm long, were all sexually Mature. The maximum age of fish reached 12+ years with a length of no more than 32 cm. Individual absolute fertility ranged from 1,75 to 178,46 thousand eggs. The value of relative fertility ranged from 79 to 308, with an average of 171 eggs/g. The result concluded that high absolute and relative fecundity of roach and dynamic changes of fertility in terms of increase in average water temperature and absolute improvement of fodder after the invasion of Dreissena bugensis, may indicate the attainment of specific limit reproductive capacity of this species.

Precocious puberty in a 24 month old Nigerian girl: case report

Precocious puberty refers to the appearance of signs of puberty at an earlier age than is considered normal. It occurs ten times more commonly in girls than in boys. The overall incidence ranged from 1/5000 to 1/10,000 children. The cause is idiopathic in 90% of cases of female precocious puberty. We present BA a 24 month old female toddler who presented with one year history of progressive breast development and 6 month history of pubic hair growth. There was associated increasing weight, height and vaginal secretion. There was no similar occurrence in the family. Mother attained menarche at 14 years of age. Essential finding at presentation revealed a toddler who is heavy and tall for age with a weight of 17kg (&gt;95th percentile for age and sex), height of 90.5cm (90th percentile for age and sex), Occipito-frontal circumference of 49cm (normal). Her sexual maturityrating was Tanner stage 3 for breasts and stage 2 for pubic hair. An assessment of precocious puberty was made. Her investigation result showed an advanced bone age of 5 years; elevated serum gonadotrophins in the pubertal range; and essentially normal cranial CT. Abdomino-pelvic USS showed an enlarged uterus for age, and a dominant right follicle with internal echo measuring 17.1mm X 15.2mm. Parents were counseled on the need for treatment to arrest the progression of precocious puberty but yet to respond because of financial constraint after 2 years of diagnosis. Female precocious puberty is ten times more common than male precocious puberty. The aetiology is idiopathic in 90% of cases and It is amenable to treatment. Integration of the investigation and treatment of childhood endocrine disorders into the National Health Insurance scheme will be a great panacea to the challenge of prompt management in developing countries.&#x0D; Keywords: Precocious, puberty, 24 months old, female, idiopathic, poverty, Nigeria

Natural selection on anthropometric traits of Estonian girls

Abstract Natural selection is a key mechanism of evolution, which results from the differential reproduction of individuals due to differences in phenotype. We describe fecundity selection on 13 anthropometric traits in a sample of 4000–10,000 of Estonian girls, who were born between 1937 and 1962 and measured at around 13 years of age. Direct selection favoured shorter, slimmer and lighter girls with smaller heads, more masculine facial and body shapes and slower rates of sexual maturation. Selection was stabilizing for weight, body mass index and face roundness. Direct selection was absent on two markers of general health and viability – handgrip strength and vital lung capacity – although these traits experience negative indirect selection due to their association with educational attainment. Similarly, indirect selection, mediated by educational attainment, accounted for a significant portion of selection for girls with smaller heads, narrower faces, and higher shoulder/hip ratios. These traits are thus subject to gene-culture coevolution, in that selection on body dimensions arises via cultural and behavioural mechanisms.

A prospective study on growth pattern and effect of serum ferritin, haemoglobin levels on physical growth inthalassemic childern

Background: Thalassemia major is one of the most common genetically transmitted diseases in the world and is associated with reduced synthesis of structurally normal hemoglobin. -thalassemia is an autosomal recessive single gene disorder. Keeping this in mind, present study was undertaken to study the growth pattern of transfusion dependent thalassaemic children and to assess the effect serum ferritin, haemoglobin levels on physical growth pattern in thalassemic children. Methods: A prospective observational study was done on 52 thalassaemic patients (aged 2 years-12 years) who attended Department of Pediatrics. Anthropometric measurements like weight and height were taken from all patients. Sexual maturity rating was done in girls 10 years and boys11 years. Lab parameters included pre-transfusion hemoglobin (Hb) and serum ferritin. Percentiles for weight, height and body mass index were calculated using WHO (2007) reference data. Results: Among 52 transfusion dependent thalassemic children majority i.e. 35(67.3%) were more than 6years.Mean age was 8 3.05. Majority of children i.e. 32(61.6%) were male. Mean age of diagnosis was 9.2 months, youngest case being diagnosed at 3rd month, and oldest being at 20 months. Among 52 children most of them are born to non-consanguineous couple. Majority of the children had pre-transfusion haemoglobin in the range of 5-7 gm%. Among 52 children 61.6 % (n=32) had mean serum ferritin more than 2500, and 38.4% (n=20) had mean serum ferritin value less than 2500. The present results showed that, Among 52 children according to IAP standards weight for age less than 50th percentile was 86.5% (n=45) and more than 50th percentile was 13.5%(n=7). Among 45(86.5%) children who had less than 50th percentile weight for age, 19.2 % (n=10) children were less than 6 years of age, remaining 67.3% (n=35) children belongs to 7 to 12 years of age. Among weight for age

Phenology of reproductive condition varies with age and spring weather conditions in male Myotis daubentonii and M. nattereri (Chiroptera: Vespertilionidae)

We examine the extent to which intrinsic and extrinsic factors influence reproductive phenology in male bats at the population level. Using data from thirteen breeding seasons (2006–2018), encompassing the reproductive histories of 1546 Myotis daubentonii and 530 M. nattereri males, we compare rates of sexual maturation and the temporal distribution of phases of spermatogenesis between juvenile (born that season) and adult (born in previous seasons) males. We found that (i) higher proportions of M. daubentonii (50.81%) than M. nattereri (12.85%) became sexually mature as juveniles, (ii) the proportion of juveniles in reproductive condition per annum was influenced by spring weather conditions, (iii) in both species males that reached puberty as juveniles had higher body mass, on average, than immature juveniles, (iv) older males (aged ≥4 years old) commenced spermatogenesis earlier than young adult males (aged 1–3 years old), whilst juveniles that commenced spermatogenesis did so later in the year than adults, in both species, and (v) M. daubentonii commenced and completed spermatogenesis earlier than M. nattereri in the equivalent age class. Our findings suggest that selection pressure exists for early mating readiness and synchronisation with female receptivity.

Limb-Bone Development of Seymouriamorphs: Implications for the Evolution of Growth Strategy in Stem Amniotes

Tetrapod life on land was the result of a lengthy process, the final steps of which resulted in full independence of amniotic tetrapods from the aquatic environment. Developmental strategies, including growth rate and the attainment of sexual maturity, played a major role in this transition. Early amniotes, such as Ophiacodon, tended to reach sexual maturity in a year while most non-amniotic Paleozoic tetrapods (including Devonian tetrapods and temnospondyls) became adult after 3 to 11 years. This ontogenetic transition is accompanied by a drastic change in growth rate and bone microstructure suggesting faster growth dynamics in early amniotes than in Devonian tetrapods and temnospondyls. Was the acquisition of a faster development (earlier sexual maturity and faster growth rate) a drastic evolutionary event or an extended process over geological time? To answer this question, the limb bone histology of two Early Permian (i.e. 270-290 million-year-old) stem-amniote seymouriamorphs, Seymouria sanjuanensis and Discosauriscus austriacus, were investigated. We used three-dimensional bone paleohistology based on propagation phase-contrast synchrotron microtomography. Both seymouriamorphs display relatively fast bone growth and dynamics (even though cyclic in the humerus of D. austriacus). This significantly contrasts with the slow primary bone deposition encountered in the stylopods of temnospondyls and Devonian (i.e. 360 million-year-old) stem tetrapods of similar sizes. On the basis of skeletochronological data, the seymouriamorph D. austriacus retained a long pre-reproductive period as observed in Devonian tetrapods and most temnospondyls. The combination of characteristics (faster growth rate but long pre-reproductive period) suggests that the shift towards an amniotic developmental strategy was an extended process in the evolutionary history of amniotes.

Zebrafish an emerging model for Preclinical Drug Discovery

For understanding the pathogenesis of human disease at cellular and molecular level bio medical research depends on the use of animal models. Maximum animal models used in medical research of human disease are basically performed in murine system. Though there are advantages of using these animals, murine’s have long gestational period, sexual maturation rate and are expensive. These invertebrates lack many structures and organ systems that are involved in human disease pathogenesis. Forward-genetic screens and random mutagenesis based reverse genetics though possible in mouse and are currently underway; they cannot be done on a large scale as they require considerable staff and infrastructure support. Due to these drawbacks other animal models have been developed that provide initial genetic or drug target information. For developmental genetic studies larval zebrafish are used extensively due to their small size, external development, optical transparency, and accessibility in large numbers. Not only are they vertebrate, they also have high fecundity, which can be easily visualised and experimentally manipulated. 70% of all human disease genes have functional homologous in zebrafish. There has been significant increase in the use of zebra fish to elucidate the etiology of human disease like cancer, infectious diseases, cardiovascular disease, kidney disease, diabetes, blindness, deafness, digestive diseases, hematopoiesis, muscle disorders and neural disorders.

Maturity, growth and natural mortality rate of the introduced fish Parachromis managuensis (Perciformes: Cichlidae) in the semiarid region of Brazil

Abstract: Aim The present study aimed to estimate the sexual maturity, growth and mortality rates of the jaguar guapote, Parachromis managuensis, an introduced fish species in the Brazilian semiarid region. Methods Specimens were collected between December 2015 and November 2016, in a dam at Serra Talhada municipality, Pernambuco, where the species was abundant. Results In total, 520 individuals were analyzed, with total lengths (TL) varying between 23 and 240 mm. Length at sexual maturity varied between 130.87 and 140.68 mm TL (Mean = 135.78 mm TL). Through the modal progression of monthly frequency distributions, the following growth parameters were obtained according to the von Bertalanffy model: L∞ = 341.40 mm, k = 0.840 and t0 = -0.035 year-1. Ages varied from 0.05 to 1.41 year, and an age at first maturity between 0.48 and 0.53 year (Mean = 0.51 year), with the largest part of the sample (72.64%) made of adult individuals. Natural mortality rates (M) were estimated from three different methods, varying between 1.53 and 3.04, with a mean of 2.27. Conclusions P. managuensis shows fast growth and maturation and high natural mortalities, which grants it biological advantages when compared to native species from the same trophic level in the area.

Central precocious puberty: A case report

Precocious puberty is the onset of physical and hormonal signs of pubertal development before the age of 9 years in males and 8 years in females. A 2-year 9-month-old Indian male child was brought to the hospital with complaints of excessive growth of pubic hair and increase in penile length for 2 months. On local examination of the genitalia, pubic hair was ++, his penile length was 9 cm, and testicular volume was >4 mL. The Sexual Maturity Rating score was A1P2T4. Serum testosterone levels – 592.76 mmol/L; serum luteinizing hormone – 2.18 IU/L; and serum follicle-stimulating hormone – 4.69 IU/L. His X-ray of the left wrist showed bone age >6 years. Magnetic resonance imaging of the brain showed an ill-defined, T2-hyperintense mass involving the right half of the tuber cinereum. A diagnosis of central precocious puberty due hypothalamic hamartoma was made. During the course of his admission, he was given injection leuprodex 3.75 mg intramuscularly once in 28 days. He was discharged with a plan to follow-up on outpatient basis.

Fixation of genetic variation and optimization of gene expression: The speed of evolution in isolated lizard populations undergoing Reverse Island Syndrome.

The ecological theory of island biogeography suggests that mainland populations should be more genetically divergent from those on large and distant islands rather than from those on small and close islets. Some island populations do not evolve in a linear way, but the process of divergence occurs more rapidly because they undergo a series of phenotypic changes, jointly known as the Island Syndrome. A special case is Reversed Island Syndrome (RIS), in which populations show drastic phenotypic changes both in body shape, skin colouration, age of sexual maturity, aggressiveness, and food intake rates. The populations showing the RIS were observed on islets nearby mainland and recently raised, and for this they are useful models to study the occurrence of rapid evolutionary change. We investigated the timing and mode of evolution of lizard populations adapted through selection on small islets. For our analyses, we used an ad hoc model system of three populations: wild-type lizards from the mainland and insular lizards from a big island (Capri, Italy), both Podarcis siculus siculus not affected by the syndrome, and a lizard population from islet (Scopolo) undergoing the RIS (called P. s. coerulea because of their melanism). The split time of the big (Capri) and small (Scopolo) islands was determined using geological events, like sea-level rises. To infer molecular evolution, we compared five complete mitochondrial genomes for each population to reconstruct the phylogeography and estimate the divergence time between island and mainland lizards. We found a lower mitochondrial mutation rate in Scopolo lizards despite the phenotypic changes achieved in approximately 8,000 years. Furthermore, transcriptome analyses showed significant differential gene expression between islet and mainland lizard populations, suggesting the key role of plasticity in these unpredictable environments.

Dynamic Changes of RFRP3/GPR147 in the Precocious Puberty Model Female Rats.

Pubertal development is a complex physiological process regulated by the neuroendocrine system and hypothalamic-pituitary-gonadal axis. Sexual precocity is a common childhood endocrine disease.The pathogenesis of sexual precocity has not been fully elucidated. RFRP3/GPRl47 signal pathway is able to inhibit the reproductive capability in avians and mammals, probably by acting on the GnRH neuron and pituitary to regulate gonadotrophin synthesis and release. However, little is known about the role of RFRP3 in puberty development and sexual precocity. To observe the dynamic changes of RFamide related peptide 3/G proteincoupled receptor 147 (RFRP3/GPR147) in hypothalamic during puberty development and explore their role in precocious puberty based on a female rat model. The Sprague-Dawley female rats were randomly divided into three groups, normal, vehicle, and precocious puberty model. At 5 days old, the rat model with precocious puberty was prepared by subcutaneously injecting a mixture of danazoldissolved ethanol and glycol. At different day-age (15, 25, 30, 35, and 40 days), the levels of estradiol(E2), follicle-stimulating hormone(FSH), and luteinizing hormone (LH) in the peripheral blood were detected by the enzyme-linked immunosorbent assay, the messenger ribonucleic acid (mRNA) expressions of RFRP3, gonadotropin releasing hormone and GPR147 were examined by real-time polymerase chain reaction(R-T PCR). RFRP3 positive cells were observed using Immunofluorescence confocal microscopy. At 25 and 30 days, the levels of sex hormones and the uterus coefficients were significantly higher in the precocious puberty model group than those in the normal and vehicle groups. The ovarian morphological development in the precocious puberty model rats was significantly earlier than those in the normal and vehicle groups. The mRNA expressions of RFRP3/GPR147 and GnRH in the precocious puberty model group gradually increased and peaked at 25 days. The different day-age and the interaction have significant statistical significance on the expression of RFRP3 mRNA, while the levels of RFRP3 mRNA in the model group and vehicle groups have no significant statistical significance. There was statistical significance between the model group and vehicle groups in different day-age on the expression of GPR147 mRNA.The expression of hypothalamic RFRP3/GPR147 mRNA and RFRP3 positive cells gradually decreased with puberty onset. At 35 days, the levels of RFRP3 mRNA and GPR147 mRNA were significantly lower in the precocious puberty model group than those in the vehicle groups. Meanwhile, the levels of LH in the precocious puberty model rats reached its peak at this age. In the vehicle group, the levels of RFRP3 mRNA and serum LH were gradually increased and LH nearly peaked at 35 day-age. Subsequently, it gradually decreased and reached the lowest level at 35 day-age. The expression of RFRP3 mRNA and LH were positively correlated. The findings suggested that RFRP3/GPR147 signaling pathway may be involved in the pathogenesis of sexual precocity by regulating puberty development and sexual maturity in rats.

Variation in menarcheal age of school-aged female athletes engaged in different types of sport

It is widely known from the literature that the rate of physical development and sexual maturation is not only determined by genetics but is also modified by physical workload factors. Given the above, this paper aimed at comparing menarcheal age of school-aged athletes engaged in different types of sport (and their respective workloads) after controlling for physical traits such as body height and mass and slenderness ratio. The survey was conducted in 2017 in 6 sports schools in Poland’s Lubuskie Voivodeship. Body height and mass were measured and the slenderness index (body height divided by the cubic root of body weight) was used to establish body build. The age of menarche was self-reported by the study participants. In total, 232 girls aged 9–18 were included in the research, of whom 125 reported their age of menarche. The age-adjusted arithmetic means and standard deviations were calculated for the study participants’ estimates. The raw data were then transformed into standardized z-score values. The significance of differences was assessed by Mann-Whitney U test as an alternative to the t-test when a given variable was not normally distributed. Pearson’s correlation coefficient was used to evaluate linear relations between the workload and age at menarche. It was found that dancers, acrobats and swimmers reported the youngest menarcheal ages. Girls practising most demanding sport disciplines (in terms of strength and endurance) reported the oldest age at menarche. Girls practising more than 3 times per week had menarche later than those practising 3 times a week or less, and the difference was statistically significant. In conclusion it might be stated that the type of sport discipline has a significant effect on age of menarche. Sport disciplines involving heavier physical exertion usually delay the onset of menstruation.

Bilateral Inguinal Hernias in a Phenotypic Female Should Raise the Suspicion for a Disorder of Sexual Development (DSD)

Disorders of sexual development are rare and carry high potential for medical and psychosocial co-morbidities. A 4 year old female presented with non-reducible bilateral inguinal masses. Physical exam was consistent with a healthy child with a normal external female genitourinary exam, sexual maturity rating 1. Elective general surgical repair was scheduled. Pre-surgical ultrasound imaging revealed isolated loops of bowel in each inguinal canal, no other inguinal masses, a visualized cervix, and non-visualized uterus. Further work up was initiated upon recommendations from urology given rare …

Study of sexual maturity rating among female adolescents who have attained menarche

Background: Adolescence is an important stage in the growth and development of girls. There has been a change in sectorial trend in the onset of puberty menarche. Thus, this study is designed to study the change in sectorial trend. In this background the study has been framed with these objectives, to correlate the attainment of menarche among the subjects in relation to their mothers and to study the sexual maturity rating among the study subjects.Methods: This is a cross sectional observation study involving 70 female adolescent girls. Data was collected from study subjects admitted in the department of Pediatrics, KIMS Hospital and those visiting KIMS OPD during September 2018 to February 2019. Study Subjects were examined by the female investigator and data on anthropometry, Tanner SMR staging, and other examination findings were collected using a pretested standardized questionnaire, after taking the consent. Subjects were divided based on the age into six groups.Results: The mean age of attainment of menarche is 12.36 years and mean age at menarche in mothers was 14.18 years. In this study the earliest age of attainment of menarche is 10 years. In our study most of the subjects attained menarche between 12-13 years, which is earlier than the age of attainment of menarche in the mothers which was 14-15 years. All the subjects who attained menarche were in SMR Breast stages 3, 4 and 5 and pubic hair stages 2, 3, 4 and 5.Conclusions: There is a positive correlation between the ages of attainment of menarche in the mothers and the subjects in this study it is found that there is a positive correlation between age, weight, height and SMR Stages.

A study of growth pattern in regularly transfused thalassaemic children of age group of 2 years to 12 years

Background: ß-thalassemia is an autosomal recessive single gene disorder. Physical growth failure is one of the most important complications of thalassemia. Very few data regarding growth pattern of thalassemic children is available from India especially North East part. Keeping this in mind, present study was undertaken. To study the growth pattern of transfusion dependent thalassaemic children and to compare growth pattern between regularly and irregularly transfused children.Methods: A cross-sectional observational study was done on 38 thalassaemic patients (aged 2 years-12 years) who attended Department of Pediatrics, Assam Medical College Dibrugarh. History, physical examination and investigations were done and filled in predesigned proforma. Anthropometric measurements like weight and height were taken from all patients. Sexual maturity rating was done in girls ≥10 years and boys ≥11 years. Lab parameters included pre-transfusion hemoglobin (Hb), serum ferritin, LFT, RFT, Thyroid profile. Percentile for weight, height and body mass index were calculated using WHO (2007) reference data. Collected data were compared with age and sex matched normal children.Results: About 34.21% transfusion dependent children had under-nutrition and 50% had stunting. 42% had thinness. Stunting was more in irregularly transfused children (81.25%) as compared to regularly transfused children which was highly significant (p&lt;0.001). Under-nutrition among irregularly transfused children was more (40%) compared to regularly transfused children (28.57%). Pubertal spurt was delayed in 66% children. Those who had Hb &lt;5 gm/dl had 100% stunting and under-nutrition.Conclusions: Regular blood transfusion with growth monitoring and appropriate iron chelation (Sr. Ferritin &gt;1000 ng/ml) is of utmost importance in transfusion dependent thalassaemic children.

Relationship between serum ferritin and endocrinopathies in thalassemic children: A hospital-based study

Background: Thalassemia syndromes are a heterogeneous group of Mendelian disorders characterized by lack of/decreased synthesis of either ?/? globin chains of hemoglobin. It results in ineffective erythropoiesis and lysis of mature red blood cells in the spleen. Thalassemic patients require multiple blood transfusions which led to iron overload. Objective: This study evaluated endocrinopathies in thalassemic patients of age 5–18 years and relationship with serum ferritin level. Materials and Methods: This is a hospital-based cross-sectional study on 70 transfusion dependent thalassemic children of 5–18 years attending the department of pediatrics, of a tertiary care hospital in Assam. Their height, sexual maturity rating, serum ferritin, calcium, alkaline phosphatase, phosphate, thyroid stimulating hormone, random blood sugar, and fasting blood sugar were recorded. The data were analyzed statistically and p &lt;0.05 was considered to be statistically significant. Results: The study population (70) consisted of 40 males and 30 females. In this study, 91.67% (22 of 24 cases) had delayed puberty, 50% (35 of 70) were stunted, 37.14% (26 of 70 cases) were hypocalcemic, 31.43% (22 of 70) had hypothyroidism, and 2.85% (2 of 70) were diabetic. The mean serum ferritin level was 2845±859.40 ng/ml in delayed puberty while it was 2027.50±21.92 ng/ml (p&gt;0.05) in thalassemic children with normal puberty. The mean serum ferritin in stunted thalassemic children was 2909.86±820.93 ng/ml and it was 1421.43±283.41 ng/ml (p&lt;0.001) in normal height thalassemics. The mean serum ferritin in hypothyroid thalassemics is 2806.59±929.57ng/ml and is 1885.75±846.89 ng/ml (p&lt;0.001) in euthyroid cases. The mean serum ferritin in diabetic thalassemics was 2772.50±1092.48 ng/ml and it was 2147.16±965.63 ng/ml (p&gt;0.05) in nondiabetic thalassemics. Conclusion: Growth retardation and endocrinal complications significantly occur in transfusion-dependent thalassemic children. Adequate chelation therapy helps in controlling serum ferritin levels thereby enabling chronically transfused thalassemic children to grow normally without early endocrinal complications.