Two cases of Klinefelter syndrome with precocious puberty due to mediastinal germ cell tumor.

Background: Klinefelter syndrome may present as precocious puberty, which can be either central precocious puberty or peripheral precocious puberty, caused by an extragonadal germ cell tumor. Objective: Report two cases of Klinefelter syndrome that presented with precocious puberty due to a ?-hCG producing mediastinal tumor. Method: Review of the clinical history, physical examination, and laboratory investigations. Results: Pseudo-precocity developed some years before diagnosis of -hCG producing tumor. The patients did not have typical physical features of this syndrome. The testes were small and had loose consistency. Conclusion: Klinefelter syndrome must be excluded in all boys presenting with precocious puberty due to a ?-hCG producing tumor. Conversely, patients with Klinefelter syndrome should be regularly checked for ?-hCG and ?-fetoprotein levels. In those cases, the patients can be diagnosed and treated early. With the early treatment, they will be able to attain normal adult height and have fewer complications from the tumor.

Bone mineral density and body composition in prepubertal and adolescent patients with the classical form of 21-hydroxylase deficiency.

OBJECTIVES: To evaluate bone mineral density (BMD) and body composition in prepubertal and adolescent patients with the classical form of 21-hydroxylase deficiency. MATERIAL AND METHOD: The authors measured height, weight and used dual energy x-ray absorptiometry (DEXA) to evaluate lumbar spine, whole body BMD and body composition in 10 prepubertal and adolescent patients with the classical form of 21-hydroxylase deficiency. Serum hormone concentrations (17-hydroxyprogesterone, dehydroepiandrosterone sulfate) were also measured. Results were compared with those of age- and sex-matched controls. RESULTS: Seven patients were adolescent (4 girls and 3 boys; age range, 9.0-19.6 years) and three patients were prepubertal. (2 girls and 1 boy; age range, 6.5-8.6 years). There were no significant differences in age, height z-score, weight z-score and body mass index between the patients with congenital adrenal hyperplasia (CAH) and controls. DEXA showed no differences between each group in whole body BMD, but showed significantly elevated areal regional BMD at the lumbar spine (L1-L4) in CAH patients. However four of the 10 CAH patients and 6 of the 10 controls had osteopenia. The BMD z-score at the lumbar spine was significantly correlated with increasing weight z-score in both CAH patients and controls. When CAH patients with osteopenia were compared with those with normal BMD, there was a trend toward lower weight z-score, higher dose of glucocorticoids and longer duration of treatment among the osteopenic patients, but it did not reach statistical significance. CONCLUSION: Classical 21-hydroxylase deficiency patients treated with long-term glucocorticoids did not have impaired bone mineral density compared with healthy, age and sex-matched controls. However, the reference data for BMD in the Thai pediatric population is lacking and the number of studied participants was limited so we need further studies.

Diagnosis and growth hormone (GH) therapy in children with GH deficiency: experience in King Chulalongkorn Memorial Hospital, Thailand.

BACKGROUND: Diagnosis of growth hormone deficiency (GHD) needs both clinical and biological aspects such as auxological data and GHprovocative tests, and active metabolites of GH including IGF-I and IGFBP-3. In GHD children, rhGH has been used worldwide with minimal serious side effects. The aims of the present study were to describe the experience in King Chulalongkorn Memorial Hospital regarding diagnosis and treatment with rhGH in GHD children. MATERIAL AND METHOD: Clinical data of 173 short children was retrospectively reviewed. Two GH provocative tests used in the present study were insulin tolerance test (ITT) and clonidine test. To make the diagnosis of GHD, the children had to fail both GH provocative tests (peak GH < 10 ng/ml). Baseline clinical data, IGF-I, and IGFBP-3 were compared between the group with true positive test and the group with false positive test. Thirty-five children with GHD, who had been treated with rhGH, were evaluated in terms of growth response, changes of IGF-I SDS and the relationship between these parameters. RESULTS: From the present study, ITT could diagnose GHD with true positive 57% and false positive 43% and clonidine could diagnose with true positive 67% and false positive 33%. Clinical data including chronological age, bone age, HtSDS, WtSDS, IGF-I SDS, and IGFBP-3 SDS were not different between the true positive and false positive group. rhGH with a mean dose of 29.3 +/- 4.6 microg/kg/day increased height velocity (HV) from 3.9 +/- 2.5 to 9.3 +/- 2.5, 8.1 +/- 1.5, 7.2 +/- 2.2, 6.8 +/- 2.2, 7.6 +/- 2.4, and 6.5 +/- 1.8 cm/yr after 6 months, 1, 2, 3, 4, and 5 years after treatment, respectively. This also improved HtSDS during treatment and brought the HtSDS into the target range after 3 years of treatment. At the end of the first year of treatment, the difference of IGF-I SDS (DeltaIGF-I SDS) > or = 1 could predict a good response (DeltaHtSDS > or = 0.5) with sensitivity of 88.9% and specificity of 60% respectively. At the end of the second year, DeltaIGF-I SDS > or = 1 could predict a good response with sensitivity and specificity of 100% and 29%, respectively. CONCLUSION: From the present study, the authors demonstrated the investigation and treatment practices of short children with GHD. The growth response is satisfactory even with a lower dose than suggested. In addition, measurement of IGF-I and IGFBP-3 cannot be used in diagnosing GHD but can predict the height outcome at least by the first 2 years of the treatment. However long-term outcome need to be clarified.

Bone mineral density and body composition in Thai Precocious Puberty girls treated with GnRH agonist.

Treatment of true Precocious Puberty (PP) with GnRH agonist can improve final adult height by suppressing gonadotropin and sex hormone levels that delays the fusion of long bone epiphyseal growth plates. However, deprivation of estrogen may affect the acquisition of peak bone mass, especially in individuals with low calcium intake. Ten Thai girls with idiopathic true PP were evaluated for Bone Mineral Density (BMD) and body composition by DXA scanner (Hologic, Inc) before and after GnRH agonist therapy for 1 year. During treatment, all children were allowed to consume a normal diet without extra calcium supplementation. In addition, serum calcium, phosphate, alkaline phosphatase and osteocalcin were also measured. The results showed that GnRH agonist could improve predicted adult height from 149.4 +/- 5.4 to 153.6 +/- 6.8 cm (p < 0.001). Serum osteocalcin, representing the bone marker formation, decreased from 184.2 +/- 66.7 to 108.6 +/- 35.3 ng/mL (p = 0.012) However, the treatment had no negative effects on BMD lumbar spine and total BMD but increased percentage of fat mass from 25.7 +/- 5.2 to 31.6 +/- 5.5%. (p =0.007). In conclusion, treatment with GnRH agonist in Thai girls with true PP for 1 year can improve PAH without negative effects on BMD but a longer period of treatment needs to be studied.

Thailand diabetes registry project: type of diabetes, glycemic control and prevalence of microvascular complications in children and adolescents with diabetes.

OBJECTIVE: To determine the etiology, glycemic control and prevalence of microvascular complications in Thai diabetic patients who were diagnosed at the age of less than 18 years and who attended diabetes clinics in university or tertiary care hospitals. MATERIAL AND METHOD: A cross-sectional, multi-center, hospital-based diabetes registry was carried out from diabetes clinics of 11 tertiary centers. Demographic data including laboratory results and microvascular complications were recorded. RESULTS: Two-hundred-and-fifty out of the 9419 (2.66%) diabetic patients were diagnosed before the age of 18 years, 78% had Type 1 diabetes (T1DM), 18.4% had Type2 diabetes (T2DM) and 3.6% had other types of diabetes. Mean +/- SD HbAlc of T1DM was 9.3 +/- 2.5, T2DM was 9.7 +/- 2.6 and other types of diabetes were 8.6 +/- 4%. The majority of patients had poor glycemic control according to ADA and WHO guidelines. The percentage of patients who had diabetes for more than 5 years but had not been screened for nephropathy and retinopathy were 57.7% and 16% in T1DM and were 46.4% and 14.2% in T2DM respectively. A significant correlation between microvacular complications (nephropathy and retinopathy) and duration of disease was found in T1DM (p < 0.001). CONCLUSION: The majority of Thai children and adolescents with diabetes had TIDM and unsatisfactory glycemic control. Screening for microvascular complications was under international standard. The national strategic plan for management of this disease especially in this age group should be urgently implemented.

Water and sodium disorders in children undergoing surgical treatment of brain tumors.

BACKGROUND: Many children with brain tumors have abnormalities in water and sodium homeostasis, such as diabetes insipidus (DI), the syndrome of inappropriate antidiuretic hormone secretion (SIADH) and cerebral salt wasting syndrome (CSW), either pre-operatively or post-operatively. But little data have been published on the incidence, time of onset and clinical course of water and sodium disorders in children with brain tumors. OBJECTIVES: To characterize the water and sodium disorders in children undergoing surgical treatment for brain tumors. PATIENTS AND METHOD: The medical records of children with brain tumors admitted for surgery at King Chulalongkorn Memorial Hospital from January 1997 to December 2002 were retrospectively reviewed. RESULTS: There were 79 patients, 46 males and 33 females included in this study. Water and sodium disorders occurred in 36 patients (45.57%), 23 patients had DI, 12 patients had SIADH and 1 patient had cerebral salt wasting syndrome (CSW). Nine of the 36 patients had pre-operative onset of water and sodium disorders (8 DI and 1 SIADH). The development of DI and SIADH was associated with the location of the brain tumors. Patients with sellar and suprasellar tumors had the highest incidence of DI (75.86%). Patients with tumors of the cerebral hemispheres had the highest incidence of SIADH (44.44%). Onset of post-operative DI ranged from 2 to 19 hours (mean 9.68 hours) and most patients developed SIADH within 24 hours post-operatively. CONCLUSIONS: DI and SIADH are common problems in children with brain tumors, especially after surgical treatment and the onset usually occurs within 24 hours after surgery. Patients with sellar and suprasellar tumors are more likely to have DI, patients with tumors of cerebral hemispheres and posterior fossa tumors are more likely to have SIADH.

Final adult height in "early normal pubertal girls" treated with gonadotropin releasing hormone agonists.

Gonadotropin releasing hormone (GnRH) agonist has been used worldwide for the treatment of central precocious puberty. However, the results on final adult height (FAH) are discrepant in various studies especially in girls with normal early puberty. Fourteen girls with normal early puberty who were treated with depot GnRH agonists 3.75 mg intramuscular (i.m.) monthly for a mean period of 1.5 +/- 0.4 yr were retrospectively studied. The chronological age and bone age at the beginning of treatment were 9.9 +/- 0.7 yr and 12.6 +/- 0.9 yr, respectively. When the treatment was stopped, all the girls were followed-up until they reached their final adult heights. The results showed that the mean FAH was 154.0 +/- 6.9 cm, which was not significantly different from the predicted adult height (PAH) at start of treatment, 153.1 +/- 6.2 m. All the girls were divided into 2 groups. Group A was girls who had FAH-PAH at the start of treatment > or = 1.5 cm and group B, FAH-PAH at the start of treatment < 1.5 cm. The authors found that only the duration of treatment was different between these 2 groups, 1.7 +/- 0.3 yr in group A and 1.3 +/- 0.3 yr in group B (p = 0.015). In conclusion, GnRH agonist cannot improve the final height outcome in girls with normal early puberty. However, a longer period of treatment may improve the height prognosis.

Basal luteinizing hormone/follicle stimulating hormone ratio in diagnosis of central precocious puberty.

BACKGROUND: Precocious puberty is characterized by breast development in girls prior to 8 years old and may have acne, adult odor, growth spurt and menstruation. Conventionally, gonadotropin releasing hormone (GnRH) stimulation test is a gold standard for diagnosis of central precocious puberty but it is a time-comsuming procedure that is not practical on an out patient basis. OBJECTIVE: To evaluate the basal luteinizing hormone (LH)/follicle stimulating hormone (FSH) ratio in diagnosis of central precocious puberty in order to save time and cost. SUBJECTS AND METHOD: The GnRH stimulation tests were performed on 51 girls with breast development before 8 years old. The 51 girls were divided into 2 groups, 24 girls with central precocious puberty (CPP) and 27 girls with premature thelarche (PT), and the clinical data and GnRH stimulation tests data were compared between the 2 groups. The authors also compared the clinical data and GnRH stimulation tests data between 13 girls with PT and 12 girls with thelarche variants (TV) who developed puberty approximately 1 year later as confirmed by GnRH stimulation test. RESULTS: Girls with CPP had a large bone age and chronological age ratio and advancement of breast staging. Girls with TV had a greater level of basal luteinizing hormone (LH), peak LH and 120 min estradiol than girls with PT. Basal luteinizing hormone and follicle stimulating hormone (FSH) ratio greater than 0.2 can be used to diagnose CPP with 75 per cent sensitivity, 85 per cent specificity, 82 per cent positive predictive value (PPV) and 82 per cent negative predictive value (NPV). CONCLUSION: Girls with CPP have a basal LH/FSH ratio greater than 0.2 and this can be used as a cut-off point for the diagnosis CPP.

Measurement of IGF-1, IGFBP-3 and free IGF-1 levels by ELISA in growth hormone (GH) deficient children before and after GH replacement.

Serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) levels reflect the growth hormone (GH) status. A few percent of IGF-1 circulate in a free form which is believed to represent the IGF biological activity. We retrospectively studied the changes of serum IGF-1, serum IGFBP-3, and plasma free IGF-1 levels in growth hormone deficient (GHD) children before and after treatment with recombinant human growth hormone (rhGH) for a period of 6 months and 1 year. Twenty-one GHD children (16 boys and 5 girls) who had the mean chronological and bone ages of 7.7 +/- 0.7 and 4.8 +/- 0.6 years, respectively, were treated with a mean rhGH dose of 11.66 +/- 0.42 U/m2 body surface area/week. Serum IGF-1 level increased from 162.5 +/- 42.9 ng/ml before treatment to 252.8 +/- 49.5 ng/ml (p = 0.007) and 282.7 +/- 86.9 ng/ml after treatment for 6 months and 1 year, respectively. Plasma free IGF-1 also increased from 0.38 +/- 0.30 ng/ml before treatment to 1.21 +/- 0.30 (p = 0.001) and 1.17 +/- 0.42 ng/ml after 6 months and 1 year of treatment. However, serum IGFBP-3 did not significantly increase after treatment. In addition, the free/total IGF-1 ratio decreased after treatment with rhGH. The height velocities at 6 months and 1 year after treatment were negatively correlated with plasma free IGF-1 before treatment. In conclusion, therefore, plasma free IGF-1 levels could serve as a good predictor of growth hormone responses. Furthermore, their circulating levels would be modified by serum IGF-1 status, and possibly, IGFBP-3 protease activity.

Linear growth of prepubertal asthmatic Thai children receiving long-term inhaled corticosteroids.

BACKGROUND: Long-term inhaled corticosteroids are recommended in persistent asthma in children. The aim of this study was to determine the growth rate of asthmatic Thai children who received long-term inhaled corticosteroids. METHOD: This controlled clinical trial was carried out on 145 prepubertal asthmatic Thai children, 81 in the study group (the group who received long-term inhaled corticosteroids) and 64 in the control group. RESULTS: The mean age of the patients when the study began was 5.9 years in the study group and 4.7 years in the control group. The average dose of inhaled corticosteroids used was 300 microg/day. The average duration overtime of inhaled corticosteroid usage was 2 years (9 months-5 years). The study showed that inhaled corticosteroids (300 microg/day for 9-60 months) have no significant effects on growth rate. From linear regression analysis, the factors that influenced the growth rate of asthmatic patients were the severity of the asthma and the age of the patients when nasal corticosteroids were started. Insulin-like Growth Factor 1 (IGF 1) and Insulin-like Growth Factor Binding Protein 3 (IGFBP 3), were measured in 7 patients in the study group and 5 in the control group. All were normal, the height standard deviation score (Ht.SDS) was below average. CONCLUSIONS: The average dose of inhaled corticosteroids (300 microg/day for 9 months) has no significant effects on the growth rate of prepubertal asthmatic Thai children.

Adrenal functions in children with adrenoleukodystrophy.

BACKGROUND: Adrenoleukodystrophy refers to an inherited disorder that mainly affects the adrenal gland, and the nervous system. The most common type is X-linked adrenoleukodystrophy (XALD). The main presenting symptoms are behavioral changes. However, endocrinological manifestations are also important and need to be clarified especially adrenal insufficiency which is a lifethreatening condition that can be prevented. OBJECTIVE: To review the endocrinological and the adrenal functions in X-linked ALD. SUBJECTS AND METHOD: The medical records of four patients diagnosed with ALD at the Endocrinology and Metabolic Unit, Department of Pediatrics, King Chulalongkorn Memorial Hospital between 1998 and 2000 were reviewed. The diagnoses were confirmed by elevated very long chain fatty acid (VLCFA) levels and the typical changes seen on magnetic resonance imaging (MRI) of the brain. The adrenal functions in these patients were studied. RESULTS: All patients presented between 7-11 years of age with learning problems and behavioral changes, without symptoms of adrenal insufficiency such as nausea, vomiting and abdominal pain. However, the physical signs of adrenal insufficiency such as generalized hyperpigmentation particularly on the nipples, skin creases and genitalia were present. The laboratory investigations revealed normal blood sugar and serum electrolytes. The adrenal functions were revealed as follows. Basal ACTH levels were high in 2 cases (290, > 1,250 pg/mL). Basal cortisol level was low in 1 case. ACTH stimulation tests revealed subnormal responses in 3 cases. Magnetic Resonance Imaging of the brain showed white matter degeneration in the occipital area in 2 cases and frontal area in 2 cases. CONCLUSION: Adrenal insufficiency can be detected by laboratory evaluation despite the lack of symptoms, therefore, the adrenal function should be evaluated in X-ALD at diagnosis for proper management.

Sitting height and subischial leg length of Thai schoolboys in Bangkok.

Anthropometry is an important way to evaluate a child's growth pattern. The measurement of sitting height helps to define body proportion. Every country should have its own normal data and use it as a national reference. The measurements of standing height, sitting height, and weight were performed in 2,650 schoolboys, aged 11-18 years, and then the subischial leg length, and the sitting height/height (SH/H) ratio were calculated. The results were compared to the data of British boys studied by Tanner et al. The authors found that, in boys aged less than 15 years, the sitting height in Thai schoolboys was similar to that of British boys but the subischial leg length was longer. However, the sitting height and subischial leg length in Thai schoolboys became shorter than that of British boys after 15 years of age. This resulted in shorter adults when compared with British boys. In addition, the SH/H ratio in Thai schoolboys was the same as that in British boys when they reached the near final height. More data in both genders should be studied in order to construct our national references.

Self-assessment of sexual maturation in Thai children by Tanner photograph.

Assessment of sexual maturation in adolescence is crucially important in clinical practice, because the possibility of concealed disease during the earlier period of child development needs to be detected. However, to undress children sometimes is problematic and unethical in several countries. Therefore, in this study the authors evaluated the concordance between children's self-assessment by Tanner photograph with a written description, and examination by pediatricians. One hundred and ninety four children (100 girls, 94 boys), aged 7-15 years, were recruited in this study. The outcome demonstrated that the two processes of investigation were in good concord: the weight kappa of 0.76 and 0.79 for the breast stage (B) and pubic hair stage (PH), respectively, in girls. In boys, the weight kappa were 0.59 and 0.73 for genital stage (G) and pubic hair (PH) stage, respectively. However, the weight kappa for genital stage was improved after the subjects were provided more time to examine themselves before choosing the photograph. In girls, the mean chronological age (CA) for B stage II, III, IV was 10.4+/-1.3, 12.5+/-1.3, 13.6+/-0.7 years and for PH stage II, III, IV was 11.9+/-1.4, 12.9+/-1.2, 13.5+/-0.9 years. The normal CA for the onset of puberty in girls was between 7.8-13.0 years. In boys, the mean CA for G stage II, III, IV was 11.3+/-1.7, 12.4+/-1.2, 13.2+/-1.2 years, and for PH II, III, IV was 12.2+/-1.3, 13.1+/-1.0, 13.9+/-1.7 years. The normal CA for the onset of puberty in boys was 7.9-14.7 years. In addition, the authors constructed the normal value for penile length in Thai boys, aged between 9-15 years, to be used as a reference. Therefore, this study demonstrated a good concordance between the self-assessment by Tanner photograph and examination by pediatricians. This can be applied for use in filed research, school screening and clinical practice.

Insulin-like growth factors and their binding proteins in children with IDDM.

The structure of insulin-like growth factor (IGF), especially IGF-I, and its receptor is similar to that of insulin. Therefore, the changes of IGFs and IGF-binding proteins may be related to glucose homeostasis in children with insulin dependent diabetes mellitus (IDDM). Sixty-three fasting blood samples of 21 children with IDDM attending 3 consecutive diabetic clinics were studied. The HbA1c progressively decreased from the 1st to the 3rd visit. IGF-I levels, both total and free forms, were not significantly different from that of control. IGFBP-3 levels in 3 visits (3406+/-305, 3376+/-252, and 2406+/-247 ng/mL) were significantly lower than that of control (5020+/-415 ng/mL) with the p value of 0.007, 0.002, and < 0.001 respectively. IGFBP-1 levels in the 1st and 2nd visits (102.1+/-12.9 and 114.1+/-14.5 ng/mL) were significantly higher than that of control (60.1+/-15.2 ng/mL) with the p value of 0.03 and 0.01 respectively, but not in the 3rd visit. IGF-I level had a positive correlation with IGFBP-3 (R=0.56, p=0.01) and free IGF-I (R=0.53, p=0.01). Free IGF-I had a negative correlation with IGFBP-1 (R=-0.64, p=0.01). IGF-II at the 15 visit had a negative correlation with HbA1c (R=-0.49, p=0.047). The authors found no correlations between IGF-I, IGFBP-3, free IGF-I, IGFBP-1 and HbA1c in the study. The patients' height SDS followed the genetic height potential. It was, therefore, postulated that a near normal free IGF-I level in diabetic children resulted from a balance of interaction between IGFBP-1 and IGFBP-3 to total IGF-I in order to keep the normal metabolic status as much as possible.

Studies of GH gene defects in familial Isolated Growth Hormone Deficiency (IGHD).

One family with isolated GH deficiency (IGHD) was Studied. To determine GHI gene deletions, PCR and Southern blot analyses were used. None of the possible deletions were found in the subjects but the GH1 gene mutation was found. The family consisted of parents (both 140 cm) and their three children with isolated GHD. The daughter and two sons were first seen between 2.9 and 5.3 years of age when their HtSDSs were -2.2 to -3.6, and peak GHs were 0.9 to 4.0 mg/ml. The GH1 gene change was found in G-->A substitution at +28 in the intervening sequence or intron 3 (IVS3+28 G-->A). This change is a dominant-negative mutation which has never occurred in any reports in any reports in Thailand and we were the first group to report here. The segregation which and expression studies of the IVS3+28 G-->A variant are underway to confirm whether it is a new dominant-negative mutation that causes GHD by perturbing mRNA splicing.

Improving height velocity in boys with constitutional delayed growth and puberty (CDGP) by using oral testosterone undecanoate.

Treatment with oral testosterone undecanoate (40 mg daily) in 9 boys diagnosed as having constitutional delayed growth and puberty (CDGP) was studied. The mean chronological age was 12.1+ 1.7 years and the mean duration of treatment was 0.7+ 0.3 year. The height velocity was increased from 3.9+ 2.1 cm per year before treatment up to 8.1+ 2.3 cm per year treatment (p=0.003). However, the predicted adult height was not different between pre and post treatment. Eighty-nine percent of children reached the pubertal testicular size after treatment. Therefore, oral testosterone undecanoate was proved to be effective to improve height velocity but the predicted adult height in CDGP boys.

Serum IGF-I, IGF-II and IGFBP-3 levels in children with isolated growth hormone deficiencies and panhypopituitarism.

Serum IGF-I,IGF-II and IGFBP-3 levels were measured by radio-immunometric assay in 24 children, 16 with isolated growth hormone deficiencies (GHD) and 8 with panhypopituitarism. AII serum parameters were significantly lower in panhypopituitarism than in isolated GHD. Serum IGF-I levels were lower than the normal range in 50 percent of the patients. However, 66.7 percent of isolated GHD children older than 10 years had serum IGF-I levels lower than the normal range. AII isolated GHD children had serum IGFBP-3 levels lower than the 50th percentile or mean of normal range. Combined IGF-I and IGF-II levels had no more advantage than IGF-L levels in the diagnosis of isolated GHD. Serum IGFII levels were lower than normal range in 87.5 percent. In conclusion, serum IGFBP-3 measurement is useful than IGF-I in all age groups. Low serum IGF-II levels and panhypopituitarism were demonstrated in this study.

Precocious puberty.

Sixty-five patients who presented with clinical features of precocious puberty during 1987-1993 at pediatric endocrinology unit, Siriraj ?Hospital, were studied. Female to male ratio was 6:1. Idiopathic true precocious puberty was the most frequent diagnosis in girls (56%) but 40 percent in boys had CNS lesions. Discriminant analysis showed that the difference between none age and chronological age (BA-CA) and height standard deviation score (htSDS) could discriminate between premature thelarche and precocious puberty with 92 percent accuracy, but not serum baseline FSH, LH and estradiol. Twenty seven percent of premature thelarche had remission in two years. Seventy eight percent of congenital adrenal hyperplasia were salt-losing type.

Pseudoprecocious puberty due to “juvenile granulose cell tumor”: A case report.

The granulose cell tumor of the ovary is an uncommon cause of precocious puberty and only about 5% of these tumors occur in premenarchal girls. However 90% of them have the distinctive features of “Juvenile granulose cell tumor”. We report a case of a 2 ½ year old girl with pseudoprecocious puberty due to juvenile granulose cell tumor. Clinical features in this girl included bilateral breast enlargement, vaginal bleed-ing and a 7-cm-diameter mass in the lower abdomen. Investigations revealed advanced bone age, elevated level of serum estradiol with low levels of serum FSH and LH. A mixed solid and cystic mass at the right side of the ovaries was identified by an ultrasound examination. Right salpingo-oophorectomy was performed and histo-logical examination showed typical Juvenile granulose cell tumor.