Onset of obesity in children through the recall of parents: relationship to parental obesity and life events.

Obesity is a multifactorial disease due to the influence of both genetic and environmental factors. Parents of 886 obese patients (427 boys and 459 girls, aged 1-18 years) were investigated by means of a questionnaire in order to study the factors associated with the onset of obesity in children and the relationship to genetic background. At presentation obese patients had a very high mean ideal body weight percent (IBW%) (154 +/- 19%, median 152%, range 120-246). A significantly higher mean IBW% was found in children of obese parents (one obese parent: 158 +/- 21%, median 156%, range 120-246; two obese parents 160 +/- 18%, median 158%, range 123-226) in comparison to children of normal weight parents (150 +/- 18%; median 147%, range 120-235; p = 0.0001 for children of one or two obese parents versus children of normal weight parents). Parents of 414 subjects (46.7%) (Group A) answered that obesity had always been present. The remaining 472 parents (53.3%) (Group B) stated that obesity had had a beginning at a mean age of 5.3 +/- 2.6 years (median 5.0 years, range 1.0-17.0). No difference was found in age at presentation, sex distribution, birth weight and number of obese parents between the two groups. Parents in Group B recalled an event associated with obesity onset in 197 cases (health event: 119 answers, psycho-social event: 78 answers). Genetic background did not influence this pattern of feedback. In conclusion, parents of obese subjects seek medical advice when obesity is severe. Pediatricians should exert strict surveillance on weight from a very young age. The recall in 22% patients of health or psychosocial events at the onset of obesity emphasizes that medical counselling is important when the occurrence of particular events in life may cause erroneous eating habits.

Liver involvement in obese children. Ultrasonography and liver enzyme levels at diagnosis and during follow-up in an Italian population.

Our aim was to evaluate incidence and risk factors of liver involvement in obese Italian children as assessed by both ultrasonographic and biochemical parameters. In seventy-five consecutive obese children (age 9.5 +/- 2.9 years, males/females 41/34), serum levels of enzymes and ultrasonography of the liver were evaluated. Tests were repeated one, three, and six months after starting a moderate hypocaloric diet and an exercise program. Three obese children who were found to have chronic viral hepatitis were excluded from the study. Thirty-eight of 72 (53%) obese children had an ultrasonographic image of bright liver consistent with liver steatosis. The latter was severe in nine children, moderate in 16, and mild in 13. Eighteen obese children (25%) had elevated transaminase levels. Bright liver and hypertransaminasemia were not due to any of the most common causes of liver disease. Both were rapidly responsive to loss of weight, confirming that liver involvement was secondary to obesity and that steatosis or steatohepatitis rather than fibrosis were involved. Obesity duration not more than three years (odds ratio = 4.77), a higher degree of obesity (odds ratio = 2.09), and hypertransaminasemia (odds ratio = 2.15) appeared as important predictive factors of liver involvement at ultrasonography. Incidence of liver involvement assessed by means of ultrasonography is significantly higher than that revealed by measurement of serum liver enzymes. A short duration of obesity emerged as a potentially new risk factor of liver involvement in the pediatric obese population and needs to be confirmed in future studies.

Pubertal growth, sexual maturation, and final height in children with IDDM. Effects of age at onset and metabolic control.

OBJECTIVE: To evaluate growth and pubertal development in children with IDDM and the influence of the age at onset of IDDM and the degree of metabolic control on final height. RESEARCH DESIGN AND METHODS: We conducted a retrospective evaluation of 62 subjects followed longitudinally both clinically and metabolically from the onset of IDDM until final height was reached. RESULTS: Height at diagnosis was within the normal percentiles in boys (0.5 +/- 1.0 standard deviation score [SDS]) and girls (0.4 +/- 1.0 SDS), but above the genetic target height (-1.0 +/- 0.9 SDS in boys and -1.1 +/- 0.6 SDS in girls; P = 0.0001 for both comparisons). Although a lesser height gain was observed during the ensuing years, the final height reached by boys (-0.4 +/- 1.1 SDS) and girls (-0.4 +/- 0.9 SDS) was higher than the genetic target height. Blunted total pubertal growth was observed both in boys (24.5 +/- 3.6 cm) and girls (20.1 +/- 4.2 cm). The decrease in height gain was independent of the duration of IDDM, the degree of metabolic control, or the insulin requirement. The greater the height at diagnosis, with respect to the genetic target height, the lesser was the subsequent height gain to reach final adult height (r = 0.34, p < 0.01). BMI increased with age as normally occurs in healthy children, independent of the duration of disease and the degree of metabolic control. Pubertal development began and progressed normally both in boys and girls. In boys, a testicular volume of 4 ml was reached at a mean age of 12.1 +/- 0.9 years. In girls, breast enlargement occurred at a mean age of 10.4 +/- 1.2 years and the mean age of menarche was 12.8 +/- 1.4 years. Pubertal development and progression occurred independent of the age at onset of IDDM, the glycemic control, or the insulin requirement during the pubertal period. CONCLUSIONS: Children with IDDM have normal onset of puberty and normal sexual maturation. Even though final height falls within the normal percentiles, the diminished height gain after diagnosis requires further investigation.

Assessment of red blood cell indices in growth-hormone-treated children.

In order to evaluate the effect of growth hormone (GH) on erythropoiesis, red blood cell (RBC) indices (hemoglobin, Hb; hematocrit, Ht; RBC count, and mean corpuscular volume, MCV) of 19 GH-deficient children (12 with isolated GH deficiency and 7 with multiple pituitary hormone deficiencies) between 2 months and 15 years of age were compared to those of 57 sex- and age-matched short normal controls before starting treatment with recombinant human GH (rhGH). The RBC indices were expressed as standard deviation score (SDS). Moreover, the RBC indices in the GH-deficient group were analyzed after the first 3 and 6 months of GH treatment and compared to those of 9 Ullrich-Turner syndrome (UTS) patients with GH therapy. Both patients with isolated and those with multiple pituitary hormone deficiencies presented significantly lower values of Hb-SDS (-1.6 +/- 1.0 and -2.0 +/- 1.4, respectively; p = 0.004), Ht-SDS (-1.55 +/- 0.9 and -2.5 +/- 2.1, respectively; p = 0.001) and RBC-SDS (-0.6 +/- 1.6 and -1.2 +/- 0.9, respectively; p = 0.002) when compared to controls (Hb-SDS: -0.6 +/- 1.4; Ht-SDS: -0.1 +/- 1.9; RBC-SDS: 0.17 +/- 1), in the presence of comparable MCV-SDS values. In contrast, RBC indices did not differ between patients with isolated and those with multiple pituitary hormone deficiencies. When the variations of RBC indices were analyzed after 3 and 6 months of rhGH therapy in the 19 GH-deficient children, an increase in the Hb-SDS (p = 0.01), Ht-SDS (p = 0.03) and RBC-SDS was observed, indicating an early stimulatory effect on RBC proliferation in these patients. However, an analysis of the RBC indices in the group of UTS patients did not reveal any significant change after both 3 and 6 months of therapy with rhGh. The increase in Hb, Ht, and RBC count observed during GH treatment confirms the in vivo erythropoietic growth-promoting effects of GH. However, this effect seems to be related only to conditions of GH deficiency. When GH deficiency is associated with multiple pituitary hormone deficiencies there are pathological influences on erythropoiesis which are not corrected until Gh treatment is started, indicating a 'permissive' role of GH in the hematopoietic system.

Growth pattern during the first 36 months of life in congenital adrenal hyperplasia (21-hydroxylase deficiency).

The longitudinal growth pattern during the first 36 months of life was studied in 24 patients (17 females) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency by analyzing the mean required daily dose of cortisone with respect to steroid suppression, height and weight growth velocities and bone age maturation. All patients were treated with cortisone acetate and 9-fluorohydrocortisone. The standard deviation score for length (SDS-L), the percentage of ideal body weight (% IBW) and biochemical parameters, 17-hydroxy-progesterone (17-OHP) and androstenedione (A) were evaluated every 3 months; bone age (BA) was evaluated annually. At diagnosis, the female population of patients with respect to the males were younger (chronological age (CA): 15 +/- 14 vs. 45 +/- 16 days, p < 0.005) and had a higher % IBW (91.7 +/- 8.0 vs. 76.3 +/- 16.7%, p < 0.05). At 3 months of age (45 days after initiating treatment) the % IBW in males normalized (97 +/- 19%) and was similar to that found in females (101 +/- 12.8%). No differences were noted in SDS-L at the moment of diagnosis (females-1.1 +/- 1.1 vs. males -0.5 +/- 0.7); however, at 3 months of age the SDS-L in females increased (0.41 +/- 0.88, p < 0.005 vs. diagnosis SDS-L) whereas that of males progressively decreased to reach the nadir at 6 months (-1.41 +/- 0.96). No differences between males and females were noted throughout this time with regard to: (a) A or 17-OHP levels (neither of which were suppressed to 'control values'); (b) the dosage of cortisone received (13.5-17.8 mg/m2/day), and (c) change in BA/CA ratio. In all patients the SDS-target height (TH) correlated with the SDS-L at 2 years (r = 0.74, p < 0.0005) and at 3 years (r = 0.60, p < 0.02) of age. In 12 patients who reached 7 years of age the SDS-L correlated with both SDS-predicted adult height (PAH) (r = 0.75, p < 0.001) and SDS-TH (r = 0.80, p < 0.005). Although the commonly accepted definition of "good control' for patients with CAH has generally included, in addition to adequate suppression of hormone markers, normal growth and skeletal maturation, the present data suggest that normal growth and BA maturation are the most useful parameters to follow and not necessarily strive for hormone suppression. Early diagnosis and replacement therapy using cortisone doses less than those currently recommended allow normal growth within the genetic potential at least for the first 7 years of life.

Anemia in infants with congenital hypothyroidism diagnosed by neonatal screening.

Although anemia is a common finding in adult hypothyroid patients, there are no studies on anemia in hypothyroid infants. The aim of this study, therefore, was to review the hematologic status during the first year of life in 50 infants with congenital hypothyroidism detected through the regional neonatal screening program. The mean age at diagnosis was 23.7 +/- 6.5 days and treatment was initially begun with a mean L-thyroxine dose of 6.8 +/- 1.3 micrograms/kg/day. Clinical and haematological assessments were performed at diagnosis, 3, 6 and 12 months of age. The patients were divided in 2 groups based on whether T4 serum concentration at diagnosis was < 3 micrograms/dl (Group A) or > or = 3 micrograms/dl (Group B). Data for hemoglobin (Hb), hematocrit (Ht), red cells count (RCC), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), serum iron and ferritin were expressed as Standard Deviation Score (SDS). Although at diagnosis the mean value of Hb-SDS, Ht-SDS and RCC-SDS were in the low-normal range in both groups, at 3 months of age the values in Group A (Hb -1.9 +/- 0.79; Ht -2.34 +/- 1.02; RCC -1.56 +/- 1.25) were significantly lower than in Group B (Hb -1.14 +/- 0.78, p < 0.005; Ht -1.59 +/- 0.94, p < 0.05; RCC -0.55 +/- 1.32, p < 0.02). A rise of the Hb, Ht and RCC values was observed in both groups from 6 to 12 months. The mean values of MCV-SDS and MCH-SDS were in the normal range at diagnosis in both groups, decrease progressively at 3 and 6 months and returned to normal at 12 months of age; no differences were found between the 2 groups at any time. Mean Hb levels at 3 months of age were correlated with mean serum T4 at diagnosis (r = 0.30, p < 0.05). The present results indicate that anemia is a frequent finding in infants with congenital hypothyroidism and is depended on the degree of neonatal hypothyroidism and imply that hypothyroidism during development may produce persisting changes even after thyroid replacement has begun.

[Variable expression of non insulin dependent diabetes mellitus in childhood]. / Variabilità di espressione del diabete mellito non insulino dipendente in età pediatrica.

Non Insulin Dependent Diabetes Mellitus (NIDDM) in pediatric age remains not well defined as a nosological entity. The so-called Maturity Onset Diabetes of the Young (MODY), for example, recognizes three different etiologies. Three cases are presented as an example of different possibilities of onset and evolution in pediatric age: a 12.5 year old girl presents a positive familial history of NIDDM, a slow onset of the disease and goes on well with administration of an oral hypoglycemic drug; a 12.5 year old girl too, with a positive familial history of NIDDM, with acute onset of the disease, needed an oral hypoglycemic drug when she was overweight. Having lost the ponderal excess, she goes on very well with the diet alone; a 13 year old girl without any familial history of NIDDM, who presented a dramatic onset in occasion of an intercurrent illness; she required insulin therapy only during a steroidal therapy period, administered for ovaric polycystosis. The authors argue over the age of onset, the familial history and the therapeutic approach to NIDDM in pediatric age.

Diabetes mellitus in Kearns-Sayre syndrome: a case with a 10-year follow-up.

An 11-year-old girl with Kearns-Sayre Syndrome developed diabetes mellitus with ketoacidosis at onset and immediate insulin-requirement. In a 10-year follow-up, while neuromuscular disease was progressively increasing, diabetes was well controlled by once-a-day insulin therapy. Insulin secretion appears low, but without impairment during the years. This case points out the diabetes' features in Kearns-Sayre syndrome.

Prognostic factors in the intellectual development at 7 years of age in children with congenital hypothyroidism.

The influence of the initial L-T4 dose and some other factors on the intellectual development was evaluated at 7 years of age in 47 congenitally hypothyroid children detected by the regional screening program. All patients were initially treated with 25 micrograms LT4/kg/day that represented a mean replacement dose of 6.8 +/- 1.3 micrograms/kg. Despite the "low" initial dose mean IQ at 7 years resulted within normal range (96 +/- 9). Twenty-eight patients initially treated with 6.0 +/- 0.6 micrograms L-T4/kg/day had a mean IQ (96 +/- 9) which was not different with respect to 19 patients (IQ 94 +/- 7) treated with a significantly higher L-T4 dose (8.1 +/- 0.9 micrograms/kg/day; p < 0.0001). The initial L-T4, dose did not correlate with IQ at 7 years whereas a significant correlation was found between IQ and serum T4 concentration at diagnosis (r = 0.35; p < 0.01) regardless of the fact that serum T4 concentration normalized after the first 2 months of therapy in both groups. Twenty-three patients whose serum T4 at diagnosis was < 2 micrograms/dl (1.0 +/- 0.5) had a mean IQ at 7 years (92 +/- 9) which was significantly lower than the 24 patients (IQ 98 +/- 7; p < 0.02) whose serum T4 was > 2 micrograms/dl (5.7 +/- 2.4; p < 0.001). The present findings suggest that the severity of neonatal hypothyroidism is an important factor in determining subsequent intellectual development of congenitally hypothyroid children.

[Turner syndrome and insulin-dependent diabetes mellitus]. / Syndrome de Turner et diabète insulinodépendant.

BACKGROUND: Cases of diabetes mellitus associated with Turner syndrome are noninsulin-dependent. We report a case of insulin-dependent diabetes mellitus (IDDM) with Turner syndrome. CASE REPORT: An 11 year-old girl with Turner syndrome was investigated for glucose tolerance that was normal with glycosylated hemoglobin at 5.1%. The patient was then given growth hormone plus oxandrolone until the age of 15 yr 3 mo; estrogen replacement was initiated at 15 yr 9 mo and progesterone 6 months later. At the age of 16.5 yrs, the patient developed manifestations of diabetes mellitus with polyuria, polydipsia, weight loss. Blood glucose concentration was 415 mg/dl with ketoacidosis. She was given insulin; at that time, blood insulin level was 7 mU/ml, C-peptide 0.8 ng/ml and glycosylated hemoglobin Alc 8.2%. Islet cell auto-antibodies were positive (1/80) and histocompatibility antigens were A1, A2, B14, B18, CW7, DR7, DR11, DQ7. After 6 months of treatment, blood insulin and C-peptide after IV glucagon were nil. There was no familial case of IDDM. CONCLUSIONS: This case of IDDM seems to be the first reported in Turner syndrome. This IDDM could be a manifestation of autoimmunity.