Effects of a gluten-free diet on catch-up growth and height prognosis in coeliac children with growth retardation recognized after the age of 5 years.

The effects of a gluten-free diet on catch-up growth and predicted height were evaluated in 12 children with coeliac disease diagnosed after the age of 5 years and followed for 2-5.5 years. In the majority of the patients, height and bone age were retarded at the time of diagnosis. Under a gluten-free diet growth velocity, age-related height, predicted height and relative bone age increased, height for bone age slightly decreased. In four patients the predicted height remained below the target height, indicating incomplete catch-up growth.

Adult height in thalassaemia major without hormonal treatment.

Height and sitting height were measured in all the 28 patients (19 females and 9 males) of our Thalassaemic Centre who exhibited evidence of cessation of growth. Their average final stature, which had been spontaneously achieved at an age ranging from 15.0-21.5 years (mean 17.9), was slightly lower with respect to the mean standards for women (159.1 +/- 8.4 vs 160.0 +/- 6.0 cm) and men (167.8 +/- 6.4 vs 172.5 +/- 6.0 cm). Height deficiency exceeded 2 SD scores in only 3/28 patients. If compared to the familial target height, patients' stature fell within the familial range in 19/24 cases and in only one case was it below the lower limit. In the whole sample the patients' height was positively related to their familial target height (r = 0.72, P less than 0.0002) and also to mid-parental stature (r = 0.45, P less than 0.05). Heights of both females and males were superimposable on those of their own mothers and fathers respectively. Average values of either SH (76.4 +/- 3.8 vs 79.9 +/- 3.4 cm, 2 P less than 0.02) or SH/H ratio (48.9 +/- 2.5 vs 51.5 +/- 1.0, 2 P less than 0.005) were significantly lower in the female thalassaemics than in the sex-matched controls. Three females and one male showed subnormal values of both SH and SH/H ratio. Two out of these patients with eunochoid body proportions and another one with a decreased SH/H ratio have hypogonadotropic hypogonadism and are undergoing a chronic substitutive treatment with sexual hormones, which was instituted after the achievement of final stature.

Dissociation between adrenarche and gonadarche in two longstanding hypothyroid youngsters.

An evident dissociation between adrenarche and gonadarche was found in two longstanding hypothyroid youngsters, one male and one female, with advanced gonadal development and absent sexual hair. This discrepancy rapidly vanished after the institution of thyroxine substitutive treatment. These case reports underline the important relationships existing between thyroid function and endocrine systems involved in the regulation of adrenarche and gonadarche.

Effects of intravenous TRH on growth hormone and cortisol serum levels in children and adolescents with insulin dependent diabetes mellitus.

The effects of an iv thyrotropin releasing hormone (TRH) bolus on serum growth hormone (GH) and cortisol levels were evaluated in 59 children and adolescents with insulin dependent diabetes mellitus (IDDM) and in 24 healthy, age-matched control subjects. In the IDDM group GH baseline levels sharply rose within 30 min after TRH and successively normalized. On the contrary, TRH injection failed to affect GH serum concentrations in the control group. The GH increase after TRH in IDDM patients was positively correlated to age, but unrelated to other variables, such as sex, pubertal stage, duration of disease, glycemia, glycosylated hemoglobin, thyrotropin and T4 concentrations. Twenty-one out of 59 diabetics and only 1/24 controls exhibited a paradoxical GH response to TRH, arbitrarily defined as a precocious increase (within 30 min), of more than 100% with respect to the baseline value, associated with a GH peak greater than 10 ng/ml. Eighteen IDDM patients underwent a second TRH test 12 to 24 months later and substantially exhibited the same GH pattern documented the first time. The mechanism responsible for such anomalous GH responsiveness to TRH in IDDM is unclear. However, it cannot be attributed to a nonspecific stress reaction, as proven by the lack of a concomitant increase of cortisol serum levels in the same subjects.

Changes in thyroid function tests induced by 2 month carbamazepine treatment in L-thyroxine-substituted hypothyroid children.

In five L-thyroxine-substituted hypothyroid children with partial epilepsy serum total thyroxine (T4) and free T4 (FT4) significantly (P less than 0.01) decreased following 2 months of carbamazepine (CBZ) administration (20 mg/kg per BW per day) from mean (+/- SD) values of 12.7 +/- 1.1 micrograms/dl and 15.5 +/- 1.8 pg/ml to mean values of 7.5 +/- 2.3 and 10.1 +/- 1.7, respectively. In all but one patient important changes in both serum total and free triiodothyronine (T3, FT3) were not observed; consequently T3:T4 and FT3:FT4 ratios significantly (P less than 0.05) increased in the whole series. Three subjects had post-treatment serum TSH that rose to hypothyroid levels parallel to a T4 decrease. The negligible thyroid hormone secretion and the unmodified T3-uptake (T3U) or T4-binding globulin (TBG) exclude direct effects of CBZ on thyroid gland and on carrier serum proteins, respectively. The findings observed, instead, might be due to accelerated T4 metabolic clearance together with augmented T4 to T3 conversion rate, as previously demonstrated for diphenylhydantoin. The sharp reduction in T4 and FT3 concentrations is the peripheral display of this event, which is associated with a decompensation of the metabolic status, as indicated by serum TSH enhancement. In all cases a supplement of L-thyroxine by itself was able to restore euthyroid TSH serum concentrations, suggesting that hypothyroidism in patients with partial epilepsy to whom CBZ had been administered requires a higher L-T4 substitutive regimen.

[Cholestatic hepatopathy in a child with congenital hypopituitarism]. / Hépatopathie cholestatique chez un enfant avec hypopituitarisme congénital.

The present case report concerns a male infant affected by congenital hypopituitarism presenting with neonatal hypoglycaemic attacks, microphallus and bilateral cryptorchidism. A transient cholestasis observed during the 2nd month of life and reversed by hydrocortisone treatment, is to be considered, in Authors' opinion, in the context of the endocrine syndrome, probably as consequence of the adrenal failure.

True precocious puberty: a long-term complication in children with shunted non-tumoral hydrocephalus.

In 4/37 (10.8%) children, adolescents and young adults with successfully shunted hydrocephalus, puberty occurred or was occurring precociously, at an age ranging from 7.5 to 8.6 years, with a consequent impairment of their effective or predicted adult height as compared to the familial target height. All four patients had undergone a surgical intervention for the insertion of a ventricular-atrial or a ventricular-peritoneal shunt during the first year of life; since the last surgical shunt revision (at the age of 5 years) no relapse of hydrocephalus had been recorded. The authors conclude that precocious puberty is to be regarded as a not infrequent long-term complication in patients with successfully shunted hydrocephalus.