Patients (pts) with IDDM can develop delayed gastric emptying (GE) that produces dyspeptic symptoms and adversely affects glucose control. In 40 IDDM pts (age range: 6-19 yrs; median: 9 yrs), without autonomic nerve dysfunction at specific tests, GE time (minutes) of a mixed solid-liquid meal and gastric electrical activity were measured by antral ultrasound and electrogastrography (EGG), respectively; 15 healthy children (age range 4-15 yrs) served as controls. EGG was performed in the fasting and fed periods by placing two pairs of Ag-AgCl electrodes on the epigastric skin area; signals were analyzed by the fast Fourier transformation. The following EGG variables were measured:% of dysrhythmias, fed/fasting ratio of the dominant power (PR). A dominant frequency of > 0.5 and <2 cpm was defined as bradygastria and > 4 and ≤ 9 cpm as tachygastria, with absence of normal gastric rhythm (2-4 cpm), and for at least 2 minutes. Blood glucose (mg/dl) was measured at the start of the meal (T0) and 180 minutes later (T180); Hb 1Ac was also determined. Results: GE time (median and ranges) was significantly delayed in 26 pts (Group I) (255; 195-285), whereas in 24 (Group II) (165; 135-180) it was in the range of control values (150; 110-180). Variables (mean ±DS) are reported as follows: Table Conclusions: delay of GE and gastric electrical dysrhythmias are detected in a high proportion of IDDM children and can contribute to poor glycemic control, most likely, by causing a mismatch between the onset of insulin action and the delivery of nutrients into the small intestine. Children with IDDM and unexplained poor glycemic control should be screened for abnormalities in gastric motility.
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 ± 8.0vs.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-F at the moment of diagnosis (females -1.1 ± 1.1 vs. males -0.5 ± 0.7); however, at 3 months of age the SDS-F in females increased (0.41 ± 0.88, p < 0.005 vs. diagnosis SDS-F) 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-F 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-F 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.
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.
