PubMed Health⌕ Search

Biomedical subjects

F Schaefer

Publications and source records attributed to F Schaefer.

At least 73 records · Page 4Linked to original sources
PubMed1996

Growth charts for prepubertal children with chronic renal failure due to congenital renal disorders. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

Despite the high prevalence of and therapeutic attention to growth failure in children with chronic renal failure (CRF), systematic evaluations of spontaneous growth in CRF are lacking. Therefore, we collected retrospectively longitudinal growth and biochemical data in 321 prepubertal patients treated for CRF due to congenital renal disorders. Data were recorded at 3-month intervals during the first 2 years of life and 6-monthly thereafter, up to the age of 10 years. Around 100 measurements were available per age interval. Mixed-longitudinal percentile curves of height and height velocity were constructed. Moreover, a statistical comparison with the heights and height velocities of healthy children and an evaluation of the effect of biochemical parameters on growth was performed. The CRF children had normal heights at birth but dropped below the 3rd normal percentile during the first 15 months of life. Thereafter, growth patterns usually were percentile parallel, with a mean height standard deviation score (SDS) of -2.37 +/- 1.6. Height velocities were consistently lower in patients with glomerular filtration rates (GFRs) below one-third of the lower normal limit (25 ml/min per 1.73 m2 for patients > 1 year) than in patients with better renal function. This difference in growth rates resulted in a mean height SDS of -1.65 +/- 1.5 SDS and -2.79 +/- 1.4 SDS (age 1-10 years) in the subgroups with relatively better and worse GFR, respectively. Regression analysis confirmed that GFR was a weak but significant predictor of height velocity SDS in most age groups.

Child↗
PubMed1996

Short dialyzed children respond less to growth hormone than patients prior to dialysis. German Study Group for Growth Hormone Treatment in Chronic Renal Failure.

Recombinant human growth hormone (rhGH) is a new treatment modality for short children with chronic renal failure (CRF) prior to and during dialysis. It is difficult to analyze whether dialysis patients respond less to rhGH than children with CRF on conservative treatment because they are older and often in a pubertal age range. One hundred and eight patients were treated with 28-30 IU rhGH/kg per week for at least 1 year. We analyzed the growth response to rhGH in 56 prepubertal patients aged less than 10 years at the start of rhGH treatment; 38 children with a mean age of 6.5 +/- 2.4 years were on conservative treatment (CT) and 18 patients with a mean age of 6.5 +/- 2 years on dialysis treatment (D). Mean height velocity was 4.9 +/- 2.3 cm/year in children on CT and 4.6 +/- 1.8 cm/year in children on D. During the 1st treatment year, height velocity was 9.5 +/- 3.8 cm/year in CT patients and 7.3 +/- 1.3 cm/year in D patients (P < 0.05). The change in height was +1.1 +/- 0.8 standard deviation (SD) in CT patients and +0.5 +/- 0.4 SD in D patients (P < 0.005). During the 2nd treatment year, the change in height was again greater in CT patients (0.5 +/- 0.4 SD vs. 0.2 +/- 0.4 SD; P < 0.05). The difference in height velocity and change in height standard deviation score was also significant when a subgroup of patients was matched for sex, age, height. Height velocity and the change in height velocity during rhGH treatment were not correlated with residual renal function, the degree of anemia, or metabolic acidosis. We conclude that short children on D respond less to rhGH than short children on CT, indicating a greater insensitivity to rhGH during D treatment.

Acidosis↗
PubMed1996

Bilateral renal venous thrombosis in a neonate associated with resistance to activated protein C.

Renal venous thrombosis (RVT) is a serious complication of neonates. In most cases the underlying cause of RVT remains unclear. Here we report a neonate with bilateral RVT and adrenal haemorrhage associated with a heterozygous mutation of the gene encoding for clotting factor V, resulting in resistance to activated protein C. Vigorous thrombolytic therapy with urokinase followed by recombinant tissue plasminogen activator dissolved the thrombus in the inferior vena cava and restored perfusion of both kidneys. However, a haemorrhagic rupture of the right kidney occurred, requiring emergency nephrectomy. Despite reperfusion of the left kidney and resumption of urine output, the patient remained dialysis dependent. Due to persistent adrenal insufficiency, long-term substitution of hydrocortisone was necessary. The patient was prophylactically treated with coumarin during the first 6 months of life and is now waiting for renal transplant at the age of 1 year.

Anticoagulants↗
PubMed1996

Influence of dialysis on plasma lipid peroxidation products and antioxidant levels.

In patients with end-stage renal failure (ESRF), the incidence of atherosclerosis and cancer is increased. The importance of lipid peroxidation (LPO) products in the pathogenesis of these complications has recently been emphasized. The LPO products malondialdehyde (MDA) and hexanal, lipophilic antioxidants and erythrocyte glutathione (GSH) were estimated in 10 pediatric hemodialysis (HD) patients before and after HD and in 11 peritoneal dialysis (CPD) patients. Before HD, MDA was elevated [median (interquartile range): 384.5 (110 to 501) nM; normal < 150 nM], whereas plasma hexanal levels were normal in all patients [130.5 (88 to 222) nM; < 320 nM]. HD decreased MDA concentrations on average by 88% but did not change hexanal levels. CPD patients exhibited high plasma MDA concentrations [371 (287 to 468) nM], whereas hexanal was in the low normal range [56 (51 to 81) nM]. Antioxidants were normal in both groups and unchanged during HD. GSH decreased slightly during HD. We hypothesize that MDA may accumulate in ESRF due to reduced plasma clearance. Our results argue against a general increase of LPO in uremia.

Adolescent↗
PubMed1996

Physiological changes during computer tasks: responses to mental load or to motor demands?

Cardiac and electrodermal measures are regarded as indicators of user strain during computer work, not taking the possible influence of finger and hand movements on these measures into account. For the evaluation of such effects, motor demands and mental load were both varied as independent factors in two experiments. As a motor task, subjects had to produce compensatory keystrokes at different speed levels to keep a moving mark within a target area on the computer screen. Mental load was varied in experiment 1 by manipulation of keystroke synchronization accuracy, and in experiment 2 by manipulation of memory load. Physiological measures were affected by motor activity only at a very high typing speed. At lower levels of motor activity physiological effects were dependent exclusively on mental load. Frequency of skin conductance responses was the most sensitive indicator of the emotional consequences of mental load and mean heart period reflected its attentional aspects. The study supports the assumption that physiological effects of motor activity can be neglected during typical computer tasks, demanding keystrokes at intervals of 300 ms or longer.

Adult↗
PubMed1996

Growth hormone as a new treatment modality for short children with chronic renal failure. The German Study Group for Growth Hormone Treatment in Chronic Renal Failure.

Recombinant human growth hormone (rhGH) has become a new treatment modality for short children with chronic renal failure (CRF) and after renal transplantation. The rationale for high-dose rhGH treatment is the insensitivity of the uremic organism to GH. As the insensitivity to GH is expressed more in end-stage renal failure than in earlier stages of CRF, patients on dialysis respond less to rhGH. In transplanted children, rhGH can counterbalance the growth-depressing effects of corticosteroids. In prepubertal children, rhGH improves the height standard deviation score by a mean of +2 within 5 years. The effect of rhGH treatment on final height remains to be studied.

Child↗
PubMed1996

Multifactorial control of the elimination kinetics of unbound (free) growth hormone (GH) in the human: regulation by age, adiposity, renal function, and steady state concentrations of GH in plasma.

To evaluate the principal determinants of the MCR and plasma t1/2 of unbound (free) GH in man, we performed steady state infusions of 3 doses of recombinant human GH during pharmacological suppression (iv octreotide) of endogenous GH secretion in 24 healthy adults and 12 patients (6 adults and 6 children) with chronic renal failure (CRF). Free plasma GH was calculated from total plasma GH (measured by immunoradiometric assay) and GH-binding protein activity (radioligand assay). The MCR of free GH was determined from free plasma GH and the rate of recombinant human GH infusion. The t1/2 of free plasma GH, and the concentration and the in vivo dissociation constant (Kd) of GH-binding protein (GHBP) were estimated by dynamic modeling of the postinfusion total plasma GH concentration decay curves. The MCR of free GH decreased and the plasma GH t1/2 increased significantly with increasing plasma GH concentrations. The MCR of free GH over its physiological concentration range was positively correlated with the body mass index as a measure of relative obesity and negatively related to age, but only at supraphysiological GH concentrations. In the adult patients with CRF, the MCR of free GH was decreased at each infusion rate by 25-38%, and the t1/2 was increased by 80-170%. Children with CRF showed a significantly lower MCR and higher t1/2 of plasma free GH than adult patients. Modeling and direct measurements of the off-rate of GH from its high affinity GHBP indicated normal dissociation rate constants but decreased molar concentrations of the GHBP in uremic plasma. We conclude that the rate of elimination of free GH from plasma in man is controlled by 1) plasma total free GH concentrations, 2) relative obesity, and 3) renal function within the physiological GH concentration range, whereas 4) age is a negative predictor of MCR only at supraphysiological GH concentrations.

Adolescent↗
PubMed1996

Control of pulsatile and tonic parathyroid hormone secretion by ionized calcium.

To investigate the effect of changes in ionized calcium on instantaneous PTH secretion, we examined seven young healthy volunteers by 1-min blood sampling under conditions of normo-, hypo-, and hypercalcemia. After a baseline period of 75 min, ambient ionized calcium was either increased or decreased by 0.2 mmol/L for 105 min by clamped infusion of calcium gluconate or sodium citrate. The characteristics of PTH secretion were analyzed by a deconvolution technique, accounting for subject-specific plasma PTH disappearance half-life, as measured during the first 15 min of calcium infusion (range, 2.04-2.93 min). The process regularity of pulsatile PTH secretion was evaluated by an approximate entropy statistic. Under baseline conditions, 32% of total PTH secretion was released in a pulsatile fashion, with a burst frequency of 6.9 +/- 0.8 h-1 and a PTH mass per burst of 2.6 +/- 0.9 pmol/L. The remaining 68% of total secretion was attributed to tonic hormone release. During the initial 30 min of induced hypocalcemia, pulsatile secretion increased by 1140%, whereas tonic secretion did not change. The preferential increase in pulsatile PTH secretion was mediated by a combined rise in burst frequency and mass released per burst. During subsequent steady state hypocalcemia, the tonic secretion rate increased (255% of baseline), whereas burst frequency and burst mass decreased (to 103% and 189% of the baseline values), restoring the baseline ratio of tonic to pulsatile PTH secretion. The regularity of PTH release increased during steady state hypocalcemia. During hypercalcemia, tonic secretion, burst mass, and burst frequency decreased by 75%, 82%, and 32%, respectively, and remained constant throughout the clamp period. We conclude that acute hypocalcemia elicits an immediate pulsatile and a delayed tonic secretory response of the parathyroid gland with increased regularity of PTH release. Acute hypercalcemia suppresses both the pulsatile and the tonic component of PTH secretion.

Adult↗
PubMed1996

Assessment of total body water in paediatric patients on dialysis.

BACKGROUND: Various anthropometric techniques are used to assess total body water in children on dialysis; however, their predictive accuracy and precision has not been validated. METHODS: We compared total body water measurements obtained by deuterium oxide (D2O) dilution with predictions of total body water from (1) height and weight, (2) skinfold measurements, and (3) bioelectrical impedance analysis, using previously published formulae for healthy children. Measurements were performed in 14 patients on peritoneal and in nine patients on haemodialysis, aged 4-22 years. RESULTS: In the total population of dialysed patients, weight was the strongest single predictor of total body water (R2 = 0.93), followed by the resistance index (RI = height2/impedance; R2 = 0.85) and height (R2 = 0.93). A prediction formula based on height and weight predicted total body water with a residual mean square error (RMSE) of 1.97 1 (coefficient of variation (CV) = 10.0%) and with a systematic overestimation of true total body water by 0.4%. A prediction equation based on skinfold measurements yielded a total body water estimate with an RMSE of 2.15 1 (CV = 10.5%) and overpredicted true total body water by an average of 2.2%. Using three published prediction equations incorporating RI, RMSEs of 2.78 1 (CV = 14.1%) with a mean under- or overestimation of true total body water by 6.9, 7.1, and 0.8% respectively, were achieved. The prediction of total body water was optimized by linear combinations of RI or the log-transformed sum of four skinfolds (logsum) with weight by the following equations: total body water (1) = 9.97 - 3.13 x logsum (1) +0.59 x weight (kg) (R2 = 0.951; RMSE = 1.67 1; CV = 8.17%). total body water (1) = 1.99 + 0.144 x RI (Ohm/cm2) (2) + 0.40 x weight (kg) (R2 = 0.949; RMSE = 1.67 1; CV = 8.53%). The fit of these prediction formulae, which were derived from the total population, did not differ significantly between haemo- and peritoneal dialysis patients or between boys and girls. CONCLUSIONS: Both skinfold measurements and bioelectrical impedance analysis can be used to improve the height- and weight-based prediction of total body water in children on dialysis.

Adolescent↗
PubMed1996

Effect of growth hormone treatment on pubertal growth in a boy with cystinosis and growth failure after renal transplantation.

Recombinant human growth hormone (rhGH) has proven effective in improving growth in short prepubertal children with chronic renal failure (CRF) before and after renal transplantation. However, its effect in pubertal patients is still doubtful. We report the case of a boy with nephropathic cystinosis and persistent growth failure despite successful renal transplantation who was treated with rhGH (30 i.u./m2 body surface area/week sc) from early puberty up to final height.

Child↗
PubMed1996

Pathogenic mechanisms underlying the variable response to recombinant human growth hormone in children with chronic renal failure.

As with spontaneous growth, the height gain achieved by stimulation with recombinant human growth hormone (rhGH) in children with chronic renal failure appears to be inversely related to the degree of renal dysfunction. The secondary complications of CRF that may independently interfere with growth, such as metabolic acidosis, anaemia, and secondary hyperparathyroidism, do not explain the poorer response to rhGH in children with end-stage renal failure. Certain abnormalities of the somatotropic hormone axis, the severity of which is related to the degree of renal dysfunction, are more likely to explain the dependence of the rhGH treatment response on glomerular filtration rate. These derangements include partial GH receptor deficiency, increased plasma binding of IGF-I, and accumulation of non-competitive inhibitors of IGF-I action. Strategies to optimise growth-promoting treatment in end-stage renal failure will depend on the relative contribution of these different somatotropic hormone axis alterations to uraemia.

Glomerular Filtration Rate↗
PubMed1995

Disproportionate growth following long-term growth hormone treatment in short children with X-linked hypophosphataemia.

UNLABELLED: Three short prepubertal children with X-linked hypophosphataemia were treated with 1 IU recombinant human growth hormone (rhGH)/kg per week sc in addition to calcitriol and phosphate supplementation over a period of 3 years. Improvement of height standard deviation score (SDS) ranged from 1.0-1.7 SD based on an increase in sitting height of 1.5-2.9 SD, whereas subischial leg length improved only slightly by 0.3-0.9 SD. In all three patients, renal phosphate threshold concentration increased slightly and transient hyperparathyroidism was noted. CONCLUSION: Treatment of stunted children with X-linked hypophosphataemia is effective in improving growth velocity, but appears to aggravate the pre-existent disproportionate stature of such children.

Anthropometry↗
PubMed1995

Treatment of peritoneal dialysis-associated peritonitis with continuous versus intermittent vancomycin/teicoplanin and ceftazidime in children: preliminary results of a prospective randomized trial. Members of APN Arbeitsgemeinschaft Paidiatrische Nephrologie.

A recent treatment update for continuous peritoneal dialysis (CPD)-associated peritonitis recommends first-line use of continuously or intermittently administered intraperitoneal vancomycin and ceftazidine. Teicoplanin has recently been introduced as a potentially less toxic alternative glycopeptide antibiotic. However, efficacy and safety have not been demonstrated for intermittent treatment schedules or for the use of teicoplanin in prospective studies. Therefore, a prospective trial on the treatment of CPD-associated peritonitis was started in 15 pediatric dialysis units using vancomycin or teicoplanin, in combination with ceftazidine. Vancomycin or teicoplanin was administered either continuously with each bag of dialysate for 10 days or as a single dose on days 1 and 8, and ceftazidime either continuously or in one bag of dialysate per day. Until December 31, 1994, 81 episodes of peritonitis including 16 relapses occurred in a cohort of 120 patients. The incidence of peritonitis was 1 episode/13.7 months, regardless of treatment modality [continuous ambulatory peritoneal dialysis (CAPD) or continuous cycling peritoneal dialysis (CCPD)]. Thirty-six (57%) primary peritonitis episodes were caused by staphylococci (21 Staphylococcus aureus, 15 coagulase-negative), 12 (19%) by gram-negative bacteria, 7 by other germs, and in 8 (13%) cases cultures remained sterile. Primary treatment response was achieved in 43/46 (93%) gram-positive and 4/12 (33%) gram-negative peritonitis episodes. Relapses occurred only with gram-positive bacteria (16/63, 25%). In cases of gram-positive peritonitis, no differences in primary response (25/25 vs 15/18) or relapse rates (10/36 vs 6/27) were observed between groups on continuous and intermittent treatment, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Bacterial Agents↗
PubMed1994

Residual renal function in children on haemodialysis and peritoneal dialysis therapy.

Residual renal function was studied in 28 haemodialysis (HD) and 31 peritoneal dialysis (PD) patients aged 1-20 years observed over 6-43 (median 19) months. After the start of dialysis urine volume (UV) decreased to 57%, 46% and 26% of initial mean values in HD patients after 6, 12 and 24 months, respectively. In PD patients the corresponding figures were 57%, 69% and 62%. Mean UV calculated from all individual mean UV measurements observed was higher in PD than HD patients (954 vs. 537 ml/m2 per 24 h, P < 0.01). A better conservation of diuresis in PD patients was also suggested by a significantly longer persistence of a UV greater than 500 ml/m2 per 24 h compared with HD patients. Cox proportional hazard analysis identified dialysis modality and pre-dialysis UV of less than 1,000 ml/m2 per 24 h as the only significant risk factors for UV survival. However, the decline of UV per time was similar in both modes of treatment. No significant changes of glomerular filtration rate were observed during both HD and PD treatment.

Adolescent↗
PubMed1994

Immunoreactive and bioactive luteinizing hormone in pubertal patients with chronic renal failure. Cooperative Study Group on Pubertal Development in Chronic Renal Failure.

Disturbed pulsatile LH secretion has been suggested to play a role in the etiology of delayed puberty and disturbed reproductive function in chronic renal failure (CRF), but interpretation of gonadotropin secretion from plasma concentration measurements is confounded by alterations in hormone metabolic clearance. To simultaneously investigate LH secretion and clearance in children, we performed multiple-parameter deconvolution analysis of 11-hour over-night serum LH concentration-time series of bioactive (bio-LH) and immunoreactive (i-LH) hormone in 36 pubertal patients (18 boys) with various degrees of CRF and 10 healthy controls matched for sex and pubertal stage. Twelve patients received conservative treatment for advanced but compensated CRF, 12 were treated by dialysis, and 12 were studied after successful renal transplantation. We observed that: (1) the mean (+/- SE) plasma half-lives of bio-LH and i-LH were increased in the dialysis group (155 +/- 47 and 201 +/- 31 min) and in the patients on conservative treatment (148 +/- 45 and 135 +/- 70 min) compared to controls (59 +/- 28 and 63 +/- 21 min; all P < 0.05). The plasma half-life of bio-LH in patients on conservative treatment or after renal transplantation was inversely correlated with glomerular filtration rate (GFR) (r = -0.70; P < 0.0001). (2) Pulsatile bio-LH production rate was independently affected by pubertal stage (P = 0.018) and treatment status (P = 0.017), increasing across pubertal stages and being significantly lower in dialysis patients (20 +/- 4 IU/liter * 11 hr) and patients on conservative treatment (28 +/- 9) than in controls (43 +/- 9; all P < 0.05). In patients on conservative treatment or after transplantation, a significant positive correlation between pulsatile bio-LH production rate was observed (r = 0.53; P < 0.008). Pulsatile i-LH secretion rate was significantly reduced only in dialysis patients (15 +/- 34 vs. 46 +/- 18; P < 0.05). (3) The reduction of pulsatile i-LH and/or bio-LH production rates was attributable to a halving of the LH mass secreted per burst in patients on conservative (bio-LH: 4.9 +/- 1.9 IU/liter) and dialysis treatment (bio-LH: 3.2 +/- 0.7, i-LH: 2.4 +/- 0.6 IU/liter) versus controls (bio-LH: 6.9 +/- 1.3, i-LH: 5.4 +/- 2.1 IU/liter), whereas the LH pulse frequency was not different between controls and treatment groups.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1994

Less acidic forms of luteinizing hormone are associated with lower testosterone secretion in men on haemodialysis treatment.

OBJECTIVE: Men with chronic renal failure treated by haemodialysis have raised levels of bioactive LH (B-LH) and immunoreactive LH (I-LH) but reduced B-LH:I-LH (B:I) ratio and testosterone (T) secretion. This study investigated the LH isoform distribution in serum from normal adult males and males on regular haemodialysis treatment. DESIGN: Four blood samples (2 ml) were obtained at 15-minute intervals from a group of men on regular haemodialysis treatment. These samples were part of a larger pulse profile series and showed no evidence of LH pulsatility. The serum was pooled for each individual patient. Blood (10 ml) was also drawn randomly from healthy male volunteers. The sera were chromatofocused on a 4-ml mono-P column attached to a fast performance liquid chromatography system. This procedure separates the LH isoforms according to their isoelectric point. The pH gradient was between pH 7 and pH 4. PATIENTS: The five men with chronic renal failure were aged between 18 and 40 years and had been on haemodialysis for a mean of 10 months (5-20). They were sampled the night prior to a dialysis session. The five normal healthy volunteers had never had any endocrine disorder diagnosed. MEASUREMENTS: An immunoradiometric assay and a commercially available (Delfia) immunofluorimetric assay were employed for detection of LH in the sera and in chromatofocusing fractions. B-LH and testosterone were also measured in the sera. RESULTS: Hormone data (mean +/- SEM for normal and renal subjects respectively) were 15.5 IU/l +/- 1.2 and 26.9 +/- 7.2 (B-LH), 6.0 IU/l +/- 0.3 and 16.5 +/- 4.8 (irmaLH), 5.7 +/- 0.5 and 13.6 +/- 4.8 (fluorLH), 25.2nmol/l +/- 2.0 and 12.1 +/- 1.2 (T). The serum B:I ratios were 2.6 +/- 0.1 and 2.6 +/- 0.2 (controls, irmaLH and fluorLH respectively) and 1.7 +/- 0.1 and 2.1 +/- 0.1 (chronic renal failure group). Recovery of LH from the column was 111 +/- 12% (mean +/- SEM) by IRMA and 104 +/- 7% by IFMA for the ten FPLC runs. The median pI for the LH distribution measured by both assays was in the region 6.54-6.40 for subjects with chronic renal failure and 6.09-5.95 for controls. Median pI was negatively correlated to the B-LH:irmaLH (P < 0.0001) and B-LH:fluorLH ratios (P = 0.002) in the serum. Furthermore, the proportion of isoforms recovered in the pH region 6.25-5.50 increased with increasing T levels in the serum (P < 0.004). CONCLUSION: The distribution of LH in serum of men on haemodialysis is more basic than in normal men. The greater the proportion of more acidic LH species, particularly those with a pI of between 5.50-6.25, the higher the ratio of LH bioactivity to immunoactivity and consequently testosterone levels.

Adolescent↗
PubMed1994

Growth and skeletal maturation in children with phenylketonuria.

Growth and skeletal maturation was evaluated in 82 children participating in the German Collaborative Study of Children Treated for Phenylketonuria (PKU). Height, weight, head circumference and bone age were recorded at regular intervals for the first 6 years of life. The mean SD score (SDS) for height was not significantly different from zero at study entry, but decreased mainly during the second year of life to a nadir of -0.78 in boys and -0.54 in girls at 2.5 years. During the subsequent years, a significant trend towards a regain of height SDS was noted in both sexes. Weight-for-height SDS was close to zero in both sexes, with a significant continuous increasing trend throughout the observation period. Head circumference SDS decreased in boys during the first year of life from -0.28 to -0.68, whereas girls showed only a minor change. During the further follow-up period, head circumference SDS remained at approximately -0.3 in boys and 0.0 in girls. While the mean verbal and performance IQ of the total study population at 5 and 6 years of age did not differ from a group of 212 healthy non-PKU children, patients with a head circumference SDS less than the population median at 2 years of age exhibited poorer cognitive abilities at school age than those patients with a relative head size greater than the population median. The children with a head circumference less than the median at 2 years had smaller head sizes already at birth; in addition, the change in relative head size during the first 2 years was correlated significantly with cognitive abilities at school age in boys. Mean bone age was identical to chronological age at each time point of observation. The rate of maturation was one year of bone age per year of chronological age. No correlation between phenylalanine intake or phenylalanine concentrations and the rates of body or head growth or skeletal maturation could be established. We conclude that despite adequate weight gain, moderate growth retardation occurred during the first 2 years of life in this group of children treated for PKU. Growth was more compromised in boys than in girls and tended to be compensated during later follow-up. Early infantile head circumference and growth appear to be predictors of cognitive development.

Age Determination by Skeleton↗