Endocrinologic Consequences of Pediatric Posterior Fossa Tumours.

Intracranial tumors are the second most frequent malignancies in children and posterior fossa is a common location for these neoplasias during childhood. Recent advances in surgical techniques, radiotherapy and chemotherapy resulted in dramatic increase in the survival rates of these children, however they are still source of a significant morbidity and mortality. Endocrinological complications and late sequelae of childhood posterior fossa tumours are common among the survivors of these tumours and include growth retardation, hypothyroidism, pubertal disorders, gonadal dysfunction and osteopenia. These complications have significant impact on the quality of life of the survivors of childhood posterior fossa tumours. In this paper, the frequency, etiology, and management of these complications will be reviewed.

Impact of strontium on skeletal deformities in Baltic cod (Gadus morhua callaris L.).

A study was conducted to examine spinal deformities such as lordosis, scoliosis, and dwarfism in cod (Gadus morhua callaris L.) that were caught in the southern Baltic. The bone tissue of the spine and the muscle of the deformed cod were analyzed for concentration of macroelements (Sr, Ca and P) and toxic metals (Cd, Pb and Hg). Healthy specimens of the same body length that were caught in the same hauls were also tested, and these comprised the reference material. The study was undertaken to verify the hypothesis that lowered values of Ca/Sr and P/Sr ratios caused skeletal deformities. Toxic metals were also tested to determine whether they had an impact on the deformities of cod inhabiting Baltic waters. In cod with deformities, a significant decrease in Ca/Sr ratios were noted in 86% of the spine and 97% of the muscle. Decreases in the values of the P/Sr ratios were confirmed in 57% of the bone tissue and 78% of the muscle tissue of individuals with skeletal deformities. Toxic metals (Cd, Pb and Hg) occurred in the bone and muscle tissues of deformed and healthy cod on the low levels. It was not differences in concentrations of these elements, and thus could not have had an impact on the occurrence of deformities. Skeletal deformities could have resulted from lowered values of the Ca/Sr and P/Sr ratios of the spinal bone and muscle tissues of cod. Lower values of these coefficients should be linked to the varied salinity (5-21‰) and strontium (5-15Bqm(-3)) concentrations of Baltic waters.

Successful treatment of dwarfism secondary to long-term steroid therapy in steroid-dependent nephrotic syndrome.

Prolonged steroid therapy is generally used for steroid-dependent nephrotic syndrome in pediatric patients. However, dwarfism secondary to a long-term regimen and its successful reverse is rarely reported. The underlying mechanism of dwarfism is still poorly understood, as both long-term steroid use and nephrotic syndrome may interact or independently interfere with the process of growth. Here, we present a 17-year-old patient with dwarfism and steroid-dependent nephrotic syndrome and the successful treatment by recombinant human growth factor and cyclosporine A with withdrawal of steroid. We also briefly review the current understanding and the management of dwarfism in pediatric patients with nephrotic syndrome.

Generalized metaphyseal modification with cone-shaped epiphyses following long-term administration of 13-cis-retinoic acid.

UNLABELLED: We report on a 6-year-old girl with short stature which developed following the administration of 13-cis-retinoic acid (a synthetic derivative of vitamin A or retinoid) for 40 months as adjunct chemotherapy for neuroblastoma. Radiographic examination suggested osteophyte formation in the cervical spine, which is the most common skeletal manifestation of retinoid toxicity [10, 11]. In addition, severe metaphyseal cupping with a cone-shaped epiphysis primarily affecting rapidly growing long bones was found, which represented impaired enchondral ossification. This epi-metaphyseal alteration, though unusually severe, was reminiscent of the premature epiphyseal closure which has been described as an adverse effect of 13-cis-retinoic acid [10-12]. Other minor skeletal changes included posterior scalloping of the vertebral bodies and increased interpediculate distances, which were related to a widened spinal canal found on CT. A literature search disclosed several primary skeletal dysplasias with superficial radiological similarities to those of the present patient. However, these entities showed significant clinical and radiological differences from our patient. CONCLUSION: The precise cause of the generalized skeletal alteration in the present patient remained unknown, but it conceivably resulted from the administration of 13-cis-retinoic acid.

Growth suppression in children receiving acetazolamide with antiepileptic drugs.

To clarify the effect of the clinical dosage of acetazolamide on growth in children with epilepsy or febrile convulsion, the standard scores of height and weight in 17 subjects receiving acetazolamide as an adjunct to unchanged monotherapy of antiepileptic drug were compared longitudinally through four phases: before antiepileptic drug administration, with monotherapy of antiepileptic drug, with acetazolamide in addition to monotherapy, and after acetazolamide discontinuation. The standard scores of both height and weight in the subjects were significantly reduced during the phase of acetazolamide administration. During this period, serum concentrations of potassium and total CO2 decreased while that of chloride increased, suggesting the existence of metabolic acidosis in the subjects. For both height and weight, there was no correlation between the degree of standard score reduction during acetazolamide administration and age at the time of acetazolamide initiation, duration of acetazolamide administration, dosages of acetazolamide, and variety of antiepileptic drugs concomitantly administered with acetazolamide. We speculate that metabolic acidosis induced by acetazolamide suppressed the growth of the subjects and that there were large individual differences in the susceptibility to acetazolamide for growth suppression among patients receiving acetazolamide.

Growth deficits in ADHD children revisited: evidence for disorder-associated growth delays?

OBJECTIVE: To reevaluate the hypothesis that stimulants cause growth deficits in children with attention-deficit hyperactivity disorder (ADHD). METHOD: Growth deficits in height and weight were examined in 124 children and adolescents with ADHD and 109 controls, using appropriate correction by age and parental height measures and attending to issues of pubertal stage, treatment, and psychiatric comorbidity. RESULTS: Small but significant differences in height were identified between ADHD children and controls. However, height deficits were evident in early but not late adolescent ADHD children and were unrelated to use of psychotropic medications. There was no evidence of weight deficits in ADHD children relative to controls, and no relationship between measures of malnutrition and short stature was identified. CONCLUSIONS: ADHD may be associated with temporary deficits in growth in height through mid-adolescence that may normalize by late adolescence. This effect appears to be mediated by ADHD and not its treatment.

Patterns of growth deficiency in rats exposed in utero to undernutrition, ethanol, or the neuroteratogen methylazoxymethanol (MAM).

Children and experimental animals exposed to ethanol (EtOH) in utero commonly have low birthweights, and many remain small at maturity. Low body weight or small stature in adulthood may reflect an inability to recover from in utero growth retardation, or it may reflect a separate, postnatal growth deficiency. In this study, daily body weights (postnatal days 1 to 60) were compared among the offspring of the following groups of Long Evans rats: dams fed liquid diet containing 35% EtOH-derived calories; their pair-fed and chow-fed controls; and dams exposed to methylazoxymethanol (MAM) in two previous studies, in which offspring exhibited reduced numbers of growth hormone releasing factor (GRF) neurons. All treatments produced a number of offspring with weight deficits beginning after birth and persisting into maturity. Three distinct patterns of growth deficiency were observed: (1) weight loss relative to controls in the first weeks of life, seen in offspring exposed to EtOH, pair feeding, or MAM on gestation day 13 (G13); (2) a delay in the onset of the prepubertal growth spurt, seen in all EtOH-exposed offspring and in G13 MAM-exposed dwarfs; and (3) failure to sustain the prepubertal growth spurt, seen only after exposure to MAM on G14. The results of this study support the view that prenatal EtOH exposure is capable of affecting postnatal growth specifically; moreover, the pattern of growth deficiency seen in EtOH-exposed offspring was distinct from that of the undernourished offspring of pair-fed dams.

Mycobacterium fortuitum in systemic lupus erythematosus.

When she was five years old, this patient - aged 20 time of death - had had two diagnoses: Leri-Weill's disease and SLE. The latter led to uninterrupted use of systemic corticosteroids. Twelve months before death, multiple purulent bursitis were followed by cutaneous nodules. From the latter, but not from the former, Mycobacterium fortuitum was isolated. Our case is in agreement with what is generally accepted: this saprophyte organism becomes pathogenic in disseminated infections, only if the immune system deteriorates.

Dwarfism in a swine herd: suspected vitamin A toxicosis.

Dwarfism was observed in a group of 30 crossbred pigs. Affected pigs had short limbs and retarded growth. Reduced long-bone length as well as flattening and caudal rotation of the humeral head and the distal femoral condyles were seen at necropsy. Metaphyseal growth plates in vertebrae were narrow and, in long bones, were closed prematurely. There was a sparing of growth plates in traction epiphyses. Vitamin A toxicosis was considered as a possible cause.

[Diastrophic dwarfism (author's transl)]. / Nanosomia diastrophica.

On the basis of their own patient the authors discuss clinical and radiological features of diastrophic dwarfism. In this rare disorder the infant is dwarfed, the limbs are shortened. It is associated with marked talipes equinovarus, limited movements and contractures of other joints. Dislocation in the hip or knee and development of kyphoscoliosis leads to furhter deformity. As aetiopathogenesis the authors consider the role of infection in the mother during her early pregnancy or teratogenic aspects of some drugs (tetracyclines, septrim).