RESUMEN
This study highlights the need for the development of simple, efficient, and cost-effective farm-scale applications to treat wastewater arising from vegetable-peeling operations. The aim was to evaluate two full-scale biological wastewater treatment systems, a sequencing batch reactor (SBR) and a biofilter, and a chemical wastewater treatment system on farms carrying out peeling of vegetables. The types, design criteria and parameters of the processes, as well as properties of the untreated and treated wastewaters were presented and evaluated. Seven-day biochemical oxygen demand (BOD7) entering the SBR was 3100 +/- 529 mg l(-1) (mean +/- standard deviation). The results showed that the SBR was very stable and effective in the treatment of carrot-processing wastewaters, the BOD7 for effluent being about 10 mg l(-1). The biofilter examined did not operate well because the pH too low: the reduction for BOD7 was 63% and, for COD, 58%. When wastewater from potato processing was treated with aluminium sulphate and conveyed to an artificial pond, removal of BOD7 was 67% and that of COD 69%. This method is only suitable for pre- or post-treatment of these wastewaters. Control of the treatment processes appeared to be essential for their proper functioning.
Asunto(s)
Agricultura/métodos , Reactores Biológicos , Verduras , Eliminación de Residuos Líquidos/métodos , Manipulación de Alimentos/métodos , Estaciones del AñoRESUMEN
BACKGROUND: Noonan syndrome (NS) is an autosomal dominant inherited disease, characterized by a distinctive facial appearance, congenital heart defects, and short stature. Treatment with growth hormone (GH) is an option to enhance height, but long-term effects are still unclear. PATIENTS AND METHODS: A cohort of 402 patients (269 males, 133 females), mean age 9.7 years at start with GH, was studied within the KIGS International growth database with respect to long-term response to GH therapy and final height after GH therapy. RESULTS: At the start of GH therapy median height was -2.61 SDS (Tanner 1966 standards). Seventy-three patients who were followed longitudinally for 3 years had an increment in height SDS (Ht SDS) over the first 3 successive years of 0.54, 0.13 and 0.13, respectively. Twenty-four patients had reached their final height after 4-12 years of GH treatment. Their Ht SDS increased from a median of -3.28 to a median of -2.41 at final height. CONCLUSION: This group of patients with NS showed an early response to GH treatment, with an attenuation of this effect thereafter. At final height the median increment of final height was 0.61 SDS according to Tanner standards and 0.97 SDS according to Noonan standards. No serious side effects were reported.
Asunto(s)
Estatura/efectos de los fármacos , Hormona de Crecimiento Humana/uso terapéutico , Síndrome de Noonan/tratamiento farmacológico , Proteínas Recombinantes/uso terapéutico , Niño , Estudios de Cohortes , Bases de Datos Factuales , Femenino , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVE: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is characterized by chronic mucocutaneous candidiasis and autoimmune destruction of endocrine organs. The resulting endocrinopathies and their treatment may impact bone health. The purpose of our study was to assess bone health and its correlates in adult patients with APECED. PATIENTS AND METHODS: Twenty-five adults (12 males) with APECED were prospectively assessed. Data on their previous medical history were collected from hospital records. Areal bone mineral density (aBMD) for the lumbar spine (L1-L4), femoral neck and whole body as well as volumetric BMD (vBMD) for the lumbar spine (L2-L4) were measured with dual-energy X-ray absorptiometry (DXA). RESULTS: Mean age was 34 years (range 21-59 years). All patients had 1-4 autoimmune endocrinopathies, the most common being adrenocortical failure (20 patients) and hypoparathyroidism (18 patients). Osteopaenia or osteoporosis was present in 28%. The median (range) aBMD Z-scores were for the lumbar spine -0.3 (-2.3 to +3.3) and for the femoral neck, -0.1 (-2.2 to +2.0). The BMD Z-scores tended to be higher in patients with hypoparathyroidism than in patients with normal parathyroid function (at the lumbar spine +0.4 vs.-1.2, P = 0.016, and at the femoral neck +0.3 vs.-0.4, P = 0.090). Adrenocortical failure had a negative impact on BMD. Six patients had had low-impact fractures and three were diagnosed with compression fractures. CONCLUSIONS: Despite the complex endocrine problems, the overall prevalence of symptomatic osteoporosis is low in adults treated for APECED. Osteopaenia is frequently observed and warrants follow-up. Treated hypoparathyroidism may have a positive, and adrenocortical failure a negative, impact on bone health.
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Densidad Ósea , Poliendocrinopatías Autoinmunes/fisiopatología , Absorciometría de Fotón , Adulto , Enfermedades Óseas Metabólicas/complicaciones , Enfermedades Óseas Metabólicas/fisiopatología , Distribución de Chi-Cuadrado , Femenino , Cuello Femoral/fisiopatología , Fracturas Óseas/complicaciones , Fracturas Óseas/fisiopatología , Humanos , Hipoparatiroidismo/complicaciones , Hipoparatiroidismo/fisiopatología , Vértebras Lumbares/fisiopatología , Masculino , Persona de Mediana Edad , Poliendocrinopatías Autoinmunes/complicaciones , RiesgoRESUMEN
The aim of pubertal induction by estrogen in hypogonadic girls is to achieve physical and psychological development similar to that in natural puberty. We investigated the use of percutaneous estradiol gel for induction of puberty in girls with Turner syndrome (TS).Twenty-three girls with TS and hypogonadism were included in the study. The initial percutaneous dose of 0.1 mg ended as 1.5 mg in the fifth year. The efficacy of the treatment was monitored by measuring height, weight, skeletal age, pubertal status, and serum hormone levels and gynecological ultrasonographic examinations throughout the study. Mean serum estradiol concentrations increased from 22.2 pmol/liter at baseline to 162.2 pmol/liter, and mean FSH levels decreased from 77.4 IU/liter at baseline to 19.2 IU/liter after 5 yr. There were no significant differences between GH users and nonusers with regard to height sd score, weight sd score, bone age acceleration, or adult height. The development of secondary sexual characteristics and uterine growth progressed gradually during the study. All girls reached at least stage B4P4. With percutaneous estradiol gel, the development of secondary sexual characteristics and uterine growth proceeded gradually, mimicking natural puberty. Estradiol gel was safe, easy to use, and well accepted by the subjects and provides an excellent way to individualize pubertal induction.
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Estradiol/administración & dosificación , Pubertad/efectos de los fármacos , Síndrome de Turner/tratamiento farmacológico , Síndrome de Turner/fisiopatología , Administración Cutánea , Adolescente , Niño , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Estradiol/efectos adversos , Femenino , Geles , Crecimiento , Hormonas/sangre , Humanos , Hipogonadismo/etiología , Caracteres Sexuales , Síndrome de Turner/complicaciones , Útero/crecimiento & desarrolloRESUMEN
The diabetes predisposing effect of HLA genes is defined by a complex interaction of various haplotypes. We analyzed the disease association of HLA DRB1-DQA1-DQB1 genotypes in a large nuclear family cohort (n = 622) collected in Finland. Using the affected family based artificial control approach we aimed at characterizing all detectable disease-specific HLA haplotype and genotype effects. The DRB1*0401-DQB1*0302 haplotype was the most prevalent disease susceptibility haplotype in the Finnish population followed by (DR3)-DQA1*05-DQB1*02 and DRB1*0404-DQB1*0302. DRB1*0405-DQB1*0302 conferred the highest disease risk, although this haplotype was very rare. The DRB1*04-DQB1*0304 was also associated with increased disease risk, an effect detected for the first time in the Finnish population. The following haplotypes showed significant protection from the disease and are listed in decreasing order of the strength of their effect: (DR7)-DQA1*0201-DQB1*0303, (DR14)-DQB1*0503, (DR15)-DQB1*0602, DRB1*0403-DQB1*0302, (DR13)-DQB1*0603, (DR11/12/13)-DQA1*05-DQB1*0301, (DR1)-DQB1*0501. In addition to the DRB1*0401/0404-DQB1*0302/(DR3)-DQA1*05-DQB1*02 genotype and DRB1*04-DQB1*0302 homozygous genotypes, heterozygous combinations DRB1*0401-DQB1*0302/(DR13)-DQB1*0604, approximately /(DR8)-DQB1*04, approximately /(DR9)-DQA1*03-DQB1*0303, approximately /(DR1)-DQB1*0501 and approximately /(DR7)-DQA1*0201-DQB1*02 were also disease-associated. As a new finding in this population, the (DR3)-DQA1*05-DQB1*02 homozygous and (DR3)-DQA1*05-DQB1*02/(DR9)-DQA1*03-DQB1*0303 heterozygous genotypes conferred disease susceptibility. Similarly, the DRB1*0401-DQB1*0302/(DR13)-DQB1*0603 genotype was disease predisposing, implying that DQB*0603-mediated protection from diabetes is not always dominant. Comparison of our findings with published data from other populations indicates a significant disease-specific heterogeneity of the (DR8)-DQB1*04, (DR7)-DQA1*0201-DQB1*02 and (DR3)-DQA1*05-DQB1*02 haplotypes.
Asunto(s)
Diabetes Mellitus Tipo 1/genética , Predisposición Genética a la Enfermedad , Antígenos HLA-DQ/genética , Antígenos HLA-DR/genética , Adulto , Niño , Femenino , Finlandia , Haplotipos , Humanos , MasculinoRESUMEN
AIMS: To study whether post-prandial insulin lispro (PL) could be used as a part of insulin therapy instead of premeal human regular insulin (HR) in prepubertal children with Type 1 diabetes mellitus (Type 1 DM). PATIENTS AND METHODS: In this open, randomized cross-over study patients used either PL or HR at breakfast and at dinner. After a 1-month screening period, patients were randomized to treatment with PL or HR for 3 months and then they crossed over to the other insulin for an additional 3 months. The patients were 24 prepubertal children with Type 1 DM (median age 6.2 years, duration of diabetes 37 months). Home monitoring of 1-day glucose profiles at meals (premeal, 1 h and 2 h after breakfast and after dinner) and HbA1c were measured before randomization, before cross-over, and at the last visit. Data on hypoglycaemic episodes were collected at each of the seven visits. The variables were compared between the two treatments. RESULTS: Of the patients 22/24 completed the study. There were no major differences in the glucose excursions between PL and HR after breakfast (mean +/- SD: 1-h PL 3.7 +/- 4.7 vs. HR 2.9 +/- 3.9 mmol/l, P = 0.3; 2-h -0.9 +/- 5.4 vs. 0.3 +/- 4.5 mmol/l, P = 0.2, respectively) or after dinner (1-h PL -2.5 +/- 4.8 vs. HR -0.4 +/- 3.7 mmol/l, P = 0.07, 2-h -4.1 +/- 5.2 vs. -0.7 +/- 5.0 mmol/l, P = 0.05, respectively). Mean change of HbA1c was similar in both treatment groups (PL 0.2 +/- 0.8% vs. HR -0.4 +/- 0.7%, P = 0.1). The frequency of hypoglycaemic episodes was 4.9 per patient per month during treatment with PL, and 4.4 during HR (P = 0.3). CONCLUSION: Treatment with post-prandial lispro as a meal insulin is as effective and safe as traditional treatment with regular insulin in young children.
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Diabetes Mellitus Tipo 1/tratamiento farmacológico , Hipoglucemiantes/administración & dosificación , Insulina/análogos & derivados , Insulina/administración & dosificación , Insulina/uso terapéutico , Periodo Posprandial , Niño , Preescolar , Estudios Cruzados , Diabetes Mellitus Tipo 1/psicología , Esquema de Medicación , Familia , Humanos , Hipoglucemiantes/uso terapéutico , Insulina Lispro , Satisfacción del Paciente , Pubertad , Factores de TiempoRESUMEN
BACKGROUND: The role of oestrogens in the closure of growth plates in both sexes is unequivocal. We postulated that inhibition of oestrogen synthesis in boys with delayed puberty would delay maturation of the growth plates and ultimately result in increased adult height. METHODS: We did a randomised, double-blind, placebo-controlled study in which we treated boys with constitutional delay of puberty with testosterone and placebo, or testosterone and letrozole. Boys who decided to wait for the spontaneous progression of puberty without medical intervention composed the untreated group. FINDINGS: Letrozole effectively inhibited oestrogen synthesis and delayed bone maturation. Progression of bone maturation was slower in the letrozole group than in the placebo group. In 18 months, bone age had advanced 1.1 (SD 0.8) years in the untreated group and 1.7 (0.9) years in the group treated with testosterone and placebo, but only 0.9 (0.6) years in the letrozole group (p=0.03 between the treatment groups). Predicted adult height did not change significantly in the untreated group and in the placebo group, whereas in the group treated with letrozole the increase was 5.1 (3.7) cm (p=0.004). INTERPRETATIONS: Our findings suggest that if oestrogen action is inhibited in growing adolescents, adult height will increase. This finding provides a rationale for studies that aim to delay bone maturation in several growth disorders.
Asunto(s)
Inhibidores de la Aromatasa , Estatura/efectos de los fármacos , Nitrilos/uso terapéutico , Pubertad Tardía/tratamiento farmacológico , Testosterona/uso terapéutico , Triazoles/uso terapéutico , Adolescente , Método Doble Ciego , Quimioterapia Combinada , Humanos , Letrozol , Masculino , Testosterona/sangreRESUMEN
BACKGROUND: Hyperinsulinism in childhood is often caused by genetic defects involving the regulation of insulin secretion leading to recurrent episodes of hypoglycaemia. We report two patients with exercise induced hypoglycaemia. METHODS: Standardised short exercise tests with frequent blood glucose and plasma insulin measurements were performed in the patients and young healthy controls. RESULTS: Short term exercise resulted in insulin induced hypoglycaemia 15 to 50 minutes after the end of exercise. A massive burst of insulin secretion was observed within a few minutes of the start of exercise in both patients. By contrast glucose and insulin concentrations remained unchanged in healthy controls. CONCLUSIONS: Hyperinsulinaemic hypoglycaemia after moderate physical exercise represents a rarely described phenotype of hyperinsulinism with an as yet unknown defect in the regulation of insulin secretion. It should be suspected in individuals with recurrent exercise related syncope or disturbance of consciousness.
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Ejercicio Físico/fisiología , Hiperinsulinismo/complicaciones , Hipoglucemia/etiología , Adolescente , Estudios de Casos y Controles , Prueba de Esfuerzo , Femenino , Humanos , Lactante , Masculino , Convulsiones/etiología , Síncope/etiologíaAsunto(s)
Aminas , Benzodiazepinas , Ácidos Ciclohexanocarboxílicos , Hipotiroidismo/complicaciones , Personas con Discapacidades Mentales/psicología , Trastornos Psicomotores/etiología , Ácido gamma-Aminobutírico , Acetatos/uso terapéutico , Adulto , Ansiolíticos/uso terapéutico , Anticonvulsivantes/uso terapéutico , Clobazam , Epilepsia/complicaciones , Epilepsia/tratamiento farmacológico , Femenino , Gabapentina , Humanos , Hipotiroidismo/tratamiento farmacológico , Tirotropina/sangre , Tiroxina/sangre , Tiroxina/uso terapéuticoAsunto(s)
Glucemia/metabolismo , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Carbohidratos de la Dieta , Hipoglucemia/prevención & control , Insulina/efectos adversos , Adolescente , Niño , Preescolar , Diabetes Mellitus Tipo 1/enfermería , Finlandia , Humanos , Hipoglucemia/sangre , Hipoglucemia/inducido químicamente , Grupo de Atención al Paciente , Enfermería Pediátrica , Pediatría , Personal de Hospital , Encuestas y CuestionariosAsunto(s)
Coma/etiología , Hiperamonemia/etiología , Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa/complicaciones , Adolescente , Humanos , Hiperamonemia/sangre , Hiperamonemia/genética , Masculino , Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa/diagnóstico , Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa/terapia , Resultado del TratamientoRESUMEN
BACKGROUND: Eye fundus destruction and type II muscle fiber atrophy in gyrate atrophy of the choroid and retina with hyperornithinaemia (GA) may be mediated by elevated ornithine concentrations which strongly inhibit creatine biosynthesis. This results in deficiency of creatine phosphate (PCr), a key intracellular energy source, as we have demonstrated in skeletal muscle of the patients by 31P magnetic resonance spectroscopy (31P MRS). MATERIALS AND METHODS: Possible correction of the relative PCr deficiency by long-term daily exogenous supplementation of creatine or its precursors was investigated in four GA patients receiving creatine and in five patients treated with guanidinoacetic acid-methionine combination. The relative PCr concentration, expressed as PCr/Pi (Pi; inorganic phosphate) or as PCr/ATP ratios, was compared with the values of untreated GA patients, and matched healthy volunteers. RESULTS: Muscle PCr/Pi ratios (mean +/- SD) of the untreated and creatine supplemented GA patients and controls were 4.9 +/- 1.4, 7.9 +/- 0.4 and 8.4 +/- 1.3. Guanidinoacetate-methionine combination was similarly effective (respective PCr/Pi ratios: 4.9 +/- 0.7, 6.3 +/- 1.1 and 10.7 +/- 2.8). CONCLUSION: Supplementation with creatine or creatine precursors almost normalised low muscle PCr/Pi ratios of patients with GA.
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Creatina/uso terapéutico , Atrofia Girata/metabolismo , Músculo Esquelético/metabolismo , Ornitina/metabolismo , Adolescente , Adulto , Niño , Suplementos Dietéticos , Femenino , Glicina/análogos & derivados , Glicina/uso terapéutico , Atrofia Girata/complicaciones , Atrofia Girata/tratamiento farmacológico , Humanos , Espectroscopía de Resonancia Magnética , Masculino , Metionina/uso terapéutico , Persona de Mediana Edad , Músculo Esquelético/efectos de los fármacos , Isótopos de FósforoRESUMEN
OBJECTIVE: To analyze in vivo brain creatine (Cr) content in gyrate atrophy of the choroid and retina with hyperornithinemia (GA). BACKGROUND: GA is caused by inherited deficiency of ornithine-delta-aminotransferase activity. Patients lose their vision by middle age and develop selective atrophy of type II skeletal muscle fibers. As demonstrated by MRS, the patients' skeletal muscles have diminished stores of high-energy Cr phosphate. Minor structural and electrophysiologic abnormalities in the brain of these patients also imply that the CNS may be affected. METHODS: The authors acquired proton MR spectra of the basal ganglia of 22 healthy control subjects and 20 GA patients. Nine patients received supplementary Cr or its precursors, and one child was on an arginine-restricted diet to normalize plasma ornithine concentration. The ratios of N-acetylaspartate (NAA) to Cr, NAA to choline (Cho), and Cho to Cr, and the ratios of NAA, Cho, and Cr to tissue water were calculated. RESULTS: NAA/Cr (Cho/Cr) in the untreated and treated patients and control subjects were (mean +/- SD) 3.3+/-0.4, 2.0+/-0.4, and 1.5+/-0.7 (1.9+/-0.3, 1.3+/-0.4, and 0.9+/-0.2), indicating that Cr content in untreated GA patients was proportionally and markedly diminished, and partially corrected by therapy (p < 0.0001). NAA/Cho was similar in all three groups. Cr/water in the untreated patients was only 46%, and increased to 75% of the control ratios in the treated patients (p < 0.0001). CONCLUSIONS: Hyperornithinemia-associated Cr deficiency in GA also affects the CNS, further supporting the possibility that Cr deficiency also has a pathogenetic role in the retina. The deficiency was partially corrected by Cr supplementation and an arginine-restricted diet.
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Encéfalo/metabolismo , Coroides/metabolismo , Creatina/química , Ornitina-Oxo-Ácido Transaminasa/deficiencia , Retina/metabolismo , Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Espectroscopía de Resonancia Magnética , Masculino , Persona de Mediana EdadRESUMEN
Rapid infantile growth was the first clinical sign in patients (n = 51) with aspartylglycosaminuria, a lysosomal storage disorder. Even if young children with aspartylglycosaminuria were tall for their age, an early but weak pubertal growth spurt in both sexes resulted in reduced adult heights.
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Acetilglucosamina/análogos & derivados , Crecimiento , Enfermedades por Almacenamiento Lisosomal/fisiopatología , Pubertad/fisiología , Acetilglucosamina/orina , Adolescente , Adulto , Niño , Preescolar , Femenino , Finlandia , Humanos , Lactante , Recién Nacido , Enfermedades por Almacenamiento Lisosomal/orina , Masculino , MenarquiaRESUMEN
The aims of this study were to evaluate the efficacy and safety of different doses of growth hormone (GH) treatment in prepubertal short children born small-for-gestational-age (SGA). Forty-eight children born SGA from Sweden, Finland, Denmark and Norway were randomly allocated to three groups: a control group of 12 children received no treatment for 2 y, one group was treated with GH at 0.1 IU/kg/d (n=16), and one group was treated with GH at 0.2 IU/kg/d (n=20). In total 42 children completed 2 y of follow-up, and 24 children from the treated groups completed 3 y of treatment. Their mean (SD) age at the start of the study was 4.69 (1.61) y and their mean (SD) height was -3.16 (0.70) standard deviation scores (SDS). The children remained prepubertal during the course of the study. No catch-up growth was observed in the untreated group, but a clear dose-dependent growth response was found in the treated children. After the third year of treatment, the group receiving the higher dose of GH, achieved their target height. The major determinants of the growth response were the dose of GH used, the age at the start of treatment (the younger the child, the better the growth response) and the family-corrected individual height deficit (the higher the deficit, the better the growth response). Concentration of insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 increased during treatment. An increase in insulin levels was found without negative effects on fasting glucose levels or glycosylated haemoglobin levels. GH treatment was well tolerated. In conclusion, short prepubertal children born SGA show a dose-dependent growth response to GH therapy, and their target height SDS can be achieved within 3 y of treatment given GH at 0.2 IU/kg/d. However, the long-term benefit of different regimens of GH treatment in children born SGA remains to be established.
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Estatura/efectos de los fármacos , Trastornos del Crecimiento/tratamiento farmacológico , Hormona de Crecimiento Humana/uso terapéutico , Recién Nacido Pequeño para la Edad Gestacional , Preescolar , Dinamarca , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Finlandia , Trastornos del Crecimiento/etiología , Humanos , Lactante , Recién Nacido , Masculino , Estadísticas no Paramétricas , Suecia , Resultado del TratamientoRESUMEN
Aspartyglucosaminuria (AGU) is a lysosomal storage disease with autosomal recessive inheritance that is caused by deficient activity of aspartylglucosaminidase (AGA), a lysosomal enzyme belonging to the newly described enzyme family of N-terminal hydrolases. An AGU mouse model was generated by targeted disruption of the AGA gene designed to mimic closely one human disease mutation. These homozygous mutant mice have no detectable AGA activity and excrete aspartylglucosamine in their urine. Analogously to the human disease, the affected homozygous animals showed storage in lysosomes in all analyzed tissues, including the brain, liver, kidney and skin, and lysosomal storage was already detected in fetuses at 19 days gestation. Electron microscopic studies of brain tissue samples demonstrated lysosomal storage vacuoles in the neurons and glia of the neocortical and cortical regions. Magnetic resonance images (MRI) facilitating monitoring of the brains of living animals indicated cerebral atrophy and hypointensity of the deep gray matter structures of brain-findings similar to those observed in human patients. AGU mice are fertile, and up to 11 months of age their movement and behavior do not differ from their age-matched littermates. However, in the Morris water maze test, a slow worsening of performance could be seen with age. The phenotype mimics well AGU in humans, the patients characteristically showing only slowly progressive mental retardation and relatively mild skeletal abnormalities.
Asunto(s)
Acetilglucosamina/análogos & derivados , Aspartilglucosilaminasa/genética , Modelos Animales de Enfermedad , Discapacidad Intelectual/genética , Enfermedades por Almacenamiento Lisosomal/genética , Ratones Noqueados/genética , Acetilglucosamina/orina , Animales , Aspartilglucosaminuria , Encéfalo/metabolismo , Encéfalo/patología , Progresión de la Enfermedad , Marcación de Gen , Genes Recesivos , Humanos , Hígado/patología , Enfermedades por Almacenamiento Lisosomal/enzimología , Imagen por Resonancia Magnética , Aprendizaje por Laberinto , Ratones , Microscopía Electrónica , FenotipoRESUMEN
UNLABELLED: Eighty-two children with malignant brain tumours were treated according to the "8 in 1" chemotherapy protocol in Finland during 1986 to 1993. Thirty-seven with brain tumours not involving the hypothalamic-pituitary region are still alive and tumour-free. The growth and response to growth hormone (GH) therapy in these children was analysed. Children who received craniospinal irradiation had the most severe loss of height SDS, being -1.07 within 3 years of the diagnosis. Even children with no irradiation to the hypothalamic-pituitary axis had a mean change in height SDS of -0.5 after 3 years. Fifteen of 23 children who received craniospinal irradiation and two out of eight children who received cranial irradiation have received GH therapy. A catch-up growth response to the daily GH therapy with the mean dose of 0.7 IU/kg per week was complete in 3 years (+1.87 SDS), irrespective of craniospinal irradiation, in children who were treated at prepubertal age but was seen in none of the children who had reached pubertal age. CONCLUSION: Growth impairment and GH deficiency are common in children treated for malignant brain tumours. The response to GH therapy is good in prepubertal children in terms of increased growth velocity, although the final height is not yet known.
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Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Neoplasias Encefálicas/tratamiento farmacológico , Enanismo/terapia , Hormona de Crecimiento Humana/administración & dosificación , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Estatura/efectos de los fármacos , Neoplasias Encefálicas/radioterapia , Niño , Preescolar , Terapia Combinada , Irradiación Craneana , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Enanismo/etiología , Femenino , Humanos , Sistema Hipotálamo-Hipofisario/efectos de los fármacos , Sistema Hipotálamo-Hipofisario/efectos de la radiación , Lactante , Masculino , Sistema Hipófiso-Suprarrenal/efectos de los fármacos , Sistema Hipófiso-Suprarrenal/efectos de la radiación , Radioterapia AdyuvanteRESUMEN
We studied psychosocial development and skeletal growth in 19 newly diagnosed patients with Legg-Calvé-Perthes disease (LCPD). Eleven patients had problems in visuospatial skills and five of 12 school-aged children had learning difficulties. The growth velocity of the patients was evaluated from 4 years before until 2 years after the diagnosis was made. Eight patients had a catch-up growth with +1.2 (0.9-1.7) delta SDS score (SDS: mean and ranges) before the diagnosis. Four patients with short stature and retarded bone age slightly diminished their growth velocity. Overnight serum growth hormone (GH) concentration and insulin-like growth factor I (IGF-I) levels were examined in the first nine consecutive patients. One patient had a high and another had a low mean GH concentration level, whereas all patients had IGF-I levels within normal limits. These results suggest that different kinds of growth disturbances may be associated with LCPD.
Asunto(s)
Discapacidades del Desarrollo/etiología , Crecimiento , Enfermedad de Legg-Calve-Perthes/complicaciones , Percepción Visual , Niño , Preescolar , Femenino , Hormona del Crecimiento/sangre , Humanos , Factor I del Crecimiento Similar a la Insulina/análisis , Discapacidades para el Aprendizaje/complicaciones , Enfermedad de Legg-Calve-Perthes/sangre , Enfermedad de Legg-Calve-Perthes/patología , Enfermedad de Legg-Calve-Perthes/psicología , Masculino , Pruebas Neuropsicológicas , Trastornos de la Percepción/complicaciones , PsicofisiologíaRESUMEN
GH provocative testing does not predict subsequent growth response, and is not therefore required before initiation of rhGH treatment in children with renal and liver transplants. However, therapeutic doses of glucocorticoids may potentially inhibit GH secretion in some patients, and determining GH secretory status by provocation tests and measuring spontaneous GH secretion is advisable at some point after organ Tx to exclude GH deficiency.