scholarly journals AGE-RELATED CHANGES IN ADRENAL SIZE DURING THE FIRST YEAR OF LIFE: COMPARISON OF ULTRASOUND EVALUATION AND DHEA-S IN NORMAL INFANTS AND A NEWBORN WITH CONGENITAL ADRENAL HYPERPLASIA (CAH)

1986 ◽  
Vol 20 (11) ◽  
pp. 1182-1182
Author(s):  
B P Hauffa ◽  
D Menzel
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
First Year ◽  
Adrenal Hyperplasia ◽  
Ultrasound Evaluation ◽  
Age Related ◽  
First Year Of Life ◽  
Age Related Changes

AGE-RELATED CHANGES IN THICKNESS OF CORTEX AND ITS LAYERS IN THE PARAMEDIAN LOBULE OF THE CEREBELLUM IN CHILDREN

2020 ◽  
pp. 14-18
Author(s):  
Татьяна Александровна Цехмистренко ◽  
Аслан Батразович Мазлоев ◽  
Дмитрий Константинович Обухов
Keyword(s):  
Right Hemisphere ◽  
Molecular Layer ◽  
First Year ◽  
Age Related ◽  
Average Group ◽  
Computer Morphometry ◽  
The Right ◽  
First Year Of Life ◽  
Granular Layers ◽  
Age Related Changes

Цель - изучение возрастных изменений толщины коры и ее слоев в парамедианной дольке мозжечка у детей. Материал и методы. Работа выполнена на постмортальном материале (62 мозжечка), полученном от детей в возрасте от рождения до 12 лет, умерших в результате травм без повреждений головного мозга. С помощью компьютерной морфометрии на окрашенных методом Ниссля фронтальных гистологических срезах коры, взятой билатерально в области парамедианной (тонкой) дольки (HVIIB) на вершине листков мозжечка, измеряли толщину коры, а также толщину ее молекулярного и зернистого слоев. Анализ количественных данных проводили в годовых интервалах. Результаты. В парамедианной дольке мозжечка увеличение толщины коры происходит в четыре этапа: в правом полушарии - от рождения к 1, 3, 5 и 9 годам, в левом полушарии - к 1, 5, 7 и 9 годам. Левосторонняя асимметрия толщины коры мозжечка отмечается у детей 1 и 2 лет, толщины молекулярного слоя - у детей 3 лет жизни. Правосторонняя асимметрия характерна для толщины зернистого слоя у детей 3 лет и поперечника коры, в целом, у детей 6 лет. Толщина коры и слоев в области парамедианной дольки мозжечка по среднегрупповым показателям достигает уровня взрослых людей к 9 годам. Выводы. Толщина коры мозжечка и ее слоев в области дольки H VII B увеличивается гетерохронно и гетеродинамически в правом и левом полушариях мозжечка у детей на первом году жизни, а также в периоды раннего, первого и второго детства. Уменьшения поперечника коры и слоев в парамедианной дольке мозжечка у детей от рождения до 12 лет не обнаружено. Objective - to study the age-related changes in the thickness of the cortex and its layers in the paramedian lobule of the cerebellum in children. Material and methods. The work was performed on postmortem material (62 cerebellums) obtained from children aged from birth to 12 years who died from injuries but without brain damage. The thickness of the cortex, as well as the thickness of its molecular and granular layers, were measured using computer morphometry on the Nissl-stained frontal histological sections of the cortex taken bilaterally in the region of the paramedian (gracile) lobule (HVIIB) at the top of the folia of cerebellum. Analysis of quantitative data was performed at annual intervals. Results. In the paramedian lobule of the cerebellum, the increase in the thickness of the cortex occured in four stages: in the right hemisphere - from birth to 1, 3, 5 and 9 years, in the left hemisphere - to 1, 5, 7 and 9 years. Left-sided asymmetry of the cortical thickness of the cerebellum was observed in 1 and 2-year old children, the thickness of the molecular layer - in 3-year old children. Right-sided asymmetry was characteristic for the thickness of the granular layer in 3-year old children and a cross-section of the cortex in 6-year old children. The thickness of the cortex and layers in the area of the paramedian lobule of the cerebellum on the average group indicators reached the level of adults by 9 years. Conclusions. The thickness of the cerebellar cortex and its layers in the area of the lobule HVIIB increased heterochronically and heterogeneously in the right and left hemispheres of the cerebellum in children of the first year of life, and in the periods of early, first and second childhood. No reduction in the diameter of the cortex and layers in the paramedian lobule of the cerebellum of children from birth to 12 years was found.

scholarly journals Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose

2017 ◽  
Vol 89 (1) ◽  
pp. 7-12 ◽  
Author(s):  
Walter Bonfig ◽  
Friedhelm Roehl ◽  
Stefan Riedl ◽  
Jürgen Brämswig ◽  
Annette Richter-Unruh ◽  
...  
Keyword(s):  
Sodium Chloride ◽  
Congenital Adrenal Hyperplasia ◽  
Routine Care ◽  
Sodium Content ◽  
Adrenal Crisis ◽  
First Year ◽  
Adrenal Hyperplasia ◽  
Salt Wasting ◽  
First Year Of Life ◽  
Oral Sodium

Introduction: Sodium chloride supplementation in salt-wasting congenital adrenal hyperplasia (CAH) is generally recommended in infants, but its implementation in routine care is very heterogeneous. Objective: To evaluate oral sodium chloride supplementation, growth, and hydrocortisone and fludrocortisone dose in infants with salt-wasting CAH due to 21-hydroxylase in 311 infants from the AQUAPE CAH database. Results: Of 358 patients with classic CAH born between 1999 and 2015, 311 patients had salt-wasting CAH (133 females, 178 males). Of these, 86 patients (27.7%) received oral sodium chloride supplementation in a mean dose of 0.9 ± 1.4 mmol/kg/day (excluding nutritional sodium content) during the first year of life. 225 patients (72.3%) were not treated with sodium chloride. The percentage of sodium chloride-supplemented patients rose from 15.2% in children born 1999–2004 to 37.5% in children born 2011–2015. Sodium chloride-supplemented and -unsupplemented infants did not significantly differ in hydrocortisone and fludrocortisone dose, target height-corrected height-SDS, and BMI-SDS during the first 2 years of life. Conclusion: In the AQUAPE CAH database, approximately one-third of infants with salt-wasting CAH receive sodium chloride supplementation. Sodium chloride supplementation is performed more frequently in recent years. However, salt supplementation had no influence on growth, daily fludrocortisone and hydrocortisone dose, and frequency of adrenal crisis.

Low Adrenomedullary Function Predicts Acute Illness in Infants with Classical Congenital Adrenal Hyperplasia

2021 ◽  
Author(s):  
Jonathan Weber ◽  
Veeraya K Tanawattanacharoen ◽  
Amy Seagroves ◽  
Mark C Liang ◽  
Christina M Koppin ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Acute Illness ◽  
First Year ◽  
Plasma Epinephrine ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Young Infants ◽  
Increased Risk ◽  
21 Hydroxylase Deficiency ◽  
First Year Of Life

Abstract Context Youth with classical congenital adrenal hyperplasia (CAH) exhibit abnormal adrenomedullary function with decreased epinephrine levels noted in newborns and young infants. Little is known about how this relates to morbidity during the first year of life. Objective To study plasma epinephrine levels in infants with classical CAH and examine the clinical significance of epinephrine deficiency in the first year of life. Design Prospective cohort study. Setting Study participants were recruited from a pediatric tertiary care center. Patients or Other Participants 36 infants with classical CAH due to 21-hydroxylase deficiency and 27 age-matched unaffected controls with congenital hypothyroidism. Main Outcome Measures Plasma epinephrine levels (N=27), CYP21A2 genotype (N=15), and incidence of acute illnesses from birth to 1 year of age (N=28). Results Epinephrine levels in CAH infants independently predicted illness incidence in the first year of life (β=-0.018, R=-0.45, P=0.02) and were negatively correlated with 17-hydroxyprogesterone at diagnosis (R=-0.51, P=0.007). Infants with salt-wasting CAH exhibited lower epinephrine levels as newborns than simple-virilizing infants (P=0.02). CAH patients had lower epinephrine as newborns than controls (P=0.007) and showed decreases in epinephrine from birth to 1 year of age (P=0.04). Null genotype was associated with lower newborn epinephrine and more illness in the first year of life, compared to less severe mutation categories. Conclusions Lower epinephrine levels are associated with increased risk of illness among CAH infants. While not currently part of clinical standard of care, measuring epinephrine levels and assessing genotype may help predict acute illness in the first year of life.

Patterns of growth from birth to maturity in infants and children with congenital adrenal hyperplasia

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S295-S304 ◽  
Author(s):  
George W. Clayton
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Growth Failure ◽  
Normal Growth ◽  
Growth Patterns ◽  
Height Velocity ◽  
Growth Spurt ◽  
First Year ◽  
Adrenal Hyperplasia ◽  
Pubertal Growth Spurt ◽  
First Year Of Life

Abstract Patterns of growth of 30 children with Congenital Adrenal Hyperplasia of the 21-hydroxylase type were studied from infancy to maturity. These children were compliant as to therapy. Intramuscular (I.M.) Cortisone (40mg/M2) given every three days resulted in growth failure after the first year of life. A marked increase in height velocity occurred when oral Cortisone (20-25mg/M2) was given at approximate] 3 years. Growth was relatively normal during childhood in both boys and girls but there was a marked increase in mean weight in both sexes. Pubertal growth spurt occurred normally in males but was delayed in females. Weight decreased in both sexes at puberty but not to normal. Better methods of monitoring this condition should result in therapeutic regimes which allow normal growth patterns as well as normal mature height.

Specific clinical and hormonal features of the non-classical form of 21-hydroxilase deficiency in the children during the first year of life detected from the results of neonatal screening

2014 ◽  
Vol 60 (3) ◽  
pp. 23-29
Author(s):  
T A Ionova ◽  
A N Tyulpakov
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Neonatal Screening ◽  
Molecular Genetic Analysis ◽  
Reproductive Age ◽  
First Year ◽  
Healthy Children ◽  
Adrenal Hyperplasia ◽  
Classical Form ◽  
First Year Of Life ◽  
Group 1

The non-classical form of 21-hydroxilase deficiency (21-OHD) has up to now been fairly well studied only in the patients of prepubertal and pubertal age as well as in the women of reproductive age. The advent of neonatal screening for congenital adrenal hyperplasia (CAD) made it possible to more frequently detect 21-OHD in the children during the first year of life. The domestic literature proposes no clinical and laboratory criteria for 21-OHD in the young children. The results of neonatal screening of 50 children presenting with elevated 17-hydroxyprogesterone (17-OHP) levels carried out in the period from 2011 to 2013 were used to form two groups of patients, one comprised of 20 children with verified 21-OHD (group 1), the other containing 30 "healthy" children showing the false-positive elevation of 21-OHD levels. All the patients underwent the comprehensive clinical and hormonal examination supplemented by the molecular-genetic analysis. The main criterion for the inclusion of children in group 1 was the presence of mutations in the CYP21 gene It was shown that determination of 21-OHD by tandem mass-spectrometry (TMS) including that in the framework of multisteroid analysis yields the most specific and accurate data in the children below one year of age; such analysis is indicated to all patients in whom the neonatal screening for congenital adrenal hyperplasia reveals the slightly elevated 17-OHP levels. The analysis for the group of patients with verified 21-OHD has demonstrated the absence of clinical and laboratory signs of adrenal insufficiency and/or hyperandrogenism in the children aged below 1 year.

Age-related changes in oxygen saturation over the first year of life: A longitudinal study

1994 ◽  
Vol 30 (5) ◽  
pp. 423-428 ◽  
Author(s):  
I. B. MASTERS ◽  
A. M. GOES ◽  
L. HEALY ◽  
M. O'NEIL ◽  
D. STEPHENS ◽  
...  
Keyword(s):  
Longitudinal Study ◽  
Oxygen Saturation ◽  
First Year ◽  
Age Related ◽  
First Year Of Life ◽  
Age Related Changes
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