ABSTRACT
Aims: To examine psychometric properties including the factor structure of the German versions of the Diabetes Treatment Satisfaction Questionnaire for teens and parents (DTSQ-T/-P). Methods: Linguistically validated questionnaires were completed by 363 adolescents with type 1 diabetes and 655 parent-caregivers in a multicenter study. Confirmatory factor analysis (CFA), reliability, and correlations were examined. Results: CFA confirmed the 2-factor model of treatment satisfaction (TS) & perceived diabetes control (PDC) with an adjustment of removing the "medical support" item from the TS and examining it as a single item in this study. Cronbach's α of TS for DTSQ-T/-P was 0.82 & 0.83, respectively, and α of the two-item PDC factor was 0.70 & 0.60, respectively. The DTSQ scale scores positively correlated with time in range and inversely correlated with HbA1c. Scale scores of DTSQ-T/-P showed significantly positive relations to the KIDSCREEN-10 Index and negative associations with the Problem Areas in Diabetes (PAID). The TS of the parents was correlated with depressive symptoms measured in the Patient Health Questionnaire-9. Conclusions: The DTSQ-T/-P produced psychometrically sound scores in measuring diabetes treatment satisfaction in German teens with type 1 diabetes and their parents. German DTSQ versions for teens and parents are recommended in research and clinical practice.
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AIMS: Transition from pediatric to adult care is difficult for patients with chronic diseases. In this study, factors associated with metabolic control in childhood-onset type 1 diabetes (T1D) after transfer to adult care were analyzed. METHODS: Overall, 224 persons with T1D were contacted yearly from 1998 to 2019. They voluntarily answered a questionnaire about their current hemoglobin A1c (HbA1c) levels, diabetes-associated complications, kind of care, living conditions, and family situation. Then, mixed longitudinal-cross-sectional analyses were carried out. RESULTS: Overall, 190 patients answered at least once (mean age: 26.6 years). Diabetes complications were mentioned by 10 patients (5 microalbuminuria, 5 retinopathy). Most patients (92.6%) were in diabetes-specific care during the first year after transfer, with a trend to leave diabetes-specific care during the observation period. Patients in diabetes-specific care displayed lower HbA1c levels (%/mmol/mol) (7.1/54 vs. 7.5/58). An important predictor for HbA1c after transfer was HbA1c during the year before transfer (r=0.67, p <0.001). Patients living alone showed no difference in HbA1c levels from those living with their parents. Married patients had lower HbA1c levels (7.0/53 vs. 7.3/56, p<0.05) than unmarried ones. Patients with children (15.8%) presented lower HbA1c levels (6.9/52 vs. 7.3/56, p <0.01) than those without. CONCLUSIONS: Good metabolic results are favored in patients followed-up in specialized care, are married, and are parents. We recommend transfer to a diabetologist with experience in T1D at an individual age.
Subject(s)
Diabetes Complications , Diabetes Mellitus, Type 1 , Transition to Adult Care , Humans , Child , Adult , Diabetes Mellitus, Type 1/therapy , Diabetes Mellitus, Type 1/complications , Glycated Hemoglobin , Cross-Sectional StudiesABSTRACT
Background: Short children born small for gestational age (SGA) often have low muscle mass. Studies on maximal isometric grip-force (MIGF) observed lower muscle strength in these children. In contrast to MIGF, jumping is an everyday muscle activity for children. Our hypothesis was that GH treatment would cause an increase in jumping strength. So, we aimed to study jumping by mechanography in short SGA children before and during GH treatment. Methods: Monocentric prospective longitudinal study in a tertiary pediatric endocrinology center. We studied 50 prepubertal short children (23 females) born SGA (mean age 7.2 y, height -3.24 SDS) during GH treatment (mean dose 45 µg/kg/d). Main outcome measures were Peak jump force (PJF) and peak jump power (PJP) measured by Leonardo® ground reaction force plate at baseline and after 12 months of GH treatment. Mechanography data were compared to sex, age and height related references (SD-Score). Fitness was estimated as PJP/kg body weight by use of the Esslinger-Fitness-Index (EFI). Results: At start of GH treatment PJP/body weight was low at -1.52 SDS and increased significantly to -0.95 SDS during 12 months of treatment (p<0.001). PJF was low-normal compared to height dependent references and remained unchanged. PJP was normal compared to height dependent references and increased only slightly from -0.34 to -0.19 SDSHT. Conclusions: Jumping performance (EFI) measured by mechanography increased during one year of GH treatment in short children born SGA.
Subject(s)
Body Height , Infant, Small for Gestational Age , Infant, Newborn , Female , Humans , Child , Prospective Studies , Longitudinal Studies , Body Height/physiology , Body WeightABSTRACT
OBJECTIVES: Data on bone density and stability in Turner syndrome (TS) are contradictory. A confounding factor for interpretation is short stature. The aim was to measure bone density, geometry and stability in girls with TS compared to idiopathic short stature (ISS). METHODS: From 1999 to 2008, 59 girls with TS (35 prepubertal) were evaluated by pQCT. Mean age was 8.9 in prepubertal and 17.3 years in adolescent girls. Mean height was -3.1 and -1.8 SDS in prepubertal treatment-free and in adolescent, formerly rhGH-treated girls. For comparison, 18 prepubertal ISS girls were studied (age 7.7 years; height -3.3 SDS). Examination of radius with pQCT (XCT 2000). Cortical (CD) and trabecular density (TD), total bone area (TBA), cortical area (CA), cortical thickness, muscle area and strength strain index (SSI) were determined and compared with height related references. RESULTS: In prepubertal girls with TS, TD and CD were normal (0.55 and 0.90 SDS) and comparable to ISS (0.95 and 1.53 SDS). TBA was greater in girls with TS than in ISS (0.87 vs. -0.33 SDS) whereas CA was similar (1.48 vs. 1.43 SDS). The SSI was comparable (1.61 vs. 1.56 SDS). Adolescent girls with TS showed similar results with a TD of 0.48 SDS, a CD of -0.32, TBA of 1.99, a CA of -0.05 and an SSI of 0.88 SDS. CONCLUSIONS: The observations are consistent with normal bone density and stability but altered bone geometry in prepubertal and substituted adolescent girls with TS. This peculiarity may reflect SHOX deficiency. We therefore think that timely and adequate estrogen substitution could prevent bone loss in TS.
Subject(s)
Dwarfism, Pituitary , Turner Syndrome , Female , Adolescent , Humans , Child , Male , Bone Density/physiology , Bone and Bones , Radius , Short Stature Homeobox ProteinABSTRACT
OBJECTIVE: Copeptin is secreted in isomolar amounts along with arginine vasopressin peptide (AVP) from the neurohypophysis. Its stability makes it a perfect candidate for the endocrine approach in the diagnosis of AVP deficiency (AVPD; cranial diabetes insipidus; CDI). However, pediatric reference values are lacking. DESIGN AND PATIENTS: This is a monocentric retrospective analysis of donated residual serum samples from 72 children and adolescents who underwent arginine or growth hormone-releasing hormone-arginine stimulation to test GH secretory capacity from 2018 to 2022. MEASUREMENTS: Copeptin was measured in baseline, 30-, and 60-min samples by BRAHMS Copeptin proAVP Kryptor immunofluorescence assay. RESULTS: Of the 72 patients, 4 suffered from complete AVPD (CDI). The baseline level of copeptin in the 68 non-AVPD (non-CDI) patients was highly variable (range: 1.3-44.4 pmol/L). The increase after arginine was moderate (30 min range: 1.6-40.4 pmol/L). The median baseline and peak copeptin levels were 5.6 and 8.0 pmol/L, respectively. The 2.5th percentile of the baseline and peak values of copeptin were 2.1 and 3.3 pmol/L, respectively. The increase and peak value of copeptin were inversely related to age (R = -.405; p = .011, and R = -.335; p = .0072, respectively) but not to gender, body mass index (standard deviation score) or GH secretion. In the four patients with AVPD (CDI), baseline or stimulated copeptin was below the 2.5th percentile of non-AVPD (non-CDI) patients. CONCLUSIONS: Stimulated copeptin is a promising parameter for the differential diagnosis of polyuria-polydipsia syndrome. However, the low copeptin increase after arginine and the high limit of quantification of the assay are problematic for use in paediatrics.
Subject(s)
Arginine , Diabetes Insipidus, Neurogenic , Humans , Child , Adolescent , Retrospective Studies , GlycopeptidesABSTRACT
[This corrects the article DOI: 10.3389/fendo.2022.897897.].
ABSTRACT
Background: The methylation of IGF1 promoter P2 was reported to negatively correlate with serum IGF-1 concentration and rhGH treatment response in children with idiopathic short stature. These findings have not yet been confirmed. Objective: This study aimed to determine IGF1 promoter P2 methylation in short children treated with rhGH and correlate clinical parameters with the methylation status. In addition, long-term stability of methylation during rhGH treatment was studied. Design: This was a single tertiary center study analyzing clinical GH response and IGF-1 serum concentration changes in patients with GHD (n=40), SGA short stature (n=36), and Turner syndrome (n=16) treated with rhGH. Data were correlated to the methylation of two cytosine residues (-137, +97) of the P2 promoter of IGF1 in blood cells measured by pyrosequencing in 443 patient samples. Results: Basal and stimulated IGF-1 concentrations, first year increment in height velocity and studentized residuals of a prediction model did not correlate to the methylation of -137 und +97 in IGF1 P2 promoter. The methylation of these two sites was relatively stable during treatment. Conclusions: This study did not confirm IGF1 P2 promotor being a major epigenetic locus for GH responsiveness in patients treated with a normal dose of rhGH. Additional studies are warranted.
Subject(s)
DNA Methylation , Human Growth Hormone , Insulin-Like Growth Factor I , Turner Syndrome , Body Height/genetics , Child , Human Growth Hormone/deficiency , Human Growth Hormone/therapeutic use , Humans , Infant, Small for Gestational Age , Insulin-Like Growth Factor I/genetics , Insulin-Like Growth Factor I/metabolism , Promoter Regions, Genetic , Turner Syndrome/drug therapy , Turner Syndrome/genetics , Turner Syndrome/metabolismABSTRACT
OBJECTIVE: Neurosecretory dysfunction (NSD) causes growth hormone deficiency (GHD). Data on adult height after recombinant human growth hormone (rhGH) treatment are lacking. DESIGN AND PATIENTS: We collected treatment data of all patients with NSD seen between 1990 and 2017 at our outpatient department (tertiary centre) and measured adult height. For comparison, patients with idiopathic GHD were used. Diagnoses were based on short stature (<-2 standard deviation score [SDS]), continuously low height velocity (<25th percentile), delayed bone age (by >1 SD) and low serum IGF-1 concentration (<-2 SDS). NSD was defined by normal GH challenge results, but subnormal spontaneous GH secretion. Exclusion criteria were no information on adult height, underweight and other short stature disorders. RESULTS: Out of 67 patients diagnosed with NSD, six were still growing, 31 had test results exceeding validated GH cut-offs and three had other disorders causing short stature. Out of the 25 eligible patients with NSD, 21 could be recruited. These patients reached an adult height of -0.85 SDS (mean); 0.34 SDS below midparental height. Height gain during treatment was 2.01 SDS. This outcome was not different to 32 patients with idiopathic GHD. CONCLUSIONS: Long-term results suggest the viability of the diagnosis of NSD and the efficacy of rhGH treatment.
Subject(s)
Dwarfism, Pituitary , Human Growth Hormone , Adult , Body Height , Growth Disorders , Human Growth Hormone/therapeutic use , Humans , Recombinant ProteinsABSTRACT
CONTEXT: Aromatase excess syndrome (AEXS) is a very rare disorder characterized by prepubertal gynecomastia, bone age acceleration, and early growth arrest. Heterozygote submicroscopic rearrangements within the promotor of CYP19A1 result in overexpression of aromatase and enhanced aromatization of androgens. OBJECTIVE: The objective was to study long-term treatment effects of an aromatase inhibitor. METHODS: Data from 7 boys with AEXS were retrospectively collected. Genetic analysis revealed upstream of CYP19A1 a 165 901 bp deletion in 4 German cousins, a 198 662 bp deletion in 2 Japanese brothers, and a 387 622 bp tandem duplication in a Japanese boy. RESULTS: All boys developed prepubertal gynecomastia, at median 9.0 years of age (range: 7.0-11.0). Height was +1.20 standard deviation score (SDS) (-0.24 to +1.98); predicted adult height was -1.29 SDS (-3.29 to +1.09). Four boys were treated with 1.0 mg of anastrozole daily, while 3 reached adult height untreated. Treatment with anastrozole was stopped after 5.6 years (4.0-6.8). Three treated boys exceeded their prognosis by 2.4, 6.9, and 8.1 cm, while 1 untreated boy fell below the prognosis by 8.6 cm. One treated with a low dose and 2 untreated reached their prognosis. Adult heights were -0.91 SDS with anastrozole (-2.86 to -0.29) and -0.15 SDS without (-2.31 to -0.03). Distance to target height was -0.22 SDS with anastrozole (-1.72 to +0.52) and +0.54 SDS without (+0.23 to +1.30). CONCLUSION: Spontaneous growth in AEXS varied, even in the same family. Our data suggest that early started, long-term inhibition by anastrozole promotes adult height in boys with AEXS.
Subject(s)
46, XX Disorders of Sex Development/drug therapy , Aromatase Inhibitors/therapeutic use , Aromatase/genetics , Child Development/drug effects , Gynecomastia/drug therapy , Infertility, Male/drug therapy , Metabolism, Inborn Errors/drug therapy , Adolescent , Anastrozole/pharmacology , Anastrozole/therapeutic use , Aromatase/metabolism , Aromatase Inhibitors/pharmacology , Body Height/drug effects , Child , Germany , Humans , Japan , Male , Retrospective Studies , Siblings , Time FactorsABSTRACT
OBJECTIVE: Dietary proteins raise blood glucose levels; dietary fats delay this rise. We sought to assess the insulin amount required to normalize glucose levels after a fat- and protein-rich meal (FPRM). METHODS: Sixteen adolescents (5 female) with type 1 diabetes (median age: 18.2 years; range: 15.2-24.0; duration: 7.1 years; 2.3-14.3; HbA1c: 7.2%; 6.2-8.3%) were included. FPRM (carbohydrates 57 g; protein 92 g; fat 39 g; fibers 7 g; calories 975 Kcal) was served in the evening, with 20 or 40% extra insulin compared to a standard meal (SM) (carbohydrates 70 g; protein 28 g; fat 19 g; fibers 10 g; calories 579 Kcal) or carbohydrates only. Insulin was administered for patients on intensified insulin therapy or as a 4-hour-delayed bolus for those on pump therapy. The 12-hour post-meal glucose levels were compared between FPRM and SM, with the extra insulin amount calculated based on 100 g proteins as a multiple of the carbohydrate unit. RESULTS: Glucose levels (median, mg/dL) 12-hour post-meal with 20% extra insulin vs. 40% vs. insulin dose for SM were 116 vs. 113 vs. 91. Glucose-AUC over 12-hour post-meal with 20% extra insulin vs. 40% vs. insulin dose for SM was 1603 mg/dL/12 h vs. 1527 vs. 1400 (no significance). Glucose levels in the target range with 20% extra insulin vs. 40% were 60% vs. 69% (p=0.1). Glucose levels <60 mg/dL did not increase with 40% extra insulin. This corresponds to the 2.15-fold carbohydrate unit for 100 g protein. CONCLUSIONS: We recommend administering the same insulin dose given for 1 carbohydrate unit (10 g carbs) to cover 50 g protein.
Subject(s)
Diabetes Mellitus, Type 1/drug therapy , Diabetes Mellitus, Type 1/metabolism , Dietary Fats/metabolism , Dietary Proteins/metabolism , Hypoglycemic Agents/administration & dosage , Insulin/administration & dosage , Meals , Adolescent , Adult , Female , Humans , Male , Young AdultABSTRACT
OBJECTIVE: Severe growth hormone deficiency causes lean body mass loss in male adolescents and increased fat mass in both sexes. The changes appear after a 6 month GH pause. AIM: The aim was to examine bone density and structure changes in adolescents with severe GHD during a 6-month rhGH treatment interruption. PATIENTS AND METHODS: In total, 113 adolescents (20 females) paused rhGH treatment for 6 months at near-final height, and they were retested with arginine-GHRH challenge and basal IGF-1. Severe GHD was diagnosed in 19 individuals (5 females, GH peak <16 ng/ml and IGF-1 < -1.9 SDS) and excluded in 94 (15 females). Bone density and structure were measured by pQCT of the forearm and DXA of the total body at cessation of rhGH and 6 months later. RESULTS: In severe adolescent GHD (sGHD) patients, trabecular density (mg/cm3) decreased from 214 to 202 (p < 0.01); changes in the adolescents with normal test results (tGHD) were from 221 to 214 (p < 0.05). Cortical density (mg/cm3) increased from 1077 to 1099 (p < 0.01) in sGHD patients and from 1060 to 1082 in tGHD patients (p < 0.001). The strength strain index (mm3) showed no significant changes in sGHD patients (306 to 307) but changed from 302 to 315 in tGHD patients (p < 0.05). Total bone area (mm2) shifted from 145.1 to 145.2 in sGHD patients and from 153 to 156 in tGHD patients. Total body aBMD (g/cm2) increased in both groups: from 1.10 to 1.12 in sGHD patients and from 1.11 to 1.14 in tGHD patients (p < 0.01). All bone measurements remained within the reference ranges, and there were no differences between sGHD and tGHD patients. CONCLUSION: During a 6-month pause of rhGH treatment, the bone structure and density of adolescents with sGHD did not show changes implying harm. Routine retesting of adolescents, including 6 months without GH, is unlikely to be detrimental to the bone.
Subject(s)
Bone Density , Human Growth Hormone , Adolescent , Body Composition , Female , Growth Disorders , Growth Hormone , Humans , Insulin-Like Growth Factor I/metabolism , MaleABSTRACT
BACKGROUND: Pathogenic CDKN1C gain-of-function variants on the maternal allele were initially reported as a cause of IMAGe syndrome characterized by intrauterine growth retardation, metaphyseal dysplasia, primary adrenal insufficiency and genital anomalies. Recently, a maternally inherited CDKN1C missense mutation (p.Arg279Leu) was identified in several members of a single family clinically diagnosed with Silver-Russell syndrome (SRS) but without adrenal insufficiency. Thereafter, two half siblings from UK with familial SRS were described who carried the same mutation. This specific amino acid change is located within a narrow functional region containing the mutations previously associated with IMAGe syndrome. RESULTS: Here, we describe a third familial case with maternally inherited SRS due to a missense variant affecting the same amino acid position 279 but leading to a different amino acid substitution (p. (Arg279Ser)). The two affected family members (mother and son) presented with the complete SRS phenotype (both Netchine-Harbison CSS score 5 of 6) but without body asymmetry or adrenal insufficiency. CONCLUSIONS: In comparison with loss-of-function genomic IGF2 mutations, CDKN1C gain-of-function mutations are a less frequent cause of SRS and seem to affect a cluster of few amino acids.
Subject(s)
Adrenal Insufficiency/genetics , Cyclin-Dependent Kinase Inhibitor p57/genetics , Fetal Growth Retardation/genetics , Insulin-Like Growth Factor II/genetics , Osteochondrodysplasias/genetics , Silver-Russell Syndrome/genetics , Urogenital Abnormalities/genetics , Adrenal Insufficiency/diagnosis , Alleles , Amino Acid Substitution/genetics , Child, Preschool , Female , Fetal Growth Retardation/diagnosis , Genetic Variation/genetics , Heterozygote , Human Growth Hormone/therapeutic use , Humans , Male , Mothers , Mutation, Missense , Osteochondrodysplasias/diagnosis , Pedigree , Phenotype , Silver-Russell Syndrome/diagnosis , Silver-Russell Syndrome/drug therapy , Treatment Outcome , Urogenital Abnormalities/diagnosisABSTRACT
OBJECTIVE: Restarting rhGH in adolescents with childhood-onset (CO-) GHD is usually based on GH retest, IGF-1, additional pituitary hormone deficiencies, pituitary morphology and history. Short-term changes in body composition in adolescents with CO-GHD when off rhGH may contribute to the identification of those in need of treatment continuation. DESIGN: This is a longitudinal single-centre study. PATIENTS AND MEASUREMENTS: The body composition of 90 male adolescents with low-likelihood severe GHD of adolescence was measured by DXA at the time of rhGH discontinuation and 6 months thereafter. At diagnosis, mean age was 5.4 years, height was -2.68 SDS and stimulated GH peak was 5.1 ng/mL. RhGH treatment was stopped at 16.7 years at near-final height of -0.44 SDS. The adolescents were re-examined after 3 months off rhGH using both IGF-1 and GHRH-arginine tests. Severe GHD of adolescence was defined both by stimulated GH < 16 ng/mL and by IGF-1 < -1.90 SDS. RESULTS: Males with severe GHD of adolescence (n = 8) gained more relative and absolute fat mass and lost significantly more relative lean body mass after 6 months off rhGH than healthy individuals (n = 82; P < 0.001). The sum of absolute fat mass gain and lean body mass loss (=body composition changes score; BCC score) correlated highly with the GH peak (R = 0.17; P < 0.001). A BCC score >7.0 kg was 88% sensitive and 94% specific for detecting severe GHD of adolescence (AUC = 0.975). CONCLUSIONS: Short-term body composition changes when off rhGH are good clinical markers of severe GHD in male adolescents. The novel BBC score is an aggregate of these changes.
Subject(s)
Body Composition , Human Growth Hormone/deficiency , Absorptiometry, Photon , Adolescent , Age of Onset , Human Growth Hormone/therapeutic use , Humans , Insulin-Like Growth Factor I/analysis , Longitudinal Studies , Male , Substance Withdrawal SyndromeSubject(s)
Diabetes Mellitus, Type 1/diagnosis , Diabetes Mellitus, Type 1/therapy , Adolescent , Child , Female , Humans , Male , Practice Guidelines as TopicABSTRACT
Background Children with non-acquired (na) growth hormone deficiency (GHD) diagnosed over decades in one center may provide perspective insight. Methods naGHD is divided into idiopathic GHD (IGHD), GHD of known cause (cGHD) and GHD neurosecretory dysfunction (NSD); time periods: <1988 (I); 1988-1997 (II); 1998-2007 (III); 2008-2015 (IV). Descriptive analyses were performed at diagnosis and during first year GH treatment. Results Patients (periods, N): I, 87; II, 141; III, 356; IV, 51. In cGHD (all), age, maximum GH, insulin-like growth factor-I (IGF-I), and insulin-like growth factor-binding protein-3 (IGFBP-3) (5.1 years, 3.6 µg/L, -5.3 standard deviation score [SDS], -3.7 SDS) were lower than in IGHD (all) (6.8 years 5.8 µg/L, -2.5 SDS, -1.0 SDS), but not height (-3.1 vs. -3.2 SDS). Characteristics of NSD were similar to that of IGHD. Patients with IGHD - not cGHD - diagnosed during 2008-2015 (IV) were the youngest with most severe GHD (maxGH, IGF-I, IGFBP-3), and first year height velocity (HV) and ∆ IGF-I (10.5 cm/year, 4.0 SDS) but not ∆ height SDS were the highest on recombinant human growth hormone (rhGH) (27 µg/kg/day). Conclusions Although during 1988-2007 patient characteristics were similar, the recently (>2008) stipulated more stringent diagnostic criteria - HV before testing, sex steroid priming, lower GH cut-off - have restricted diagnoses to more severe cases as they were observed before the rhGH era.
Subject(s)
Body Height/drug effects , Growth Disorders/drug therapy , Human Growth Hormone/therapeutic use , Hypopituitarism/drug therapy , Adolescent , Child , Child, Preschool , Female , Human Growth Hormone/deficiency , Human Growth Hormone/pharmacology , Humans , Male , Treatment OutcomeABSTRACT
The objective of this study was to determine the major nutrient composition of Indian rhinoceros milk ( Rhinoceros unicornis) over the first 13 mo of an 18-mo lactation period and to compare the results to those of previous studies on rhinoceros, African elephant ( Loxodonta africana), and horse milk ( Equus ferus caballus). The following parameters were measured: dry matter (DM), crude ash (ASH), crude protein (CP), ether extract (EE), nitrogen-free extract (NFE; calculated), lactose, calcium (Ca), phosphorus (P), magnesium (Mg), fatty acids (FAs), and gross energy (GE). DM, ASH, CP, and EE were determined with a proximate analysis, lactose with infrared spectroscopy and an enzymatic method, minerals with an autoanalyzer, FA with gas chromatography, and GE with bomb calorimetry. Milk samples were collected from two Indian rhinoceros cows from Zoo Basel. Rhino A gave birth to her third calf on 10 September 2012; three samples were collected and analyzed (colostrum, milk 1 wk and 2 wk postpartum). Rhino B gave birth to her eighth calf on 05 October 2013; samples were collected and 15 were chosen for the analyses (from colostrum to 13 mo postpartum). The composition of rhino B's colostrum was 13.8% DM (wet-weight basis), 4.8% ASH, 61.8% CP, 0.7% EE, 32.6% NFE, 26.7% lactose, 0.59% Ca, 0.54% P, 0.2% Mg (DM basis), and 20.3 MJ GE/kg DM. Rhino B's sample collected 13 mo postpartum averaged 8.0% DM (wet-weight basis), 3.6% ASH, 16.3% CP, 1.8% EE, 78.3% NFE, 84.7% lactose, 0.54% Ca, 0.48% P, 0.09% Mg (on DM basis), and 17.43 MJ GE/kg DM. The main FAs in rhino B's and rhino A's samples were C10 : 0, C12 : 0, C16 : 0, C18 : 1n9c, and C18 : 2n6c. Milk of the Indian rhinoceros is low in fat and protein but high in lactose, which is comparable to the milk composition of other rhinoceros species and horses, but not African elephants.
Subject(s)
Lactation/physiology , Milk/chemistry , Perissodactyla/physiology , Animals , Animals, Zoo , Fatty Acids/chemistry , Fatty Acids/metabolism , FemaleABSTRACT
BACKGROUND: Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA. METHODS: A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire. RESULTS: Eleven patients were included in the study. The median age at surgery was 6 months (range: 3-72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n = 6), ileorectal anastomosis (n = 1), J-pouch (n = 1) and Duhamel procedure (n = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27-199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life. CONCLUSION: The majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.