Zaburzenia oddychania w czasie snu u pacjentów z zespołem Pradera-Willego w zależności od okresu leczenia hormonem wzrostu

Introduction: Sleep-related breathing disorders (SRBD) are commonly present in patients with Prader-Willi syndrome (PWS). Recombinant human growth hormone (rhGH) treatment is reported to improve breathing function in PWS, but the findings are not explicit. Material and methods: Screening polysomnography- polygraphy (PSG), assessing nasal respiratory flow, respiratory effort, and blood oxygen saturation, was used. Group 1 — before rhGH therapy (n = 11, mean age 3.0 years); PSG was repeated after the start of rhGH therapy in a mean time of 0.9 years in six patients (Group 1a). Group 2 — on rhGH treatment, for a mean time of four years (n = 17, mean age 8.8 years). Group 3 — without rhGH therapy due to severe obesity (n = 8, mean age 13.1 years). Results: Group 1 — mean apnoea-hypopnoea index (AHI) was 10.2, oxygen desaturation index (ODI)- 36.3, Group 1a- AHI 12.0, ODI 60.9, Group 2-AHI 9.0, ODI 25.1, Group 3- AHI 8.2, ODI 22.0. ODI was significantly higher in Group 1a than in the other groups (p < 0.005), but not strictly related to SRBD. The results in Group 2 did not differ significantly from those of Group 1. Conclusions: Our study proves the high frequency of SRBD among PWS patients, with worsening of ODI after short-term rhGH therapy.Wstęp: Zaburzenia oddychania w czasie snu (sleep-related breathing disorders, SRBD) występują często u pacjentów z zespołem Pradera­-Willego (PWS). Opisywana jest poprawa funkcji oddechowych w trakcie leczenia preparatem ludzkiego rekombinowanego hormonu wzrostu (GH), jednak dane te nie są jednoznaczne. Materiał i metody: Przesiewowa ocena polisomnograficzna — poligrafia (PSG), oceniająca przepływ powietrza w trakcie oddychania, wysiłek oddechowy, saturację. Grupa 1 — przed leczeniem GH (n = 11, średni wiek 3 lata); PSG powtórzono po rozpoczęciu leczenia GH średnio po 0,9 roku u 6 pacjentów (grupa 1a). Grupa 2 — w trakcie leczenia GH, większość pacjentów &gt; 4 lata terapii (n = 17, średni wiek 8,8 roku). Grupa 3 — bez leczenia GH z powodu znacznej otyłości (n = 8, średni wiek 13,1 roku). Wyniki: Grupa 1 — średni wskaźnik bezdech-spłycenie oddychania (apnea-hypopnea index, AHI) wynosił 10,2, wskaźnik desaturacji (oxygen desaturation index, ODI) — 36,3, group 1a — AHI 12,0, ODI 60,9, group 2 — AHI 9,0, ODI 25,1, group 3 — AHI 8,2, ODI 22,0. ODI było znacząco wyższe w grupie 1a w porównaniu do pozostałych grup (p &lt; 0,005), ale nie wykazywało ścisłego związku z pozosta­łymi wskaźnikami SRDB. Porównanie wyników grupy 2 i grupy 1 nie wykazało różnic istotnych statystycznie. Wnioski: Przedstawione wyniki potwierdzają wysoką częstość występowania SRBD u pacjentów PWS, z istotnym pogorszeniem ODI po krótkim okresie leczenia GH

Diabetic Retinopathy in Children with Type 1 Diabetes—Occurrence and Screening Using Optical Coherence Tomography

Purpose: To describe the occurrence of diabetic retinopathy, the principles for pediatric care of patients with diabetes, and the utility of optical coherence tomography. Pediatric patients with type 1 diabetes should be screened for diabetic retinopathy upon the lapse of 5 years following the diagnosis. The patients in the time of puberty, who should be screened promptly after the diabetes diagnosis, and patients with type 2 diabetes are the exceptions. Special attention must be paid not only to retinopathy, but also to other possible concomitant conditions, such as cataract, refractive errors, or neuropathy. New techniques, such as optical coherence tomography angiography (OCTA), may contribute greatly to the early detection of retinopathy, facilitating the decision to modify the treatment. The application of modern insulin pumps with continuous glucose monitoring systems has greatly diminished the incidence rate of early symptoms of diabetic retinopathy in the pediatric population

Gender-Specific Risk Factors for the Development of Retinal Changes in Children with Type 1 Diabetes

The aim of the study was to determine gender-specific risk factor sets which could influence optical coherence tomography (OCT) results in children with type 1 diabetes (T1D). Material and Methods: 175 children with T1D without symptoms of diabetic retinopathy were enrolled, but 330 eyes were used for the final analysis (168 children, mean age 12.81 ± 3.63 years, diabetes duration 4.59 ± 3.71 years). The multivariate regression models for retinal thickness (foveal FT, and parafoveal PFT) and vascular densities (superficial and deep) were carried out separately for both genders using all metabolic and demographic parameters. Results: In the statistically significant multiple regression models for all analyzed OCT parameters for both genders, pH at the onset of diabetes were in existence, as well as for retinal thickness current HbA1c. Duration of continuous insulin infusion (CSII) was an important factor in all parameters, except PFT. For the girls, the most significant factors were daily insulin dose, uric acid, and triglycerides, but for the boys, it was serum creatinine, systolic pressure, and free thyroxine level. Conclusions: We detected significant risk factors set for development of OCT parameters changes, and they were not identical for both genders. Current metabolic control, diabetic ketoacidosis at the disease onset, serum creatinine and longer use of CSII are the most important factors for retinal thickness and vessel densities in both genders in children with type 1 diabetes. For the girls, elements of metabolic syndrome (uric acid and triglycerides) and parameters of insulin amount were more pronounced

Thyroid Hormones, Peripheral White Blood Count, and Dose of Basal Insulin Are Associated with Changes in Nerve Conduction Studies in Adolescents with Type 1 Diabetes

Type 1 diabetes (T1D) in the child population is the third most common chronic disease. Diabetic peripheral neuropathy (DPN) is a very disabling and silently developing complication. This prospective, observational study enrolled 182 (93 girls) patients with T1D, aged 16.5&ndash;18 years. The aim of the study was to assess the correlation between factors of diabetes metabolic control, blood count, thyroid hormones, thyroid-stimulating hormone (TSH), level of cortisol, vitamin D3, metabolic factors, demographic data, and nerve conduction study (NCS) parameters. We revealed that in multivariate regression models for almost all NCS parameters, beside height and diabetes duration, significant factors were basal insulin dose per kilogram of weight (BID/kg), body mass index (BMI), and thyroid hormones. For conduction velocities of the motor nerves, mean HbA1c exists in models. In all models for all NCS parameters there exists at least one parameter of peripheral white blood cell counts (predominantly monocytes). There is a significant influence of thyroid hormones, peripheral blood white cells count, and BID per weight on parameters of NCS. It is essential to take care of the proper insulin dose per weight of patients and the adequate proportion of basal to prandial insulin

Foot Plantar Pressure Abnormalities in Near Adulthood Patients with Type 1 Diabetes

Increased ulcer risk diminishes the quality of life in diabetes. This study assessed abnormalities in foot plantar pressure distribution in adolescents with T1D to detect early signs of ulcer risk. A total of 102 T1D patients, without diabetic neuropathy, were included (mean age 17.8 years, mean diabetes duration 7.4 year). Pedography was captured using Novel emed. Data from the study group were compared with reference data. The study revealed a statistically significant reduced foot contact area in both feet in the entire foot and under the head of the fifth metatarsal bone and the second toe. In both feet, the peak pressure was increased under the entire foot, hindfoot, midfoot, first metatarsal head, big toe, and second toe. There was no statistically significant difference in peak pressure. The mean plantar pressure rating was statistically significantly increased in both feet across the entire sole, in the hindfoot, midfoot, and first metatarsal head. T1D patients of age near adulthood without neuropathy have increased values in mean pressure and reduced contact area, pointing to the need of monitoring and preventive measures. These results point to the need of further research and analysis which should include various risk factor such as foot anatomy, body posture, or certain metabolic factors

Comparison of Frequency and Severity of Sleep-Related Breathing Disorders in Children with Simple Obesity and Paediatric Patients with Prader–Willi Syndrome

Sleep-related breathing disorders (SRBDs) can be present in children with simple obesity and with Prader–Willi syndrome (PWS) and influence an individual diagnostic and treatment approach. We compared frequency and severity of SRBDs in children with simple obesity and with PWS, both without and on recombinant human growth hormone (rhGH) treatment, and correlation of SRBDs with insulin resistance tests. A screening polysomnography-polygraphy (PSG), the oral glucose tolerance test (OGTT) and homeostasis model assessment of insulin resistance (HOMA-IR) were analysed in three groups of patients—with simple obesity (group 1, n = 30, mean age 14.2 years), patients with PWS without the rhGH therapy (group 2, n = 8, mean age 13.0 years) and during the rhGH treatment (group 3, n = 17, mean age 8.9 years). The oxygen desaturation index (ODI) was significantly higher in groups 2 and 3, compared to group 1 (p = 0.00), and hypopnea index (HI) was higher in group 1 (p = 0.03). Apnea–hypopnea index (AHI) and apnea index (AI) results positively correlated with the insulin resistance parameters in groups 1 and 3. The PSG values worsened along with the increasing insulin resistance in children with simple obesity and patients with PWS treated with rhGH that may lead to a change in the patients’ care

Influence of Metabolic Parameters and Treatment Method on OCT Angiography Results in Children with Type 1 Diabetes

Aim. To evaluate the influence of metabolic parameters and the treatment method in children with type 1 diabetes (T1D) on the optical coherence tomography angiography (OCTA) results as early markers of diabetic retinopathy (DR). Material and Methods. This prospective study enrolled 175 consecutive children with T1D. OCTA was performed using AngioVue (Avanti, Optovue). Whole superficial capillary vessel density (wsVD), fovea superficial vessel density (fsVD), parafovea superficial vessel density (psVD), whole deep vessel density (wdVD), fovea deep vessel density (fdVD), parafovea deep vessel density (pdVD), foveal thickness (FT), parafoveal thickness (PFT), and foveal avascular zone (FAZ) in superficial plexus were evaluated and analyzed in relation to individual characteristics, i.e., sex, weight, height, body mass index (BMI), and metabolic factors: current and mean value of glycated hemoglobin A1c (HbA1c). Furthermore, the analysis concerned the diabetes duration, age at the T1D onset, and type of treatment—multiple daily insulin injections (MDI) or continuous subcutaneous insulin infusion (CSII). Results. In the study group, we did not identify any patient with DR in fundus ophthalmoscopy. Age at the onset of diabetes correlated negatively with FAZ (r=−0.17, p<0.05). The higher level of HbA1c corresponded to a decrease of wsVD (r=−0.13, p<0.05). We found significantly lower fsVD (32.25±.1 vs. 33.98±.1, p<0.01), wdVD (57.87±.1 vs. 58.64±.9, p<0.01), and pdVD (60.60±.2 vs. 61.49±.1, p<0.01) and larger FAZ area (0.25±.1 vs. 0.23±.1, p<0.05) in the CSII vs. MDI group. Conclusion. The metabolic parameters, age of the onset of diabetes, and treatment method affected the OCTA results in children with T1D. Further studies and observation of these young patients are needed to determine if these findings are important for early detection of DR or predictive of future DR severity

Associations of nerve conduction parameters and OCT angiography results in adolescents with type 1 diabetes.

AimTo evaluate dependence of abnormalities in peripheral nerves and retina in children with type 1 diabetes (T1D) using optical coherence tomography angiography (OCTA) and nerve conduction studies (NCS).Material and methods50 adolescents with T1D without any signs and symptoms of diabetic retinopathy and neuropathy (mean age 16.92±1.6 years, diabetes duration 6.88 ±4.34years) were included. In OCTA capillary plexuses superficial (SCP) and deep (DCP) vessel density: whole, foveal and parafoveal, ganglion cell complex (GCC), loss volume focal (FLV) and global loss volume (GLV) were analyzed in relation to NCS parameters (motor nerves median and tibial potential amplitude (CMAP), velocity (CV), distal latency (DML) and F wave and sensory nerves median and sural potential amplitude (SNAP), CV and distal latency (DSL).ResultsWe detected the correlations between median sensory SNAP and GCC (r = -0.3, p ConclusionsIn adolescents with T1D without diabetic neuropathy and retinopathy we detected associations between NCS and OCT and OCTA parameters, regarding decreased GCC and density of superficial and deep vessel plexuses in relation to DML and CV and amplitudes of sensory and motor potential

Choroidal Thickness and Ganglion Cell Complex in Pubescent Children with Type 1 Diabetes without Diabetic Retinopathy Analyzed by Spectral Domain Optical Coherence Tomography

Aim. To assess the retinal and choroidal thickness and ganglion cell complex (GCC) in pubescent children with type 1 diabetes (T1D) without diabetic retinopathy (DR), using spectral domain optical coherence tomography (SD-OCT). Materials and Method. Sixty-four right eyes of 64 subjects with T1D and 45 right eyes of 45 age-matched healthy volunteers (control group) were enrolled in this study. The mean age of the subjects and controls was 15.3 (±SD = 2.2) and 14.6 (±SD = 1.5), respectively. SD-OCT was performed using RTVue XR Avanti. Ganglion cell complex (GCC), GCC focal loss volume (FLV), GCC global loss volume (GLV), choroidal thickness (CT), foveal (FT) and parafoveal thickness (PFT), and foveal (FV) and parafoveal volume (PFV) data were analyzed. Results. There was no significant difference between subjects and controls in the CT in the fovea and nasal, temporal, superior, and inferior quadrants of the macula. There were no significant correlations between CT, duration of diabetes, and HbA1C level (p=0.272 and p=0.197, resp.). GCC thickness did not differ significantly between the groups (p=0.448), but there was a significant difference in FLV (p=0.037). Significant differences between the groups were found in the PFT and PFV (p=0.004 and p=0.005, resp.). There was a significant negative correlation between PFT, PFV, and HbA1C level (p=0.002 and p=0.001, resp.). Conclusions. Choroidal thickness remains unchanged in children with T1D. Increased GCC FLV might suggest an early alteration in neuroretinal tissue. Parafoveal retinal thickness is decreased in pubescent T1D children and correlates with HbA1C level. OCT can be considered a part of noninvasive screening in children with T1D and a tool for early detection of retinal and choroidal abnormalities. Further OCT follow-up is needed to determine whether any of the discussed OCT measurements are predictive of future DR severity

Lack of effect of Lactobacillus rhamnosus GG and Bifidobacterium lactis Bb12 on beta-cell function in children with newly diagnosed type 1 diabetes: a randomised controlled trial

Introduction The gut microbiota may be relevant in the development of type 1 diabetes (T1D). We examined the effects of Lactobacillus rhamnosus GG and Bifidobacterium lactis Bb12 on beta-cell function in children with newly diagnosed T1D.Research design and methods Children aged 8–17 years with newly (within 60 days) diagnosed T1D were enrolled in a double-blind, randomised controlled trial in which they received L. rhamnosus GG and B. lactis Bb12 at a dose of 109 colony-forming units or placebo, orally, once daily, for 6 months. The follow-up was for 12 months. The primary outcome measure was the area under the curve (AUC) of the C-peptide level during 2-hour responses to a mixed meal.Results Ninety-six children were randomised (probiotics, n=48; placebo n=48; median age 12.3 years). Eighty-eight (92%) completed the 6-month intervention, and 87 (91%) completed the follow-up at 12 months. There was no significant difference between the study groups for the AUC of the C-peptide level. For the secondary outcomes at 6 months, there were no differences between the study groups. At 12 months, with one exception, there also were no significant differences between the groups. Compared with the placebo group, there was a significantly increased number of subjects with thyroid autoimmunity in the probiotic group. However, at baseline, there was also a higher frequency of thyroid autoimmunity in the probiotic group. There were no cases of severe hypoglycemia or ketoacidosis in any of the groups. No adverse events related to the study products were reported.Conclusions L. rhamnosus GG and B. lactis Bb12, as administered in this study, had no significant effect in maintaining the residual pancreatic beta-cell function in children with newly diagnosed T1D. It remains unclear which probiotics, if any, alone or in combination, are potentially the most useful for management of T1D.Trial registration number NCT03032354