Effect of steroid therapy in a 16-year-old girl observed for lymphocytic hypophysitis.

Not required for Clinical Vignette.

Long-term Survival in a Child with Malignant Insulinoma After Liver Transplantation.

Insulinoma belongs to pancreatic neuroendocrine tumors and is immensely rare in children. The tumor leads to severe consequences of hypoglycemia caused by excessive insulin release. We report a pediatric patient with malignant insulinoma linked with liver transplantation due to metastases to this organ. A 13-year-old girl presented with symptoms of hypoglycemia due to hyperinsulinism. In computed tomography (CT), a polycyclic lesion in the head of the pancreas and enlarged lymph nodes were revealed. Modified Whipple's operation was performed, and histological examination confirmed pancreatic neuroendocrine tumor. CT of the body showed an enlarged liver with numerous metastases. Allogeneic liver transplantation was carried out successfully. Positron emission tomography-computed tomography (PET/CT) using 68Ga-DOTA-labeled somatostatin analogs at the age of 22 confirmed the complete metabolic remission. The patient currently remains under immunosuppressive and antiproliferative treatment. Multiple surgical interventions, liver transplantation combined with somatostatin analogs, and immunosuppressive medication could be effective in malignant insulinoma.

Pituitary Stalk Thickening: Causes and Consequences. The Children's Memorial Health Institute Experience and Literature Review.

Background: Pituitary stalk thickening (PST) is a rare abnormality in the pediatric population. Its etiology is heterogeneous. The aim of the study was to identify important clinical, radiological and endocrinological manifestations of patients with PST and follow the course of the disease. Materials and Methods: It is a study conducted in 23 patients (13 boys) with PST with/without central diabetes insipidus (CDI) diagnosed between 1990 and 2020 at Children's Memorial Health Institute (CMHI) in Warsaw, Poland. We analyzed demographic data, clinical signs and symptoms, radiological findings, tumor markers, hormonal results, treatment protocols and outcomes. Results: The median age at the diagnosis of PST was 9.68 years (IQR: 7.21-12.33). The median time from the onset of the symptoms to the diagnosis was 2.17 years (IQR: 1.12-3.54). The most common initially reported manifestations were polydipsia, polyuria and nocturia (82.6%); most of the patients (56.5%) also presented decreased growth velocity. Hormonal evaluation at the onset of PST revealed: CDI (91.3%), growth hormone deficiency (GHD) (56.5%), hyperprolactinemia (39%), central hypothyroidism (34.8%), adrenal insufficiency (9%), precocious puberty (8.7%). The majority of the patients were diagnosed with germinoma (seventeen patients - 73.9%, one of them with teratoma and germinoma). Langerhans cell histiocytosis (LCH) was identified in three patients (multisystem LCH in two patients, and unifocal LCH in one patient). A single case of atypical teratoid rhabdoid tumor, suspected low-grade glioma (LGG) and lymphocytic infundibuloneurohypophysitis (LINH). The overall survival rate during the observational period was 87.0%. Conclusions: The pituitary infundibulum presents a diagnostic imaging challenge because of its small size and protean spectrum of disease processes. Germinoma should be suspected in all children with PST, especially with CDI, even when neurological and ophthalmological symptoms are absent.

Pituitary stalk thickening in patients under 18 years of age - the most common causes and diagnostic procedures.

INTRODUCTION: Pituitary stalk thickening (PST) is a rare abnormality in children, and it may be challenging due to its diverse clinical picture. AIM OF THE STUDY: The aim of the study is to summarize the data on the causes and diagnostic procedures of PST. MATERIAL AND METHODS: Papers were searched in the PubMed database identifying published randomized clinical trials, reviews, systematic reviews, meta-analyses, and case reports. RESULTS: The most common causes of a thickened pituitary stalk in children are germ cell tumours (GCTs), Langerhans cell histiocytosis (LCH), and lymphocytic infundibulo-neurohypophysitis (LINH). Neurosarcoidosis, pituitary tuberculosis, granulomatosis, or specific inflammations were only reported in the paediatric population as case studies. PST mainly affects teenagers and is often detected with brain magnetic resonance imaging (MRI) in patients with central diabetes insipidus (CDI). It is not possible to differentiate the causes of PST with the use of the MRI image alone. Although various biochemical and oncological markers and other imaging tests are used, the diagnosis of PST remains a significant diagnostic challenge for clinicians. The final diagnosis is made based on histopathological examination. The indications for a biopsy are not uniform. Most experts, including the authors of the 2021 British consensus, recommend biopsy in the case of PST with a stalk lesion diameter ≥ 6.5-7 mm. CONCLUSIONS: The differential diagnosis of PST is a challenge. The diagnostic and treatment strategy should be individually adapted. Patients should be diagnosed in large clinical centres with experience in this field.

Influence of emotional intelligence on glycemic control in adolescents with diabetes type 1.

INTRODUCTION: Psychological factors can have a significant impact on diabetes control. We aimed to evaluate the correlation between emotional intelligence and glycemic control in type one diabetes (T1D) adolescents. MATERIAL AND METHODS: This prospective study enrolled 97 consecutive children admitted to our department and aged 15 to 17 with T1D. The Emotional Intelligence Questionnaire INTE was used to measure emotional intelligence. The results were correlated with a glycemic control status, measured by current and mean (since the diagnosis of T1D, minimum four tests per year) and hemoglobin A1c (HbA1c). An additional questionnaire collected the demographic and social data. RESULTS: Our study found a significant, negative correlation between HbA1c level and the ability to utilize emotions to support thinking and actions (Factor I of the INTE questionnaire). There was no significant correlation between emotional intelligence General Score or Factor II (the ability to recognize emotions) and glycemic control. CONCLUSIONS: A higher ability to utilize emotions to support thinking and actions positively correlates with metabolic control in the adolescent population with T1D. The appropriate emotional intelligence training and better psychological care may improve the metabolic outcomes of children with T1D. This merits further study.

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 16.5-18 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.

Endocrine disorders in a patient with a suspicion of a mitochondrial disease, MELAS syndrome - a case report and literature review.

MELAS syndrome (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes) is a genetically determined disease caused by mutations in mitochondrial DNA. We present a girl who was suspected of MELAS syndrome during the diagnostic evaluation of short stature. The patient suffered from symptoms potentially indicating mitochondrial disease, such as muscular weakness, cranial nerve VI palsy, headaches, retinitis pigmentosa, sensory-neural hearing loss, and elevated lactic acid. T2-weighted brain MRI showed hyperintense lesions in the white matter. Muscular biopsy revealed ragged red fibres. Genetic evaluation did not detect the most common mutations in the MT-TL1 gene and MT-ND5 gene. Endocrine tests led to the confirmation of growth hormone deficiency, and so replacement treatment was started. After 1 year of recombinant growth hormone therapy the patient was diagnosed with diabetes. At the age of 14 years the LH-RH test showed prepubertal values. Endocrine disorders may be one of the first manifestations of MELAS syndrome. In differential diagnosis of short stature, less common causes, such as mitochondrial diseases, should be taken into consideration.

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.

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

AIM: To 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 METHODS: 50 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). RESULTS: We detected the correlations between median sensory SNAP and GCC (r = -0.3, p <0.04), motor nerves tibial DML and CV and FLV (respectively r = -0.53, p<0.001, and r = -0.34, p<0.05), and median DML and GLV (r = 0.47, p<0.001). Vessel densities were related to changes in motor nerves tibial velocity (whole SCP r = 0.43, p <0.01, parafoveal SCP r = 0.41, p <0.01), CMAP (parafoveal SCP r = -0.35, p<0.03), median DML (whole DC r = 0.36, p<0.03, foveal DCP r = 0.37, p<0.02) and in sensory median SNAP (whole SCP r = -0.31, p<0.05). CONCLUSIONS: In 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.

Influence of puberty on retinal microcirculation in children with type 1 diabetes without retinopathy using optical coherence tomography angiography.

BACKGROUND: This study aimed to assess the influence of pubertal status on the results of optical coherence tomography angiography (OCTA) in children with type 1 diabetes (T1D). METHODS: We enrolled 167 consecutive children with T1D. Retinal superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density data underwent analysis. We divided the study population into three subgroups depending on the pubertal status. RESULTS: Analysis of the prepubertal and pubertal subgroups revealed statistically significant differences in foveal thickness (FT) (p < 0.05) and foveal SCP (p < 0.02). Analyzing subgroups of the prepubertal and postpubertal children, we observed statistically significant differences in FT (p < 0.03), whole SCP (p < 0.02), and foveal SCP (p < 0.02). Comparison of the pubertal and postpubertal subjects revealed differences in parafoveal DCP (p < 0.003). In the groups matched depending on diabetes duration, we observed differences between prepubertal, pubertal, and postpubertal children in FT, PFT, and parafoveal SCP and DCP. CONCLUSION: Our data suggest that in a cohort of pubertal children with a short duration of diabetes, alterations in retinal vessel density occur early and progress during puberty.

The effect of coexisting autoimmune thyroiditis in children with Type 1 diabetes on optical coherence tomography results.

OBJECTIVE: To assess the influence of thyroid hormones status and coexistence of autoimmune thyroiditis on optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) results in children with Type 1 diabetes (T1D). RESEARCH DESIGN AND METHODS: In the prospective, observational study (n = 175) we analyzed the impact of thyroid hormones on OCT results and the differences between the matched groups of children with T1D (n = 84; age = 13.14 ± 3.6; diabetes duration = 5.99 ± 3.3 years) and the children with T1D and autoimmune thyroiditis (AT) (n = 20; age = 13.94 ± 3.6; diabetes duration = 6.7 ± 4 years). We analyzed the following parameters: fovea avascular zone (FAZ), foveal thickness (FT), parafoveal thickness (PFT), ganglion cell complex (GCC), loss volume (global-GLV, focal-FLV), capillary vessel density: superficial (whole-wsVD, foveal-fsVD, parafoveal-psVD), and deep (whole-wdVD, foveal-fdVD, parafoveal-pdVD. The differences between the groups were tested by the unpaired t-Student test, Mann-Whitney U test as appropriate, whereas p level .05 was recognized as significant. RESULTS: We detected the significant correlations between thyroid-stimulating hormone (TSH) level and PFT (r = -0.14; p < .05), psVD (r = -0.18; p < .005). The level of free triiodothyronine (FT3) was correlated with psVD (r = -0.14; p < .05). We found significant correlation between free thyroxine (FT4) and fsVD (r = -0.17; p < .01). In the studied T1D and AT groups there were statistical differences in FT (p < .005), PFT (p < .03), GCC (p < .01), and GLV (p < .003). We did not observe any significant differences in the FAZ area between the groups. CONCLUSIONS: In our patients the co-occurrence of T1D and AT worsens the status of retinal parameters. Further studies are necessary to observe these relations and their potential influence on the occurrence of diabetic retinopathy (DR).

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.

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.

Peripheral Neuropathy as a Complication of Diabetic Ketoacidosis in a Child with Newly Diagnosed Diabetes Type 1: A Case Report

Neurological complications of diabetic ketoacidosis are considered to be a serious clinical problem. The most common complication is cerebral edema. However, these neurological complications also include less common entities such as ischemic or hemorrhagic stroke, cerebral venous and sinus thrombosis or peripheral neuropathy. We present a case of a 9-year old girl admitted to our intensive care unit with new onset type 1 diabetes, diabetic ketoacidosis, cerebral edema, multifocal vasogenic brain lesions and bilateral lower limb peripheral paresis. The patient developed polydipsia and polyuria one week before admission. The initial blood glucose level was 1136 mg/dL and severe acidosis was present (pH 7.1; BE-25.9). Computed tomography scan showed brain edema and a hypodense lesion in the left temporal region. Brain magnetic resonance imaging revealed more advanced multifocal brain lesions. Nerve conduction studies demonstrated damage of the motor neurons in both lower limbs with dysfunction in both peroneal nerves and the right tibial nerve. With treatment and physiotherapy, the patient's health gradually improved. Acute neuropathy after ketoacidosis is a rare complication and its pathogenesis is not clear. Patients with diabetic ketoacidosis require careful monitoring of neurological function, even after normalization of their glycemic parameters.

Optical coherence tomography angiography vessel density in children with type 1 diabetes.

PURPOSE: To assess the optical coherence tomography angiography (OCTA) retinal vessel density and foveal avascular zone (FAZ) in children with type 1 diabetes (T1D) and compare potential pathologic early changes in this population to healthy age-matched controls. METHODS: This study included 130 pubescent children: 94 with T1D (188 eyes) and 36 of their age-matched control group (60 eyes). OCTA was performed using AngioVue (Avanti, Optivue). FAZ area (mm2) in superficial plexus, 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) (µm) and parafoveal thickness (PFT) (µm) were taken into analysis. Among the studied patients with T1D there were assessed codependences regarding the investigated foveal and parafoveal parameters and selected potential predictors, i.e. patient's age (years), diabetes duration time (years), age of onset of the disease (years), mean level of glycated hemoglobin (HbA1C) (%), and concentration of serum creatinine (mg/dL). RESULTS: None of the abovementioned OCT and OCTA parameters was statistically significantly different between the groups. The patient's age statistically significantly did not influent any of the OCT and OCTA parameters. Yet an elevated level of HbA1C tended to reduce the parafovea superficial vessel density (p = 0.039), and parafoveal thickness (p = 0.003) and an increased serum creatinine level correlated with the decreased whole deep vessel density (p < 0.001). The parafovea deep vessel density in the diabetic patients decreased when the serum creatinine level (p = 0.008), age of onset of the disease (p = 0.028), and diabetes duration time (p = 0.014) rose. CONCLUSIONS: Vessel density, both in superficial and deep plexuses, and FAZ area are normal in pubescent children with T1D comparing to healthy subjects. An elevated level of HbA1C correlated with reduced psVD and PFT. Longitudinal observation of these young patients is needed to determine if any of these OCTA measurements are predictive of future DR severity.