Does metabolic control of the disease related with bone turnover markers in children with type 1 diabetes mellitus in Turkey?

BACKGROUND: The aim was to evaluate the effect of metabolic control on bone biomarkers in children with type I diabetes. MATERIALS AND METHODS: The children were divided into two groups according to their glycated hemoglobin (HbA1c) (%) levels: a group with HbA1c levels < 8% (n = 16) and: a group with HbA1c levels > 8% (n = 18). The serum total oxidative status (TOS) (µmol/L), total antioxidant status (TAS) (mmol/L), alkaline phosphatase (ALP) (IU/L), osteocalcin (OC) (ng/ml), procollagen type-1-N-terminal peptide (P1NP) (ng/ml), and vitamin D (IU) levels and food consumption frequencies were determined. RESULTS: When patients were classified according to HbA1c (%) levels, those with HbA1c levels < 8% were found to have lower TOS (µmol/L) values (8.7 ± 6.16, 9.5 ± 5.60) and higher serum OC (ng/mL) (24.2 ± 16.92, 22.0 ± 6.21) levels than those with HbA1c levels > 8% (p < 0.05). Regardless of the level of metabolic control, there was a statistically significant association between serum TOS (µmol/L) and P1NP (ng/ml) (p < 0.05) levels, with no group-specific relationship (HbA1c levels <%8 or HbA1c levels >%8). CONCLUSION: HbA1c and serum TOS levels had an effect on bone turnover biomarkers in individuals with type I diabetes.

Association of serum periostin levels with clinical features in children with asthma.

BACKGROUND: The aim of this study was to investigate the association of serum periostin levels with clinical features in children with asthma. METHODS: Children with physician-diagnosed asthma who attended regularly to an outpatient pediatric allergy and asthma center were enrolled in the study along with control subjects. Asthma severity and control status of the patients were evaluated according to the recent GINA guidelines. RESULTS: A total of 158 children (125 with asthma and 33 age- and sex-matched control subjects) with a median age of 10.2 years (range 5.9-17.0) were enrolled. Asthma severity was mild in 41 (32.8%), moderate in 63 (50.4%), and severe in 21 (16.8%) children. Children with asthma had significantly higher periostin levels than controls (53.1 ± 13.1 vs 43.0 ± 11.2 ng/mL, P < .001). The mean serum periostin levels in children with severe asthma (63.8 ± 10.8) were significantly higher than in children with moderate asthma (53.3 ± 12.7) and mild asthma (47.4 ± 11.1) (P < .001). Results of multivariable logistic regression analysis demonstrated an association between serum periostin levels and asthma severity in children (OR, 1.10; 95% CI, 1.04-1.15, P < .001). When analyzed for the best cut-off value with the highest combined sensitivity and specificity, a cut-off value of 52 ng/mL for serum periostin level was obtained with sensitivity, specificity, PPV, and NPV of 100%, 50%, 29%, and 100%, respectively. CONCLUSION: Although serum periostin levels are higher in children with asthma, its diagnostic role in identifying children with severe asthma is limited.

Children's Power of Food Scale: Turkish validity and reliability study.

OBJECTIVE: The aim of the present study was to validate the Turkish version of the Children's Power of Food Scale (C-PFS-T) after translation of the original version. DESIGN: The data were collected via face-to-face interviews using the C-PFS-T and a socio-demographic information form. BMI was calculated by dividing body weight by the square of the height. After the adaptation of the scale to Turkish language, validity and reliability analysis were conducted for the C-PFS-T. SETTING: Gülhane Training and Research Hospital Department of Child Health and Diseases Nutrition and Diet Unit in Ankara. PARTICIPANTS: This research was conducted with volunteer children and adolescents between the ages of 9 and 16 years (n 268). RESULTS: It was concluded that the 15-item C-PFS-T was collected under three factors as in the original version of the child version. Cronbach's α coefficient was found to be 0·878 for the scale. The confirmatory factor analysis results showed the acceptability and applicability of adapting the version of the C-PFS-T in terms of χ2/df (= 3·816), adjusted goodness-of-fit index (AGFI = 0·931), root mean square error of approximation (RMSEA = 0·082) and goodness-of-fit index (GFI = 0·852) fit indices. C-PFS-T total score's median value of obese group wasn't substantially different from normal weight group.Conclusions:It was concluded that the Turkish version of the C-PFS, which provides an assessment of the hedonic hunger status of children and adolescents with fifteen items and threesubdimensions, has sufficient reliability and validity to be applied to these subjects.

Mosquito hypersensitivity may be associated with atopic background in children.

BACKGROUND: Many children encounter unusual or "exaggerated" reactions such as large local, atypical or systemic reactions after mosquito bites. OBJECTIVE: The aim of this study was to document the clinical features of children with mosquito allergy and investigate the possible associations between demographic features and type of reactions in this population. METHODS: Children with large local or unusual reactions after mosquito bites who attended to our outpatient pediatric allergy department were enrolled in the study along with control subjects. RESULTS: A total of 180 children (94 with mosquito allergy and 86 age and sex-matched control subjects) with a median age of 6.8 years (IQR 5.5-9.3) were enrolled. Atopy (35.1% vs. 11.6%, p < 0.001) and grass pollen sensitization (28.7% vs. 8.1%, p < 0.001) were significantly more frequent in children with mosquito allergy. Skin prick test with mosquito allergen was positive in only 6 children (6,4%). Grass pollen sensitization was most common in children (28.7%) followed by sensitization to house dust mite (9.6%). 30 children (31.9%) had an accompanying atopic disease such as allergic rhinitis, asthma or atopic dermatitis. Bullae were significantly more frequent in children with asthma (41.7% vs.15.9, p = 0.034). The median duration of symptoms after onset were significantly longer in patients with ecchymosis, with immediate wheals and in children whose symptoms start in 20 min to 4 hours after mosquito bites. CONCLUSION: There is an association between unusual, large local or exaggerated reactions after mosquito bites and allergic diseases in children. The severity of reactions increases with age and particularly in children with atopic background.

Perinatal risk factors for asthma in children with allergic rhinitis and grass pollen sensitization.

BACKGROUND: Results of epidemiologic studies have determined several risk factors for asthma in school-age children. OBJECTIVE: To examine whether parental and perinatal risk factors, along with infantile feeding patterns, were associated with asthma in children with grass pollen allergy and allergic rhinitis. METHODS: We retrospectively analyzed the data of our cohort, which consisted of children with allergic rhinitis. Only children with grass pollen sensitization were enrolled. A detailed questionnaire regarding demographic features and perinatal events was given to the parents. RESULTS: A total of 293 children (200 boys [68.3%]; with median age, 10.2 years [interquartile range {IQR}, 7.4-13.0 years]) were included. A total of 109 children (37.2%) had accompanying asthma. The median age of onset of rhinitis symptoms was earlier (5.3 years [IQR, 4.0-8.0 years] versus 7.0 years [IQR, 5.0-10.0 years]; p = 0.001), histories of prematurity (16.7 versus 6.5%; p = 0.006), preeclampsia (5.5 versus 0%; p = 0.001), neonatal intensive care unit admission (15.1 versus 6.0%; p = 0.01), phototherapy (17.9 versus 7.1%; p = 0.004), early formula feeding (58.7 versus 41.2%; p = 0.006), and parental asthma (25.0 versus 11.4%; p = 0.002) were more frequent in children with asthma. Multivariate logistic regression analysis revealed prematurity (odds ratio [OR] 2.78 [95% confidence interval [CI],1.24-6.24]; p = 0.013), history of formula feeding (OR 1.81 [95% CI, 1.09-3.01]; p = 0.022), and parental asthma (OR 2.37 [95% CI, 1.22-4.63]; p = 0.011) were associated with asthma in school-age children with grass pollen-induced allergic rhinitis. CONCLUSION: Close monitoring of patients with these risk factors may help with an earlier diagnosis of asthma and prompt initiation of therapeutic interventions in children with allergic rhinitis and who were sensitized to grass pollen.

Vitamin D status and premature adrenarche.

BACKGROUND: Vitamin D, an important factor in calcium-phosphate homeostasis, has recently been suggested to play an important role in the pathogenesis of numerous chronic conditions such as hyperandrogenism. The aim of this study was to investigate the relationship between vitamin D status and premature adrenarche (PA). METHODS: A total of 71 girls with PA and 52 healthy girls, as the control group, were recruited. Axillary and/or pubic hair development before the age of 8 years was defined as PA. Bone age and anthropometric measures including height, weight, and body mass index (BMI) were obtained. 25-Hydroxyvitamin D (25(OH)D), fasting plasma glucose and insulin were measured. Vitamin D insufficiency was defined as <20 ng/mL. RESULTS: The PA patients had older bone age, higher BMI standard deviation score, homeostasis model of assessment-insulin resistance (HOMA-IR), and androgen but lower 25(OH)D than the control group. HOMA-IR and dehydroepiandrosterone sulfate were also higher in PA patients with vitamin D insufficiency compared with those with normal vitamin D. There was a negative correlation between 25(OH)D and HOMA-IR. CONCLUSION: Low vitamin D is associated with PA; and insulin resistance may be a factor in this association.

Association of neck circumference and pulmonary function in children.

BACKGROUND: Childhood obesity leads to many complications including impaired respiratory function. There are various anthropometric parameters related to obesity. OBJECTIVE: To investigate the correlation between anthropometric indices and pulmonary function test results in children without asthma. METHODS: Children without any respiratory disorders were enrolled in this study. Anthropometric measurements, such as height, weight, neck circumference (NC), and waist circumference, were obtained from the enrollees and body mass index was calculated. Afterward, pulmonary function tests were performed using spirometry. RESULTS: A total of 178 children (106 boys, 59.5%) with a mean age of 9.7 years were included the study. NC was above the 90th percentile in 65 children. Importantly, pulmonary parameters, such as forced expiratory volume during the first second (FEV1) and the ratio of FEV1 to forced vital capacity (FVC), were lower in subjects with a large NC. Similarly, waist circumference was above the 90th percentile in 67 children, and FEV1/FVC was significantly lower in children with a large waist circumference. Moreover, there was a statistically significant negative correlation among FEV1, FEV1/FVC, and body mass index SD score. Also, multivariable linear regression analysis showed that an NC above the 90th percentile was associated with lower FEV1 and FEV1/FVC values. CONCLUSION: We identified NC as a novel anthropometric index that is strongly correlated with respiratory functions in children. Therefore, close monitoring of respiratory symptoms, particularly in children with obesity and a large NC, could help with early and prompt determination of respiratory complications of obesity.

Association of wider neck circumference and asthma in obese children.

BACKGROUND: Obesity is a well-established risk factor for asthma. Previous studies have reported that central obesity is associated with asthma. OBJECTIVE: To investigate the association between fat distribution, which is determined by anthropometric measures, including neck circumference (NC), and asthma in school-aged children. METHODS: Children diagnosed as having asthma were enrolled along with controls who were admitted to our outpatient department with allergic symptoms, such as rhinitis, urticaria and atopic dermatitis. Anthropometric measures, including height, weight, NC, waist circumference, and hip circumference, were obtained. Skin prick tests, blood eosinophil counts, and serum total IgE level measurements were performed. RESULTS: A total of 196 children (92 male [46.9%]) were included. Asthma was present in 102 patients (52.1%). Ninety-one of the patients (46.4%) were overweight, and 45 patients (22.9%) were obese. The NC of children with asthma was significantly higher than that of children in the control group. Grades defined according to NC percentiles were also significantly different between groups. In children with asthma, the prevalence of children with an NC higher than the 90th percentile (grade 6) was more frequent when compared with controls. The median NC of obese-overweight children with asthma was significantly higher compared with obese-overweight controls without asthma. Results of multivariable logistic regression analysis revealed that the presence of an NC in the greater than 90th percentile was associated with asthma in obese-overweight children. CONCLUSION: This study found that NC, which is a simple anthropometric measure, is associated with asthma in obese children.

Association of nerve conduction impairment and insulin resistance in children with obesity.

AIM: The objective of our study was to investigate nerve conduction in normoglycemic obese children. METHODS: A total of 60 children with obesity (30 female and 30 male) and 30 healthy children (15 female and 15 male) were enrolled in the study. Insulin resistance (IR) and other metabolic disturbances were investigated and nerve conduction was measured in all participants. Obese children were divided into groups according to the presence of IR. All results were compared between these subgroups. RESULTS: The nerve conduction velocity (NCV) of motor median nerves in the IR+ group was significantly higher than that in the IR- group and lower than that in the control group. The NCV of the motor peroneal nerve in the IR+ group was significantly lower than that in the IR- group. The sensory nerve action potential (SNAP) of the sensory median nerve was significantly lower in the IR+ group compared to that in the IR- group. The sensory sural nerve's SNAP was significantly lower in the IR+ group than that in the control group. CONCLUSION: Nerve conduction tests may help to detect early pathologies in peripheral nerves and to decrease morbidities in obese children.

Mid-wave infrared metasurface microlensed focal plane array for optical crosstalk suppression.

Spatial crosstalk is one of the fundamental drawbacks of diminishing pixel size in mid-wave infrared focal plane arrays (IR-FPAs). We proposed an IR-FPA using the concept of optical phase discontinuities for substantial optical crosstalk suppression. This IR-FPA consists of asymmetrically tailored V-shaped optical antennas. Full-wave simulations confirmed major improvements in narrowing the intensity distribution of incident light beam by over 30-folds and concentrating these distributions in the central pixel of IR-FPA by achieving optical crosstalks of <1%.

Observation of selective plasmon-exciton coupling in nonradiative energy transfer: donor-selective versus acceptor-selective plexcitons.

We report selectively plasmon-mediated nonradiative energy transfer between quantum dot (QD) emitters interacting with each other via Förster-type resonance energy transfer (FRET) under controlled plasmon coupling either to only the donor QDs (i.e., donor-selective) or to only the acceptor QDs (i.e., acceptor-selective). Using layer-by-layer assembled colloidal QD nanocrystal solids with metal nanoparticles integrated at carefully designed spacing, we demonstrate the ability to enable/disable the coupled plasmon-exciton (plexciton) formation distinctly at the donor (exciton departing) site or at the acceptor (exciton feeding) site of our choice, while not hindering the donor exciton-acceptor exciton interaction but refraining from simultaneous coupling to both sites of the donor and the acceptor in the FRET process. In the case of donor-selective plexciton, we observed a substantial shortening in the donor QD lifetime from 1.33 to 0.29 ns as a result of plasmon-coupling to the donors and the FRET-assisted exciton transfer from the donors to the acceptors, both of which shorten the donor lifetime. This consequently enhanced the acceptor emission by a factor of 1.93. On the other hand, in the complementary case of acceptor-selective plexciton we observed a 2.70-fold emission enhancement in the acceptor QDs, larger than the acceptor emission enhancement of the donor-selective plexciton, as a result of the combined effects of the acceptor plasmon coupling and the FRET-assisted exciton feeding. Here we present the comparative results of theoretical modeling of the donor- and acceptor-selective plexcitons of nonradiative energy transfer developed here for the first time, which are in excellent agreement with the systematic experimental characterization. Such an ability to modify and control energy transfer through mastering plexcitons is of fundamental importance, opening up new applications for quantum dot embedded plexciton devices along with the development of new techniques in FRET-based fluorescence microscopy.

Genotype-phenotype characteristics of 57 patients with Prader-Willi syndrome: a single-center experience from Turkey.

OBJECTIVES: Prader-Willi syndrome (PWS) is a rare and complex genetic disorder caused by the loss of expression of the paternal copy of the imprinted genes on chromosome 15q11-q13. A variety of findings have been reported on the phenotypic differences between the genetic subtypes of PWS. This article compares the clinical findings of 57 PWS patients by genetic subtype and explores possible associations in this context. METHODS: Methylation­specific multiplex ligation-dependent probe amplification and single nucleotide polymorphism microarrays were used to diagnose deletion and uniparental disomy (UPD). For phenotype-genotype correlation, clinical data were collected and genetic subgroups were compared statistically, and P  < 0.05 was considered to indicate statistical significance. RESULTS: These 57 patients consisted of 15 type I deletions, 20 type II deletions, six atypic deletions, 11 heterodisomy UPD, four isodisomy UPD, and one translocation-type PWS. All patients had hypotonia, poor neonatal sucking, and feeding difficulties during infancy. Other PWS-related clinical findings, such as speech articulation problems (85.9%), sleep apnea (77.2%), normal birth length (71.9%), small hands/feet (71.9%), childhood polyphagia (57.9%), clinodactyly (56.1%), thick viscous saliva (54.4%), and behavioral problems (50.9%) were observed at varying rates with no statistical difference between genetic subtypes in general. CONCLUSION: This study highlights the phenotype-genotype associations on PWS from a cohort of Turkish pediatric patients as a single-center experience.

Transient Neonatal Diabetes Mellitus and Seizure with an Unknown Etiology.

Neonatal diabetes mellitus (NDM) is a monogenic form of diabetes, usually occurring in the first 6 months of life. Here, we present a newborn, which was admitted with epileptic seizure on the postnatal second day of life. Sepsis and meningitis were ruled out. Cranial imaging and electroencephalography revealed normal. She developed transient NDM on the follow-up and was diagnosed to carry an ABCC8 mutation. Although the neurological features are more common in patients with KCJN11 mutations, patients with ABCC8 mutations could also represent with subtle neurodevelopmental changes or even with epileptic seizures. The genetic testing and appropriate therapy is important in this patient group for predicting clinical course and possible additional features.

The Relationship Between Premature Adrenarche and Platelet Aggregation

Objective: Premature adrenarche (PA) has been associated with an increase in adrenal androgens, and the hyperandrogenic hormonal environment is known to lead to increased platelet (PLT) aggregation. Here, we evaluated the effects of PA on PLT aggregation in PLT-rich plasma samples from female patients. Methods: The study included 40 female patients diagnosed with PA between February, 2014 and June, 2018 and 30 healthy female individuals as a control group. Adenosine diphosphate (ADP) and collagen-induced PLT aggregation were studied via the photometric aggregometry method. Results: There were no significant differences in the PLT count or volume values between those participants with PA and the control group. Additionally, the ADP-induced maximum aggregation time, value, and slope values did not significantly differ between the patient and control groups (p>0.05). However, the collagen-induced maximum aggregation time, value, and slope values were significantly higher in the studygroup (p<0.001). Conclusion: Increased collagen-induced PLT aggregation was detected in female patients with PA. As PA is associated with a higher risk of cardiovascular events later in life, close follow-up of PA in this respect may be beneficial.

Evaluation of the nutritional status of children with type 1 diabetes and their healthy siblings.

BACKGROUND: Although there are studies evaluating the psychological adjustment of healthy children when their siblings have type 1 diabetes mellitus (T1DM), no study evaluating their nutritional status exists. Thus, this study aimed to determine the nutritional status of children with T1DM and their healthy siblings. MATERIAL AND METHODS: Data from a control group, healthy siblings, and patients who were followed and treated for T1D in the Paediatric Gastroenterology and Paediatric Endocrinology outpatient clinics of the Health Sciences University Gülhane Medical Faculty between November 2019 and November 2020 were analysed and compared. The groups were compared in terms of nutritional characteristics, daily macro and micronutrient intakes and classified according to their body mass index (BMI) Z-scores. RESULTS: The study population consisted of 29 children with T1DM (51.7% female, 48.3% male; age: 11.00 ±3.66 years), 36 healthy siblings of children with T1D (50% female, 50% male; age: 9.61 ±4.84 years), and a control group of 58 healthy children (51.7% female, 48.3% male; age: 10.68 ±3.01years).The BMI Z-score of 28.6%of healthy siblings and 25% of children with T1D was > 1 SD or overweight. All of the control group children were of normal weight. None of the children were obese; however, the overweight rate was significantly higher in the healthy siblings and diabetes groups compared to the control group (p = 0.012). Daily energy intake (%) was significantly higher in the control group than in the healthy siblings (p < 0.001). CONCLUSIONS: A quarter of the children with T1D over five years of age were overweight. In addition, healthy siblings were found to have higher BMI Z-scores than controls. This is the first study to evaluate the nutritional status of siblings of patients with T1D and will hopefully lead to more comprehensive studies that will also assess their daily exercise and physical activity.

Evaluation of the resilience of the girls with central precocious puberty treated with gonadotropin-releasing hormone analog.

OBJECTIVES: The aim of this study was to evaluate the resilience of girls with central precocious puberty (CPP) during treatment with a gonadotropin-releasing hormone agonist (GnRHa) and compare these results with their healthy peers. METHODS: The Connor-Davidson Resilience Scale (CD-RISC) is a self-report scale used to quantify resilience, which is divided into seven subgroups (hardiness, coping, flexibility, purpose, optimism, regulation of emotion and cognition (REC), and self-efficacy). Fifty-one girls with CPP receiving GnRHa treatment and 51 healthy controls were involved in the study. Anthropometric measurements were evaluated and CD-RISC was performed at least six months after the initiation of GnRHa treatment. RESULTS: There was no statistically significant difference between the anthropometric evaluations of girls with CPP and the control group. Similarly, the total score and subgroup scores of patients with CPP and the control group showed no statistically significant difference. In the correlation analysis, there was a weak negative correlation between height and flexibility (r=-0.314 p=0.025), height SDS and flexibility (r=-0.254 p=0.092), height SDS, and purpose (r=-0.285 p=0.058). Also, there was a weak negative correlation between REC and weight (r=-0.435 p=0.003), REC and weight SDS (r=-0.461 p=0.002), REC and height (r=-0.269 p=0.077), REC and height SDS (r=-0.322 p=0.033), REC and BMI (r=-0.289 p=0.058), and REC and BMI SDS (r=-0.353 p=0.019). CONCLUSIONS: The resilience of girls with CPP treated with GnRHa was found to be similar to their healthy peers. The early diagnosis of the disease and adequate treatment may decrease the discrepancy of somatic changes between girls with CPP and their peers, which may help them to overcome the stress of CPP and long-term treatment.

Two Subsequent Metachroneus Solid Tumors: Oncocytic Variant Adrenocortical Carcinoma and Rhabdomyosarcoma of Childhood: Case Report and Literature Review

Most cases of malignancies appear to be sporadic, but some syndromes are associated with malignancies with germline variants. Herein, a child with an unusual association of oncocytic variant adrenocortical carcinoma (ACC) and rhabdomyosarcoma (RMS) was presented. An 18-month-old-boy was admitted with virilization of the genital area, penis enlargement and erection, which had begun six months earlier. Serum total testosterone (457 ng/dL; NR <10), androstenedione (3.35 ng/mL; NR <0.5) and dehydroepiandrosterone-SO4 (206 mcg/dL; NR<35) were above the normal ranges. Right adrenal mass was detected. After adrenalectomy, histopathological examination revealed an oncocytic variant ACC. Three-month after surgery, he then presented with 6x8 cm sized swelling of the left leg. Histopathological examination revealed embryonal RMS. Testing for tumor protein (TP53) variant by DNA sequence analysis was positive; however; fluorescence in situ hybridization analysis was negative. After chemotherapy and local radiotherapy, the patient is in good condition without tumor recurrence. Only about one-third of these tumors have a variant of TP53. This status also applies to other genetic variants related to cancer. However, a significant association of malignancies strongly suggests a problem in tumor suppressor genes or new variants. Another as yet unidentified suppressor gene may also be present and effective in this locus. The occurrence of ACC as a part of a syndrome and positive family history of malignancies in patients are clinically important. These patients and their families should be scanned for genetic abnormalities. The patient with ACC should be followed-up carefully for other tumors to detect malignancy early.

A Rare Cause of Hyperinsulinemic Hypoglycemia: Kabuki Syndrome

Kabuki syndrome (KS) is a disease characterized by distinctive facial features, skeletal anomalies and delay in neuromotor development. KS 1 is an autosomal dominant condition caused by mutations in the KMT2D gene, whereas KS 2 is an X-linked disorder caused by mutations in the KDM6A gene. In the majority of KS patients who present with hypoglycemia, KDM6A is the defective gene. A 9-month old girl was admitted to our emergency department due to a seizure. On physical examination, hypotonia, mild facial dysmorphism, brachydactyly of the 5th finger, prominent finger pads and pansystolic murmur were detected. A fasting glucose tolerance test was performed the next day due to her history of hypoglycemia, but she had convulsions at the fifth hour of the test. Her serum glucose was 24 mg/dL, insulin 1.94 mIU/L, C-peptide 0.94 ng/mL, growth hormone 11 ng/mL, anti-insulin antibody 4.2 IU/mL, cortisol 19.8 µg/dL, and adrenocorticotropic hormone 9.3 pg/mL. A diagnosis of hyperinsulinemic hypoglycemia was considered. Given the abnormalities, genetic analysis for congenital hyperinsulinism, including the genes causing KS was performed. A heterozygous frameshift mutation (c.2579del, p.Leu860Argfs*70) was detected in the KMT2D gene. Epilepsy and other neurological symptoms may be seen in KS patients and in some of these the neurological symptoms are the result of hypoglycemia. In such cases, the detection and prevention of hypoglycemia can help prevent the progression of neurological symptoms. We suggest considering the diagnosis of KS for patients with hypoglycemia and dysmorphic features, even if the patient does not manifest all features of KS.