Pneumococcal carriage and serotype distribution in children with nephrotic syndrome.

BACKGROUND: Patients with nephrotic syndrome (NS) are at a higher risk of developing invasive pneumococcal disease (IPD). Pneumococcal carriage studies are helpful tools for detecting potentially infectious serotypes and guiding immunization efforts. Pneumococcal nasopharyngeal colonization is common, and IPD can easily occur in an immunosuppressed state. Limited information is available regarding the frequency of pneumococcal carriage in individuals with NS. The aim of this study was to evaluate pneumococcal carriage and serotype distribution in children with NS. METHODS: Pneumococcal carriage was detected by real-time PCR assays from nasopharyngeal swab samples from 98 children with NS, and 100 healthy controls. Isolates were serotyped by real-time PCR. RESULTS: The pneumococcal carriage rate was 44.9% in children with NS. Regarding the recommendation about pneumococcal immunization in children with NS, the vaccination rate was low. Also, non-PCV13 serotypes have been detected in at least 25% of PCV13-vaccinated children. There is no statistically significant difference in total pneumococcal carriage rate, PCV13 serotype carriage rate, or non-PCV13 serotype carriage rate between children with NS and healthy controls (p > 0.05 for all). CONCLUSIONS: The pneumococcal carriage rate was similar between children with NS and healthy controls. However, because children with NS have an increased risk for IPD, the serotype distribution of children with NS can demonstrate the improved protection offered by new pneumococcal vaccines. Regular monitoring for IPD is crucial for assessing the evolving sero-epidemiology of pneumococcal infections and evaluating the effectiveness of vaccines for children with NS.

Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date. METHODS: A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed. RESULTS: The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04). CONCLUSIONS: Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.

The effectiveness of a fall detection device in older nursing home residents: a pilot study.

BACKGROUND: Real-world research to evaluate the effect of device technology in preventing fall-related morbidity is limited. This pilot study aims to investigate the effectiveness of a non-wearable fall detection device in older nursing home residents. METHODS: The study was conducted in a nursing home with single-resident rooms. Fall detection devices were randomly set up in half of the rooms. Demographic data, comorbidities, lists of medications, and functional, nutritional, and frailty status were recorded. The residents were followed up for 3 months. The primary outcome was falls and the secondary outcome was all-cause mortality. RESULTS: A total of 26 participants were enrolled in the study. The study group consisted of 13 residents who had a fall detection device in their rooms. The remaining 13 residents on the same floor formed the control group. Participants had a mean age of 82 ± 10 years and 89% of the residents were female. The most prevalent comorbidity was dementia. Two residents from the control group and one resident from the study group experienced a fall event during follow-up. The fall events in the control group were identified retrospectively by the nursing home staff, whereas the fall in the study group received a prompt response from the staff who were notified by the alarm. One resident was transferred to the hospital and died due to a non-fall related reason. CONCLUSION: Device technology may provide an opportunity for timely intervention to prevent fall-related morbidity in institutionalized older adults.

High prevalence of muscle weakness and probable sarcopenia in patients with inflammatory bowel disease.

BACKGROUND: This study aimed to determine the prevalence of probable sarcopenia and sarcopenia in patients with inflammatory bowel disease (IBD) by using the European Working Group on Sarcopenia in Older People (EWGSOP2) diagnostic criteria. METHODS: Sarcopenia was assessed by using the sequential four-step algorithm. (1) Find: Sarcopenia risk by simple clinical symptom index (strength, assistance walking, rise from a chair, climb stairs, and falls [SARC-F questionnaire]). (2) Assess: Probable sarcopenia by low muscle strength on handgrip. (3) Confirm: Confirmed sarcopenia by low appendicular skeletal muscle mass on bioimpedance analysis. (4) Severity: Severe sarcopenia by low 4-m gait speed test. RESULTS: A total of 129 adult patients with IBD younger than 65 years and 50 age- and sex-matched healthy control (HC) participants were included to the study. Handgrip strength, gait speed, and SARC-F scores were significantly lower in patients with IBD than in the HCs (P = 0.032, <0.0001, and <0.0001, respectively). Based on the EWGSOP2 definition, 17.8% of patients with IBD had probable sarcopenia, and six patients had confirmed sarcopenia. According to the ethnicity-based population thresholds, 34.9% of patients with IBD had probable sarcopenia, and two patients had confirmed sarcopenia. Corticosteroid use within the past year was identified as an independent risk factor for low muscle strength (P = 0.012; odds ratio, 4.133), along with advanced age and disease activity. CONCLUSION: One-third of the patients younger than 65 years with IBD had probable sarcopenia, defined as low muscle strength, whereas the incidence of confirmed sarcopenia remained relatively low.

Comprehensive evaluation of patients with primary hyperoxaluria type 1: A nationwide study.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey. METHODS: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey. Detailed patient data was collected which included demographic, clinical and laboratory features. Patients were classified according to their age and characteristics at presentation: patients presenting in the first year of life with nephrocalcinosis/nephrolithiasis (infantile oxalosis, Group 1), cases with recurrent nephrolithiasis diagnosed during childhood (childhood-onset PH1, Group 2), and asymptomatic children diagnosed with family screening (Group 3). RESULTS: Forty-eight patients had a mutation consistent with PH1. The most common mutation was c.971_972delTG (25%). Infantile oxalosis patients had more advanced chronic kidney disease (CKD) or kidney failure necessitating dialysis (76.9% vs. 45.5%). These patients had much worse clinical course and mortality rates seemed to be higher (23.1% vs. 13.6%). Patients with fatal outcomes were the ones with significant comorbidities, especially with cardiovascular involvement. Patients in Group 3 were followed with better outcomes, with no kidney failure or mortality. CONCLUSION: PH1 is not an isolated kidney disease but a systemic disease. Family screening helps to preserve kidney function and prevent systemic complications. Despite all efforts made with traditional treatment methods including transplantation, our results show devastating outcomes or mortality.

Developing a virtual patient: design, usability, and learning effect in communication skills training.

BACKGROUND: Literature shows that Virtual Patients (VPs) find extensive usage in the field of health sciences, especially in the post-pandemic period. VPs are successfully utilized in developing various effective skills like medical interview. However, this technology is quite new in Turkey and has not yet been used in communication skills training in a structured form. This research aimed to develop a virtual patient to improve the communication skills of medical students. METHODS: Developmental research method was used in the study. The implementation phase involved the one group posttest quasi-experimental design. The study group comprised of experts in various fields and 213 medical students. Needs Analysis Form, Scenario Building Form, System Validation Form, Communication Skills Assessment Form, and Interview Form were used as data collection tools. The research primarily concentrated on ensuring minimal errors within the system and enhancing students' communication skill scores. RESULTS: The study found that VP was effective in teaching communication skills. Communication skills improved from a mean score of 36.74 in the first interview with 15 students to 74.2 in the final application with 198 students. It was determined that the students who practiced repeatedly (n = 26) made 17% more effective interviews than their first practices (score: 89.2). The script matching of the VP was 83%. Other data obtained from the students generally showed that the VP application was developed in accordance with the purpose, that it was user-friendly, and that the scenarios were adequate. CONCLUSION: VPs like this have the potential to develop skills such as history taking, clinical reasoning, etc., which are very important in the field of health sciences.

The value of 2019 EULAR/ACR classification criteria in predicting lupus nephritis in childhood-onset systemic lupus erythematosus.

BACKGROUND: We investigated the role of European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for the prediction of LN among children with SLE. METHODS: The data of the patients with childhood-onset SLE diagnosed based on 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were retrospectively evaluated. Based on 2019 EULAR/ACR classification criteria, the scoring was done at the time of renal biopsy. RESULTS: Fifty-two patients (12 with LN, 40 without LN) were included. The mean score was higher in patients with LN than those without (30.8±6.14, 19.8±7.76, respectively, p=0.000). The score value had indicative value for LN (area under curve [AUC]:0.863±0.055, cut-off value:22.5, p=0.000). Lymphocyte counts had a predictive value for LN (cut-off value:905/mm3, AUC:0.688±0.087, p=0.042). The score was positively associated with SLE disease activity index (SLEDAI) and activity index (r=0.879, p=0.000; r=0.811, p=0.001, respectively). There were significant negative associations between score value and GFR (r=-0.582, p=0.047). The patients with renal flare had higher the mean score than those of without renal flare (35±2/25.4±5.57, respectively, p=0.019). CONCLUSIONS: The EULAR/ACR criteria score could reflect the activity of disease and severity of nephritis in childhood-onset SLE. A point of 22.5 as score value might be an indicator for LN. During scoring, it should be taken into account that lymphopenia might guide the prediction of LN.

Comparative Analysis of Intensive Care Prognosis Scoring Systems and Acute Kidney Injury Scores (AKIN and pRIFLE) in Critically Ill Children.

The development of AKI (acute kidney injury) in critically ill patients in pediatric intensive care units (PICUs) is one of the most important factors affecting mortality. There are scoring modalities used to predict mortality in PICUs. We compared the AKIN (Acute Kidney Injury Network) and pRIFLE (pediatric risk, injury, failure, loss, and end stage) AKI classifications and PICU scoring modalities in this study. METHODS: A total of 716 children, whose serum creatinine levels were within the normal limits at the time of admission to the PICU between January 2018 and December 2020, were included. Along with the demographic and clinical variables, AKIN and pRIFLE classifications were recorded at the most advanced stage of AKI. Along with the PIM-2, PRISM III, and PELOD-2 scores, the highest value of the pSOFA score was recorded. RESULTS: According to the pRIFLE and AKIN classifications, 62 (8.7%) patients developed kidney injury, which had a statistically significant effect on mortality. The occurrence of renal injury was found to be statistically strongly and significantly correlated with high PRISM III, PELOD-2, and pSOFA scores. When the stages of kidney injury according to the AKIN criteria were compared with the PRISM III, PELOD 2, and pSOFA scores, a significant difference was found between the patients who did not develop AKI and those who developed stage 1, stage 2, and stage 3 kidney injury. For the PRISM III, PELOD 2, and pSOFA scores, there were no significant differences between the stages according to the AKIN criteria. A substantial difference was discovered between the patients who did not develop AKI and those who were in the risk, injury, and failure plus loss stages according to the pRIFLE criteria. According to the PIM-2 ratio and pRIFLE criteria, there was a statistically significant difference between patients in the injury and failure plus loss stages and those who did not develop AKI. CONCLUSIONS: Our study is the first pediatric study to show a substantial correlation between the variables associated with the PICU scoring modalities in critically ill children with AKI. Identifying the risk factors for the development of AKI and planning antimicrobial regimens for patients with favorable prognoses at the time of PICU admission could lower mortality rates.

Immature granulocytes as biomarkers of inflammation in children with predialysis chronic kidney disease.

BACKGROUND: Chronic inflammation in patients with predialysis chronic kidney disease (CKD) is quite common. We aimed to investigate the relationship of the percentage of immature granulocytes (IG%) and immature granulocyte count (IGC) with inflammation in children with predialysis CKD. METHODS: The data from children with stage 2-4 CKD and a control group of healthy children were evaluated retrospectively. A highly-sensitive C-reactive protein (hs-CRP) level above 5 mg/dL was considered the presence of inflammation. The IGCs were calculated in the white cell differential channel of the Sysmex XN-9000 using the fluorescent flow cytometry method. The IG% was expressed as percentage of total leucocyte concentration. RESULTS: The data from 57 patients (30 stage 2 CKD, 15 stage 3 CKD, 12 stage 4 CKD) and 46 controls were analyzed. hs-CRP levels, IG%, IGC, white blood cell (WBC) and neutrophil counts, and neutrophil-to-lymphocyte ratio (NLR) were higher in patients than the control group (p < 0.000, p < 0.000, p < 0.000, p = 0.001, p = 0.002, p < 0.000, respectively). Both IG% and IGC were positively correlated with hs-CRP, WBC and neutrophil counts, and NLR (r = 0.485, p < 0.000; r = 0.379, p = 0.004; r = 0.543, p < 0.000; r = 0.628, p < 0.000 for IG%; r = 0.379, p = 0.004; r = 0.351, p = 0.007; r = 0.525, p < 0.000; r = 0.601, p < 0.000 for IGC, respectively). A ROC analysis of the relationship between IGC, IG%, and inflammation showed IGC and IG% had predictive value for the presence of inflammation (cut-off value: 0.035 × 106/mL, AUC: 0.799 ± 0.061, sensitivity: 74.2%, specifity: 63%, p < 0.001 for IGC; cut-off value: 0.45%, AUC: 0.838 ± 0.056, sensitivity: 70.8%, specifity: 67.3%, p = 0.001 for IG%). CONCLUSIONS: Immature granulocytes may be used as a biomarker of inflammation in children with predialysis CKD. A higher resolution version of the Graphical abstract is available as Supplementary information.

Associations between obesity, self-reported weakness and their combinations with mortality in nursing home residents.

OBJECTIVES: There are studies on associations between obesity and mortality in nursing home (NH) residents, but the presence of concomitant muscle weakness has not been examined. We considered that self-reported weakness might be a low muscle strength proxy marker. We aimed to examine associations of obesity alone, self-reported muscle weakness alone, and their combination with mortality in NH residents. METHODS: This is a retrospective longitudinal follow-up study. We noted age, sex, nutritional status, functionality, number of chronic diseases, and regular medication. Obesity was assessed by the body fat-percentage method estimated by bioimpedance analysis. Weakness was identified by self-reported muscle weakness. Survival was evaluated with a univariate log-rank test and multivariate Cox regression analyses. RESULTS: We included 214 participants. In a median follow-up time of 46 months, mortality occurred in 37.4%. In multivariate analysis adjusted by age, sex, undernutrition, number of chronic diseases, and regular medication, functional scores; 'non-weak non-obese' participants or 'weak alone' participants or 'weak+obese' participants had higher mortality risk when compared with the 'obesity alone' participants [hazard ratio (HR) = 2.6, 95% confidence interval (CI) = 1.2-5.5, p = 0.01; HR = 2.6, 95% CI = 1.2-5.9, p = 0.02; HR = 3.0, 95% CI = 1.2-7.7, p = 0.02]. CONCLUSION: This is the first report showing that obesity was associated with lower mortality risk if the weakness was not present in NH residents. However, obesity with concomitant weakness was associated with mortality risk similar to non-weak non-obese or weak alone participants. Our study suggests a simple consideration of weakness that can easily be integrated into everyday practice.

Evaluation of Arterial Stiffness and Carotid Intima-Media Thickness in Children with Primary and Renal Hypertension.

Hypertension is an increasing disease in children and the risk of endothelial damage and target organ damage increases in the presence of additional risk factors such as obesity. In our study, the effect of hypertension on early atherosclerotic changes and target organ damage in children was investigated. Twenty four-hour ambulatory pulse wave analysis was performed by oscillometric method in 71 children aged 8-18 years, 17 of whom were diagnosed with primary hypertension without obesity, 18 had both primary hypertension and obesity, and 16 had renal hypertension. Twenty healthy normotensive children were included as the control group. Carotid intima-media thickness (CIMT) and Left Ventricular Mass Index were measured. Central systolic blood pressure (cSBP), central diastolic blood pressure (cDBP), systolic blood pressure (SBP) and diastolic blood pressure (DBP) were higher in the primary hypertension group compared to controls (p = 0.001, p = 0.005, p = 0.001, p = 0.009, respectively), cSBP was higher in the renal hypertension group than the control group (p = 0.018). There was no difference between the groups in terms of pulse wave analysis parameters, CIMT, or left ventricular mass index (p > 0.05). Pulse wave velocity was positively correlated with SBP, DBP, cSBP, cDBP (p < 0.001). Augmentation index was positively correlated with DBP and cDBP (p = 0.01, p = 0.002, respectively). Our findings show that high blood pressure is associated with arterial stiffness and target organ damage beginning in childhood. The detection of early atherosclerotic vascular changes using pulse wave analysis allows to take necessary precautions such as lifestyle changes to prevent target organ damage in hypertensive children.

The efficacy of interventions provided by nurses to improve glycemic control of children with type 1 diabetes: A systematic review.

PURPOSE: Nurses have a very important role in diabetes education of children and adolescents, however, no systematic review has been performed that evaluates the effectiveness of the independent interventions of the nurses. This systematic review aims to identify and assess randomized controlled trials (RCT) that included interventions implemented by nurses to improve glycemic control for children and adolescents with type 1 diabetes. ELIGIBILITY CRITERIA: PubMed, the Cochrane Central Register of Controlled Trials, Scopus, Web of Science, Science Direct databases were systematically searched up to 2022. Interventions provided by nurses for any child ≤18 years diagnosed with type 1 diabetes mellitus were included. SAMPLE: Seven RCT articles met our review criteria. RESULTS: All included studies were published between 2005 and 2021. Studies varied in terms of intervention type; telephone case management, coping skills training, motivational interview as well as home visit education programs. Interventions were found to significantly decrease the HbA1c level in three of seven studies. CONCLUSIONS: Our systematic review suggests that nurses can improve the glycemic control of children with type 1 diabetes by conducting care interventions. According to current evidence, this study suggests that telephone contact by nurses and motivational interviewing can be beneficial in improving glycemic control in this population. IMPLICATIONS: Nurses may encourage children and adolescents with diabetes to engage in self-management of their glucose levels. There is a need for more randomized controlled studies assessing the efficacy of nursing interventions for children with type 1 diabetes.

Diagnostic Values of Immature Granulocytes Detected by the Sysmex XN 9000 Hematology Analyzer in Children with Urinary Tract Infections.

Immature granulocytes (IGs) are used as markers of infection and systemic inflammation. We aimed to investigate the diagnostic value of IGs in children with urinary tract infections (UTIs). Children with their first UTIs were included in this observational study. Blood samples were obtained before antibiotic therapy. The blood analysis was repeated 2 weeks after the treatment ended. In total, 194 children (95 with febrile UTI, 58 with cystitis, and 41 controls) were included. The percentage of IGs (IG%) and IG count (IGC) measured at the time of admission were higher in the patients with febrile UTI than in the patients with cystitis and the controls (P = 0.000). The IGC and IG% after treatment were higher in patients with renal scarring than in those without scarring (P = 0.012 and P = 0.021, respectively). Cox's regression analysis showed the significant associations of renal scarring with both IGC and IG% (hazard ratio: 8.181, P = 0.002; hazard ratio: 5.106, P = 0.033, respectively). Both IGC and IG% were positively associated with severe vesicoureteral reflux (VUR) [odds ratio (OR): 22.235, P = 0.025; OR: 15.597, P = 0.038, respectively]. In conclusion, the IG% and IGC, which can be easily measured in a routine complete blood count without the need for additional effort, could be used as biomarkers for predicting febrile UTI, renal scarring, and severe VUR in children.

Potentially inappropriate medications based on TIME criteria and risk of in-hospital mortality in COVID-19 patients.

OBJECTIVE: This study aimed to evaluate the relationship between hospital admission potentially inappropriate medications use (PIM) and in-hospital mortality of COVID-19, considering other possible factors related to mortality. METHODS: The Turkish inappropriate medication use in the elderly (TIME) criteria were used to define PIM. The primary outcome of this study was in-hospital mortality. RESULTS: We included 201 older adults (mean age 73.1±9.4, 48.9% females). The in-hospital mortality rate and prevalence of PIM were 18.9% (n=38) and 96% (n=193), respectively. The most common PIM according to TIME to START was insufficient vitamin D and/or calcium intake per day. Proton-pump inhibitor use for multiple drug indications was the most prevalent PIM based on TIME to STOP findings. Mortality was related to PIM in univariate analysis (p=0.005) but not in multivariate analysis (p=0.599). Older age (hazards ratio (HR): 1.08; 95% confidence interval (CI): 1.02-1.13; p=0.005) and higher Nutritional Risk Screening 2002 (NRS-2002) scores were correlated with in-hospital mortality (HR: 1.29; 95%CI 1.00-1.65; p=0.042). CONCLUSION: Mortality was not associated with PIM. Older age and malnutrition were related to in-hospital mortality in COVID-19.

Impact of flipped classroom approach on undergraduate nursing student's critical thinking skills.

BACKGROUND: Enriching the undergraduate nursing main courses innovative approaches such as flipped classroom method, which may aid improving students' metacognitive learning strategies, are regarded to be very important in terms of enhancing critical thinking and problem-solving skills in nursing care. AIM: This study aimed to determine if there were significant differences in performance and critical thinking skills of nursing students who were taught using the flipped classroom method versus those who were taught using the online traditional learning approach. Another aim was for students to describe their opinions of using the flipped classroom method. METHODS: This study adopted a quasi-experimental study design. A total of 63 nursing students participated in the study. The experimental group followed the flipped classroom learning approach, while the control group followed the online traditional learning approach. The study involved a pre- and post-test assessment to examine the students' learning achievements and critical thinking skills. RESULTS: The results indicate that the flipped classroom approach has no significantly different effect on students' academic achievement and critical thinking, compared to online traditional lecturing. However, in contrast to the analysis of the results, students generally stated that learning with a flipped classroom approach was more helpful and efficient, and that the method provided a dynamic learning session during lectures. CONCLUSIONS: Flipped classroom approach planned for a part of the pediatric nursing education did not show significant difference compared to online traditional lecturing.

A case of familial recurrent 17q12 microdeletion syndrome presenting with severe diabetic ketoacidosis.

BACKGROUND: Heterozygous intragenic mutations of the hepatocyte nuclear factor 1 homeobox b gene (HNF1B) located on chromosome 17 and microdeletion of 17q12 region (17q12MD) leads to the complete loss of this gene, which causes renal cystic disease, diabetes mellitus (MODY5), hypomagnesemia, hyperuricemia, liver enzyme abnormalities, genital tract abnormalities and exocrine pancreatic insufficiency. In addition, patients with 17q12MD also have facial dysmorphism, neuro-developmental and neuropsychiatric disorders. CASE: A 16-year-old girl with obesity and mild facial dysmorphism was admitted to the hospital with symptoms of diabetes that started two days prior to her admission. She was diagnosed with severe diabetic ketoacidosis and treated accordingly. She had been followed up with the diagnoses of multicystic renal disease, hydronephrosis, hepatosteatosis, hypomagnesemia and hyperuricemia since the age of six. She had mild intellectual disability. Her menarche started two months ago. Cranial magnetic resonance imaging revealed mild diffuse cerebral and cerebellar atrophy and a partial empty sella. Her mother had diabetes, hypomagnesemia and mild intellectual disability and her maternal grandfather and uncle had diabetes. Her grandfather also had renal cystic disease. All of them are on oral antidiabetic medication. The genetic analysis of the patient and her mother revealed a loss of 1.6 megabases in chromosome 17q12. CONCLUSIONS: MODY5 should be kept in mind in patients with diabetes who present with extra pancreatic findings, especially with renal cystic disease, more over, a genetic analysis including the study of 17q12MD should be carried out in patients who present with additional neuropsychiatric findings. Ketoacidosis can be seen in patients with MODY5. Ketoacidosis and renal anomalies and dysfunction are factors that increase and affect the severity of each other in these patients.