A case of down syndrome with basal ganglia calcification

Sekiz yasında, Down sendromlu, erkek hasta pnömoni ve solunum yetmezligiyle ÇocukYogun BakımÜnitesine kabul edildi. Izleminde tremor, strabismus, idrar retansiyonu ve klonus fark edildi. Bu nörolojik bulgular için çekilen bilgisayarlı beyin tomografisinde bilateral bazal gangliyon kalsifikasyonu gösterildi. Ayrıca nörolojik bulgular kusmalarını önlemek amacıyla baslanan metoklopramide baglandı. Hastanın bazal gangliyon kalsifikasyonunun TORCH, metabolik ve endokrin nedenlere baglı olmadıgı gösterilerek Down sendromuna baglı oldugudüsünüldü.Eight-year-old boy with Down syndrome was accepted to The Pediatric Intensive Care Unit for pneumonia and respiratory failure. Tremor, strabismus, urinary retantion and clonus were observed during his hospitalization. Bilateral basal ganglia calcification was demonstrated by computerized tomography neurologic findings were included to be due to metoclopramid which had been begun for vomiting. Bilateral basal ganglia calcification was decided to be due toDownsyndrome after excludingTORCH,metabolic and endocrin causes

Outcome of treosulfan-based reduced-toxicity conditioning regimens for HSCT in high-risk patients with primary immune deficiencies

Introduction: HSCT is the curative therapeutic option in PIDs. Due to the increase in survival rates, reduced-toxicity conditioning regimens with treosulfan have become another alternative. The purpose of this retrospective study was to analyze the outcome of treosulfan-based conditioning before HSCT for patients with PID. Method: A total of 15 patients that received a treosulfan-based conditioning regimen for HSCT were recruited. Type of diagnosis, donor and stem cell source, pretransplant organ damage, infections, engraftment, chimerism, and transplant-related toxicities were analyzed. Results: At a median follow-up time of 32 months, the overall survival was 86.7%. Following HSCT, 14 of 15 patients had engraftment, with 86.7% of the cohort having full-donor chimerism. The most common toxicity was seen on the skin (53.3%). Acute GVHD and chronic GVHD were documented in 53% and 20% of the study population, respectively. Although the cohort consisted of patients with pretransplant liver damage, SOS manifestations were documented in 20%. Conclusion: Treosulfan-based conditioning regimens before HSCT are associated with lower toxicity compared to myeloablative regimens, are safe, and have high engraftment rates with full-donor chimerism in patients having PID, regardless of the specified genetic diagnosis and donor type

Efficiency of Fluid Treatments with Different Sodium Concentration in Children with Type 1 Diabetic Ketoacidosis

Objective: The management of children with diabetic ketoacidosis (DKA) continues to be a controversial issue with regard to amount of intravenous fluid to be given, rate of delivery of fluid, and type of fluid to be used. We aimed to analyze the results obtained by administration of rehydration fluids of two different sodium (Na) concentrations (75 mEq/L vs. 100 mEq/L ) in the treatment of children with DKA

Impact of the COVID-19 pandemic on diabetic ketoacidosis management in the pediatric intensive care unit

Background Diabetic ketoacidosis (DKA) is a common endocrine emergency in pediatric patients. Early presentation to health facilities, diagnosis, and good management in the pediatric intensive care unit (PICU) are crucial for better outcomes in children with DKA. Methods This was a single-center, retrospective cohort study conducted between February 2015 and January 2022. Patients with DKA were divided into two groups according to pandemic status and diabetes diagnosis. Results The study enrolled 59 patients, and their mean age was 11±5 years. Forty (68%) had newly diagnosed type 1 diabetes mellitus (T1DM), and 61% received follow-up in the pre-pandemic period. Blood glucose, blood ketone, potassium, phosphorus, and creatinine levels were significantly higher in the new-onset T1DM group compared with the previously diagnosed group (P=0.01, P=0.02, P<0.001, P=0.01, and P=0.08, respectively). In patients with newly diagnosed T1DM, length of PICU stays were longer than in those with previously diagnosed T1DM (28.5±8.9 vs. 17.3±6.7 hours, p<0.001). The pandemic group was compared with pre-pandemic group, there was a statistically significant difference in laboratory parameters of pH, HCO3, and lactate and also Pediatric Risk of Mortality (PRISM) III score. All patients survived, and there were no neurologic sequelae. Conclusion Patients admitted during the pandemic period were admitted with more severe DKA and had higher PRISM III scores. During the pandemic period, there was an increase in the incidence of DKA in the participating center compared to that before the pandemic

ÇOCUK COVID-19 HASTASININ ACİL HAVAYOLU YÖNETİMİ VE TRAKEAL ENTÜBASYONU İÇİN ÖNERİLER

[Abstract Not Available

COVID-19 İLİŞKİLİ DOLAŞIM BOZUKLUĞU OLAN ÇOCUKLARDA ŞOKUN TANIMLANMASI VE TEDAVİ İLKELERİ

[Abstract Not Available

COVID-19 PEDİYATRİK AKUT RESPİRATUVAR DİSTRESS SENDROMU (PARDS) PROTOKOLÜ

[Abstract Not Available

CASE REPORT - Hyponatremia as a cause of prolonged seizures in a child with sickle cell anemia

We report a successful outcome on recurrent hyponatremic seizures, treated with agressive sodium replacement therapy in a 12-year-old girl with sickle cell anemia. The cause of her hyponatremia was probably tubular damage due to vaso-occlusive crisis. We achieved rapid correction in neurologic findings, serum sodium level and urinalysis with sodium replacement and fluid therapy in this patient. We excluded stroke based on the findings in cranial magnetic resonance imaging. We conclude that severe seizures found in sickle cell anemia may result from hyponatremia that can be treated by sodium replacement therapy. (J Pediatr Neurol 2004; 2(4): 231-233)