Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

Clinical, electrophysiological and neuropsychological findings of twenty-two children with mesial temporal sclerosis.

Twenty-two patients, followed with the diagnosis of epilepsy between the ages of 8.5 and 19 years who were found to have mesial temporal sclerosis (MTS) on brain magnetic resonance imaging (MRI) were evaluated according to their clinical, electrophysiological and neuropsychological characteristics, and assessed for the significance of neuropsychological tests on lateralization. In both MTS groups, a remarkable number of patients had their intial seizure before one year of age (45.5% in the right MTS group, and 36.4% in the left MTS group). Half of the patients were seizure-free for a period of two months-9.5 years (average 3.9 years). The duration between the febrile seizure and first non-febrile seizure was 6.2 years. Eleven patients had MTS on the left temporal lobe. Interictal EEG was normal in five (22.5%) patients; 10 (45.5%) had temporal spike on the corresponding side with MTS. Six (27.3%) patients had paroxysmal activity and voltage asymmetry on cortical areas other than temporal region. The Wechsler Memory Scale (WMS), Visual Reproduction Subtest and Auditory Verbal Learning Test (AVLT) Delayed Recall and Retrieval Subtest performances were poor in the right MTS group.

Rhombencephalosynapsis associated with cutaneous pretibial hemangioma in an infant.

Rhombencephalosynapsis is a rare congenital abnormality characterized by noncleavage of the cerebellar hemispheres and dentate nuclei with agenesis or severe hypoplasia of the cerebellar vermis. We report an 8-month-old boy with hypotonia, mental and motor retardation, and capillary hemangiomas on his pretibial region. Cranial magnetic resonance imaging revealed vermian hypoplasia with fused cerebellar hemispheres called rhombencephalosynapsis. Other cerebellar and cerebral structures were normal. With this constellation of findings, we attempt to identify the pathogenesis of this posterior fossa malformation. We also discuss cerebellar embryogenesis to differentiate this condition from other posterior fossa malformations such as Dandy-Walker malformation and Joubert's syndrome.

Hemophagocytic syndrome as an initial presentation of miliary tuberculosis without pulmonary findings.

A 9-y-old girl was admitted with fever, weakness and weight loss. She had pancytopenia in peripheral blood, hypocellularity and hemophagocytosis in bone marrow. Disseminated tuberculosis was diagnosed after a long delay, with involvement of the lungs, bone marrow, liver, spleen and central nervous system. Tuberculosis can be a cause of hemophagocytosis and should be taken into account in the differential diagnosis of fever of unknown origin associated with pancytopenia and hemophagocytosis.

Pontocerebellar hypoplasia in two siblings with dysmorphic features.

We present two siblings with pontocerebellar hypoplasia who have progressive microcephaly, mental and motor retardation, truncal ataxia, strabismus, and progressive spasticity and hyperreflexia of the lower limbs. Extrapyramidal dyskinesia and epilepsy, other main clinical features of pontocerebellar hypoplasia, are absent. The older sibling also has a high arched palate, triangular-shaped face, thoracolumbar scoliosis, pectus carinatum, kyphosis, cubitus valgus, arachnodactyly, long extremities, and a tall stature, which were not previously reported in association with pontocerebellar hypoplasia. The clinical phenotype should be expanded, especially within type II, with the reports of additional cases.