Objective: To evaluate patients who underwent acute and chronic peritoneal dialysis under the age of one one in terms of etiology, complications, and prognosis over 20 years and to compare the results between the first and last 10 years of acute and chronic peritoneal dialysis. Material and Method: Seventy-four peritoneal dialysis patients under the age of one in the Division of Pediatric Nephrology and Neonatal Intensive Care Unit of our hospital between January 2002 and December 2023 were evaluated retrospectively. The patients were divided into two groups: patients admitted in the 2002-2013 period (Group I) and patients admitted in the 2013-2023 period (Group II). Patients in Group I and Group II were compared in terms of acute and chronic peritoneal dialysis etiology, complications, and prognosis. Result: Forty-four of the patients (60%) were newborns, and the remaining 30 were infants (40%). There were 39 patients in Group I and 35 patients in Group II. There was no difference between Group I and Group II for acute dialysis in terms of neonatal and infant diagnoses, infectious and non-infectious complications, and prognosis (p>0.05). There was no difference between Group I and Group II in terms of neonatal and infant diagnoses, infectious and non-infectious complications, and infant prognosis (p>0.05). There was no death in the newborns in Group II and patient survival was higher than in the newborns in Group I (p=0.019). Conclusion: Peritoneal dialysis is the most commonly used method for renal replacement therapy in children facing both acute and chronic renal failure. In the last decade, despite high-quality care in the neonatal care unit, positive technological developments and effective management of PD, the most common infectious complication was still peritonitis, and the non-infectious complication was dialysate leakage, as in the previous 10 years.
The phenotypic spectrum of Bruck syndrome has broadened since its first descrip - tion. Besides its orthopedic manifestations, other findings such as myopathy or car - diac disease have been reported in previous studies. A case is presented with Pierre Robin syndrome and subglottic mass. Because of the clinical picture of congenital high airway obstruction syndrome, emergency tracheotomy was performed upon delivery. An excisional biopsy under direct laryngoscopy was done later. The patient died of congestive heart failure when 8 months old. In conclusion, Bruck syndrome types 1 and 2 may not be phenotypically equivalent and there may be unexpected upper respiratory system findings.
Inviting Innovations their inter connections, anterior cerebral artery, pericallosal artery, thalamoperforating artery and quadrigeminal arteries also support the blood supply.Mural type (Type II), has a single or multiple arteriovenous fistulas (AVF) which drain the median prosencephalic vein at the inferolateral mural side.The shunt is usually fed by collicular and posterior choroidal arteries.The resultant AVF leads to an abnormal flow which prevents the regression of the embryonic vein, generating VGAM.There are also mixed types of VGAM carrying the characteristics of both choroidal and mural types.Yasargil classification has four types.In types I, II and III, all lesions have direct fistulous connections with the vein of Galen.In type IV, there is a paranchymal arteriovenous malformation which drains into the vein of Galen.In Type I, there is an AVF between vein of Galen and pericallosal branches of leptomeningeal arteries and/or any branch of posterior cerebral artey.In Type II, vein of Galen is fed by two branches of posterior cerebral artery and thalamoperforating vessels.Type III is a mixture of type I and type II and is the most common form.Type IV is known as the secondary type and has three subtypes: In Type IVA an aneurysmal dilatation develops in the vein of Galen by the neighbouring thalamic arteriovenous malformation.Type IVB is similar to type IVA, but arteriovenous malformation is mesencephalic rather than thalamic.In Type IVC thalamomesencephalic or mesodiencephalic plexiform malformation drains into the vein of Galen by a nearby and different cisternal AVF.Yasargil classification is helpful when an open surgery is indicated, whereas Lasjaunias classification is helpful if endovascular intervention is the treatment option [4].Prenatal diagnosis is made by color Doppler ultrasound examination in the third trimester of pregnancy.Aneurysmatic dilatation localized at the midline and posterior to the third ventricle, venous and arterial turbulent blood flow in hypoechoic structures can be shown [7].Fetal magnetic resonance imaging (MRI) is needed both to confirm VGAM and to exclude the diagnosis of arachnoidal,
Objective: To assess the clinical features, diagnosis, management strategies and results of newborns born between the years 2005-2015 with Vein of Galen Malformation (VGAM). Material and Method: Eight newborn patients with diagnosis of VGAM were assessed retrospectively in terms of clinical admission symptoms, diagnosis, treatment strategies and follow-up based on the information retrieved from patient files. Three of four patients who survived had neurological assessment while the information about the remaining patient whose family moved to another city were obtained via phone call. Results: Seven out of 8 patients (1 female, and 7 male patients) had antenatal diagnosis. In all cases severe heart failure and pulmonary hypertension were present from the first days of their life, later on development of hypotension, multiorgan failure, hydrocephalus and seizures was seen. VGAM and its feeder arteries were mapped by cranial magnetic resonance imaging and magnetic resonance angiography. Transarterial embolization therapy was performed successfully in four patients. Despite similar treatment modalities three of these patients died, and one patient was lost before initiation of embolization procedure. Conclusion: VGAM has high mortality and morbidity rates because of its complicated anatomy, pathophysiology leading to severe neurological sequelae. Prognosis in high risk neonates can be improved with aggressive medical support and early endovascular embolization therapy.
We report the occurrence of supraventricular tachycardia during intravenous immunoglobulin (IVIG) infusion. Supraventricular tachycardia was observed in two newborn patients during IVIG infusion. Both of the babies responded to adenosine treatment. Cardiorespiratory monitoring during IVIG infusion can be recommended because of the possibility of this potentially lifethreatening adverse effect.
The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors, particularly hypertension in juvenile-onset systemic lupus erythematosus (SLE).
Macrocephaly-capillary malformation syndrome is characterized by cutaneous vascular malformations with associated anomalies as macrocephaly, macrosomia, hemihypertrophy, hypotonia, developmental delay, lax joints, loose skin, polysyndactyly, and neuroimaging abnormalities. We present a newborn with a prenatal diagnosis of macrosomia and tetralogy of Fallot. He also had macrocephaly; a high forehead; capillary hemangioma on the forehead, upper lip, and philtrum; generalized loose skin; postaxial polydactyly of both hands and feet, with neuroimaging findings of polymicrogyria and thrombosis in sagittal sinus and sinus rectus. His condition was diagnosed as macrocephaly-capillary malformation syndrome in the neonatal period and he died suddenly during sleep at 6 months of age. The clinical course in this syndrome is not as benign as was previously thought. Careful follow-up of these patients with particular emphasis on neuroradiologic and cardiologic evaluation might help decrease the risk of sudden death and to improve long-term outcome.
A 7-year-old boy with pica presented with a diagnosis of leukemia. He did not have hepatosplenomegaly, lymphadenopathy, or infection. Laboratory results were as follows: hemoglobin (Hb): 6.9 g/dL; mean corpuscular volume (MCV): 66 fL; red cell distribution width (RDW): 20.3%. Iron studies results were consistent with iron deficiency, as follows: serum iron: 13 μg/dL; total iron binding capacity: 416 μg/dL; ferritin level: 10 ng/mL. The patient’s white blood cell (WBC) count was 2.9×103/μL (1.5×103/μL neutrophils) and platelet count was 28×103/μL. Bone marrow aspiration showed a normal number of megakaryocytes, low-level stored iron, and no blasts. The patient was diagnosed as IDA. On d 5 of iron treatment the patient’s findings were as follows: Hb: 7.4 g/dL; MCV: 68 fL; RDW: 25.7%; WBC: 5.6×103/μL; platelet count: 430×103/μL.
Dandy-Walker variant (DWV) might be considered as one of the borderlines in the examination of fetal brain. We report the first case of a DWV in a fetus conceived by ICSI. A 34 year-old woman underwent assisted reproductive treatment due to male factor infertility. A twin gestation was achieved, one of the fetuses was diagnosed as DWV on 22 weeks' ultrasound examination. Fetal MRI confirmed the diagnosis. The couple did not allow fetal karyotyping. Pregnancy continued uneventfully. Associated cardiac and minor skeletal anomalies were seen in the postpartum examination of the affected newborn. She died six days after birth due to pulmonary insufficiency. Karyotype analysis of the affected infant performed after birth was reported to be partial trisomy 9. Parental karyotype analysis was further performed and balanced reciprocal translocation t(4;9) was found in the mother. A careful posterior fossa evaluation should be performed as part of routine anatomical survey in ICSI conceptions. Upon the detection of an anomaly, a comprehensive fetal ultrasound for associated abnormalities and karyotype analysis are vital for obstetric management and neonatal survival.