Our patient was a 6-month-old infant referred to our hospital with the chief complaints of fever, rapid breathing, and poor feeding from three days ago. There was no history of chocking, coryza, or gastrointestinal symptoms. She was the first child of the family. The mother conceived this girl after six years of infertility by in-vitro fertilization (IVF). She was delivered through cesarean section at 37 weeks of gestation and was hospitalized for nine days due to respiratory distress. There was no specific complaint after discharge from the hospital until the age of five months. The mother mentioned poor weight gain and occasional coughs, which had gradually exacerbated in the last month. The weight upon referral was 5. 2 kilograms, which was between 2nd and 5th percentile. Before admission to our hospital, she was referred to a pediatrician’, s office, and sweat test was performed and repeated, which revealed borderline results. She had received Creon capsule, montelukast tablet, and fluticasone spray for one month because of suspected cystic fibrosis (CF). She was also receiving levothyroxine because of congenital hypothyroidism...

Introduction: Cardiac catheterization is a common procedure which needs a careful coagulation monitoring. In our study, we aimed tofind factors influencing active clotting time (ACT) following heparin therapy.Methods: ACT of 71 patients who were scheduled to undergo transcutaneous diagnostic catheterization and angiography were measured at baseline, 2 and 60 minutes after 50 IU/kg heparin loading. ACT in two groups of patients (cyanotic and non-cyanotic) was compared. All data were analyzed with Wilcoxon, Mann-Whitney test and Pearson in SPSS 16, P value less than 0.05 was considered significant.Results: ACT following heparin at 2nd and 60th minutes was not significantly different in cyanotic and non-cyanotic groups. At 60th minute following heparin administration, ACT decreased more dramatically in older children.Conclusion: CYANOSIS does not affect ACT measures following heparin treatment. Moreover, after 60 minutes, heparin efficacy (ACT values) decreased more with increase in patients’ age.

Today surgical repair of different types of atrial septal defect (ASD) is considered as relatively a simple and safe procedure with minor to nil morbidity and mortality. When a complication occurs after ASD repair, is associated with considerable morbidity and mortality, which is much higher than primary surgical procedure. We report two patients that underwent ASD repair with caval inflow occlusion without CPB with late presentations of iatrogenic diversion of inferior vena cava to left atrium and subsequent complications of right to left shunting.

Dear Editor, The clinical signs and symptoms of perinatal arterial ischemic stroke (PAIS) may be subtle and nonspecific. Imaging studies provide a good choice for early diagnosis. We report a case of extensive arterial ischemic infarct in a full-term neonate, with a focus on ultrasound and MRI findings.

Introduction: Unilateral and bilateral tongue CYANOSIS usually occurs due to the Raynaud syndrome and in the underlying severe types of vasculitis and rheumatology. Case Presentation: The present study was conducted on a 54-year-old woman who referred to the emergency department with complaints of sudden and painless discoloration of the left half of the tongue. The patient had no history of disease other than diabetes controlled with glibenclamide. Clinical examination of the head and neck revealed evidence of unilateral CYANOSIS in the left half of the tongue without pain, whose discoloration did not improve with warming of the tongue. Conclusion: CYANOSIS in the emergency department can be managed appropriately by considering some parameters including history taking, history of CYANOSIS occurrence, history of cardiopulmonary disease, cold sensitivity and history of rheumatologic diseases, presence or absence of nail clubbing, arterial blood oxygen saturation and arterial blood gas test results. These parameters can be effective in designing a treatment regimen, while differentiating the causes of central from peripheral CYANOSIS.

Introduction: Congenital portosystemic shunts constitute a rare disorder in childhood with a wide spectrum of symptoms and signs, one of which may be CYANOSIS. Case Presentation: We describe for the first time in Iran a 9-year-old boy with a portacaval shunt presenting with CYANOSIS. He was diagnosed, managed, and treated successfully. The postoperative period was complicated by portal vein thrombosis, which was gradually resolved with appropriate treatment. Conclusions: CYANOSIS is one of the hallmarks of cardiopulmonary disease; however, as was the case in our patient, a secondary cause of pulmonary disease should be considered. The portacaval shunt is a rare but important cause of CYANOSIS and should be considered in all patients in whom CYANOSIS is unexplained.

Background: Hypertrophic cardiomyopathy (HCM) is defined by the presence of significant left ventricular hypertrophy (LVH) in the absence of secondary factors like systemic hypertension, aortic stenosis, and athlete's heart syndrome. Case presentation: A 67-year-old woman, with a complaint of severe fatigue, peripheral CYANOSIS on normal daily activity life, and paroxysmal nocturnal dyspnea, was admitted to Cardiac Care Unit, Razavi Hospital, Mashhad, Iran. In the primary physical examination, cardiac auscultation revealed pathologic S4 sound. Clinical investigations such as electrocardiography, chest X-ray, and echocardiography approved Apical Hypertrophic Cardiomyopathy (AHCM). Only administration of Metoprolol succinate with a short-term follow-up showed completely relieved pathologic presentation of this case. Conclusion: In this case report, the management of a patient with peripheral CYANOSIS on normal activity, paroxysmal nocturnal dyspnea, and AHCM was emphasized. This case showed that early diagnosis followed by medication and supportive care, can control the patient's symptoms and postpone the progression of heart failure symptoms.

To evaluate the prognosis of BHS after iron treatment, 35 children, including 19 boys, and 16 girls, between 3 to 60 months (median age of onset 6 to 24 months) with these attacks were followed prospectively for a period of 3 months after iron treatment. 31 patients had CYANOSIS, 3 had pallor, and one had mixed attacks. Frequency and severity of their attacks were recorded, before and after our treatment, by their mothers. All patients were evaluated initially for hematological indices. Complete responses were noticed in 24 patients, favorable response in 9, poor response in 1, and one with no response at all, which were placed in different categories, according to their iron deficiency levels.Among these patients, 14 had iron deficiency anemia, 20 had Iron deficient erythropoieses, and only one with no anemia. Only 2 of them had received regular iron supplement diets, 3 non-regulars, and 30 of them had received no iron supplements. But no marked differences were seen in their responses to the treatment. The results indicate that iron therapy has an impressive effect in diminution of BHS attacks in infants.  

Background: We report five term neonates born at an altitude of 1890 meters with transient early neonatal CYANOSIS due to right-to-left shunting at atrial level through patent foramen ovale.Case Presentation: The five neonates with no clinical sign or symptom other than marked CYANOSIS were examined in two neonatal units of Erzurum city. Hematologic and radiologic examinations were normal. Partial oxygen pressure (PO2) in the arterial blood samples was lower than 45 mmHg in all of the patients, and did not increase more than 15 mmHg in any of the patients after inhalation of 100% oxygen. Echocardiography revealed normal intracardiac structure. The right-to-left interatrial shunt at diastole was detected through a patent foramen ovale in all of these infants. By only observation with no treatment, diastolic right-to-left shunt disappeared in 40.15±9.52 hours. Oxygen saturation was increased from 69.80±9.55 percent to 90.40±8.80 percent. The patients were discharged from the hospital at 5.6±0.4 days of life. Follow up for 6 months revealed no clinical problem in any of the cases.Conclusion: Transient CYANOSIS can be seen in the very early neonatal period because of interatrial right-to-left shunting in some healthy term neonates born at an altitude of 1890 meters. Decreased right atrial compliance due to relative hypoxia at that altitude can be speculated to be the causative mechanism.

Congenital methemoglobinemia is a rare cause of CYANOSIS. We report a case of a girl, 17 years old with peripheral CYANOSIS and normal cardio-pulmonary system. She was diagnosed as acase of methemoglobinemia based on findings of polycythemia and HbM band on hemoglobin electrophoresis. We emphasize the importance of this rare entity in the differential diagnosis of CYANOSIS.