Exchange Transfusion Trends and Risk Factors for Extreme Neonatal Hyperbilirubinemia over 10 Years in Shiraz, Iran.

Background: Exchange transfusion (ET) is an effective treatment for acute bilirubin encephalopathy and extreme neonatal hyperbilirubinemia (ENH). It can reduce mortality and morbidity. This study aimed to investigate the trends and risk factors of ENH requiring ET in hospitalized neonates in Iran. Methods: A retrospective analysis of medical records of neonates who underwent ET due to ENH was conducted from 2011 to 2021, in Shiraz, Iran. Clinical records were used to gather demographic and laboratory data. The quantitative data were expressed as mean±SD, and qualitative data was presented as frequency and percentage. P<0.05 was considered statistically significant. Results: During the study, 377 ETs were performed for 329 patients. The annual rate of ET decreased by 71.2% during the study period. The most common risk factor of ENH was glucose-6-phosphate dehydrogenase (G6PD) deficiency (35%), followed by prematurity (13.06%), ABO hemolytic disease (7.6%), sepsis (6.4%), Rh hemolytic disease (6.08%), and minor blood group incompatibility (3.34%). In 28.52% of the cases, the cause of ENH was not identified. 17 (5.1%) neonates had acute bilirubin encephalopathy, of whom 6 (35.29%) had G6PD deficiency, 6 (35.29%) had ABO incompatibility, and 2 (11.76%) had Rh incompatibility. Conclusion: Although the rate of ET occurrence has decreased, it seems necessary to consider different risk factors and appropriate guidelines for early identification and management of neonates at risk of ENH should be developed. The findings of the study highlighted the important risk factors of ENH in southern Iran, allowing for the development of appropriate prevention strategies.

Generalized cutis marmorata telangiectatica congenita or neonatal lupus? A case report and literature review.

Cutis marmorata telangiectatica congenita (CMTC) presents as marbled erythema and may exhibit diverse associated anomalies. Thorough multidisciplinary evaluation is crucial. Treatment varies with inconclusive evidence, necessitating further research. Our case underscores CMTC's rarity and heterogeneous nature, advocating for comprehensive management approaches and ongoing research.

Operation of a giant occipital encephalocele in an infant: A surgical case report.

INTRODUCTION AND IMPORTANCE: Encephalocele is a rare medical condition where certain parts of the central nervous system protrude through a skull defect, resulting in a deformity where the head size is smaller than the protrusion. This condition is relatively uncommon, and only a few cases have been reported worldwide. CASE PRESENTATION: We present a case of a 13-day-old neonate with a giant occipital encephalocele who underwent a successful surgical intervention in a resource-limited setting. CLINICAL DISCUSSION: The diagnosis of encephaloceles is frequently by clinical examination, although sonography could be helpful before birth. It is crucial that this patient receives immediate surgical intervention. In cases where hydrocephalus and ventriculomegaly are absent, we predict a better prognosis. The prone position is preferred in these operations, and Anesthesia is a real challenge. CONCLUSIONS: Congenital giant occipital encephaloceles can be identified clinically shortly after birth. They cause a substantial surgical challenge due to their massive size. Surgical repair must be performed as early as possible.

Unilateral diaphragmatic paralysis and complete brachial plexus injury in a complex birth injury: A rare case report mimicking Tarlov cyst and review of literature.

The rare occurrence of Tarlov cysts in pediatric patients, particularly in the context of complex birth injuries, necessitates thorough evaluation and tailored management approaches. A comprehensive understanding of the clinical significance and optimal treatment strategies for this unique combination is crucial to ensure effective and individualized care for affected children.

Necrotizing fasciitis in neonates: A case report and review of literature.

Early recognition and treatment of necrotizing fasciitis reduce mortality rate which is 24.1% among neonates. Antibiotics and debridement are common treatments. Hyperbaric oxygen and negative pressure wound healing show potential but need further investigation.

Persistent pulmonary hypertension of the newborn due to methylmalonic acidemia: a case report and review of the literature.

BACKGROUND: Persistent pulmonary hypertension of the newborn manifesting with refractory and severe cyanosis is the consequence of high pulmonary vascular resistance causing extrapulmonary right-to-left shunt. Acidosis and hypoxemia produce pulmonary vasoconstriction. Persistent pulmonary hypertension of the newborn occurs due to numerous disorders and has been rarely reported as a manifestation of methylmalonic acidemia. We report a newborn with methylmalonic acidemia who presented with persistent pulmonary hypertension of the newborn. CASE PRESENTATION: A 1-day-old Iranian girl presented with respiratory distress and refractory metabolic acidosis. She was born at 39 + 5 weeks gestational age with Apgar scores of 8 and 9 in the 1st and 5th minutes, respectively, and was in good condition up to 10 hours of life. After that, she presented with cyanosis, tachypnea, retraction, and hypotonia. Despite receiving oxygen, she had low oxygen saturation. Echocardiography revealed severe pulmonary hypertension and right-to-left shunt through patent ductus arteriosus and foramen ovale. Her acidosis worsened despite receiving full support and medical therapy. So, she was started on peritoneal dialysis. Unfortunately, she did not respond to treatment, and after she had died, biochemical tests confirmed methylmalonic acidemia. CONCLUSION: Persistent pulmonary hypertension of the newborn is a very rare manifestation of methylmalonic acidemia. Severe inborn errors of metabolism may cause irreversible damage with adverse lifelong morbidity, and early diagnosis may help to prevent such complications. Furthermore, diagnosis of these disorders aids in prenatal diagnosis through the use of cultured amniocytes or chorionic villi to detect gene mutations, as well as biochemical analyses of amniotic fluid for subsequent pregnancies.

Adrenal insufficiency as a rare manifestation of secondary antiphospholipid syndrome: A pediatric case report and review of articles.

Key Clinical Message: Adrenal insufficiency is a rare, important manifestation of secondary antiphospholipid syndrome (APS) in pediatrics. In the presence of hematologic disorders such as thrombosis, we should consider APS. Abstract: Adrenal insufficiency can rarely occur in the context of vascular disorders and thrombosis in patients with antiphospholipid syndrome. There are few case reports in pediatrics. Here, we present a pediatric case-the first pediatric case report in Iran-and review articles in this age group.

Giant umbilical cord in a normal preterm infant: a case report and review of the literature.

BACKGROUND: Giant umbilical cord, defined as a cord diameter of more than 5 cm, is an extremely rare malformation. There are few case reports of giant umbilical cord often associated with patent urachus duct or cystic malformation. These cases are usually managed by surgical excision and repair of patent urachus or cyst resection. CASE PRESENTATION: We report the case of a 1-day-old Iranian boy with giant umbilical cord detected postnatally. The pregnancy course was uneventful, except for preterm premature rupture of the membrane and preterm delivery. There was no relevant family history. The patient was delivered by vaginal delivery with a good Apgar score. On clinical examination, the umbilical cord was very thick (about 6 cm in diameter), and huge fluctuating Wharton's jelly was observed. Other organs were normal. During the hospital stay, the patient did not develop any complications except borderline hyperbilirubinemia, which improved with conventional phototherapy. Since the umbilical cord had no discharge and was dried, the newborn was discharged with advice for cord drying care. CONCLUSION: The newborn was well, and the dried umbilical stump was detached after 32 days, leaving a granulomatous structure without discharge. The patient was followed up for 4.5 months and had no problems except delayed separation of the umbilical cord.

Complications and Related Risk Factors of Peripherally Inserted Central Catheters in Neonates: A Historical Cohort Study.

BACKGROUND: Peripherally inserted central catheters (PICCs) are an effective method for medication and nutrition infusion in preterm neonates. The present study aimed to identify the incidence of the most common complications of PICC implantation and evaluate the risk factors of each complication. METHODS: This historical cohort study was conducted on 2500 neonates with birth weights (BWs)≥500 g and gestational age (GA)>24 weeks who had a history of PICC inserted in three NICUs between August 2015 and August 2018. Data were collected by reviewing medical records. Demographic data and indices of catheter placement, duration of catheter placement, and common complications were recorded. Data analysis was done using SPSS-21. RESULTS: The median cubital vein had the most PICC placement (43%). The most common complication was tip malposition (48.2%). The incidence rates (95% CI) of the main complications such as malposition, edema/occlusion, and PICC migration were 0.0356 (0.0337-0.0377), 0.0134 (0.0122-0.0147), and 0.0088 (0.0079-0.0099), respectively. PICC insertion position was the strongest predictor of malposition for the cephalic vein. Besides, the incidence of malformation in the cephalic vein was about six times higher than in the median cubital vein. Independent risk factors for non-technical complications included BW (OR=0.59, 95% CI 0.44-0.79), administration of hyperosmolar medications (OR=3.43, 95% CI 2.62-4.51), position (OR=2.43, 95% CI 1.92- 3.08), and duration of catheter presence (OR=1.02, 95% CI 1.01-1.03) (P<0.001). CONCLUSION: The most common complication was malposition related to catheter placement in an emergency. Moreover, BW, administration of hyperosmolar medications, and duration of catheter presence were the most critical risk factors for non-technical complications. Therefore, it is recommended to educate the PICC insertion team to reduce tip malposition and replace long-term catheters.

Epidemiologic study of patients with thrombotic events referred to a tertiary hospital in Southern Iran.

BACKGROUND AND AIM: Thromboembolic events mainly occur in older age is related with high morbidity and mortality, and considerable health-care costs particularly in developing countries. Both arterial and venous thromboembolism has known risk factors such as hyperlipidemia, obesity, diabetes, cancer, major surgery, central catheter. We aimed to evaluate the occurrence of thrombotic events and related risk factors in a group of Iranian patients. METHODS: In this cross-sectional study, all patients (n = 99) who were complicated by thrombotic events referred to the Hematology Research Center of Shiraz University of Medical Sciences were investigated from 2015 to 2017, in Shiraz, Southern Iran. Data were collected from their medical records by a designed data gathering form. RESULTS: The median age of the occurrence of thrombosis was 51 (IQR: 31) years. From all thrombotic events 52.5% occurred in females. Venous thrombosis was more prevalent than arterial (61.6% vs. 38.4%). Hypertension, diabetes mellitus and ischemic heart disease were the most associated disease with thrombosis. Most of the patients (79.8%) had no episodes of relapse and the occurrence of relapse had no significant relationship with thrombophilia and underlying disease. Acceptable response rate for warfarin therapy was achieved in 46.5% with 5 mg and 43.4% with 5-7.5 mg. CONCLUSION: Knowing the frequency and risk factors for thrombotic events lead to timely diagnosis and management of thrombosis. Atrial fibrillation and valvular rheumatic heart disease are the most common risk factors of thrombosis in our study showing prophylaxis is necessary in high-risk patients.

Orbital mucormycosis in immunocompetent children; review of risk factors, diagnosis, and treatment approach.

BACKGROUND: Orbital mucormycosis is a rare but potentially severe and troublesome invasive fungal infection that could be occurred even in healthy individuals. The initial clinical presentation is similar to bacterial pre-septal or septal cellulitis, especially in early stages. CASE PRESENTATION: Herein, we describe the successful management of a series of five cases presenting with orbital mucormycosis in previously healthy children. CONCLUSIONS: Orbital mucormycosis is extremely rare in healthy children and maybe life-threatening when diagnosis delayed given a similar clinical presentation with bacterial septal cellulitis. Intravenous antifungal therapy with amphotericin B and timely surgical drainage is live-saving.