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.

The Effect of Oral Protein Supplementation on the Growth of Very Low Birth Weight Preterm Infants Admitted to the Neonatal Intensive Care Unit: A Randomized Clinical Trial.

BACKGROUND: During NICU admission, extra-uterine growth retardation that can affect the neurodevelopmental outcome is a challenging problem in extremely preterm infants. This trial aimed to determine the effect of additional enteral protein supplementation on the growth velocity of the anthropometric parameters. METHOD: In this randomized controlled trial, 77 preterm infants (gestational age ≤33 weeks and birth weight <1500 g) who reached full enteral feeding with either fortified breast milk or preterm formula were included. They were randomized to receive either 4-<5 g/kg/day protein through extra protein supplementation (intervention) or 3-<4 g/kg/day protein. Weight gain, as well as length and head circumference growth, were monitored daily and weekly, respectively. Venous blood gas, blood urea nitrogen (BUN), and albumin levels were checked weekly. RESULTS: Five out of 77 participants were excluded due to feeding intolerance. Analyses were conducted on 36 neonates with protein intake of 3.66 ± 0.22 gr/kg/day and 36 with extra protein intake. Baseline characteristics were similar between the groups. An additional protein supply of 0.89 gr/kg/day, resulting in an average protein intake of 4.55 ± 0.18 in the intervention group, increased the postnatal weight gain, linear growth, and head circumference growth (7.98 gr/kg/day, 0.347 cm/week, and 0.38 cm/week, respectively). The albumin levels were significantly increased, but the BUN levels were not significantly increased in the intervention group. None of the patients developed necrotizing enterocolitis or significant acidosis. CONCLUSION: Protein supplementation significantly improves the growth of the anthropometric parameters. An increase in serum albumin and no increase in serum urea can indicate the anabolic effect of extra protein. Protein supplementation can add to routine feeding protocols of VLBW infants without any short-term adverse effect; however, further study for evaluation of long-term complications is needed.

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.

Delayed cord clamping for prevention of intraventricular hemorrhage in preterm neonates: a randomized control trial.

BACKGROUND: Intraventricular hemorrhage (IVH) is a common condition in preterm neonates and is responsible for substantial adverse neurodevelopmental outcome in preterm neonates. Prevention of IVH is an important intervention for better neurological outcome in these preterm neonates. AIMS AND OBJECTIVE: This study aimed to determine whether delayed cord clamping (DCC) was superior to immediate cord clamping (ICC) for the prevention of IVH in preterm neonates. PATIENTS AND METHODS: In this two centered prospective double-blind randomized controlled trial, eligible neonates with gestational age from 26 to 34 weeks were randomized to receive either ICC (cord clamped in 10-15 s) or DCC (cord clamped in 30-45 s) groups. The grading and severity of IVH were evaluated by cranial ultrasound scan done on the 3-4th and 7-10th days after birth. RESULTS: Among the 148 enrolled neonates, 79 were in the ICC group and 69 were in the DCC group. There was no difference in maternal and neonatal baseline characteristics except the neonates in the DCC group weighed more (ICC 1528.77 ± 365.5 g vs. DCC 1658.11 ± 419.52 g; p = .047) at birth. There was no significant difference in the incidence of any grade of IVH in both groups (ICC 12.8% vs. DCC 14.5%; p = .745). There was a significantly higher incidence of grade I IVH (ICC 2.5% vs. DCC 13%; p = .024) in the DCC group. The incidence of grade II IVH (ICC 5.1% vs. DCC 0%; p = .123); grade III IVH (ICC 3.8% vs. DCC 1.4%; p = .623); and grade IV IVH (ICC 1.3% vs. DCC 0%; p>.999) were comparable between the two groups. The incidence of a significant IVH (grades II, III, and IV) was significantly less in the DCC group (ICC 10.1% vs. DCC 1.4%, p = .036). The mean initial hemoglobin levels were significantly higher in neonates enrolled in DCC (15.41 ± 2.1 vs. 16.46 ± 2.45 g/dL; p = .007). There was a significant reduction in the number of days of hospital stay (ICC 18.78 ± 15.42 vs. DCC 13.21 ± 16.16; p = .002). There was no difference in initial hematocrit, platelet count, maximum bilirubin level, and Apgar score (p>.05). CONCLUSIONS: Although there was no reduction in any grade of IVH, the incidence of significant IVH (grades II, III, and IV) was significantly decreased with the use of DCC in preterm neonates. Delayed cord clamping also resulted in a significant increase in birth weight, higher hemoglobin levels, and shorter hospital stays without any increase in the risks of hyper-bilirubinemia, low Apgar score, and neonatal mortality. TRIAL REGISTRY: IRCT2014031116936N1, https://www.irct.ir/trial/15707.

Outcome evaluation of COVID-19 infected patients by disease symptoms: a cross-sectional study in Ilam Province, Iran.

BACKGROUND: Novel coronavirus disease-19 (COVID-19) was declared as a global pandemic in 2020. With the spread of the disease, a better understanding of patient outcomes associated with their symptoms in diverse geographic levels is vital. This study aimed to evaluate clinical outcomes of COVID-19 patients by disease symptoms in Ilam province, Iran. METHODS: This was a cross-sectional study. Data were collected from integrated health system records for all hospitals affiliated with the Ilam University of Medical Sciences between 26-Jan-2020 and 02-May-2020. All patients with a confirmed positive test were included in this study. Descriptive analyses, chi-square test, and binary logistic regression model were performed by using SPSS version 22. RESULTS: The mean age of participants was 46.47 ± 18.24 years. Of the 3608 patients, 3477 (96.1%) were discharged, and 129 (3.9%) died. 54.2% of the patients were male and were in the age group of 30-40 years. Cough, sore throat, shortness of breath or difficulty breathing, and fever or chills were the most common symptoms. Patients with symptoms of shortness of breath, abnormal radiographic findings of the chest, and chest pain and pressure were relatively more likely to die. According to binary logistic regression results, the probability of death in patients with shortness of breath, abnormal chest radiographic findings, and chest pain was 1.34, 1.24, and 1.32 times higher, respectively, than for those without. CONCLUSION: Our study provides evidence that the presentation of some symptoms significantly impacts outcomes of patients infected with SARS-CoV-2. Early detection of symptoms and proper management of outcomes can reduce mortality in patients with COVID-19.

Congenital Hypothyroidism in Preterm Newborns: A Retrospective Study Arising from a Screening Program in Fars Province, Southwestern Iran.

OBJECTIVES: There is a high interest in the early diagnosis of hypothyroidism in preterm newborns for preventing any intellectual disability. Our study sought to determine the incidence of abnormal thyroid-stimulating hormone (TSH) between three and six days old and in weeks two, six, 10, and 12 after birth to evaluate the validity of repeating the test in premature infants. METHODS: We conducted a retrospective review of 320 886 live births in Fars province, Southwestern Iran, from March 2014 to October 2017. TSH levels in premature infants were measured by heel prick test, and the data was collected from the central newborn screening center of Fars province. RESULTS: The number of premature newborns was 15 381, and the prevalence of hypothyroidism was 2.3%. Among 355 premature newborns with high TSH, 31.3% was detected in three to six days of life, 43.9% in the second week, 14.4% in the sixth week, 9.9% in the tenth week, and 0.6% in the twelfth week as hypothyroidism. CONCLUSIONS: Our results showed that thyroid screening of preterm infants needs retesting in two, six, and 10 weeks after to detect cases in newborns who would not otherwise be identified.

Incidence, risk factors and causes of severe neonatal hyperbilirubinemia in the South of iran (fars province).

BACKGROUND: Today, Severe hyperbilirubinemia is the most common cause of neonatal readmissions. Identification of the cause of neonatal hyperbilirubinemia is useful in determining whether therapeutic interventions can prevent severe hyperbilirubinemia. OBJECTIVES: We conducted this study to estimate the incidence of severe hyperbilirubinemia in Fars province and to determine the underlying causes and risk factors, which would be of value in identifying and implementing strategies to prevent morbidity from this condition. PATIENTS AND METHODS: All infants less than 28 days referred due to severe indirect hyperbilirubinemia were included. Complete history, physical examination and lab work up were performed. This is a longitudinal prospective study in 2009-2010. RESULTS: More common causes of severe indirect hyperbilirubinemia were blood group incompatibility, G6PD deficiency, sepsis and unknown. Risk factors of severe hyperbilirubinemia were Male sex, previous siblings with severe hyperbilirubinemia, early discharge, NVD, Breast feeding and cultural background of mothers. CONCLUSIONS: Our study showed severe neonate indirect hyperbilirubinemia is still prevalence in Fars province and ethnic and cultural background of the mothers was more effective than school education in preventing hyperbilirubinemia complication.

The value of bilicheck® as a screening tool for neonatal jaundice in the South of iran.

The gold standard to assess jaundice in neonates is the serum bilirubin measurement. Blood sampling for the determination of total serum bilirubin (TSB) is painful for newborns and stressful for parents. The Bilicheck®, a new transcutaneous bilirubinometer, is considered as a more accurate measurement of bilirubin compared to the previous bilirubinometers courtesy of its advanced technology. The objective of this study was to evaluate the correlation between transcutaneous bilirubin (TcB) measurements using the Bilicheck® device and TSB in some Iranian neonates and to determine the most reliable cut-off value with the highest sensitivity and desirable specificity for bilirubin measured by the Bilicheck® on the forehead. This prospective observational study was conducted in 2011 on 560 healthy neonates with jaundice. TcB was measured using the Bilicheck® (Respironic, USA) within 30 minutes of TSB measurement via direct spectrophotometry. The results were assessed by simple linear regression analysis and receiver operative characteristic curve. There was good a correlation between TcB and TSB (r=0.969, r(2)=0.94), and this was not affected by sex, gestational age, postnatal age, and birth weight. TSB can be calculated through the measurement of TcB and use of the linear regression equation: TSB=-0.99+1.06TcB. Sensitivity and specificity of the Bilicheck® at the most reliable cut-off value (15 mg/dl) were 96.6% and 99%, respectively. The findings of the present study indicate that the Bilicheck® is a non-invasive, simple, easy, and reliable method for bilirubin measurement in neonatal jaundice, especially in neonates with bilirubin levels ≤15 mg/dl.

Prevalence of Gilbert syndrome in parents of neonates with pathologic indirect hyperbilirubinemia.

BACKGROUND AND OBJECTIVES: The cause of hyperbilirubinemia cannot be found in about 45% of cases of neonatal jaundice. Gilbert syndrome (GS) is the most common congenital disease associated with bilirubin metabolism in the liver. Since the screening value of genetic tests cannot be fully determined until accurate data on the prevalence and penetrance of the GS genotype are known, we sought to estimate whether the prevalence of GS is higher in the parents of neonates with severe unexplained indirect hyperbilirubinemia. DESIGN AND SETTING: Case-control study of parents of neonates with severe unexplained indirect hyperbilirubinemia admitted to a neonatal ward. METHODS: We used the rifampin test (checked bilirubin before and 4 hours after administration of 600 mg rifampin) for diagnosis of GS in parents of 115 neonates with severe unexplained indirect hyperbilirubinemia. We compared the prevalence of GS in these parents with that of a control group of 115 couples referred for premarital counseling. RESULTS: The 115 neonates were aged 5.2 (1.6) days (mean, standard deviation), all were breast-fed, and males constituted 56.5%. Mean total serum bilirubin (TSB) level was 20.96 (5.48) mg/dL. 14.8% were glucose 6 phosphate dehydrogenase (G6PD) deficiency was present in 14.8%, and 10.4% had A, B or O blood group (ABO) incompatibilities with their mothers. There was no difference in the prevalence of GS between parents of the group with hyperbilirubinemia (22.2%) and the control group (19.13%) (P=.42). Mean TSB in neonates with parents who had GS was more (about 3 mg/dL) than in neonates with normal parents (P=.004). Fathers had GS twice as often as the mothers among the parents of neonates with hyperbilirubinemia (P=.003), among the control group (P=.009) and among neonates (P=.014). CONCLUSION: This study showed that GS cannot cause severe indirect hyperbilirubinemia by itself, but it may have a summative effect on rising bilirubin when combined with other factors, for example, G6PD. Our results showed that in GS, males are affected about twice as much as the females.