Stomnaroska Damchevski, Orhideja

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Stomnaroska Damchevski, Orhideja

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orhideja.sd@medf.ukim.edu.mk

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SITUS INVERSUS TOTALIS IN NEWBORN: A CASE REPORT
(Macedonian Association of Anatomists, 2024)
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Janchevska, Aleksandra
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Ristovska, Sanja
Situs inversus totalis (SIT) is a rare congenital abnormality and a complex disorder of embryological morphogenesis. It is characterized by a mirror-image transposition of the abdominal and the thoracic organs. Frequently it is associated with other congenital anomalies, congenital heart malformations being found in 3-9% of patients with SIT. Isolated SIT is also reported. We report a preterm newborn treated with non-invasive ventilation (CPAP) for respiratory distress. The X-ray of the lungs revealed the expected respiratory distress syndrome (RDS), but surprisingly and accidentally dextrocardia and left sided liver. Heart ultrasound showed dextrocardia without congenital anomaly. Abdominal ultrasound confirmed left-sided liver, and right sided spleen. After a lengthy 30 days stay the baby was discharged at home without any complication. SIT is an extraordinary rare condition in neonates. Timely diagnosis in-utero is important for prenatal and postnatal monitoring of newborns. Most of the patients with SIT without congenital anomalies do not have any complications in their life. However, appropriate information about the condition is important to prevent complications during some invasive and surgical interventions.
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Hypoxic Ischemic Encephalopathy (HIE) in Term and Preterm Infants
(Macedonian Academy of Science and Arts/Sciendo, 2022-04-22)
Ristovska Sanja
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Danilovski Dragan
Hypoxic-ischemic syndrome (HIS) and Hypoxic-ischemic encephalopathy (HIE) are conditions that affect term and premature babies, with different pathophysiology and different brain disorders. HIE appears in 1-6 / 1000 live births and 26/1000 live births in developing countries. 15-20% die in the early neonatal period, while surviving babies have severe neurological impairment, including cerebral palsy, epilepsy, visual and hearing impairment, cognitive impairment, intellectual, behavioural, and social disorders. The hypoxic-ischemic event occurs before, during or after birth. The reasons may be related to the mother, the way of birth, the placenta, and the newborn. The criteria for diagnosis of HIE include a combination of perinatal factors, the need for resuscitation, standard neurological examinations, neurophysiological monitoring, neuroimaging methods and biochemical markers. The most effective treatment for HIE is hypothermia in combination with pharmacological therapy. HIE and HIS are problem that still persist in developing countries due to inadequate obstetric care, neonatal resuscitation, and hypothermia. Current and emerging research for HIE examines new markers for early recognition, treatment, and appropriate neuroprotection of high-risk term and premature infants.
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NEURO DEVELOPMENTAL CONSEQUENCES OF NEONATAL HYPOGLYCEMIA
(Macedonian Academy of Sciences and Arts / Sciendo, 2020-09)
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Valentina Dukovska
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Neonatal hypoglycemia (HG) can cause neurologic damage, epilepsy, mental retardation, behavioral and personality disorders and death. The longest the HG lasts and the greatest the glucose nadir the consequences are more pronounced. Comorbidities are rather important in development of neurological damage. Hypoxemia and ischemia can cause permanent brain damage. Small for gestational age (SGA), large for gestational age (LGA), intrauterine growth restriction, gestational age bellow the 37th week, low Apgar score, sepsis, children whose mothers have toxemia, diabetes or chorioamnionitis are all newborns with increased HG risk. Comparing 34 patients with NH and 34 children without NH with similar GA, BW, BL, the Apgar score, we found statistically significant differences in motor and mental development using the Griffith scale. Children with neonatal HG fared significantly worse than those without neonatal HG. Therefore, CBG measurements and early recognition of neonatal HG is of significant importance in preventing motor and mental damage in children. A larger and well-balanced cohort of patients followed for a longer period is also necessary to clarify and discern in detail the importance of neonatal HG and other perinatal factors in neurodevelopmental damage.
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NEONATAL HYPOGLYCEMIA: RISK FACTORS AND OUTCOMES
(Macedonian Academy of Sciences and Arts / Sciendo, 2017-03)
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Elizabeta Petkovska
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Snezana Jancevska
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Background and aims: Severe neonatal hypoglycemia (HG) leads to neurologic damage, mental retardation, epilepsy, personality disorders, impaired cardiac performance and muscle weakness. We aimed to assess the clinical characteristics of children with hypoglycemia in a random population of newborns. Patients, methods and results: We investigated 84 patients (M:F=35:48) born at the University Clinic for Gynecology and Obstetrics in Skopje (hospitalized in the NICU) who were found to have hypoglycemia. In total 89.25% of the babies were premature. The mean birth weight was 1795.95 +/596.08 grams, the mean birth length was 41.92+/- 4.62 cm, while the mean gestational age was 33.05±3.19 weeks. 32 children (38.08%) were very low birth weight (<1500g), 38 (45.22%) were low birth weight (1500-2500g), while there were 8 children (9.52%) appropriate for age BW and no high BW for age patients (>4000 g). HG duration was 2.42+/-2.41 hours. In the group as a whole, hypoxic-ischemic encephalopathy (HIE) was found in 3 children (3.57%), infections in 22 (26.18%), respiratory distress syndrome (RDS) in 9 patients (10.62%), intracranial haemorrhage in 2 patients (2.38%). There were no inborn errors of metabolism. There were two deaths (2.38%). Conclusion: Neonatal HG is a significant factor in the overall neonatal mortality. HG can also cause severe invalidity. We found that infections, LBW and low gestational age were most commonly associated with neonatal HG. However the Spearman test showed weak direct correlation, without statistical significance. Neonatal HG requires complex and team interaction of prenatal and postnatal approaches to reduce the incidence of seizures, their consequences and the overall mortality. Special consideration is to be taken in measures that avoid neonatal infections, HIE, LBW and low gestational age. Further studies on a larger population are needed to fully understand and prevent the phenomenon of HG in newborns.
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SITUS INVERSUS TOTALIS IN A NEWBORN WITH CONGENITAL HEART DISEASE
(Македонско лекарско друштво = Macedonian medical association, 2024)
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Jancevska, Aleksandra
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Ristovska, Sanja
Introduction. Situs inversus totalis (SIT) is rarely re- ported in newborns. Isolated or associated with other congenital abnormalities, most often with congenital heart malformations (3-9%), SIT can often be an acci- dental finding. Case report. We report a term-newborn with SIT and complex congenital heart defect, diagnosed prenatally by fetal ultrasound. SIT was confirmed with plane film X-ray (liver on a left side, spleen on right side). Heart ultrasound was done and revealed a complex cardiac malformation (CHM) - dextrocardia, single ventricle, tricuspidal valvular atresia, hypoplastic aortae, ASD II, PDA. The baby was transferred to a heart surgery center where the neonate was treated but unfortunately pa- ssed away. Conclusion. SIT with CHM is a rare condition in neonatal period. Although a myriad of congenital malformations can be accompanying, isolated SIT is the most common. SIT and CHM is a condition challenging for surgical treatment.
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Transient Neonatal Myasthenia Gravis: A Case Report
(Macedonian Academy of Science and Arts/Sciendo, 2023-07-15)
Sanja Ristovska
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Renata Dimitrioska
Transient neonatal myasthenia gravis (TNMG) is a neuromuscular disorder that occurs in infants born from mothers with myasthenia gravis (MG) due to transplacental transfer of antibodies against the acetylcholine receptor. TNMG is a rare form occurring in 10-15% of infants born from mothers with MG. We present a case of a newborn with TNMG with generalized hypotonia and respiratory distress. The newborn shows symptoms of hypotonia, weakened reflexes, poor crying, difficult sucking and potentiated tachydyspnea after 24 hours of birth and needs of assisted mechanical ventilation. Based on the mother's positive history of MG and the high titer of mother's (8.43nmol/l) and newborn's (9.088nmol/l) anti-AChR antibodies, TNMG was diagnosed. The baby was treated with assisted mechanical ventilation and neostig-mine until the anti-AChR antibody titer was negative. Adequate management of the newborn resulted in a positive outcome and evident withdrawal of the symptoms. Although TNMG is one of the rare neuromuscular disorders in newborns that can be treated, a multidisciplinary approach in the management of pregnant women with MG and newborns through timely diagnosis and early appropriate treatment, results in successful resolution of this condition.
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LARGE NECK TERATOMA IN A NEWBORN WITH RESPIRATORY DISTRESS SYNDROME
(Macedonian Academy of Sciences and Arts / Sciendo, 2021-04)
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Goran Kocovski
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Neonatal tumours in the neck region are a rare finding. Teratomas typically comprise all three germ cell layers with tissues usually foreign to the anatomic site of origin. Head and neck teratomas account a smaller part of congenital teratomas. They can cause major airway obstruction due to the external compression that oropharyngeal or neck masses produce. In addition, there can be an intrinsic lesion in the larynx or trachea. We describe a premature, 30-gestational week-old newborn with large subcutaneous neck mass. Pre-delivery ultrasound showed heterogeneous tumor structure and displaced larynx. The intubation was successful. The newborn developed respiratory distress syndrome immediately after birth which rendered the surgical removal of the neck tumor impossible. An autopsy was done, and the histopathology revealed mature teratoma comprising muscle, brain, salivary and pulmonary tissues, as well as well-developed hyaline membranes in the alveoli. The combination of the respiratory distress syndrome and the neck tumor compression proved fatal. Prenatal diagnosis, therapeutic options and ex utero intrapartum treatment (EXIT) procedures are discussed for the diagnosis and management of this very rare tumor.
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Hypoglycaemia in the newborn
(Macedonian Academy of Sciences and Arts / Sciendo, 2017-09)
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Elizabeta Petkovska
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Sanja Ivanovska
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Snezana Jancevska
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Aim: Severe neonatal hypoglycaemia (HG) leads to neurologic damage, mental retardation, epilepsy, impaired cardiac performance and muscle weakness. The aim was to assess the frequency and severity of HG in a population of newborns. Patients and methods: We investigated 739 patients with neonatal hypoglycaemia (HG) (M:F=370:369) born at the University Clinic for Gynaecology and Obstetritics in Skopje in the period 2014-2016 and treated at the neonatal intensive care unit (NICU). 1416 babies were treated in the same period in NICU, and HG was observed in 52.18%. The birth weight was dominated by children with low birth weight: very low birth weight (VLBW)(<1500g) 253 children, (34,23%), low birth weight (1500-2500g) 402 (54.39%), appropriate for gestational age (AGA) 78(10.55%), and high birth weight (>4000g) 6 babies (0.81%). The gestational age was also dominated by children with low gestational age: gestational week (GW) 20-25 four children (0.54%), 26-30 GW 133 babies (17.99%), 31-35 GW472 (63.87%), and 36-40 GW130 neonates (17.59 %). 241 mothers (32.61%) have had an infection during pregnancy, 82 preeclampsia or eclampsia (11.09%), 20 diabetes mellitus (2.70%), 78 placental situations (placenta previa, abruption) (10.55%). In this study 47 babies (6.35%) with HG and co-morbidities died. There was a significant positive correlation between HG birth weight (p<0.01), gestational age (p<0.05), and the lowest Apgar score (p<0.01). Neonatal deaths were significantly correlated with GA (р>0,01), co-morbidities of the mothers (р>0,05) but not with the birth weight (р>0,05). In contrast, a significant positive correlation was found between convulsions and body weight (р<0.05). The lowest Apgar score was positively correlated with the gestational age (0.01), but not with the birth weight (0.05). Conclusion: Low birth weight, low gestational age, maternal risk factors, hypoxic-ischemic encephalopathy and neonatal infections are associated with HG and are a significant factor in overall neonatal mortality. Those results indicate that diminishing the frequency of the neonatal HG and the rates of neonatal mortality requires complex interaction of prenatal and postnatal interventions.
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THE CRIB II (CLINICAL RISK INDEX FOR BABIES II) SCORE IN PREDICTION OF NEONATAL MORTALITY
(Macedonian Academy of Sciences and Arts / Sciendo, 2020-12)
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Predicting the outcome of neonatal critical patients remains elusive. The multiple factors of maternal state of health (infections, diabetes, gestosis), the placental situation (premature rupture of membranes) as well as multiple factors from the baby (small for gestational age, low Apgar score, low birth infections, mechanical ventilation, hypoglycaemia hyperglycamiea) render the approach to treatment of each patient individual and the outcome uncertain. Several approaches and scales are developed in order to assess the mortality risk in those rather complicated situations. We used the CRIB-II scale to assess the mortality risk in 80 patients delivered in a large tertiary level hospital with more than 4,000 deliveries yearly. The patients were stratified according to all the neonatal risk factors and comorbidities. The CRIB-II scale identified well the mortality rates, but not the outcomes. A large and well-balanced cohort of patients followed for a longer period is required to discern in detail the importance of CRIB-II scale in predicting outcomes in high-risk new-borns. This could serve as an assistance to personalized approach to severely sick children. In addition, it is a valuable method in comparing outcomes in different NICUs and outcomes in different times in the same NICU, thus rendering possible improvements in the same unit and among several NICU departments.
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Potocki-Lupski Syndrome Dup17p11.2 in a Girl with Hypotonia and Early Behavioural Disturbances
(Macedonian Academy of Science and Arts/Sciendo, 2021-10-26)
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Neskovska, Lidija
Potocki-Lupski syndrome (PTLS) is a contiguous gene syndrome caused by duplication of chromosome 17p11.2. PTLS is characterized by hypotonia, failure to thrive, congenital anomalies (particularly of the cardiovascular system), intellectual disability, and behavioural disturbances. The patient was a full-term baby girl, 2,750 grams at birth, delivered via an uncomplicated vaginal delivery with pronounced hypotonia at birth. Nevertheless, there was failure to thrive (weight 7.6 kg; 2.8 SD). Micrognathia, epicanthal skin folds, and megalocornea were noticeable. There was a harsh continuous systolic murmur, and the ultrasound of the heart revealed a persistent arteriosus duct which was surgically closed. At the age of 18 months, the girl could not sit without support, and she could not utter simple words. The girl is often moody, angry, and aggressive. She is hyperactive and unable to establish contacts with family members. A 17p12-p11.2 microduplication was identified via MLPA. Muscle hypotonia, congenital heart malformation, failure to thrive, developmental delay, behavioural disturbances (or autism spectrum disorder), and intellectual disability are early signs of PTLS. The presence of PTLS was proven by an MLPA analysis.

Stomnaroska Damchevski, Orhideja

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