The Extremely Low Birth Weight Infant

Extremely low birth weight infants (ELBW) are defined by birth weight of less than 1000 g and are frequently born at 27 weeks’ gestation (GW) or younger. The neonatologists’ efforts focused on improvement of intact survival rate, especially for those born at the frontiers of viability at 22/23 GW. Survival rates of >80% for the advanced gestations and > 50% for 23–24 GW have been reported. Higher gestational age and birth weight, female gender, better maternal education, and white race have been recognized as significant predictors of decreased morbidity in ELBW infants. Although the mortality rate has significantly contracted for this group with improved technology and better understanding of pathophysiology, the proportion of surviving infants without sequelae, has not improved as noticeably. We review the short and long-term morbidities in ELBW infants and compare own and literature data. We analyze some of the specific immediate problems for this group such as: respiratory problems, infection, thermoregulation, impaired glucose homeostasis and disturbed cardiovascular and excretory functions as well as late morbidities such as bronchopulmonary dysplasia, late-onset infections, central nervous system occurrences, retinopathy and anemia of prematurity. We also deal with preventive and therapeutic strategies for improved outcome in this sensitive group of patients

Neonatal Hyperbilirubinemia in Newborns of the Republic of North Macedonia

Neonatal indirect hyperbilirubinemia is one of the most frequent neonatal problems that affect almost two thirds of term infants. Although etiology of jaundice has been widely studied, identification of pathological causes presents constant clinical challenge. Our study group performed an extensive retrospective study of etiology of neonatal hyperbilirubinemia and showed high frequency (44.37%) of jaundice of undefined etiology. The group included exaggerated physiological jaundice, early- and late-onset breast-milk jaundice, and no identifiable etiology. Other etiologies were neonatal infection, prematurity, birth trauma, and hemolysis represented with 15%. We described hematological parameters in both non-hemolytic and hemolytic type of jaundice; a significant correlation of relevant laboratory findings with etiology was established. In this chapter we will present our own data and perform a data-relevant literature review. Furthermore, investigation and management plan of neonatal indirect hyperbilirubinemia will be presented in accordance with own data and available literature

Kлинички тек и иÑход кај пациенти Ñо рано дијагноÑтицирани конгенитални малформации на бубрезите и уринарниот тракт

Congenital anomalies of the kidney and urinary tract- CAKUT are common childhood pathology accounting for 20-30% of all perinatally detected congenital anomalies. The importance of CAKUT is the risk they represent to kidney function deterioration and end-stage renal disease development.The aim of this study was to analyze the clinical course and outcome in patients with early diagnosed CAKUT. The study was designed as retrospective observational study.  A total of 100 patients with early diagnosed CAKUT at University Children's Hospital, Skopje, were enrolled in the study. The results obtained in this study present the clinical course and outcome in the study group. Urinary tract  infections were seen in 30% of patients enrolled in this study; they were predominantly girls (65%), mostly  diagnosed with posterior urethral  valve, VUR (vesicouretral reflux) and UPJO (ureteropelviuc junction obstruction). Surgical treatment  was required in 28% of patients, and chronic renal failure was diagnosed in 4% of the total number of patients in this study.This study aims to contribute to understanding the early diagnosed CAKUT as well as to establishing a protocol for early detection and adequate treatment in order to prevent renal function deterioration. ÐšÐ¾Ð½Ð³ÐµÐ½Ð¸Ñ‚алните малформации на бубрезите и уринарниот тракт (congenital anomalies of the kidney and urinary tract- CAKUT) Ñе чеÑти во детÑката возраÑÑ‚ и Ñочинуваат помеѓу 20 и 30% од Ñите конге- нитални аномалии во перинаталниот период. Значењето на конгениталните аномалии на бубрезите и уринарниот тракт е ризикот што тие го ноÑат за влошување на бубрежната функција Ñо развој на прогреÑивна бубрежна болеÑÑ‚ и бубрежна инÑуфициенција. Целта на оваа Ñтудија беше да Ñе опи- ше клиничкиот тек и иÑходот кај пациентите Ñо рано дијагноÑтициран CAKUT. Студијата  Ð±ÐµÑˆÐµ ди- зајнирана како ретроÑпективна опÑеревациона Ñтудија. Таа опфати 100 иÑпитаници Ñо пренатално поÑтавено Ñомнение за конгенитална малформација на бубрезите и уринарниот тракт. Пациентите беа прегледани на УниверзитетÑката клиника за детÑки болеÑти, Скопје. Резултатите ги прикажуваат клиничкиот тек и иÑходот кај пациентите Ñо рана дијагноза на конгенитални аномалии на бубрезите и уринарниот тракт. Уринарни инфекции во тек на Ñледењето беа региÑтрирани кај 30% од пациентите, почеÑто кај девојчиња (65%), во поголем процент заÑтапени кај пациенти Ñо валвула на задна уретра, VUR (vesicouretral reflux) и UPJO (ureteropelviuc junction obstruction). Оперативен третман беше не- опходен кај 28% од пациентите, а хронична бубрежна болеÑÑ‚ беше дијагноÑтицирана кај 4%. Оваа Ñтудија има за цел да придонеÑе во разбирањето на карактериÑтиките на рано детектираните конге- нитални малформации и поÑтавување на Ñтратегија за рана поÑтнатална потврда на пренатално по- Ñтавеното Ñомнение за малформација и ран, оптимизиран третман, Ñо цел редукција или одложување на прогреÑија на детериорација на бубрежната функција и терминална бубрежна болеÑÑ‚

Referentne vrijednosti za kvantitativno ispitivanje G6PD deficita kod novorođenčadi iz Republike Sjeverne Makedonije

Glucose-6-phosphate dehydrogenase is a key regulatory enzyme in the pentose-phosphate cycle that participates in the formation of reduced equivalents to maintain the cellular redox status. The G6PD enzyme activity is crucial in protecting cells from oxidative stress. Deficit of the glucose-6-phosphate dehydrogenase (G6PD) has been recognized as the most common inherited enzymopathy worldwide. In the Republic of North Macedonia (RNM), the deficit of glucose-6-phosphate dehydrogenase has been infrequently investigated. Moreover, no reports exist on quantitative testing of G6PD in newborns from the RNM. SCOPE: The aim of our study was to determine the reference values for the level of G6PD in erythrocytes of newborns from the Republic of North Macedonia. For this purpose, eighty-two healthy newborns were selected and tested for G6PD by the quantitative spectrophotometric method. RESULTS: The mean ± SD of the G6PD quantitative value in the examined group was 229.12 ± 24.2 mU/109Er ranging from a minimum of 191.7 to a maximum of 288, and a median of 228.0 mU/109Er, values that were lower than the preset reference values of the diagnostic test in use. CONCLUSION: We speculated that by establishing a specific reference value (range) for the target population and for the appropriate diagnostic test, we would gain increased sensitivity of the test. This would help optimize detection of G6PD deficient newborns, mild-variant hemizygotes and female heterozygotes for the deficiency.Glukoza-6-fosfat dehidrogenaza je ključni regulatorni enzim u pentoza-fosfatnom ciklusu koji sudjeluje u stvaranju reduciranih ekvivalenata za održavanje staničnog redoks statusa. Aktivnost enzima G6PD ključna je u zaštiti stanica od oksidativnog stresa. Deficit glukoza-6-fosfat dehidrogenaze (G6PD) prepoznat je kao najčešća nasljedna enzimopatija u svijetu. U Republici Sjevernoj Makedoniji (RSM) deficit glukoza-6-fosfat dehidrogenaze je rijetko istraživan. Štoviše, iz RSM-a ne postoje izvješća o kvantitativnom ispitivanju G6PD u novorođenčadi. CILJ: Cilj našeg istraživanja bio je odrediti referentne vrijednosti za razinu G6PD u eritrocitima novorođenčadi iz Republike Sjeverne Makedonije. U tu svrhu odabrano je osamdeset i dvoje zdravih novorođenčadi i testirano na G6PD kvantitativnom spektrofotometrijskom metodom. REZULTATI: Srednja vrijednost ± SD kvantitativne vrijednosti G6PD u ispitivanoj skupini bila je 229,12 ± 24,2 mU/109Er u rasponu od minimalno 191,7 do maksimalno 288 te medijan od 228,0 mU/109Er, vrijednosti koje su bile znatno niže od unaprijed postavljenih referentnih vrijednosti dijagnostičkog testa u uporabi. ZAKLJUČAK: Pretpostavili smo da ćemo uspostavljanjem specifične referentne vrijednosti (raspona) za ciljnu populaciju i za odgovarajući dijagnostički test dobiti povećanu osjetljivost testa. To bi pomoglo optimizirati otkrivanje novorođenčadi s nedostatkom G6PD, hemizigota za blage varijante deficita i ženskih heterozigota na deficit G6PDa