Late-onset fungal sepsis in very low birth weight newborns

Gljivama uzrokovana kasna novorođenačka sepsa u novorođenčadi rodne mase (RM) <1500 g je češća nego se prije znalo, odnosno, vjerojatno je učestalost narasla uz veće stope preživljavanja ove djece. Unatoč tomu, u nekim istraživanjima pokazano je smanjivanje učestalosti gljivične sepse zadnjih godina. U svakom slučaju, gljivičnu sepsu u ove djece treba očekivati i za njom aktivno tragati. Opisani su rizični čimbenici, osobitosti obrane od infekcije, osobitosti pojedinih vrsta gljiva, klinička slika, načini liječenja, osobitosti lijekova, te komplikacije i prevencija gljivične sepse ove novorođenčadi. Naveden je i plan dijagnostike diseminirane bolesti i kriteriji izlječenja. Predložena je i profilaksa gljivične infekcije/sepse u novorođenčadi RM <1500 g flukonazolom. Razmotrena je i situacija u Hrvatskoj u pogledu kasne novorođenačke gljivične sepse i moguće preventivne i profilaktičke mjere.Late-onset fungal sepsis in very low birth weight (VLBW) newborns (BW <1500 g) is more common than it was known before, the incidence possibly increased over time due to higher survival rate of these infants. In spite of that, some research have shown a decrease of such infections in the last years. However, late-onset fungal sepsis should be expected in these infants, and active investigations should be performed. The paper describes risk factors, defense mechanisms, particular fungal species, clinical presentation, modalities of treatment, characteristics of the drugs, complications and prevention of fungal sepsis in these infants. Also, the diagnosis of disseminated sepsis and criteria of successful treatment are presented. Prophylaxis of fungal colonization/infection by fluconazole in VLBW infants is proposed. Situation in Croatia concerning late-onset fungal sepsis in VLBW infants and possibilities of preventive and prophylactic measures are discussed

Appendicitis within inguinal hernia – case report of a premature newborn with Amyand’s hernia

Upala crvuljka unutar preponske kile ekstremno je rijetka pojava u nedonoščadi s procijenjenom incidencijom od oko 0,08-0,13%. Dijagnoza ovog izuzetnog entiteta, nazvanog Amyandova kila prema autoru koji ga je prvi opisao, najčešće je slučajna i obično se postavlja tek operativnim zahvatom. U prikazanog muškog nedonoščeta rođenog u 28. tjednu gestacije znaci ukliještenja desnostrane preponske kile javili su se tridesetog dana života. Kirurški zahvat je bio i dijagnostička i terapijska metoda. Nalazom neperforiranog, gangrenozno promijenjenog crvuljka unutar preponske kile postavljena je dijagnoza Amyandove kile. Nakon operativnog zahvata i uz dugotrajno antibiotsko liječenje uslijedio je potpuni oporavak. Prikaz bolesnika je podsjetnik na to da unatoč rijetkoj pojavnosti na Amyandovu kilu treba pomisliti u diferencijalnoj dijagnozi ukliještene desnostrane preponske kile u nedonoščadi.Acute appendicitis within an inguinal hernia is an extremely rare condition among premature newborns with estimated incidence ranging from 0.08% to 0.13%. The diagnosis of this extraordinary entity, known as Amyand’s hernia according to the author who fi rst described it, is often accidental and usually established intraoperatively. We report a case of a boy born at 28 weeks of gestation, who presented with incarcerated right inguinal hernia on his thirty day of life. Surgical intervention was both a diagnostic and therapeutic procedure. Intraoperative diagnosis of Amyand’s hernia was established based on the fi nding of non-perforated, gangrenous appendicitis within the inguinal hernial sac. Appendectomy and hernia repair followed by long-term antibiotic treatment led to complete infant’s recovery. This case report reminds that regardless of its rarity, clinicians should be aware of Amyand’s hernia in the evaluation of incarcerated right side inguinal hernia in preterm newborns

Survival prediction of high-risk outborn neonates with congenital diaphragmatic hernia from capillary blood gases

BACKGROUND: The extent of lung hypoplasia in neonates with congenital diaphragmatic hernia (CDH) can be assessed from gas exchange. We examined the role of preductal capillary blood gases in prognosticating outcome in patients with CDH. ----- METHODS: We retrospectively reviewed demographic data, disease characteristics, and preductal capillary blood gases on admission and within 24 h following admission for 44 high-risk outborn neonates. All neonates were intubated after delivery due to acute respiratory distress, and were emergently transferred via ground ambulance to our unit between 1/2000 and 12/2014. The main outcome measure was survival to hospital discharge and explanatory variables of interest were preductal capillary blood gases obtained on admission and during the first 24 h following admission. ----- RESULTS: Higher ratio of preductal partial pressure of oxygen to fraction of inspired oxygen (PcO2/FIO2) on admission predicted survival (AUC = 0.69, P = 0.04). However, some neonates substantially improve PcO2/FIO2 following initiation of treatment. Among neonates who survived at least 24 h, the highest preductal PcO2/FIO2 achieved in the initial 24 h was the strongest predictor of survival (AUC = 0.87, P = 0.002). Nonsurvivors had a mean admission preductal PcCO2 higher than survivors (91 ± 31 vs. 70 ± 25 mmHg, P = 0.02), and their PcCO2 remained high during the first 24 h of treatment. ----- CONCLUSION: The inability to achieve adequate gas exchange within 24 h of initiation of intensive care treatment is an ominous sign in high-risk outborn neonates with CDH. We suggest that improvement of oxygenation during the first 24 h, along with other relevant clinical signs, should be used when making decisions regarding treatment options in these critically ill neonates

EARLY AND LATE NEONATAL MORTALITY OF INFANTS BIRTH-WEIGHT 500–1499 GRAMS IN CROATIA IN THE YEAR 2003

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, zadnjih godina je manji od fetalnog¬ mortaliteta. RNM je za 2003.g. u Hrvatskoj bio 76% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 53% za novorođenčad PT 750–999 g, 19% za novorođenčad PT 1000–1249 g, i 4% za novorođenčad PT 1250–1499 g. Neonatalni mortalitet (NM) je za skupine novorođenčadi iste porodne težine bio 81%, potom 70%, zatim 25% i 5%. Kasni neonatalni mortalitet (KNM) u novorođenčadi PT 500–999 g iznosi najviše 12,3%, dok je u skupini novorođenčadi PT 1000–1499 g najviše 9,1%. To pokazuje da RNM nije podcijenjen na račun visokog KNM, i da pedijatrijska – neonatalna služba ne ostvaruje smanjenje RNM na račun kasnijeg povišenja KNM. U neposrednoj budućnosti treba u cijeloj državi ostvariti praćenje preživljavanja sve novorođenčadi do otpusta iz bolnice. Ti podatci predstavljat će osnovu za planiranje potreba neonatološke službe, izradu smjernica za prenatalno i postnatalno usmjeravanje novorođenčadi i za davanje vjerodostojnijih¬ prognoza roditeljima novorođenčadi najnižih porodnih težina.Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2003 in Croatia 76% for newborns of birth-weight (BW) 500–749 g; 53% for those 750–999 g, 19% for those 1000–1249 g, and 4 % for newborns of BW 1250–1499 g. Neonatal mortality for newborns in these birth-weight groups was 81%, 70%, 25% and 5% respectively. Late neonatal mortality (LNM) is maximal 12.3% for newborns of BW 500–999 g, and 9.1% for newborns of BW 1000–1499 g. ENM was not underestimated instead of possible higher LNM, pediatric-neonatal services didn’t reduce ENM on expenses of LNM. In close future is necessary to follow survival or mortality of all newborns up to discharge from hospital. These data will give us benchmark for planning of neonatal resources, development of recommendations in perinatology-neonatology for prenatal and postnatal transfer, and for more exact prognoses to parents of the smallest newborns

Survival of outborns with congenital diaphragmatic hernia: the role of protective ventilation, early presentation and transport distance: a retrospective cohort study

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a congenital malformation associated with life-threatening pulmonary dysfunction and high neonatal mortality. Outcomes are improved with protective ventilation, less severe pulmonary pathology, and the proximity of the treating center to the site of delivery. The major CDH treatment center in Croatia lacks a maternity ward, thus all CDH patients are transferred from local Zagreb hospitals or remote areas (outborns). In 2000 this center adopted protective ventilation for CDH management. In the present study we assess the roles of protective ventilation, transport distance, and severity of pulmonary pathology on survival of neonates with CDH. ----- METHODS: The study was divided into Epoch I, (1990-1999, traditional ventilation to achieve normocapnia), and Epoch II, (2000-2014, protective ventilation with permissive hypercapnia). Patients were categorized by transfer distance (local hospital or remote locations) and by acuity of respiratory distress after delivery (early presentation-occurring at birth, or late presentation, ≥ 6 h after delivery). Survival between epochs, types of transfers, and acuity of presentation were assessed. An additional analysis was assessed for the potential association between survival and end-capillary blood CO2 (PcCO2), an indirect measure of pulmonary pathology. ----- RESULTS: There were 83 neonates, 26 in Epoch I, and 57 in Epoch II. In Epoch I 11 patients (42%) survived, and in Epoch II 38 (67%) (P = 0.039). Survival with early presentation (N = 63) was 48 % and with late presentation 95% (P <0.001). Among early presentation, survival was higher in Epoch II vs. Epoch I (57% vs. 26%, P = 0.031). From multiple logistic regression analysis restricted to neonates with early presentation and adjusting for severity of disease, survival was improved in Epoch II (OR 4.8, 95%CI 1.3-18.0, P = 0.019). Survival was unrelated to distance of transfer but improved with lower partial pressure of PcCO2 on admission (OR 1.16, 95%CI 1.01-1.33 per 5 mmHg decrease, P = 0.031). ----- CONCLUSIONS: The introduction of protective ventilation was associated with improved survival in neonates with early presentation. Survival did not differ between local and remote transfers, but primarily depended on severity of pulmonary pathology as inferred from admission capillary PcCO2

Postoperative gastric perforation in a newborn with duodenal atresia

Gastric perforation (GP) in neonates is a rare entity with high mortality. Although the etiology is not completely understood, it mostly occurs in premature neonates on assisted ventilation. Combination of duodenal atresia and gastric perforation is very rare. We present a case duodenal atresia who developed gastric perforation after operetion for duodenal atresia. Analysis of the patient medical record and histology report did not reveal the etiology of the perforation

SIGNIFICANCE OF NEONATAL SCREENING FOR CYSTIC FIBROSIS

Cistična fibroza (CF) je autosomno recesivno nasljedna bolest i smatra se najčešćom smrtonosnom bolešću u bijele rase. Dijagnostički pristup bolesti temelji se na dijagnostičkom konsenzusu po kojem se sumnja na CF može ¬postaviti među ostalim i na osnovi pozitivnog nalaza novorođenačkog probira. Dok je u svijetu zadnjih godina sve više djece koja se otkrivaju u programu novorođenačkog probira, u Hrvatskoj se on ne provodi. Bolesnici u kojih se dijagnoza postavi nakon novorođenačkog probira imaju neke prednosti, rizike ali i nove probleme, te izazove za medicinsku struku s kojima se nismo susretali u dosadašnjoj praksi. Prednost probira najviše se očituje u boljoj uhranjenosti i boljem potencijalu kognitivnih funkcija te u izbjegavanju komplikacija koje su posljedica malnutricije. Prednost glede očuvanja plućne funkcije sadrži više kontroverza. Rano prepoznavanje bolesti često dovodi do ranog izlaganja bolesnika riziku ¬infekcija Pseudomonasom zbog odlaska u ustanove u kojima susreće druge bolesnike s CF. Poznavanje prednosti i rizika novorođenačkog probira na CF omogućuje procjenu njegove važnosti ne samo za bolesnika ili njegovu obitelji već i za čitavu zajednicu.Cystic fibrosis is among Caucasians the most common lethal autosomal recessive inherited disease. Diagnosis is based on meeting the criteria published as an expert consensus. Neonatal screening is one of the mentioned criteria. The number of children diagnosed with cystic fibrosis through neonatal screening is increasing throughout the world, but is not performed in Croatia. Early identification of these patients carries some advantages and some risks, posing new challenges for health workers. The most pronounced advantage is better growth and cognitive potentials for screened children, as well as less complications arising from malnutrition. Benefits regarding preservation of lung function are more controversial. Detection of patients through screening programs often means early exposure to Pseudomonas infections due to contacts with other CF patients in specialized centers. Recognizing potential advantages and risks of neonatal screening programs for CF brings new knowledge not only for individual patients and their families, but for the entire community

MORTALITY OF NEWBORNS IN CROATIA IN 2005

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, i zadnjih godina je manji od fetalnog mortaliteta. RNM je u 2005. g. u Hrvatskoj bio 71% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 32% za novorođenčad PT 750–999 g, 16% za novorođenčad PT 1000–1249 g, i 9% za novorođenčad PT 1250–1499 g. Mortalitet do otpusta iz bolnice (MOB) je za skupine novorođenčadi iste porodne težine bio 84%, potom 48%, zatim 20% i 10%. U skupinama novorođenčadi veće porodne težine RNM i MOB bili su još niži. RNM sve novorođenčadi >500 g bio je 3,4‰, neonatalni mortalitet (NM) je bio 4,1‰ i MOB je bio 4,4‰. RNM novorođenčadi PT >1000 g bio je 2,2‰, NM je bio 2,6‰ i MOB je bio 2,9‰. RNM je za svu novorođenčad PT >500 g činio prosječno 76,7% smrtnosti (145/189), dok je ostalih 44 djece (23,3%) umrlo nakon prvog tjedna života. To ukazuje da RNM nije podcijenjen na račun visokog kasnijeg moraliteta, i da pedijatrijska-neonatalna služba ne ostvaruje smanjenje RNM na račun kasnijeg povišenja ¬mortaliteta ili MOB. U 2005. godini je kao i u 2003. i 2004. tri četvrtine novorođenčadi PT 500–1499 g rođeno u rodilištima III. razine. Za vjerodostojnu analizu podataka ishoda novorođenčadi i djece planirana je izrada novih obrazaca perinatalnih zbivanja, uz prikupljanje podataka o postnatalnom transportu novorođenčadi i mjestu liječenja novorođenčeta. Potrebno je nastaviti prikupljati detaljne podatke o vitalnim događajima do otpusta iz bolnice. Ti podatci predstavljat će osnovu za planiranje potreba neonatološke službe, izradu smjernica za prenatalno i postnatalno usmjeravanje novorođenčadi i za davanje vjerodostojnijih prognoza roditeljima novorođenčadi najnižih porodnih težina.Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2004 in Croatia 71% for newborns of birth-weight (BW) 500–749 g; 32% for those 750–999 g, 16% for those 1000–1249 g, and 9% for newborns of BW 1250–1499 g. Mortality to discharge from hospital (MDH) for newborns in these birth-weight groups was 84%, 48%, 20% and 10%, respectively. In groups of newborns with larger BW over 1500 g ENM and MDH were even lower. ENM for all newborns BW >500 g was 3,4‰, neonatal mortality (NM) was 4,1‰, and MDH was 4,4‰, respectively. ENM for newborns of BW > 1000 g was 2,2‰, NM was 2,6‰, and MDH was 2,9‰, respectively. ENM made 76,7% mortality of all newborns (BW >500 g) (145/189), while the rest of 44 newborns (23,3%) died after the first week of life. Therefore, ENM was not underestimated due to possible higher late neonatal mortality, pediatric-neonatal services didn\u27t reduce ENM on expenses of higher late neonatal mortality or MDH. In the year 2005, as in 2003 and 2004, three fourths of newborns of BW 500–1499 grams were born in maternities of 3rd level. Within the aims of the proper analysis of newborns’ outcome data, is creation of new certificates of vital events with details of postnatal transport and place of treatment of newborn. It is necessary to continue to follow survival or mortality of all newborns to discharge from the hospital. These data will give us benchmark for planning of neonatal resources, development of recommendations in perinatology-neonatology for prenatal and postnatal transfer, and for more exact prognoses of the smallest newborns in the process of decision making

Successful separation of xypho-omphalopagus conjoined twins with extrauterine twin-twin transfusion syndrome: a case report

Conjoined twining is a rare medical phenomenon, with an overall prevalence of 1.47 per 100 000 births. This report describes a successful separation of xypho-omphalopagus conjoined twins complicated by unbalanced blood shunting through the porto-systemic anastomoses within the shared liver parenchyma. Significant extrauterine twin-twin transfusion syndrome caused by unbalanced shunting is an extremely rare, and probably under-recognized, hemodynamic complication in conjoined twins necessitating urgent separation. Progressive deterioration with a poor outcome can be prevented if the condition is recognized in a timely manner

MORTALITY OF NEWBORNS IN REPUBLIC OF CROATIA IN THE YEAR 2008

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, zadnjih godina je manji od fetalnog mortaliteta. RNM je u 2007. godini u Hrvatskoj bio 69% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 30% za novorođenčad PT 750–999 g, dok je 2008. godine RNM za djecu PT ≤999 g bio 42%. Po 100-gramskim razredima RNM je bio za djecu PT <500 g 100%, za djecu PT 500–599 g 72,2%, za djecu PT 600–699 g 77,8%, za djecu PT 700–799 g 46,4%, za djecu PT 800–899 g 20% i za djecu PT 900–999 g RNM je bio 16,2%. Za novoro|enčad PT 1000–1249 g RNM je bio 9,6% i za novoro|enčad PT 1250–1499 g 2,4%. Mortalitet do otpusta iz bolnice (MOB) je za skupine novorođenčadi iste porodne težine bio: za djecu <500 g 100%, za djecu PT 500–599 g 94,4%, za djecu PT 600–699 g 77,8%, za djecu PT 700–799 g 57,1%, za djecu PT 800–899 g 42,9%, za djecu 900–999 g 21,6%, za djecu PT 1000–1249 g 12% i za djecu PT 1250–1499 g 2,4%. Sve su te vrijednosti niže od odgovarajućih za prethodno razdoblje. U skupinama novorođenčadi veće porodne težine RNM i MOB bili su još niži. RNM sve novorođenčadi bio je 2,7‰, neonatalni mortalitet (NM) je bio 3,1‰ i MOB je bio 3,5‰, što je tako|er manje nego prethodnih godina. RNM novorođenčadi PT ≥1000 g bio je 1,3‰, NM 1,5‰ i MOB 1,7‰, manje nego za 2007. godinu. RNM je za svu novorođ enčad činio prosječno 77,1% smrtnosti (118/153), dok je ostalih 35 djece (22,9%) umrlo nakon prvog tjedna života. To poka zuje da RNM nije podcijenjen na račun visokog kasnijeg mortaliteta, i da pedijatrijska-neonatalna služba ne ostvaruje smanjenje RNM na račun kasnijeg povišenja neonatalnog mortaliteta ili mortaliteta do otpusta iz bolnice. U razdoblju 2003.–2006. godine je tri četvrtine novorođenčadi PT 500–1499 g ro|eno u rodilištima III. razine. U 2007. godini su četiri petine ove djece rođene u rodilištima III. razine. U 2008. godini je 18,5% djece PT ≤1499 g rođeno u rodilištima izvan III. razine. Trend rađanja ove djece usmjeren je prema rodilištima III. razine, ali još ima mjesta povećanju broja poroda ove djece u rodilištima III. razine. Usporedbe RNM i NM s europskim zemljama ukazuju na postojanje daljnjih mogućnosti poboljšanja perinatalnih pokazatelja. Za vjerodostojnu analizu podataka ishoda novorođenčadi i djece planirana je izrada novih obrazaca perinatalnih zbivanja, uz prikupljanje podataka o postnatalnom transportu novorođenčadi i mjestu liječenja novorođenčeta. Potrebno je nastaviti prikupljati detaljne podatke o vitalnim događajima do otpusta iz bolnice. Ti podatci predstavljat će osnovu za planiranje potreba neonatološke službe, izradu smjernica za prenatalno i postnatalno usmjeravanje novorođenčadi i za davanje vjerodostojnijih prognoza roditeljima novorođenčadi najnižih porodnih težina.Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2008 in Croatia 69% for newborns of birth-weight (BW) 500–749 g; 30% for those 750–999 g, 16% for those 1000–1249 g, and 6% for newborns of BW 1250–1499 g. In newborns divided by 100-grams, ENM for infants BW <500 g was 100%, for those of BW 500–599 g was 72,2%, for infants BW PT 600–699 g 77,8%, for infants of BW 700–799 g 46,4%, for infants of BW 800–899 g 20% and for infants of BW 900–999 g ENM was 16,2%, respectively. For infants of BW 1000–1249 g ENM was 9,6% and for infants of BW 1250–1499 g was 2,4%, respectively. Mortality to discharge from hospital (MDH) for subgroups of infants of the same BW was 100% for infants of BW <500 g, for infants of BW 500–599 g 94,4%, for infants of BW 600–699 g 77,8%, for infants of BW 700–799 g 57,1%, for infants of BW 800–899 g 42,9%, for infants of BW 900–999 g 21,6%, for infants of BW 1000–1249 g 12%, and for infants of BW 1250–1499 g was 2,4%, respectively. All these values were lower than corresponding in previous period. In groups of infants of larger BW, ENM and MDH were lower. ENM of all newborns was 2,7‰, neonatal mortal-1,3‰, NM was 1,5‰, and MDH was 1,7‰, respectively, lower than in the year 2007. ENM was 77,1% (118/153) of all infants deaths, while the remained 35 infants (22,9%) died after the first week. Therefore, ENM was not underestimated instead of possible higher late neonatal mortality, pediatric-neonatal services didn’t reduce ENM on expenses of higher late neonatal mortality or MDH. In the years 2003–2006, three fourths of newborns of BW 500–1499 g were born in maternities of IIIrd level. In the year 2007 four fifths of these newborns were born in maternities of IIIrd level. In the year 2008 18,5% of infants of BW ≤1499 g were born in maternities outside of IIIrd level. Through the years the proportion of these infants born in maternities of IIIrd level is increasing, but that proportion can be even larger. Comparisons of ENM and NM with some European countries show the possibility of further improvement in perinatal markers. In the aim of the proper analysis of newborns’ outcome data, creation of new certificates of vital events is planned with details of postnatal transport and place of the treatment of newborn. It is necessary to continue to follow survival or mortality of all newborns up to discharge from hospital. These data will give us benchmark for planning of neonatal resources, development of recommendations in perinatology-neonatology for prenatal and postnatal transfer, and for more exact prognoses to parents of the smallest newborns