Improving feeding skills and transition to breastfeeding in early preterm infants: a randomized controlled trial of oromotor intervention.

Introduction: Oromotor therapy exercises used for preterm infants in the NICU might promote oral-motor skills and shorten discharge day. This study investigates the impact of an oral-motor therapy program on the successful transition to breastfeeding (BF) and the enhancement of feeding skills in preterm infants below 30 weeks of gestational age who experience feeding intolerance. Methods: The intervention group received oral-motor therapy programme for one month, while the control group did not. The feeding skills were evaluated by Early Feeding Skills Assessment Tool (EFS) and Preterm Oral Feeding Readiness Scales (POFRAS). Results: There was a significant difference in EFS and POFRAS scores, transition to bottle feeding at discharge and transition to BF after discharge between babies given oral-motor therapy programme and controls (p < 0.05). While the transition time to full enteral feeds did not vary significantly between the groups, noteworthy outcomes were observed in the intervention group, including differences in feeding type at discharge, the nature of feeds at discharge, and the success of transitioning to breastfeeding after discharge. Discussion: We conclude that the oromotor therapy exercises in NICU improves the quality of sucking, contributes to better oromotor skills and promotes transition to enteral feeding and BF in preterm babies. Clinical Trial Registration: ClinicalTrials.gov, identifier (NCT05845684).

Neonatal Resuscitation Practices in Turkey: A Survey of the Turkish Neonatal Society and the Union of European Neonatal and Perinatal Societies.

OBJECTIVE: Optimal care in the delivery room is important to decrease neonatal morbidity and mortality. We aimed to evaluate neonatal resuscitation practices in Turkish centers. MATERIALS AND METHODS: A cross-sectional survey consisted of a 91-item questionnaire focused on delivery room practices in neonatal resuscitation and was sent to 50 Turkish centers. Hospitals with <2500 and those with ≥2500 births/year were compared. RESULTS: In 2018, approximately 240 000 births occurred at participating hospitals with a median of 2630 births/year. Participating hospitals were able to provide nasal continuous-positiveairway-pressure/high-flow nasal cannula, mechanical ventilation, high-frequency oscillatory ventilation, inhaled nitric oxide, and therapeutic hypothermia similarly. Antenatal counseling was routinely performed on parents at 56% of all centers. A resuscitation team was present at 72% of deliveries. Umbilical cord management for both term and preterm infants was similar between centers. The rate of delayed cord clamping was approximately 60% in term and late preterm infants. Thermal management for preterm infants (<32 weeks) was similar. Hospitals had appropriate equipment with similar rates of interventions and management, except conti nuous-positive-airway-pressure and positive-end-expiratory-pressure levels (cmH2O) used in preterm infants (P = .021, and P = .032). Ethical and educational aspects were also similar. CONCLUSIONS: This survey provided information on neonatal resuscitation practices in a sample of hospitals from all regions of Turkey and allowed us to see weaknesses in some fields. Although adherence to the guidelines was high among centers, further implementations are required in the areas of antenatal counseling, cord management, and circulation assessment in the delivery room.

Demographic and clinic characteristics and risk factors of molluscum contagiosum in children.

OBJECTIVE: To address the gap in evidence related to molluscum contagiosum in children by focusing on demographic and clinical features as well as risk factors. Methods: The multicentre, prospective, clinical study was conducted at four hospitals in Ankara and Tokat cities of Turkey from August 1, 2014, to August 5, 2019, and comprised patients aged ≤18 years diagnosed with molluscum contagiosum. Data about demographics, day nursery and preschool attendance, the seasons when the disease occurred, any use of Turkish baths and swimming pools, history of personal/familial atopy, coexistence of diseases, disease duration, courses, number of lesions and anatomic localisation. Data was analysed using SPSS 19. RESULTS: Of the 286 patients, 130(45.5%) were girls and 156(54.5%) were boys. The overall mean age was 5.94±3.95 years. The median duration of the disease was 5 weeks (interquartile range: 3.00-12.00 weeks). There was a significant number of cases with family history 18(48.6%) in the 0-3 age group (p=0.027). History of personal atopy was significantly high in the winter season (p<0.05). Patients with >20 lesions had used swimming pools significantly more frequently than the rest (p=0.042). The trunk was the most commonly involved region 162(56.6%). CONCLUSIONS: Providing prospective data about demographics, clinical characteristics and risk factors of molluscum contagiosum in children will lead to appropriate preventive and therapeutic measures.

Ursodeoxycholic acid protects neonatal rats from necrotizing enterocolitis: a biochemical, histopathological, and immunohistochemical study.

BACKGROUND: The pathophysiology of necrotizing enterocolitis (NEC) includes the massive production of endogenous cytokines with exaggerated activation of inflammatory pathways. Ursodeoxycholic acid (UDCA) has been used as an anti-inflammatory, antioxidant, and anti-apoptotic agent. We investigated the possible protective effects of UDCA in a neonatal rat pup model of NEC. METHODS: We randomly divided rat pups into three groups: a control group, a non-treated NEC group, and a UDCA-treated NEC group. We induced NEC by feeding formula enterally and via hypoxia/reoxygenation. Intestinal samples were collected for histopathological and immunohistochemical evaluation. Blood samples were taken for biochemical analyses. RESULTS: UDCA significantly reduced the extents of terminal ileal and jejunal injuries compared to the NEC group (p < .01), reduced Bax and caspase-3 immunoreactivities (both p < .01), and lowered serum levels of platelet-activating factor and intestinal fatty acid-binding protein (p < .01, p = .023, respectively). CONCLUSIONS: In a rat model of NEC, UDCA protects against adverse intestinal histological, immunohistochemical, and biochemical changes. UDCA significantly reduces the effects of NEC on the rat pup intestine.

Analysis of late-onset neonatal sepsis cases in a level three neonatal intensive care unit.

OBJECTIVE: Newborns in neonatal intensive care units (NICUs) are at high risk for developing nosocomial infections (NIs), which may result in morbidity and mortality. In this study, we aimed to ascertain the bacteriological profiles and their antimicrobial susceptibility patterns in NIs. METHODS: We conducted a cross-sectional study in the NICU. Late-onset sepsis (LOS) cases confirmed with blood cultures were evaluated retrospectively. Laboratory parameters, demographics, and clinical data were collected and analyzed from hospital records retrospectively. RESULTS: Of the 1210 infants in the NICU, 76 (6.3%) had LOS. A total of 86 episodes of LOS were documented; in 10 infants, two pathogens were identified. The mean gestational age (GA) of the infants with LOS was 33.2±4.8 weeks (23 to 42 weeks). Gram-positive cocci (GPC) caused most of the LOS episodes (65.8%, 50/76), with coagulase-negative Staphylococcus (CoNS) as the most common cause of LOS (50%, 38/76). Gram-negative rod species (GNRs) accounted for 32.9% (25/76) of the LOS cases, and fungi accounted for 1.3% (1/76). The mortality rates for GNR and GPC were 17.9% and 6.4%, respectively (p>0.05). The mean CRP and conjugated bilirubin levels for the GPC and GNR groups were 37.5 vs. 29.5 mg/dl and 0.7 vs 1.5 mg/dl, respectively (p>0.05). GNRs had a 20-25% ceftriaxone resistance. Two (4%) GPC species were resistant to linezolid, while all were susceptible to vancomycin. All of the GNRs were susceptible to carbapenems. CONCLUSION: These results underscore the recent emergence of CoNS in NICUs. LOS due to GNRs seems to display higher C-reactive protein and conjugated bilirubin values than those due to GPC. Clinical monitoring of NIs and bacterial resistance profiles are required in all NICUs.

Neonatal Thrombocytopenia and the Role of the Platelet Mass Index in Platelet Transfusion in the Neonatal Intensive Care Unit

Background: Neonatal thrombocytopenia is a common hematological abnormality that occurs in 20­35% of all newborns in the neonatal intensive care unit. Platelet transfusion is the only known treatment; however, it is the critical point to identify neonates who are really at risk of bleeding and benefit from platelet transfusion as it also has various potential harmful effects. Aims: To investigate the prevalence and risk factors of neonatal thrombocytopenia and its relationship to intraventricular hemorrhage in the neonatal intensive care unit and to determine whether the use of platelet mass index-based criteria could reduce the rate of platelet transfusion. Study Design: Retrospective cohort study. Methods: This study was conducted in the neonatal intensive care unit of a tertiary university hospital. The medical records of neonates in the neonatal intensive care unit with platelet counts <150×109/L between January 2013 and July 2016 were analyzed. Results: During the study period, 2,667 patients were admitted to the neonatal intensive care unit, and 395 (14%) had thrombocytopenia during hospitalization. The rate of intraventricular hemorrhage was 7.3%. Multiple logistic regression analysis showed that although lower platelet counts were associated with a higher intraventricular hemorrhage rate, the effects of respiratory distress syndrome, sepsis, and patent ductus arteriosus were more prominent than the degree of thrombocytopenia. Thirty patients (7%) received platelet transfusion, and these patients showed a significantly higher mortality rate than their non-platelet transfusion counterparts (p<0.001). In addition, it was found that the use of platelet mass index-based criteria for platelet transfusion in our patients would reduce the rate of platelet transfusion by 9.5% (2/21). Conclusion: Neonatal thrombocytopenia is usually mild and often resolves without treatment. As platelet transfusion is associated with an increased mortality rate, its risks and benefits should be weighed carefully. The use of platelet mass index-based criteria may reduce platelet transfusion rates in the neonatal intensive care unit, but additional data from prospective studies are required.

An Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence.

Prune belly syndrome (PBS) is characterized by a classical triad of congenitally absent abdominal muscles, bilateral cryptorchidism, and a malformed urinary tract. Urorectal septum malformation sequence (URSM) is identified with the absence of urogenital and anal openings. This case report describes a 15-week-old female fetus with megacystis, ascites and oligohydramnios in a 19-year-old nulliparous woman. The patient underwent preterm labor at the 33rd gestational week and delivered a female newborn weighing 2250 grams who died three days later due to progressive respiratory insufficiency. To the best of our knowledge, this is the third case of an overlap between PBS and URSM in literature. Such an overlap refers to the existence of left renal agenesis, right renal cystic dysplasia, bilateral club foot and lumbar scoliosis as well as the absence of abdominal wall muscles, internal genital organs, urethral, vaginal and anal openings. This case report aims to remind the obstetricians about the concurrent occurrence of PBS with URSM and its poor prognosis.

Early neonatal outcomes of very-low-birth-weight infants in Turkey: A prospective multicenter study of the Turkish Neonatal Society.

OBJECTIVE: To investigate the early neonatal outcomes of very-low-birth-weight (VLBW) infants discharged home from neonatal intensive care units (NICUs) in Turkey. MATERIAL AND METHODS: A prospective cohort study was performed between April 1, 2016 and April 30, 2017. The study included VLBW infants admitted to level III NICUs. Perinatal and neonatal data of all infants born with a birth weight of ≤1500 g were collected for infants who survived. RESULTS: Data from 69 NICUs were obtained. The mean birth weight and gestational age were 1137±245 g and 29±2.4 weeks, respectively. During the study period, 78% of VLBW infants survived to discharge and 48% of survived infants had no major neonatal morbidity. VLBW infants who survived were evaluated in terms of major morbidities: bronchopulmonary dysplasia was detected in 23.7% of infants, necrotizing enterocolitis in 9.1%, blood culture proven late-onset sepsis (LOS) in 21.1%, blood culture negative LOS in 21.3%, severe intraventricular hemorrhage in 5.4% and severe retinopathy of prematurity in 11.1%. Hemodynamically significant patent ductus arteriosus was diagnosed in 24.8% of infants. Antenatal steroids were administered to 42.9% of mothers. CONCLUSION: The present investigation is the first multicenter study to include epidemiological information on VLBW infants in Turkey. Morbidity rate in VLBW infants is a serious concern and higher than those in developed countries. Implementation of oxygen therapy with appropriate monitoring, better antenatal and neonatal care and control of sepsis may reduce the prevalence of neonatal morbidities. Therefore, monitoring standards of neonatal care and implementing quality improvement projects across the country are essential for improving neonatal outcomes in Turkish NICUs.

Clinical and Demographic Evaluation According to MEFV Genes in Patients with Familial Mediterranean Fever.

The present study examined the relationship between clinical findings and mutation analyses in children with Familial Mediterranean Fever (FMF) in the inner Black Sea region of Turkey. This retrospective, cross-sectional study included patients with FMF who were evaluated between 2007 and 2015. FMF was diagnosed according to the Tel Hashomer criteria. FMF mutations were analyzed using a Real-time PCR System (Roche Diagnostics, Mannheim, Germany), and patients were classified into three groups according to allele status. The most common symptom was abdominal pain (99%, n = 197). The most frequent mutations were M694V and R202Q. Chest pain was reported more often in patients homozygous for M694V (61.4%). Although fever, abdominal pain, and arthritis were more commonly observed with the M694V mutation, chest pain was the most common symptom in R202Q carriers (n = 10, 32.3%). Proteinuria was observed in 42 (21.2%) patients, frequently accompanied by the M694V mutation (28.6%). The most common mutations in children with FMF in Turkey were M694V and R202Q. Recurrent abdominal pain and arthritis/arthralgia were commonly observed in patients with M694V and R202Q mutations. Moreover, chest pain was commonly seen with the R202Q mutation. Thus, R202Q might be a disease-causing mutation in FMF patients.

Lead and Mercury Levels in Preterm Infants Before and After Blood Transfusions.

Very low birth weight (VLBW) infants usually receive packed red blood cell unit (pRBC) transfusions. Heavy metal transfer via pRBCs is not widely discussed before. This study aimed to determine pre-/post-transfusion erythrocyte lead and mercury levels in infants and to correlate these levels to heavy metal concentrations in pRBCs. VLBW infants (n = 80), needing pRBC transfusion for the first time, were enrolled. Erythrocyte heavy metal levels were determined in pre-/post-transfusion blood samples and also in pRBC units. Mean lead and mercury levels in the pRBCs were found to be 16.3 ± 10.8 and 3.75 ± 3.23 µg/L, respectively. Of the infants, 69.7% received lead above reference dose. Erythrocyte lead levels increased significantly after transfusions (10.6 ± 10.3 vs. 13 ± 8.5, p < 0.05) with significant correlated to amount of lead within pRBCs (r = 0.28). Mean pre-/post-transfusion erythrocyte mercury levels were 3.28 ± 3.08 and 3.5 ± 2.83 µg/L, respectively (p > 0.05). There was a significant correlation between mean difference of mercury levels after transfusion and amount of mercury delivered by pRBCs (r = 0.28). Infants can be subject to high levels of lead and mercury through pRBC transfusions.

Perfusion index and pleth variability index in the first hour of life according to mode of delivery.

Yigit S, Takci S, Bozkaya D, Yurdakök M. Perfusion index and pleth variability index in the first hour of life according to mode of delivery. Turk J Pediatr 2018; 60: 421-425. Perfusion index (PI) is a relative assessment of the pulse strength at the monitoring site. The plethysmographic variability index (PVI, pleth variability index) is a noninvasive and continuous measure of the dynamic change in PI that occurs during respiratory cycles. In this study PI and PVI changes were compared in babies born with cesarean section (C/S) or spontaneous vaginal delivery (SVD); 125 healthy term infants were monitored with pulse oxymeter after first ten minutes of life. Data were recorded from this monitor on a personal computer and analyzed by generalized estimating equations (GEE) method. Pulse rate was lower in babies born via SVD. Perfusion index were higher in C/S group. Pleth variability index were higher in C/S group. Higher pulse rate, PI and PVI values in babies born via C/S may be indicative of more significant hemodynamic changes in these infants during early transitional period after birth.

Association between vitamin D levels and cardiovascular risk factors in obese children and adolescents.

BACKGROUND AND AIM: Childhood obesity is associated with an increased risk of chronic disease. We aimed to determine the association between vitamin D deficiency and cardiovascular risks in obese children. METHOD: The studied children were selected from obese children who were followed up at obesity clinic, aged 6-17 years. Basic demographic information and laboratory data were collected retrospectively from hospital records. RESULTS: A total of 310 students (178 [57.4%] girls) were evaluated for 25-hydroxyvitamin D (25[OH] D) levels in late winter/spring. The prevalence rates of vitamin D deficiency, insufficiency, and sufficiency were 62.3%, 34.5%, and 3.2%, respectively. Insulin resistance was observed in 146 (47.1%) children; the frequencies of dyslipidemia and hypertension were 31% and 19.4%, respectively. The mean atherogenic dyslipidemia ratio was higher in the deficient group (p = 0.049). Inverse correlations of 25(OH) D levels were observed with homeostasis model assessment of insulin resistance values (r = -0.146, p = 0.010). The mean values of 25(OH) D (ng/mL) were lower in girls (12.15 ± 6.60) than in boys (16.48 ± 8.69) (p < 0.05) and in children with hypertension (11.92 ± 5.48) than in those without (14.50 ± 8.24) (p < 0.05). CONCLUSIONS: Vitamin D deficiency is observed more frequently than expected in obese children and adolescents. Our findings indicate that low 25(OH) D levels are associated with insulin resistance. Vitamin D deficiency could contribute to the morbidities associated with childhood obesity, such as insulin resistance or diabetes mellitus, increased cardiovascular/cardiometabolic risks, atherogenic dyslipidemia, and hypertension.

A rare large mutation involving two exons of the SP-B gene in an infant with severe respiratory distress.

Takci S, Anuk-Ince D, Louha M, Couderc R, Çakar N, Köseoglu RD, Ates Ö. A rare large mutation involving two exons of the SP-B gene in an infant with severe respiratory distress. Turk J Pediatr 2017; 59: 483-486. Hereditary surfactant protein-B (SP-B) deficiency is a rare autosomal recessive disease of newborn infants causing severe respiratory failure and death within the first year of life. The most common cause of SP-B deficiency is a frameshift mutation in exon 4 (121ins2) in the gene encoding SP-B. We report a term infant with unremitting respiratory distress who was unresponsive to all treatment modalities. The parents were consanguineous and a term sibling of the infant had died due to respiratory failure without a certain diagnosis. In the first step of the diagnostic work-up, common genetic mutations for SP-B, surfactant protein C and ATP-binding cassette s3 were absent, however sequencing of SP-B gene revealed a large homozygous genomic deletion covering exon 8 and 9. In this case report, we aimed to emphasize further genetic evaluation in all cases suggestive of surfactant dysfunction, even if common mutations are absent.

Copper, zinc and iron levels in premature infants following red blood cell transfusion.

This study aimed to investigate effect of erythrocyte suspension (ES) transfusion on Cu, Zn, and Fe levels. It was conducted on 53 premature infants who were admitted to Hacettepe Hospital and received EST for first time. Blood samples were drawn before and 96h after ES transfusion to determine Cu, Zn, and Fe levels in plasma and/or erythrocytes. The mean plasma Cu levels were 99±3µg/dl and 113±3µg/dl; Zn levels were 105±2µg/dl and 115±23µg/dl; mean plasma Fe level was 58.1±19.4 and 75.2±25.4µg/dl and mean erythrocyte Fe level was 4182±2314µg/ml and 7009±5228µg/ml, before and after ES transfusion. The differences between before and after ES transfusion in Cu, Zn and Fe levels were significant. Correlation between plasma and erythrocyte Fe levels was significant both before and after ES transfusion. Though Fe overload is a major cause of morbidity/mortality after ES transfusion, alterations in trace elements should also be considered when transfusing blood to infants and children.

Prevalence of hypercalciuria and urinary calcium excretion in school-aged children in the province of Tokat.

AIM: Hypercalciuria is an important cause of urinary tract symptoms, and also frequently results in urolithiasis. Urinary calcium excretion varies for geographic areas. We aimed to assess percentiles of urinary calcium excretion and prevalence of hypercalciuria for school-aged children in Tokat (city located in inner northern region of Turkey). MATERIAL AND METHODS: One thousand three hundred seventy-five children aged 6 to 18 years were enrolled in the study. Urine samples were obtained randomly. The children's variables as sex, age, length, and weight were recorded. Urinary calcium and creatinine determined from the urine samples and urinary calcium/creatinine ratios (mg/mg) were calculated. Percentiles of urinary calcium/creatinine ratios were also assessed for each age of the children. RESULTS: Six hundred eighty-three of the 1 375 children were girls and 692 were boys. The mean age of the children was 11.68±3.43 years. Some 23.9% of the children were living in rural regions and 76.1% were were living in urban regions. The mean urinary calcium/creatinine ratio was 0.080±0.24 and the 95th percentile value of the urinary calcium/creatinine ratio was 0.278. The hypercalciuria prevalence for school-aged children was 4.7% when the urinary calcium/creatinine ratio value for hypercalciuria was accepted as ≥0.21. The prevalence of hypercalciuria in rural and urban regions was 7.60% and 3.82%, respectively (p<0.05). Hypercalciuria was present in 7 of 141 patients who were obese (4.96%) and 58 of 1 234 patients who were not obese (4.70%) (p>0.05). CONCLUSION: The prevalence of hypercalciuria and urinary calcium excretion vary for different geographic areas, not only for countries. The percentiles of urinary calcium excretion should be assesed for every geographic region and the prevalance of hypercalciuria should be determined with these values. There is controversy as to whether obesity is a risk factor for hypercalciuria.

A Turkish BCS1L mutation causes GRACILE-like disorder.

A full-term growth-restricted female newborn (1790 g), presented with lactic acidosis (12.5 mmol/L) after birth. She had renal tubulopathy, cholestasis and elevated serum ferritin concentration (2819 ng/ml). Two similarly affected sisters had died before 3 months of age. Mitochondrial disorder was suspected since the disease resembled the Finnish GRACILE syndrome, caused by a homozygous mutation (c.232A > G) in BCS1L. Thus, we sequenced the BCS1L gene, encoding the assembly factor for respiratory chain complex III. The patient had a homozygous mutation (c.296C > T; p.P99L), for which both parents were heterozygous. In four previously published patients of Turkish origin, the same homozygous mutation resulted in complex III deficiency, tubulopathy, encephalopathy, and liver failure. The p.P99L mutation seems to be specific to Turkish population and leads to GRACILE-like or Leigh-like condition. Assembly defects in complex III should be investigated in the affected tissues, since fibroblasts may not exhibit the deficiency.

A new approach to an old hypothesis; phototherapy does not affect ductal patency via PGE2 and PGI2.

OBJECTIVE: Numerous investigations have demonstrated that phototherapy (PT) directly or indirectly causes ductal patency by photorelaxation effect. In this observational study, we aimed to assess the effect of PT on the incidence of patent ductus arteriosus (PDA) together with prostaglandins (PGE2) and (PGI2) levels in preterm infants. METHODS: Preterm infants whose gestational age<34 weeks and who required PT in the first 3 d of life were enrolled in this prospective study. The clinical signs of PDA, the data of detailed echocardiographic study were recorded and plasma PGE2 and PGI2 levels were measured before and after PT. The outcome measures were the status of ductus arteriosus and alterations of PGE2 and PGI2 levels under the effect of PT. RESULTS: A total of 44 preterm infants were enrolled in the study, of these 21 (47.7%) were in Group 1 (Non-PDA Group) and 23 (52.3%) were in Group 2 (PDA Group). After PT, ductal reopening occurred in three infants (14.3%) in Group 1, while ductus closed in four infants in Group 2 (17.3%). PT does not seem to effect ductal patency for both groups (p=0.250 and p=0.125, respectively). PGE2 levels were not different before and after PT for both groups (p=0.087, p=0.408, respectively). However, PGI2 levels were significantly decreased after PT in both groups (p=0.006, and p=0.003, respectively). CONCLUSION: There was no effect of PT on ductal patency. We can conclude that PGs were eliminated simultaneously with ductal closure and photorelaxation effect did not influence PG levels.

Cotreatment of congenital measles with vitamin a and intravenous immunoglobulin.

Although the measles vaccine has been part of routine national childhood vaccination programs throughout Europe, measles remains a public health concern. High numbers of cases and outbreaks have occurred throughout the European continent since 2011, and an increasing number of cases have been reported in Turkey since 2012. During a recent measles outbreak in Turkey, 2 pregnant women contracted measles prior to delivering preterm infants at Hacettepe University Hospital. Measles virus genomic RNA and IgM antibodies against measles were detected in the cord blood of infants and mothers in both cases. The infants were treated with intravenous immunoglobulin (IVIG) and vitamin A. Transient thrombocytopenia was present in 1 infant and treated with an additional dose of IVIG and vitamin A. The infants were discharged, without complications, within 10 days of birth. The successful treatment of these cases suggests that infants who have been exposed to, or infected with, measles may benefit from cotreatment of vitamin A and IVIG.

Pregnancy in women with primary biliary cirrhosis.

BACKGROUND & AIMS: Primary biliary cirrhosis (PBC) may present in all decades of life, also in childbearing age. Data on maternal and fetal outcome is limited. We aimed to investigate the impact of pregnancy and childbirth on the disease course and possible effects of PBC on fetal outcome. METHODS: Retrospective study of local cases and a compact review of published reports between 1950 and 2014. RESULTS: Our cases along with literature review provided 98 pregnancies in 72 PBC patients. PBC was diagnosed during pregnancy in 26 (36%) patients and 46 (64%) had the diagnosis before conception. Twenty-four (30%) of the pregnancies were associated with biochemical flares and 55 (70%) with clinical improvement or stabilization. De novo onset or worsening of pruritus was seen in 49% (45/92). No maternal deaths were reported. Post-partum disease activation was observed in 60% (53/88). One patient was referred for liver transplantation after delivery. A miscarriage rate of 24% and three stillbirths were reported. Most patients were treated with ursodeoxycholic acid (UDCA) during breastfeeding and 12 patients also received UDCA during the first trimester without any identified side effects. CONCLUSION: Most women with PBC maintain a stable disease during pregnancy, but post-partum biochemical flares are common. Symptomatic pruritus may be challenging in pregnant PBC patients. UDCA appears to be safe during pregnancy and breastfeeding. A successful pregnancy outcome is a realistic expectation for women with PBC.