Should Ocular Hemorrhage Screening be Conducted in Newborns with Acidosis?

OBJECTIVE: Ocular hemorrhages (OHs) may cause visual disturbances and incidence vary from 18 to 39% in newborns. Precipitated/instrumental delivery and perinatal asphyxia were predefined risk factors. Acidosis can interfere with coagulation and disrupt the pressure of ocular capillaries and put infants with moderate acidosis with or without hypoxic-ischemic encephalopathy at risk for OH. We aimed to evaluate the OH in neonates with fetal acidosis. STUDY DESIGN: Neonates >34 weeks are included if pH < 7.10 and BE < -12 mmol/L within the first hour. Ophthalmologic examinations for retinal (RH), vitreous, and anterior chamber (hyphema) hemorrhage were done within the third day. RH was staged according to Egge's classification. Follow-up of the patients was continued until the age of 2. Clinical characteristics of newborns were analyzed. RESULTS: Sixty-two neonates (38 ± 2.3 weeks, 2,971 ± 612 g) were included. pH = 6.91 ± 0.16, BE = - 17.2 ± 5.3 mmol/L. OH was found in 22 (36.7%) neonates (hyphema n = 2, vitreous n = 2, RH n = 21). Thirty-eight eyes with RH were staged (Stage 3: n = 15 [39.5%]; Stage 2: n =11 [28.9%]; Stage 1: n = 12 [31.6%]). Vaginal delivery (OR: 4.9, 95% CI [1.4-17.8]) and advanced resuscitation at the delivery room (OR: 8.8; 95% CI [1.9-41.7]) were found to increase the risk of RH. CONCLUSION: Approximately one-third of neonates with moderate to severe acidosis exhibited RH when examined on the third day. Contrary to previous studies that reported mild RH in otherwise healthy neonates, our findings revealed that neonates with moderate to severe acidosis predominantly presented with Stage 3 RH. While the higher incidence of RH in vaginally delivered infants is consistent with previous studies, the identification of advanced resuscitation as a risk factor is a new addition to the literature. The findings in our study highlight the importance of retinal examination in neonates with acidosis in the presence of intubation during resuscitation. KEY POINTS: · One-third of neonates with moderate to severe acidosis exhibited RH.. · Stage 3 RH was identified as the most prevalent.. · Advanced resuscitation was identified as an independent risk factor for RH..

The effect of oscillometric blood pressure measurement on pain response in preterm infants.

AIM: Preterm neonates are exposed to many painful procedures in neonatal intensive care units. This study aims to evaluate the effect of oscillometric blood pressure (BP) measurement on pain response in preterm infants. METHODS: This prospective study was performed over 4 months in a level III neonatal intensive care unit. Premature neonates whose gestational age was <34 weeks and postmenstrual age <36 weeks were included if they had no systemic diseases. BP measurement was performed on the right arm. The Premature Infant Pain Profile-Revised (PIPP-R) scores were evaluated three times before, during, and 10 min after BP measurement. RESULTS: During the 5-month period, 100 preterm neonates (53 male infants) were included in the study. Median birth weight and gestational age of the infants were 1148 (IQR: 1015-1300) g and 28 (IQR: 27-30) weeks, respectively. PIPP-R scores were found to be ≥7 in 34% of neonates. PIPP-R scores increased during BP measurement and decreased after. CONCLUSION: Our results demonstrated that oscillometric BP measurement which is generally accepted as a non-invasive tool for monitoring can produce mild pain in premature neonates of postmenstrual age <36 weeks.

In-Hospital Newborn Falls and Near Miss Events: A Need to Report.

OBJECTIVE: This study aimed to evaluate the clinical characteristics of the neonates who fell in the maternity ward and identify the incidence of near miss events during the immediate postpartum period. STUDY DESIGN: The study consisted of two steps. The retrospective part included the evaluation of admissions due to the in-hospital newborn fall for 6 years. The prospective part included the assessment of the near miss events (any probability of falling of the newborn; either cosleeping or an incident with a possible consequence of falling of the newborn) in the postpartum clinic (<72 hours after delivery) for a period of 4 weeks. The details of the events and clinical outcomes were recorded. A questionnaire about fatigue was administered to mothers who experienced near miss event. RESULTS: Seventeen in-hospital newborn falls were recorded: 1.8 to 2.4/10,000 live births. The median age of the neonates when the fall occurred was 22 (16-34) postnatal hours. Fourteen events (82%) occurred between 10 p.m. and 6 a.m. All neonates who experienced a fall were discharged without any known adverse outcomes. Twelve mothers (71%) had experienced a near miss event before. In the prospective arm of the study, 67 out of 804 mothers (8.3%) were found to experience a near miss event (44/1,000 days of postpartum hospitalization). Thirty-two events (49%) occurred in the first postpartum day. Fifty-two events (78%) occurred between 10 p.m. and 6 a.m. Fifty-eight mothers (86%) had no companion. Sixty-three percent of the mothers expressed intense fatigue after delivery. CONCLUSION: In-hospital newborn fall may occur in the postpartum period, and near miss events should warn clinicians for a probable fall event. The nighttime shift requires more attention regarding the prevention of both the fall and the near miss events. Immediate postpartum mothers are needed to be observed carefully. KEY POINTS: · In-hospital newborn falls occurred mainly during the night shift.. · Nearly two-third of the mothers whose newborn was fallen experienced a previous near miss event.. · Any near miss event in the hospital was detected in 8.3% of mothers..

Umbilical Cord Separation Time and Influencing Factors in Very-Low-Birth-Weight Preterm Neonates.

OBJECTIVE: The average time for umbilical cord separation in term neonates is 7 to 10 days. Prematurity, phototherapy, prolonged duration of antibiotic treatment, and parenteral nutrition are other factors which were demonstrated to delay cord separation. In the previous studies including greater premature infants, the time of separation of the umbilical cord was shown to vary 2 to 3 weeks. We aimed to determine the cord separation time and associated factors in very-low-birth-weight (VLBW) infants. STUDY DESIGN: In this retrospective study, VLBW infants (birth weight [BW] <1,500 g, gestational age [GA] < 32 weeks) without umbilical catheterization were included. Specific cord care was not applied. The cord separation time, gender, mode of delivery, presence of sepsis, duration of antibiotic treatment, serum free thyroxine, free triiodothyronine (FT3), thyroid-stimulating hormone, lowest leukocyte, polymorphonuclear leukocytes (PMNLs), and platelet counts were recorded. RESULTS: The study included 130 infants (GA: 29 ± 2 weeks, BW: 1,196 ± 243 g). Mean cord separation time was 14 ± 5 days, while 95th percentile was 22nd day of life. A positive correlation was demonstrated between duration of antibiotic treatment and cord separation time (p < 0.001, r: 0.505). Cord separation time did not differ regarding gender or mode of delivery. Corrected leukocyte count (p = 0.031, r: -0.190) and PMNL count (p = 0.022, r: -0.201), and serum FT3 level (p = 0.003, r: -0.261) were found to be negatively correlated with cord separation time. The cord separation time was found to be delayed in the presence of sepsis (with sepsis: 18 ± 7 days and without sepsis: 13 ± 3 days; p = 0.008). Sepsis was found to delay the cord separation time beyond second week (odds ratio = 6.30 [95% confidence interval: 2.37-15.62], p < 0.001). CONCLUSION: The 95th percentile for cord separation time was 22nd day. Sepsis might be either the reason or the consequence of delayed cord detachment. The exact contribution of low serum FT3 levels to the process of cord separation should be investigated in further studies. KEY POINTS: · Mean cord separation time was 14 ± 5 days, while 95th percentile was 22nd day, in VLBW infants.. · Sepsis was found to delay the cord separation time by sixfold beyond second week.. · Serum free triiodothyronine level was negatively correlated with cord separation time..

Inactivation of viruses on surfaces by infrared techniques.

Several studies on vaccines and medicines against virus-based illnesses (COVID-19, SARS, MERS) are being conducted worldwide. However, virus mutation is an issue. Therefore, inactivation and disinfection of viruses are crucial. This paper presents a method for virus inactivation by physical techniques. The infrared (IR) technique is preferred over other disinfection techniques such as ultraviolet (UV) and chemical disinfectants (alcohol) due to the associated health and environmental benefits. In this study, IR sources with various wavelengths were characterized and a far infrared (FIR) source was used to inactivate viruses. FIR sources have a therapeutic effect on the human body and have been used in medical centers. Virus spread is highly affected by environmental conditions such as temperature, humidity, and airflow. A setup with IR sources, an IR camera, an automatically controlled humidity chamber, and an airflow unit was constructed to study the viability of viruses in stationary droplets as a function of relative humidity and temperature. Bacteriophage Phi6 was used as a model organism for studying enveloped viruses such as influenza and coronavirus. IR techniques were used for studying virus inactivation. The effect of various physical conditions such as temperature, humidity, and airflows was considered to study the effect of radiation on the stationary droplets of Phi6. All measurements were performed under laboratory conditions with controlled temperature and humidity. The IR camera system was used to measure the surface temperature of Phi6 suspension droplets. The samples subjected to IR radiation were processed for plaque assay preparation and counting. Measurements were carried out to reduce and eliminate droplets, which are one of the transmission pathways of viruses. IR was radiated in closed and open-air conditions with appropriate humidity and temperature. This study reports the effective inactivation of viruses by FIR. The inactivation rate under 50 %rh for IR radiated at 1.4 m height for 3 h in closed environmental chamber was 90%, and that under an airflow rate of 0.20 m/s for 10 min in open-air conditions at a height of 1.0 m was 45.7%.

Comparison of placental α microglobulin-1 protein assay (Amnisure) with speculum examination for the diagnosis of premature preterm rupture of membranes (PPROM): a clinical evaluation.

In this study, we aimed to compare the clinical outcomes of Premature Preterm Rupture of Membranes (PPROM) cases diagnosed by classical speculum examination and by placental alpha microglobulin-1 protein (PAMG-1) assay. The medical records of all patients with singleton pregnancies that were diagnosed with PPROM were retrospectively reviewed. Singleton pregnancies with PPROM diagnosis that was confirmed either by direct visualisation of amniotic fluid leaking through the cervix or by placental alpha microglobulin-1 protein (PAMG-1) assay if no amniotic fluid leakage was documented were included in the study. Demographics, prenatal and postnatal characteristics were reviewed from the medical charts and were recorded. The study included 138 pregnancies with PPROM; 111 patients in clinical speculum examination group and 27 in PAMG-1 assay group. There were no significant differences in maternal and pregnancy characteristics between the clinical speculum examination and PAMG-1 assay groups. Foetal outcomes were comparable between clinical speculum examination and PAMG-1 assay groups. In the clinical speculum examination group, there were nine (8.1%) chorioamnionitis cases, however, there were no chorioamnionitis cases in the PAMG-1 assay group during the latency period (p = .21).Impact statementWhat is already known on this subject? Placental alpha microglobulin-1 protein assay uses immunochromatography method to detect trace amount of placental alpha microglobulin-1 protein in vaginal fluids and has high sensitivity and specificity for ROM diagnosis. However, to the best of our knowledge, the clinical outcome of ROM cases detected by classical speculum examination and by placental alpha microglobulin-1 protein assay has not been compared in the literature previously.What do the results of this study add? Although statistically insignificant, cases diagnosed by PAMG-1 assay had lower risk of chorioamnionitis during latency period.What are the implications of these findings for clinical practice and/or further research? Whether cases diagnosed by PAMG-1 assay represent a milder form of rupture of membranes than cases diagnosed by classical speculum examination group warrants further research.

Minor dysmorphic features in a patient with papillorenal syndrome: A Case Report.

Papillorenal syndrome, also known as renal coloboma syndrome, is characterised by congenital optic disc anomalies and renal abnormalities. Mutations in the PAX2 gene, which plays a critical role in embryogenesis, cause this syndrome. Other related anomalies are less commonly observed. To our knowledge, this is the first case reported in the literature in which Papillorenal syndrome accompanied various dysmorphic features.

Severe Anemia Is Associated with Intestinal Injury in Preterm Neonates.

OBJECTIVE: A temporal relationship has been reported between necrotizing enterocolitis, anemia, and red blood cell transfusion (RBCT) in preterm neonates. However, the mechanism underlying this association is not clearly defined. Intestinal (I-) and liver (L-) fatty acid binding proteins (FABPs) have been proposed as plasma markers for the detection of acute intestinal injury. This study aimed to investigate the effect of anemia and RBCT on intestinal injury in preterm neonates by measuring serum I-FABP and L-FABP levels. STUDY DESIGN: A prospective cohort study including preterm neonates with gestational age <32 weeks and/or birth weight <1,500 g and requiring erythrocyte transfusions for anemia after day 15 of life was conducted. Stable growing preterm infants with hemoglobin values ≥ 10 g/dL were taken as controls. I-FABP and L-FABP levels of the neonates with anemia were compared with levels of the control group. In addition, pretransfusion I-FABP and L-FABP levels of the neonates with anemia were compared with posttransfusion levels. RESULTS: In total, 39 infants transfused for anemia and 20 controls were enrolled. L-FABP levels were significantly higher in neonates with anemia compared with controls (p < 0.001), whereas I-FABP (p = 0.695) was not different. L-FABP and I-FABP levels were similar before and after transfusion in neonates with anemia. L-FABP levels before transfusion were negatively correlated with pretransfusion hemoglobin levels (p < 0.001). CONCLUSION: Anemia is associated with intestinal injury documented by increased L-FABP levels in preterm infants, and this injury is more severe with lower hemoglobin levels.

Neonatal outcomes in Syrian and other refugees treated in a tertiary hospital in Turkey

Background/aim: Turkey accepts refugees from many countries, principally Syria. More than 2.7 million refugees live in Turkey.We evaluated the neonatal outcomes of refugees. Materials and methods: We retrospectively reviewed the clinical and demographic characteristics of refugee infants born in our hospital between August 2013 and September 2016. Results: Refugees (718 Syrian, 136 Iraqi, 32 Afghani, and 21 of other nationalities) accounted for 907 of 49,413 births. The mean refugee maternal age was lower than that of Turkish women, whereas the gestational age (GA) and birthweight were similar. Refugees required fewer cesarean sections but exhibited greater small- and large-for-GA rates (P < 0.05). Refugee and Turkish infant mortality rates did not differ significantly (0.8 vs. 0.4%). Eighty-nine (12.3%) refugee neonates and 6682 (13.5%) Turkish neonates were admitted to our neonatal intensive care unit (NICU). Jaundice and perinatal asphyxia were significantly more common in refugees, whereas respiratory distress syndrome, GA ≤32 weeks, and infant birthweight <2000 g were more common in Turkish infants. The total NICU admission cost of approximately 450,000 USD was paid by the Turkish government. Conclusion: The numbers of refugees and refugee births continue to grow. The Turkish people and government have provided medical, social, and economic support to date; international assistance is needed.

The effect of midkine on growth factors and oxidative status in an experimental wound model in diabetic and healthy rats.

Wound healing is important for longevity. Midkine is a cytokine involved in controlling tissue repair and new tissue development, and in regulating inflammation. We investigated the effect of midkine on wound healing in rats. In total, 108 Wistar albino rats were used: 12 as healthy and diabetic controls; 96 were split into 4 groups: healthy, saline treated; healthy, midkine (10 ng/kg, 48 h intervals) treated; diabetic, saline treated; and diabetic, midkine treated. Following wound creation, 6 rats per group were euthanized on days 3, 7, 14, and 28; the wounded skin was removed. Levels of epidermal growth factor (EGF), matrix metalloproteinase-8 (MMP-8), transforming growth factor beta (TGF-ß), platelet-derived growth factor (PDGF), vascular endothelial growth factor (VEGF), and thiobarbituric acid reactive substances (TBARS) were measured. MMP-8 and PDGF levels fluctuated in all groups; TGF-ß fluctuated in the diabetic groups and was significantly higher in the HM group than other groups after 14 days. EGF and VEGF levels were increased in the HM group after 3 days. TBARS levels were highest in the diabetic groups. Macroscopically, the midkine-treated groups healed better. Midkine can accelerate wound healing by influencing growth factors and oxidative status in wound tissues.

A Newborn with Transient Diabetes Mellitus Accompanied by Ketoacidosis Attributable to a ZFP57 Mutation.

Hyperglycemia commencing within the first 6 months of life requires exogenous insulin therapy and, if the condition persists for >2 weeks, is termed neonatal diabetes mellitus (NDM). This rare illness is of two types: transient and permanent NDM. Most cases come to medical attention because of nonspecific symptoms, including intrauterine growth retardation, dehydration, difficulties in feeding and inadequate weight gain. In the present article, we describe an infant who smelt of ketones during examination and who was diagnosed with transient NDM caused by a ZFP57 mutation, accompanied by ketoacidosis. This is the first report of such a condition.

Central venous catheter insertion into the false lumen of a complicated aortic dissection.

Thoracic endovascular repair is considered the first-line treatment in complicated acute type B dissection. Central venous catheters provide valuable vascular access during endovascular treatments. However, central venous catheters are not without complications. Herein, we report a case of central venous catheter insertion into the false lumen of a complicated acute type B aortic dissection by direct aortic puncture. The tip of the central venous catheter was in the false lumen. The central venous catheter was left in place initially and was removed after graft stent deployment. This case illustrates the importance of image guidance during central venous catheter insertion, which may further complicate an already complicated aortic dissection case.

Corkscrew Collateral Vessels in Buerger Disease: Vasa Vasorum or Vasa Nervorum.

PURPOSE: To investigate the origin of "corkscrew" collateral vessels around the occluded popliteal artery in patients with Buerger disease by Doppler ultrasound (US) and magnetic resonance (MR) imaging in tandem with digital subtraction angiography (DSA). MATERIALS AND METHODS: Between January 2013 and June 2015, 42 patients diagnosed with Buerger disease were identified retrospectively. Patients in whom occlusion of the popliteal artery was found on DSA of the lower extremity were subjected to Doppler US and MR imaging prospectively. Fifteen of 42 patients were identified as having the required characteristics, of whom 10 participated in the present study. RESULTS: Ten patients with occlusion of the popliteal artery were selected for inclusion, and 12 lower limbs of these patients were investigated. The study cohort comprised one woman and nine men with a mean age of 41 years ± 10 (standard deviation; range, 39-58 y). Corkscrew collateral vessels identified on DSA examinations were also identified on secondary imaging (Doppler US and MR imaging) in all patients except one in whom the popliteal artery was reconstituted after short-segment occlusion. The origin of the corkscrew collateral vessels was identified as the vasa nervorum of the tibial nerve in nine patients. CONCLUSIONS: Data from the present study suggest that corkscrew collateral vessels at the knee level in patients with Buerger disease originate from the vasa nervorum of the tibial nerve rather than the vasa vasorum of the popliteal artery if the latter is occluded.

A Previously Healthy Premature Infant Treated With Thrombolytic Therapy for Life-threatening Pulmonary Artery Thrombosis.

Pulmonary artery thrombosis is rarely reported in preterm neonates. Although treatment of neonatal thrombosis remains controversial, thrombolytic agents must be considered when the thrombosis is life threatening. We herein present a case of a preterm newborn with pulmonary artery thrombosis accompanied by acute-onset respiratory failure and cyanotic congenital heart disease. The thrombosis was successfully treated using tissue plasminogen activator. In conclusion, the thrombolytic therapy should be considered in treatment of patients in whom the thrombosis completely occludes the pulmonary arteries.

Central corneal thickness and intraocular pressure in premature infants.

To evaluate the central corneal thickness (CCT) and intraocular pressure (IOP) of premature infants and to document correlation of them with gestational age, chronological age, and birth weight of infants. Using a hand-held applanation tonometer and a portable pachymeter, IOP and CCT of 170 premature infants were measured just before initial retinopathy of prematurity screening examination and re-measured 4 weeks after the first visit. The CCT and IOP were positively correlated during the first (r = 0.616, p < 0.001) and second (r = 0.564, p < 0.001) visits. The mean CCT at first and second visits were 568.1 ± 22.1 (527-628) and 561.6 ± 21.4 (520-619) µm, consecutively (p < 0.001). Gestational age, chronological age and birth weight of infants were found to be negatively correlated with CCT at first (r = -0.751, p < 0.001; r = -0.745, p < 0.001; r = -0.581, p < 0.001, consecutively) and second (r = -0.729, p < 0.001; r = -0.729, p < 0.001; r = -0.555, p < 0.001, consecutively) visits. The mean IOP at first and second visits were 14.1 ± 1.9 (11-19) and 13.7 ± 1.7 (11-18) mmHg, consecutively (p < 0.001). Gestational age, chronological age, and birth weight of infants were found to be negatively correlated with IOP at first (r = -0.724, p < 0.001; r = -0.715, p < 0.001; r = -0.558, p < 0.001, consecutively) and second (r = -0.704, p < 0.001; r = -0.703, p < 0.001; r = -0.518, p < 0.001, consecutively) visits. CCT and IOP of the premature infants with a smaller gestational age were found to be higher (p < 0.001 for both). Premature infants with smaller gestational age have higher CCT and IOP values when compared to older infants. These values tend to become lower 4 weeks after the first examination as infants become older. The CCT and IOP were positively correlated with each other and both were negatively correlated with gestational age, chronological age, and birth weight during first and second visits.

Severe leukemoid reaction in a preterm infant with congenital cytomegalovirus infection.

Leukemoid reaction, defined as a total leukocyte count of >50,000/mm, is most commonly related to antenatal administration of steroids, infections, and transient myeloproliferative disorder of Down syndrome in newborns. Atypical presentations of viral infections can be a diagnostic challenge in the newborn period. Cytomegalovirus (CMV) infection causes a multisystem disease, and symptomatic infants generally present with intrauterine growth restriction, hepatosplenomegaly, cholestasis, rash, thrombocytopenia, and microcephaly. We present a case of a preterm infant with severe myeloid leukemoid reaction (leukocyte count >100,000/mm) at birth who was diagnosed with congenital CMV infection on the basis of CMV polymerase chain reaction results after the appearance of cholestasis, blueberry muffin rash, and hepatosplenomegaly.

Congenital megalourethra presenting with large cystic mass in the inguinal area.

Congenital megalourethra is a rare urogenital malformation characterized by severe dilatation and elongation of the penile urethra. It causes functional obstruction of the lower urinary system. Herein, we present a newborn with congenital megalourethra with single umbilical artery.

A newborn with widespread skin lesions and extremity anomalies.

A variety of diseases can cause vesicular or pustular eruptions in newborns. A thorough clinical history and physical exam provide important clues for differential diagnosis. Goltz syndrome, also known as focal dermal hypoplasia, is a rare genodermatosis and generally diagnosed by the presence of the characteristic skin lesions. We present an infant with aplasia cutis, atrophic skin lesions, syndactyly and eye abnormalities who was diagnosed with Goltz syndrome soon after birth. Goltz syndrome should be considered in differential diagnosis of hypoplastic or aplastic skin lesions in neonates in the presence of skeletal or ophthalmic anomalies.

The incidence and risk factors of severe retinopathy of prematurity in extremely low birth weight infants in Turkey.

BACKGROUND: The aim of this study was to identify the incidence of severe retinopathy of prematurity (ROP) requiring laser treatment and the overall incidence of ROP in extremely low birth weight (ELBW) infants, and to evaluate the associated risk factors for developing severe ROP in ELBW infants in Turkey. MATERIAL AND METHODS: This retrospective, case-control, tertiary care-hospital based study included 235 ELBW infants screened for ROP. The incidence of the onset of ROP and severe ROP requiring laser treatment were assessed. The relationship between various clinical risk factors, and the development of severe ROP and onset of ROP were analyzed using univariate analysis and multivariate logistic regression analysis. RESULTS: The overall incidence of any stage ROP and severe ROP requiring laser treatment in ELBW infants was 75.5% and 38.7%, respectively. Severe ROP requiring laser treatment in ELBW infants was significantly associated with various independent risk factors, including blood transfusion (P=0.002), gestational age at birth (P<0.001), ges- tational weight at birth (P=0.001), culture-proven sepsis (P=0.047). CONCLUSIONS: The severity of ROP may increase as weight at birth and gestational age at birth decrease, and in those with culture-proven sepsis as well as in those that receive blood transfusion. Clinicians should be aware of the presence of these risk factors when treating ELBW premature infants. Early detection and prevention of sepsis and reducing the number of blood transfusions may decrease the incidence of severe ROP requiring laser treatment.

Fetal echogenic bowel in association with Zellweger syndrome.

Increased echogenicity of fetal bowel in the second trimester obstetrical ultrasound has been described in association with several pathologic conditions, such as growth restriction, aneuploidy, cystic fibrosis, congenital infections, and gastrointestinal malformations. Zellweger syndrome (ZS) is the prototype of peroxisomal disorders characterized by craniofacial dysmorphism and severe neurologic abnormalities. We report two cases with fetal echogenic bowel (FEB) but no associated anomalies and normal fetal growth. After birth, clinical and laboratory findings led to diagnosis of ZS. Association of FEB with neurometabolic disorders is limited to a few case reports in the medical literature. To the best of our knowledge, this is the first report of ZS associated with FEB.