About

Danitza M. Velazquez, MD, is an Assistant Professor of Pediatrics at Rutgers New Jersey Medical School within the Department of Pediatrics. She completed her MD at UMDNJ-NJMS in 2011 and earned her BA from Rutgers, The State University in 2007. Her postgraduate training includes an internship and residency at Rutgers RWJMS from 2011 to 2014, followed by a fellowship at Albert Einstein College of Medicine- The Childrens Hospital at Montefiore in Bronx, NY, from 2014 to 2017. Dr. Velazquez's clinical expertise encompasses general pediatrics and neonatology, and she is involved in patient care at University Hospital, Newark. Her research contributions include publications on neonatal conditions such as persistent hypoglycemia, congenital diarrhea, patent ductus arteriosus, and other neonatal syndromes, reflecting her focus on neonatal and pediatric health issues.

Research topics

• Medicine
• Pediatrics
• Surgery
• Anesthesia
• Pharmacology
• Internal medicine
• Pathology

Selected publications

• A Newborn Infant with Hand Nodule Following Intravenous Extravasation

  NeoReviews · 2022 · 1 citations

  Senior authorCorresponding
  • Medicine
  • Anesthesia
  • Pathology

  A male infant is born at 39 weeks and 5 days of gestation to a 22-year-old primigravida via spontaneous vaginal delivery. The labor course is complicated by premature rupture of membranes (ROM) for 22 hours, preeclampsia, and non-reassuring fetal heart rate. The mother receives azithromycin, cefazolin, and penicillin G for prolonged ROM. The infant’s birthweight is 3,240 g (41st percentile), length 48.3 cm (20th percentile), and head circumference 34 cm (36th percentile). His Apgar scores are 9 and 9 at 1 and 5 minutes, respectively. The infant is initially admitted to the well newborn nursery. Within the first hour after birth, he begins to show signs of respiratory distress with grunting, nasal flaring, and retractions. He is transferred to the NICU for further management. NICU and admission vital signs are as follows: temperature 99.1°F (37.2°C), heart rate 178 beats/min, respiratory rate 48 breaths/min, and oxygen saturation 89% in room air.In the NICU, the infant is given continuous positive airway pressure of +5, requiring 25% fraction of inspired oxygen to maintain normal oxygen saturations. A partial sepsis evaluation is initiated and the infant is started on ampicillin and gentamicin. Blood culture, complete blood cell count, and C-reactive protein measurements are obtained. The infant is initially made nil per os and intravenous fluids are started with D10W at 60 mL/kg per day. Respiratory distress resolves on day 1 after birth and the infant makes a successful transition to room air. Intravenous fluids are weaned as the infant is successfully advanced to full enteral feeds. Chemistry findings on day 1 after birth are significant for a serum calcium level of 7.1 mg/dL (1.77 mmol/L). Repeat chemistry on day 2 shows further decrease of serum calcium to 6.3 mg/dL (1.57 mmol/L) for which he receives calcium gluconate 200 mg/kg via peripheral intravenous catheter in the left hand. On completion of the infusion, the insertion site is noted to have a 1 × 1 cm area of white discoloration and swelling. Hyaluronidase is administered subcutaneously to the site. Plastic surgery and wound care services are consulted and recommendations are made for hand elevation, and bacitracin and xeroform gauze are applied to the site.Repeat calcium levels improve to 7.3 mg/dL (1.82 mmol/L) on day 3 and 7.5 mg/dL (1.88 mmol/L) on day 4, with a phosphorus level of 8.1 mg/dL (2.62 mmol/L; high), magnesium of 1.6 mg/dL (0.66 mmol/L; normal), and 25-hydroxyvitamin D of 8.4 ng/mL (21 nmol/L; low). Pediatric endocrinology is consulted at this point. The etiology of the hypocalcemia and low vitamin D level is determined to be secondary to low maternal stores and the infant is subsequently started on 1,600 IU of ergocalciferol by mouth once daily.The extravasation lesion on the dorsum of the left hand progresses to blanching erythema with swelling extending from the dorsum of the hand to the forearm by day 6 after birth. An initial radiograph of the left upper extremity shows normal soft tissue with no bony abnormalities (Fig 1). On day 9, the soft tissue swelling becomes indurated and tender to touch concerning for cellulitis. Due to the rapid progression of the cellulitis, a sepsis evaluation including a lumbar puncture is performed. The infant is started on vancomycin and cefepime for cellulitis with methicillin-resistant Staphylococcus aureus coverage for 10 days. The cerebrospinal fluid culture is negative, and parameters are within normal limits and not concerning for infection. The blood culture is also negative. On completion of the 10-day antibiotic course, nontender induration of the forearm remains, and a new firm, nontender nodule is noted on the dorsum of the left hand. Mobility of all joints of the left upper extremity remains unrestricted. Repeat radiography shows diffuse extraosseous calcification of the soft tissue of the forearm and hand, with sheetlike distributions along fascial planes of extensor and flexor muscle groups (Fig 2). Orthopedic surgery evaluates the infant and recommends no further acute orthopedic intervention. The infant is discharged from the hospital on day 18 after birth. Anticipatory guidance is provided to the parents before discharge to reinforce that intradermal calcifications most often reabsorb spontaneously over time over the course of weeks to months. Close follow-up with pediatric endocrinology, orthopedics, pediatrics, and neonatal high-risk clinic is set up for the infant.Calcinosis cutis is a common but overlooked condition that is seen in both the pediatric and neonatal populations. It is characterized by the presence of precipitated calcium crystals in soft tissue. The condition is divided into 5 subtypes based on etiology and severity: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. (1) Dystrophic calcinosis cutis is caused by local trauma to the tissue, such as by repeated intravenous access, heel sticks, or other procedures, and is present in patients with normal calcium and phosphorus levels. (1) Metastatic calcinosis cutis occurs in patients with underlying disorders of calcium or phosphorus metabolism (and therefore abnormal calcium and phosphorus levels) and can also be associated with calcium deposition in blood vessels, lungs, kidneys, and intestines. (1) Iatrogenic calcinosis cutis is caused by the extravasation of intravenous calcium gluconate, calcium chloride, or fluids containing phosphorus. (1) Idiopathic calcinosis cutis occurs in the absence of underlying causes like metabolic disorders or tissue damage. Calciphylaxis is the most serious subtype, defined as a vasculopathy of small and medium vessels that lead to ischemic necrosis. It is also known as uremic gangrene syndrome or calcific uremic arteriolopathy and most often seen in patients with chronic renal failure. (1)Iatrogenic calcinosis cutis generally presents days to weeks after the administration of intravenous calcium with initial erythema, swelling, and local tenderness at the intravenous site. The appearance of the lesion develops over time as the extravasated calcium spreads along fascia, often resulting in large areas of swelling and erythema. (1) Eventually the lesion progresses to hard, tender nodules that persist for weeks to months.Existing cases in the literature involve patients who present when their lesions have already progressed to the nodular stage, or report patients whose lesions are not described starting from the day of intravenous calcium administration. The early presentation of calcinosis cutis, as observed in our patient, is similar to cellulitis and often initially diagnosed as cellulitis. (2) However, unlike cellulitis, calcinosis cutis is not associated with systemic signs of infection such as fever, elevated leukocytes, or elevated C-reactive protein, though altered vital signs like tachycardia related to pain can be present.The first-line imaging modality is radiography, which confirms the presence of calcifications in soft tissue. Radiographs should be taken 7 days to 3 weeks after the initial administration of calcium. (1)Iatrogenic calcinosis cutis is generally self-limiting. Nodules and swelling regress slowly over time and eventually disappear over a period of weeks to months, fully clearing by 6 months after the initial insult. (3) Symptomatic treatment with hot and cold compresses can be helpful, as is the administration of hyaluronidase immediately after extravasation. (3) Conservative approaches are preferable, especially in younger children, though surgical intervention with excision of calcified nodules can be considered. Although this condition is usually benign, it is also important to note that idiopathic calcinosis cutis has been documented as the cause of compartment syndrome in the neonatal population. (4)Idiopathic calcinosis cutis is a complication that can occur in neonates after the administration of intravenous calcium.Radiographic findings may not be present until 7 days after extravasation of calcium.The condition is self-resolving, typically in weeks to months.Conservative treatment may be advisable with resolution of findings in weeks to months.

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• Delayed Presentation of Neonatal Drug Withdrawal in Neonate With Congenital Hypothyroidism

  Clinical Pediatrics · 2022 · 2 citations

  Senior authorCorresponding
  • Medicine
  • Pediatrics
  • Internal medicine
  PublisherDOI

• Case 1: Persistent Hypoglycemia in a 46,XX Newborn with Ovarian Dysgenesis

  NeoReviews · 2020-01-01

  articleSenior author

  A 4-day-old female newborn is transferred from an outside hospital for further evaluation and management of hypoglycemia. She had been born at 37 weeks’ gestation to a 23-year-old gravida 2, para 1 woman via spontaneous vaginal delivery. The prenatal history was unremarkable. She had a birthweight of 2,815 g (50th percentile) and length of 47 cm (40th percentile). She was initially discharged from the hospital on day 2 after birth. On day 4, she is brought back to the emergency department after 5 episodes of watery, nonbloody diarrhea and nonbloody, nonbilious emesis at home. In the emergency department, her vital signs are within the normal range for age. She is found to be hypoglycemic, with a blood glucose level of 31 mg/dL (1.7 mmol/L) for which she received a bolus of 10% dextrose in water. The repeat glucose level after the bolus is 84 mg/dL (4.6 mmol/L) and she is subsequently transferred to the NICU. On arrival at the NICU, her admission vital signs are within the normal range for age, and physical examination reveals a moderately dehydrated, nondysmorphic infant who is alert and fussy. She has jaundice to the umbilicus and a dermal melanocytosis spot near her sacrum. The remainder of her physical examination findings are within the range for age. Her blood glucose concentration is 53 mg/dL (2.9 mmol/L). Her admission blood gas measurements and complete blood cell counts are unremarkable. She is started on 10% dextrose water in addition to feedings of expressed breastmilk and supplemental infant formula as needed, at least every 3 hours. Blood and stool cultures are sent and prophylactic antibiotics initiated. Total and direct bilirubin concentrations are normal—9.6 mg/dL (164.2 μmol/L) and 0.3 mg/dL (5.1 μmol/L), respectively. Antibiotics are discontinued after the blood culture is found to be negative for 48 hours. Stool …

  PublisherDOI

• Case 1: Vomiting and Bradycardia in a Newborn Infant

  NeoReviews · 2019-04-01 · 1 citations

  articleSenior author

  A male infant is born at 39 weeks and 5 days’ gestational age via normal spontaneous vaginal delivery to a 21-year-old gravida 1, para 0 woman with no known medical problems. The pregnancy is uncomplicated and prenatal laboratory results are all unremarkable. Apgar scores are 8 and 9 at 1 and 5 minutes, respectively. Initial newborn examination findings are within normal limits. The infant is born with a birthweight of 3.34 kg, head circumference of 32.3 cm, and length of 52.1 cm, with all measurements plotted to be appropriate for gestational age. The infant is noted to be tolerating breast and bottle feeds well and is voiding and passing stools normally. He is discharged from the newborn nursery 2 days after birth with normal physical examination findings and a weight of 3.3 kg, which is 1.2% below birthweight.Four days after birth, the infant is brought back to the birth hospital for feeding intolerance and having increased nonbloody, nonbilious vomiting with phlegm after each feeding. In the emergency department (ED), the infant is noted to have lost 12% of his birthweight. He is also found to have self-limiting episodes of bradycardia with heart rates in the 70 beats/min range. Electrocardiography is performed, which shows sinus bradycardia. He receives two 10-mL/kg boluses of normal saline, and the heart rate improves to around 100 beats/min with intermittent dips to around 90 beats/min. The infant’s other vital signs are otherwise within normal limits for age. A full sepsis evaluation is initiated in the ED and the infant is started on ampicillin and gentamicin treatment for presumed sepsis. The lumbar puncture is unsuccessful, but blood and urine culture specimens are obtained. Rapid testing for respiratory syncytial virus and influenza A and B has negative results and the chest radiograph is normal. Complete blood cell count reveals a white blood cell count of 8,000/μL (8.8×109/L), with a normal differential, hemoglobin of 17.6 g/dL (176 g/L), hematocrit of 53.2%, and platelet count of 409×103/μL (4.09×109/L). The basic metabolic panel is significant for hypernatremia, with a sodium level of 151 mEq/L (151 mmol/L). The sample is hemolyzed and the potassium level is not reported. Other values are within normal limits with chloride of 112 mEq/L (112 mmol/L), total bicarbonate of 22 mEq/L (22 mmol/L), glucose of 73 mg/dL (4.05 mmol/L), blood urea nitrogen of 11 mg/dL (3.9 mmol/L), creatinine of 0.55 mg/dL (48.6 μmol/L), and calcium of 10.1 mg/dL (2.5 mmol/L).The infant is transferred to a level III NICU for further evaluation and management. Abdominal radiography is performed on admission to the unit, which shows an overall ‘gasless’ abdomen and a cystic lucency projecting over the lower mediastinum (Fig 1). He is given nothing by mouth on admission and started on intravenous fluids. Pediatric surgery is consulted and computed tomography (CT) of the chest, abdomen, and pelvis is completed, which shows a complex gastric hernia with the gastroesophageal junction above the diaphragm (Fig 2). A subsequent barium esophagography confirms a hiatal hernia or intrathoracic stomach with significant partial obstruction (Fig 3). Echocardiography is also performed, which shows a structurally normal heart with good function but an external impingement on the posterior aspect of the left atrium is noted.A diagnosis of a hiatal hernia with compression of the heart is made, which is likely the factor contributing to the episodic bradycardia. The infant is taken to the operating room 8 days after birth for open repair of the hiatal hernia with gastrostomy tube placement. A gastrostomy is placed to provide support or hold the stomach to the anterior abdominal wall. Intraoperatively, the infant is found to have a small diaphragmatic defect and a large portion of the proximal stomach in the chest, with only the distal stomach and pylorus in the thoracic cavity. The stomach is reduced and the hiatal defect is repaired with crural approximation.The infant has no postoperative complications and is able to tolerate ad libitum feeds by mouth before discharge on postoperative day 6. No further episodes of vomiting or bradycardia are noted and he demonstrates appropriate weight gain. The infant is discharged from the hospital with instructions to the family to follow up with pediatric surgery 2 months later. On outpatient follow-up, he continues to gain weight appropriately, and the gastrostomy was reversed successfully without complications.Congenital hiatal hernias are very rarely seen in the neonatal period and are characterized by a herniation of the abdominal organs, most commonly the stomach, into the thorax from a physiologic opening caused by the laxity of the attachment of the stomach and gastroesophageal junction. Congenital hiatal hernia has to be distinguished from the congenital diaphragmatic hernia, which is a separate entity where there is a pathologic defect in the diaphragm. There are 4 anatomic classifications of hiatal hernias, types I, II, III and IV. Type I or a sliding-type hernia is the most common type, accounting for more than 90% of cases. (1) Type II or a paraesophageal-type hernia, although considered a rarer type, has a higher risk of complications such as incarceration, strangulation, complete gastric herniation with organoaxial volvulus, and a perforation of herniated viscera. (2)(3) Type III is a combination of type I and II, and type IV is characterized by the presence of a structure other than the stomach, such as the omentum, colon, or small bowel within the hernia sac. (1)Congenital hiatal hernias may present with symptoms such as respiratory distress, vomiting, poor feeding, failure to thrive, poor feeding, or signs such as bradycardia as observed in the current case when the hernia is massive enough to cause cardiac compression. These signs and symptoms have a broad list of differential diagnoses including sepsis, gastroesophageal reflux disease, pneumonia, pneumatocele, pneumothorax, pleural effusion, and congenital diaphragmatic hernia. The diagnosis of hiatal hernias is usually made with a barium swallow esophagography, upper endoscopy, or CT scan showing the herniation of intra-abdominal contents into the thorax. (1)Although rare, there have been 9 reported cases of congenital hiatal hernia diagnosed antenatally. (4)(5)(6)(7)(8)(9) All of the reported cases were identified during the third trimester of pregnancy. It is postulated that the late onset of findings is because of the fact that the fetus may only be able to develop sufficient pressure to dilate the intrathoracic stomach by the third trimester. Hence, with the development of adequate pressure during swallowing and likely with the effect of reflux of gastric secretions into the esophagus, the dilated esophagus/stomach may be visualized during third-trimester ultrasonography. (8) This finding has been described as a presence of a cystic mass in the posterior mediastinum, usually located behind the heart juxtaposed to the vertebral body and connecting to the intra-abdominal stomach. (7)Surgical correction is the treatment of choice, especially in symptomatic paraesophageal hernias with obstructive symptoms. Patients may also be asymptomatic; however, because of the risk of subsequent complications, elective surgical treatment is necessary shortly after diagnosis. (10)

  PublisherDOI

• Congenital diarrhea in a newborn infant: A case report

  World Journal of Clinical Pediatrics · 2019-08-29 · 2 citations

  articleOpen accessSenior author
  PublisherDOI

• Congenital diarrhea in a newborn infant: A case report

  World Journal of Clinical Pediatrics · 2019-08-29

  articleOpen accessSenior author
  PublisherDOI

• Congenital diarrhea in a newborn infant: A case report

  World Journal of Clinical Pediatrics · 2019-08-29 · 4 citations

  articleOpen accessSenior author

  BACKGROUND: Microvillus inclusion disease (MVID) is a rare autosomal recessive cause of severe congenital diarrhea with significant morbidity and mortality. Definitive treatment involves bowel transplant. The diagnosis of this condition can be challenging and a few genetic panels are available for the identification of the most common mutations. We present the case of an infant with MVID due to a mutation not reported in the literature before. CASE SUMMARY: gene [homozygous mutation for MYO5B c.1462del, p. (Ile488Leufs*93)] giving us the diagnosis of MVID. She remains on TPN while awaiting bowel transplant at the time of the compilation of this case report. CONCLUSION: We report a novel mutation involved in MVID and highlight the importance of considering this disease when faced with a newborn presenting with life threatening diarrhea. At the time of this publication, 232 allelic variations of this gene (MIM#606540) exist in National Center for Biotechnology Information's database. Our patient's mutation has not been reported in literature as a cause of MVID.

  PublisherOA PDFDOI

• The effect of hemodynamically significant patent ductus arteriosus on acute kidney injury and systemic hypertension in extremely low gestational age newborns

  The Journal of Maternal-Fetal & Neonatal Medicine · 2018-04-12 · 32 citations

  article1st authorCorresponding

  Background: Acute kidney injury (AKI) in preterm neonates is becoming an increasingly recognized morbidity in the neonatal intensive care unit neonatal intensive care unit (NICU), yet its epidemiology, delineation and relation to numerous toxic exposures and common morbidities such as systemic hypertension is just evolving. With a frequency of the patent ductus arteriosus (PDA) as high as 70% in preterm infants born before 28-week gestation, the role of the hemodynamically significant PDA (hs-PDA) remains unclear.Objective: To determine if AKI and systemic hypertension is more common in extremely low gestational age newborns (ELGAN) with hs PDA compared to ELGAN with no or non-hs PDA using modified AKIN and Neonatal Risk, Injury, Failure, Loss of Kidney Function, and End-stage (N-RIFLE) scoring systems.Methods: This was a retrospective cohort study of infants ≤28 weeks gestational age born between 2010 and 2016 who had echocardiographic PDA evaluation completed for hemodynamical significance as well as serial serum creatinine and urine output measurement documented, needed for the two AKI scoring systems: modified AKIN (based on serial serum creatinine) and N-RIFLE (using urine output data). Blood pressure measurements and therapy were evaluated during the hospitalization and on the day of NICU discharge. Baseline characteristics and outcome variables were compared between the hs-PDA and no or non-hs PDA using unpaired t-tests for continuous variables and chi square tests for categorical data.Results: One hundred fifty-one infants were eligible of which 110 had hs-PDA. Infants with hs-PDA were smaller (777 versus 867 g, p = .026), less mature (25.8 versus 26.4 weeks, p = .023) and had greater exposure to nephrotoxic drugs (14 versus 9.4 days, p = .001). Other clinical and demographic variables were similar between the two groups. The overall incidence of AKI was not different between the hs-PDA and no PDA or non-hs PDA groups when evaluated by the acute kidney injury network (AKIN) or N-RIFLE staging; however, preterm newborns with hs-PDA demonstrated a trend towards increased risk of AKI injury (12.7 versus 0.02%, p = .06). The N-RIFLE and AKIN scoring systems demonstrated very poor degree of agreement (kappa = 0.00853) in our study. There was no difference in the rates of hypertension during the hospitalization as well as on the day of NICU discharge.Conclusion: Preterm neonates with hs-PDA had similar rates of AKI and hypertension as neonates with no or non-hs PDA.

  PublisherDOI

• Case 2: A Neonate Affected by Maternal Pica

  NeoReviews · 2017-03-01

  article1st authorCorresponding

  A male infant is born at 38 6/7 weeks’ gestation via vaginal delivery to a 23-year-old gravida 3, para 2-0-0-2 woman. On the first day after birth, he is found to have a critically elevated blood lead level (BLL). The infant was tested because of the mother’s prenatal history of an elevated BLL. History revealed the mother had ingested soil from her backyard and used ethnic products from a local store (not tested for lead) throughout the pregnancy. The backyard soil was later tested by the New York City Department of Health and Mental Hygiene (NYC DOHMH), which revealed high lead levels. Five months before delivery, the maternal BLL was 10 μg/dL (0.48 μmol/L). Repeat maternal BLL on postpartum day 2 was 49 μg/dL (2.3 μmol/L).At birth, the newborn Apgar scores were 9 and 9 at 1 and 5 minutes, respectively, the birthweight was 3.57 kg, and the length was 50 cm (body surface area of 0.21 m2). The infant’s venous BLL on the first and second day after birth was 78 μg/dL (3.8 μmol/L). On admission, the infant had unremarkable routine chemistry studies, a hemoglobin of 17 g/dL (170 g/L), and mean corpuscular volume of 90 um3 (90 fL).On the third day after birth, the newborn was transferred from the NICU of an affiliate hospital to our institution for dual chelation therapy with calcium disodium versenate (CaNa2EDTA) and succimer, as recommended by the Centers for Disease Control and Prevention (CDC) and NYC DOHMH guidelines. Succimer is a chelating agent that has 2 sulfhydryl groups capable of complexing to lead, making it water soluble, and thus increasing the urinary excretion of lead. CaNa2EDTA is another chelating agent that chelates lead that has taken the place of calcium and promotes its urinary excretion. CaNa2EDTA was not available initially, so the infant received oral succimer 350 mg/m2 every 8 hours as monotherapy. On the sixth day after birth, CaNa2EDTA was started at 1,000 mg/m2 per day as a continuous infusion for a total of 5 days. A BLL measurement before the initiation of CaNa2EDTA revealed that the lead level had decreased from 76 μg/dL (3.7 μmol/L) to 48 μg/dL (2.3 μmol/L; 37% reduction) while receiving succimer monotherapy. On completion of dual therapy, oral succimer was continued at two-thirds of the initial dose to complete a 19-day course. BLL was 17 μg/dL (0.8 μmol/L) on day 11 after birth. A test for glucose-6-phosphate dehydrogenase deficiency was normal. A measure of lead’s toxic effect, the blood zinc protoporphyrin, was elevated at 274 μg/dL (4.9 μmol/L). Iron studies revealed ferritin level of 431 ng/dL (968 pmol/L); iron, 37 μg/dL (6.6 μmol/L); transferrin, 195 mg/dL (24 μmol/L); total iron-binding capacity, 244 μg/dL (43.7); and saturation, 15%.The infant’s condition was monitored with daily bilirubin levels, basic metabolic panels, and urinalyses, as well as biweekly liver function tests to monitor for medication tolerance and side effects. On the 2nd day of treatment with succimer, he developed electrolyte abnormalities of hyperphosphatemia, hyponatremia, hypocalcemia, and hypomagnesemia, for which he received oral supplementation of these elements and his formula was changed to a low phosphorus formulation to reduce phosphate intake. At the time of discharge, these electrolyte abnormalities had resolved and the infant was back to feeding standard term formula. Chelation was well tolerated by the neonate. Breastfeeding continued to be avoided because maternal BLL was elevated to more than 40 μg/dL (1.9 μmol/L).The BLL rebounded to 28 μg/dL (1.3 μmol/L) on day 22 after birth (day 20 of succimer). The patient was discharged from the hospital to complete the course of succimer as an outpatient.Current guidelines from the CDC and the NYC DOHMH recommend dual drug therapy for treating children with a BLL greater than or equal to 70 μg/dL (3.3 μmol/L). (1)(2) However, the infant described herein was first treated solely with succimer because CaNa2EDTA was unavailable. Because of the high price of CaNa2EDTA, the treating institution and 14 surrounding hospitals no longer provided this option. However, monotherapy with succimer has been used to successfully treat moderate to severe lead poisoning in children in resource-poor countries and did provide some benefit in this case. (3)The fetus is at risk of lead toxicity from the mother because of the passage of lead across the placenta. (4) After birth, the main portals of entry into the body are via ingestion or inhalation and, very rarely, transdermally. Lead is primarily excreted through the gastrointestinal and urinary systems. Nonexcreted lead primarily accumulates in the bone, but is distributed to all tissues in the body, affecting the hematologic, renal, and neurologic systems.Lead toxicity occurs by interference with the function of metalloproteins when it substitutes for essential metals such as calcium, iron, and zinc. Its effect on the developing nervous systems is believed to occur through interference with neurotransmission at the synapse (dopamine and acetylcholine) and interference with cell adhesion molecules. In disrupting the intercellular junctions, it increases permeability, leading to a weaker blood-brain barrier. This increases intracranial fluid accumulation and intracranial pressure, which eventually leads to tissue ischemia and atrophy. (5) In bones, lead alters the activity of osteoblasts through calcium-binding protein synthesis as well as vascularization of new bone and cartilage mineralization. (4) In the kidneys, it particularly affects the proximal tubules, resulting in proteinuria, glycosuria, and decreased calcitriol synthesis, thereby further impairing calcium absorption. Lead is a potent inhibitor of multiple enzymes in the heme synthesis pathway, resulting in the accumulation of potentially toxic precursors and leading to normocytic and microcytic variants of anemia. (4)Lead poisoning is often diagnosed with the finding of an elevated BLL. A complete blood cell count should be checked to assess for anemia. Checking for levels of erythrocyte protoporphyrin is a pertinent test for severe lead toxicity; levels generally begin to rise when the BLL is more than 20 μg/dL. Other causes of an elevated protoporphyrin level include iron deficiency and inflammatory disease. Ferritin levels should also be checked to assess for adequate iron stores. Long bone radiography may reveal dense lines at the metaphysis after prolonged or chronic exposure, but such studies are rarely needed for diagnostic purposes.Of the 4 steps for lead treatment, the first 3 are universally applicable: 1) eliminate the source of exposure; 2) stop the pathway into the body; 3) maximize optimal nutrition of essential metals especially calcium and iron. Iron deficiency is associated with increased lead absorption, retention, and toxicity. Both iron deficiency and lead toxicity affect brain development independently and the combination may be synergistic. Moreover, iron-deficient children excrete less lead in response to a chelating agent for any given BLL compared with iron-sufficient children. However, dimercaprol, which is a congener of succimer, forms a toxic chelate with iron; therefore, iron therapy should be avoided during chelation with dimercaprol. Presumably, decreased lead may be excreted when succimer and iron treatment are given concomitantly.For a fetus, treatment requires reducing maternal BLL or inducing delivery of the infant. Chelation therapy is the fourth step and is reserved for children with BLLs greater than or equal to 45 μg/dL (2.1 μmol/L). The 3 most commonly used chelation drugs in the United States are succimer, CaNa2EDTA, and dimercaprol. A fourth, weak chelating agent, penicillamine, is used in resource-poor countries when the preferred drugs are unavailable.Succimer is administered orally and is the initial drug of choice. CaNa2EDTA can be administered as an infusion or intramuscular injection, and requires careful monitoring for subsequent renal impairment and hypercalcemia. It can be added to succimer for children with BLLs greater than or equal to 70 μg/dL (3.4 μmol/L) or as a substitute for succimer when it is not tolerated. Dimercaprol can only be given intramuscularly. It is more toxic than the other drugs and has fallen into disuse. In the present case, only 1 agent was initially available and the decision to proceed was predicated on concern for ongoing toxicity from such high BLLs and any delay in therapy. Three days of solo therapy reduced the BLL significantly. A greater reduction in BLL was subsequently achieved during dual drug treatment. A rebound in BLL was evident soon after completion of the course of CaNa2EDTA while the infant was still receiving succimer, perhaps indicating that bone lead stores were still substantial. Furthermore, we did not identify a cause for the electrolyte abnormalities because they are not normally associated with either lead poisoning or chelation in the absence of renal disease.Some women develop behaviors related to pica during pregnancy. Although the cause for this behavior is not known for certain, it is speculated that it may be related to iron deficiency as the body attempts to obtain vitamins or minerals that are missing from the mother’s diet. In other instances, it may even be related to physical or mental illness. In New York State, obstetrics providers are mandated by the department of health to perform a risk assessment for lead exposure in pregnant women. It is the provider’s choice whether to use a risk questionnaire or to proceed directly to BLL measurement. A positive risk assessment should also result in a BLL measurement. For children, as of 2012, the CDC has determined that a BLL greater than or equal to 5 μg/dL (0.24 μmol/L) is the reference level for identifying the 2.5% of children younger than 5 years with the highest BLLs. It is not a toxicologic threshold; that has yet to be discovered. Rather, it is the point at which primary care physicians are encouraged to assess for sources of exposure, give guidance to prevent lead poisoning progression, and initiate treatment intervention. It is important to note that chelation therapy does not reverse the cognitive effects of toxicity. As the pregnant woman is carrying a potential child, it is reasonable to use the pediatric standard in the interpretation of maternal BLLs and the approach to treatment.

  PublisherDOI

• Case 2: Neonate with Cholestasis, Thrombocytopenia, and Hypoglycemia

  NeoReviews · 2016-07-01 · 2 citations

  article1st authorCorresponding

  A male infant is born at 36 4/7 weeks’ gestation to a 23-year-old gravida 1, para 0 woman via spontaneous vaginal delivery. He is admitted to the intensive care nursery for growth restriction, hypoglycemia, and thrombocytopenia. The prenatal history is unremarkable. Routine resuscitation is performed in the delivery room. Apgar scores are 8 and 9 at 1 and 5 minutes, respectively. The admission weight is 1.67 kg (0.3 percentile), length 43.0 cm (3.1 percentile), and head circumference 30.0 cm (4.4 percentile). Vital signs at admission and physical examination findings are within normal limits.Admission laboratory values are remarkable for a platelet count of 25 × 103/μL (25 × 109/L), with the remainder of the complete blood cell count within normal limits. Initial glucose concentration by dextrose stick is 20 mg/dL (1.11 mmol/L).By day 2, the infant requires 20% dextrose for persistent hypoglycemia and 2 platelet transfusions. Laboratory tests reveal a total bilirubin of 11.4 mg/dL (195 μmol/L), direct bilirubin of 6.3 mg/dL (108 μmol/L), and aspartate aminotransferase of 107 U/L (1.8 μkat/L). C-reactive protein is elevated, at 4.5 mg/L (42.8 nmol/L). Significant coagulopathy is noted, with an international normalized ratio of 10 on day 3, and the infant receives additional doses of vitamin K, cryoprecipitate, and fresh frozen plasma. Given this early presentation of liver disease, intravenous immunoglobulin is administered, with a consideration of gestational alloimmune liver disease (GALD). Salivary gland biopsy does not demonstrate any evidence of siderosis seen in neonatal hemochromatosis, which is typically associated with GALD. Head ultrasonography shows a wedge-shaped area of increased echogenicity in the right parietal-occipital region representing infarction versus ischemia. Cultures for toxoplasmosis, other agents, rubella, cytomegalovirus, and herpes simplex (TORCH) syndrome, blood, and cerebrospinal fluid are negative. He receives ampicillin and gentamicin for 5 days for possible infection.Liver ultrasonography (Figs 1 and 2) on day 3 reveals dilated hepatic and portal veins with increased pulsatility that is thought to be secondary to a hepatic arterial to portal venous fistula. Magnetic resonance (MR) imaging of the abdomen confirms findings of enlarged celiac artery and hepatic artery, with prominent hepatic veins, most suggestive of a congenital intrahepatic arterioportal fistula (IAPF) in the left hepatic lobe.The infant requires intravenous dextrose with continuous feeds to maintain euglycemic levels until day 19. Angiography and endovascular occlusion of the fistula for treatment are considered but delayed due to significant clinical improvement of the infant. The infant is discharged from the hospital on day 37 with mild, improving cholestasis. He is tolerating feeds and gaining weight without evidence of heart failure or pulmonary hypertension.On outpatient follow-up, the infant undergoes MR venography/MR arteriography imaging of his abdomen, which demonstrates stable, unchanged evidence of the hepatic artery to left portal vein fistula. At 3 months of age, the cholestasis resolves. At that time, he has normal hepatic function, normal growth, and spontaneous resolution of the fistula on follow-up ultrasonography.Pediatric IAPF is a rare anomaly that typically presents before 18 years of age. Congenital arterioportal fistulas are an intrahepatic communication between the hepatic artery and portal venous system without connection with the systemic venous circulation. (1) There are 3 types of IAPFs: type 1 unilateral lesions involve only 1 of the hepatic arteries, type 2 bilateral lesions involve both of the hepatic arteries, and type 3 complex lesions involve at least 1 hepatic artery as well as another nonhepatic artery. (2)The median age at presentation is 3 years. (2) In older children, the most common presentations include upper gastrointestinal bleeding, failure to thrive, abdominal pain, and chronic diarrhea or steatorrhea. (3) Patients can also present with neurologic symptoms or hepatic encephalopathy with elevated ammonia levels. Dyspnea may be present due to pulmonary hypertension. Pulmonary hypertension occurs when vasoactive substances bypass normal liver metabolism and cause vasoconstriction of the small pulmonary arteries. (4) Pediatric IAPF may also present with hypoglycemia due to defective liver uptake of glucose and reduced or impaired hepatic degradation of normal insulin levels. (4) Despite the presence of an arteriovenous anastomosis with IAPF, congestive heart failure is rare, possibly related to the protective effect of the hepatic sinusoids interposed between the fistula and the right heart cavities. (1)Additional associated malformations are more common in extrahepatic shunts. IAPF has been reported with atrial septal defect, ventricular septal defect, patent ductus arteriosus, coarctation of aorta, and dextrocardia. Other associated findings include polysplenia, biliary atresia, skeletal abnormalities, and renal tract abnormalities. (4)Ultrasonography with Doppler flow is the first-line imaging modality. Hepatic angiography confirms the diagnosis and provides a more exact estimation of size and location before intervention.Therapy depends on the fistula location and severity of the symptoms. Percutaneous transarterial embolization is usually the preferred method of action for most children with noncomplex congenital IAPF and is usually curative. Surgical approaches are considered if the fistula persists or the patient has a complex congenital IAPF. Sometimes, multiple interventions are needed for complete elimination of the fistula. Routine follow-up with ultrasonography is required to verify resolution of flow through the fistula. In addition, complex congenital IAPFs are prone to collateralization or recurrence after radiologic intervention. (3)Spontaneous resolution of the fistula, as seen in the current patient, is unusual.Most children become asymptomatic, with resolution of symptoms with intervention. Morbidity is usually avoided with early diagnosis. If left unrecognized and untreated, IAPF can lead to life-threatening portal hypertension.

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Frequent coauthors

• Omer Choudry

  Rutgers, The State University of New Jersey

  10 shared

• Onajovwe Fofah

  Rutgers, The State University of New Jersey

  8 shared

• Thomas Havranek

  Children's Hospital at Montefiore

  6 shared

• Mehrin Sadiq

  6 shared

• Arun Kumar

  George Institute for Global Health

  6 shared

• Olga Sudol

  Rutgers, The State University of New Jersey

  4 shared

• Kyrillos Attaalla

  Rutgers New Jersey Medical School

  4 shared

• Elaine M. Pereira

  Columbia University

  4 shared

Education

• Medical Doctor

  Rutgers New Jersey Medical School