Newborn infant with lethargy, poor feeding, dehydration, hypothermia, hyperammonemia, neutropenia, and thrombocytopenia

Abstract

History on admission. A.P. was a 10-day-old Hispanic girl, admitted to the hospital because of a 3-day history of lethargy and increased sleeping. For one day, there was also inadequate feeding and low body temperature. There was no history of irritability or crying episodes, runny nose, cough, respiratory distress, apnea, or cyanosis. Vomiting and diarrhea were not present and there was no history of bloody stools. The mother stated that the infant was apparently voiding well.The infant was born at term, after an uneventful pregnancy, with a normal spontaneous vaginal delivery. Apgar scores were 8 at 1 minute and 9 at 5 minutes. Birth weight was 3.2 kg, length 47 cm, and head circumference 34 cm. Examination in the nursery on admission and on discharge revealed no morphological anomalies. Mother's rapid plasma reagin (RPR) and human immunodeficiency virus (HIV) were nonreactive, and there was no history of alcohol or other drug use. The infant was Type O+, and Coombs test was nonreactive. Mother and infant were discharged after two days. There were no pets at home. The mother was a smoker. There were three sibs, ages 9, 3, and 1 and 3/12 years. Family history was unremarkable.Before admission, the patient was receiving a diet of Similac 20 with iron, 2-3 ounces every three hours, which she accepted well until the day prior to admission.Physical examination on admission. Weight was 3.14 kg; temperature 35°C; pulse 112; respirations 20; and BP 114/63 mmHg. The infant was in no acute distress, was sleeping but arousable. The anterior fontanel was open and wide. The pharynx was normal, with no evidence of oral candida. Ear drums were normal. Pupils were equal and reacted to light and accommodation. Lungs were clear to percussion and auscultation, and there was no respiratory distress. The heart had a regular rate and rhythm and there were no murmurs. The abdomen was soft; no masses were palpable; the umbilicus was still crusted, but dry. Genitalia were normal for age. No skin rashes or petechiae were noted. Reflexes were present and normal.Laboratory findings on admission. Findings are shown in Tables I and II. The patient was dehydrated, leukopenic, and thrombopenic. A spinal tap was bloody (Table III).Course in hospital. On admission to the PICU, the patient was rehydrated intravenously. After culture of blood for sepsis, she was treated empirically with ampicillin and gentamicin. Her condition improved, and she was started on infant formula on day 3. Within 12 hours, she became hypothermic, hypotensive, and acidotic. She was given IV fluids and transfused with blood and platelets. Platelet count again fell rapidly.Her condition remained poor. She was poorly rousable, arid, and had poor Moro and sucking reflexes. Blood ammonia was 385 UM/L on day 7. Immunoglobulins (Table III) were normal. Serum free and total carnitine were reduced. Urinary carnitine esters were 80% of total. Additional studies were ordered.A bone marrow examination carried out because of the neutropenia and thrombocytopenia showed arrest of myel...

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“…They also are the precursors of catecholamines in the body (tyramine, dopamine, epinephrine and norepinephrine). Additionally, a previous study has reported the increased amount of tyrosine in the blood of premature infants, which is marked by a decreased motor activity, lethargy and poor feeding (Elfenbein, Barness, Pomerance, & Barness, ). Phenylalanine also is the precursor of tyrosine.…”

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