Ingestions
A previously healthy 3-year old female swallowed a quarter, which settled in the gastric antrum. The coin was retrieved by endoscopy as it did not pass beyond the pylorus. Coins smaller than a quarter typically pass through the intestinal tract without difficulty. Another 3-year old child swallowed a screw, which was located in the stomach on admission. It was not located via endoscopy and passed through the intestinal tract without complication. Sharp objects, such as small safety pins, are often retrieved more for parental anxiety than documented risks. Sharp objects longer than 1.5 inches however, should be retrieved.

Medication Overdose
A 17-year old female with a history of depression ingested up to 10 isoniazid tablets. She had two brief, self resolving seizures and was treated with pyridoxine to reverse the neurotoxicity of isoniazid. She recovered uneventfully and was discharged home with close follow up with her primary physician and the psychiatrist. Poison control was notified and assisted in the case.

3-year old Girl with Acute Mental Status Change
A previously healthy 3-year old female was transferred from an outside ED for further evaluation and management of her altered mental status. The mother and her boyfriend stated that the patient was in her usual state of good health the morning of admission. The mother’s boyfriend took care of her during the day and reported that she ate an early breakfast and had a muffin later that morning. She took a brief nap and awoke with notable grogginess, ataxia and drooping eyes. She ate lunch and another muffin. She continued to be slightly disoriented and unsteady on her feet, but was able to make eye contact and respond appropriately to questions. Her mother returned home later that evening and noted the mental status change and brought her to the nearest ED.

Her triage vital signs were stable and work-up of her altered mental status included blood, urine, and CSF testing, as well as a head CT. All tests were normal except for urine toxicology screen was significant for marijuana ingestion. Upon further history, the mother reported that the family recently hosted some out-of-town friends. The friends left behind some marijuana-filled muffins which the child retrieved from the container herself. The boyfriend smelled the muffins after the positive urine toxicology results were known. The toddler recovered completely and remained in the hospital until the CPS evaluation was completed. She was safely discharged home with close follow up with her primary physician.

Impressive Oncological Presentation
A 17-year old boy presented to his primary physician with a chief complaint of a left buttock mass. He was referred to the ED for further evaluation of this buttock mass. A CT scan at the outside ED indicated multiple masses, and a grossly elevated WBC of 452,000. The ED physician unsuccessfully attempted to admit the patient to the adult oncology service. As there is not a pediatric oncology service at the outside hospital, the patient was discharged home from the ED to follow up with his primary physician and await a referral to a pediatric oncologist. The patient’s physician referred him to our PICU the following day upon learning of his WBC. The young man took the bus and walked into the PICU and complained only of a mild headache and his buttock mass.

Further history revealed an unintentional 60 pound weight loss over the last 9 months. He noted early satiety but did not notice that his abdomen was getting larger or firmer. He denied fevers, night sweats, abdominal pain or bone pain. He also denied blurry vision, dizziness, paresthesias, weakness or mental status changes. He had mild fatigue and a one-week history of worsening morning headaches. He also had a one-month history of skin nodules on his upper extremity.

His WBC increased from 452,000 to 1.88 million upon admission to the PICU. A large groin central line was placed and the patient received leukopheresis therapy to remove some of the excess white blood cells. A bone marrow biopsy and lumbar puncture indicated chronic myelogenous leukemia with the BCR/ABL mutation and CNS involvement. The buttock mass was biopsied and revealed a leukemic chloroma. He was started on chemotherapy and had an excellent response with minimal tumor lysis syndrome, despite the initial quantity of leukemic cells. He was discharged home with close follow-up with his primary physician and the pediatric oncologist. He was also referred to the pediatric bone marrow transplant team at UCSF.

Bitten by the Family Pet
An otherwise healthy 3 -year old female was bitten by the family’s pet Labrador when she was trying to teach the dog to sit. Her mother applied localized first aid and brought the child to her physician the following morning when the wound appeared pururlent. The wound was cultured and the patient was prescribed amoxicillin/clavulanate and then discharged home. The patient developed fevers with an expanding, erythematous wound. She was therefore referred to the Pediatric Ward for parenteral antibiotics. She improved significantly and remained afebrile for 24 hours. Her wound culture was notable for Pasturella multocida and the patient was transitioned back to amoxicillin/clavulante. She tolerated oral therapy and continued to improve and was discharged home with close follow-up with her pediatrician.

Severe Hemolysis and Direct Hyperbilirubinemia in a Newborn
A 2-day old 38-week female was born via SVD to 26yo G2P1-2 mom with normal prenatal labs. Delivery was precipitous and the baby was depressed at birth with apgars 4 and 7. She was admitted to the ICN for observation and continued to require supplemental oxygen for one day. However, she was noted to be jaundiced at 11 hours of life with an initial total bilirubin level of 11.3. She was started on phototherapy. At 16 hours of life, her total and direct bilirubin levels were increasing to 12.3 and 4.2, respectively. At 40 hours of life, the total and direct bilirubin levels continued to rise and the hematocrit was noted to be 27, with a reticulocyte count of 33% and LDH of 2075. Maternal and infant blood types were both O+, and the Kleihaur-Betke and Coombs tests were both negative. There was no reported birth trauma, abruption or other bleeding disorder. Head and abdominal ultrasounds were normal. The baby appeared clinically well and was feeding vigorously.

The patient was referred to our service for further evaluation and management of her direct hyperbilirubinemia and severe hemolysis. The differential diagnosis included: minor antigen incompatibility, G6PD deficiency, hereditary spherocytosis, hemoglobinopathy, pyruvate kinase deficiency, hypothyroidism, hemochromatosis, hemophagocytic lymphohistiocytosis, sepsis, and TORCH infections. Obstructive conditions such as biliary atresia was also included in the differential diagnosis, but considered less likely given her early presentation and associated hemolysis. The patient’s father also had a history of neonatal jaundice and anemia requiring blood transfusion, but has been healthy since. Laboratory tests to evaluate the parents were all normal.

The patient continued to hemolyze briskly over the next several days, with a stable bilirubin level despite intensive phototherapy and excellent oral intake. She required a PRBC transfusion on day of life 5 for her continued hemolysis. The hemolysis stabilized after the transfusion, and both the total and direct bilrubin levels declined to 11 and 3, respectively. There was no significant rebound. She remained stable and was discharged home to complete an outpatient evaluation. She was discharged home with ADEK vitamins, ursodiol to help her excrete the direct bilirubin, and folate to help her replete her red blood cells. She had scheduled close follow up with her pediatrician and the pediatric hematologist. She was later diagnosed with pyruvate kinase deficiency.

Unusual Presentation of Kawasaki’s Disease
A 6-year old boy presented to an outside ED with a 5-day history of worsening neck pain, stiffness, throat pain and tactile temperatures. He also had a 2-day history of a pruritic rash that began around his knees and then spread to his lower extremities, groin, trunk and upper extremities. The patient was given acetaminophen as needed for his tactile temperatures at home. He was also applying caladryl lotion for his rash. A CT scan of the neck was obtained given his history of throat pain and difficulty with opening his mouth. A 1 x 5 cm retropharyngeal phlegmon was found on the CT scan and the patient was referred to the Pediatric Unit for further evaluation and management of his presumed retropharyngeal abscess. His admission examination was significant for a non-exudative, bilateral conjunctivitis, pronounced strawberry tongue, erythematous and fissured lips, unilateral cervical adenopathy. His findings were consistent with Kawasaki’s disease. The patient was initially treated with parenteral antibiotics for his retropharyngeal abscess but he remained febrile and did not clinically improve. Pediatric infectious disease was consulted and a literature search revealed several case reports of Kawasaki’s disease presenting with a retropharyngeal abscess. The antibiotics were discontinued on his second hospital day and the patient was treated with IVIG. The patient had an excellent response to the IVIG and did not have any coronary aneurysms on his echocardiogram. He was discharged home with close follow up with his primary physician and the cardiologist.

Overview
Adrenal insufficiency can be the result of primary, secondary, or tertiary causes. Disease intrinsic to the adrenal cortex is classified as primary adrenal insufficiency. Defective adrenocorticotropic hormone (ACTH) release or effect from the pituitary gland is classified as secondary adrenal insufficiency. Defective corticotropin releasing factor (CRF) secretion or action from the hypothalamus results in tertiary adrenal insufficiency.

Presentation
The adrenal cortex synthesizes glucocorticoids, mineralcorticoids and adrenal androgens. The clinical findings depend on the severity of the hormone class involved. Children often present with fatigue, nausea, and vomiting. Glucocorticoid deficiency manifests with fasting hypoglycemia, muscle weakness, increased insulin sensitivity, and morning headache. Hyperpigmentation as a consequence of increased pro-opiomelanocortin (ACTH precursor) production can be noted in the axillae, palmar creases, gingiva, elbows, and knees. Signs and symptoms of sodium loss are consistent with mineralcorticoid deficiency. The clinical findings include hypotension, dizziness, salt-craving, anorexia, weight loss, hyponatremia, hyperkalemia, and metabolic acidosis. Pubertal females with adrenal androgen deficiency present with decreased libido, axillary, and pubic hair. Pubertal males produce the majority of their androgens in the testes and therefore remain asymptomatic.

Acute adrenal insufficiency can result in an adrenal crisis. This syndrome manifests in children with hypotension due to the mineralcorticoid deficiency. In extreme situations, progressively fatal shock may occur. The primary adrenal insufficiency leading to the adrenal crisis can result from congenital or acquired etiologies or to inappropriate replacement therapy in patients with diagnosed adrenal insufficiency.

Neonates with classic congenital adrenal hyperplasia, most commonly from 21-hydroxylase deficiency, present within the first few days to weeks of life with an adrenal crisis. The clinical presentation includes vomiting, diarrhea, hyponatremia, hyperkalemia, hypoglycemia, hypovolemia and hypotension. Female neonates exhibit ambiguous genitalia. Infants and older children with adrenal crisis develop weight loss, hyponatremia, hyperkalemia, and hyperpigmentation, often following an antecedant precipitating stress.

Bilateral adrenal hemorrhage can occur with septic shock or anticoagulation therapy. The adrenal crisis presents with shock unresponsive to vigorous fluid resuscitation and inotropic support. Additionally, there is significant hyponatremia and hyperkalemia.

Treatment
The treatment for primary adrenal insufficiency includes glucocorticoid and mineralcorticoid replacement therapy. Hydrocortisone is a low potency, short acting glucocorticoid often used in infants and children. The daily oral dose is double the normal daily cortisol secretion rate because of its short half-life and partial degradation by stomach acids. Hydrocortisone can by administered every eight hours. Prednisone or pednisolone is an alternative choice and can be prescribed every 12 hours because of its longer half-life and increased potency. The glucocorticoid requirements relative to body surface area are greatest during infancy and puberty. The physiologic replacement dosage therefore needs to be adjusted to the child’s somatic growth. Children with excessive glucocorticoid replacement will exhibit weight gain, decreased height velocity, and signs or symptoms of Cushing’s syndrome.

The glucocorticoid dosage should be increased during acute, stressful conditions. The dosage may need to be doubled during a moderate illness or increased up to four times the normal dosage during a severe illness. If the child is unable to tolerate oral therapy during a severe illness, then an intramuscular injection of hydrocortisone can provide six hours or coverage while the family seeks medical attention. It is recommended that the patient wear a medical alert bracelet indicating the diagnosis of adrenal insufficiency.

The glucocorticoid requirement will also increase with general anesthesia and surgical procedures. Patients undergoing a surgical procedure that lasts less than 45 minutes are given an IV bolus of hydrocortisone along with a continuous IV infusion. The stress dose is rapidly tapered as the patient improves. Surgeries lasting longer than 45 minutes may require a rapid bolus of hydrocortisone prior to anesthesia followed by a 24 hour continuous IV infusion if the patient is unable to tolerate oral intake. High dosage replacement therapy for the next few days will provide both glucocorticoid and mineralcoticoid activity. The stress dosage is continued and slowly tapered until the patient is afebrile, hemodynamically stable, and able to tolerate oral intake.

Mineralcorticoid replacement therapy with 9-alpha-fluorocortisol is also required in primary adrenal insufficiency. As aldolsterone secretion is fairly constant throughout life, the mineralcorticoid dosage does not need to be adjusted by age or body surface area. However, infants younger than 12 months of age may require additional sodium chloride supplementation due to the low sodium content in breastmilk and formula. Dehydration, salt-craving, failure to thrive, hyponatremia and hyperkalemia are some findings consistent with insufficient mineralcorticoid replacement.

Ingestions
A previously healthy 3-year old female swallowed a quarter, which settled in the gastric antrum. The coin was retrieved by endoscopy as it did not pass beyond the pylorus. Coins smaller than a quarter typically pass through the intestinal tract without difficulty. Another 3-year old child swallowed a screw, which was located in the stomach on admission. It was not located via endoscopy and passed through the intestinal tract without complication. Sharp objects, such as small safety pins, are often retrieved more for parental anxiety than documented risks. Sharp objects longer than 1.5 inches however, should be retrieved.

Medication Overdose
A 17-year old female with a history of depression ingested up to 10 isoniazid tablets. She had two brief, self resolving seizures and was treated with pyridoxine to reverse the neurotoxicity of isoniazid. She recovered uneventfully and was discharged home with close follow up with her primary physician and the psychiatrist. Poison control was notified and assisted in the case.

3-year old Girl with Acute Mental Status Change
A previously healthy 3-year old female was transferred from an outside ED for further evaluation and management of her altered mental status. The mother and her boyfriend stated that the patient was in her usual state of good health the morning of admission. The mother’s boyfriend took care of her during the day and reported that she ate an early breakfast and had a muffin later that morning. She took a brief nap and awoke with notable grogginess, ataxia and drooping eyes. She ate lunch and another muffin. She continued to be slightly disoriented and unsteady on her feet, but was able to make eye contact and respond appropriately to questions. Her mother returned home later that evening and noted the mental status change and brought her to the nearest ED.

Her triage vital signs were stable and work-up of her altered mental status included blood, urine, and CSF testing, as well as a head CT. All tests were normal except for urine toxicology screen was significant for marijuana ingestion. Upon further history, the mother reported that the family recently hosted some out-of-town friends. The friends left behind some marijuana-filled muffins which the child retrieved from the container herself. The boyfriend smelled the muffins after the positive urine toxicology results were known. The toddler recovered completely and remained in the hospital until the CPS evaluation was completed. She was safely discharged home with close follow up with her primary physician.

Impressive Oncological Presentation
A 17-year old boy presented to his primary physician with a chief complaint of a left buttock mass. He was referred to the ED for further evaluation of this buttock mass. A CT scan at the outside ED indicated multiple masses, and a grossly elevated WBC of 452,000. The ED physician unsuccessfully attempted to admit the patient to the adult oncology service. As there is not a pediatric oncology service at the outside hospital, the patient was discharged home from the ED to follow up with his primary physician and await a referral to a pediatric oncologist. The patient’s physician referred him to our PICU the following day upon learning of his WBC. The young man took the bus and walked into the PICU and complained only of a mild headache and his buttock mass.

Further history revealed an unintentional 60 pound weight loss over the last 9 months. He noted early satiety but did not notice that his abdomen was getting larger or firmer. He denied fevers, night sweats, abdominal pain or bone pain. He also denied blurry vision, dizziness, paresthesias, weakness or mental status changes. He had mild fatigue and a one-week history of worsening morning headaches. He also had a one-month history of skin nodules on his upper extremity.

His WBC increased from 452,000 to 1.88 million upon admission to the PICU. A large groin central line was placed and the patient received leukopheresis therapy to remove some of the excess white blood cells. A bone marrow biopsy and lumbar puncture indicated chronic myelogenous leukemia with the BCR/ABL mutation and CNS involvement. The buttock mass was biopsied and revealed a leukemic chloroma. He was started on chemotherapy and had an excellent response with minimal tumor lysis syndrome, despite the initial quantity of leukemic cells. He was discharged home with close follow-up with his primary physician and the pediatric oncologist. He was also referred to the pediatric bone marrow transplant team at UCSF.

Bitten by the Family Pet
An otherwise healthy 3 -year old female was bitten by the family’s pet Labrador when she was trying to teach the dog to sit. Her mother applied localized first aid and brought the child to her physician the following morning when the wound appeared pururlent. The wound was cultured and the patient was prescribed amoxicillin/clavulanate and then discharged home. The patient developed fevers with an expanding, erythematous wound. She was therefore referred to the Pediatric Ward for parenteral antibiotics. She improved significantly and remained afebrile for 24 hours. Her wound culture was notable for Pasturella multocida and the patient was transitioned back to amoxicillin/clavulante. She tolerated oral therapy and continued to improve and was discharged home with close follow-up with her pediatrician.

Severe Hemolysis and Direct Hyperbilirubinemia in a Newborn
A 2-day old 38-week female was born via SVD to 26yo G2P1-2 mom with normal prenatal labs. Delivery was precipitous and the baby was depressed at birth with apgars 4 and 7. She was admitted to the ICN for observation and continued to require supplemental oxygen for one day. However, she was noted to be jaundiced at 11 hours of life with an initial total bilirubin level of 11.3. She was started on phototherapy. At 16 hours of life, her total and direct bilirubin levels were increasing to 12.3 and 4.2, respectively. At 40 hours of life, the total and direct bilirubin levels continued to rise and the hematocrit was noted to be 27, with a reticulocyte count of 33% and LDH of 2075. Maternal and infant blood types were both O+, and the Kleihaur-Betke and Coombs tests were both negative. There was no reported birth trauma, abruption or other bleeding disorder. Head and abdominal ultrasounds were normal. The baby appeared clinically well and was feeding vigorously.

The patient was referred to our service for further evaluation and management of her direct hyperbilirubinemia and severe hemolysis. The differential diagnosis included: minor antigen incompatibility, G6PD deficiency, hereditary spherocytosis, hemoglobinopathy, pyruvate kinase deficiency, hypothyroidism, hemochromatosis, hemophagocytic lymphohistiocytosis, sepsis, and TORCH infections. Obstructive conditions such as biliary atresia was also included in the differential diagnosis, but considered less likely given her early presentation and associated hemolysis. The patient’s father also had a history of neonatal jaundice and anemia requiring blood transfusion, but has been healthy since. Laboratory tests to evaluate the parents were all normal.

The patient continued to hemolyze briskly over the next several days, with a stable bilirubin level despite intensive phototherapy and excellent oral intake. She required a PRBC transfusion on day of life 5 for her continued hemolysis. The hemolysis stabilized after the transfusion, and both the total and direct bilrubin levels declined to 11 and 3, respectively. There was no significant rebound. She remained stable and was discharged home to complete an outpatient evaluation. She was discharged home with ADEK vitamins, ursodiol to help her excrete the direct bilirubin, and folate to help her replete her red blood cells. She had scheduled close follow up with her pediatrician and the pediatric hematologist. She was later diagnosed with pyruvate kinase deficiency.

Unusual Presentation of Kawasaki’s Disease
A 6-year old boy presented to an outside ED with a 5-day history of worsening neck pain, stiffness, throat pain and tactile temperatures. He also had a 2-day history of a pruritic rash that began around his knees and then spread to his lower extremities, groin, trunk and upper extremities. The patient was given acetaminophen as needed for his tactile temperatures at home. He was also applying caladryl lotion for his rash. A CT scan of the neck was obtained given his history of throat pain and difficulty with opening his mouth. A 1 x 5 cm retropharyngeal phlegmon was found on the CT scan and the patient was referred to the Pediatric Unit for further evaluation and management of his presumed retropharyngeal abscess. His admission examination was significant for a non-exudative, bilateral conjunctivitis, pronounced strawberry tongue, erythematous and fissured lips, unilateral cervical adenopathy. His findings were consistent with Kawasaki’s disease. The patient was initially treated with parenteral antibiotics for his retropharyngeal abscess but he remained febrile and did not clinically improve. Pediatric infectious disease was consulted and a literature search revealed several case reports of Kawasaki’s disease presenting with a retropharyngeal abscess. The antibiotics were discontinued on his second hospital day and the patient was treated with IVIG. The patient had an excellent response to the IVIG and did not have any coronary aneurysms on his echocardiogram. He was discharged home with close follow up with his primary physician and the cardiologist.