A 7-month old male infant arrived in the ED at 7:45 a.m. after passing a dark maroon colored stool with clots at 7:00 a.m. He has passed small amounts of blood in his stools intermittently since 4 months of age, but this current episode is the worst it has ever been.
He has been taking amoxicillin-clavulinic acid for the past 7 days for otitis media. He also has some nasal congestion and coughing. He passed a normal stool at 4:00 a.m. earlier. His past medical history is unremarkable except for the intermittent blood in his stool (small amounts).
Stool cultures and examinations for ova and parasites done in the past as an outpatient were negative. His growth has been good. His development has been normal. His diet consists of soy formula, baby foods, and table foods.
T37.0 (tympanic), P192, R40, BP 154/128, oxygen saturation 94-95% in room air. Alert, crying, in no distress. Skin warm and dry. Color pink. Anterior fontanelle soft and flat. Pupils reactive. TM’s normal. Oral mucosa moist. Throat clear. Neck supple. Heart tachycardic, regular, without murmurs. Lungs good aeration, clear.
Abdomen soft, slightly distended, non-tender, bowel sounds active, no masses, no hepatosplenomegaly. Peripheral pulses good. Capillary refill time 2 seconds. Excoriation noted over the perianal region. Muscle tone good. Moves all extremities well.
Stool is guaiac positive.
Laboratory studies: CBC WBC 10,900, 11 bands, 32 segs, 51 lymphs, 2 monos, 3 eos, Hgb 11.4, Hct 34.6, platelet count 176,000. Prothrombin time and partial thromboplastin times are normal.
Urinalysis specific gravity 1.020, pH 6, 3-5 WBC’s and 3-5 RBC’s per high power field.
An IV is started and a clot tube for blood type and screening for possible transfusion is sent to the lab. An abdominal series is ordered.
His repeat vital signs at 8:35 a.m. show P172, R40, BP 141/100. He passes two more bloody stools in the ED which appear to resemble currant jelly (blood mixed with mucus somewhat resembling strawberry jelly without the seeds).
A nasogastric tube is placed. An aspirate of his gastric contents is guaiac negative.
At 9:40 a.m. his blood pressure falls. He passes more blood per rectum. He is transfused with packed RBC’s. After stabilization, a water soluble contrast enema is performed to rule out intussusception. This study is negative.
In reviewing his abdominal series, the abdomen portion of the study is non specific. However, the chest portion of the upright abdomen is positive for interstitial infiltrates.
In retrospectively reviewing his vital signs, his oxygen saturation of 94-95% in room air before he developed hemodynamic decompensation is suggestive of a significant ventilation perfusion mismatch suggesting some type of pulmonary condition. Reactive airway disease is the most common cause of this, but since wheezing was not noted on examination, an occult pneumonia should be suspected. Most infants with normal lungs should have an oxygen saturation of 98-100% in room air. This is especially true of young infants less than 3 months of age who almost always have an oxygen saturation of 100% in room air. Anything less than 98% should be viewed with some suspicion in this age group.
This interstitial pneumonia was appreciated in the ED, however, the oxygen saturation rose to 100% with some blowby oxygen. The hemodynamic stabilization seemed to have a greater priority at the time. After stabilization in the intensive care unit, he was placed on cimetadine and IV fluids. A Meckel’s diverticulum was suspected. His pneumonia was felt to be viral in etiology and he was not placed on any antibiotics. His oxygen saturation stabilized at 99% with 1 liter per minute of oxygen by nasal cannula. After multiple consultants became involved in his care, he was started on antibiotics.
A nuclear medicine Meckel’s scan was negative. Endoscopy and colonoscopy were both negative. He continued to bleed intermittently, requiring additional transfusions of RBC’s and platelets. Stool, blood, and urine cultures were negative. He underwent an exploratory laparotomy. Frozen section examination of intestinal biopsies were positive for viral inclusion bodies, suggesting cytomegalovirus enteritis.
Probable severe CMV infection and the interstitial pneumonia raised the possibility of HIV infection. His mother was not known to be HIV positive nor at high risk for HIV. An HIV ELISA study was positive and an HIV p24 antigen assay was also positive. He underwent extensive treatment with gamma globulin, anti-viral agents, antibiotics, and intensive care support.
He eventually developed respiratory insufficiency and expired 15 days after presenting to the ED The classification of HIV is very detailed ranging from asymptomatic HIV infection to AIDS associated manifestations including specific secondary infections, recurrent serious bacterial infections, progressive neurologic disease, lymphoid interstitial pneumonitis, specific secondary malignancies (lymphomas and Kaposi sarcoma), and HIV wasting syndrome. The details of this classification are beyond the scope of this case. Manifestations of HIV infection in children are extensive. They are summarized here briefly by organ system.
Systemic manifestations include failure to thrive and recurrent or chronic fever. The failure to thrive is usually due to reduced caloric intake secondary to anorexia and acclerated energy expended in fighting chronic infection. Examination findings include hepatosplenomegaly, lymphadenopathy, and dermatitis.
Cardiac manifestations are rarely the presenting finding in a new patient. Cardiomyopathy, conduction abnormalities, and congestive heart failure may result directly from HIV or from other opportunistic viral infections.
Pulmonary manifestations can be categorized as infectious and non-infectious. Recurrent bacterial pneumonia, pneumocystis carinii pneumonia (PCP), viral pneumonia (including CMV), mycobacterium avium complex infections, and mycobacterium tuberculosis are some of the common pulmonary infections. Tuberculosis infections are often highly drug-resistant. Pneumocystis carinii pneumonia (PCP) is a diffuse, desquamative, interstitial infection resulting in hypoxemia, fever, productive cough, respiratory distress, and poor exercise tolerance. PCP and Mycobacterium avium complex disease are two of the more severe infections affecting the later stages of HIV infection. Reactive airway disease is common among all immunocompromised children, probably due to chronic airway inflammation due to recurrent or chronic infections. Lymphoid interstitial pneumonitis is the most common noninfectious pulmonary condition seen in pediatric HIV infection. This is characterized by diffuse interstitial infiltrates. Pulmonary fibrosis is also seen with HIV. Children with advanced HIV encephalopathies may develop aspiration pneumonia.
GI manifestations include candida esophagitis, CMV esophagitis, salmonellosis, shigellosis, campylobacteriosis, rotavirus gastroenteritis, etc. These infections are usually more severe than in non-HIV children. Mycobacterium avium complex, cryptosporidium, CMV, and other viruses may all induce opportunistic enteropathies. Clostridium difficile (pseudomembranous) colitis may develop in any patient treated with broad spectrum antibiotics for long periods of time. HIV children are at risk of developing chronic hepatitis due to CMV or hepatitis B virus. CMV and microsporidiosis have been identified as causes of biliary tract infection. Pancreatitis may be caused by CMV or drug toxicity (pentamidine, didanosine, zalcitabine).
Neurological manifestations result from progressive encephalopathy and retarded CNS development. There is progressive motor dysfunction and loss of developmental milestones. Exam findings include weakness, flaccidity, hyperreflexia, spasticity, and gait abnormalities. Seizures, cerebrovascular accidents, CNS lymphoma, and meningitis may also develop. Children may do well for several years. Behavioral changes associated with chronic disease, interaction with peers, or frequent medical visits may mimic neurologic changes. Zoster and varicella infections can be severe.
Eye manifestions include frequent conjunctivitis and blepharitis. Severe retinopathies are uncommon.
ENT manifestations include chronic otitis media and sinusitis. Eradication of these infections may be difficult. These children are at risk of developing mastoiditis. Tonsillar and adenoidal hypertrophy may result along with generalized lymphatic hypertrophy. Oral infections include thrush, periodontitis, severe dental caries, parotitis, and cervical lymphadenopathy. Renal manifestations include nephrosis and recurrent urinary tract infections. Children with HIV often manifest short stature and delayed puberty. Myopathies and non-specific rheumatologic conditions may develop.
Anemia, granulocytopenia, lymphopenia, and thrombocytopenia all occur frequently with HIV. Lymphopenia is a later manifestation of progressive immune deficiency.Patients may often present with findings similar to ITP (idiopathic thrombocytopenic purpura) or aplastic anemia. Therapeutic agents such as trimethoprim-sulfamethoxasole, zidovudine, and conventional antibiotics may add to bone marrow depression. Acquired circulating anticoagulants result in prolonged coagulation times. Lymphomas occur in 3% of patients. Children are less susceptible to Kaposi’s sarcoma.
Making the diagnosis of HIV infection in young children can be very difficult. ELISA and Western blot assays detecting anti-HIV IgG antibody are highly sensitive and specific when used in adults. However, these assays are often falsely positive in young children due to the detection of maternal antibodies in the infant’s serum which can be detectable for as long as 18 months after birth. Only about 13% to 39% of babies born to HIV-infected mothers will develop HIV infection. Although overly sensitive in infants, these commonly used tests are able to identify infants at risk of developing HIV infection by identifying infected mothers.
More specific tests for young children include in vitro production of HIV-specific antibodies by the child’s lymphocytes, detection of IgA anti-HIV antibodies, detection of HIV p24 antigen (after decomplexing HIV antigen-antibody complexes), HIV culture, and polymerase chain reaction for detection of HIV-specific nucleic acid sequences. However, these tests are not as widely available.
As this case points out, any patient could be potentially infected with HIV. Universal precautions should be practiced routinely to prevent occupational and nosocomial transmission. The child in this case came from a low risk family residing in a low risk community. In contrast to hospital personnel working in a high risk community who routinely practice universal precautions, hospital personnel in low risk settings may actually be at higher risk by not practicing universal precautions routinely.
- Church JA. Clinical Aspects of HIV Infection in Children. Pediatric Annals 1993;22(7):417-427.
- Church JA. The Diagnostic Challenge of the Child Born At Risk For HIV Infection. Pediatric Clinics of North America 1994;41(4):715-725.