Splenic rupture in a newborn with severe hemophilia--case report and review.

SUMMARY: Life-threatening splenic rupture is rare in neonates with severe hemophilia. There are only 3 cases of splenic rupture in neonates with hemophilia reported in the literature. We present the case of an infant, born to a hemophilia A carrier mother. The infant was asymptomatic until discharge at 48 hours of age, but presented on the third day of life with shock, abdominal distension, and severe anemia. Computed tomography of the abdomen confirmed the diagnosis of splenic rupture with hemoperitoneum. The infant recovered after extensive supportive care surgery and factor replacement.

Severe acute interstitial nephritis secondary to minocycline use in an adolescent girl.

Acute interstitial nephritis is an uncommon but classic complication of minocycline therapy for acne. A 14-year-old African American girl was started on oral minocycline for the treatment of acne 6 weeks before presentation. After 4 weeks on minocycline, she developed a generalized rash, anasarca, fever, myalgia, nausea, vomiting, sore throat, and generalized body weakness. The evaluation showed increased levels of serum creatinine, urea nitrogen, and serum alanine and aspartate aminotransferases. Renal ultrasonography showed bilateral enlarged, echogenic kidneys, and percutaneous renal biopsy showed features of acute allergic interstitial nephritis. Treatment included methylprednisolone and intravenous fluids and discontinuation of minocycline. The elevated serum creatinine level (12.9 mg/dL (reference, 0.40-0.70 mg/dL)) suggests marked renal impairment corresponding with Kidney Disease Improving Global Outcomes acute kidney injury classification stage 3. The kidney injury improved from stage 3 to stage 1 within 3 days, and early treatment with steroids might have prevented chronic renal failure. The creatinine level promptly decreased to normal, and liver enzyme results also improved. In summary, the diagnosis of acute interstitial nephritis should be considered in patients who present with renal failure associated with recent use of minocycline, and treatment with corticosteroids should be considered early during the hospitalization.

Guillain-Barré syndrome with unilateral peripheral facial and bulbar palsy in a child: A case report.

Guillain-Barré syndrome is characterized by progressive motor weakness, sensory changes, dysautonomia, and areflexia. Cranial nerve palsies are frequent in Guillain-Barré syndrome. Among cranial nerve palsies in Guillain-Barré syndrome, facial nerve palsy is the most common affecting around half of the cases. Facial palsy in Guillain-Barré syndrome is usually bilateral. We describe a pediatric Guillain-Barré syndrome variant presenting with unilateral peripheral facial palsy and dysphagia. A 5-year-old boy had progressive lower extremity weakness and pain 3 days prior to onset of unilateral peripheral facial palsy. On presentation, diagnosis of Guillain-Barré syndrome was supported by areflexia and albuminocytologic dissociation. His condition deteriorated with a decline in his respiratory effort and inability to handle secretions. He was given non-invasive ventilation to prevent worsening of his acute respiratory failure. Brain and spine magnetic resonance imaging scans showed enhancement of the left bulbar nerve complex and anterior and posterior cervical nerve roots with gadolinium. Treatment with intravenous immunoglobulin led to an uneventful clinical course with partial recovery within 2 weeks. In summary, Guillain-Barré syndrome should be considered as a possible cause of unilateral peripheral facial palsy. Guillain-Barré syndrome patients with facial nerve and bulbar palsy require close monitoring as they are at risk of developing acute respiratory failure. Early intervention with intravenous immunoglobulin may benefit these patients. Magnetic resonance imaging findings may lend support to early intervention.

Epidemiology of meningitis in an HIV-infected Ugandan cohort.

There is limited understanding of the epidemiology of meningitis among human immunodeficiency virus (HIV)-infected populations in sub-Saharan Africa. We conducted a prospective cohort study of HIV-infected adults with suspected meningitis in Uganda, to comprehensively evaluate the etiologies of meningitis. Intensive cerebrospiral fluid (CSF) testing was performed to evaluate for bacterial, viral, fungal, and mycobacterial etiologies, including neurosyphilis,16s ribosomal DNA (rDNA) polymerase chain reaction (PCR) for bacteria, Plex-ID broad viral assay, quantitative-PCR for HSV-1/2, cytomegalovirus (CMV), Epstein-Barr virus (EBV), and Toxoplasma gondii; reverse transcription-PCR (RT-PCR) for Enteroviruses and arboviruses, and Xpert MTB/RIF assay. Cryptococcal meningitis accounted for 60% (188 of 314) of all causes of meningitis. Of 117 samples sent for viral PCR, 36% were EBV positive. Among cryptococcal antigen negative patients, the yield of Xpert MTB/RIF assay was 22% (8 of 36). After exclusion of cryptococcosis and bacterial meningitis, 61% (43 of 71) with an abnormal CSF profile had no definitive diagnosis. Exploration of new TB diagnostics and diagnostic algorithms for evaluation of meningitis in resource-limited settings remains needed, and implementation of cryptococcal diagnostics is critical.

Asymptomatic DNAemia heralds CMV-associated NEC: case report, review, and rationale for preemption.

Human cytomegalovirus (CMV) infection may be acquired in very low birth weight and extremely low birth weight (ELBW) infants from breast milk. The clinical relevance of such infections is uncertain. There is no consensus on whether screening breast milk for CMV, freezing/pasteurizing milk before feeding, or performing virological monitoring on at-risk infants is warranted. We describe an ELBW infant who acquired CMV postnatally from breast milk and developed CMV sepsis syndrome and clinical evidence of necrotizing enterocolitis (NEC) at ≈ 5 weeks of age. The availability of serial dried blood spots from day of life (DOL) 4 to 21, coincidentally obtained for a metabolic study, provided the novel opportunity to retrospectively test for and quantify the magnitude of CMV DNAemia. DNAemia was present for several weeks before the onset of severe CMV disease, first being noted on DOL 18 and increasing in magnitude daily to 4.8 log10 genomes/mL on DOL 21, approximately 8 days before the onset of abdominal distension and 15 days before the onset of CMV sepsis syndrome and NEC. After surgical resection, supportive care, and ganciclovir therapy, the infant recovered. This case underscores the importance of including CMV infection in the differential diagnosis of sepsis and NEC in premature infants. This case also suggests the value of prospective virological monitoring in at-risk low birth weight and ELBW infants. Future studies should examine the potential utility of preemptive monitoring for, and possibly treatment of, CMV DNAemia in premature infants, which may herald the onset of serious disease.