Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia.

BACKGROUND: To describe the first case of optic perineuritis because of meningeal involvement of early stage chronic lymphocytic leukemia (CLL). METHODS: A case report and review of the literature. RESULTS: A case of unilateral optic neuropathy associated with enhancement of the optic nerve sheath is described in a patient with a prior 2-year history of Rai Stage 0 CLL. Lumbar puncture revealed a lymphocytic pleocytosis. Cerebrospinal fluid flow cytometry revealed a monoclonal expansion of CD5+ B cells compatible with CLL, matching the flow cytometry characteristics of his peripheral blood. CONCLUSIONS: Optic perineuritis is often initially diagnosed as optic neuritis, yet the 2 have different etiologies and follow a different clinical course. Orbital MRI with contrast structurally separates the 2, revealing a characteristic pattern of peripheral optic nerve sheath rather than primary optic nerve enhancement. Etiologies of optic perineuritis are varied and include inflammatory, infectious, neoplastic, and toxic entities. Central nervous system (CNS) involvement by chronic lymphocytic leukemia is unusual, but cranial nerve and meningeal involvement have been reported. This case adds central nervous system chronic lymphocytic leukemia to the list of differential diagnostic possibilities for optic perineuritis. It also alerts clinicians to consider optic perineuritis as a potential presenting feature of CNS involvement in otherwise asymptomatic and stable CLL.

Clinical impact of NEC-associated sepsis on outcomes in preterm infants.

OBJECTIVE: To determine risk factors and outcomes of necrotizing enterocolitis (NEC)-associated sepsis in infants with NEC. METHODS: A retrospective review comparing demographic and clinical information in infants with and without NEC-associated sepsis (defined as positive blood culture at the time of NEC onset). RESULTS: A total of 209 infants with medical (n = 98) and surgical NEC (n = 111) had a median gestational age of 27 weeks (IQR 25; 30.5) and a median birth weight of 910 g [IQR 655; 1138]. Fifty of 209 (23.9%) infants had NEC-associated sepsis. Infants with NEC-associated sepsis had lower median GA (26.4 vs. 27.4 weeks; p = 0.01), lower birth weight (745 vs. 930 g; p = 0.009), were more likely mechanically ventilated [p < 0.001], received dopamine [p < 0.001], had more evidence of acute kidney injury [60% vs. 38.4%, p = 0.01], longer postoperative ileus (16 [13.0; 22.0] vs. 12 [8; 16] days; p = 0.006), higher levels of C-reactive protein, lower platelet counts, longer hospitalization compared to infants without NEC-associated sepsis. On multivariate regression, cholestasis was an independent risk factor for NEC-associated sepsis (OR 2.94; 95% CI 1.1-8.8, p = 0.038). CONCLUSION: NEC-associated sepsis was associated with greater hemodynamic support, acute kidney injury, longer postoperative ileus, and hospitalization on bivariate analysis, and cholestasis was associated with higher odds of sepsis on multi regression analysis. IMPACT: NEC-associated sepsis was present in 24% of infants with NEC. Gram-positive bacteria, Gram-negative bacteria, and Candida were found in 15.3%, 10.5%, and 2.8% of cases, respectively. Infants with NEC-associated sepsis had a greater inflammatory response (CRP levels), received more blood transfusion before NEC onset, frequently needed assisted ventilation ionotropic support, and had acute kidney injury after NEC onset. NEC infants with Gram-negative sepsis had higher portal venous gas, received more platelet transfusions before NEC onset, and had higher CRP levels and lower median lymphocyte counts at 24 h after NEC onset than those with Gram-positive sepsis.

Otoscopy and tympanometry outcomes from the National Health and Nutrition Examination Survey (NHANES).

PURPOSE: The purpose of the study was to estimate the prevalence of conductive pathologies based on tympanometric and otoscopic findings in adults in the U.S. and examine relationships between abnormal findings and audiometrically defined or reported perceived hearing loss and tinnitus. MATERIALS AND METHODS: Data from 3409 individuals aged 20-69 years from the 1999-2000 and 2000-2002 cycles of the National Health and Nutrition Examination Survey (NHANES) were analyzed. Outcomes included report of ear tubes, self-perceived hearing loss, and tinnitus (yes or no). Otoscopy involved screening for presence of cerumen (ear wax) and physical abnormality for each ear. Ear-specific tympanometry included measures of middle ear pressure, external ear volume, tympanometric width, and admittance/compliance for each ear. Mean and prevalence estimates are provided applying sample weights. A multivariate ordinal regression model adjusting for age, sex, race/ethnicity, and education was used to examine relationships between otoscopy or tympanometry measures with hearing loss and tinnitus. RESULTS: Approximately 12% of the sample showed evidence of abnormal tympanometry and 12% showed evidence of abnormal otoscopy in at least one ear. Measured hearing loss was consistently related to tympanometry outcomes, in both univariate and adjusted models, but perceived hearing loss and tinnitus were not related to abnormal tympanometry or otoscopy. The most common reason for abnormal otoscopy was presence of excessive or impacted cerumen, representing an estimated 10% of the population. CONCLUSIONS: With 1 out of 10 adults having excessive cerumen, adults considering over-the-counter (OTC) hearings aids may benefit from an ear examination prior to purchase.

Severe acute kidney injury in neonates with necrotizing enterocolitis: risk factors and outcomes.

BACKGROUND: To study the risk factors and outcomes of severe acute kidney injury (AKI) in neonates with necrotizing enterocolitis. METHODS: Retrospective chart review of 202 neonates with necrotizing enterocolitis (NEC) (Bell stage >IIa) from 2013 to 2018. AKI was defined as per-modified neonatal Kidney Disease: Improving Global Outcomes criteria. Demographic, clinical, and outcome data were compared between neonates without severe AKI (stage 0 and 1 AKI) and those with severe AKI (stage 2 and 3 AKI). RESULTS: Severe AKI occurred in 66/202 (32.6%) of neonates after NEC diagnosis and after 61/104 (58.7%) of surgical NEC diagnoses. On adjusted model, surgical NEC [adjusted odds ratio (aOR) = 30.6; 95% confidence interval (CI) = 8.9, 130.6], outborn [aOR = 3.9; 95% CI = 1.54, 11.0], exposure to antenatal steroids [aOR = 3.0; 95% CI = 1.1, 8.9], and positive blood culture sepsis [aOR = 3.5; 95% CI = 1.3, 10.0] had increased odds for severe AKI. Those with severe AKI required longer hospitalization [124 days (interquartile range (IQR) 88-187) vs. 82 days (IQR 42-126), p < 0.001]. CONCLUSIONS: Severe AKI is common in neonates with NEC who require surgical intervention, are outborn, have positive blood culture sepsis, and receive antenatal steroids. Severe AKI is associated with a significantly longer length of hospitalization. IMPACT: Neonates with NEC, who are transferred from outside hospitals, require surgical NEC management, and/or have a positive blood culture at NEC onset are at the highest odds for severe (stages 2 and 3) AKI. Assessment of urine output is important for patients with NEC. Without it, 11% of those with severe AKI would have been misdiagnosed using serum creatinine alone. Kidney-protective strategies in the pre-, peri-, and postoperative period may improve the morbidity and mortality associated with severe AKI in neonates with NEC.