Infantile pyknocytosis is a rare cause of neonatal hemolytic anemia, which presents in the first few weeks of life. We report a classic case of infantile pyknocytosis that presented to our ...institution with rebound hyperbilirubinemia after receiving phototherapy. The infant was found to have a hemoglobin of 5.8 g/dL, requiring a total of 15 mL/kg of red blood cells (in 2 separate transfusions) before discharge. The diagnosis was ultimately made by a review of the peripheral blood smear. We review the literature and suggest pediatricians consider infantile pyknocytosis on their differential when hemolytic anemia presents in the newborn period.
Kaposi sarcoma (KS) is the most common HIV-associated malignancy in children and adolescents in Africa. Pediatric KS is distinct from adult disease. We evaluated the clinical characteristics ...associated with long-term outcomes. We performed a retrospective observational analysis of 70 HIV-infected children and adolescents with KS less than 18 years of age diagnosed between 8/2010 and 6/2013 in Lilongwe, Malawi. Local first-line treatment included bleomycin and vincristine plus nevirapine-based highly active anti-retroviral therapy (HAART). Median age was 8.6 years (range 1.7-17.9); there were 35 females (50%). Most common sites of presentation were: lymph node (74%), skin (59%), subcutaneous nodules (33%), oral (27%), woody edema (24%), and visceral (16%). Eighteen (26%) presented with lymphadenopathy only. Severe CD4 suppression occurred in 28%. At time of KS diagnosis, 49% were already on HAART. Overall, 28% presented with a platelet count < 100 x 109/L and 37% with hemoglobin < 8 g/dL. The 2-year event-free (EFS) and overall survival (OS) were 46% and 58% respectively (median follow-up 29 months, range 15-50). Multivariable analysis of risk of death and failure to achieve EFS demonstrated that visceral disease (odds ratios OR 19.08 and 11.61, 95% CI 2.22-163.90 and 1.60-83.95 respectively) and presenting with more than 20 skin/oral lesions (OR 9.57 and 22.90, 95% CI 1.01-90.99 and 1.00-524.13 respectively) were independent risk factors for both. Woody edema was associated with failure to achieve EFS (OR 7.80, 95% CI 1.84-33.08) but not death. Univariable analysis revealed that lymph node involvement was favorable for EFS (OR 0.28, 95% CI 0.08-0.99), while T1 TIS staging criteria, presence of cytopenias, and severe immune suppression were not associated with increased mortality. Long-term complete remission is achievable in pediatric KS, however outcomes vary according to clinical presentation. Based on clinical heterogeneity, treatment according to risk-stratification is necessary to improve overall outcomes.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background
Anemia is associated with intensive care unit (ICU) outcomes, but data describing this association in sub-Saharan Africa are scarce. Patients in this region are at risk for anemia due to ...endemic conditions like malaria and because transfusion services are limited.
Methods
This was a prospective cohort study of ICU patients at Kamuzu Central Hospital (KCH) in Malawi. Exclusion criteria included age <5 years, pregnancy, ICU readmission, or admission for head injury. Cumulative incidence functions and Fine-Gray competing risk models were used to evaluate hemoglobin (Hgb) at ICU admission and hospital mortality.
Results
Of 499 patients admitted to ICU, 359 were included. The median age was 28 years (interquartile ranges (IQRs) 20-40) and 37.5% were men. Median Hgb at ICU admission was 9.9 g/dL (IQR 7.5-11.4 g/dL; range 1.8-18.1 g/dL). There were 61 (19%) patients with Hgb < 7.0 g/dL, 59 (19%) with Hgb 7.0-8.9 g/dL, and 195 (62%) with Hgb ≥ 9.0 g/dL. Hospital mortality was 51%, 59%, and 54%, respectively. In adjusted analyses, anemia was associated with hospital mortality but was not statistically significant.
Conclusions
This study provides preliminary evidence that anemia at ICU admission may be an independent predictor of hospital mortality in Malawi. Larger studies are needed to confirm this association.
Background
A major barrier in improving cancer outcomes in Botswana and other low‐ and middle‐income countries is timely access to care. Understanding time to diagnosis of pediatric cancers in ...Botswana and evaluating factors contributing to delays was necessary to inform interventions.
Methods
A retrospective cohort study of children diagnosed with cancer at Princess Marina Hospital from 2008 to 2015 was performed utilizing the Botswana Pediatric Oncology Database. The time to diagnosis, pretreatment center delay, and pathology turnaround time were calculated. Time to diagnosis was analyzed using univariate and multivariate analyses to determine association with age, sex, distance to a treatment center, HIV status, cancer type, outcome, and presence of metastasis at diagnosis.
Results
The median time to diagnosis was 10.7 weeks, median pretreatment center delay was 9.6 weeks, and median pathology turnaround time was 3 weeks. Longer time to diagnosis was significantly correlated with presence of metastasis at diagnosis. Age, sex, distance to a treatment center, HIV status, cancer type, and outcome were not significantly associated with diagnostic delay.
Conclusion
Children with cancer in Botswana have more than three months of symptoms prior to diagnosis, which is associated with metastasis at diagnosis. Efforts should be made to empower and promote awareness of pediatric cancer symptoms among caregivers and community healthcare providers in order to shorten time to presentation at a treatment center.
Kaposi sarcoma (KS) is among the most common childhood malignancies in central, eastern, and southern Africa. Although its unique clinical features have been established, biological mechanisms ...related to the causative agent, KS‐associated herpes‐virus (KSHV), have yet to be explored in children. We performed a prospective observational pilot study to explore associations between KSHV viral load (VL), human interleukin‐6 (IL‐6) and IL‐10 levels, and clinical characteristics of 25 children with KS in Lilongwe, Malawi from June 2013–August 2015. The median age was 6.4 years. Lymphadenopathy was the most common site of KS involvement (64%), followed by skin and oral mucosa (44% each), woody edema (12%), and pulmonary (8%). Baseline samples for plasma KSHV VL, IL‐6 and IL‐10 analyses were available for 18/25 patients (72%) at time of KS diagnosis. KSHV VL was detectable at baseline in 12/18 (67%) patients, the median baseline IL‐6 level was 8.53 pg/mL (range 4.31–28.33), and the median baseline IL‐10 level was 19.53 pg/mL (range 6.91–419.69). Seven (39%) patients presented with an IL‐6 level > 10 pg/mL (exceeding twice the upper limit of normal). Detectable KSHV VL was significantly associated with lymphadenopathic KS (p = 0.004), while having undetectable KSHV VL was associated with a higher likelihood of presenting with hyperpigmented skin lesions (p = 0.01). Detectable KSHV VL and elevated IL‐6 levels are present in a subset of children with KS. Lytic activation of KSHV and associated elevation in KSHV VL may contribute to the unique clinical manifestations of pediatric KS in KSHV‐endemic regions of Africa.
What's new?
Kaposi sarcoma is among the most common childhood malignancies in regions endemic for Kaposi sarcoma‐associated herpesvirus (KSHV), but virological characteristics of the disease remain undefined. Here the authors studied 25 children with Kaposi sarcoma in southeastern Africa and established links between distinct clinical patterns and elevated KSHV viral load and interleukin‐6 levels. The results points to a role of KSHV lytic activation and a potential KSHV inflammatory cytokine syndrome in the lymphadenopathic form of childhood Kaposi sarcoma.
Abstract
We describe 7 human immunodeficiency virus–infected Malawian children with Kaposi sarcoma who met criteria for Kaposi sarcoma herpesvirus (KSHV) inflammatory cytokine syndrome. Each ...presented with persistent fevers, bulky lymphadenopathy, massive hepatosplenomegaly, and severe cytopenias. Plasma analyses were performed in 2 patients, both demonstrating extreme elevations of KSHV viral load and interleukin 6.
In areas of high HIV and human herpes virus 8 prevalence, life-threatening forms of Kaposi sarcoma (KS) can occur in HIV-positive women during pregnancy. Treating KS in pregnancy must balance both ...the well-being of the mother with the health of the fetus, yet data and recommendations on the best treatment approach for KS during pregnancy are limited. Without effective treatment, which can be difficult to obtain in low income countries (LICs), the mother and infant are at risk for poor outcomes. A successful case report is used as teaching example, followed by a detailed review of the literature that culminates in recommendations for treating KS during pregnancy among HIV-positive women in LICs. A 31-year-old HIV-positive woman presented for care in April 2016 at 28 weeks gestation with extensive KS skin lesions, KS lymphadenopathy, and a large oropharynx KS lesion causing partial airway obstruction. She had initiated antiretroviral therapy (ART) months prior and was virally suppressed, suggesting KS-immune reconstitution inflammatory syndrome. Due to the severity of KS and her third trimester status, combination chemotherapy was initiated using bleomycin, vincristine, and doxorubicin followed by maintenance therapy with paclitaxel. She showed remarkable response to the chemotherapy and had a normal vaginal delivery of a healthy baby at full term. Full clinical remission was achieved, and her baby was HIV-negative with no negative health effects of the KS or the chemotherapy. Review of the sparse existing literature demonstrates the importance, safety, and effectiveness of treating KS during pregnancy. We offer simple adaptable treatment recommendations for use in treating HIV-positive women with KS during pregnancy in LICs. Life-threatening KS can be treated using chemotherapy and ART in resource-limited settings, allowing for good outcomes in mother and infant. While monotherapy with liposomal doxorubicin or paclitaxel is preferred, these are often not available in LICs. As alternatives, bleomycin, vincristine, and doxorubicin can be safely used during the second and/or third trimesters for treating KS. Following a simple treatment approach can be an effective way to treat KS in pregnancy for pregnant women living with HIV in an LIC setting.
Approximately 91% of the world's children living with HIV (CLWH) are in sub-Saharan Africa (SSA). Living with HIV confers a risk of developing HIV-associated cancers. To determine the incidence and ...risk factors for cancer among CLWH, we conducted a nested case-control study of children 0-18 years from 2004-2014 at five centers in four SSA countries. Incident cases of cancer and HIV were frequency-matched to controls with HIV and no cancer. We calculated the incidence density by cancer type, logistic regression, and relative risk to evaluate risk factors of cancer. The adjusted incidence density of all cancers, Kaposi sarcoma, and lymphoma were 47.6, 36.6, and 8.94 per 100,000 person-years, respectively. Delayed ART until after 2 years of age was associated with cancer (OR = 2.71, 95% CI 1.51, 4.89) even after adjusting for World Health Organization clinical stage at the time of enrolment for HIV care (OR = 2.85, 95% CI 1.57, 5.13). The relative risk of cancer associated with severe CD4 suppression was 6.19 (
= 0.0002), 2.33 (
= 0.0042), and 1.77 (
= 0.0305) at 1, 5, and 10 years of ART, respectively. The study demonstrates the high risk of cancers in CLWH and the potential benefit of reducing this risk by the early initiation of ART.
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