Central Venous Pressure and Impaired Renal Function in Children and Young Adults With Cardiovascular Disease.

Background: Traditionally, low cardiac output has been considered the primary hemodynamic driver of renal function and injury. Adult data suggest that central venous pressure (CVP) is a more important factor. Objectives: The authors hypothesized that in children with cardiovascular disease, higher CVP predicts lower estimated glomerular filtration rate (eGFR) and worsening renal function (WRF). Methods: We performed a single-center cohort study of patients aged 3 months to 21 years with biventricular circulation undergoing cardiac catheterization. Pearson's correlation and linear and Cox regression analyses were performed to determine associations with eGFR at the time of catheterization and WFR within 180 days after catheterization. Results: 312 patients had median age 7.9 years (IQR: 2.3 to 14.5 years), median eGFR 97 mL/min/1.73 m2 (IQR: 81-118 mL/min/1.73 m2), median CVP 7 mm Hg (IQR: 5-9 mm Hg), and median cardiac index 3.7 mL/min/m2 (IQR: 2.9-4.6 mL/min/m2). Nearly half (48%) were transplant recipients. In multivariable analysis, CVP was independently associated with eGFR (ß = -2.65; 95% CI: -4.02, -1.28; P < 0.001), as was being a transplant recipient (ß = -10.20; 95% CI: -17.74, -2.65; P = 0.008), while cardiac index was not. Fifty-one patients (16%) developed WRF. In a proportional hazards model adjusting for cardiac index, only higher CVP (HR: 1.10; 95% CI: 1.04-1.17; P = 0.002) and greater contrast volume by weight (HR: 1.05; 95% CI: 1.01-1.10; P = 0.021) predicted WRF. CVP ≥7 mm Hg likewise predicted WRF (HR: 2.57; 95% CI: 1.29-5.12; P = 0.007). Conclusions: Among children with a spectrum of cardiovascular disease, higher CVP is associated with lower eGFR and development of WRF, independent of cardiac index.

Atypical infiltrates on endomyocardial biopsy are associated with adverse outcomes in pediatric heart transplantation.

BACKGROUND: The significance of atypical infiltrates (eosinophils or plasma cells) on endomyocardial biopsy (EMB) after pediatric heart transplant (HTx) is not known. We hypothesized that atypical infiltrates are associated with worse post-HTx outcomes. METHODS: We performed a retrospective cohort study of consecutive patients <21 years old who underwent primary HTx between 2013 and 2017. Multiorgan transplants were excluded. The presence of atypical infiltrates and burden of atypical infiltrates (rare vs predominant) on EMB were recorded. Primary outcome was a composite of cardiac allograft vasculopathy, graft failure (relisting or retransplant), or death. Presence of atypical infiltrates was evaluated: (1) overall using Cox regression with time-dependent covariates and (2) if present by 1 year post-HTx using Kaplan-Meier analysis. RESULTS: Atypical infiltrates were present in 24 out of 95 patients (25%) and were associated with a higher likelihood of reaching the composite outcome (hazard ratio (HR) 6.22, 95% confidence interval (CI) 2.60-14.89, p < 0.0001). This persisted when controlling for rejection in multivariable analysis. There was also a greater risk of the composite outcome if ≥2 nonconsecutive EMBs had atypical infiltrates (HR 11.80, 95%CI 3.17-43.84, p = 0.0002) or if atypical infiltrates were the predominant feature on EMB (HR 30.58, 95%CI 9.34-100.06, p < 0.0001). Patients with atypical infiltrates by 1-year post-HTx had a 5-year freedom from the composite outcome of 48%, compared to 90% if no atypical infiltrates had been present by this timepoint (log rank p = 0.002). CONCLUSIONS: The presence of atypical infiltrates on EMB is associated with significantly worse outcomes in children following HTx. These patients require closer follow-up to assess for developing graft dysfunction.

Influence of Parental Awareness Drive on Preoperative Fasting Compliance in Pediatric Day Care Surgery.

Context: Optimum preoperative fasting is imperative for the prevention of aspiration in pediatric patients. The current guidelines advocate 2-4-6 rule for the same. However, direct supervision is lacking in large volume centers. Aims: Thus, we aimed to determine the fasting compliance of children preoperatively and to ascertain whether parents understood the significance and purpose of optimum fasting. Materials and Methods: Design - A prospective questionnaire-based study regarding preoperative fasting was performed in pediatric patients aged 1-10 years scheduled for "day care surgery" or "same day admission surgery" over 12 weeks. Thereafter, parental awareness drive was carried out, and a re-audit was performed with a questionnaire in the next cohort of patients. Results: The number of patients in the pre and postcounseling groups were 98 and 99. Thirteen percent of the patients were optimally fasted for solids initially. Re-audit confirmed compliance increased to 46%. Patients fasting adequately (2-3 h) for clear fluids increased from 22.4% to 51.5% postcounseling. The number for optimally breast-fed children increased postaudit (23.1%-39.1%). Consequent to the drive, parental awareness increased and 49.5% parents knew that only plain water was permitted during fasting. Number of parents considering preoperative fasting important increased from 39.8% to 79.7%. Initially, 27.6% of the parents did not know the reason for fasting, which reduced to 3% postaudit. Conclusion: Parents are misinformed and ignorant about optimum preoperative fasting. Adequate education and awareness to improve their knowledge was associated with increased compliance for optimal fasting.

Diagnostic and therapeutic application of thoracic epidural in a child with intractable cardiac arrhythmias: A case report.

A 3-year-old boy presented with episodes of uneasiness and transient loss of consciousness. Atrial tachyarrhythmias with rapid ventricular rate was diagnosed and initially unsuccessfully treated with oral antiarrhythmic drugs. Subsequent Holter monitoring revealed ventricular arrhythmias. Despite pharmacologic treatment, he needed numerous cardioversions. Surgical sympathectomy was planned. Initially, sympathectomy was achieved using a continuous high thoracic epidural block and was performed to ascertain the efficacy of the thoracic sympathectomy. This successfully reduced the ventricular arrhythmias and the need for antiarrhythmic agents. The epidural infusion was also used for pain relief following the subsequent surgical sympathectomy.

Ventricular assist device support for children with chemotherapy-induced cardiomyopathy and advanced heart failure: Perspectives gained from a single-center experience.

BACKGROUND: Guidance and data on ventricular assist device (VAD) support for children with chemotherapy-induced cardiomyopathy, particularly within the first 2 years after chemotherapy, are limited. METHODS: We performed a single-center retrospective case series, reviewing medical records of children <18 years of age with chemotherapy-induced cardiomyopathy and advanced heart failure (HF) who received durable VAD support. RESULTS: Six patients met inclusion criteria-5 HeartWare™ HVAD, 1 Berlin Heart EXCOR® . Median age at cancer diagnosis was 6 years (IQR 4.5-10 years). Median dose of anthracycline received was 540 mg/m2 (IQR 450-630 mg/m2 ). All patients developed HF within 1 year after initiation of cancer treatment (median 8 months, IQR 6-11.5 months) and were initiated on durable VAD support at a median of 8 months after completion of cancer treatment (IQR 3.3-43.5 months). Four patients had significant right ventricular dysfunction needing oral pulmonary vasodilator therapy, one patient had a major bleeding complication, and two patients had thromboembolic strokes while on VAD support. Median duration of VAD support was 7.5 months (IQR 3-11.3 months). Two patients underwent VAD explant due to recovery of LV function, one died due to cancer progression, and three underwent heart transplantation. CONCLUSIONS: Durable VAD support should be considered as a therapeutic option for children who have advanced HF due to chemotherapy-induced cardiomyopathy, even within 2 years of completing cancer treatment. A multi-disciplinary approach is essential for appropriate patient selection prior to implant and to ensure comprehensive care throughout the duration of VAD support.

Neurodevelopmental Outcomes in Infants With Cardiac Surgery Associated Acute Kidney Injury.

BACKGROUND: Infants who undergo surgery for congenital heart disease are at risk of neurodevelopmental delay. Cardiac surgery-associated acute kidney injury (CS-AKI) is common but its association with neurodevelopment has not been explored. METHODS: This was a single-center retrospective observational study of infants who underwent cardiac surgery in the first year of life who had neurodevelopmental testing using the Bayley Scale for Infant Development, third edition. Single and recurrent episodes of stages 2 and 3 CS-AKI were determined. RESULTS: Of 203 children with median age at first surgery of 12 days, 31% had one or more episodes of severe CS-AKI; of those, 16% had recurrent CS-AKI. Median age at neurodevelopmental assessment was 20 months. The incidence of delay was similar for patients with and patients without CS-AKI but all children with recurrent CS-AKI had a delay in one or more domains and had significantly lower scores in all three domains, namely, cognitive, language, and motor. CONCLUSIONS: This study has assessed the association of CS-AKI with neurodevelopmental delay after surgery for congenital heart disease in infancy. Infants who have recurrent CS-AKI in the first year of life are more likely to be delayed and have lower neurodevelopmental scores.

Poor efficacy of oral iron replacement therapy in pediatric patients with heart failure.

INTRODUCTION: Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure. METHODS: We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test. RESULTS: Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy. CONCLUSIONS: This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.

Management of Advanced Heart Failure in Children with Cancer Therapy-Related Cardiac Dysfunction.

The evolution of cancer therapies has led to marked improvement in survival of those affected by childhood malignancies, while also increasing the recognition of early and late toxicities associated with cancer therapies. Cardiotoxicity can include cardiomyopathy/heart failure, coronary artery disease, stroke, pericardial disease, arrhythmias, and valvular and vascular dysfunction as a result of exposure to chemotherapy and/or radiation. Anthracyclines remain the most common cause of chemotherapy-induced cardiomyopathy (CCM) with varying clinical presentations including: acute, early onset, and late-onset. Many individuals develop cardiac dysfunction over the long-term, ranging from subclinical cardiac dysfunction to end-stage symptomatic heart failure. The focus of this review is on characterization of symptomatic heart failure in children with cancer therapy-related cardiac dysfunction (CTRCD) primarily due to CCM and utilization of advanced heart failure therapies, including ventricular assist device (VAD) support and heart transplantation, with consideration of unique patient-related factors.

Mechanical Support for The Failing Single Ventricle at Pre-Fontan Stage: Current State of The Field and Future Directions.

With the substantial growth of pediatric ventricular assist device (VAD) support, there has been an expansion of the target population towards more complex patients, including congenital heart disease (CHD) with single ventricle (SV) physiology. The outcomes of Stage I and Stage II SV-CHD patients on VAD support from the Pedimacs database are poor, with less than 50% survival on VAD by the 3-month mark in both. The primary objective of this article is to describe the current state of VAD support for the failing Stage I and II SV-CHD circulation, to provide insight into potential areas of outcome improvement. We reviewed the published literature in the form of database and registry reports as well as single-center studies to discuss the outcomes of Stage I and Stage II SV-CHD patients on VAD support. Registry-based studies suggest that VAD support for the failing Stage I and Stage II SV-CHD circulations is challenging. However, the more promising outcomes in several single-institutional reports for both Stage I and Stage II SV-VAD indicate that the grim picture from the databases does not reflect the best outcomes that are possible to be achieved, potentially at experienced centers with higher volumes. Areas of future study and potential improvement including timely initiation of VAD support in the cohort of patients expected to not be a candidate for standard SV palliations, pump selection and the benefits of continuous-flow devices, and the decision-making for setting up the optimum circulation for VAD support, be it Fontan completion if feasible or takedown to shunt physiology.

Iron Deficiency Is Associated with Adverse Outcomes in Pediatric Heart Failure.

OBJECTIVE: To evaluate the prevalence of iron deficiency and its association with outcomes in children with heart failure. STUDY DESIGN: A single-center retrospective cohort study of patients with heart failure aged 1-21 years from July 2012 to June 2017 with available serum iron studies was performed. Subjects were analyzed in 2 groups: biventricular systolic heart failure (BiV) and single-ventricle congenital heart disease with systolic heart failure (SV). Iron deficiency was defined as ≥2 of the following: serum iron <50 µg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, or transferrin saturation <15%. The primary outcome was a composite adverse event (CAE) of ventricular assist device implantation, heart transplantation, or death, at 3 and 6 months from time of iron studies. RESULTS: Of the 107 subjects (77 BiV, 30 SV) included in the study, 56% were iron deficient. Demographics, etiology of heart failure, and chronicity of heart failure symptoms were not associated with iron deficiency. On multivariable analysis, in group BiV, iron deficiency was associated with CAE at 3 months (79% iron deficiency in CAE group vs 37% iron deficiency in non-CAE, P = .001, OR 7, 95% CI 2-21) and 6 months (76% iron deficiency in CAE vs 35% iron deficiency in non-CAE, P = .002, OR 7, 95% CI 2-24). In group SV, iron deficiency was associated with CAE at 6 months (79% iron deficiency in CAE vs 29% iron deficiency in non-CAE, P = .014, OR 8, 95% CI 2-32). CONCLUSIONS: Iron deficiency was present in 56% of the pediatric patients with heart failure who were evaluated with iron studies. Iron deficiency was associated with greater risk of ventricular assist device implantation, heart transplantation, or death.

A novel approach to bedside pretransfusion identity check of blood and its components: the Sandesh Positive-Negative protocol.

BACKGROUND: Blood component mistransfusion is generally due to preventable clerical errors, specifically pretransfusion misidentification of patient/blood unit at bedside. Hence, electronic devices such as barcode scanners are recommended as the standard instrument used to check the patient's identity. However, several healthcare facilities in underdeveloped countries cannot afford this instrument; hence, they usually perform subjective visual assessment to check the patient's identity. This type of assessment is prone to clinical errors, which precipitates significant level of anxiety in the healthcare personnel transfusing the blood unit. Hence, a novel objective method in performing pretransfusion identity check, the 'Sandesh Positive-Negative (SPON) protocol,' was developed. METHODS: A nonrandomized study on bedside pretransfusion identity check was conducted, and 75 health care personnel performed transfusion. The intervention was performed by matching a custom-made negative label with blood component with the positive label of the same patient available at bedside who was about to receive transfusion. RESULTS: In total, 85.3% of the subjects were anxious while performing pretransfusion identity check based on the existing standard practice. After the implementation of the SPON protocol, only 38.7% experienced either mild, moderate or severe anxiety. The overall level of satisfaction also increased from 8.0% to 38.7% and none were dissatisfied. Although only 9.3% were dissatisfied about the existing practice, approximately 70.7% felt the need for a better/additional protocol. Clerical error was not observed. CONCLUSIONS: The SPON protocol is a cost-effective objective method that reduces anxiety and increases satisfaction levels when performing final bedside identity check of blood components.

Missed Diagnosis of New-Onset Systolic Heart Failure at First Presentation in Children with No Known Heart Disease.

OBJECTIVE: To determine frequency of missed heart failure diagnosis at first presentation among children with no known heart disease admitted with new-onset heart failure. STUDY DESIGN: Using a retrospective design, we reviewed electronic medical records of all patients aged <21 years with no known heart disease, hospitalized with new-onset heart failure during 2003-2015 at a tertiary-quaternary care institution. We assessed records for missed diagnosis of heart failure (primary outcome), associated process breakdowns, and clinical outcomes using a structured data collection instrument. RESULTS: Of 191 patients meeting inclusion criteria, 49% (94/191) were missed on first presentation. Most common incorrect diagnostic labels given to "missed" patients were bacterial infection (29%; 27/94), followed by viral illness (22%; 21/94) and gastroenteritis/hepatitis (21%; 20/94). On multivariable analysis, presentation to primary care provider (PCP), longer duration of symptoms (median 7 days), more than 2 symptoms of heart failure, and nausea/emesis were associated with missed diagnosis. On examining process breakdowns, 49% had errors in history-taking and 50% had no documentation of differential diagnoses. There was no difference in hospital mortality, length of stay, or mechanical circulatory support in missed vs not-missed cohorts. Unnecessary noninvasive and invasive tests were performed in 18% and 4% of patients, respectively. CONCLUSIONS: Nearly one-half of children with no known heart disease hospitalized with systolic heart failure were missed at first presentation and underwent significant nonrelevant treatment and testing. Initial presentation to the PCP, longer duration of symptoms before presentation, and nausea/emesis were associated with missed diagnosis.

Characteristics and outcomes of children with ductal-dependent congenital heart disease and esophageal atresia/tracheoesophageal fistula: A multi-institutional analysis.

BACKGROUND: Extracardiac birth defects are associated with worse outcomes in congenital heart disease (CHD). The impact of esophageal atresia/trachea-esophageal fistula (EA/TEF) on outcomes after surgery for ductal-dependent CHD is unknown. METHODS: Retrospective matched cohort study using the Pediatric Health Information System database from 07/2004 to 06/2015. Hospitalizations with ductal-dependent CHD and EA/TEF, undergoing CHD surgery were included as cases. Admissions with ductal-dependent CHD without EA/TEF were matched 3:1 for age at admission and Risk Adjustment for Congenital Heart Surgery-1 classification. Comparisons were performed using generalized estimating equations. RESULTS: There were 124 cases and 372 controls. Cases included 32 (25.8%) low-risk, 86 (69.3%) intermediate-risk, and 6 (4.8%) high-risk patients. Cases had more females compared to controls (53.2% vs 41.1%, P = .022). Cases were more likely to be premature (28.2% vs 13.7%, P = .001) and low birth weight (29.8% vs 11.8%, P < .001). Cases had a similar frequency of Down syndrome, and DiGeorge/Velocardiofacial syndrome, but a higher frequency of anorectal malformations (4.3% vs 2.4%, P < .001) and renal anomalies (27.4% vs 9.9%, P < .001) than controls. Cases had a higher mortality on univariate (22.0% vs 8.4%, P < .001) and multivariable analysis (odds ratio 2.45, 95%, confidence interval 1.34 - 4.49). Prematurity also was significantly associated with mortality on multivariable analysis. Cases had a longer duration of mechanical ventilation, longer hospital duration of stay, and higher total cost than controls (all P < .001). CONCLUSION: In children with ductal-dependent CHD, EA/TEF is associated with increased morbidity, mortality and resource utilization. A majority of patients undergo EA/TEF repair prior to congenital heart disease surgery. (Surgery 2017;160:XXX-XXX.).

Prevalence of Anemia and Iron Deficiency in Pediatric Patients on Ventricular Assist Devices.

The prevalence and characteristics of anemia and iron deficiency in children supported by a ventricular assist device (VAD) are unknown. Patients <21 years of age on durable VAD support for ≥7 days at Texas Children's Hospital from 2006 to 2015 were retrospectively reviewed. Red blood cell (RBC) and iron deficiency indices in pulsatile VAD (P-VAD) and continuous-flow VAD (CF-VAD) were evaluated. Anemia, iron deficiency, and iron therapy regimens were identified. Seventy-six VAD implants in 74 patients were included: 45 P-VAD and 31 CF-VAD. Overall, 48% (36/75) of patients were anemic at VAD implant, with 67% of CF-VAD and 34% of P-VAD affected. Iron deficiency was seen in 52% (39/75) of patients at implant (similar in both groups). At explant, 71% (53/75) had anemia (similar in both groups). No patients had microcytosis. Iron supplementation was given to 20 patients, with four receiving target replacement therapy (2-6 mg/kg/d × 90 days). Red blood cell transfusion volumes were higher for P-VAD versus CF-VAD. We concluded that anemia and iron deficiency are common in pediatric VAD patients. Pulsatile VAD patients tend to develop anemia over the course of VAD support. Lack of microcytosis, likely masked by high RBC transfusions, suggests that specific iron studies are necessary to identify iron deficiency.

Bartonella Osteomyelitis of the Acetabulum: Case Report and Review of the Literature.

INTRODUCTION: Bartonella henselae commonly involves the mononuclear phagocyte system (MPS), and its most common presentation is lymphadenitis. Rarely, it can cause isolated osteomyelitis. We present a case of a 3 year old with constitutional symptoms and new onset of limp. Previously reported cases of osteomyelitis due to B. henselae are also reviewed here, keeping the index case in mind. METHODS: We conducted a Medline search using MeSH subject headings Bartonella and osteomyelitis, limited to humans. RESULTS: The index case is a 3-year-old female who had a subacute presentation with new-onset leg pain and fever. Subsequent imaging demonstrated osteomyelitis of the acetabulum. Multiple diagnostic attempts were unsuccessful, and the patient did not respond to empiric therapy. Despite indeterminate serology, the diagnosis of Bartonella osteomyelitis was eventually confirmed by PCR on bone biopsy of the lesion. The literature search revealed 48 publications, which were reduced to 28 when limiting articles to the English language and the pediatric population. After a report of 36 pediatric cases in 2007, there have been an additional 12 pediatric cases since 1998. Generally, these patients had a subacute presentation with relatively mild constitutional symptoms. Most commonly, bone involvement occurred as osteolytic lesions of the axial skeleton. Of the total 48 cases reported, only four reported involvement of the axial skeleton. CONCLUSION: We present the first case, to our knowledge, of pediatric osteomyelitis of the pelvis due to B. henselae with indeterminate serologic and positive PCR results. Bartonella osteomyelitis should be included in the differential diagnosis when typical pathogens are not identified or if the patient is slow to respond to standard therapies. The sensitivity of tissue PCR for Bartonella osteomyelitis is now better than the current gold standard of serology, and new management guidelines may need to reflect this.

Association of Chlamydia trachomatis infection with human papillomavirus (HPV) & cervical intraepithelial neoplasia - a pilot study.

BACKGROUND & OBJECTIVES: Human papillomavirus (HPV) is the necessary cause of cervical cancer and Chlamydia trachomatis (CT) is considered a potential cofactor in the development of cervical intraepithelial neoplasia (CIN). The objective of this pilot study was to determine the association of CT infection with HPV, other risk factors for cervical cancer, and CIN in symptomatic women. METHODS: A total of 600 consecutively selected women aged 30-74 yr with persistent vaginal discharge, intermenstrual/postcoital bleeding or unhealthy cervix underwent conventional Pap smear, Hybrid Capture 2 (HC2) testing for HPV and CT DNA and colposcopy, with directed biopsy of all lesions. RESULTS: HPV DNA was positive in 108 (18.0%) women, CT DNA in 29 (4.8%) women. HPV/CT co-infection was observed in only four (0.7%) women. Of the 127 (21.2%) women with Pap >ASCUS, 60 (47.2%) were HPV positive and four (3.1%) were CT positive. Of the 41 women with CIN1 lesions, 11 (26.8%) were HPV positive, while two were CT positive. Of the 46 women with CIN2+ on histopathology, 41 (89.1%) were HPV positive, two (4.3%) were CT positive and one was positive for both. The risk of CIN2+ disease was significantly increased (P<0.05) by the following factors: age <18 yr at first coitus, HPV infection and a positive Pap smear. Older age (>35 yr), higher parity, use of oral contraceptives or smoking did not show any significant association with HPV or abnormal histopathology. Parity >5 was the only risk factor positivity associated with CT infection (P<0.05). INTERPRETATION & CONCLUSIONS: Our findings showed that CT infection was not significantly associated with CIN, and most of its risk factors, including HPV infection, in symptomatic women. Longitudinal studies with carefully selected study sample would be able to answer these questions.

Adjunctive testing for cervical cancer screening in low resource settings.

BACKGROUND: Cervical cancer screening using cytology is expensive and requires repeat testing with loss to follow-up for triage. Adjunctive testing using two tests in a parallel or sequential combination can improve diagnostic accuracy, decrease costs and minimise referral rates. AIM: To evaluate the performance of visual inspection with acetic acid (VIA), human papillomavirus (HPV) DNA and cervical cytology in sequential and parallel combinations, to determine the optimum screening strategy. METHODS: A total of 548 symptomatic women presenting to a gynaecology clinic were screened using conventional cytology; HPV testing of self- and physician-collected samples using HC2 assay; and VIA. All women underwent colposcopy with biopsy from all lesions as the reference standard. Test characteristics were calculated individually, and then applied jointly in parallel and sequential combinations. RESULTS: Biopsy-proven CIN2+ was present in 40 (7.8%) women. The sensitivity and specificity, respectively, of the various tests were as follows - VIA 82.5, 66.9%, Pap (≥ASCUS) 77.5, 86.8%, HPV-Physician (HPV(P)) 90.0, 91.5%, HPV-Self (HPV(S)) 80.0, 88.1%. Among the adjunctive testing scenarios, sequential testing performed better than parallel. VIA followed by HPV test had the highest specificity (90.4%) and diagnostic accuracy (90.5%), comparable to HPV-P followed by Pap. CONCLUSIONS: Sequential testing combination of VIA and HPV, followed by colposcopy and directed biopsy for diagnosis, combines economic viability with high scientific fidelity. The high specificity and negative predictive value reduce referral and treatment rates, as well as number of visits required for diagnosis. HPV (P) followed by reflex Pap is a suitable option for high resource situations with access to liquid-based cytology.

The role of fluorodeoxyglucose positron emission tomography-computerized tomography in resolving therapeutic dilemmas in pediatric Hodgkin lymphoma.

INTRODUCTION: Hodgkin lymphoma (HL) is a highly curable lymphoma with cure rates of over 80% and even higher with limited stage disease. Computerized tomography (CT) scan is currently the recommended modality in staging and assessment of response to therapy in patients with HL. However, CT has its limitations. This study describes our experience with patients of HL where fluorodeoxyglucose positron emission tomography (FDG-PET)-CT scan helped decide further management, after completion of chemotherapy. METHODOLOGY: This is a retrospective review of the records of children diagnosed with HL at our center. Patients with post-treatment CT scan showing evidence of residual disease, who underwent FDG-PET-CT for deciding further management, were included in the study. RESULTS: Thirty one patients were diagnosed with HL during this period. Nine patients were eligible and underwent PET-CT. In 8 out of 9 patients, PET-CT showed no scan evidence of active disease. In one patient, FDG-PET-CT carried out after completion of chemotherapy showed evidence of active disease and was given radiotherapy. CONCLUSION: FDG-PET-CT is a promising modality in deciding further management when there is discordance between the post-treatment CT scan and clinical condition of the patient with HL thus avoiding unnecessary chemotherapy/radiotherapy.

Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic study of 5 cases.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma is as yet a poorly characterized subtype of cutaneous T-cell lymphomas. It is difficult to diagnose and lacks a standard treatment protocol. MATERIALS AND METHODS: We report our experience with five such cases in patients with age ranging from 13 to 45 years, wherein we reviewed their clinical presentations, histopathological findings, treatment and clinical outcome. RESULTS: Three out of the five cases are alive of which two are in complete remission. Our series stresses on the need to be aware of this uncommon entity in a dermatology clinic. Facial and upper extremity skin nodules with involvement of the lower dermis on light microscopical examination and suggestive immunohistochemical findings were frequently observed in our patients in contrast to previously described cases.

Diagnostic dilemma of JMML coexisting with CMV infection.

Juvenile myelomonocytic leukemia (JMML) is a rare mixed myeloproliferative and myelodysplastic disorder of early childhood, characterized by excessive proliferation of monocytic and granulocytic cells, along with myelodysplastic features. There are reports of viral infections mimicking JMML, with all clinical and hematological parameters normalizing on resolution of infection. The authors describe a 1- year- old boy with concomitant JMML and CMV infection. The diagnostic dilemma, the significance of distinguishing it from a mimicking viral infection, as well as potential synergistic effect of concomitant infections on the presentation or actual disease severity of underlying JMML will be discussed.