Recurrent intussusception with ileal perforation as a complication of Blue Rubber Bleb Nevus Syndrome in a pediatric patient

Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital condition, characterized by multiple venous malformations that may involve any organ system, most commonly the skin or the gastrointestinal tract. These lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may cause severe complications such as intussusception, volvulus, and intestinal infarction. Intussusception as a complication of BRBNS, although a known complication of the disease, has rarely been reported, especially in the Philippines. In the Philippine Society for Orphan Disorders, only 2 cases of BRBNS are currently included in the organization, including the patient presented in the case report. The treatment of BRBNS that involves the gastrointestinal tract depends on the extent of intestinal involvement and severity of the disease. The treatment aims to preserve the GI tract as much as possible due to the high recurrence in the disease. In this case report, we present a 13 year-old male with BRBNS with previous history of intussusception, successfully managed conservatively;however, upon recurrence, underwent exploratory laparotomy wherein a subcentimeter perforation in the antimesenteric border of the proximal ileum was noted, together with a gangrenous intussuscipiens, and multiple mulberry-like formations on the antimesenteric border of the small bowels. Histopathological findings of the resected bowels showed multiple cavernous hemangiomas consistent with BRBNS. The postoperative course of the patient was unremarkable.Copyright © 2023 The Authors

Bilateral optic neuritis and myocarditis after the first dose of COVID-19 vaccine

Since the introduction of COVID-19 vaccine, various adverse events have been reported including injection site pain, fatigue, headaches, and myocarditis. Cranial neuropathies and optic neuritis, have been also rarely reported, however, the significance of these autoimmune manifestations after the administration of COVID-19 vaccine remain controversial. In this report we present a case of myocarditis and bilateral optic neuritis that occurred in a young healthy male patient after the administration of first dose of mRNA-1273 vaccine (Moderna).Copyright © 2022 The Author(s)

Successful Treatment of Subclavian Artery Thrombosis by Catheter-Based Thrombus Aspiration and Thrombolytic Therapy: A Case Report

Clinical Information Patient Initials or Identifier Number: BP4****/22 Relevant Clinical History and Physical Exam: A 55 Y / Female C/C : Pain, numbness, cold sensation & weakness of left upper limb for 2 hours. Risk Factor : Hypertension, diabetes mellitus O/E : Pale, cold and absent of radial, ulnar, brachial pulse of left upper limb. Muscle power 3/5 left side. So2 86%, BP undetectable. Right upper limb were normal. BP 160/90 mm of hg, pules : 112 b/min, RR : 26/min. Body Temperature 37.5 C [Formula presented] [Formula presented] Relevant Test Results Prior to Catheterization: CBC : WBC 7450, HB % 10.8 g/dl, ESR 20mm in 1st hour, Platelets : 262000, SARS Cov2 Antigen : Negative PT 14.3 sec, INR : 1.07 APTT : 32.4 sec. blood group: O positive Serum Creatinine : 1.1 mg/dl Plasma glucose 9.7 mmmol/l HIV Ab : Negative HBs Ag : Negative Anti-HCV : Negative Urine R/E : Normal lipid profile : Cholesterol 280mg/dl Vascular duplex ultrasound of left upper limb : A dilated echogenic thrombus had blocked the left subclaviav artery lumen. Relevant Catheterization Findings: Conventional angiography with the lowest amount of contrast agent through the right femoral artery, revealed that left subclavian artery thrombosis with total occlusion distal to Left internal mammary artery. [Formula presented] [Formula presented] [Formula presented] Interventional Management Procedural Step: A5Fr MPA catheter with side holes was negotiated through a right femoral sheath and was placed in the left subclavian artery. Initially thrombus aspiration was done with Eliminate aspiration catheter (TERUMO) with no success. Then suction was done with the MPA catheter itself with partial removal of thrombus. Then a 5Fr Pigtail catheter was placed inside the thrombus and kept in situ. For residual thrombus 250,000u of Inj. Streptokinase as a thrombolytic drug was given through the Pigtail catheter as bolus over 30 min. The maintenance dose 100,000 u per hour was given over 24 hours through the Pigtail catheter via infusion pump. After 24 hours of thrombolytic therapy, her pain was reduced, the left hand became slightly warm, and distal pulses were feebly palpable. Moreover, the skin colour returned to near normal with improvement of pallor. Bleeding was well controlled at the catheter site. Doppler sounds revealed partial improvement of arterial flow. After evaluation of partial improvement, a low dose 1000 iu per hour of heparin (UFH)was infused intravenously for 24 hours. After 48 hours, repeat angiography via the inserted catheter at the site did not reveal any atherosclerotic plaque and confirm the thrombosis-dissolution. The latter practice demonstrated a good blood flowto the left upper distal limb leaving a little thrombus in the superficial palmer arch. [Formula presented] [Formula presented] [Formula presented] Conclusion(s): Catheter-based thrombus aspiration and thrombolytic therapy is primarily reserved for patients with acute viable limb ischemia. As observed in the presented case, thrombus aspiration and thrombolytic therapy is recommended to be considered as an alternative therapeutic method for patients with arterial thrombosis due to the rapid response, shorter treatment time and lower cost, compared to common and sometimes unsuccessful therapies.Copyright © 2023

First reported case of parvovirus triggering painful widespread livedoid vasculopathy: another virus causing hypercoagulability?

Livedoid vasculopathy (LV) is a noninflammatory thrombotic disease caused by occlusion of dermal small vessels associated with systemic autoimmune disorders and coagulopathies. However, LV is often reported as being 'idiopathic', despite extensive investigation. We report a case of severe LV in an otherwise healthy 27-year-old woman, associated with parvovirus infection. The patient presented with a short history of a livedoid rash initially covering her torso, which spread to acral sites. Burning pains in the lower limb caused reduced mobility;systemically, she remained well and stable throughout. Examination revealed generalized acral skin pallor, livedoid patches of violet erythema and purpura with deep serpiginous ulcerations over extensor aspects of upper and lower limbs with a more broken/racemosa nonulcerated livedoid appearance on the trunk. On admission a transaminitisareas continued to ulcerate. Codeine was present with a creatine kinase of 1569 U L.1, but other blood test results were unremarkable including erythrocyte sedimentation rate, complement, cryoglobulins, antinuclear antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigen, rheumatoid factor, myositis screen, antiphospholipid screen and thrombophilia screen. Parvovirus IgG and IgM were both positive and tested for, as the patient's young daughter had recently been diagnosed with 'slapped cheek disease'. Magnetic resonance imaging of the thighs showed a diffuse mild myositis;electromyography, nerve-conduction studies, barium swallow and computed tomography of the chest, abdomen and pelvis were all normal. An incisional skin biopsy was performed, which revealed a blood vessel with organizing (Solimani F, Mansour Y, Didona D et al. Development of severe pemphigus vulgaris following SARS-CoV-2 vaccination with BNT162b2. J Eur Acad Dermatol Venereol 2021;35: e649- 51) have been reported. The main proposed mechanisms for AstraZeneca vaccine-induced pemphigus could be a hyperimmune reaction in genetically predisposed individuals, with eventual formation of anti-desmoglein antibodies. An alternative hypothesis is that vaccine components could act as foreign antigens resulting in a cross-reaction with pemphigus antigens. The close association of COVID-19 vaccination with the acute onset of pemphigus in our patient, as well as exacerbations after subsequent vaccine administration, is more than coincidental. Considering the recent pandemic with COVID-19 and the widespread administration of the COVID-19 vaccine, continued observation and documentation of true adverse events is essential.

Acute limb ischemia secondary to vaccine-induced inmune thrombotic thrombocytopenia (VITT) after ChAdOx1 nCoV-19

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a syndrome that resembles to heparin-induced thrombocytopenia (HIT). Platelet factor 4 (PF-4) reacts to a vaccine component resulting formation of immune complex that stimulates an autoimmune reaction triggering platelet consumption causing thrombus formation and producing thrombotic events. When suspected is important to confirm for make a correct anticoagulation management to avoid complications related to unfractioned and low weight heparins use. In this report we describe a case of acute limb ischemia secondary to ChAdOx1 nCoV-19 vaccine (Astrazeneca, Cambridge, UK)Copyright © 2022

Pediatric ovarian torsion: A diagnostic challenge in covid-19 times

Ovarian torsion is a rare gynecological emergency in children and an early surgical intervention is needed to salvage the ovary. Herein, we present a case of eight year old girl who presented with complaints of lower abdominal pain during ongoing COVID-19 pandemic. She was diagnosed as a case of ovarian torsion on right side. She underwent exploratory laparotomy and ovarian cystectomy was performed. Delay in diagnosis and treatment of ovarian torsion may have grave consequences, resulting in functional loss of the ovary. Copyright © 2020, Kathmandu University. All rights reserved.

Acute renal infarction presenting as hypertensive urgency

Case Report: A 46-year-old lady with medical history of hypertension, diabetes mellitus, and peripheral neuropathy was admitted to the hospital with the diagnosis of sepsis without septic shock secondary to a right foot pressure ulcer. Her presentation was non-specific as she was complaining of fatigue, myalgia, fever, and chills. Routine COVID-19 test was done on admission and it came back positive despite her denying having any respiratory symptoms. She was being treated with fluids and antibiotics until her third night of admission. According to the patient, she got up to use the restroom when she suddenly noticed weakness in her lower extremities. She thought it may be due to a muscle spasm;hence, she did not notify the medical team. Later, her nurse was routinely checking the patient's blood pressure when she noted a blood pressure of 220/105 mmHg. She then received a total of 3 doses of intravenous labetalol over three hours;however, her blood pressure continued to be elevated. Patient did endorse right flank pain but it was responding to intravenous fentanyl. The on-call physician then proceeded to perform a full physical examination and noted paleness, weakness, and absent pulses in bilateral lower extremities. A STAT computed tomography (CT) angiogram of the abdominal aorta and iliofemoral arteries was performed and it revealed low-density defects in the right kidney compatible with infarcts, occlusive thrombus in the infrarenal abdominal aorta and extensive bilateral arterial thrombosis. Vascular surgery was immediately consulted and they kept the patient on heparin drip and took her to the operation room within few hours for thrombectomies. Her blood pressure improved following the removal of the thrombus and there were no other documented occurrence of uncontrolled hypertension during her hospitalization. Discussion(s): Acute renal infarction is an arterial vascular event that leads to sudden disruption of blood flow in the renal artery. It can often be diagnosed late due to its rare incidence. In addition, it has a nonspecific clinical presentation that can mimic many common causes. The most common causes of renal infarction include atrial fibrillation, endocarditis, ischemic heart disease, hypercoagulable disorders, and spontaneous renal artery dissection. A case report published by Bourgault M, et al. on renal infarction suggested that around 97% had abdominal/flank pain and 48% of patients had marked uncontrolled hypertension at initial presentation of renal infarction. Our patient did not have any of the afore mentioned risk factors except for a possible hypercoagulable state from her COVID-19 infection and she did present with the two most common presentations. In conclusion, clinicians should have a low threshold for the suspicion of renal ischemia in patients with severe hypertension and flank pain. Copyright © 2023 Southern Society for Clinical Investigation.

Severe progression of autoimmune hepatitis in a young COVID-19 adult patient: A case report

Rationale: The impact of COVID-19 in patients with autoimmune liver disease treated with immunosuppressive therapy has not been described so far. This case report describes the clinical course of a patient with autoimmune hepatitis (AIH) who developed COVID-19 and the features of cytokine syndrome leading to its deterioration in our intensive care unit. Patient's Concern: A 28-year-old male presented with generalized anasarca for two weeks and chronic liver disease for 8 months. Diagnosis: AIH and Covid-19 with features of cytokine storm syndrome. Interventions: Intravenous furosemide, mannitol, syrup lactulose, steroids (prednisolone 40 mg), azathioprine 1 mg/kg body weight, rifaximin, vitamin K, and blood products. Outcomes: The patient had hepatic encephalopathy and AIH and died on the 10th day after admission despite ventilatory support, sustained low-efficiency hemodialysis, and resuscition. Lessons: The dramatic release of cytokines and the inflammatory-immune responses not only alter the pathophysiology but also affects the onset and severity of disease progression in patients with AIH.

LATE ONSET PRIMARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS in AN INDIAN ADOLESCENT BOY during A PEAK of POST COVID-19 MULTISYSTEM INFLAMMATORY SYNDROME

Background: Familial Hemophagocytic lymphohistiocytosis (fHLH) categorized as FHL2 (PRF1), FHL3 (UNC13D), FHL4 (STX11), and FHL5 (STXBP2) encoding for Perforin, Munc13-4, Syntaxin11, and Syntaxin binding protein 2, respectively. There is limited information available about the clinical and mutational spectrum of FHL patients in Indian population. Objectives: To delineate clinical and laboratory features of late onset familial Hemophagocytic Lymphohistiocytosis. Methods: A 12-years-old well nourished sick looking boy, born to a non-consanguineous parents with normal birth, development and immunization history with uneventful past presented to us with 6 days history of high fever, cough, breathing difficulty and severe headache. He had occasional vomiting, abdominal pain, polyarthragia & chest pain from last 10 days. Mother also had given history of throat pain, backache & some non-specifc papular rashes over face before the onset of fever. His vitals were normal. Examination revealed faint diffuse fxed erythematous rash all over the body, pallor, icterus and hepatos-plenomegaly. Musculoskeletal examination was unremarkable. Lab evaluation revealed HB 8.9gm%, TLC 4700/cumm with neutrophils 40% and lymphocytes 56% with 8-9% activated lympocytes. Further evaluation showed low ESR 6mm/hr, fbrinogen 97mg% and albumin 2.2 gm% with elevated CRP 40mg/L, ferritin 2000ng/ml, LDH 658IU/L, SGPT 110IU/L, SGOT 221 IU/L, total bilirubin 6mg%, D-dimer 4355 ng:EFU/ml and Triglycerides 441mg%. His blood, urine, CSF and bone marrow cultures were sterile for endemic bacterial and viral infections in our area. His EBV PCR, CoVID RT PCR and CoVID antibody (Total & IgG) test were negative. His immunoglobulin leves were normal. HRCT Chest showed bilateral mild-moderate plural effusions, mild interstitial thickening in both the lower lobes, few fbrotic opacities & old areas of consolidation bilaterally. 2D echo showed mild pericardial effusion. Bone marrow examination showed Hypercellular marrow with iron depletion and occasional hemophagocytosis with CD8 T lymphocytes proliferation (55.2%) and double positive CD4 & CD8 (1.2%). He was initially commenced on supportive therapy, oxygen & intravenous antibiotics. In view of most probable non-infectious, non-malignant hemophagocytic lymphohistiocytosis, he was fnally given intravenous immunoglobulin (2gm/kg) and intravenous pulse methylprednisolone (30mg/kg). He responded well to above regimen within 3 days. He was discharged with tapering steroids over few weeks. Clinical exome by NGS revealed Homozygous Mutation in STXBP2 gene Intron 14, c.1280-1G>C (3' Splice Site) His parents has been counselled for hematopoietic stem cell transplantation and their decision is still pending. Results: We compared our patietnt with a reference to the largest Indian series of pediatric HLH1. Conclusion: Primary HLH type 5 can present frst time during childhood and adolescence. Any child presenting with unexplained HLH features should undergo genetic analysis irrespective of person's past and family history.

Immune-mediated Acute Limb Ischemia: A Rare Complication of SARS-CoV-19 Infection

Occurrence of acute limb ischaemia (ALI) in patients with SARS-CoV-2 is an uncommon complication. COVID-19 has been associated with thrombotic disease secondary to a hypercoagulable state. COVID-19 appears to cause a hypercoagulable state through mechanisms unique to SARS-CoV-2 and centres on the cross-talk between thrombosis and inflammation. The proposed hypothesis includes a severely heightened inflammatory response that leads to thrombotic inflammation, through a mechanism such as cytokine storm, complement activation, and endothelitis. The innate and adaptive immune responses result in immunemediated thrombosis, leading to thrombotic complications, such as myocardial infarction, pulmonary embolism, deep vein thrombosis, and stroke. The activation of coagulation (D-dimer) and thrombocytopenia are important prognostic markers in SARSCoV- 19 infections. At our institution, we found six patients to have ALI and reviewed their characteristics and outcomes. Our findings showed that in severe COVID-19 disease, the association of ALI had high mortality. Materials and methods: It is a retrospective observational study performed at Bangalore Baptist hospital during the COVID-19 pandemic (August 2020 to August 2021). We report a case series of 6 ALI patients aged between 30 and 55 years. All the patients were tested positive for SARS-CoV-2 disease. All our patients received standard treatment care as per institution protocol for SARS-CoV-2 disease. They were all commenced on therapeutic anticoagulation at admission to ICU. Baseline coagulation profile and inflammatory markers and their trends were followed in all patients. The diagnosis of ALI in all ventilated patients was done clinically by the presence of pallor, pulselessness, acrocyanosis, blisters, and dry care unit with SARS-CoV-2 disease, 6 patients had developed limb ischemia (1.4%). Male and female preponderance was equal. Among 6 patients, 1 was newly detected diabetes mellitus, 2 were diabetic and hypertensive of which one had right upper limb post-polio paralytic sequelae, and the rest had no co-morbidities. The mean duration of ICU stay and mechanical ventilation days was 22 days and 17.8 days, respectively. All the patients had lower limb ischemia of which 3 were unilateral. Discoloration extended up to the ankle joint in almost all cases. As these patients were on the ventilator secondary to severe hypoxemia or vasopressor support, they were managed conservatively. Two patients presented with stroke, pyelonephritis with acute kidney injury, and septic shock requiring high vasopressor support. 5 of 6 patients died during the course of treatment (mortality 83%). All patients showed high inflammatory markers especially D-dimer during the initial development phase of limb ischemia. 1 survived patient required bilateral foot amputation due to dry gangrene. Conclusion: Limb ischemia with tissue necrosis is a dreadful complication and is associated with high mortality. High incidence of thrombosis despite therapeutic anticoagulation raises a question about pathophysiology unique to COVID-19. Evidence of inflammatory-mediated thrombosis and endothelial injury are possible explanations which would support the use of immunotherapy in addition to anticoagulation for the treatment of thrombotic events. Further insight into the cause and management of thrombosis is needed.

Retinal Manifestations in Rhinoorbito-cerebral Mucormycosis: A Cross-sectional Study

Introduction: Rhino-orbito-cerebral Mucormycosis (ROCM) is an uncommon but devastating fungal infection caused by Mucoraceae family fungi, which are angiotropic and filamentous, with significantly high morbidity and mortality despite treatment. Post Coronavirus Disease-2019 (COVID-19), there was a sudden surge in ROCM cases nationwide due to immunologically and metabolically compromised status. Aim: To describe retinal manifestations in ROCM in a tertiary eye care centre of Northern India. Materials and Methods: An analytic, cross-sectional and hospital-based study was conducted in Regional Institute of Ophthalmology, PGIMS Rohtak, Haryana, India, from May 2021 to September 2021. This study was conducted on 200 admitted patients of RCOM in the institute, which was only designated Nodal centre in Haryana, India. Detailed history was recorded in every patient regarding presenting symptoms, history of COVID-19, hospital stay, oxygen inhalation, steroid intake and immunisation. Thorough ocular examination was done in every patient including visual acuity, ocular movements and pupillary reactions. Dilated fundus examination was done by Indirect Ophthalmoscopy (IDO) for posterior segment evaluation. Contrast Enhanced Magnetic Resonance Imaging (CE-MRI) brain with orbit and Paranasal Sinus (PNS) was done in every patient to see the extent of spread and planning further management. Results: Out of 200 patients of ROCM, majority of patients (64/200) were of 51-60 year age group (32%) followed by 41-50 year age group (28%). Out of 200 cases of ROCM, 146 patients (73%) had history of COVID-19 infection in past and 134 (67%) patients had history of hospital stay during COVID-19 infection. Oxygen (O2) supplementation was given to 98 patients either at home or during hospital stay. History of steroid intake was present in 34 patients and 46 patients received injection Remedsivir. Only 12 patients had vaccine against COVID-19 and none of them were fully vaccinated. Most common presenting symptom was unilateral nasal stiffness (22%) followed by loss of vision (17%). Most common predisposing factor was Diabetes Mellitus (DM) in 78 patients (39%) followed by steroid intake in 34 patients (17%). Out of 200 patients, only 60 patients had retinal manifestations and most common was Central Retinal Artery Occlusion (CRAO) (35/60) and the main mechanism is the direct infiltration of central retinal artery due to angioinvasion of fungi from the orbit. Conclusion: CE-MRI brain with orbit is an important tool in diagnosing and monitoring progression of RCOM but it cannot provide information regarding retinal findings like CRAO, central retinal venous occlusion (CRVO), disc pallor and optic atrophy. Thus, the fundus examination of every ROCM patient should be emphasised, as it not only helps in categorising ROCM but also tells about the visual potential of affected eye. Patients with CRAO and combined vascular occlusion should be considered for exenteration on urgent basis, so that intracranial spread can be prevented and patient's life can be saved.

The Prolactinoma Predicament;To Treat Medically Or Surgically

Introduction: Hormonal effects of prolactinomas include central hypogonadism, infertility, and breast symptoms. When the mass is large enough to abut or compress the optic chiasm, prompt treatment is critical to preserve vision. Prolactin secretion is usually proportional to the size of the adenoma. This case describes a patient who presented with prominent vision changes and was found to have a large pituitary macroadenoma with lower than expected prolactin elevation, highlighting the dilemma between medical versus surgical treatment. Case Description: A previously healthy 32 year old male first noticed vision changes in October 2019 when he was unable to see the dots to focus a camera. Due to the COVID-19 pandemic, he delayed seeking care. When he visited a neuro-ophthalmologist in September 2020, he was noted to have dense left eye and mild right eye temporal defects, left afferent pupillary defect, and left greater than right optic disc pallor suspicious for a sellar lesion. MRI in September 2020 showed a sellar and suprasellar mass measuring 3.4 cm tall with left greater than right optic chiasm compression. He was referred to endocrinology, where further history revealed low libido. Physical exam showed BMI 38.7, lipomastia, normal body hair, and normal testicular volume bilaterally. In September 2020, labs showed prolactin 302.4 ng/ml [reference range (RR) 4-15.2 ng/ml], confirmed by dilution to eliminate hook effect, testosterone 119 ng/dl [RR 250-110 ng/dl], FSH 2.0 mIU/ml [RR 1.6-8 mIU/ml], and LH 1.7 mIU/ml [RR 1.5-9.3 mIU/ml], confirming hyperprolactinemia and central hypogonadism, though prolactin was lower than expected given the size of the pituitary mass. Cabergoline was started with goals to shrink the adenoma, alleviate optic chiasm compression and restore gonadal function. By October 2020, the patient’s prolactin normalized and he was tolerating cabergoline. By December, there was marked reduction in the size of the pituitary mass on MRI to 2.1 cm in height. He had significant improvement in visual fields, improved visual acuity, slightly less prominent afferent papillary defect, and improving libido. Discussion: This case illustrates the classic presentation of a patient with a large prolactinoma with hormonal and mass effects. It highlights the role of primary dopamine agonist therapy to treat large prolactinomas causing visual impairment with lower than expected prolactin given the size of the pituitary mass. In this case, the pituitary adenoma may have been an inefficient prolactin secretor due to being poorly differentiated or partially cystic, but treating medically was effective in quickly shrinking the mass and improving his vision, thus avoiding the need for surgery.

Post-COVID pulmonary aspergillosis - A case report

Introduction: Novel coronavirus disease started in December 2019 and declared pandemic on march 11 2020. Viral pneumonia increases patients susceptibility to bacterial and fungal infections and those who were admitted to ICU had a higher probability of infection. Case Report: A 33yr old female came to the hospital with chief complaints of cough with expectoration associated with streaky haemoptysis and generalised weakness since 20 days. There was a h/o hospital admission for covid and patient was on NIV support. Patient was denovo diabetic. On examination pallor present and decreased breath sounds in bilateral infrascapular and infraaxillary areas. Investigations: Chest X ray showed bilateral mid and lowerzone non-homogenous opacities. Sputum for AFB, CBNAAT and KOH were negative. Sputum culture & sensitivity showed streptococcus. HRCT chest was done which showed multiple cavitary lesions in bilateral lung fields. Bronchoscopy was done and washings showed branching septate hyphae on KOH mount suggestive of aspergillosis. Patient was started on antifungals and symptomatic improvement was seen. Conclusion: Pulmonary aspergillosis is a serious complication in postcovid patients and high index of clinical suspicion and focused diagnostic approach is essential for early diagnosis and treatment which may improve the outcome.

Clinicohaematological Patterns of Anemia and Correlation with Clinical Conditions in Children Aged 5 to 12 Years in Tertiary Care Centre

Objective: To classify the haematological pattern, severity of anemia in children 5-12 years age admitted and to find its correlation with the clinical conditions. Methods Crossectional study of 160 patients in two years was done. Patients satisfying the inclusion criteria were selected for study. Relevant clinical data were recorded in a structured proforma including detailed history was recorded with particular symptoms suggestive of anemia such as weakness and easily fatigability, breathlessness on exertion and pica. A thorough clinical examination of every child was done followed by routine investigations for anemia Results Patients between 7-8 year were found to be the most affected. Anemia was found to be more common in female children as compared to male children (F:M=1.13). Anemia is more common in undernourished child. Most common presenting symptoms were gastrointestinal including vomiting, diarrhea and pain abdomen. Most common sign was Pallor followed by other common signs included signs of dehydration associated with diarrhea, hepatosplenomegaly. microcytic hypochromic anemia was the most common morphological type of anemia and macrocytic anemia was the least common.Thalassemia cases were most common among hemolytic anemias. Iron Deficiency Anemia (Nutritional Anemia) was the most common etiology of anemia. Conclusion Dietary deficits affect children aged 5 to 12, creating financial, emotional, and psychological burden for patients and their families, as well as depleting critical national resources. As a result, screening for these illnesses, as well as early detection of anemia and related problems, is essential.

Impact of Time and Mechanical Ventilation on Convalescent Plasma in Severe/Critically Ill COVID-19 Patients

Background: Convalescent plasma (CP) transfusion is one of the suggested treatments for Coronavirus disease 2019 (COVID-19) especially in critically ill patients. Objectives: This study aimed to investigate the efficacy and safety of CP transfusion were investigated in severe/critically ill COVID-19 patients. Methods: This study was performed on 50 consecutive COVID-19 patients with severe/critically ill disease. Severe disease was defined as having at least one of the following symptoms: shortness of breath, respiratory frequency ≥ 20/min, blood oxygen saturation ≥ 93%, partial pressure of arterial oxygen to fraction of inspired oxygen ratio < 300, lung infiltrates > 50% within the last 24-48 h. Critically ill disease was identified by intensive care unit admission, respiratory failure, septic shock, or multiple organ dysfunction or failure. Primary outcomes included the safety of CP transfusion, 14-day and 30-day survival rate, and change in lung computed tomography (CT) scan score. Several other clinical and laboratory features were evaluated as the secondary outcomes. Results: Based on the results, 21 out of 50 consecutive patients were on mechanical ventilation at the time of CP transfusion. In total, 32 patients (64%) survived 30 days after CP transfusion. The survival rates were 74% and 44% in patients who received CP < 7 and ≥ 7 days after admission, respectively. While 92% of patients without mechanical ventilation survived, the survival rate of patients on mechanical ventilation was 29%. Moreover, the CT scan score and some other clinical features were improved in the group that received CP transfusion, and no adverse effects were observed. Conclusion: The CP transfusion is a safe and effective treatment in severe/critically ill COVID-19 patients. The best outcome can be achieved in patients who are not on mechanical ventilation, especially early in the disease course.

Clinicopathological Parameters of Haemolytic Anaemia in COVID-19 Infection: A Series of Three Cases

Coronavirus Disease 2019 (COVID-19) patients show various haematological abnormalities like cytopenia and coagulation disorders. Corona virus can induce an inflammatory state, leading to extensive coagulation manifestations. Association between COVID-19, Autoimmune Haemolytic Anaemia (AIHA) and thrombotic state is still the subject of extensive research. In this study, three cases of haemolytic anaemia are discussed. First case was a 28-year-old female with a history of abruptio placentae who presented with complaints of generalised weakness and oliguria for five days. She was diagnosed as thrombotic microangiopathy based on peripheral smear finding of schistocytes and spherocytes and few polychromatophils and normal prothrombin time (International Normalized Ratio (INR)) with very high D-dimer levels on coagulation profile. Second case was of a 25-year-old female who presented with complaints of fatigue, rashes, dark urine, nausea and abdominal pain. She was diagnosed as a case of AIHA based on peripheral smear finding of Red Blood Cells (RBC) clumping and positive direct coomb test. Third case was of a two-month-old child who presented with respiratory distress and pallor. He was diagnosed as a case of haemolytic anaemia either due to direct effect of COVID-19 infection or Cytomegalovirus (CMV) and mycoplasma infection. Thus, COVID-19 infection can directly or indirectly lead of a wide spectrum of haemolytic manifestations and every patient with anaemia should be thoroughly investigated for early detection and treatment.

An Unusual Case of Pancytopenia

Introduction: HLH should be considered in patients with underlyingautoimmune disorder, immunodeficiency or infection presenting withfever, pancytopenia, organomegaly.Aims &Objectives: Treatable cause of pancytopenia in post-covidpatient must be evaluated.Materials &Methods: A 58 yr female, known case of Type IIDiabetes Mellitus, Hypertension and Rheumatoid Arthritis on treatment for 10 years;and Severe COVID-19 pneumonia 2 months back,presented with generalized weakness and easy fatiguability for1.5 months and fever for 1 month.Patient had no history of cough,breathlessness, recent increase in joint pain, rash, bleeding or anyother localizing sign of infection.On examinations patient was febrile (101.7 F) with PR-108/min andBP-150/88 mm.Pallor was present, Rest all general and systemicexamination finding was unremarkable. On Investigating patient wasfound to have pancytopenia without Neutrophylic/lymphocyticreversal, with correct retic count of 3.3%, Ferritin-628 ng/ml,LDH-370 U/L, Fasting Triglyceride-310 mg/dl, Procalcitonin-0.5 ng/dl, Other causes of pancytopenia were ruled out.Result: Bone marrow aspiration was done which showed hypercellular particulate marrow with many macrophage exhibitingerythrophagocytosis. HLH 2004 Diagnostic Criteria score was 5/8suggestive of HLH. H-Score of the patient 183. Patient was treatedwith oral prednisolone rest of the complains was managed symptomatically. Fever and pancytopenia improved and patient wasdischarged with stable vitals.Conclusions: HLH is a rare syndrome in adult. Delay in diagnosisdue to lack of suspicion and non-specific clinical and lab findings. AllHLH patient dont need aggressive chemotherapy. Clinicallystable patients should undergo treatment for the trigger.

Acquired Aplastic Anemia in Immunocompetent Pediatric Patients with Asymptomatic COVID-19 Infection: A Case Series

Background: In pediatrics, acquired aplastic anemia (AA) is most commonly due to infection, particularly viruses, when a cause can be identified. Coronavirus disease 2019 (COVID-19) has affected more than 197 million people worldwide, and children typically experience a less severe disease course. COVID-19 is known to cause transient hematologic abnormalities, including leukopenia, lymphopenia, anemia and thrombocytosis or thrombocytopenia in severe cases. Objectives: Describe three cases of COVID-19 associated acquired aplastic anemia in immunocompetent pediatric patients. Design/Methods: Case series established by retrospective review of the electronic medical record. Results: Case 1: An 8-year-old Hispanic male presented with a three-week history of increased bruising and a one-week history of progressive exercise intolerance, shortness of breath, pallor and fatigue. Labs showed pancytopenia. Bone marrow aspirate and biopsy was markedly hypocellular at 5-10% consistent with aplastic anemia (Figure 1). Work-up for the etiology of his aplastic anemia was only significant for positive SARS-COV-2 antibodies and a SEC23B variant of unknown significance on a comprehensive bone marrow failure (BMF)/myelodysplastic syndrome (MDS)/leukemia panel from the Children's Hospital of Philadelphia (CHOP). He was treated with eltrombopag olamine and then proceeded to immunotherapy with cyclosporine (CsA) and horse antithymocyte globulin (ATG) when a sibling match was not identified for hematopoietic stem cell transplant (HSCT). Three months later, his peripheral blood counts have improved, and he is no longer transfusion-dependent. Repeat bone marrow aspirate and biopsy continues to show markedly hypocellularity at <5%. Case 2: A 5-year-old non-Hispanic white female presented with a two-week history of easy bruising, petechial rash, fatigue and bone pain. Labs showed pancytopenia, and bone marrow aspirate and biopsy showed marked hypocellularity at 5-10% consistent with aplastic anemia (Figure 2). Her aplastic anemia work-up was significant for positive SARS-COV-2 antibodies and subclinical RBC and WBC paroxysmal nocturnal hemoglobinuria (PNH) clones. She was started on eltrombopag olamine and then proceeded to immunotherapy with CsA and ATG when a matched sibling donor was not identified. Three months later, she continues to be severely neutropenic, anemic and thrombocytopenic requiring multiple transfusions. Repeat bone marrow aspirate and biopsy showed variable cellularity with some areas 10-20% and others 70% with an overall cellularity of 50%. Case 3: An 8-year-old non-Hispanic white female presented with a 10-day history of fatigue, bilateral leg pain and pallor. Labs showed pancytopenia, elevated inflammatory markers and elevated hemoglobin F. Bone marrow aspirate and biopsy demonstrated mild-moderate hypocellularity at 40-50%, left-shifted myelopoiesis and dyspoiesis in the erythroid and megakaryocytic cell lines (Figure 3). MDS and acute lymphoblastic leukemia (ALL) fluorescence in situ hybridization (FISH) panels were negative. Additional work-up revealed positive SARS-COV-2 antibodies. Her pancytopenia resolved within two weeks of her initial hospitalization. Four months later, she presented with increased bruising and fatigue. Labs showed leukocytosis, thrombocytopenia, anemia and circulating peripheral blasts. Bone marrow aspirate and biopsy was consistent with B-cell ALL. She is receiving chemotherapy on study COG AALL1732. Conclusion: Severe aplastic anemia (SAA) has high morbidity and mortality, and timely diagnosis is needed for appropriate treatment. Multiple different viral infections have been known to cause acquired aplastic anemia. Data on all the sequelae of COVID-19 infection is still emerging, but it is plausible that COVID-19 infection may cause SAA. All three patients were found to have positive COVID-19 antibodies but did not have any evidence of previous COVID-19 infection. Further research and follow-up is needed to determine if previous COVID-19 infection is indeed a risk factor for development of S A. [Formula presented] Disclosures: No relevant conflicts of interest to declare.

Secondary acrocyanosis: Case report

Introduction: Acrocyanosis is a painless condition characterized by discoloration of different shades of blue in the distal parts of the body, marked by symmetry and persistence of color changes exacerbated by cold exposure. It is less common than other acrosyndromes (erythromelalgia, Raynaud's phenomenon, erythema pernio) According to its ethiology could be classified: a) Primary: mostly in young adults. B) Secundy: anorexia nervosa, mitochondrial diseases, neoplasms, connective tissue/rheumatologic disorders, cryoglobulinemia, exposure to drugs among others. We describe a case of secondary acrocyanosis. Clinical case: A 15-year-old boy with atopic dermatitis since childhood, was referred by Dermatology to rule out a possible contact eczema in hands, four months long. In the outpatient allergy office he showed painless cold symmetrical erythematous-violaceous macules affecting fingers and hands that aggravated by cold exposure. There was also local hyperhidrosis on a scaly-itchy base in distal areas of both hands without paroxysmal pallor or chilblains. These symptoms had been more evident a month ago and did not respond to topical corticosteroids and anti-H1. Neurologist had prescribed four weeks before amitriptyline to treat headache. Material and methods: Patch test with the standard series of the Spanish Contact Dermatitis and analysis for acrocyanosis screening were performed. Results: Patch tests with the standard battery: negative. Hemoglobin concentration: 16 g/dl, white blood cell count: 5.000/ mm3, platelet: 182.000/mm3, erythrocyte sedimentation rate: < 10 mm/h. Coagulation, urinalysis, immunoglobulin concentrations, C3, C4: normal. Cryoagglutinins, rheumatoid factor, anti-double stranded DNA antibody (Anti-ds-DNA), anti-cardiolipin and anti-Scl 70 antibodies: absents. COVID-19 RT-PCR-test: negative. Lues, HIV and Hepatitis B, C serologies:negatives. Arterial blood gas: pH: 7.43, PaO2: 113 mmHg, PaCO2: 31 mmHg. Chest radiographs: normal. Amitriptyline treatment was discontinued after suspicion of being the causal agent and 15 days later hands were pink and warm. Conclusions: We report a case of secondary acrocyanosis due to amitriptyline, a serotonin reuptake inhibitor. We emphasize the importance of a detailed clinical history to achieve the diagnosis and to verify the reversibility of acrocyanosis when its etiology is due to medication. Physicians should be aware of this possible side effect of tricyclic antidepressants.