Laboratory Profile Research Articles

The Correlation of Human Leukocyte Antigen (HLA) Haplotypes with Clinical and Laboratory Profiles of Celiac Disease in Children

The Correlation of Human Leukocyte Antigen (HLA) Haplotypes with Clinical and Laboratory Profiles of Celiac Disease in Children

Study of clinico-etiological and laboratory profile of chronic liver disease in children and adolescents in tertiary care center

Study of clinico-etiological and laboratory profile of chronic liver disease in children and adolescents in tertiary care center

Profile of patients referred for lung transplant and their transplant-free survival.

Lung transplant (LTx) is a potential treatment option for all patients with chronic, end-stage respiratory disease, who are refractory to optimal medical therapy or where no medical therapy exists. In India, LTx is still in its evolving stages and published literature is sparse. The current study was carried out to study the selection criteria for lung transplant and to evaluate the clinical and socio-economic profile of patients referred for the same at a tertiary health care facility. The study was a descriptive, prospective, observational study. All adults referred for lung transplant were evaluated for clinical and laboratory profiles. All enrolled patients were assessed for presence of referral criteria, listing criteria, contraindications, and willingness for lung transplant. These patients were followed up for 2 years for transplant-free survival, and the Cox proportional hazards model was used to determine independent predictors of all-cause mortality. A total of 103 were included in study. The most common diagnosis was interstitial lung disease (57.2%), followed by bronchiectasis (17.5%) and COPD (13.6%). Most patients were referred for LTx at an advanced stage as 90% met listing criteria. Fifty-four (52.4%) patients had an absolute or relative contraindication to transplant; however, the majority of those contraindications were modifiable. Patients with a lower socio-economic status were less likely to be willing for LTx. The median survival was 757 days. A 6-minute walk distance (6MWD) lesser than 250 m was found to be an independent predictor of mortality. Making patients aware about lung transplant early in their treatment may give them sufficient time to come to terms with their disease and understand the risk and benefits associated. Efforts should be focused on screening and early treatment of reversible contraindications for the eligible patients. Patients with 6MWD < 250 m are at increased risk of mortality.

Comparative analysis of prognostic scoring systems in predicting severity and outcomes of Omicron variant COVID-19 pneumonia.

The global spread of Coronavirus Disease 2019 (COVID-19) underscores the urgent need for reliable methods to forecast the disease's severity and outcome, thereby facilitating timely interventions and reducing mortality rates. This study focuses on evaluating the clinical and laboratory profiles of patients with Omicron variant-induced COVID-19 pneumonia and assessing the efficacy of various scoring systems in prognosticating disease severity and mortality. In this retrospective analysis, we examined the clinical records of 409 individuals diagnosed with Omicron variant COVID-19 pneumonia. We documented the Pneumonia Severity Index, CURB-65, and MuLBSTA scores within the first 24 h and analyzed the sensitivity, specificity, positive predictive value, negative predictive value, and the area under the receiver operating characteristic curve for each scoring system to ascertain their predictive accuracy for disease severity and fatality risk. The cohort's median age was 78 years, predominantly presenting with fever, cough, expectoration, fatigue, and gastrointestinal symptoms. Factors such as expectoration, fatigue, Glasgow Coma Scale score, lactate dehydrogenase levels, procalcitonin, creatinine levels, and co-occurrence of acute respiratory distress syndrome were identified as independent predictors of disease severity. Furthermore, age, oxygenation index, glucose levels, lactate dehydrogenase, and septic shock were independently associated with mortality. For severe disease prediction, the CURB-65, PSI, and MuLBSTA scores demonstrated sensitivities of 65.9%, 63.8%, and 79.7%, respectively, with specificities of 63.8%, 76.8%, and 60.9%, and AUROCs of 0.707, 0.750, and 0.728. To predict mortality risk, these scores at cutoffs of 1.5, 102.5, and 12.5 exhibited sensitivities of 83.3%, 96.3%, and 70.4%, specificities of 59.4%, 60.8%, and 65.4%, and AUROCs of 0.787, 0.850, and 0.736, respectively. The study cohort predominantly comprised elderly individuals with pre-existing health conditions. Elevated lactate dehydrogenase emerged as a significant marker for both disease severity and prognosis, sputum production, gastrointestinal symptoms, GCS score, creatinine, PCT, and ARDS as independent predictors of disease severity, and age, oxygenation index, glucose levels, and septic shock as independent mortality predictors in COVID-19 pneumonia patients. Among the scoring systems evaluated, Pneumonia Severity Index demonstrated superior predictive capability for both disease severity and mortality, suggesting its utility in forecasting the clinical outcomes of Omicron variant COVID-19 pneumonia.

Hormonal and Laboratory Profile of Polycystic Ovarian Syndrome (Pcos) Women Visiting Fertility Center in Indonesia

Our study aims to scrutinize the hormonal and laboratory profile of women with PCOS visiting a fertility center in Indonesia. A computerized database of 120 PCOS women and 120 controls was retrospectively reviewed and analyzed. According to analysis, the concentration of AMH and basal LH were significantly more elevated in the PCOS group than in the control group (6.77 vs. 2.05, P&lt;0.001; and 6.8 vs. 5.3, P&lt;0.001, respectively). The level of basal FSH was significantly lower in the PCOS group (5.83 vs. 7.86; P&lt;0.001). After an IVF cycle, PCOS women had significantly higher retrieved oocytes and mature oocytes compared to controls (18 vs. 8, P&lt;0.001; 12 vs. 6, P&lt;0.001). The number of fertilizations, cleavages, and top-quality cleavages were also higher in the PCOS group than in the control group (10 vs. 4, P&lt;0.001; 10 vs. 4, P&lt;0.001; 4 vs. 2, P&lt;0.001). At the blastocyst stage, patients with PCOS had a higher number of blastocysts and top-quality blastocysts than control (9 vs. 4, P&lt;0.001); 4 vs. 1, P&lt;0.01). However, the clinical pregnancy was significantly higher in the control group than in the PCOS group (52.6% vs. 16.7%; P = 0.045).

Clinical and Laboratory Profile of Dengue Syndrome in Pediatric patients: Study in a Tertiary Care Hospital, Dhaka, Bangladesh

Introduction: Dengue fever is a mosquito-borne arboviral (arthropod-borne virus) disease which is of global concern. This fever is a benign syndrome caused by an arthropod-borne virus and is characterized by Biphasic fever, myalgia, arthralgia, rash, leukopenia, and lymphadenopathy. Aim of the study: This study aimed to evaluate the clinical and laboratory profile of dengue fever in children less than 15 years old admitted to an urban hospital. Methods: This retrospective study was conducted in the Department of Paediatrics of Anwer Khan Modern Medical College Hospital (AKMMCH), Dhaka, Bangladesh during the period from 4 April 2019 to 9 September 2019. They have followed up on the symptoms, platelet count and management of the patients. Data were analysed by using MS-Excel-2016. Result: In total 98 patients from both groups completed the study. In our study, we found a maximum of 54.08% were male, more than half 53(54.08%) patients were below five years of age, about 95(96.94%) of the patients had a fever. Maximum patients, 81(82.65%), were managed by I/V fluid, more than 72(73.47%) of the patients were managed by paracetamol. Others by antibiotics 16(16.33%), platelet transfusion 14(14.29%) and blood transfusion 12(12.24%). Other management also includes plasma, &amp; dopamine. Conclusion: These study children about 0-15 years of age more commonly suffer from dengue fever. As the vaccine is not available in Bangladesh, these patients need a timely diagnosis and critical monitoring during the disease course to prevent severity and mortality.

CLINICAL PROFILE OF EXPANDED DENGUE SYNDROME IN CHILDREN.

The objective is to study the clinical profile and outcome of expanded dengue syndrome (EDS) in children. We conducted a retrospective cohort study and enrolled consecutive children (1 month to 18 years) who presented with dengue fever for over 1 year. The diagnosis of dengue fever was confirmed by the presence of dengue NS1 antigen and/or dengue IgM positivity. Subsequently, we identified children exhibiting EDS according to the revised World Health Organization guidelines (2011). We compared the clinical and laboratory profiles of children diagnosed with EDS and those without EDS. Of 178 children with dengue fever, 33 (18.5%) had EDS [95% confidence interval (CI): 13.1%-25.0%]. In these 33 children, neurological involvement was the most common manifestation [n = 16/33 (49%, 95% CI: 32%-65%)], namely encephalopathy, febrile seizures and encephalitis, respectively. Gastrointestinal involvement was the second most common [n = 10/33 (30%, 95% CI: 17%-47%)], which included fulminant hepatic failure, acalculous cholecystitis and acute pancreatitis. Renal presentation [n = 7 (21%, 95% CI: 10%-37%)] was limited to acute kidney injury. Children with EDS were significantly anemic and had leukocytosis, hyperkalemia, azotemia, hyperbilirubinemia, raised serum transaminases and fluid-refractory shock than their non-EDS counterparts. Children with EDS had a trend toward higher mortality (P = 0.07) and the survivors had a longer duration of hospital stay (5 days vs. 4 days in non-EDS, P = 0.001). In conclusion, we observed a high prevalence (18.5%) of EDS among children hospitalized for dengue fever. The common manifestations of EDS include neurological, renal and gastrointestinal involvement. Children with EDS showed a trend toward higher mortality and longer duration of hospital stay than children without EDS.

Clinical and Laboratory Profile of Macrophage Activation Syndrome in Kawasaki Disease: A Single Centre Cross-Sectional Study

Clinical and Laboratory Profile of Macrophage Activation Syndrome in Kawasaki Disease: A Single Centre Cross-Sectional Study

Predictive factors of hyponatremia in subarachnoid hemorrhage and outcomes

Background &amp; Objective: The prevalence of hyponatremia in subarachnoid hemorrhage (SAH) was 30-55%. There was lack of evidence about predictive factors and clinical outcomes of hyponatremia in SAH patients. This study aimed to evaluate factors associated with hyponatremia following SAH and to review the clinical outcomes and treatment of hyponatremia in SAH patients. Method: This is a retrospective cohort study. SAH patients presented during January 2013 to January 2019 were reviewed. They were divided into 2 groups: SAH with normonatremia and SAH with hyponatremia. Clinical data, laboratory profile, aneurysm characteristics and clinical outcomes were recorded and analyzed. Result: A total number of 278 patients with SAH were included, 139 patients in each group. The populations were female 66% with mean age of 56 years old and mean serum sodium (Na) level 132 mmol/L. Aneurysm location associated with hyponatremia; anterior cerebral artery (ACA) (OR 4.2, 95%CI 1.4-13.0, p-value 0.009) and posterior cerebral artery (PCA) (OR 3.7, 95%CI 1.2-11.5, p-value 0.017). Aneurysms clipping procedure was also associated with hyponatremia (OR 4.0, 95%CI 1.8-8.8, p-value &lt; 0.001). Conclusion: ACA and PCA aneurysms and aneurysms clipping procedure were risk factors for hyponatremia following SAH. Mild hyponatremia was not associated with morbidity and mortality in SAH patients.

A Cross-Sectional Study of Congenital Adrenal Hyperplasia.

Congenital adrenal hyperplasia is an autosomal recessive disease caused by the deficiency of one of the enzymes of adrenal steroidogenesis, the most common of which is the deficiency of 21-hydroxylases. It represents a significant cause of morbidity and mortality in the pediatric population, especially in the absence of systematic neonatal screening in Morocco, which makes the management of these patients difficult for clinicians. This study aimed to describe the epidemiological, clinical, laboratory, evolutionary, and therapeutic profile of children followed for congenital adrenal hyperplasia at the pediatric endocrinology unit, Abderrahim Harrouchi Children's Hospital, Casablanca, Morocco. Materialsand methods: A retrospective cross-sectional study including 184 children followed for congenital adrenal hyperplasia over a period of 11 years (from January 1, 2013, to December 31, 2023). The diagnosis was confirmed by molecular biology, and all clinical, laboratory, and radiological data were collected retrospectively from medical records. The median age at diagnosis was1.5 months (birth: 13 years). The consanguinity rate was 54.4% (n=100). A history of death in the family was found in 16.3% (n=30) of cases in a table of salt wasting and infections. The classic form was observed in 72% (n=132) of children compared to 28.3% (n=52) for the non-classical form. The virilizing form with salt wasting and the pure virilizing form represented 45.6% (n=84) and 26% (n=48) of cases, respectively. Deficiency in 21-hydroxylase was found in 91.8% (n=169) of children, while deficiency in 11-β-hydroxylase was identified in 4.9% (n=9) of cases, and in 3-β-hydroxysteroid dehydrogenase in 3.2% (n=6) of cases. A total of 40.7% (n=75) of children underwent corrective surgery of the external genitalia. Congenital adrenal hyperplasia is a group of rare diseases. The best therapeutic alternative would be newborn screening and antenatal diagnosis.

Comparing FGFR-3 and TS-HDS Seropositive Small Fiber Neuropathy: Unique Patient Features, Symptoms, Laboratory, and Nerve Conduction Study Findings.

Small fiber neuropathy presents a significant diagnostic and therapeutic challenge. To solve this challenge, efforts have been made to identify autoantibodies associated with this condition. Previous literature has often considered tri-sulfated heparin disaccharide (TS-HDS) and fibroblast growth factor receptor 3 (FGFR3) as a singular seropositive group and/or focused primarily on symptomatic associations. One hundred seventy-two small fiber neuropathy patients with a Washington University Sensory Neuropathy panel were selected for TS-HDS seropositivity, FGFR-3 seropositivity, and seronegative controls. Data were collected to on the demographic, symptomatic, and laboratory profiles of each subgroup. Percent female (P = 0.0043), frequency of neuropathic pain symptoms (P = 0.0074), and erythrocyte sedimentation rate (P = 0.0293), vitamin D (P < 0.0001), and vitamin B12 (P = 0.0033) differed between the groups. Skin biopsy was more frequently normal within both the FGFR-3 and the TS-HDS cohort (P = 0.0253). TS-HDS and FGFR-3 display a distinct phenotype from both controls and one another. Immunoglobulin M (IgM) against FGFR-3 and IgM against TS-HDS may be individually valuable markers for the development of distinct clinical phenotypes.

Analysis of COVID-19 Infection in Kidney Transplant Recipients

BackgroundThe Coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2 was responsible for higher morbidity and mortality in renal transplant recipients (RTx). The objective of the study was to evaluate the impact of COVID-19 infection on RTx in a single center in Brazil. MethodsA cohort of 135 RTx was evaluated between December 2019 to June 202l and demographics, clinical and, laboratory profiles were analyzed from deceased donors with COVID-19. ResultsDiabetic and RTx from extended criterion donors presented more frequently the severe form of the disease. Serum creatinine (sCr) after 3 months of diagnosis of COVID-19 varied according to the severity of infection. The lethality rate was higher in the group with severe symptoms (65%) compared with those with mild infection (1.5%). ConclusionThe increase in sCr was associated with disease severity. The lethality rate for COVID-19 was 26.6%. These rates are 10 to 20 times higher than those reported in the general population and suggest that rigorous observation, early diagnosis, and disease prevention measures are crucial in RTx.

Evaluation of clinical and laboratory characteristics of dengue viral infection and risk factors of dengue hemorrhagic fever: a multi-center retrospective analysis

BackgroundDengue Viral Infection (DVI) has become endemic in Pakistan since the first major outbreak in Karachi in 1996. Despite aggressive measures taken by relevant authorities, Pakistan has been dealing with a worsening dengue crisis for the past two decades. DHF is severe form of dengue infection which is linked with significant morbidity and mortality. Early identification of severe dengue infections can reduce the morbidity and mortality. In this context we planned current study in which we find out the different factors related with DHF as well as clinical laboratory features of DHF and compare them to DF so that patients can be best evaluated for DHF and managed accordingly at admission.MethodsRetrospective study conducted over a period of 6 years (2013–2018) in two tertiary care hospitals in Pakistan. Data were collected by using a pre-structured data collection form. Data were statistically analyzed to determine the clinical and laboratory characteristics of DVI and risk factors of dengue hemorrhagic fever (DHF).ResultsA total 512 dengue cases (34.05 ± 15.08 years; Male 69.53%) were reviewed. Most common clinical manifestations of DVI were fever (99.60%), headache (89.1%), chills (86.5%), rigors (86.5%), myalgia (72.3%). Less common clinical manifestations were vomiting (52.5%), arthralgia (50.2%) and skin rashes (47.5%). Furthermore, nasal bleeding (44.1%), gum bleeding (32.6%), pleural effusion (13.9%) and hematuria (13.1%) were more profound clinical presentations among DHF patients. Mortality rate was 1.5% in this study. Logistic regression analysis indicated that delayed hospitalization (OR: 2.30) and diabetes mellitus (OR:2.71), shortness of breath (OR:2.21), association with risk groups i.e., living near stagnant water, travelling to endemic areas, living in endemic regions (OR:1.95), and presence of warning signs (OR:2.18) were identified as risk factors of DHF. Statistically we found that there is strong association of diabetes mellitus (DM) with DHF while the patient suffering from DM individually had higher odds (2.71) of developing DHF than patients without disease.ConclusionsThe current study demonstrated that the clinical and laboratory profiles of DF and DHF are significantly distinct. Significant predictors of DHF were advanced age, diabetes mellitus, ascites, pleural effusion, thick gallbladder and delayed hospitalization. The identification of these factors at early stage provides opportunities for the clinicians to identify high risk patients and to reduce dengue-related morbidity and mortality.

Clinical and laboratory profiles of systemic lupus erythematosus patients in a new rheumatology clinic in southwestern Nigeria.

The aims were to study the sociodemographic characteristics of patients presenting to the clinic and to study the clinical and serological pattern of systemic lupus erythematosus (SLE) in a new rheumatology clinic of a predominantly Yoruba population. This was a retrospective, cross-sectional study conducted over 7 years (January 2017 - December 2023). Patients who satisfied the 1997 American College of Rheumatology (ACR) and/or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria were enrolled using their medical records. Patients with overlap syndromes and other inflammatory or noninflammatory rheumatic diseases were excluded from the study. Their sociodemographic, clinical, laboratory, and treatment data were retrieved from their medical records and analysed using IBM SPSS version 23.0 software. A total of 65 patients were diagnosed with SLE with a frequency of 15.8%. The mean age ±SD of the patients at presentation was 33.85 years ±11.01 and the female to male ratio was 9.8 : 1. The median (IQR) duration of symptoms at presentation was 7.0 months (3-24). The common clinical presentations included synovitis (86.2%), acute cutaneous rash (53.8%), oral ulcers (52.3%), nonscarring alopecia (50.8%), and serositis (47.7%). Proteinuria was seen in 37.7% of the patients and the predominant renal histopathological feature was Class IV. Antinuclear antibody was 100% positive with 50.94% of the patients having a titre of 1 : 5,120 and above. Anti-double-stranded deoxyribonucleic acid and anti-Smith antibodies each had 50% prevalence. Dyslipidaemia was found in 76.7% of the patients. The study's findings are largely consistent with similar studies done in Africa. Further prospective multi-centred studies are needed to further determine the epidemiological characteristics of the disease in Nigeria with a multi-ethnic population.

CLINICAL AND LABORATORY PROFILE OF PATIENTS WITH HEPATITIS B INFECTION ATTENDING THE OUTPATIENT DEPARTMENT OF A TERTIARY CARE HOSPITAL IN NORTHEAST INDIA

Objective: The objective of this study was to analyze the clinical and laboratory profile of patients with hepatitis B infection-attending outpatient departments of tertiary care hospitals in northeast India. Methods: This was a cross-sectional study conducted in the outpatient department of a tertiary care medical institute situated in northeast India. Eighty patients infected with hepatitis B (on the basis of serological markers) were included in this study on the basis of predefined inclusion and exclusion criteria. Demographic details such as age, gender, and socioeconomic status of all the patients were noted. A detailed history was taken including any history of intravenous drug use, tattooing, sexual contacts, history of blood transfusions, and familial liver disease. Clinical and laboratory profiles (liver function test and serological markers) of all the patients were studied. Ultrasound imaging was done in all the cases. p&lt;0.05 was taken as statistically significant. Results: Out of 80 studied cases, there were 49 (61.25%) males and 31 (38.75%) females. The mean age for males and females was 32.30±8.03 and 34.70±13.52 years, respectively. There was no significant difference in mean age between males and females (p=0.3218). Mother-to-child transmission was evenly distributed with 7 males (8.75%) and 9 females (11.25%) affected. Other risk factors, such as dental implants and surgeries, showed a lower prevalence, with dental implants impacting 2 males (2.5%) and 3 females (3.75%) and surgeries concerning 5 males (6.25%) and 3 females (3.75%). Majority of the patients (52.5%) were asymptomatic and were found to be hepatitis B surface antigen (HBsAg) positive incidentally. HBsAg was positive in all cases. 12 (15%) patients showed the presence of hepatitis B e antigen suggestive of high infectivity. Anti-HBc and anti-HBe were positive in 68 (85%) patients each. Hepatomegaly was seen in 23 cases (28.75%) on ultrasound. Moderate fibrosis on elastography was seen in 2 (2.5%) cases. Conclusion: In cases of hepatitis B, the majority of the patients remained asymptomatic, and many cases no risk factor for hepatitis B virus infection could be identified. It is therefore important to screen the general population for hepatitis B infection.

Clinical Manifestations of Dengue in Children and Adults in a Hyperendemic Region of Colombia.

Dengue is the most common arboviral disease in the world. Traditionally, it has affected more adults, but the incidence in children has increased in recent years. Colombia is no stranger to this change; therefore, we aimed to find the differences in signs, symptoms, and clinical, hematological, and hemogram characteristics between children under 12 years old and individuals aged 12 years and older in an endemic region of Colombia in 2020-2022. The analyses were conducted with baseline data, corresponding to a cross-sectional design. Multiple correspondence analysis was used for general, dermatological, and clinical symptom profiles. Discriminant analysis was used for laboratory profiles. Multiple correspondence analysis was applied to nominal categorical data, employing Euclidean distances to analyze age groups. Discriminant analysis was applied to a training sample and validated on a test sample. The overall agreement of the model's discrimination, sensitivity, specificity, and fit indicators was calculated. The results indicated that individuals under 12 years exhibited distinct dermatological and clinical features, including rash, pruritus, hypotension, lymphocyte count, and platelet count, compared with those aged 12 years and older. In contrast, those 12 years and older were profiled for general and clinical symptoms such as pain (back pain, retro-orbital pain, headache), dizziness, chills, hematuria, tachypnea, and elevated/high hematocrit, hemoglobin, and basophil values. These findings are crucial to understanding the high incidence in children; they also facilitate rapid understanding of the disease in clinical care settings and differentiate it from other febrile outbreaks. This will affect disease control, particularly in severe cases, and reduce mortality.

Clinical and Laboratory Profile, Management and Outcome of Pediatric SARS-CoV-2 Infection Admitted at the Philippine General Hospital.

The Philippines has recorded over 470,000 COVID-19 cases in children, with over 1,500 deaths during the same period. Although a Philippine online pediatric COVID-19 registry exists, this only relies on passive surveillance. This study determined the clinical and laboratory profile, risk factors for severe COVID-19, and mortality, management, and outcome of pediatric SARS-CoV-2 patients admitted at the Philippine General Hospital (PGH) from April 2020 to June 2022 to fill in knowledge gaps on the epidemiology of COVID-19 infection in children. This was a retrospective cohort study of pediatric COVID-19 cases admitted at the PGH, a designated COVID referral center during the study period. Demographic and clinical profile, risk factors, comorbidities, laboratory and radiologic findings, management, and outcomes across different disease severity states were gathered by chart review and the data were analyzed using STATA 17. There were 448 pediatric patients admitted and diagnosed with COVID-19 during the study period. Most patients belonged to the 1-5-year age group (25.9%), had no known exposure to a COVID-19 case (65.4%), were mild cases (37.3%%), and did not receive any dose of the COVID-19 vaccine (96.7%). The most common presenting symptoms across all disease categories were fever (44.4%), cough (28.6%), and shortness of breath (26.6%). Multisystem inflammatory syndrome in children (MIS-C) presented with fever (100%) and rash (53.9%). The risk factors for severe disease were the presence of a congenital anomaly lung disease, and elevated procalcitonin. Most patients with MIS-C were previously well with no comorbidities. Laboratory findings which were markedly elevated among severe and critical cases were ESR, CRP, D-dimer, LDH, and IL-6. Ferritin, procalcitonin (PCT) and IL-6 were elevated only in severe to critical COVID-19 cases and remained within normal for the other disease categories. As to treatment, asymptomatic, mild, and moderate cases were given supportive medications (zinc, vitamin D, and vitamin C), while investigational drugs particularly corticosteroids, IVIG, and remdesivir, were used in severe cases.Antibiotics were given to 71.7% of patients at the outset. As to the outcomes, 89% recovered, while 8.9% died. The case fatality rate from COVID-19 infection was at 2.2%. Admitted pediatric COVID-19 cases are generally mild but admission is due to underlying illness or comorbidities. Those with severe to critical cases have underlying comorbidities and had either progression or complications due to COVID disease. D-dimer, LDH, IL-6, ferritin and procalcitonin were elevated among severe and critical cases which can be utilized as inflammatory markers.

Pyruvate kinase deficiency: A case series of congenital non-spherocytic hemolytic anemia

BackgroundPyruvate kinase deficiency (PKD) is a common cause of congenital non-spherocytic hemolytic anemia (CNSHA). The heterogeneity of clinical presentation complicates the diagnosis and management of this disease. The objective was to describe the clinical, laboratory, and genetic profile of pediatric patients with PKD. Material and methodsIt was a retrospective, observational study. Children and their family members presenting to the Department of Pediatric Hematology and Oncology, St John's Medical College Hospital Bangalore, India, and diagnosed with PKD during 2011–2023 were enrolled. ResultsEight patients were identified through retrospective chart reviews. Four cases were clustered in one family and affected twins in the second family. All patients had a history of neonatal jaundice; however, they varied in their severity and the requirement for transfusion support. Some patients underwent splenectomy and one had hematopoietic stem cell transplant. ConclusionPKD is a known cause of congenital non-spherocytic hemolytic anemia with heterogeneous presentation. A high index of suspicion, enzyme assay, and genetic studies are required for diagnosis.

The microbiological profile of necrotising fasciitis at a secondary level hospital in Gauteng.

Necrotising fasciitis (NF) is a fulminant soft tissue infection that requires timely diagnosis, urgent surgical debridement, and appropriate antimicrobial therapy. The choice of empiric antimicrobial therapy depends on the microorganisms cultured and the antimicrobial resistance profile of the institution. Necrotising fasciitis has not been studied in our setting. The aim of the study was to audit the microbiological profile of NF and antimicrobial susceptibility profile. This was a retrospective study in a secondary level hospital from the period of 2014-2020. The patients' demographic data, clinical features, location of infection, comorbidities, laboratory and microbiological profiles were analysed. There were 53 patients during 2014-2020 with median age of 45.5 (38.5-56.0) years. The majority of the patients were males (35 [66.04%]), had no comorbidities (25 [47.17%]), and the lower limb was the most common anatomic site (17 [32.08%]). Type II (monomicrobial) NF was the predominant type (31 [58.49%]). Staphylococcus aureus was the most prevalent Gram-positive bacteria (18 [38%]) and Escherichia coli, the main species isolated in the Gram-negative bacteria (14 [36%]) with susceptibility to cloxacillin (94%) and amoxicillin and/or clavulanic acid (92%), respectively. Staphylococcus aureus and Escherichia coli were the most common bacteria with low rate of antimicrobial resistance. Amoxicillin and/or clavulanic acid and an adjunctive clindamycin are appropriate antimicrobial therapy for empiric treatment for NF in our setting. Amoxicillin and/or clavulanic acid and an adjunctive clindamycin can be used as an empiric treatment for NF.

Male patients with primary Sjögren's syndrome have unique clinical manifestations and circulating lymphocyte profiles.

We aimed to explore the relationship between clinical characteristics and circulating lymphocyte profiles in Chinese male patients with primary Sjögren's syndrome (pSS). Data from 397 patients with pSS were analyzed retrospectively. 37 were male, which is a prevalence of 9.3%. The clinical, laboratory, and immunophenotypic profiles of peripheral blood lymphocyte subsets were compared between male and female pSS patients. Male patients with primary Sjögren's syndrome have unique clinical manifestations and circulating lymphocyte profiles. Male patients complained more about xerophthalmia and presented with more extra-glandular manifestations as compared with female patients. The CD4+/CD8+ ratio (P = 0.030), the prevalence of CD4-CD8- T cells in lymphocytes (P = 0.020), the absolute number of CD4-CD8- T cells (P = 0.035), the prevalence of CD4+ T cells in lymphocytes (P < 0.001), and the absolute number of CD4+ T cells (P = 0.023) were significantly lower in male patients compared to female patients. On the other hand, the prevalence of CD8+CD28+ T cells (P = 0.030) and CD4+CD25high T cells (P = 0.040) in lymphocytes was significantly higher in male patients than in female patients. Moreover, compared to females with pSS, an elevated serum IgG level, low C3 and C4 levels, anti-SSB positivity, and ANA titers of ≥ 1:160 positivity were more frequent in male with pSS. Male patients with pSS have distinctive peripheral blood lymphocyte subpopulations, present with more severe clinical symptoms and immunological features, and have an unfavorable prognosis. Key Points • Male patients with pSS have more severe clinical symptoms and specific characteristics of peripheral blood lymphocyte subsets. • Male pSS patients exhibit a higher intensity of the disease (as evaluated by ESSDAI). • Male patients with pSS require individualized treatment regimens and closer follow-up.