Assessment of occupational musculoskeletal disorders (MSDs) among heavy vehicle drivers.

BACKGROUND: Professional driving requires long hours of work, uncomfortable seats, negotiating rough terrain and highways, and possibly minor repairs and other auxiliary transportation duties. Heavy vehicle drivers driving vehicles such as trucks, bulldozers, etc. due to such working structures are more prone to various musculoskeletal disorders (MSDs) and pain, which is of great concern. OBJECTIVES: In the present study, it is planned to investigate possible ergonomic risk factors such as age, weight, driving exposure, seat suspension systems, lifting heavy weights causing MSDs in drivers of various heavy vehicles. The results of the study are expected to help drivers reduce the risk of MSDs. METHODS: For the present study, the Nordic questionnaire on musculoskeletal disorders was modified and standardized and was administered to the 48 heavy vehicle drivers randomly selected to collect the data. RESULTS: The analysis divulged that over the past 12 months, lower back pain (LBP) emerged as the most dominant pain experienced by 56% of drivers, followed by knee pain (KP) (43%) and neck pain (NP) (39%) respectively. The prevalence of shoulder pain (SP) was observed to be much lower than in previous literature. The logistic regression model further revealed that increasing age, poor suspension system and poor body posture were significantly associated with lower back pain. Additionally, a poor suspension system and lifting heavy weights had significant effect on the drivers' knee pain. CONCLUSION: The results demonstrated the evident necessity for ergonomic consideration in vehicle designing and ergonomic training for heavy vehicle drivers.

Clinical Spectrum, Treatment and Outcome of Children with Autoimmune Encephalitis.

OBJECTIVE: To assess the clinical spectrum, treatment, and outcome of children with autoimmune encephalitis (AE). STUDY DESIGN: Descriptive study. Place and Duration of the Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2017 to December 2021. METHODOLOGY: Medical records of children with a diagnosis of AE were reviewed for clinical features, treatment details, and outcomes. Outcome was defined as good (0-2) or poor (3-6) based on a modified Rankin Scale (mRS) score at 3-month follow-up. Descriptive statistics were reported and logistic regression was used to assess the prognostic factors associated with outcome. RESULTS: Thirty-three patients were identified with AE. Thirteen (39.3%) were antibody positive. Anti-N-methyl-D-aspartate receptor (NMDAR) antibody was seen in 92% of positive cases. Behavioural abnormalities (87.8%), seizures (81.8%), movement disorders (66.6%), psychiatric symptoms (63.6%), and mutism (33.3%) were the prominent symptoms. Thirty (91%) patients received first-line immunotherapy. Good outcome was seen in 14 (48.2%) patients. Univariable analysis showed that the odds of having poor outcome were 2.5 (95% confidence interval [CI] 0.37-16.88, p=0.34) in patients with chorea. In addition, an elevated cerebrospinal fluid (CSF) protein had an odds ratio (OR) of 8.6 (CI 0.88-84.83, p=0.064) and positive CSF antibodies had an OR of 3.7 (CI 0.79-17.72, p=0.095) for a poor outcome. Mortality was seen in 4 (12.1%) patients. CONCLUSION: A very low threshold is needed for the diagnosis of AE in children presenting with behavioural symptoms and chorea. Although the odds for poor prognosis were higher in patients with chorea, elevated CSF protein and positive CSF antibodies, the p-value did not come out significant. KEY WORDS: Autoimmune encephalitis, Antibodies, NMDAR, Immunotherapies, mRS score, Outcome.

Inner Ear Function Evaluation in Mobile Phone Users: A Cross-Sectional Study From a Tertiary Care Centre in North India.

Background India has approximately 1.02 billion mobile phone users. The electromagnetic radiations emitted by telecommunication systems are absorbed by the recipient's body, leading to changes in brain electrical activity, sensations of warmth or burning around the ear, and alterations in the blood-brain barrier. The inner ear, being the closest organ during mobile phone use, directly receives these electromagnetic radiations. This study aims to assess the inner ear function among mobile phone users, investigate the impact of mobile phones on the hearing thresholds of volunteers through pure-tone audiometry (PTA), and delve into the same using otoacoustic emissions (OAE). Methodology A cross-sectional study was conducted at a single center in North India from September 2020 to March 2021. The sample size of around 100 was determined using G Power software (G Power, Aichach, Germany), including volunteers aged 18-25, using mobile phones for over a year with normal hearing. Exclusions involved various ear-related histories or chronic systemic illnesses. Dominant and non-dominant ear groups were formed based on mobile phone usage. The study involved comprehensive ENT examinations, pure-tone audiometry, and otoacoustic emissions. We performed statistical analyses using SPSS version 22.0 (IBM Corp., Armonk, NY), which presented descriptive statistics and employed tests for group comparisons. Results Most participants were in the 21-23 age group (56%), with a mean age of 22.16 ± 1.77 years. There were 45 males and 55 females. The mean mobile phone usage was 6.6 ± 1.98 years, with varying daily durations. The dominant ear for mobile phone usage was predominantly the right ear (75 participants). Pure-tone audiometry results showed no statistically significant differences between dominant and non-dominant ears. Among the 24 participants with absent OAE, no significant association was found with mobile phone usage duration. Notably, the highest incidence of absent OAE occurred in the 120-180-minute usage category. Conclusion Mobile phones have seamlessly integrated into the lives of individuals, witnessing an exponential increase in users over time. The inner ear, situated in proximity to mobile phone usage, is of particular concern. While there is existing evidence indicating potential adverse effects of mobile phones on the inner ear, further long-term studies involving larger populations are essential to comprehensively evaluating the impact on inner ear function among mobile phone users.

Kisspeptin stimulates oestradiol biosynthesis by upregulating steroidogenic transcripts and proliferation markers in the bubaline granulosa cells in vitro.

Kisspeptin (Kp), an upstream regulator of GnRH release, is essential for the development and function of reproductive axis. Previously, we demonstrated the localization of Kp and its receptor (Kiss1r) in the active follicle in the bubaline ovary. Present study aimed to determine the effect of Kp on granulosa cell (GCs) functions, especially oestradiol (E2 ) and progesterone (P4 ) production, and differential expression of genes regulating the proliferation, apoptosis and steroidogenesis in the buffalo. The ovaries with 6-10 mm size follicles obtained from the cyclic buffaloes after slaughtering were used for isolation of GCs for in vitro study. The primary GCs culture was treated with Kp (0, 10, 50 and 100 nM) and incubated for 48 h. Production of E2 and P4 was estimated in the culture supernatant by ELISA. The expression of gonadotropin receptors (FSHR and LHR), steroidogenic genes (STAR, 3ß-HSD, CYP19A1), proliferation marker (PCNA), apoptotic factors (CASP3 and BCL2) and Kp signalling molecule (extracellular signal-regulated kinase 1/2, ERK1/2 and p-ERK1/2) was studied in the GCs by qPCR. Significant E2 production was found in the Kp 50 and 100 nM groups (p < .05), whereas P4 production was reduced in Kp 100 nM group (p < .05). There was concomitant upregulation of FSHR, ERK1/2, STAR and CYP19A1 in the Kp 100 nM treated GCs. In addition, Kp at 100 nM stimulated the proliferation of GCs by upregulating the expression of BCL2 (5.0 fold) and PCNA (94.9 fold). Further, high immunoreactivity of p-ERK1/2 was observed in the Kp-treated GCs. It was concluded that Kp at 100 nM concentration stimulated E2 production by upregulating the steroidogenic pathway through ERK1/2, STAR and CYP19A1 and modulating PCNA and BCL2 expressions in the GCs. Further experiments are warranted using Kp antagonist in different combinations to establish the signalling pathway in Kp-mediated steroidogenesis in the GCs for developing strategies to control ovarian functions.

Diagnostic dilemmas and challenges in the management of myasthenia in infants and toddlers: A case report.

Myasthenia in the infancy and toddler age group is rare and often presents a challenge to treating pediatric neurologists. Our report addresses the challenges encountered when distinguishing myasthenia in infants and toddlers from similar illnesses, as well as the differentiation between congenital myasthenia, transient myasthenia, and autoimmune myasthenia. We present four cases of myasthenia between the ages of 10 and 30 months. The diagnosis and management of these cases were challenging due to the variability in clinical presentation. Four cases of myasthenia were diagnosed, with three having autoimmune myasthenia and one having congenital myasthenic syndrome. One patient initially tested negative for acetylcholine receptor antibodies, but later tested positive after 4 months and had a rare facial diplegia finding. The patient with congenital myasthenic syndrome had a novel genetic mutation, DPAGT1 homozygous variants, and also had false positive acetylcholine receptor antibodies. These cases highlight the importance of genetic testing for all infants and toddlers suspected of having myasthenia.

Effectiveness of levofloxacin in the induction of chemotherapy in high-risk acute lymphoblastic leukaemia in children in a developing country.

Background: Infections significantly predominate during induction chemotherapy for acute lymphoblastic leukaemia (ALL) in children. Antibacterial prophylaxis is one strategy that lowers the risk of these infections. This study evaluates the role of levofloxacin prophylaxis on the frequency of infections, febrile neutropenia (FN) and outcomes associated with it along with the development of drug-resistance. Subject and methods: This was a single-centre cohort study in which the data were collected from electronic health records between two cohorts of high-risk ALL patients in the induction phase: the first one before the initiation of levofloxacin prophylaxis and the second was after the implementation of levofloxacin prophylaxis. The variables were compared between both the groups and odds ratios were calculated for clinical outcomes. Results: Out of 227 patients, 115 were given levofloxacin prophylaxis and 112 were in the no prophylaxis group. Both cohorts were similar in demographic factors, treatment regimen and supportive care services. There was a significant difference in total in-patient admissions along with FN admissions (p = 0.026). Microbiologically documented infections and infection-related critical interventions were significantly higher in the no prophylaxis group (p < 0.05). Odds ratios with a 95% confidence interval were applied to both groups for clinical outcomes in patients with and without FN which also illustrated similar results. Overall mortality and drug resistance patterns were similar among both groups. Conclusion: This study emphasised that levofloxacin is effective in reducing inpatient admissions with FN and its complications but did not affect the drug-resistance pattern. Long-term monitoring for antibiotic resistance is mandatory.

HTLV seroprevalence in people using HIV pre-exposure prophylaxis in England.

OBJECTIVES: HTLV-1 is predominantly a sexually-transmitted infection but testing is not mentioned in HIV-PrEP guidelines. We ascertained HTLV-1/HTLV-2 seroprevalence amongst HIV-PrEP users in England. METHODS: An unlinked anonymous seroprevalence study. RESULTS: Amongst 2015 HIV-PrEP users, 95% were men, 76% of white ethnicity and 83% had been born in Europe. There were no HTLV-1/HTLV-2 seropositive cases (95% confidence interval 0% - 0.18%). CONCLUSIONS: There were no HTLV positive cases, likely reflecting the demographic of mostly white and European-born individuals. Similar studies are needed worldwide to inform public health recommendations for HIV-PrEP using populations, particularly in HTLV-endemic settings.

An Unusual Etiology: Subarachnoid Hemorrhage Resulting in Transient Apical Ballooning Syndrome.

Intracranial bleeds, such as subarachnoid hemorrhage, carry high morbidity and mortality rates. Often intracranial hemorrhages result in debilitating residual neurological symptoms but can be so extensive that cardiac complications can also be seen. We present a rare case of a patient who was found to have a subarachnoid hemorrhage that incited the development of Takotsubo cardiomyopathy, which subsequently progressed to an acute myocardial infarction. The aim of this case report is to explore the underlying pathophysiology of how cerebral hemorrhage can result in apical ballooning of the left ventricle through various mechanisms including sympathetic-induced surge in catecholamines and neurogenic damage to the myocardium. We also intend to highlight the importance for clinicians to consider brain bleeds in the differential diagnosis when a patient presents with an acute myocardial infarction as treatment with heparin is generally contraindicated.

Enhancement of Electrochemical Stability Window and Electrical Properties of CNT-Based PVA-PEG Polymer Blend Composites.

New polymer blend composite electrolytes (PBCEs) were prepared by the solution casting technique using poly(vinyl alcohol) (PVA)-polyethylene glycol (PEG), sodium nitrate (NaNO3) as a doping salt and multiwalled carbon nanotubes (MWCNTs) as fillers. The X-ray diffraction pattern confirms the structural properties of the polymer blend composite films. FTIR investigations were carried out to understand the chemical properties and their band assignments. The ionic conductivity of the 10 wt % MWCNTs incorporated PVA-PEG polymer blend was measured as 4.32 × 10-6 S cm-1 at 20 °C and increased to 2.253 × 10-4 S/cm at 100 °C. The dependence of its conductivity on temperature suggests Arrhenius behavior. The equivalent circuit models that represent the R s(Q1(R1(Q2(R2(CR3))))) were used to interpret EIS data. The dielectric behavior of the samples was investigated by utilizing their AC conductance spectra, dielectric permittivity, dielectric constant (εi and εr), electric modulus (Mi and Mr), and loss tangent tan δ. The dielectric permittivity of the samples increases due to electrode polarization effects in low frequency region. The loss tangent's maxima shift with increasing temperature; hence, the peak height rises in the high frequency region. MWCNTs-based polymer blend composite electrolytes show an enhanced electrochemical stability window (4.0 V), better transference number (0.968), and improved ionic conductivity for use in energy storage device applications.

Altered subgenomic RNA abundance provides unique insight into SARS-CoV-2 B.1.1.7/Alpha variant infections.

B.1.1.7 lineage SARS-CoV-2 is more transmissible, leads to greater clinical severity, and results in modest reductions in antibody neutralization. Subgenomic RNA (sgRNA) is produced by discontinuous transcription of the SARS-CoV-2 genome. Applying our tool (periscope) to ARTIC Network Oxford Nanopore Technologies genomic sequencing data from 4400 SARS-CoV-2 positive clinical samples, we show that normalised sgRNA is significantly increased in B.1.1.7 (alpha) infections (n = 879). This increase is seen over the previous dominant lineage in the UK, B.1.177 (n = 943), which is independent of genomic reads, E cycle threshold and days since symptom onset at sampling. A noncanonical sgRNA which could represent ORF9b is found in 98.4% of B.1.1.7 SARS-CoV-2 infections compared with only 13.8% of other lineages, with a 16-fold increase in median sgRNA abundance. We demonstrate that ORF9b protein levels are increased 6-fold in B.1.1.7 compared to a B lineage virus in vitro. We hypothesise that increased ORF9b in B.1.1.7 is a direct consequence of a triple nucleotide mutation in nucleocapsid (28280:GAT > CAT, D3L) creating a transcription regulatory-like sequence complementary to a region 3' of the genomic leader. These findings provide a unique insight into the biology of B.1.1.7 and support monitoring of sgRNA profiles to evaluate emerging potential variants of concern.

Characterising within-hospitalSARS-CoV-2 transmission events using epidemiological and viral genomic data across two pandemic waves.

Hospital outbreaks of COVID19 result in considerable mortality and disruption to healthcare services and yet little is known about transmission within this setting. We characterise within hospital transmission by combining viral genomic and epidemiological data using Bayesian modelling amongst 2181 patients and healthcare workers from a large UK NHS Trust. Transmission events were compared between Wave 1 (1st March to 25th J'uly 2020) and Wave 2 (30th November 2020 to 24th January 2021). We show that staff-to-staff transmissions reduced from 31.6% to 12.9% of all infections. Patient-to-patient transmissions increased from 27.1% to 52.1%. 40%-50% of hospital-onset patient cases resulted in onward transmission compared to 4% of community-acquired cases. Control measures introduced during the pandemic likely reduced transmissions between healthcare workers but were insufficient to prevent increasing numbers of patient-to-patient transmissions. As hospital-acquired cases drive most onward transmission, earlier identification of nosocomial cases will be required to break hospital transmission chains.

Investigations on Structural, Optical Properties, Electrical Properties and Electrochemical Stability Window of the Reduced Graphene Oxides Incorporated Blend Polymer Nanocomposite Films.

The incorporation of reduced Graphene oxides (rGO) as a nanofiller in the blend polymer nanocomposite (BPNC) based on Polyvinylpyrrolidone (PVP)-Polyvinylalcohol (PVA) and sodium bicarbonate (NaHCO3) are presented. The blend polymer electrolytes films are prepared by the standard solution cast technique, and it is characterized to investigate the structural, morphological, thermal, optical and electrochemical property. The X-ray diffraction confirms the formation of polymer nanocomposite and is agreed with FESEM analysis. The FTIR confirms the presence of various interactions between the polymer, salt and rGO, and indicates the composite formation. The DSC examines the thermal property of the blend polymer nanocomposite electrolytes system. The bandgap energy has been obtained from the UV-spectroscopy and examines the direct and indirect gap, both offer the decreases bandgap with the addition of a higher concentration of rGO as nanofillers. The highest value of ionic conductivity of the film is obtained ~1.39×10-6 S cm-1 at 15 wt.% of rGO content in polymer blend nanocomposite (BPNC) films. For these BPNC films, the electrochemical stability window (ESW) is ~4.0 V at 25 wt.% of rGO content and ionic transport number (tion) is ~0.98, for 10 wt.% of rGO content at the room temperature. These highly stable blend polymer nanocomposite electrolyte films offer the excellent properties for utilized as a separator for solid-state devices e.g., battery, supercapacitors, electrochromic display devices and other electrochemical energy storage/ conversion devices respectively.

Clinical features and outcome of COVID-19 positive children from a tertiary healthcare hospital in Karachi.

: As the coronavirus disease 2019 (COVID-19) pandemic continues to evolve, differences in epidemiological and clinical features among pediatrics have been noticed across different countries. We describe the spectrum of COVID-19 in pediatric patients treated in tertiary health care. We conducted a retrospective chart review of pediatric patients admitted to Indus Hospital & Health care network, Korangi campus, Karachi; from April 1st, 2020 to July 31st, 2020. A total of 141 COVID-19 cases were reported, males were 81 (57%) and the median age was 8 (0.3-17) years. Moderate and severe infections were noted in 36(26%), and 17(12%) children respectively. Fever (50%) was the most common clinical feature. The SF ratio less than 264 was significantly associated with severe disease (p < .05). Lab investigations that differed significantly across disease severity groups included IL-6 levels (p < .01) and Prothrombin time (p < .05). Majority of children were advised home isolation 89 (63%), 29 (20.5%) were admitted while mortality was observed in 10 (7%) children. No significant difference was observed between children with and without malignancy. Pre-existing comorbidities are significantly associated with COVID-19 infections among children. Reduced SF ratio, elevated Prothrombin time, and interleukin-6 levels are associated with greater disease severity.

Subgenomic RNA identification in SARS-CoV-2 genomic sequencing data.

We have developed periscope, a tool for the detection and quantification of subgenomic RNA (sgRNA) in SARS-CoV-2 genomic sequence data. The translation of the SARS-CoV-2 RNA genome for most open reading frames (ORFs) occurs via RNA intermediates termed "subgenomic RNAs." sgRNAs are produced through discontinuous transcription, which relies on homology between transcription regulatory sequences (TRS-B) upstream of the ORF start codons and that of the TRS-L, which is located in the 5' UTR. TRS-L is immediately preceded by a leader sequence. This leader sequence is therefore found at the 5' end of all sgRNA. We applied periscope to 1155 SARS-CoV-2 genomes from Sheffield, United Kingdom, and validated our findings using orthogonal data sets and in vitro cell systems. By using a simple local alignment to detect reads that contain the leader sequence, we were able to identify and quantify reads arising from canonical and noncanonical sgRNA. We were able to detect all canonical sgRNAs at the expected abundances, with the exception of ORF10. A number of recurrent noncanonical sgRNAs are detected. We show that the results are reproducible using technical replicates and determine the optimum number of reads for sgRNA analysis. In VeroE6 ACE2+/- cell lines, periscope can detect the changes in the kinetics of sgRNA in orthogonal sequencing data sets. Finally, variants found in genomic RNA are transmitted to sgRNAs with high fidelity in most cases. This tool can be applied to all sequenced COVID-19 samples worldwide to provide comprehensive analysis of SARS-CoV-2 sgRNA.

Diagnosis of SARS-CoV-2 Infection with LamPORE, a High-Throughput Platform Combining Loop-Mediated Isothermal Amplification and Nanopore Sequencing.

LamPORE is a novel diagnostic platform for the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) RNA combining loop-mediated isothermal amplification with nanopore sequencing, which could potentially be used to analyze thousands of samples per day on a single instrument. We evaluated the performance of LamPORE against reverse transcriptase PCR (RT-PCR) using RNA extracted from spiked respiratory samples and stored nose and throat swabs collected at two UK hospitals. The limit of detection of LamPORE was 10 genome copies/µl of extracted RNA, which is above the limit achievable by RT-PCR, but was not associated with a significant reduction of sensitivity in clinical samples. Positive clinical specimens came mostly from patients with acute symptomatic infection, and among them, LamPORE had a diagnostic sensitivity of 99.1% (226/228; 95% confidence interval [CI], 96.9% to 99.9%). Among negative clinical specimens, including 153 with other respiratory pathogens detected, LamPORE had a diagnostic specificity of 99.6% (278/279; 98.0% to 100.0%). Overall, 1.4% (7/514; 0.5% to 2.9%) of samples produced an indeterminate result on first testing, and repeat LamPORE testing on the same RNA extract had a reproducibility of 96.8% (478/494; 94.8% to 98.1%). LamPORE has a similar performance as RT-PCR for the diagnosis of SARS-CoV-2 infection in symptomatic patients and offers a promising approach to high-throughput testing.

The COVID-19 Sequelae: A Cross-Sectional Evaluation of Post-recovery Symptoms and the Need for Rehabilitation of COVID-19 Survivors.

Background  As of January 19, 2021, around two million fatalities and 68 million recoveries from coronavirus disease 2019 (COVID-19) have been reported around the globe. The past pandemics of severe acute respiratory syndrome (SARS) and the Middle East respiratory syndrome (MERS) hint toward a risk of occurrence of "Long-COVID" syndrome, i.e., the persistence of post-discharge symptoms among COVID-19 survivors. With the scarcity of literature addressing post-COVID-19 manifestations and little regard for the stigma associated with this disease, survivors' rehabilitation remains widely neglected. The current study aims to assess the prevalence and characteristics of post-COVID-19 manifestations and their effect on the quality of life (QoL) of COVID-19 recovered individuals. We have also analyzed the relationship of time since the recovery of COVID-19 and its severity with the post-discharge symptoms. The stigma affiliated with the infection of SARS coronavirus-2 (SARS-CoV-2) has also been highlighted. Methodology A descriptive, cross-sectional, questionnaire-based study was conducted from September 2020 to December 2020 among 158 COVID-19 recovered patients, whose information was obtained from Dow Diagnostic Laboratory, Ojha Campus, Karachi, Pakistan. The questionnaire consisted of four sections: sociodemographic data, post-COVID-19 manifestations, questions relating to the stigma, and the QoL of the recovered COVID-19 patients. We used the EuroQol five-dimension five-level questionnaire to assess the QoL, while the modified BG Prasad Socioeconomic Classification updated for 2019 was employed to determine the socioeconomic status of the participants. Data were analyzed using SPSS version 24.0 (IBM Corp., Armonk, NY, USA). Data were presented in the form of frequencies and percentages. Results An overwhelming majority (94.9%) experienced at least one post-COVID-19 symptom, with fatigue (82.9%) being the most prevalent post-discharge manifestation. We observed a significant correlation of post-COVID-19 symptoms with gender, age, and time since recovery. COVID-19 severity was found to be significantly related to the five dimensions of the QoL. A significant difference in EuroQol Visual Analog Scale health score was observed between the participants with mild, moderate, and severe COVID-19 infection (p < 0.001). Besides, the associated stigma with SARS-CoV-2 infection was found to be more prevalent in the participants belonging to the upper class as compared to the other classes (p < 0.05). Nonetheless, we also observed a significant association of disease severity with post-COVID-19 manifestations and pre-existing comorbidities. Conclusions The long-COVID syndrome is similar to the post-discharge manifestations of the survivors of prior pandemics of SARS and MERS. Multi-disciplinary rehabilitation teams, healthcare workers, and the general population should recognize the need for systematic assessment of their recovery and further rehabilitation.

Hereditary Tyrosinemia Compounded With Hyperinsulinemic Hypoglycemia: Challenging Diagnosis of a Rare Case.

Hereditary tyrosinemia type 1 (HT-1) is a rare autosomal recessive disorder caused by a deficiency in the enzyme fumarylacetoacetate hydrolase (FAH), which catalyzes the final step in the tyrosine degradation pathway. Hereditary tyrosinemia is a heterogeneous disease with a wide spectrum of clinical manifestations involving hepatic, renal, or nervous systems. It has grave consequences if left untreated. Some of the late complications of hereditary tyrosinemia include cirrhosis, liver nodules, hepatocellular carcinoma, hypophosphatemic rickets, nephrocalcinosis, glomerulosclerosis, and chronic renal failure. Rarely, infants with hereditary tyrosinemia may present with persistent hypoglycemia, which may be a result of acute liver failure or hyperinsulinism. Hyperinsulinemic hypoglycemia (HH), caused by dysregulation of insulin secretion from pancreatic ß-cells, leads to insulin driven glucose entry into the tissues and inhibits glycolysis, gluconeogenesis, fatty acid release, and ketone body synthesis. Hyperinsulinemic hypoglycemia can cause severe, persistent hypoketotic hypoglycemia. Diagnosing tyrosinemia type 1 can be a challenge as it is a heterogeneous disorder with a wide variety of clinical manifestations and complications. We herein report a rare case of a three-day-old male neonate with HT-1 compounded with HH.

Electrolyte Imbalance in Children With Severe Acute Malnutrition at a Tertiary Care Hospital in Pakistan: A Cross-Sectional Study.

Background Malnutrition is a significant public health concern and a leading contributor to the global burden of children's diseases, affecting 50 to 150 million children under the age of five years worldwide. Globally, undernutrition accounts for approximately 33% of the deaths among under-fives. South Asia alone contributes to 50% and 38.8% of the world's population of wasted and stunted children, respectively. In Pakistan, malnutrition is the leading cause of childhood mortality, accounting for nearly 35% of all deaths under five years of age. Severe acute malnutrition (SAM), the most severe form of malnutrition, is often associated with electrolyte imbalances. This study aimed to determine the frequency of electrolyte imbalance in children with SAM admitted at a tertiary care hospital. Methods This cross-sectional study includes 184 patients with SAM aged between 6 and 60 months, who were admitted at the inpatient Department of Pediatrics, Dr. Ruth K. M. Pfau, Civil Hospital, Karachi, Pakistan, from January 17, 2017 to July 16, 2017. Weight and length/height were measured, and weight-for-height was calculated. Children were labeled to have SAM when weight-for-height was below -3 standard deviation (SD). Blood samples for serum electrolytes were drawn and sent to the lab. Descriptive statistics were calculated, and stratification was performed using the chi-square test. A p-value of ≤0.05 was considered statistically significant. Results  The mean age of participants was 22.63 ± 12.71 months. Of the 184 patients with SAM, 172 (93.5%) patients had electrolyte imbalance. Hypokalemia was present in 79.9%, whereas hypocalcemia, hyponatremia, and hypomagnesemia were present in 71.7%, 48.9%, and 13.6%, respectively. Post-stratification results showed a significant association of electrolyte imbalance with gender (p = 0.005) and educational status of parents (p = 0.001). Conclusions Electrolyte disturbances are common in SAM. Serum electrolytes of every malnourished child admitted should be assessed and corrected to avoid fatal outcomes. We suggest that more research with better study designs should be conducted to develop policies and strategies for successfully combating malnutrition in Pakistan. In the meantime, we recommend adopting national guidelines for the management of acute malnutrition to reduce morbidity and mortality.