Clinical Course Research Articles

The impact of viral respiratory infection on surgical outcome of cavopulmonary shunt.

Undetected respiratory infections may adversely affect the intrapulmonary resistance after Stage 2 or Stage 3 Fontan palliation. A few studies describe a higher risk for viral pneumonia during respiratory virus season, but none of them have focused on the effect of symptomatic viral pneumonia on in-hospital clinical course after bidirectional Glenn shunt. We analysed 77 patients who underwent bidirectional Glenn shunt surgery. Six patients were detected with pneumonia and proof of viral ribonucleic acid in tracheal mucus in the very early postoperative time. We compared them retrospectively to the remaining 71 patients regarding preoperative inflammatory signs, mortality, paediatric ICU length of stay, and ventilation time. The infection rate was not seasonal dependent. Ventilation time was significantly elongated in the pneumonia group (558 h ± 634 vs. 8.7 h ± 1.9; p < 0.0001) and so was the paediatric ICU length of stay (29 days ± 26 vs. 3 days±1; p = 0.007). Significantly more patients in the pneumonia group required extracorporeal cardiac life support postoperatively. The mortality was significantly increased in patients with pneumonia. Even subclinical viral pneumonia may cause ventilation-to-perfusion mismatch by raising intrapulmonary resistance. Recorded parameters of postoperative paediatric ICU therapy showed a significant impact of a viral pneumonia on patients after bidirectional Glenn shunt. The respiratory syncytial virus vaccination does not protect these patients from infection with other respiratory viruses. The focus should be put on preoperative diagnosis of pulmonary infections in the vulnerable group of patients with univentricular hearts.

Long-term outcome followed for more than 5 years after revascularization surgery for the treatment of atherosclerotic steno-occlusive disease: poor outcome prediction using machine learning and analysis of the results.

Cerebral revascularization for the treatment of atherosclerotic steno-occlusive disease (ASOD) was found to have no benefit compared with medical treatment. However, there is also criticism that with sufficiently long-term follow-up, a crossover might emerge demonstrating the advantages of surgery. Therefore, we examined the long-term outcome of cerebral revascularization performed on patients with carefully selected ASOD at our center. Patients undergoing bypass surgery for non-moyamoya ischemic disease were retrospectively identified. The inclusion criteria were symptomatic ASOD with hemodynamic insufficiency, follow-up of more than 5 years, and stroke or surgical complications during follow-up. The clinical course and radiological findings were investigated. Poor outcomes were predicted using machine learning (ML) models, and Shapley additive explanation (SHAP) values and feature importance of each model were analyzed. A total of 109 patients were included from 2007 to 2018. The 30-day risk of any stroke or death was 6.4% (7/109). The risk of ipsilateral ischemic stroke during median follow-up of 116 months was 7.3% (8/109). The SHAP values showed that previously and empirically known stroke risk factors exert a relatively consistent effect on the prediction of models. The number of lesions with stenosis > 50% (odds ratio [OR] 5.77), age (OR 1.13), and coronary artery disease (OR 5.73) were consistent risk factors for poor outcome. We demonstrated an acceptable long-term outcome of cerebral revascularization surgery for patients with hemodynamically insufficient and symptomatic ASOD. Multicenter studies are encouraged to predict poor outcomes and suitable patients with large numbers of quantitative and qualitative data.

Atypical presentation of PJP: hypercalcemia and kidney injury in an allogeneic stem cell transplant recipient

BackgroundPneumocystis jirovecii pneumonia (PJP) is an opportunistic infection that primarily affects immunocompromised individuals. Typical symptoms of PJP include the subacute onset of dyspnea, nonproductive cough, and low-grade fever. In hematology patients, particularly those who are allogeneic stem cell transplant recipients, the disease often presents with a more aggressive clinical course. While hypercalcemia has been documented as a manifestation of PJP in some solid organ transplant recipients, it has not been reported in hematology or stem cell transplant patients.Case presentationHere, we present a case of PJP in a 56-year-old male allogeneic stem cell transplant recipient, who developed hypercalcemia and renal failure during the late post-transplant period. The patient had a history of allogeneic stem cell transplantation due to acute myeloid leukemia. He presented with symptoms of fatigue and weakness. Laboratory tests revealed hypercalcemia (13.8 mg/dL) and elevated serum creatinine levels (2.3 mg/dL). The patient was hospitalized, and despite initial treatment with hydration and furosemide, the hypercalcemia persisted, leading to the administration of denosumab. During follow-up, hypoxia was detected, and a chest CT scan revealed mosaic attenuation and ground-glass opacities. Bronchoscopy was performed, and PCR testing confirmed the presence of Pneumocystis jirovecii. Other causes of hypercalcemia were ruled out, with PTH measured at 13.8 pg/mL (normal range 15–65 pg/mL), albumin at 3.71 g/dL, 1.25-dihydroxy vitamin D3 at 96 ng/dL (normal range 26–95 ng/dL), and 25-hydroxy vitamin D at 32.5 ng/mL (normal range 20–40 ng/mL). A PET-CT scan demonstrated no pathological FDG uptake, with the exception of findings suggestive of a pulmonary infection. Following treatment with trimethoprim-sulfamethoxazole and denosumab, the patient’s hypercalcemia and infection resolved.Conclusions Although rare, PJP can present with hypercalcemia and kidney injury in allogeneic stem cell transplant recipients. Early diagnosis and treatment can improve both PJP and hypercalcemia.

Advances in the Study of the Pathogenesis of Vogt-Koyanagi-Harada Syndrome.

Vogt-Koyanagi-Harada syndrome (VKHS) is a common type of uveitis characterized by the invasion of melanocyte-rich tissues. In recent years, the incidence of VKHS has been increasing yearly, and its specific pathogenesis has not yet been elucidated. However, its pathogenesis has been a hot topic of research. The clinical course of VKHS is characterized by the early involvement of the posterior segment of the eye, including exudative retinal detachment, optic papillitis, bilateral diffuse chorioretinitis, etc. If treated improperly or with delayed treatment, the inflammation may gradually spread to the anterior segment of the eye, leading to vision loss or even vision. This study examines the pathogenesis of VKHS. It reviews the progress of research on the pathogenesis of VKHS, which will help to improve the understanding of VKHS and provide a reference for subsequent studies.

Foscarnet eyedrops for the treatment of refractory herpetic keratitis

PurposeThe purpose of this case series is to describe the clinical course of patients receiving foscarnet eyedrops for the treatment of refractory herpetic keratitis.ObservationsSix patients diagnosed with herpetic keratitis were treated with foscarnet 24 mg/mL (2.4%) eyedrops with resulting improvement in keratitis.ConclusionTopical foscarnet may be a safe and effective treatment for herpetic keratitis in conjunction with, or as an alternative to, conventional antiviral therapy. This is an off-label use of foscarnet.

Exploring the Relationship Between Hypertension and Cerebral Microvascular Disease

Background/Objectives: Hypertension exerts negative effects on the vasculature representing a key risk factor for cardiovascular disorders, cerebral and Cerebral Small Vessel Disease (CSVD). Methods: An extensive research in the literature was implemented in order to elucidate the role of hypertension in the pathogenesis of CSVD. Results: Hypertension-mediated vascular dysfunction and chronic cerebral hypoperfusion are closely linked to CSVD. CSVD encompasses a wide range of lesions depicted on brain Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) scans. The presenting symptoms and clinical course are highly variable, as a significant proportion of patients remain asymptomatic. Nevertheless, CSVD is associated with an increased risk of stroke, dementia and mobility disorders. Various randomised controlled trials have been implemented trying to shed light on the effect of vascular risk-modifying agents and lifestyle interventions on the prevention and treatment of small vessel disease. Conclusions: Hypertension has a pivotal role in the pathogenesis of CSVD. However, further research is required for a better understanding of the relationship between blood pressure levels and CSVD progression.

Nerandomilast Improves Bleomycin-Induced Systemic Sclerosis-Associated Interstitial Lung Disease in Mice by Regulating the TGF-β1 Pathway.

Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common complication of SSc and a major contributor to SSc-related deaths. Besides nintedanib and tocilizumab, there are currently no clinically approved drugs for SSc-ILD, highlighting the urgent need for new treatment strategies. Previous studies have shown that cyclic adenosine monophosphate (cAMP) plays a crucial role in the pathogenesis of SSc and lung fibrosis. Phosphodiesterases (PDEs) are enzymes that specifically hydrolyze cAMP, making PDE inhibitors promising candidates for SSc-ILD treatment. Nerandomilast, a preferential phosphodiesterase 4B (PDE4B) inhibitor currently undergoing phase III clinical trials for idiopathic pulmonary fibrosis and progressive fibrosing interstitial lung diseases (PF-ILD), has good preference for PDE4B but lacks studies for SSc-ILD. Our research demonstrates that nerandomilast effectively inhibits skin and lung fibrosis in a bleomycin-induced mouse model of SSc-ILD. For lung fibrosis, we found that nerandomilast could improve bleomycin-induced SSc-ILD through inhibiting PDE4B and the TGF-β1-Smads/non-Smads signaling pathways, which provides a theoretical basis for potential therapeutic drug development for SSc-ILD.

Central nervous system manifestations in acute and chronic graft-versus-host disease.

Despite the growing evidence supporting the existence of CNS involvement in acute and chronic graft-versus-host disease (CNS-GvHD), the characteristics and course of the disease are still largely unknown. In this multicenter retrospective study, we analyzed the clinical, biological, radiological, and histopathological characteristics, as well as the clinical course of 66 patients diagnosed with possible CNS-GvHD (pCNS-GvHD), selected by predetermined diagnostic criteria. Results were then contrasted depending on whether pCNS-GvHD occurred before or after day 100 following allogeneic hematopoietic stem cell transplantation. Median time between hematopoietic stem cell transplantation and pCNS-GvHD onset was 149 days (IQ25-75 48-321), and pCNS-GvHD onset occurred before day 100 following transplantation in 44% of patients. The most frequent findings at presentation were cognitive impairment (41%), paresis (21%), altered consciousness (20%), sensory impairment (18%), and headache (15%). Clinical presentation did not significantly differ between patients with pCNS-GvHD occurring before or after day 100 following transplantation. Brain MRI found abnormalities compatible with the clinical picture in 57% of patients, while CT detected abnormalities in only 7%. Seven patients had documented spinal cord MRI abnormalities, all of them with pCNS-GvHD occurring after day 100 following transplantation. In the cerebrospinal fluid, white blood cell count was increased in 56% of the population (median 18 cells/μL). Histopathological analyses were performed on 12 specimens and were suggestive of pCNS-GvHD in 10. All compatible specimens showed parenchymal and perivascular infiltration by CD3+ and CD163+ cells. Immunosuppressive therapy was prescribed in 97% of patients, achieving complete clinical response in 27%, partial improvement in 47% and stable disease in 6%. Response to immunosuppressive therapy did not significantly differ between patients with pCNS-GvHD occurring before or after day 100 following transplantation. Clinical relapse was observed in 31% of patients who initially responded to treatment. One-year overall survival following pCNS-GvHD onset was 41%. Onset before day 100 following hematopoietic stem cell transplantation (HR [95%CI]: 2.1 [1.0-4.5]; P=0.041) and altered consciousness at initial presentation (HR [95%CI]: 3.0 [1.3-6.7]; P=0.0077) were associated with a reduced one-year overall survival probability. Among surviving patients, 61% had neurological sequelae. This study supports that immune-mediated CNS manifestations may occur following allo-HSCT. These can be associated with both acute and chronic GvHD and carry a grim prognosis. The clinical presentation as well as the radiological and biological findings appear variable.

Understanding the interplay between psoriatic arthritis and gout: "Psout".

The interplay between Psoriatic arthritis and Gout is a current diagnostic challenge faced by many physicians and researchers. We aimed at reviewing the coexistence of gout and its features such as hyperuricemia and deposition of monosodium urate crystals in patients with psoriatic arthritis (PsA). We also focused on a brief presentation of the pathophysiology underneath the interplay between PsA and gout, and ultimately on recommendation of approaches for the differential diagnosis. The literature search for this narrative review was conducted using PubMed and Medline and after retrieving and screening the references, articles were selected according to the inclusion and exclusion criteria. Part of the assessed studies reported the coexistence of PsA and gout (Psout) and its association with several clinical outcomes among affected patients. Other studies stressed incidences of misdiagnosis of gout with PsA and vice versa. Additionally, the presence of hyperuricemia in PsA patients could interfere with the patient's characteristics and outcomes of their treatment. Further research on the assessment and clinical course of Psout is required to develop an official protocol for its diagnosis and treatment.

Subependymal giant cell astrocytoma in the absence of tuberous sclerosis: a case report and literature review

Introduction and Importance: Subependymal giant cell astrocytoma (SEGA) is a benign intraventricular tumor classically arising near the Foramen of Monro. SEGAs almost always present as a component of tuberous sclerosis complex (TSC), an autosomal dominant disorder characterized by lesions in multiple organs. Case Presentation: A 16-year-old male with no significant past medical history presented with headache and dizziness. Imaging revealed intraventricular mass within the left lateral ventricle; the post-contrast image showed a contrast-enhanced lesion suggestive of SEGA. Following resection, histopathological analysis identified the lesion as a SEGA. However, on further workup, the patient was found not to meet the clinical criteria of TSC, which exemplifies a rare case of SEGA in the absence of a TSC diagnosis. Clinical Discussion: Tuberous sclerosis Complex (TSC) is a neurocutaneous disorder with a classical triad of seizures, mental retardation, and hamartomas in multiple organs and tissues. SEGA without TSC does not have multiorgan problems and hence doesn’t need ancillary testing but despite having a good outlook still needs follow-up for interval growth or recurrence. However, overall prognosis of isolated SEGA is better than SEGA associated with TSC. Conclusion: SEGA is commonly found to be associated with TSC. Although it is challenging to diagnose isolated SEGA, hence it is essential for physicians to be aware of the possibility of SEGA in the absence of other characteristics of TSC, which has many implications for a patient’s clinical course. This case report adds to current knowledge regarding isolated SEGA.

Clinical and Financial Validation of the International Study Group for Pancreatic Surgery (ISGPS) Definition of Post-Pancreatectomy Acute Pancreatitis (PPAP): International Multicenter Prospective Study.

To validate the ISGPS definition and grading system of PPAP after pancreatoduodenectomy (PD). In 2022, the International Study Group for Pancreatic Surgery (ISGPS) defined post-pancreatectomy acute pancreatitis (PPAP) and recommended a prospective validation of its diagnostic criteria and grading system. This was a prospective, international, multicenter study including patients undergoing PD at 17 referral pancreatic centers across Europe, Asia, Oceania, and the United States. PPAP diagnosis required the following three parameters: (1) postoperative serum hyperamylasemia /hyperlipasemia (POH) persisting on postoperative days 1 and 2, (2) radiologic alterations consistent with PPAP, and (3) a clinically relevant deterioration in the patient's condition. To validate the grading system, clinical and economic parameters were analyzed across all grades. Among 2902 patients undergoing PD, 7.5% (n=218) developed PPAP (6.3% grade B and 1.2% grade C). POH occurred in 24.1% of patients. Hospital stay was associated with PPAP grades (No POH/PPAP 10 days (IQR 7-17) days, grade B 22 days (IQR 15-34) days, and grade C 43 days (IQR 27-54) days; P<0.001), as well as intensive care unit admission (No POH/PPAP 5.4%, grade B 12.6%, grade C 82.9%; P<0.010), and hospital readmission rates (No POH/PPAP 7.3%, grade B 16.1%, grade C 18.5%; P<0.05). Costs of grade B and C PPAP were 2 and 11 times greater than uncomplicated clinical course, resp. (P<0.001). This first prospective, international validation study of the ISGPS definition and grading system for PPAP highlighted the relevant clinical and financial implications of this condition. These results stress the importance of routine screening for PPAP in patients undergoing PD.

Vaccinations in pulmonary diseases - part 2: herpes zoste, RSV, pneumococcal infection and pertussis

RSV is a common cause of respiratory tract infections, posing a risk of severe disease, particularly for newborns and infants, as well as in older individuals with pre-existing conditions. Two safe and effective RSV vaccines were approved in 2023. These vaccines elicit protective antibodies and offer robust protection with no additional benefit from annual boosters. Both vaccines have been approved for individuals aged 60 years and older, while one of the vaccines has also been approved in pregnant women to elicit maternal immunity for passive protection of the unborn child. In Germany, RSV vaccination is currently recommended for all individuals aged 75 years and older, as well as people aged 60-74 years of age with severe underlying conditions. PNEUMOCOCCAL INFECTION: Streptococcus pneumoniae is a primary cause of community-acquired pneumonia (CAP). Since early 2022, a 20-valent conjugate vaccine (PCV20) is approved and recommended for people over 60 years of age and individuals over 18 years of age with risk factors. PCV20 replaces the 23-valent polysaccharide vaccine (PPV23) previously recommended for those over 60 years of age. Although viewed primarily as a childhood disease, the majority of infections affect adults. Patients with chronic respiratory diseases are at a higher risk for severe clinical course of pertussis infection. It has therefore been recommended that all adults should get a pertussis booster with their next scheduled tetanus vaccination, given as a combination vaccine (Tdap). For risk groups (healthcare personnel, community facilities) a booster vaccination every 10 years is recommended. Herpes Zoster (shingles) is caused by the varicella-zoster virus, and reactivations can lead to painful skin lesions and potential complications such as herpes zoster oticus, meningitis, or postherpetic neuralgia. People with chronic lung diseases such as COPD or asthma are at increased risk of herpes zoster-related complications. A recombinant adjuvanted inactivated vaccine was approved in 2018 and offers robust protection against herpes zoster and its complications. The vaccine is recommended for all people over 60 years of age and for certain risk groups over 50 years of age.

Diagnostic and treatment possibilities in the management of positional plagiocephaly.

Positional plagiocephaly, characterized by an asymmetric skull shape, is the most common craniofacial malformation in infancy. Associated risk factors include apreference for the supine position, first and assisted delivery, multiple pregnancy, prematurity, and congenital muscular torticollis. The diagnosis is established by clinical and anthropometric examinations. In the case of moderate or severe deformity, three-dimensional optical scanning enables adetailed depiction of the deformity and provides asafe and noninvasive tool for follow-up. Treatment mainly includes repositioning of the infant, while orthotic therapy is considered in cases of severe deformity. Cranial orthotic therapy is most effective when started between 4and 7months of age. The total duration of orthotic treatment ranges from 2to 6months. Although the clinical course of positional plagiocephaly is generally benign and the prognosis favorable, its increasing prevalence has prompted the development of novel diagnostic and therapeutic strategies over the past decade.

Pulmonary function using impulse oscillometry system and clinical outcomes at age 4 years in children born extremely preterm with or without bronchopulmonary dysplasia

This study aimed to describe the early assessment of lung function and respiratory morbidity in children born extremely preterm with or without bronchopulmonary dysplasia (BPD). MethodsThis was a prospective study including all the children born at gestational age ≤28 weeks who received treatment in the NICU of the Centre Intercommunal de Créteil in France, from January 2006 to March 2012. Lung function, using the impulse oscillometry system, respiratory morbidity and growth were assessed at age 4 years. Lung function and clinical course of children were compared in children with and without BPD. ResultsWe included 136 extremely premature children; 26 (19 %) had BPD. Children with and without BPD did not significantly differ in resistance measurements at 5 Hz (R5) and 20 Hz (R20) and reactance (X5) measurements at age 4 years. A total of 104 (76 %) pre-term children had respiratory resistance R5 above the 95th percentile for the reference population (z-score >1.64), regardless of BPD status. The mean (SD) R5 z-score for all children was 2.1 (±0.7), whereas the mean (SD) R20 was in the normal range (z-score = 1.1 [±0.3]). After treatment with bronchodilators, all children showed no significant change in resistance. The prevalence of asthma symptoms at age 4 years was common and estimated at 30 % regardless of BPD status. ConclusionEarly assessment of lung function by the impulse oscillometry system revealed that most preschool children who were born extremely preterm had abnormal total airway resistance regardless of BPD status. The system is an essential tool for the early assessment of children born prematurely.

The Chilean COVID-19 Genomics Network Biorepository: A Resource for Multi-Omics Studies of COVID-19 and Long COVID in a Latin American Population

Although a lack of diversity in genetic studies is an acknowledged obstacle for personalized medicine and precision public health, Latin American populations remain particularly understudied despite their heterogeneity and mixed ancestry. This gap extends to COVID-19 despite its variability in susceptibility and clinical course, where ethnic background appears to influence disease severity, with non-Europeans facing higher hospitalization rates. In addition, access to high-quality samples and data is a critical issue for personalized and precision medicine, and it has become clear that the solution lies in biobanks. The creation of the Chilean COVID-19 Biorepository reported here addresses these gaps, representing the first nationwide multicentric Chilean initiative. It operates under rigorous biobanking standards and serves as one of South America’s largest COVID cohorts. A centralized harmonization strategy was chosen and included unified standard operating procedures, a sampling coding system, and biobanking staff training. Adults with confirmed SARS-CoV-2 infection provided broad informed consent. Samples were collected to preserve blood, plasma, buffy coat, and DNA. Quality controls included adherence to the standard preanalytical code, incident reporting, and DNA concentration and absorbance ratio 260/280 assessments. Detailed sociodemographic, health, medication, and preexisting condition data were gathered. In five months, 2262 participants were enrolled, pseudonymized, and sorted by disease severity. The average Amerindian ancestry considering all participant was 44.0% [SD 15.5%], and this value increased to 61.2% [SD 19.5%] among those who self-identified as Native South Americans. Notably, 279 participants self-identified with one of 12 ethnic groups. High compliance (&gt;90%) in all assessed quality controls was achieved. Looking ahead, our team founded the COVID-19 Genomics Network (C19-GenoNet) focused on identifying genetic factors influencing SARS-CoV-2 outcomes. In conclusion, this bottom-up collaborative effort aims to promote the integration of Latin American populations into global genetic research and welcomes collaborations supporting this endeavor. Interested parties are invited to explore collaboration opportunities through our catalog, accessible online.

Bifidumbacterin forte in the treatment of acute viral hepatitis

Relevance. In patients with acute viral hepatitis A and B, favorable conditions are created for the development of dysbacteriosis. As a result, the toxic load on the liver increases. For effective treatment of acute viral hepatitis A and B, it is necessary to examine for intestinal dysbiosis before and after treatment and subsequent correction of identified disorders. Aim. To study the clinical course, functional state of the liver, intestinal microflora in patients with acute viral hepatitis A and B and the effectiveness of using bifidumbacterin forte for the correction of intestinal microflora. Materials and methods. The work used clinical and laboratory data from 54 patients with acute viral hepatitis A and B. The main group of patients received bifidumbacterin forte against the background of basic therapy. Patients in the comparison group were on traditional therapy. Clinical, biochemical, bacteriological parameters were observed before the start of therapy and 15 days after its start, serological markers - upon admission. Results. During the therapy, in patients of the main group, the disappearance of intoxication, lethargy, weakness, pain, and normalization of liver size were observed earlier in comparison with the control group. Symptoms of pigment metabolism disorders disappeared significantly earlier: yellowness of the skin, itching of the skin, changes in the color of urine and feces. A dynamic study of liver tests demonstrated a more rapid reverse development of biochemical disorders of the functional state of the liver in patients who received bifidumbacterin forte as part of complex treatment. Conclusion. The use of bifidumbacterin forte in patients with acute viral hepatitis A and B against the background of basic treatment has a positive effect on some clinical manifestations, biochemical parameters, and individual components of the intestinal microflora.

Eosinophilic granulomatosis with polyangiitis associated with malignant arrhythmias: a case report

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis associated with significant cardiac morbidity and mortality. This case report presents the diagnostic and management challenges of EGPA-related arrhythmias in a remote general hospital setting. Case summary A 64-year-old Caucasian male presented with an indolent prodrome of fatigue, shortness of breath and anorexia, that culminated in an acute presentation with pulmonary embolism. His complicated clinical course included intracranial haemorrhage and refractory ventricular arrhythmias. Eosinophilia and subendocardial hypoattenuation observed on chest computed tomography were key findings that led to the diagnosis of EGPA. Multiple antiarrhythmic therapies were required as temporary measures whilst control of the underlying eosinophilic inflammation was achieved. Once stable, the patient was transferred to a tertiary cardiac centre for further investigation and cardioverter-defibrillator implantation. With EGPA now well controlled, he has experienced no further ventricular arrhythmias and has fully recovered. Conclusion Cardiac complications of EGPA, including ventricular arrhythmias, are difficult to manage without concurrent immunosuppression, which may itself further destabilise cardiac electrophysiology. The role of multiple imaging modalities in the diagnosis and monitoring of EGPA is emphasised, with cardiac magnetic resonance imaging playing a crucial role in detecting subendocardial fibrosis.

The clinical course of SARS-CoV-2 infection in patients with glomerular diseases and evaluation of the subsequent risk of relapse

IntroductionThis study aimed to describe the clinical course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with glomerular diseases (GDs) and its impact on the probability of relapse.MethodsPatients with biopsy-proven GD and positive PCR test for SARS-CoV-2 from glomerular clinics across Greece were studied retrospectively. Those who received the GD diagnosis after the SARS-CoV-2 vaccination or coronavirus disease 2019 (COVID-19) or ended in ESKD prior to infection were excluded. Demographics, histopathological diagnoses, past medical history, immunosuppression, and GD activity status were recorded.ResultsA total of 219 patients with GDs and documented SARS-CoV-2 infection were included. The mean time from the diagnostic kidney biopsy to SARS-CoV-2 infection was 67.6 ( ± 59.3) months. Among the participants, 82.5% had been vaccinated against SARS-CoV-2 with three doses (range: 2.5–3) without subsequent GD reactivation in 96.2% of them. Twenty-two patients (10%) were hospitalized for COVID-19 and one (0.5%) required mechanical ventilation. Four (1.8%) died due to COVID-19 and one (0.5%) had long COVID-19 symptoms. Among patients in remission prior to SARS-CoV-2 infection, 22 (11.2%) experienced a GD relapse within 2.2 (range: 1.5–3.7) months from the diagnostic test. The relapse-free survival after COVID-19 was significantly shorter for patients with minimal change disease, pauci-immune glomerulonephritis, and focal segmental glomerulosclerosis. No difference was observed in the relapse-free survival post-COVID-19 based on the history of SARS-CoV-2 vaccination.ConclusionsSARS-CoV-2 infection appears to have a symptomatic but uncomplicated sequence in vaccinated patients with GDs, with a significant impact on the clinical course of GD, associated with an increased probability of relapse in certain histopathological types.

Long term outcome of Immunoglobulin M (IgM) Nephropathy of Children in National Institute of Kidney Diseases &amp; Urology (NIKDU), Dhaka, Bangladesh

Background: Immunoglobulin M nephropathy (IgMN) is a new clinicoimmunopathologic entity which present mainly as idiopathic nephrotic syndrome in both children and adults. The clinical manifestation of IgMN are highly variable presenting with isolated haematuria or asymptomatic proteinuria. About 6 to 23 percent of IgMN patient developed end stage kidney disease. Usually treated with immunosuppressive agents (cyclosporine/tacrolimus) along with corticosteroid has response rates up to 50%. In our institute last few years we have observed a surprisingly rise of IgM nephropathy in children. Objective: To see the response of IgMN children treated with tacrolimus or mycophenolate mofetil(MMF) and long term follow up to observe their outcome. Methodology : This was a prospective observational study done in the Department of Paediatric Nephrology, National Institute of Kidney Diseases &amp; Urology, Dhaka, starting from January/2014 to December/2017. The study population consists of 86 IgMN children, age ranges from 2 to 12 years. The study was approved by institutional ethical review committee and informed written consent was taken from every parents. Twenty seven patients(Group-I) were selected purposively and treated with MMF and 59 patients (Group- II) treated with tacrolimus for 2 years. Patients were followed up three monthly onward in a prescribed form including adverse effect of drugs. Clinical outcome data like remission, active disease, active disease with renal impairment and dependency on renal replacement therapy(RRT) were documented for statistical analysis. Results: Of 86 IgMN children most of them were older than &gt;8-12 years in both groups and the highest percentage were clinically diagnosed as frequently relapsing nephrotic syndrome. In Group I 43.48% children remain on remission, 13.04% have active disease, 30.43% are active disease with impaired renal function and 4.35 % went into RRT. In Group II 55.56% children remain on remission, 12.96 % have active disease, 16.67% are active disease with impaired renal function and 5.56% dependent on RRT. Conclusion: From above observations, the overall prognosis of IgMN children is not good. The clinical course and disease outcome did not differ significantly between two groups treated with mycophenolate mofetil and tacrolimus. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 34-38

Early detection and management of congenital pulmonary airway malformation in a newborn with stable clinical course

Introduction: Congenital pulmonary airway malformation (CPAM) is a rare developmental lung anomaly characterized by cystic lung lesions, constituting about 25% of congenital pulmonary anomalies. It typically occurs sporadically and is not significantly associated with race, age, or other maternal factors. The prenatal course of CPAM can vary based on factors such as lesion size, mediastinal shift, and associated anomalies. While the overall prognosis is generally favorable in the absence of severe complications like hydrops fetalis, which can adversely affect outcomes, management strategies include corticosteroids, thoracoamniotic shunt (TAS), and, in severe cases, open fetal surgery or the EXIT procedure. Postnatal surgical excision usually offers a curative outcome with an excellent prognosis. Without surgical intervention, there are risks of recurrent infections and, rarely, malignant transformation. Effective prenatal detection significantly impacts clinical decision-making and neonatal outcomes, and the necessity and timing of surgery for asymptomatic infants remain subjects of ongoing debate, underscoring the need for personalized, multidisciplinary care. Case Report: A female infant, delivered at 38 weeks via Cesarean section, was diagnosed with CPAM following routine prenatal ultrasound. Presence of extensive cystic regions in the right lung confirmed the diagnosis and a CPAM volume ratio (CVR) of 0.37 indicated a low risk of severe complications. The pregnancy was largely uneventful, with maternal chronic hypertension managed by Nifedipine and mild intermittent asthma. Postnatal chest radiography corroborated the CPAM diagnosis, while an abdominal ultrasound investigated intrahepatic calcifications. Maternal screening for cytomegalovirus and toxoplasmosis yielded negative results, and postnatally, the infant also tested negative for both infections. The neonate, though asymptomatic and stable, required close monitoring in the neonatal intensive care unit to prevent potential respiratory compromise. Conclusion: Advances in prenatal imaging have greatly improved the early detection and management of CPAM, allowing for targeted neonatal care and strategic planning. This case highlights the importance of a multidisciplinary approach involving obstetricians, maternal-fetal medicine specialists, neonatologists, and pediatric thoracic surgeons in the effective management of CPAM. Early identification and diligent postnatal monitoring are essential for ensuring optimal outcomes and minimizing the risk of complications. The coordinated care and strategic decision-making exemplified in this case underscore the potential for enhanced neonatal health and long-term well-being through comprehensive management of congenital anomalies.