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1.
Unfallchirurgie (Heidelb) ; 127(8): 607-612, 2024 Aug.
Article in German | MEDLINE | ID: mdl-38990311

ABSTRACT

The challenge in treating traumatic hemipelvectomy is the dynamics of the complex and life-threatening consequences of the injury. These include skin and soft tissue defects, osseous, neural and vascular injuries as well as the subsequent hemostatic derangement and organ dysfunction as part of the shock process. The treatment requires rapid and targeted decisions to save the patient's life. In this particular case a 34-year-old farmer was trapped between a wheeled loader and a stationary trailer. Upon arrival at the hospital the patient was in a state of hemorrhagic shock with accompanying acute traumatic coagulopathy and a grade III open pelvic trauma with complete ischemia of the left leg and a bladder injury. After performing emergency surgery and a two-stage approach for pelvic stabilization the patient's condition deteriorated up to multiorgan failure, necessitating left-sided hemipelvectomy as an immediate life-saving salvage procedure. In the further course multiple revision surgeries and plastic reconstructions due to wound infections and the presence of skin and soft tissue damage were required. Due to the rare confrontation with this type of injury in everyday practice and the absence of a universal treatment algorithm, the following case report is intended to contribute to a better understanding of the treatment and to illustrate the coherent interactions of the individual organ systems affected.


Subject(s)
Hemipelvectomy , Humans , Adult , Male , Hemipelvectomy/methods , Multiple Trauma/surgery , Treatment Outcome , Pelvic Bones/injuries , Pelvic Bones/surgery , Leg Injuries/surgery , Acute Care Surgery
2.
J Neuroendovasc Ther ; 18(7): 197-202, 2024.
Article in English | MEDLINE | ID: mdl-39040914

ABSTRACT

Objective: Catastrophic antiphospholipid syndrome (CAPS) is a disease characterized by a poor prognosis and a high mortality rate, leading to systemic thrombosis. Approximately two-thirds of CAPS cases are associated with conditions such as infections, malignancies, surgical interventions, and events linked to the disease activity of systemic lupus erythematosus (SLE). Herein, we present a case of CAPS with multiorgan ischemia following ischemic stroke. Case Presentation: In this case report, a 33-year-old woman with a history of SLE and prolonged steroid use manifested impaired consciousness. Detection of the right internal carotid artery (ICA) occlusion led to successful ICA recanalization through endovascular thrombectomy. Postoperatively, she experienced pulmonary embolism and renal infarction. Although antiphospholipid syndrome (APS) was suspected, APS-related antibodies were negative. Anticoagulation therapy was initiated, presuming corticosteroid-induced thrombosis. However, she developed multiorgan thrombosis, culminating in multiple organ failure. Based on her clinical course, a diagnosis of CAPS was established. Intensive care and plasma exchange therapy were instrumental in her recovery, and she was discharged with a modified Rankin Scale score of 4. Conclusion: When encountering multiorgan ischemia following ischemic stroke in a young adult patient with an autoimmune disease, the consideration of CAPS as a differential diagnosis is crucial, even if APS-related antibodies test negative.

4.
Article in English | MEDLINE | ID: mdl-38926003

ABSTRACT

OBJECTIVES: To examine trends in the prevalence of multiorgan dysfunction (MODS), utilization of multi-organ support (MOS), and mortality among patients undergoing cardiac surgery with MODS who received MOS in the United States. DESIGN: Retrospective cohort study. SETTING: 183 hospitals in the Premier Healthcare Database. PARTICIPANTS: Adults ≥18 years old undergoing high-risk elective or non-elective cardiac surgery. INTERVENTIONS: none. MEASUREMENTS AND MAIN RESULTS: The exposure was time (consecutive calendar quarters) January 2008 and June 2018. We analyzed hospital data using day-stamped hospital billing codes and diagnosis and procedure codes to assess MODS prevalence, MOS utilization, and mortality. Among 129,102 elective and 136,190 non-elective high-risk cardiac surgical cases across 183 hospitals, 10,001 (7.7%) and 21,556 (15.8%) of patients developed MODS, respectively. Among patients who experienced MODS, 2,181 (22%) of elective and 5,425 (25%) of non-elective cardiac surgical cases utilized MOS. From 2008-2018, MODS increased in both high-risk elective and non-elective cardiac surgical cases. Similarly, MOS increased in both high-risk elective and non-elective cardiac surgical cases. As a component of MOS, mechanical circulatory support (MCS) increased over time. Over the study period, risk-adjusted mortality, in patients who developed MODS receiving MOS, increased in high-risk non-elective cardiac surgery and decreased in high-risk elective cardiac surgery, despite increasing MODS prevalence and MOS utilization (p<0.001). CONCLUSIONS: Among patients undergoing high-risk cardiac surgery in the United States, MODS prevalence and MOS utilization (including MCS) increased over time. Risk-adjusted mortality trends differed in elective and non-elective cardiac surgery. Further research is necessary to optimize outcomes among patients undergoing high-risk cardiac surgery.

5.
Medicina (Kaunas) ; 60(6)2024 Jun 20.
Article in English | MEDLINE | ID: mdl-38929623

ABSTRACT

Background and Objectives: Prolidase deficiency (PD) is a rare, life-threatening, genetically determined disease with an incidence of 1-2 cases per 1 million births. The disease inhibits collagen synthesis, which leads to organ and systems failure, including hepato- and splenomegaly, immune disorders, chronic ulcerative wounds, respiratory infections, and pulmonary fibrosis. The complexity of the problems associated with this disease necessitates a comprehensive approach and the involvement of an interdisciplinary team. The objective was to present the treatment and care plan, as well as complications of PD, in a young woman following admission to an intensive care unit (ICU). Materials and Methods: A retrospective observational single-case study. Results: A 26-year-old woman with PD was hospitalized in the ICU for acute respiratory failure. The presence of difficult-to-heal extensive leg ulcers and the patient's immunocompromised condition resulted in the development of sepsis with multiple organ failure (respiratory and circulatory, liver and kidney failure). Complex specialized treatment consisting of wound preparation, limb amputation, the minimization of neuropathic pain, mechanical ventilation, renal replacement therapy, circulatory stabilization, and the prevention of complications of the disease and of therapy were applied. On the 83rd day of hospitalization, the patient expired. Conclusions: Despite the use of complex treatment and care, due to the advanced nature of the disease and the lack of therapies with proven efficacy, treatment was unsuccessful. There is a need for evidence-based research to develop effective treatment guidelines for PD.


Subject(s)
Intensive Care Units , Multiple Organ Failure , Prolidase Deficiency , Sepsis , Humans , Female , Adult , Multiple Organ Failure/etiology , Sepsis/complications , Prolidase Deficiency/complications , Retrospective Studies , Fatal Outcome
6.
CEN Case Rep ; 2024 Jun 18.
Article in English | MEDLINE | ID: mdl-38888727

ABSTRACT

Carbamazepine (CBZ) intoxication can occur due to various factors, including drug interactions and over-ingestion. Extracorporeal elimination, particularly through hemodialysis and hemoperfusion, is effective in treating severe carbamazepine intoxication. However, as the effectiveness of various modalities can differ, method selection may be based on a specific clinical situation. A 47-year-old woman who took CBZ for schizophrenia presented to our hospital with episodes of vomiting and consciousness disorder. As the CBZ concentration was > 20 µg/mL, she was admitted to the intensive care unit with a diagnosis of acute CBZ poisoning. She underwent one session of hemoperfusion for 2 h, and her CBZ level decreased from > 20 µg/mL to 6.4 µg/mL. However, she developed acute kidney and liver injuries 2 days after admission and underwent intermittent hemodialysis, plasma exchange, continuous hemodiafiltration (CHDF), and online HDF, depending on her condition. Her general condition improved, and she was transferred to the psychiatric department. To our knowledge, no case reports have described severe acute CBZ poisoning in a patient who developed multiorgan failure to date, which was successfully treated with multimodal blood purification therapy. When treating severe CBZ intoxication, blood purification therapy should be tailored to the changing pathophysiology of the condition.

7.
J Investig Med High Impact Case Rep ; 12: 23247096241258074, 2024.
Article in English | MEDLINE | ID: mdl-38813977

ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) secondary to Histoplasma capsulatum is rare, impacting <1% globally, with a mortality rate of up to 31%. Herein, we present a rare case of HLH secondary to H capsulatum, affecting a 57-year-old female with rheumatoid arthritis. Extensive investigations were unrevealing and despite broad-spectrum antibiotics, her condition worsened, leading to respiratory failure requiring extracorporeal membrane oxygenation (ECMO) support, shock requiring multiple vasopressors, and acute kidney injury (AKI) requiring hemodialysis. Diagnosis confirmed disseminated histoplasmosis (DHP), prompting Amphotericin B and methylprednisolone treatment, resulting in significant improvement and discharge with posaconazole therapy. Secondary HLH, primarily arising from severe infections like DHP, is discussed. Limited research exists on this condition in human immunodeficiency virus (HIV)-seronegative individuals. Diagnosis involves HLH-2004 and HScore criteria. Managing histoplasmosis-associated HLH remains challenging due to multiorgan failure risks and treatment complexities and needs further research.


Subject(s)
Histoplasmosis , Lymphohistiocytosis, Hemophagocytic , Humans , Lymphohistiocytosis, Hemophagocytic/diagnosis , Histoplasmosis/diagnosis , Histoplasmosis/drug therapy , Histoplasmosis/complications , Female , Middle Aged , Antifungal Agents/therapeutic use , Histoplasma/isolation & purification , Amphotericin B/therapeutic use
8.
Cureus ; 16(4): e57980, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38738098

ABSTRACT

Systemic lupus erythematosus (SLE) is a persistent autoimmune disorder that manifests across a spectrum ranging from mild to severe disease, often requiring hospitalization and critical care management. We present a severe case of systemic involvement at its onset. A young woman, with a background of arterial hypertension, presented to the emergency department exhibiting a total anterior circulation stroke and exuberant symmetric lower limb edema. Her condition rapidly deteriorated with neurological impairment, respiratory failure requiring mechanical ventilation, and acute kidney injury prompting her admission to the ICU. Following clinical investigation, a diagnosis of SLE was established, according to the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 and Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria. The patient underwent treatment involving high-dose corticosteroids, followed by the Euro-Lupus protocol, resulting in significant improvement, despite her severe neurological deficit at admission. Lupus is a complex disease that is often difficult to diagnose because of its potential to mimic various other conditions. Our report delves into a case of previously undiagnosed lupus leading the patient to the ICU. The clinical scenario described adds valuable insights to the understanding of lupus-related complications and their management through a multidisciplinary approach.

9.
Cureus ; 16(3): e55711, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38586796

ABSTRACT

Sepsis is characterized by a dysregulated immune response to an infection. It is a major public health problem owing to its high mortality and morbidity. Sepsis is a medical emergency and requires aggressive and timely management. It can cause multiorgan failure, unmask an existing but undiagnosed disease such as ornithine transcarbamylase deficiency (OTCD), or make a known well-controlled disease worse. We present the case of a 52-year-old male who was brought to the emergency department unresponsive. He was diagnosed with severe sepsis which was associated with multiorgan failure and hyperammonemia crisis. Hyperammonemia was due to a newly diagnosed, late-onset OTCD which was unmasked by severe sepsis. This case will enable physicians to be aware and consider OTCD in a patient presenting with severe sepsis, altered mentation, and seizures, with no obvious cause of hyperammonemia.

10.
Eur J Clin Invest ; : e14229, 2024 Apr 27.
Article in English | MEDLINE | ID: mdl-38676423

ABSTRACT

Oxidative stress (OS) develops in critically ill patients as a metabolic consequence of the immunoinflammatory and degenerative processes of the tissues. These induce increased and/or dysregulated fluxes of reactive species enhancing their pro-oxidant activity and toxicity. At the same time, OS sustains its own inflammatory and immunometabolic pathogenesis, leading to a pervasive and vitious cycle of events that contribute to defective immunity, organ dysfunction and poor prognosis. Protein damage is a key player of these OS effects; it generates increased levels of protein oxidation products and misfolded proteins in both the cellular and extracellular environment, and contributes to forms DAMPs and other proteinaceous material to be removed by endocytosis and proteostasis processes of different cell types, as endothelial cells, tissue resident monocytes-macrophages and peripheral immune cells. An excess of OS and protein damage in critical illness can overwhelm such cellular processes ultimately interfering with systemic proteostasis, and consequently with innate immunity and cell death pathways of the tissues thus sustaining organ dysfunction mechanisms. Extracorporeal therapies based on biocompatible/bioactive membranes and new adsorption techniques may hold some potential in reducing the impact of OS on the defective proteostasis of patients with critical illness. These can help neutralizing reactive and toxic species, also removing solutes in a wide spectrum of molecular weights thus improving proteostasis and its immunometabolic corelates. Pharmacological therapy is also moving steps forward which could help to enhance the efficacy of extracorporeal treatments. This narrative review article explores the aspects behind the origin and pathogenic role of OS in intensive care and critically ill patients, with a focus on protein damage as a cause of impaired systemic proteostasis and immune dysfunction in critical illness.

11.
Int J Mol Sci ; 25(4)2024 Feb 08.
Article in English | MEDLINE | ID: mdl-38396750

ABSTRACT

Acute-on-chronic liver failure (ACLF) is a syndrome marked by sudden liver function decline and multiorgan failure, predominantly acute kidney injury (AKY), in patients with chronic liver disease. Unregulated inflammation is a hallmark of ACLF; however, the key drivers of ACLF are not fully understood. This study explores the therapeutic properties of human mesenchymal stem cell (MSC) secretome, particularly focusing on its enhanced anti-inflammatory and pro-regenerative properties after the in vitro preconditioning of the cells. We evaluated the efficacy of the systemic administration of MSC secretome in preventing liver failure and AKI in a rat ACLF model where chronic liver disease was induced using by the administration of porcine serum, followed by D-galN/LPS administration to induce acute failure. After ACLF induction, animals were treated with saline (ACLF group) or MSC-derived secretome (ACLF-secretome group). The study revealed that MSC-secretome administration strongly reduced liver histological damage in the ACLF group, which was correlated with higher hepatocyte proliferation, increased hepatic and systemic anti-inflammatory molecule levels, and reduced neutrophil and macrophage infiltration. Additionally, renal examination revealed that MSC-secretome treatment mitigated tubular injuries, reduced apoptosis, and downregulated injury markers. These improvements were linked to increased survival rates in the ACLF-secretome group, endorsing MSC secretomes as a promising therapy for multiorgan failure in ACLF.


Subject(s)
Acute-On-Chronic Liver Failure , Humans , Rats , Animals , Swine , Acute-On-Chronic Liver Failure/therapy , Secretome , Stem Cells , Anti-Inflammatory Agents
12.
Intern Med ; 63(8): 1167-1172, 2024 Apr 15.
Article in English | MEDLINE | ID: mdl-38296478

ABSTRACT

A 45-year-old woman was hospitalized with severe coronavirus disease 2019 pneumonia. Following cytokine storm-induced multiorgan failure and lethal arrhythmia, the patient developed a sustained coma with flaccid quadriplegia. A cerebrospinal fluid examination excluded infectious and immunogenic encephalopathies, and diffusion-weighted magnetic resonance imaging demonstrated high-intensity areas in the white matter with a cortex-sparing distribution, suggesting delayed post-hypoxic leukoencephalopathy. As a result of intensive cardiopulmonary support for a month, the neurological function gradually recovered. Based on the reversible clinical course noted in this patient, accurate diagnosis and persistent medical approaches are important for the management of coronavirus disease 2019-related delayed post-hypoxic leukoencephalopathy.


Subject(s)
COVID-19 , Leukoencephalopathies , Female , Humans , Middle Aged , COVID-19/complications , Leukoencephalopathies/diagnostic imaging , Leukoencephalopathies/etiology , Hypoxia/etiology , Diffusion Magnetic Resonance Imaging , Disease Progression , Magnetic Resonance Imaging
13.
Ann Pathol ; 44(2): 150-151, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37865572

ABSTRACT

A 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in granules in PAS and Giemsa stains should establish the diagnosis of suspicion.


Subject(s)
Sea-Blue Histiocyte Syndrome , Female , Humans , Aged , Sea-Blue Histiocyte Syndrome/complications , Sea-Blue Histiocyte Syndrome/diagnosis , Ceroid , Splenomegaly/complications , Hepatomegaly/etiology
14.
Chirurgie (Heidelb) ; 95(2): 115-121, 2024 Feb.
Article in German | MEDLINE | ID: mdl-37978073

ABSTRACT

A review and discussion of the current literature on liver transplantation for acute-on-chronic liver failure (ACLF) was performed. The ACLF represents an acute deterioration of liver function with pre-existing liver disease and is associated with increasing multiorgan failure, depending on the stage. The 28-day mortality ranges to well over 70% in stage 3 and requires rapid intensive medical treatment involving an interdisciplinary team experienced in transplantation medicine. Under optimized conditions, liver transplantation provides long-term survival rates comparable to other indications. Achieving this requires a differentiated donor selection, choosing the appropriate time for transplantation in the context of a dynamic disease course and the use of appropriate surgical techniques.


Subject(s)
Acute-On-Chronic Liver Failure , Liver Transplantation , Humans , Acute-On-Chronic Liver Failure/surgery , Multiple Organ Failure
15.
Clin Case Rep ; 11(10): e7993, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37808572

ABSTRACT

Epstein-Barr virus (EBV) is a commonly asymptomatic widespread human herpes virus affecting over 90% of the population. It mostly originates complications like simple sore throat and infectious mononucleosis but severe manifestations are rare. Herein we report a 30-year-old immunocompetent man who presented with fever, sore throat, general weakness, and drowsiness. The diagnosis was formulated based on the positive RT-PCR test for EBV DNA and serological detection of IgM antibody against viral capsid antigen. The patient developed severe meningoencephalitis, myocarditis, and bowel perforation and passed away after 72 days of hospitalization.

16.
J Int Med Res ; 51(9): 3000605231197461, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37776529

ABSTRACT

OBJECTIVES: Acute pancreatitis (AP) is a severe complication of leptospirosis. This review focuses on the current evidence of AP in patients with leptospirosis. METHODS: Data on clinical characteristics, biochemical parameters, diagnosis, complications, critical care, fluid management, operative management, and outcomes were analyzed. This study was registered in PROSPERO (CRD42022360802). RESULTS: We included 35 individual case reports and 4 case series involving 79 patients. Sex was reported for 48 (60.7%) patients; 38 (48.1%) were male and 10 (12.6%) were female. The patients' mean age was 45.13 (15-83 years). Acute kidney injury, thrombocytopenia, hypotension, and liver injury were the most common complications reported. Complete recovery was reported for 36 (45.5%) patients. Biochemical and radiological recovery was reported for 10 (12.6%) and 9 (11.3%) patients, respectively. Death was reported in 18 (22.7%) patients. CONCLUSION: A high degree of clinical suspicion and different modalities of investigations are essential in the diagnosis of AP in leptospirosis. AP can be easily missed in leptospirosis because both conditions share similar clinical presentations and complications. Because of the high prevalence of acute kidney injury, judicious fluid management and close monitoring are mandatory.


Subject(s)
Acute Kidney Injury , Leptospirosis , Pancreatitis , Thrombocytopenia , Humans , Male , Female , Middle Aged , Pancreatitis/complications , Pancreatitis/therapy , Pancreatitis/diagnosis , Acute Disease , Leptospirosis/complications , Leptospirosis/epidemiology , Acute Kidney Injury/therapy , Acute Kidney Injury/complications
17.
Cureus ; 15(7): e41781, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37575835

ABSTRACT

Immune checkpoint inhibitors (ICIs) as standard of care have revolutionized the treatment of patients with metastatic melanoma. The combination of nivolumab and ipilimumab improves treatment efficacy and prolongs survival compared to monotherapy alone. However, combination therapy is also associated with an increased incidence of adverse events. We report an uncommon yet important case of multi-organ failure in a patient following a single dose of nivolumab plus ipilimumab. A 60-year-old male with a history of ulcerative colitis in remission and metastatic melanoma was admitted on February 25, 2021, for presumed sepsis, after presenting with neutropenic fever. His brain metastases were previously resected on January 14, 2021, and he was started on dexamethasone 4 mg BID for six weeks. On February 11, 2021, he received one dose of nivolumab plus ipilimumab, per the CheckMate-067 protocol. He presented 14 days later with fever, diarrhea, pancytopenia, renal failure, drug-induced hepatitis, and myocarditis. The infectious workup was negative. His neutropenia responded to growth factors. He was diagnosed with interstitial nephritis due to immunotherapy and treated with corticosteroids. His symptoms resolved with concomitant improvement of his renal, hepatic, and cardiac function. He was discharged home in a stable condition. Although these specific immune-related adverse events (irAEs) are uncommon and rarely occur simultaneously, ICIs can trigger non-specific immune system activation, resulting in widespread inflammatory effects. Since irAEs can lead to multi-organ failure, as evidenced in this case, early recognition and institution of high-dose steroids are critical to preventing rapid deterioration. Given that ICI therapy is the standard of care for several cancers and is often studied in clinical trials, increased education on irAE toxicity and updated algorithms on the management of febrile cancer patients are warranted.

18.
Cureus ; 15(6): e41054, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37519525

ABSTRACT

Secondary bacterial infections post-COVID infection posed a major challenge to the healthcare settings during the COVID pandemic. We present the case of an 81-year-old patient who was initially admitted for COVID pneumonia in a tertiary care hospital and was managed with a course of dexamethasone and had a good outcome. However, post-discharge, the patient developed symptoms of productive cough, hemoptysis and shortness of breath. Evaluation of the patient revealed that the patient had developed a secondary bacterial infection with Methicillin-resistant Staphylococcus aureus (MRSA), resulting in MRSA pneumonia and empyema. The patient was started on appropriate antibiotics and underwent thoracocentesis followed by video-assisted thoracoscopic surgery (VATS) and decortication. MRSA infection resulted in severe septic shock, acute respiratory distress syndrome (ARDS) and multiorgan failure. Successful intensive care unit (ICU) management of the life-threatening complications resulted in the remarkable recovery of the patient.

19.
BMC Complement Med Ther ; 23(1): 273, 2023 Jul 31.
Article in English | MEDLINE | ID: mdl-37525199

ABSTRACT

BACKGROUND: Xanthii Fructus was used in the treatment of rhinitis and related nasal disease. It is the most commonly used chemically active component in compounds formulated for the treatment of rhinitis. However, poisoning, resulting in serious consequences, can easily occur owing to cocklebur overdose, improper processing, or usage without processing. CASE PRESENTATION: We reported on a 55-year-old man who experienced allergic rhinitis for 2.5 years. He ingested unprocessed Xanthii Fructus for 2 months as treatment. However, he developed anorexia; nausea; abdominal pain; general weakness; hiccups; oliguria and anuria; significantly elevated serum alanine aminotransferase, aspartate aminotransferase, and creatinine levels; and abnormalities in blood coagulation series. Nutritional support; daily drugs for liver protection, gastric protection, inflammation reduction; fresh plasma; and cryoprecipitate infusion were administered. Continuous venovenous hemodialysis (Prismaflex ST100) was also administered. However, the patient's multiple organ failure gradually worsened, ultimately leading to death. CONCLUSION: Xanthii Fructus poisoning affects multiple systems, and its clinical manifestations are complex. Therefore, it is easily misdiagnosed and missed. Along with careful inquiry of medical and medication history, early diagnosis and intervention are vital for a successful treatment. It is also important to educate people and create awareness about this poisoning. Therefore, this intractable case has great clinical significance.


Subject(s)
Drugs, Chinese Herbal , Rhinitis , Male , Humans , Middle Aged , Drugs, Chinese Herbal/chemistry , Multiple Organ Failure/chemically induced , Liver , Fruit/chemistry
20.
J Investig Med High Impact Case Rep ; 11: 23247096231168111, 2023.
Article in English | MEDLINE | ID: mdl-37085971

ABSTRACT

We are reporting monochorionic, diamniotic twin premature infants born at 25 weeks and 6 days gestation with riboflavin (vitamin B2) and biotin (vitamin B7) deficiency, while on prolonged total parenteral nutrition (TPN) during vitamin shortage. They presented initially with skin rash, lactic acidosis, and thrombocytopenia. Both twins progressed to severe respiratory failure, severe lactic acidosis, with refractory vasodilatory shock, pancytopenia, ischemic bowel injury, acute kidney injury, liver injury, and capillary leak syndrome leading to death of twin A. The surviving twin B was diagnosed with riboflavin and biotin deficiency that presented with abnormal metabolic work up suggestive of maple syrup urine disease, glutaric acidemia type 2, and X-linked adrenoleukodystrophy. Twin B was started on riboflavin and biotin supplementation at 41 days of life, with rapid improvement in clinical findings and laboratory abnormalities within days of starting biotin and riboflavin supplementation. He was discharged home in stable condition at 49 weeks of postmenstrual age.


Subject(s)
Acidosis, Lactic , Exanthema , Thiamine Deficiency , Male , Infant, Newborn , Infant , Humans , Acidosis, Lactic/chemically induced , Biotin/adverse effects , Parenteral Nutrition/adverse effects , Infant, Premature , Riboflavin/adverse effects , Vitamins , Multiple Organ Failure/etiology
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