RESUMEN
INTRODUCTION: Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease, which is mainly characterized by joint swelling, pressure pain and joint destruction. Some patients may suffer from a variety of serious complications, which require prompt diagnosis and treatment. Otherwise, the patient condition may deteriorate rapidly, leading to premature death. OBJECTIVE: We reported a case of RA combined with hyperferritinemic syndrome and capillary leak syndrome (CLS) that was successfully treated with tocilizumab (TCZ), with the aim of improving diagnostic ideas for clinicians and consequently improving the diagnosis and treatment of the hyperferritinemic syndrome and CLS. CASE SUMMARY: A 55-year-old female patient was admitted to the Department of Infectious Diseases of our hospital due to "recurrent fever for more than 1 month and aggravation for 3 days." The patient was diagnosed with fever of unknown origin (lung infection?) and received anti-infective therapy with large encirclement of anti-bacterial, antifungal and empirical anti-tuberculosis successively during hospitalization in the Department of Infectious Diseases. Yet her condition continues to progress. The patient was eventually diagnosed with RA combined with hyperferritinemic syndrome and CLS. Then she received glucocorticoids (GC) (160 mg qd) combined with intravenous immunoglobulin (IVIG, 20 g/d, for 3 days). We considered that the patient also had an overwhelming proinflammatory cytokine storm, so she received a strong anti-inflammatory treatment with TCZ (400 mg qm). The patient symptoms and follow-up chest CT showed significant improvement following treatment. CONCLUSION: TCZ has good efficacy in the treatment of RA combined with hyperferritinemic syndrome and CLS and is expected to be a promising treatment.
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Anticuerpos Monoclonales Humanizados , Artritis Reumatoide , Síndrome de Fuga Capilar , Hiperferritinemia , Humanos , Femenino , Persona de Mediana Edad , Hiperferritinemia/tratamiento farmacológico , Hiperferritinemia/etiología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Artritis Reumatoide/complicaciones , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/etiología , SíndromeRESUMEN
Systemic capillary leak syndrome (SCLS) is a rare and complex adverse effect of immune checkpoint inhibitors (ICIs). The diagnosis of drug-induced SCLS is based on diffuse infusions of exudative fluid into the interstitial areas and the exclusion of other causes. The best management of ICIs-induced SCLS is not settled, though proper supportive care and corticosteroids were commonly applied as the first-line treatment. In our patient with advanced gastroesophageal junction squamous cell carcinoma, although ICIs-induced SCLS was successfully controlled with corticosteroids, the patient soon experienced cancer progress and died of pulmonary infections. Based on our experience and the reported cases by other hospitals, different stages of SCLS might respond differently to the same treatment. Therefore, a grading of ICIs-induced SCLS might help to stratify the patient for different treatment strategies. Besides, corticosteroids-sensitive patients, though waived from deadly SCLS, might be at higher risk of cancer progress and subsequent infections due to the application of corticosteroids. Considering that the inflammatory factors should be closely involved in the development of ICIs-induced SCLS, targeted therapy against the driver inflammatory cytokine might offer treatment regimens that are more effective and safer.
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Síndrome de Fuga Capilar , Carcinoma de Células Escamosas , Humanos , Síndrome de Fuga Capilar/inducido químicamente , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados/efectos adversos , Carcinoma de Células Escamosas/complicaciones , Corticoesteroides/uso terapéuticoRESUMEN
Systemic capillary leak syndrome is a rare derangement of endothelial function characterised by extravasation of plasma and proteins into the interstitial space. Primary capillary leak syndrome is a rare, episodic medical illness of unknown molecular pathology while secondary capillary leak syndrome may be precipitated by any number of inflammatory and infectious syndromes. Decompression sickness, a disorder of depressurisation, has been identified as a very rare trigger. We present a structured case report of systemic capillary leak syndrome secondary to decompression sickness following deep diving, informing physicians of this potential complication. No pharmacological therapy has substantial evidence in the treatment of acute systemic capillary leak syndrome. By review of current recommendations for acute management, we also emphasise an observed positive response to judicious fluid resuscitation and an oral cyclic AMP-elevating agent (ie, terbutaline).
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Síndrome de Fuga Capilar , Enfermedad de Descompresión , Humanos , Síndrome de Fuga Capilar/tratamiento farmacológico , Enfermedad de Descompresión/complicaciones , Terbutalina , Fluidoterapia/efectos adversos , PlasmaRESUMEN
A Japanese man experienced three episodes of hypovolemic shock and was diagnosed with systemic capillary leak syndrome (SCLS). He developed SCLS exacerbation 2 days after receiving a second dose of the Pfizer-BioNTech BNT162b2 mRNA COVID-19 vaccine, 1 year after the third episode. After fluid therapy and albumin administration, we initiated terbutaline and theophylline prophylaxis for SCLS. A literature review revealed that SCLS attacks often occur 1-2 days after the second COVID-19 vaccination. Patients with a history of SCLS should avoid COVID-19 vaccination and be carefully monitored for 1-2 days if they receive the vaccine.
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Vacunas contra la COVID-19 , COVID-19 , Síndrome de Fuga Capilar , Humanos , Masculino , Vacuna BNT162 , Síndrome de Fuga Capilar/etiología , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/tratamiento farmacológico , COVID-19/prevención & control , Vacunas contra la COVID-19/efectos adversos , TerbutalinaRESUMEN
Systemic capillary leak syndrome is a rare and life-threatening disorder, characterized by recurrent episodes of unexplained hypotension, hemoconcentration, and hypoalbuminemia. This condition is caused by leakage of plasma and proteins into the extravascular space and can be classified as either idiopathic or secondary. Secondary systemic capillary leak syndrome can result from cancer, infections, medications, or surgery. Systemic capillary leak syndrome frequently develops as a side effect of denileukin diftitox treatment of refractory cutaneous T-cell lymphoma. However, the pathophysiology of this disease is not well understood. Herein, we report a case of denileukin diftitox-induced systemic capillary leak syndrome.
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Lesión Renal Aguda , Síndrome de Fuga Capilar , Neoplasias Cutáneas , Humanos , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/inducido químicamente , Interleucina-2/efectos adversos , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Lesión Renal Aguda/inducido químicamente , Lesión Renal Aguda/complicacionesRESUMEN
BACKGROUND: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown. OBJECTIVE: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS. METHODS: We conducted a retrospective multicenter study including all adult patients with monoclonal gammopathy-associated SCLS from the EurêClark registry who received at least 1 course of IVIG. The primary end point was overall survival according to IVIG withdrawal. RESULTS: Fifty-nine patients of mean ± SD age 51 ± 13 years were included. Overall cumulative probabilities of 2-, 5-, 10- and 15-year survival were 100%, 85%, 72%, 44%, respectively. The IVIG was withdrawn at least once in 18 patients (31%; W+ group) and never in 41 patients (69%; W- group). Cumulative probabilities of 10-year survival in W+ versus W- groups were 50% and 83% (log rank test, P = .02), respectively. Relapse rate and the median number of relapses in the W+ versus the W- groups were 72% versus 58% (P = 0.3) and 2.5 (0.3-4) versus 1 (0-2) (P = .03), respectively. The IVIG tapering was not statistically associated with increased person-year incidence of attacks using a mixed linear model. CONCLUSIONS: The IVIG withdrawal was associated with increased mortality and higher rate of recurrence in SCLS patients. The IVIG tapering might be cautiously considered in stable SCLS patients.
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Síndrome de Fuga Capilar , Paraproteinemias , Adulto , Humanos , Persona de Mediana Edad , Síndrome de Fuga Capilar/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Paraproteinemias/complicaciones , Estudios Retrospectivos , IncidenciaRESUMEN
Systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare disorder of unknown aetiology. Since SCLS was first described in 1960, fewer than 500 cases have been reported. SCLS is diagnosed by the classic triad of hypotension, haemoconcentration and hypoalbuminaemia resulting from fluid extravasation. Some reports show that SCLS may sometimes occur as a side effect of adenoviral vector COVID-19 vaccines, although there is only one report (two cases) of SCLS after receiving a messenger RNA vaccine. Survival rates for SCLS are very poor without treatment, so it is crucial for clinicians to recognise this disorder. A middle-aged woman who presented with generalised malaise and anasarca after receiving the BNT162b2 COVID-19 vaccine was diagnosed with SCLS. Treatment with methylprednisolone and intravenous immunoglobulin was commenced and her symptoms resolved. We expect that this case report will add to the existing literature on this rare disorder and the side effects of vaccinations.
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COVID-19 , Síndrome de Fuga Capilar , Vacunas , Vacuna BNT162 , COVID-19/prevención & control , Vacunas contra la COVID-19/efectos adversos , Síndrome de Fuga Capilar/tratamiento farmacológico , Femenino , Humanos , Persona de Mediana Edad , ARN Mensajero/uso terapéutico , Vacunas/uso terapéutico , Vacunas Sintéticas , Vacunas de ARNmRESUMEN
Systemic capillary leak syndrome (SCLS) is a syndrome caused by many reasons and without a definitive mechanism. The main diagnostic criteria of SCLS are hemoconcentration, hypoalbuminemia, and hypotension. Though most SCLS improved spontaneously within a few days, it can be life-threatening without effective treatments. In previous literature, vascular endothelial growth factor (VEGF) inhibitor had shown its potential to be an effective treatment, but the treatment outcomes were inconsistent. This article was about a 58-year-old female suffering from refractory systemic capillary leak syndrome after bone marrow transplantation and being treated with bevacizumab, a VEGF inhibitor. In comparison with other successfully treated cases, this patient received four cycles of bevacizumab treatment without symptomatic improvement and eventually died in the intensive care unit. Further studies are needed to further confirm the role of bevacizumab in the management of SCLS.
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Síndrome de Fuga Capilar , Bevacizumab/uso terapéutico , Síndrome de Fuga Capilar/tratamiento farmacológico , Femenino , Humanos , Persona de Mediana Edad , Resultado del Tratamiento , Factor A de Crecimiento Endotelial VascularRESUMEN
Snakebite envenomation caused by the Western and Eastern Russell's Vipers (Daboia russelii and Daboia siamensis) may potentially induce capillary leak syndrome (CLS), while the use of antivenom in treating this has not been well examined. This study investigated the CLS-inducing toxicity of Russell's Viper venoms from various sources and examined the neutralization activity of regionally available antivenoms, using a newly devised mouse model. D. russelii venoms demonstrated a more consistent vascular leakage activity (76,000-86,000 CLS unit of vascular leak index, a function of the diameter and intensity of Evans Blue dye extravasation into dermis) than D. siamensis venoms (33,000-88,000 CLS unit). Both species venoms increased hematocrits markedly (53-67%), indicating hemoconcentration. Regional antivenoms (DsMAV-Thailand, DsMAV-Taiwan, VPAV-India) preincubated with the venoms effectively neutralized the CLS effect to different extents. When the antivenoms were administered intravenously post-envenomation (challenge-rescue model), the neutralization was less effective, implying that CLS has a rapid onset that preceded the neutralizing activity of antivenom, and/or the antivenom has limited biodistribution to the venom's inoculation site. In conclusion, Russell's Viper venoms of both species from various locales induced CLS in mice. Antivenoms generally had limited efficacy in neutralizing the CLS effect. Innovative treatment for venom-induced CLS is needed.
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Antivenenos/farmacología , Síndrome de Fuga Capilar/tratamiento farmacológico , Daboia , Mordeduras de Serpientes/tratamiento farmacológico , Venenos de Víboras/toxicidad , Animales , Vasos Sanguíneos/efectos de los fármacos , Síndrome de Fuga Capilar/inducido químicamente , Síndrome de Fuga Capilar/patología , Femenino , Ratones , Ratones Endogámicos ICR , Pruebas de Neutralización , Mordeduras de Serpientes/inducido químicamente , Mordeduras de Serpientes/patologíaAsunto(s)
Vacunas contra la COVID-19/efectos adversos , COVID-19/prevención & control , Síndrome de Fuga Capilar/diagnóstico , Anciano , Síndrome de Fuga Capilar/tratamiento farmacológico , Fármacos Cardiovasculares/uso terapéutico , Progresión de la Enfermedad , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Persona de Mediana Edad , Pandemias , Factores de Riesgo , SARS-CoV-2 , Terbutalina/uso terapéutico , Teofilina/uso terapéuticoRESUMEN
BACKGROUND: Capillary leakage is a key contributor to the pathological host response to infections. The underlying mechanisms remain incompletely understood, and the role of microRNAs (MIR) has not been investigated in detail. We hypothesized that specific MIRs might be regulated directly in the endothelium thereby contributing to vascular leakage. METHODS: SmallRNA sequencing of endotoxemic murine pulmonary endothelial cells (ECs) was done to detect regulated vascular MIRs. In vivo models: transgenic zebrafish (flk1:mCherry/l-fabp:eGFP-DPB), knockout/wildtype mouse (B6.Cg-Mir155tm1.1Rsky/J); disease models: LPS 17.5 mg/kgBW and cecal ligation and puncture (CLP); in vitro models: stimulated human umbilical vein EC (HUVECs), transendothelial electrical resistance. RESULTS: Endothelial MIR155 was identified as a promising candidate in endotoxemic murine pulmonary ECs (25 × upregulation). Experimental overexpression in a transgenic zebrafish line and in HUVECs was sufficient to induce spontaneous vascular leakage. To the contrary, genetic MIR155 reduction protects against permeability both in vitro and in endotoxemia in vivo in MIR155 heterozygote knockout mice thereby improving survival by 40%. A tight junction protein, Claudin-1, was down-regulated both in endotoxemia and by experimental MIR155 overexpression. Translationally, MIR155 was detectable at high levels in bronchoalveolar fluid of patients with ARDS compared to healthy human subjects. CONCLUSIONS: We found that MIR155 is upregulated in the endothelium in mouse and men as part of a systemic inflammatory response and might contribute to the pathophysiology of vascular leakage in a Claudin-1-dependent manner. Future studies have to clarify whether MIR155 could be a potential therapeutic target.
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Síndrome de Fuga Capilar/tratamiento farmacológico , Endotelio Vascular/efectos de los fármacos , MicroARNs/farmacología , Animales , Síndrome de Fuga Capilar/etiología , Endotelio Vascular/metabolismo , Humanos , Ratones , MicroARNs/uso terapéutico , Síndrome de Respuesta Inflamatoria Sistémica/complicaciones , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Pez CebraRESUMEN
A 64-year-old man had a several year history of B prolymphocytic leukaemia (PLL) which behaved indolently and had not required any treatment. Five years after diagnosis, he developed hypoalbuminaemia associated with severe lower-limb oedema, consistent with systemic capillary leak syndrome (SCLS). He recovered spontaneously but went on to have three further increasingly severe and protracted episodes over the subsequent 18 months. There was no identifiable precipitating factor for these episodes, but his peripheral lymphocyte count continued to increase slowly. The start of treatment for his PLL with chemoimmunotherapy was followed by a rapid resolution of residual oedema and normalisation of serum albumin. He has had no further attacks of SCLS in the 14 months since he started therapy for PLL. SCLS is a rare consequence of haematological malignancy which may show an excellent response to treatment of the haematological disease.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Síndrome de Fuga Capilar/etiología , Edema/etiología , Hipoalbuminemia/etiología , Leucemia Prolinfocítica Tipo Células B/complicaciones , Síndrome de Fuga Capilar/sangre , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Edema/sangre , Edema/diagnóstico , Edema/tratamiento farmacológico , Humanos , Hipoalbuminemia/sangre , Hipoalbuminemia/diagnóstico , Leucemia Prolinfocítica Tipo Células B/sangre , Leucemia Prolinfocítica Tipo Células B/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Rituximab/uso terapéutico , Albúmina Sérica Humana/análisis , Resultado del Tratamiento , Vidarabina/análogos & derivados , Vidarabina/uso terapéuticoRESUMEN
We report herein the case of a patient with low blood levels of different hormones during a systemic capillary leak syndrome (Clarkson's disease) attack.
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Aldosterona/sangre , Síndrome de Fuga Capilar/sangre , Hormonas Esteroides Gonadales/sangre , Péptido Natriurético Encefálico/sangre , Hormonas Tiroideas/sangre , Adulto , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/patología , Femenino , HumanosRESUMEN
The current World Health Organization guidance is not to start corticosteroids, but there is no robust evidence of risk in patients with cancer and coronavirus disease 2019. A risk-benefit analysis should be performed for each patient on the use of steroids in cancer care.
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Corticoesteroides/uso terapéutico , Infecciones por Coronavirus/epidemiología , Neumonía Viral/epidemiología , Guías de Práctica Clínica como Asunto , Neoplasias Urológicas/tratamiento farmacológico , Antineoplásicos Hormonales/uso terapéutico , Antineoplásicos Inmunológicos/efectos adversos , Betacoronavirus , COVID-19 , Síndrome de Fuga Capilar/inducido químicamente , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/prevención & control , Femenino , Humanos , Inflamación/inducido químicamente , Inflamación/tratamiento farmacológico , Neoplasias Renales/tratamiento farmacológico , Masculino , Pandemias , Neoplasias de la Próstata/tratamiento farmacológico , Medición de Riesgo , Factores de Riesgo , SARS-CoV-2 , Neoplasias de la Vejiga Urinaria/tratamiento farmacológicoRESUMEN
BACKGROUND: Burn injury is associated with a long-standing inflammatory reaction. The use of albumin solutions for plasma volume support is controversial because of concerns of increased capillary leakage, which could aggravate the commonly seen interstitial oedema. METHODS: In the present open controlled clinical trial, an intravenous infusion of 20% albumin at 3 mL/kg was given over 30 min to 15 burn patients and 15 healthy volunteers. Blood samples and urine were collected for 5 h. Plasma dilution, plasma albumin and colloid osmotic pressure were compared. Mass balance calculations were used to estimate plasma volume expansion and capillary leakage of fluid and albumin. RESULTS: The patients were studied between 4 and 14 (median, 7) days after the burn injury, which spread over 7-48% (median, 15%) of the total body surface area. The albumin solution expanded the plasma volume by almost 15%, equivalent to twice the infused volume, in both groups. The urinary excretion exceeded the infused volume by a factor of 2.5. Capillary leakage of albumin occurred at a rate of 3.4 ± 1.5 g/h in burn patients and 3.7 ± 1.6 g/h in the volunteers (P = 0.61), which corresponded to 2.4 ± 1.0% and 2.5 ± 1.2% per hour of the intravascular pool (P = 0.85). The median half-life of the plasma volume expansion was 5.9 (25th-75th percentiles 2.7-11.7) h in the burn patients and 6.9 (3.4-8.5) h in the volunteers (P = 0.56). CONCLUSIONS: Albumin 20% was an effective volume expander in patients at 1 week post-burn. No relevant differences were found between burn patients and healthy volunteers. TRIAL REGISTRATION: EudraCT 2016-000996-26 on May 31, 2016.
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Quemaduras/complicaciones , Síndrome de Fuga Capilar/etiología , Sustitutos del Plasma/farmacología , Adulto , Quemaduras/tratamiento farmacológico , Quemaduras/fisiopatología , Síndrome de Fuga Capilar/tratamiento farmacológico , Síndrome de Fuga Capilar/prevención & control , Femenino , Humanos , Masculino , Sustitutos del Plasma/uso terapéutico , Volumen Plasmático/efectos de los fármacos , Albúmina Sérica HumanaAsunto(s)
Síndrome de Fuga Capilar/complicaciones , Miocardio/patología , Choque Cardiogénico/etiología , Adulto , Biopsia , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/tratamiento farmacológico , Humanos , Inmunoglobulinas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Infusiones Intravenosas , Masculino , Choque Cardiogénico/diagnósticoRESUMEN
Capillary leak syndrome is a critical condition occasionally occurring posttransplant and is characterized by acute endothelial hyperpermeability leading to systemic protein-rich fluid extravasation and consequent hypovolemia, hypoperfusion, and acute kidney injury. Treatment is merely supportive and is based on osmotic drugs, diuretics, continuous renal replacement therapy, and surgical drainage. However, removal of the underlying inflammatory cause is mandatory to achieve stable resolution. Herein, we report the first successful treatment with colchicine in 2 life-threatening pediatric cases of capillary leak syndrome with renal failure occurring after transplant (heart and bone marrow) and unresponsive to any other line of therapy. Both cases were only palliated by supportive therapy and revealed an impressively rapid response to colchicine both in terms of diuresis and clinical condition recovery, allowing for the cessation of renal replacement therapy in a few hours. In both patients, colchicine was temporarily discontinued for transient leukopenia (attributed to an additive effect with mycophenolate mofetil), resulting in extravasation, and renal failure recurrence was restored only after colchicine reintroduction. Although the association of colchicine with an immunosuppressive drug was formerly contraindicated, no other adverse events were noted when using a minimized dose. Both patients are now maintaining a good renal function without recurrence of extravasation after 6 months of follow-up. In conclusion, this strikingly positive experience forces physicians to consider this old and cost-effective drug as a new, powerful rescue tool in such critical cases.
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Síndrome de Fuga Capilar/tratamiento farmacológico , Colchicina/administración & dosificación , Trasplante de Corazón/efectos adversos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Insuficiencia Renal/tratamiento farmacológico , Moduladores de Tubulina/administración & dosificación , Adolescente , Síndrome de Fuga Capilar/diagnóstico , Síndrome de Fuga Capilar/etiología , Niño , Femenino , Trasplante de Corazón/tendencias , Trasplante de Células Madre Hematopoyéticas/tendencias , Humanos , Masculino , Insuficiencia Renal/diagnóstico , Insuficiencia Renal/etiologíaRESUMEN
BACKGROUND: Postoperative fluid management in critically ill neonates and infants with capillary leak syndrome (CLS) and extensive volume overload after cardiac surgery on cardiopulmonary bypass is challenging. CLS is often resistant to conventional diuretic therapy, aggravating the course of weaning from invasive ventilation, increasing length of stay on ICU and morbidity and mortality. METHODS: Tolvaptan (TLV, vasopressin type 2 receptor antagonist) was used as an additive diuretic in neonates and infants with CLS after cardiac surgery. Retrospective analysis of 25 patients with CLS including preoperative and postoperative parameters was performed. Multivariate regression analysis was performed to identify predictors for TLV response. RESULTS: Multivariate analysis identified urinary output during 24 h after TLV administration and mean blood pressure (BP) on day 2 of TLV treatment as predictors for TLV response (AUC = 0.956). Responder showed greater weight reduction (p < 0.0001), earlier weaning from ventilator during TLV (p = 0.0421) and shorter time in the ICU after TLV treatment (p = 0.0155). Serum sodium and serum osmolality increased significantly over time in all patients treated with TLV. CONCLUSION: In neonates and infants with diuretic-refractory CLS after cardiac surgery, additional aquaretic therapy with TLV showed an increase in urinary output and reduction in bodyweight in patients classified as TLV responder. Increase in urinary output and mean BP on day 2 of treatment were strong predictors for TLV response.