scholarly journals Haemophagocytic Lymphohistiocytosis with Leptospirosis: A Rare but Devastating Complication

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
B. M. Munasinghe ◽  
A. G. Arambepola ◽  
N. Pathirage ◽  
U. P. M. Fernando ◽  
N. Subramaniam ◽  
...  

Introduction. Secondary haemophagocytic lymphohistiocytosis (sHLH), often associated with an array of infections, malignancies, and autoimmune diseases, is rarely seen with leptospirosis, which carries a relatively poor prognosis even with modern state-of-the-art medical care. We describe a patient with leptospirosis complicated by sHLH who succumbed to illness following multiorgan dysfunction. Case Description. A 74-year-old farmer presented with high-grade, unsettling fever for a week. Muddy water exposure and suggestive symptoms prompted investigation and management in the line of leptospirosis (IV ceftriaxone was instituted, and later, MAT (microscopic agglutination test) became positive). Subsequently, he developed severe acute hypoxemia requiring mechanical ventilation and acute renal failure requiring renal replacement therapy. Bone marrow biopsy and markedly elevated serum ferritin and triglyceride levels done on day 10 (with unresolving fever, hepatosplenomegaly, and pancytopaenia) confirmed the diagnosis of HLH. The routine cultures, retroviral studies, CMV, dengue, hanta and mycoplasma antibodies, tuberculosis and COVID-19 PCR, and malaria screening were all normal. There was no improvement of hypoxemia following intravenous methylprednisolone. He died on day 15 despite escalating organ support. Conclusion. Leptospirosis is a common zoonotic disease in the tropics with significant morbidity and mortality. In the case of severe leptospirosis, overlapping clinical features with sHLH make the diagnosis of the latter challenging. No assessment tools are available to date to predict the risk of developing sHLH in a patient having leptospirosis. Outcome following sHLH due to leptospirosis still remains majorly ominous. A high index of suspicion and low threshold for specific investigations could possibly alter the outcome following such an occurrence.


QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
Y M Kotb ◽  
M K Abdelfattah ◽  
I M Saleh

Abstract Objective to demonstrate the risk factors of developing subcapsular renal hematoma ( SRH) in patients who underwent ureteroscopic lithotripsy for upper ureteric stones. Materials and Methods In a randomized controlled trial, 60 patients with an upper ureteric stone underwent URS lithotripsy either by laser or pneumatic. The inclusion criteria were age above 14 years old, of both sexes with an upper ureteric stone about 2 cm in diameter. Exclusion criteria were age below 14 years old, elevated serum creatinine level, patients with coagulopathies and pregnancy. The perioperative and postoperative outcomes were evaluated and postoperative pelvi-abdominal CT with intravenous contrast was done to detect if a subcapsular hematoma has been developed. Results There is no significant association between the formation of subcapsular renal hematoma and degree of hydronephrosis, chronic kidney disease (CKD), body mass index (BMI), diabetes mellitus (DM), hypertension (HTN) and preoperative bacteriuria as risk factors. Conclusions Subcapsular hematoma post-ureteroscopic lithotripsy (URSL) for impacted upper ureteric stones is a rare but potentially serious complication. A high index of suspicion is needed when patients present with significant loin pain and fever after ureteroscopic lithotripsy for obstructing proximal ureteral stones with thin renal cortices. Stone size, degree of hydronephrosis, operation duration, and perfusion pressure of hydraulic irrigation were associated with an increased risk of SRH formation. Management of post-URSL subcapsular hematomas needs to be customized for each patient.



BioMedica ◽  
2020 ◽  
Vol 36 (4) ◽  
pp. 367-373
Author(s):  
Zi-Ang Li ◽  
Prof. Jun Lin

Background and Objective: Coronavirus disease 2019 (COVID-19) has caused a large number of casualties and economic losses. Reducing the number of hospitalization days can alleviate pressure on health services and save more people. This study was carried out to find associated factors with the duration of hospitalization at a COVID-19 dedicated hospital in China. Methods: In this retrospective study, the demographic details and laboratory tests of 556 patients were collected. These patients were cured and discharged. The data was analyzed using statistical package for the social sciences (SPSS), version 22. The Shapiro-Wilk method was used for the normality of data with P ≤ 0.05 as significant to relate factors with the duration of hospitalization. Results: A total of 556 patients with COVID-19 were included in the study; 249 were males and 307 were females. Among the factors associated with longer hospital stay, lower K+ and Na+ levels were noticed in 102 (18.5%) and 61 (11%) respectively. Among liver functions tests, elevated AST levels was found in 109 (19.6%) patients, decreased serum albumin seen in 301 (54.1%) patients, elevated serum ALT levels in 80 (14.3%) patients with longer hospital stay (P = 0.032). Increased procalcitonin (PCT) was noticed in 66 (11.8%) patients. An elevated level of plasma D-dimer was seen in 125(22.4%) and increased NT-pro BNP levels in 64 (11.5%) patients. Increased cTnT levels were found in 76(13.6%) patients and elevated creatinine levels in 114 (21%) patients.(P = 0.015). Conclusion: Patients with elevated serum total cholesterol had significantly lesser hospital stay than the normal group. Patients with temperature ≥ 38°C and elevated blood glucose had longer hospitalization stay. We recommend that health care providers should not ignore multiple organ support when treating patients.



2021 ◽  
Vol 8 (6) ◽  
pp. 1102
Author(s):  
Cuddapah Gaurav Venkat ◽  
Vallivedu Chennakesavulu Pujitha ◽  
Kanchan S. Channawar ◽  
Vadde Vasavi

Pulmonary hypertension (PH) is most commonly related either to a cardiac or a pulmonary cause. But less commonly various hematological, hepatic, genetic causes are also associated. Infantile PH due to vitamin deficiencies is very rare though few cases with thiamine deficiencies causing PH have been reported lately. Lack of awareness and late recognition of thiamine deficiency may result in high mortality. A high index of suspicion is required for early diagnosis and management to decrease the severity and morbidity and thereby preventing long term implications on neurological development. Here, we described three cases of infants admitted to Kamineni academy of medical sciences diagnosed with PH who responded dramatically to thiamine supplements. The lack of rapid diagnostic capacity and the severe outcome of thiamine deficiency justify the use of a therapeutic thiamine challenge in cases with high clinical suspicion. Increased awareness about thiamine deficiency and low threshold for thiamine use should guide clinicians in their practice.



2021 ◽  
Vol 13 (03) ◽  
pp. 123-131
Author(s):  
Sohail Qazi ◽  
David Graham ◽  
Steven Regal ◽  
Peter Tang ◽  
Jon E. Hammarstedt

AbstractThe distal radioulnar joint (DRUJ) allows supination and pronation of the distal forearm and wrist, an integral motion in everyday human activity. DRUJ injury and chronic instability can be a significant source of morbidity in patients’ lives. Although often linked with distal radius fractures, DRUJ injury may occur in a variety of other upper extremity injuries, as well as an isolated pathology. Diagnosis of this injury requires the clinician to have a high index of suspicion and low threshold for clinical testing and further imaging of the DRUJ. The purpose of this article is to provide a review on DRUJ anatomy and biomechanics, to discuss common diagnostic and treatment modalities, and to identify common injuries associated with DRUJ instability.



2021 ◽  
Vol 16 (2) ◽  
pp. 287-294
Author(s):  
Ahmad Khaldun Ismail ◽  

Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecific clinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-organ dysfunction with superimposed pneumonia, requiring mechanical ventilation. However, persistent cytopenia despite blood transfusion without evidence of haemorrhage raised the suspicion of HLH. Further blood investigations revealed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia. Bone marrow aspiration showed haemophagocytosis. Patient fulfilled the diagnostic criteria for HLH by HLH-2004 trial. Her HScore is 281, with the probability of having HLH is 99.9%. Patient’s condition improved after administration of intravenous immunoglobulin (IVIG) and intravenous dexamethasone in tapering doses. Early specific treatment of HLH with IVIG and/or corticosteroid is important but diagnosis is usually delayed due to nonspecific clinical findings and laboratory results. High index of suspicion with the aid of diagnostic criteria by HLH-2004 trial and HScore is helpful to recognise this syndrome.



2021 ◽  
Vol 9 ◽  
Author(s):  
Pai-Jui Yeh ◽  
Shiu-Feng Huang ◽  
Ming-Chou Chiang ◽  
Chao-Jan Wang ◽  
Ming-Wei Lai

Background: Gestational alloimmune liver disease (GALD) is a rare but critical cause of neonatal liver failure. After discovering the maternal–fetal alloimmune mechanism, intravenous immunoglobulin (IVIG) with or without exchange transfusion (ET) has gradually replaced antioxidant cocktails as the first-line therapy. Whether such therapy changes the outcome of neonates with GALD is yet to be defined.Method: We reported a pair of twins with discordant presentations, mild and self-limited in the older, whereas liver failure in the younger, who was successfully rescued by ET and IVIG. To investigate the outcome after therapeutic alteration, 39 cases between 2005 and 2020 from literature research were collected.Results: Half of the collected cases (47.1%) were preterm. Common presentations were ascites, jaundice, respiratory distress, hepatomegaly, and edema. Leading laboratory abnormalities were coagulopathy, hypoalbuminemia, and elevated serum ferritin. Salivary gland biopsy and magnetic resonance imaging detected extrahepatic siderosis in 70% (14/20) and 56% (14/25), respectively. IVIG, ET, and liver transplantation were performed in 19 (48.7%), 15 (38.5%), and 8 (20.5%) patients, respectively. The overall survival (OS) rate and native liver survival (NLS) rate were 64.1% (25/39) and 43.6% (17/39), respectively. Although the compiled results did not support a significant benefit, the OS and NLS were higher in the IVIG with/without ET group compared with those treated with conventional therapy [OS (70 vs. 57.9%) and NLS (55 vs. 31.6%), respectively].Conclusion: A high index of suspicion for GALD is crucial when facing a neonate with liver failure. Despite no significant influence on the outcome over conventional therapy in such a rare and detrimental disease, IVIG with or without ET can be worth trying before resorting to liver transplantation, which is resource-demanding and technique-challenging in small infants.



2019 ◽  
Vol 30 (1) ◽  
pp. 89-93 ◽  
Author(s):  
Graziela Zibetti Dal Molin ◽  
Shannon Neville Westin ◽  
Pavlos Msaouel ◽  
Larissa M Gomes ◽  
Andrea Dickens ◽  
...  

ObjectiveTo describe discrepancies in calculated and measured glomerular filtration rate in patients using PARP (poly ADP ribose polymerase) inhibitors who had an elevation in serum creatinine levels.MethodsRetrospective cohort, single center study. Patients included were those with ovarian or endometrial cancer taking olaparib, rucaparib or niraparib, and in in whom an increased serum creatinine was identified. The study cohort included those who also underwent technetium-99m radioisotope renography (glomerular filtration rate (GFR) scan). The main objective is to describe the discrepancies in calculated glomerular filtration rate using the Cockcroft-Gault method and measured glomerular filtration rate using a GFR scan.Results211 patients were included in the study; 64 (30%) had on-treatment elevated serum creatinine, and 23 (36%) underwent a GFR scan. 32 GFR scans were performed (six patients had more than one scan). Using a clinical cut-off ≥50 mL/min as normal renal function, both calculated and estimated glomerular filtration rates were below normal in 6 of 32 GFR scans. In those patients undergoing a GFR scan, serum creatinine had risen a median 49% (IQR 20–66%, range 0–144%) above baseline. Discordance between a calculated low glomerular filtration rate and an estimated normal glomerular filtration rate occurred in 63% (range of glomerular filtration rate discrepancy: −46% to +237%). Despite increases in serum creatinine on therapy and a subsequent significant decline in the per patient calculated creatinine clearance (mean 65.6 mL/min vs 43.4 mL/min; p<0.0001), the estimated glomerular filtration rate from the renal scan was nearly identical to the patient’s baseline (65.6 mL/min vs 66.1 mL/min; p=0.89).ConclusionsSerum creatinine elevation in patients taking PARP inhibitors may not be associated with a true decrease in glomerular filtration rate. A high index of suspicion should be maintained for alternative causes of elevated serum creatinine in patients treated with PARP inhibitors who lack other sources of renal injury.



Author(s):  
A. G. Peppelenbosch ◽  
Martijn Poeze

Intestinal ischaemia is caused by occlusion of the visceral arteries, thrombosis of the mesenteric veins, or by (low-flow) non-occlusive mesenteric ischaemia (NOMI). Each condition has a specific diagnostic and therapeutic work-up and prognostic significance. The incidence of acute mesenteric infarction is as low as 0.63 cases/100,000 person years, but overall mortality rates remains high at 74%. In general, a high index of suspicion is necessary and should be followed by administering therapeutic low molecular weight heparin or systemic heparin infusion. In these patients resuscitation and organ support are essential, but should not delay diagnostic work-up, including CT-angiography. With arterial occlusion, revascularization should be performed if indicated, preferentially using endovascular techniques prior to laparotomy. For venous occlusion, thrombolytic therapy directly into the superior mesenteric artery or venous thrombectomy can be performed, followed by laparotomy. The treatment of NOMI is to treat the underlying cause.



The author adverts to the deficiency of information which has hitherto existed as to the temperature and chemical composition of the springs and rivers both of India and of Egypt; and also as to their geographical and geological relations. He gives, in the present paper, the details of a great number of observations which he has made on these subjects, and which he thinks may prove a useful contribution to Indian hydrography, as well as afford more exact data for philosophical inquiry. The observations extend, at irregular intervals, from Alexandria to Malacca, or from 31º13' of northern latitude to within 2º14' of the Equator, and between the meridians of 27º and 103º of east longitude. In the columns of the register, the date of the observation, the latitude, longitude, approximate height above the sea, nature of the surrounding geological formation, depth to the surface of the water, depth of the water itself, temperature of the air, and approximate annual mean of the climate in which the wells, &c. occur, are, as far as practicable, specified. A column of remarks is added, containing observations on the chemical nature of the water, and on the size of the wells and springs, and the result obtained by other observers. It was found, in general, that in low latitudes the temperature of the deepest wells and springs is a little higher than the mean temperature of the air; although there occur a few exceptions, especially in the neighbourhood of a high range of hills, whence there probably arise cold springs, having their source at an elevation considerably above that of the plain where the water makes its appearance. Springs which are strongly saline and sulphureous, have, on the average, a higher temperature than those of pure water. Both saline and cold springs are found occurring within a few feet from thermal and freshwater springs: a fact which the author is disposed to ascribe to their rising through different seams of the subjacent strata, often much inclined; and to the different depths and heights, above and below the crust of the earth, from which the supply of water is derived. Wells, and particularly those having a small surface, which are much used for purposes of irrigation, thereby acquire an artificial increase of temperature. The temperature of shallow exposed wells, springs and rivers, especially those which have sandy beds, is subject to diurnal fluctuation from the more powerful influence of the atmosphere: and the surface water of deep wells partakes of these vicissitudes to a depth varying according to the transparency of the water, the extent of surface, degree of exposure and clearness of the sky. In muddy water, the surface is heated to a greater extent; but at the depth of a foot or two, it is less affected by the heat of the solar rays than clear water.



2020 ◽  
Vol 21 (3) ◽  
pp. 256-268 ◽  
Author(s):  
Kris Bauchmuller ◽  
Jessica J Manson ◽  
Rachel Tattersall ◽  
Michael Brown ◽  
Christopher McNamara ◽  
...  

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation, characterised by extreme inflammation, fever, cytopaenias and organ dysfunction. HLH can be triggered by conditions such as infection, autoimmune disease and malignancy, among others. Both a familial and a secondary form have been described, the latter being increasingly recognised in adult patients with critical illness. HLH is difficult to diagnose, often under-recognised and carries a high mortality. Patients can present in a very similar fashion to sepsis and the two syndromes can co-exist and overlap, yet HLH requires specific immunosuppressive therapy. HLH should be actively excluded in patients with presumed sepsis who either lack a clear focus of infection or who are not responding to energetic infection management. Elevated serum ferritin is a key biomarker that may indicate the need for further investigations for HLH and can guide treatment. Early diagnosis and a multidisciplinary approach to HLH management may save lives.



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