scholarly journals A severe case of thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly syndrome with myocardial and skeletal muscle calcification despite hypocalcemia: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Shogo Minomo ◽  
Yu Fujiwara ◽  
Shota Sakashita ◽  
Akito Takamura ◽  
Kaoru Nagata

Abstract Background TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndrome is a recently recognized disease with a variety of presentations of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported. Case presentation A 24-year-old healthy young Asian man was admitted with intermittent epigastric pain and fever for 2 weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction. However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal muscle calcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization, with residual calcific lesions. Conclusion This is the first report of a patient with TAFRO syndrome and the complication of organ calcification. The etiology of calcification in this case is not clear. Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification. It is important to carefully handle the general management of TAFRO syndrome because of the possibility of various complications.

2020 ◽  
Author(s):  
Shogo Minomo ◽  
Yu Fujiwara ◽  
Shota Sakashita ◽  
Akito Takamura ◽  
Kaoru Nagata

Abstract Background TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndromeis a recently recognized disease with a variety of presentation of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported.Case presentation A 24-year-old healthy young man was admitted with intermittent epigastric pain and fever for two weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction.However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal musclecalcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization with residual lesions of calcification.Conclusion This is the first TAFRO syndromereport with the complication of organ calcification. The etiology of calcification in this case is not clear.Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification.It is important to handle general management of TAFRO syndrome carefully because of various complications.


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Qianyun Zhou ◽  
Yuanyuan Zhang ◽  
Guangping Zhou ◽  
Jihong Zhu

Abstract Background TAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis (or renal dysfunction), and organomegaly. TAFRO syndrome has only recently been described, and many clinicians are unaware of this disease, leading to delays in diagnosis and treatment. We present two patients with TAFRO syndrome in whom renal biopsies were performed. Case presentation Both patients had subacute onset and exhibited renal insufficiency, edema, anemia, thrombocytopenia, polyserositis and lymphadenopathy over the disease course. However, there were many differences in their clinical manifestations. Case 1 was a 30-year-old woman admitted due to intermittent vaginal bleeding for 3 weeks. Laboratory tests on admission showed severe renal insufficiency (creatinine: 624 μmol/L), severe anemia (Hb: 41 g/L), and moderate thrombocytopenia (61 × 109/L). Case 2 was a 42-year-old man. Acute epigastric pain was his initial complaint, and computed tomography (CT) revealed retroperitoneal exudation around the pancreas. He was diagnosed with acute pancreatitis, and after treatment with a proton pump inhibitor (PPI) and somatostatin, his abdominal pain still recurred. During treatment, renal failure gradually increased, with oliguria, fever, anemia, thrombocytopenia, edema and massive ascites. Lymph node histologies were consistent with the hyaline-vascular (HV) type and mixed type, respectively, and renal histopathologies were consistent with thrombotic microangiopathy (TMA)-like renal lesions and membranoproliferative glomerulonephritis (MPGN), respectively. Their general conditions improved after glucocorticoid therapy, but their renal functions did not recover completely. On the basis of glucocorticoids, second-line treatments with tocilizumab and rituximab, respectively, were applied. Conclusions The diagnosis of TAFRO syndrome is based mainly on clinical manifestations and lymph node biopsies. A reliable early diagnosis and appropriate rapid treatment are essential to improve patient outcomes. Clinicians should deepen their understanding of this disease and similar conditions. Once the disease is suspected, lymph node biopsies should be performed as soon as possible. In addition, renal biopsies should be actively performed in patients with renal involvement.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Mohammad Z. Darabseh ◽  
Thomas M. Maden-Wilkinson ◽  
George Welbourne ◽  
Rob C. I. Wüst ◽  
Nessar Ahmed ◽  
...  

AbstractCigarette smoking has a negative effect on respiratory and skeletal muscle function and is a risk factor for various chronic diseases. To assess the effects of 14 days of smoking cessation on respiratory and skeletal muscle function, markers of inflammation and oxidative stress in humans. Spirometry, skeletal muscle function, circulating carboxyhaemoglobin levels, advanced glycation end products (AGEs), markers of oxidative stress and serum cytokines were measured in 38 non-smokers, and in 48 cigarette smokers at baseline and after 14 days of smoking cessation. Peak expiratory flow (p = 0.004) and forced expiratory volume in 1 s/forced vital capacity (p = 0.037) were lower in smokers compared to non-smokers but did not change significantly after smoking cessation. Smoking cessation increased skeletal muscle fatigue resistance (p < 0.001). Haemoglobin content, haematocrit, carboxyhaemoglobin, total AGEs, malondialdehyde, TNF-α, IL-2, IL-4, IL-6 and IL-10 (p < 0.05) levels were higher, and total antioxidant status (TAS), IL-12p70 and eosinophil numbers were lower (p < 0.05) in smokers. IL-4, IL-6, IL-10 and IL-12p70 had returned towards levels seen in non-smokers after 14 days smoking cessation (p < 0.05), and IL-2 and TNF-α showed a similar pattern but had not yet fully returned to levels seen in non-smokers. Haemoglobin, haematocrit, eosinophil count, AGEs, MDA and TAS did not significantly change with smoking cessation. Two weeks of smoking cessation was accompanied with an improved muscle fatigue resistance and a reduction in low-grade systemic inflammation in smokers.


2021 ◽  
Vol 14 (1) ◽  
pp. e237842
Author(s):  
Arun Ahluwalia ◽  
Matthew George Roy Allaway ◽  
Serena Giga ◽  
Richard James Curran

A 79-year-old woman presented with postprandial epigastric pain. She had normal vital signs, inflammatory markers and liver function tests. Ultrasound and CT of the abdomen demonstrated features consistent with acute cholecystitis. Her medical comorbidities and extensive abdominal surgical history prompted the decision to treat non-operatively. Despite optimal medical management, worsening abdominal pain and uptrending inflammatory markers developed. She underwent an emergency laparoscopy which revealed a necrotic gallbladder secondary to an anticlockwise complete gallbladder torsion; a rare condition associated with significant morbidity and mortality if managed non-operatively. Laparoscopic cholecystectomy was achieved without complication and the patient had an uneventful recovery. Preoperative diagnosis of torsion of the gallbladder is difficult. However, there are certain patient demographics and imaging characteristics that can help surgeons differentiate it from acute cholecystitis; a condition which can be safely managed non-operatively in selected patients. The differentiating features are elaborated on in this case report.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Andrew Leber ◽  
Raquel Hontecillas ◽  
Nuria Tubau-Juni ◽  
Victoria Zoccoli-Rodriguez ◽  
Bret Goodpaster ◽  
...  

2020 ◽  
Vol 318 (2) ◽  
pp. R296-R310 ◽  
Author(s):  
Hélène N. Daou

Cancer cachexia is a complicated disorder of extreme, progressive skeletal muscle wasting. It is directed by metabolic alterations and systemic inflammation dysregulation. Numerous studies have demonstrated that increased systemic inflammation promotes this type of cachexia and have suggested that cytokines are implicated in the skeletal muscle loss. Exercise is firmly established as an anti-inflammatory therapy that can attenuate or even reverse the process of muscle wasting in cancer cachexia. The interleukin IL-6 is generally considered to be a key player in the development of the microenvironment of malignancy; it promotes tumor growth and metastasis by acting as a bridge between chronic inflammation and cancerous tissue and it also induces skeletal muscle atrophy and protein breakdown. Paradoxically, a beneficial role for IL-6 has also been identified recently, and that is its status as a “founding member” of the myokine class of proteins. Skeletal muscle is an important source of circulating IL-6 in people who participate in exercise training. IL-6 acts as an anti-inflammatory myokine by inhibiting TNFα and improving glucose uptake through the stimulation of AMPK signaling. This review discusses the action of IL-6 in skeletal muscle tissue dysfunction and the role of IL-6 as an “exercise factor” that modulates the immune system. This review also sheds light on the main considerations related to the treatment of muscle wasting in cancer cachexia.


2013 ◽  
Vol 2 (1) ◽  
pp. 57 ◽  
Author(s):  
Bryce Weber ◽  
Gerald Todd ◽  
Ronald B. Moore

A 26-year-old male body builder diagnosed with renal artery stenosis and middleaortic syndrome underwent an autotransplantation with bench reconstructionand end-to-end anastomosis using the hypogastric artery. Shortly afterthe procedure, the patient developed rhabdomyolysis and renal insufficiency,possibly related to his increased muscle mass, potentially greater susceptibilityto hypertrophic skeletal muscle cells or his unique vascular condition. Wereview the risk factors, diagnosis, management and outcome of a case ofrhabdomyolysis in a male patient who underwent autotransplantation for renalvascular hypertension.


2019 ◽  
Vol 12 (3) ◽  
pp. e226761 ◽  
Author(s):  
Rajarshi Bhadra ◽  
Fareeha Ahmed Khan ◽  
Mona Soliman ◽  
Meyappan Somasundaram ◽  
Daniel V Iltchev ◽  
...  

Injudicious use of over-the-counter calcium supplements has resulted in increased incidences of hypercalcaemia and related complications. We present a case of acute pancreatitis in a chronic hypocalcaemic patient of DiGeorge’s syndrome. The patient came into the ED with sepsis syndrome, right upper quadrant and epigastric pain and no obvious source of infection. Lab results and imaging were indicative of acute pancreatitis. There was severe renal dysfunction. The patient needed haemodialysis and had a prolonged stay in intensive care. The medical history was negative for biliary duct pathology or alcohol use. The patient had vomiting and diarrhoea in the nursing home for about a week, but she continued to receive her regular medications that included the calcium supplements and thiazide diuretics. It is likely that a complex interplay between calcium supplementation, dehydration and thiazide diuretics resulted in the development of acute pancreatitis and severe renal dysfunction in a chronic hypocalcaemic patient.


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