Macrophage Activation Syndrome:A case series;From Sepsis to Malignancy: Early diagnosis and treatment
NTRODUCTION: Haemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome(MAS) is a syndrome of fulminant cytokine storm leading to multiorgan dysfunction and high mortality rate. HLH may be Familial or Primary(fHLH) and Secondary (sHLH).FHLH is due to mutation in gene coding for perforin or NK cell of CD8 lymphocytes. sHLH may be associated with hematological malignancies, autoimmune disorders like SLE,Still’s diseae, kawasaki disease, infections and sepsis of various etiology starting from bacteria ,viral protozoal ,fungal and zoonotic infection. Main presenting features are fever, hepatospleenomegly, cytopenia,high ferritin level, high serum triglyceride and haemophagocytosis in bone marrow spleen or lymphnode. MATERIAL AND METHOD: In this series we describe Five cases ,(two cases of SLE,one B-Cell lymphoma, one case of scrub typhus,one case of kleibsiella pneumoniae presenting as sepsis ) with their clinical, laboratory investigations, management and outcome with special correlation of ferritin level and HS score with the prognosis. All patients managed with I.V.Methyl prednisolone 30 mg/kg/day for three consecutive days and out come is assessed. CONCLUSION: MAS in SLE and sepsis is a life threatening unrecognised condition, early diagnosis and treatment can increase the survival rate by many folds . a drop of ferritin level by 15% after 48 hrs of treatment is a surrogate marker of good prognosis. From H scoring cut off value 169 corresponds to sensitivity of 93% and specificity of 86%.
Marfan’s syndrome (MFS) is an autosomal dominant disorder that affects the body’s connective tissue, characterized by changes in three major connective tissue systems: the musculoskeletal, ocular, and cardiovascular systems. Individuals with MFS can develop severe cardiovascular complications., and the life expectancy of patients with MFS is markedly reduced as the effects of the disease on the aorta often lead to progressive dilatation, with subsequent dissection and rupture. Obstructive sleep apnea (OSA) is highly prevalent in patients with cardiovascular diseases. There is a high prevalence of OSA in patients with MFS due to craniofacial dysmorphism and increased upper airway collapsibility. A high prevalence of OSA has been reported in patients with MFS. To date, however, there are only a few case reports regarding the treatment of OSA in MFS patients. We present a treatment in an adult MFS patient with OSA using an oral appliance (OA). The patient was a 48-year-old Japanese woman of MFS, diagnosed with moderate OSA (apnea hypopnea index [AHI] = 27.0; lowest oxygen saturation [SaO2] = 78%; maximum apnea time = 114 s]). An OA was selected as the treatment option, and post-treatment sleep study indicated a reduction in AHI to 8.8/h with an improvement in other factors (lowest SaO2 = 84%; maximum apnea time = 66 s). Additionally, the treatment resulted in a reduction in BNP levels (81.3 from 110.2 pg/ml), and the patient’s cardiac function was stable over the period in which the patient received OA treatment. This study provides a basis for the future appropriate understanding and management of OSA of MFS patients.
Early effectivity of teduglutide for the treatment of short bowel sydrome – our first clinical experience
Background Short bowel syndrome is an uncommon malabsorptive condition most frequently caused by massive surgical resection of the small intestine. Standard of care is home parenteral nutrition. Teduglutide, glucagon like peptide 2 analog, was approved to treat patients with short bowel syndrome, who are stable following a period of post-surgery intestinal adaptation. Case presentation We report two cases of patients with short bowel syndrome on home parenteral nutrition, treated with teduglutide, novel advanced therapy. We monitored the effectiveness and tolerance of the new treatment. Conclusion Teduglutide increases intestinal absorption, causes cryptal hyperplasia, villous hypertrophy, angiogenesis, and allows weaning from parenteral nutrition. We confirmed the early onset of treatment effectiveness in the 4th month. Early effectiveness of treatment allowed a reduction in the volume of home parenteral nutrition with an improvement in quality of life.
BACKGROUND AND AIM: Adult, non-infective, haemolytic-uremic syndrome (HUS) although a rare disease in itself, has a high likelihood of occurrence in pregnancy and immediate post partum period. It is an important differential diagnosis in the evaluation of thrombotic microangiopathies. Patients with post-partum HUS display a classical triad of microangiopathic haemolytic anaemia, acute nephropathy and thrombocytopenia. I hereby present a case of post partum HUS treated with therapeutic plasma exchange (TPE) MATERIAL AND METHODS: A total of six sessions of TPE were performed daily, three sessions for consecutive days and remaining three sessions were performed on alternate days. All the procedures were carried out with Haemonetics MCS+ exchanging one plasma volume using fresh frozen plasma and saline as replacement fluid. Haemodialysis was started and four sessions were carried out on alternate days. RESULT: A 37 year old, 85 kg female, G2 P1, underwent emergency LSCS because of foetal distress at 38 weeks of pregnancy. Post surgery she developed decreasing urine output, anuria ensued. Emergency therapeutic plasma exchange was carried out within 24 hours of diagnosis. It could be found that with TPE, patient had improvement in renal function, decrease in LDH levels and increase in platelet count. Patient had sustained remission and discontinuation of haemodialysis. CONCLUSION: HUS is a disorder with high mortality and long term morbidity, if prompt treatment is not instituted. The decision to intervene with plasma exchange should be based upon the severity of thrombocytopenia, microangiopathic haemolytic anaemia and neurological abnormalities, even if the diagnosis and nomenclature is uncertain. Improved survival after this disorder has been attributed to aggressive treatment with plasma exchange therapy.
Background: Hemorrhagic cholecystitis is an infrequent complication of acute cholecystitis associated with high mortality rate. Recognition of this acute abdominal condition is often challenging. Authors present a case of acute intraluminal gallbladder bleeding with a consequential gallbladder wall perforation and hematoperitoneum requiring emergency surgery in a patient on anticoagulant therapy. Case Presentation: An 80-year old woman with atrial fibrillation on warfarin was presented to abdominal surgeon due to acute abdominal pain with hemodynamic instability and active intraluminal gallbladder bleeding on CT-scan. An emergency laparotomy with cholecystectomy was performed. Despite intensive treatment patient died on the third postoperative day. Conclusions: Hemorrhagic cholecystitis is a potentially life-threatening condition. Patients on anticoagulant therapy with clinical signs of acute cholecystitis are more prone to develop bleeding into the gallbladder. Contrast-enhanced CT of the abdomen is the diagnostic modality of choice and cholecystectomy definitive therapy in patients with hemorrhagic cholecystitis.
Multiple septic emboli and myocardial infarction due to vaso-invasive Rhizomucor pusillus infection in a hematologic patient
We present a case of a 63-year-old hematologic patient with pulmonary vaso-invasive zygomycosis with Rhizomucor pusillus after a second stem cell transplantation (SCT) for myelodysplastic syndrome, complicated by multi organ failure, myocardial ischemia and infarction. Zygomycosis is common in immunocompromised patients, especially after hematopoietic stem cell transplantation (HSCT). Mucor species have devastating vaso-invasive properties causing hematogenic dissemination. Antemortem diagnosis may be difficult due to negative cultures. Despite adequate treatment outcome tends to be poor. Cardiac zygomycosis is rare. In our patient, the clinical course and imaging results of the myocardial infarction are most consistent with coronary plaque rupture possibly provoked by severe vaso-invasive pulmonary infection and multi-organ failure.
PICC Insertion could be an optimal choice of central venous access in prone position mechanically ventilated COVID-19 ARDS patient
During the management of critically ill covid-19 patients obtaining an appropriate centrally inserted central catheter (CICC) can be a prime necessity. Traditionally, a CICC is inserted in a supine position. However, a CICC may not be possible in some COVID-19 patients with severe hypoxia or sudden clinical deterioration who need urgent intubation and immediate proning. Therefore, CICC in pronated COVID-19 ARDS patients is challenging. Recent studies limited to case reports have shown that peripherally inserted central catheters (PICC) are safer in pronated ARDS patients. PICC lines minimize mechanical complications and lower catheter-related bloodstream infections when compared to standard CICC. However, there is a scarcity of evidence showing the efficacy of PICC in pronated COVID-19 ARDS patients, possibly due to the complex precautionary safety measures, insertion techniques, and expertise team deficit. Herein, we present a 57-year-old male as a case of COVID-19 ARDS, mechanically ventilated in a prone position with existing subcutaneous emphysema. Our case illustrates PICC insertion challenges in the prone position, ultrasound guidance in PICC insertion to comprehend the vein’s diameter for accurate vein needling in proportion to the external catheter diameter, and intracavitary electrocardiographic (ECG) navigation method to confirm catheter tip location. So that chest X-ray and radiology risk of contamination is avoided. Long-term research urged to validate the efficacy of PICC in this group of patients.
Efficacy and safety of Bosutinib in Patient with Chronic myeloid leukemia who was intolerant to DASTANIB,NILOTUNIB
Treatment for Chronic myeloid leukemia has been revolutionized because of availability of different tyrosine kinase inhibitors. Each TKI come with its on toxicity profile as this needs to be taken in account before starting therapy with particular agent in a patient. Most of the adverse effects related to TKI are mild and can be managed by either symptomatic treatment or either by dose reduction. But some patients can become intolerant and to switch to other TKI remains the only option. Bosutinib is currently approved for treatment of chronic phase CML in patients who are either resistant or intolerant to previous TKI. We present a case of 59 year old male patient with CML who was intolerant to Dastanib and Nilotinib but showed excellent hematological and major molecular response to bosutinib
Cornual pregnancy is a fearful condition with dreadful complications. Due to rarity and late presentation, it poses diagnostic and therapeutic challenges. There is no consensus on treatment strategies till date and risk of rupture after repair in subsequent pregnancy always remains a major concern. We hereby discuss a case of 27 year female who presented with ruptured cornual ectopic pregnancy, underwent cornual wedge resection with repair and had subsequent pregnancy with a successful outcome.
Pulmonary embolism occurs when thrombi enter the pulmonary arterial circulation. Most pulmonary embolisms are the result of deep venous thrombosis of the pelvic limbs, chest or pelvis, and, less commonly, the jugular veins or inferior vena cava. Venous thromboembolism includes deep vein thrombosis and pulmonary embolism. It is the third most common cardiovascular disease, with a total annual incidence of 100-200 per 100 000 population. INTRODUCTION: Acute pulmonary embolism is the most serious clinical presentation of venous thromboembolism. Overall, pulmonary embolism is a major cause of mortality, morbidity and hospitalization. Mortality in pulmonary embolism depends on haemodynamic impairment, age and co morbidities. The prognosis of patients with pulmonary embolism depends on two factors : underlying disease state plus diagnosis, and appropriate treatment. Approximately 10% of patients who develop pulmonary embolism die within the first hour, and 30% subsequently die of recurrent embolism. CASE PRESENTATION: In this presentation we present the case of a 49-year-old male patient without co morbidities, presented repeatedly to the Emergency Room for symptoms suggestive of pulmonary thromboembolism, who benefited from life-saving therapies due to timely diagnosis and appropriate treatment, with subsequent favorable outcome. CONCLUSIONS: The particularity of the case is that, on the one hand, the thromboembolic event recurred in a short time, on the other hand, the evolution was favorable in both cases, with complete recovery of right ventricular function and disappearance of pulmonary hypertension, despite the fact that the patient was non-compliant with initial anticoagulation therapy. This was due to both early diagnosis and timely administration of appropriate treatment.