Antineutrophil cytoplasmic antibody-associated vasculitis with systemic sclerosis: a fatal case report.

Introduction and importance: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical manifestations and prognosis of vasculitis in systemic sclerosis depend on organ involvement. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers get along with purpuric vasculitis in some cases reports. Reports had found that survival in those with pulmonary-renal syndrome is poor. However, high-dose corticosteroids and cyclophosphamide increase the survival percent in those patients. Case presentation: An 81-year-old female was admitted for newly diagnosed acute renal failure and highly elevated C-reactive protein levels. She was diagnosed with systemic sclerosis 8 years previously, with a 3-year history of interstitial lung disease, and a 2-year history of pulmonary hypertension. Treatment included home oxygen on demand, prednisone 5 mg/day, and azathioprine 75 mg daily. On physical examination, she had sclerodactyly, both extremities ulcers, severe livedo reticularis, and hyperpigmented papules on her hand and feet. Laboratory findings included a markedly positive MPO (p-ANCA), and anti-Scl-70. She was treated with pulse methylprednisolone without any improvement. After a day, she developed anuria and became comatose. Then, she developed cardiac arrest, leading to death. Clinical discussion: The presence of ANCA in systemic sclerosis patients ranges from 2.5 to 9% of systemic sclerosis patients. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers. The treatment with high-dose corticosteroids and cyclophosphamide is benefit. Survival in those with pulmonary-renal syndrome is poor. Conclusion: The presence of ANCA-associated vasculitis is rarely reported with scleroderma. It occurs most commonly in women with limited or Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) variants of scleroderma, as well as those with overlap features. Severe manifestations including pulmonary-renal syndrome and death may occur.

Fatal rhabdomyolysis caused by COVID-19 infection: a case report.

COVID-19 is a systemic viral disease complicated with medical conditions. Severe rhabdomyolysis during the COVID-19 course is not until now well known. Case presentation: The authors presented a 48-year-old female with fatal rhabdomyolysis caused by COVID-19 infection. She was referred to us with cough, generalized myalgia and arthralgia, and fever during the last week. Laboratory results showed an elevated erythrocyte sedimentation rate, elevated C-reactive protein level, and elevated creatine kinase. The nasopharyngeal swab confirmed the diagnosis of coronavirus 2 RNA infection. She was managed initially in the COVID-19 isolation department. Three days later, she was transferred to the intensive care unit and mechanically ventilated. Laboratory results were consistent with rhabdomyolysis. She died because of cardiac arrest due to continuous hemodynamic deterioration. Clinical discussion: Rhabdomyolysis is a serious condition that can be fatal or cause disability. Rhabdomyolysis cases have been reported in COVID-19 patients. Conclusion: Rhabdomyolysis cases have been reported in COV19 patients. Further studies are needed to understand the mechanism and to optimize the treatment.

Hyperplastic polyps after surgical management of Hirschsprung's disease in children treated with argon plasma coagulation: Case report.

Introduction and importance: The argon plasma coagulation is a technique used for noncontact thermal coagulation of tissue. Hyperplastic polyps are the most common non-neoplastic polyps in the colon. Case presentation: We presented a 3-year-old girl with a chief complaint of bloody stool and rectal tenesmus that began 5 days ago. She was previously being treated because of a history of chronic constipation. She underwent a one stage surgery for Hirschsprung's disease, after a full-thickness rectal wall biopsy was obtained and it consistent with Hirschsprung's disease. Three months after the surgery, the girl presented to our clinic with rectal blood loss and rectal tenesmus. Endoscopic examination of her colon demonstrated sessile and pedunculated polyps. The pedunculated polyps were removed endoscopically. Biopsies were taken from the sessile polyps. Histopathologic examination of the polypectomy specimen and the biopsies of the sessile polyps showed hyperplastic polyps. The endoscopy was repeated for the purpose of argon plasma coagulation ablation of the sessile polyps. Argon plasma coagulation caused an adequate ablation and the entire polyps were burned. Clinical discussion: Argon plasma coagulation is an effective and safe technique in the endoscopic management of gastrointestinal conditions. Conclusion: Further clinical trials including a comparison of argon plasma coagulation to other treatment modalities, as well as long-term follow-up after argon plasma coagulation treatment are required.

Congenital lobar emphysema case report: A frequently misdiagnosed disease.

Introduction and importance: Congenital Lobar Emphysema is a rare cystic lesion of the lung which may be misdiagnosed and managed as pneumonia or pneumothorax. Case presentation: We presented a case of a congenital cystic lung malformation, the case demonstrated a 29-day-old boy who presented with respiratory distress. He was initially diagnosed as pneumonia and was given unnecessary antibiotic treatment in another hospital without improvement. Subsequently, the diagnosis of congenital lobar emphysema of the left upper lobe was made on the basis of the clinical and radiologic features. The condition wasn't detected before birth. He underwent a successful left thoracotomy with left upper lobe lobectomy. Clinical discussion: Congenital Lobar Emphysema poses a challenge in diagnosis. It may mimic other causes of respiratory distress. Conclusion: Congenital Lobar Emphysema requires a high index of clinical and radiological suspicion to make an early diagnosis so that timely treatment will be offered.

Possible autosomal recessive inheritance in a neonate with Nager syndrome: Case report.

INTRODUCTION AND IMPORTANCE: Nager syndrome is a rare inherited disorder characterized by craniofacial malformations occurring in association with abnormalities of the thumb and radial parts of the forearm. CASE PRESENTATION: We presented a 18-day-old boy with Nager syndrome. The diagnosis based on his clinical presentation. He was born to non-consanguineous healthy parents. He had three deceased siblings who had similar clinical features. This family gave further evidence for autosomal recessive inheritance. Nager syndrome can be detected using prenatal screening ultrasound. CLINICAL DISCUSSION: The etiology of Nager Syndrome is poorly described. Most cases arise spontaneously, although autosomal recessive and autosomal dominant modes of inheritance have been reported. Nager syndrome is suspected to have an autosomal recessive inheritance pattern, when unaffected parents have more than one affected child. CONCLUSION: Treatment required the coordinated efforts of a team of specialists. Many manifestations of the disease can be improved by surgery and other supportive treatments.

Kartagener syndrome: A case report.

Kartagener syndrome is a rare, autosomal recessive genetic disorder that causes defects in the action of ciliary movement, comprises of triad situs inversus, chronic sinusitis, and bronchiectasis. We present the case of a 3-year-old boy with repeated respiratory infections and pneumonic infections presenting with acute respiratory failure. He was diagnosed with Kartagener syndrome based on his clinical presentation and imaging features. The current diagnosis was consistent with severe acute bronchitis. He was managed initially with conventional medical therapy, but he didn't respond and was transferred immediately to the pediatric intensive care unit where noninvasive ventilation was administered. He had shown significant predictors of early noninvasive ventilation failure and was mechanically ventilated, after which, he was disconnected from the ventilator and discharged without complications. In patients presenting with recurrent upper and lower respiratory tract infections, Kartagener syndrome should always be kept in mind. The correct diagnosis of this disorder in early life is very important to prevent complications and improve patients' quality of life.