Clinical features of acute fibrinous and organizing pneumonia: An early histologic pattern of various acute inflammatory lung diseases.

BACKGROUND: Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic pattern of acute lung involvement with intra-alveolar fibrin deposition. However, the clinical significance of the pathological findings of AFOP remains unclear. This study aimed to explore the clinical significance of AFOP through a comprehensive clinical examination. METHODS: The medical records of patients with lung diseases accompanied by the pathological finding of intra-alveolar organization between January 2010 and December 2019 were retrospectively reviewed. The clinical and radiological findings were compared between the groups with and without the histologic pattern of AFOP. RESULTS: We identified 34 patients with AFOP (AFOP group) and 143 without AFOP (non-AFOP group). The underlying diseases of the AFOP group were as follows: 19 patients had cryptogenic organizing pneumonia (OP), 5 had connective tissue diseases, 3 had radiation pneumonitis, 3 had chronic eosinophilic pneumonia, 2 had myelodysplastic syndromes, and 2 had drug-induced pneumonia. Fever was more common, the time from symptom onset to biopsy was shorter, and the serum C-reactive protein level was higher in the AFOP group than in the non-AFOP group. On high-resolution computed tomography, 85% of patients had OP pattern, and halo sign was more common in the AFOP group. Corticosteroids were effective in 94% of the patients in the AFOP group; however, recurrences were more frequent, and a higher corticosteroid dose was needed during recurrence. CONCLUSIONS: AFOP might be an early phase of a histologic pattern associated with known etiologies. In addition, it could be a marker indicating intense inflammatory diseases with a tendency of recurrence.

Nontuberculous mycobacterial lung disease accompanied by organizing pneumonia.

We herein report our experience with patients who had nontuberculous mycobacterial lung disease (NTM disease) accompanied by organizing pneumonia (OP). Out of 98 NTM disease patients who had undergone a biopsy or surgical resection, 11 patients had OP that was revealed histologically. After excluding six patients who had OP-related diseases (idiopathic interstitial pneumonia, rheumatoid arthritis, etc.), the remaining five patients were studied. Two of them (a 73-year-old man and a 66-year-old woman) showed common clinical feature: acute-onset symptoms of cough and fever, infiltrating shadows and dramatic improvement following treatment with a corticosteroid and anti-mycobacterial therapy. Our cases demonstrate that NTM disease is sometimes accompanied by OP histologically, and some such cases show common clinical features.

Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report.

INTRODUCTION: Refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation is rarely reported and has a poor prognosis in general (a median survival of 1.6 months). Moreover, the optimum treatment for this condition is still undecided. This is the first report on the successful use of vincristine, adriamycin and dexamethasone chemotherapy for refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis without cardiac decompensation. CASE PRESENTATION: We report the case of a 68-year old Japanese male with systemic immunoglobulin light chain amyloidosis presenting with bilateral pleural effusion (more severe on the right side) in the absence of cardiac decompensation that was refractory to diuretic therapy. The patient was admitted for fatigue, exertional dyspnea, and bilateral lower extremity edema. He had been receiving intermittent melphalan and prednisone chemotherapy for seven years. One month before admission, his dyspnea had got worse, and his chest radiograph showed bilateral pleural effusion; the pleural effusion was ascertained to be a transudate. The conventionally used therapeutic measures, including diuretics and thoracocentesis, failed to control pleural effusion. Administration of vincristine, adriamycin, and dexamethasone chemotherapy led to successful resolution of the effusion. CONCLUSION: Treatment with vincristine, adriamycin, and dexamethasone chemotherapy was effective for the refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation and appears to be associated with improvement in our patient's prognosis.

Early diagnosis and treatment may prevent the development of complications in an adult patient with glycogen storage disease type Ia.

Type Iota(a) glycogen storage disease (GSD Iota(a)) is caused by the deficiency of glucose-6-phosphatase activity, which results in metabolic disorder and organ failure, including renal failure. GSD Iota(a) patients are generally diagnosed at a median age of 6 months. However, we report a 20-year-old Japanese female with newly diagnosed GSD Iota(a) . The renal disorder of GSD Iota(a) is considered to be produced by glomerular hyperfiltration, TGF-beta expression which is induced by renin-angiotensin-aldosterone system (RAS) and uric acid, and the increase in both small dense LDL and modified LDL which is characteristic of GSD Iota(a) as well as hypertriglyceridemia. With the administration of intensive therapies, including angiotensin type 1-receptor blocker and some lipid lowering drugs, along with traditional dietary therapy, daily proteinuria of the patient improved from 2.1 g to 0.78 g. Although the patients of GSD Iota(a) should receive an early and accurate diagnosis and effective therapies before the age of 1 year, the combination of traditional dietary therapies and intensive therapies may have therapeutic potential for the complications of adult patients. In this report, we describe the management of renal disease and the characteristic features of this metabolic disorder.

Embryogenesis of fused umbilical arteries in human embryos.

OBJECTIVE: The objective of this study was to elucidate the embryologic basis of fused umbilical arteries in the human. STUDY DESIGN: Twenty-nine human embryo specimens at Carnegie stages 11 through 15 (4-5 weeks after fertilization) were examined histologically, with special reference to the development of umbilical arteries. RESULTS: All embryos at Carnegie stage 11 and 12 had fused umbilical arteries, and 66% of Carnegie stage 13 embryos and 29% of Carnegie stage 14 embryos still had the condition. None of the embryos at Carnegie stage 15 or older had fused umbilical arteries, but there were always 2 arteries present in their umbilical cords. CONCLUSION: Our data suggest that (1) a single umbilical artery splits into 2 as the developmental stage of the embryo advances, (2) that fused umbilical arteries represent a remnant of the embryonic phenotype, and (3) that fused umbilical arteries are embryologically distinct from true single umbilical artery.

Association of chromium exposure with multiple primary cancers in the nasal cavity.

A 56-year-old man who had worked at a chromate factory for 13 years developed squamous cell carcinoma of the left nasal cavity 11 years after retirement. He received intra-arterial chemotherapy, followed by surgery. Two years later, an adenocarcinoma was identified in the same nasal cavity just above the previous surgical region. He underwent medial maxillectomy in combination with postoperative irradiation. He has been disease free for 5 years after the second surgery. Microsatellite markers were examined in the second tumor specimen as a possible factor for carcinogenesis; however, replication errors were not observed in any of four loci (D2S123, D3S1067, TP53, D18S474) tested. The present case seems to have resulted from long-term exposure to chromium and, to our knowledge, is the first reported case with multiple primary cancers in the nasal cavity associated with chromium exposure.