A 36-year-old woman with a history of persistent cough and bronchiectasis was referred and admitted to our hospital for the evaluation of asymptomatic microhematuria with a urine protein-to-creatinine (Cr) ratio of 0.64 g/gCr, which had been found by her primary physician a few months previously. She was diagnosed with KS on a clinical basis in her childhood and experienced frequent respiratory tract infections, for which she received several courses of medical care, including the sporadic use of antibiotics and expectorants. A dipstick analysis performed 3 years previously revealed 1+ proteinuria and 1+ occult blood reaction when she was admitted to another hospital for bacterial pneumonia; however, her renal parameters were not regularly monitored after the analysis. She had never smoked and denied the use of any drugs. Her younger brother had also been diagnosed with KS.\nAt the time of admission, the patient was alert with a blood pressure of 120/71 mmHg. Her heart sounds were on the right side of the chest. Neither peripheral cyanosis nor clubbing of the digits was noted. Renal sonography showed that the right and left kidney long axis dimensions were 10.8 and 11.1 mm, respectively, with normal renal cortex echogenicity, while radiographic studies demonstrated bronchiectasis in the lower zone of both lungs, as well as dextrocardia (). A full blood count demonstrated a white blood cell count of 4,600/µL with normal differentials, a hemoglobin level of 12.4 g/dL, and a platelet count of 32.5×104/µL. Further laboratory analyses revealed the following results: blood urea nitrogen, 8 mg/dL; serum, Cr 0.4 mg/dL; uric acid, 3.8 mg/dL; total protein, 6.4 g/dL; serum albumin, 3.3 g/dL; sodium, 140 mmol/L; potassium, 4.1 mmol/L; chloride, 104 mmol/L; Ca 9.5 mg/dL; phosphorus, 3.2 mg/dL; IgG, 1,793 mg/dL; IgA, 680 mg/dL; IgM, 90 mg/dL; C3, 99 mg/dL; C4, 24 mg/dL; and C-reactive protein, 0.02 mg/dL. A serological study was negative for antimyeloperoxidase antineutrophil cytoplasmic antibody (ANCA), antiproteinase 3-ANCA, and anti-glomerular basement membrane antibodies. A 24-hour urine specimen contained 0.16 g of protein, with a Cr clearance rate of 145.1 mL/min. Renal biopsy, which was performed on hospital day 2 (), revealed two cores of renal parenchyma with nine glomeruli, one of which was globally sclerotic. The rest of the glomeruli demonstrated mild mesangial proliferation and moderate mesangial matrix accumulation. Predominant mesangial depositions of IgA and C3 were observed in an immunofluorescence analysis, while electron microscopy revealed paramesangial electron-dense deposits within a mildly expanded mesangium. Consequently, the patient’s renal abnormalities were ascribed to IgA nephropathy. In accordance with the Japanese therapeutic guidance, we prescribed a low protein diet and a periodic monitoring program, consisting of comprehensive renal care, was scheduled.
This was a 39-year-old smoker female, born by non-consanguineous parents. Married with two children. She was hospitalized in August 2006 for acute exacerbation of bronchitis with cough and mucopurulent expectoration, and hyperthermia. She reported recurrent episodes of common cold, with productive cough and rhinorrhea since the childhood. Chest CT scan was done on August 31, 2006, and revealed dextrocardia, and bronchiectasis with prevalent diffusion in the right lung. On auscultation, right basal crackles were audible. Electrocardiogram showed evidence of dextrocardia. Spirometric evaluation showed moderate obstruction. Therapy with the DPI fixed combination formoterol/budesonide was established. In the follow up visits the patients showed a stabilization of functional respiratory pattern. In 2007 the nasal brushing was performed, with demonstration of 100% cilia lacking outer dynein arm (Figure ). She missed the following up visits.