Articles

Introduction: Anderson-Hynes pyeloplasty remains the gold standard in the treatment of ureteropelvic junction obstruction. The diagnostic criteria for defining the failure of pyeloplasty are not well-defined or even arbitrary. Likewise, the ideal treatment of persistent hydronephrosis after pyeloplasty is not well established. We tested an innovative endourological procedure, which simultaneously allows a diagnostic definition of failure and treatment when necessary. Materials and methods: The endourological procedure was applied prospectively to 13 cases from 2006 to 2015. The mean hydronephrosis was 3 cm and all the patients showed an obstructive pattern at scintigraphie. Of these, only 2 patients had symptoms. The procedure consisted in the endoscopic calibration of the pyeloureteral junction. In case of confirmed persistent stenosis, the procedure continued with the high pressure dilation of the junction. The calibration/dilation procedure was carried out with a balloon catheter, previously used for high pressure dilation in the obstructive megaureter. In all patients, a ureteral stent was positioned for 6 - 8 weeks. The patients were then followed up using ultrasound and renoscintigraphie. Results: According to the endoscopic balloon procedure, anastomosic stenosis was confirmed in 3 cases, treated with high pressure dilation during the same procedure. In 10 cases no stenosis was found and we followed-up these patients with periodic ultrasound and scintigraphie. Conclusion: The calibration/dilation of the pyeloureteral junction represents in our opinion a useful diagnostic and therapeutic tool that allows to limit the repetition of open surgery only to symptomatic cases and those non-responders to endoscopic treatment.

We investigated the existence of microalbuminuria in children with corticosteroid-sensitive idiopathic nephrotic syndrome in complete remission. In the study of a series of 18 cases, we noted a clearly different evolution depending on the existence or absence of pathological micro albuminuria. Microalbuminuria appears to be a prognostic discrimination parameter in idiopathic nephrotic syndrome. 

Acute renal injury is a rare complication of idiopathic nephrotic syndrome with mesPGN. Here we present a 2-year-old male patient with 4 days history of anuria, generalized edema and hypervolemia. Any evidence other than proteinuria and renal failure could not be identified with laboratory tests and doppler ultrasonography. Anuric presentation was thought to be related with rapidly progressive glomerulonephritis, diffuse mesangial sclerosis or acute tubular necrosis. However, renal biopsy revealed mesangial proliferative glomerulonephritis (mesPGN). Prednisolone 2 mg/kg/day was prescribed. Diuresis was started gradually and on the 10th day of disease, anuria was resolved and acute renal injury recovered without any sequel. This case is presented because of the incompatibility between clinical findings and histopathologic diagnosis. It is concluded that although rare, anuria and acute renal injury could be the presenting symptom of idiopathic nephrotic syndrome in childhood. 

Granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis (AAV), is a rare disease with an often-occult presentation. It is more common in 4th and 5th decades of life but can be seen in all ages. This case report details a 76-year-old female presenting with abdominal pain, generalized weakness, and malaise, who was found to have pulmonary nodules on chest imaging. Biopsy of the lung nodule showed organizing pneumonia. Initially, antibiotics were used to treat the patient. However, she developed acute renal failure a few days after presentation and found to have positive serum C-ANCA as well as elevated ANCA-PR3 serologies. A subsequent kidney biopsy demonstrated pauci-immune necrotizing and crescentic glomerulonephritis that was consistent with GPA and the patient was started immediately on combination immunosuppressive therapy, plasmapheresis, and hemodialysis. GPA’s clinical and radiological presentation can mimic other common conditions such as pneumonia, malignancy, bacterial sinusitis, pulmonary tuberculosis, sarcoidosis, and urinary tract infection. Because of this, a high level of suspicion is required for early diagnosis and treatment to alter the high mortality rate in this disease entity. All forms of ANCA-associated vasculitis (AAV) should be in the differential diagnosis for all patients presenting with multiorgan system involvement particularly in individuals with pulmonary and renal manifesations.

A 61-year-old Brazilian black woman consulted with a nephrologist due to proteinuria identified on a routine urine test. She has a personal history of thymoma resection five years ago, followed by multiple episodes of pulmonary infections including mycobacteriosis, recurrent mucocutaneous candidiasis, and paraneoplastic pemphigus. Physical examination showed no edema or hypertension and laboratory tests identified proteinuria of 2.43 g/day without hematuria, serum creatinine of 0.69 mg/dl, urea 34 mg/dl, serum albumin of 2.4 g/dl, hemoglobin 10.9 g/dl, platelets 292,000/mm3, leukocytes 4950/mm3, lymphocytes 594/mm3 and neutrophils 3910/mm3. The hemolysis tests were negative and serum iron was low. Analysis of glicemia and serum lipids levels were normal as well as serum complement and imunoglobulins, except for an IgM level of 283 mg/dl (normal values 40 to 230 mg/dl) and undetectable IgE. Serologies for Syphilis, HIV, hepatitis B, C and antibodies for autoimmune diseases were negative. 

Infantile cystinosis is a lysosomal storage disease leading to end stage kidney disease at early ages. There is no effective treatment and patients require long term dialysis or kidney transplant for survival. We present our experience on three affected children who received HLA matched allogeneic stem cell transplant. The protocol used was novel and designed to promote engraftment. The primary endpoint was safety for treatment related mortality or morbidity; All three children survived without serious adverse effects during extended follow up for over 4 years. Although we could not prove engraftment, all three children met secondary end point of sustained target functions over a 6 month follow-up. Further studies are warranted to further evaluate safety and efficacy of MSC treatment for infantile cystinosis.

Sleep related breathing disorders (SRBD) are among seven well-established major categories of sleep disorders defined in the third edition of The International Classification of Sleep Disorders (ICSD-3), and Obstructive Sleep Apnea (OSA) is the most common SRBD [1,2]. Several studies have demonstrated that obstructive sleep apnea treatment increases the quality of life in OSA patients [3-8]. Indeed, excessive daytime sleepiness (EDS), cognitive impairment (e.g., deficits in attention-concentration, memory, dexterity, and creativity), traffic accidents, and deterioration of social activities are frequently reported in untreated patients [9-11]. Furthermore, an increase in cardiovascular morbidities and mortality (systemic hypertension, stroke, cardiac arrhythmias, pulmonary arterial hypertension, heart failure) [12], metabolic dysfunction, cerebrovascular ischemic events and chemical/structural central nervous system cellular injuries (gray/white matter) has been reported in OSA patients [13-17].  Continuous positive airway pressure (CPAP) therapy is considered the gold standard for treatment of moderate-severe OSA, nevertheless there is an increasing body of evidence supporting the usefulness of mandibular advancement devices (MADs) for improving quality of life and respiratory parameters even among patients with a high severity of OSA burden [5,10,18,19]. According to the standard of care of the American Academy of Sleep Medicine (AASM), MADs are indicated for mild to moderate OSA particularly in the context of CPAP intolerance or refusal, surgical contraindication, or the need for a short-term substitute therapy [9,15,20-22]. In Cuba, CPAP machines are not readily available; they are expensive and the majority of OSA patients cannot obtain this mode of therapy. Taking into account this problem, our hypothesis was based in the scientific evidences of MAD effectiveness, considering that low cost MADs could offer a reasonable alternative treatment for patients with OSA where CPAP technology are not handy. In this way our purpose was to assess the efficacy of one of the most simple, low cost, manufactured monoblock MAD models (SAS de Zúrich) in terms of improvements in cerebral function, sleep quality and drowsiness reports in a group of Cuban OSA patients with mild to severe disease. Outcome measures included changes in the brain electrical activity, sleep quality, and respiratory parameters, measured by EEG recording with qEEG analysis and polysomnographic studies correspondingly, which were recorded before and during treatment with an MAD, as well as subjective/objective improvements in daytime alertness. 

Catheter-associated urinary tract infection (CAUTI) is among the most common nosocomial infections especially in acute care settings. Its economic and unanticipated health implications make it burdensome for the healthcare providers and patients. The paper examined the perceived causes and mode of preventing urinary tract infections in patients with spinal cord injury. Qualitative research approach was utilized; the study site was a Tertiary Hospital in Nigeria. Eight (8) in-depth interviews (IDI) were conducted with healthcare providers managing patients with spinal cord injured in the hospital. The major risk factors causing urinary tract infection identified include financial problems, organization of care, human error, hospital environment and patient-related factors. To prevent urinary tract infections among patients in the hospital, a number of suggestions were made by the participants such as training of caregivers and educating patients and relations. The authors concluded that the incidence of CAUTI could be reduced in the hospital if the opinions of stakeholders are fairly considered.

Many side effects, in addition to those of the pathology itself, have occurred with hemodialysis treatment but existing literature have shown that physical activity is beneficial to hemodialysis patients. Nevertheless, our parameters have not been studied enough with a resistance training program. In our study we have observed the effect of a 12-week intradialytic resistance training program (T0 vs T12) on the quality of life using the KDQOL-SF questionnaire, blood samples biological parameters and sleep using the Epworth scale and the International restless legs syndrome study group scale. The resistance training program consists of 3 sessions per week and involved lower extremities thanks to elastic band and soft ball. After the training program, the quality of life score trend to the increase at T12 compared to T0. Among the biological parameters, the only trend decrease observed was in the C-reactive protein and a trend increase was observed in urea at T12. Dialysis efficiency presented no changes and no significant results were observed for sleep. Some trends were observed as a result of our program. The type of exercise seems to have different effects on measured parameters. Nevertheless, exercise was beneficial to chronic hemodialysis patients and seemed to improve their health.

Atrial septal defect (ASD) is common among adult congenital heart diseases but rarely causes paradoxical cerebral embolism. By sharing the ASD diagnosed after the first ischemic stroke attack at the age of 49 and a case of paradoxical cerebral embolism developing accordingly, we aimed to draw attention to the necessity of detailed cardiac examination in patients with cryptogenic stroke.