PATTERN AND OUTCOME OF RENAL DISEASES IN HOSPITALIZED CHILDREN IN TIKUR ANBESSA SPECIALIZED TEACHING HOSPITAL, ADDIS ABABA, ETHIOPIA.

Background: Renal diseases are major causes of morbidity and mortality in pediatric practice. Pediatric patients with renal disease, especially younger ones may present with nonspecific signs and symptoms unrelated to the urinary tract. Unexplained fever or failure to thrive may be the only manifestation. Most children with renal diseases in our hospital arrive very late either because of inadequate health awareness among the parents or failure of recognizing the symptoms of renal diseases at a lower health care level. This review will highlight the symptoms of renal diseases at presentation and outcomes of treatment in children in a major referral hospital. Methods: A cross-sectional retrospective chart review was done over a period of 3 years (June, 2012 to May, 2015) in 381 admitted children (Birth-17 years) at Tikur Anbessa Specialized Teaching Hospital in Addis Ababa, Ethiopia. Results: Out of 14521 pediatric ward admissions in the study period, kidney diseases accounted for 473 admissions in 381 children, accounting for 3.3% of all admissions. The three most common renal diseases observed were congenital anomalies of the kidney and urinary tract (CAKUT) seen in 127 children (26.8%), followed by nephrotic syndrome in 80 children 16.9% and acute glomerulonephritis in 58 children (12.2%). Other renal diseases observed were urinary tract infection 8.0%, urolithiasis 6.7%, Wilm's tumor 6.3%, acute kidney injury 4.2% and chronic kidney disease 4.0%. Other less frequently detected diseases were bladder exstrophy, lupus nephritis, Henock shonlein Purpura nephritis and prune-belly syndrome. Out of 381 children 207 (54.3%) recovered normal renal function, 20(5.2%) remained with proteinuria, 13(3.4%) progressed to chronic kidney disease and 11(2.9%) died. Sixty one nephrotic children (76.3%) achieved remission but 17 children (21.3%) remained with proteinuria; one steroid resistant child died of end stage renal disease. Ten children (2.6%) with different renal diseases were lost to follow-up and 5 (1.3%) discharged against medical advice. Conclusions: This data reflects that many of the renal diseases are preventable or potentially curable. Therefore, improvement of pediatric renal services and training of health workers would help in early detection and treatment of these conditions leading to reduction in their morbidity and mortality.

Cloacal exstrophy: a single center experience.

OBJECTIVE: Cloacal exstrophy is an exceedingly rare and complex anomaly. The records of 23 patients treated in a tertiary care center with limited infrastructure were analyzed for anatomic types, associated anomalies, surgical procedures adopted, and the outcome. MATERIALS AND METHODS: There were 14 males. Seventeen babies were preterm with an average weight of 1.92 kg. The time of presentation, gestational age, birth weight, position of the hemibladders and associated malformations were noted. Reconstruction procedures involved dismantling of the hemibladders and primary turn in, tubularization of the bowel with an end colostomy, and reconstruction of the abdominal wall. Results of the primary surgical repair, bowel function, and outcome of secondary procedures were analyzed. RESULTS: The position of hemibladders was lateral in 11, upper confluent in 4 and lower confluent in 8. Associated anomalies were noted in 19 patients. Four patients presented late (>5 days). Five died preoperatively, all had major associated anomalies. Four of them were preterm with average weight of 1.4 kg. Two patients refused surgery. Single-stage surgical reconstruction was done in 15 patients. Five patients died postoperatively because of associated anomalies, prematurity, and sepsis. One patient is waiting for surgery. Six patients had follow-up at 3-42 months and are awaiting further reconstruction. Four patients were lost to follow-up. CONCLUSIONS: Prematurity, late presentation, and sepsis are the major causes of high mortality noted in this series. In our experience, single-stage reconstruction without osteotomy gives satisfactory results.

Surgical repair of bladder exstrophy in the modern era: contemporary practice patterns and the role of hospital case volume.

PURPOSE: Bladder exstrophy is a rare condition, and data are lacking regarding practice patterns in its surgical management. We used a large nationwide database to investigate practice patterns of bladder exstrophy repair. MATERIALS AND METHODS: We used the Nationwide Inpatient Sample (1988 to 2000) to identify patients who underwent surgical repair of bladder exstrophy (International Classification of Disease-9 code 578.6). We analyzed factors affecting practice patterns and outcomes. Hospital volume was based on caseload during the highest volume year of study participation (high volume 5 or more, mid volume 3 to 4 and low volume less than 3 cases). RESULTS: We identified 407 cases. Approximately half of the patients (53.2%) were hospitalized within 24 hours of birth, although 28% of patients were older than 1 year. Of the patients 54% were male. Exstrophy repair is extremely resource intensive. In this series mean length of hospital stay (LOS) was 24.6 +/- 22.8 days, and mean inflation adjusted hospital charges were 62,302 dollars (median 39,978 dollars). High volume hospitals (HVHs) had lower hospital charges (37,370 dollars) than mid volume (51,778 dollars) or low volume hospitals (LVHs, 50,474 dollars, p = 0.0095). On multivariate regression HVHs had lower charges even after controlling for other significant predictors, including LOS (p <0.0001). Patients at HVHs were more likely to undergo osteotomy (p = 0.007). Six patients died after exstrophy repair (1.5%), all of whom had been born prematurely (p <0.0001). Although death was more likely at LVHs, this was due to the fact that more patients at LVHs were born prematurely (4.2% at HVHs vs 5.9% at mid volume hospitals and 11.1% at LVHs, p = 0.027). CONCLUSIONS: Bladder exstrophy repair carries a high risk of morbidity and is resource intensive. Variations between high and low volume hospitals in practice patterns and case mix may contribute to observed differences in resource use, LOS and clinical outcomes.

[Bladder exstrophy. Treatment results over 27 years]. / Die Blasenekstrophie. Behandlungsergebnisse von 27 Jahren.

Between 1960 and 1988 47 patients with bladder exstrophy were attended. 7 various operative procedures were applied. 3 patients died postoperatively, 37 of the 42 patients would be examined after 1 11/12 to 27 years. All patients with ureterosigmoidostomy exhibited electrolyte disturbance and metabolic acidosis. Patients with sigmoid conduit, rectal bladder and bladder reconstruction had unremarkable findings. The uretero-enterostomy anastomotic region was endoscopically essentially unremarkable. The control of continence was from satisfactory to good, and there was not absolute incontinence. X-ray investigation revealed both distinct and indistinct changes. The psychic and social stress was more serious in childhood than in adulthood.

Cloacal exstrophy: a 22-year experience.

Our 22-year experience in the management of 12 patients with cloacal exstrophy is discussed. All patients underwent functional bladder closure. Of 7 evaluable patients 3 (43 per cent) have continent intervals of 3 to 4 hours and constitute the first report of satisfactory urinary continence following functional bladder closure in patients with cloacal exstrophy. Of 8 patients with an XY karyotype 6 were raised as girls with satisfactory genitoplasty, while 2 raised as boys had functionally inadequate external genitalia. The management of the rudimentary hindgut varied and the alternative approaches are discussed. It appears that this patient population can be rehabilitated to lead productive lives.

Improved life expectancy for children with exstrophy of the bladder.

Life-table techniques demonstrate a 98% ten-year survival, a 96% 20-year survival, and 91% 35-year survival of children with exstrophy of the bladder. These figures represent a vast improvement over the earlier figures, due in large part to technical advances that have been made in areas of surgery, anesthesia, and antibacterial and metabolic therapy.

Long-term results of ureterosigmoidostomy in children with bladder exstrophy.

The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution of the question of disposal of the diverted urine.