scholarly journals Congenital nephrotic syndrome: preemptive bilateral nephrectomy and dialysis before renal transplantation.

1992 ◽  
Vol 3 (2) ◽  
pp. 260-263
Author(s):  
M S Kim ◽  
W Primack ◽  
W E Harmon
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Transplantation ◽  
Mortality Rate ◽  
High Mortality ◽  
Congenital Nephrotic Syndrome ◽  
First Year ◽  
Bilateral Nephrectomy ◽  
Related Complication ◽  
Fatal Disease ◽  
First Year Of Life

Congenital nephrotic syndrome (CNS) is a rare and uniformly fatal disease if it is not treated. Although renal transplantation has been a successful treatment, there remains a high mortality rate during the first year of life before transplantation. From 1979 to 1987, four patients with CNS, all of whom died before they could undergo renal transplantation were treated. On the basis of this clinical experience, early elective bilateral nephrectomy and dialysis for infants with CNS were initiated to improve overall outcome. From 1987 to 1989, this protocol has been used on four consecutive patients with CNS. In these patients, bilateral nephrectomy was performed only after patients suffered a serious CNS-related complication that occurred between 4 and 6 months of age. Despite nephrectomy and the need for chronic dialysis, all patients grew at normal or accelerated rates. By 16 months of age, all patients underwent successful renal transplantation.

Renal Transplantation in Children With Nephrotic Syndrome in the First Year of Life

2015 ◽  
Vol 47 (1) ◽  
pp. 38-41 ◽  
Author(s):  
S. Martínez Mejía ◽  
A. Alonso Melgar ◽  
M. Melgosa Hijosa ◽  
C. Fernandez Camblor ◽  
A. Peña Carrión ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Transplantation ◽  
First Year ◽  
First Year Of Life

Congenital Nephrotic Syndrome and Persistent Hypothyroidism After Bilateral Nephrectomy

2008 ◽  
Vol 21 (6) ◽  
pp. 597-602
Author(s):  
Martina Kacer ◽  
Dilys A. Whyte ◽  
Ivy Boydstun ◽  
Thomas A. Wilson
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Transplantation ◽  
Thyroid Hormone ◽  
Normal Values ◽  
Congenital Nephrotic Syndrome ◽  
Standard Of Care ◽  
Thyroid Ultrasound ◽  
Bilateral Nephrectomy ◽  
Hormone Synthesis ◽  
Finnish Type

Abstract Congenital nephrotic syndrome is commonly associated with hypothyroidism. Thyroid hormone supplementation is recommended as standard of care. The hypothyroidism is postulated to occur secondary to chronic massive proteinuria with loss of thyroid binding globulin, thyroid hormone and iodine. Previous reports have indicated that thyroxin may be discontinued following bilateral nephrectomy. We report our experience with one child with congenital nephrotic syndrome, Finnish type, and hypothyroidism who had a high requirement for thyroxin (100-150 μg/d) from infancy to 4 years of age. Hypothyroidism persisted despite bilateral nephrectomy and later following renal transplantation. However, his thyroxin requirement is now substantially lower (62.5 μg/d) at age 14 years. No goiter was detected clinically and antithyroid antibodies were negative. Thyroid ultrasound and 1231 scan revealed a thyroid gland in the anatomically normal location. 1231 uptake was elevated, 18% at 6 hours and 51% at 24 hours (normal values: 3-16% at 6 hours and 8- 25% at 24 hours). Perchlorate was unavailable for a perchlorate washout study. We speculate that this patient may have an intrinsic problem with thyroid hormone synthesis. It is unclear whether this is related or coincidental to the Finnish nephrotic syndrome. We recommend following thyroid functions closely if thyroxin is discontinued following bilateral nephrectomies in Finnish type congenital nephrotic syndrome.

Renal transplantation in the first year of life: the treatment of choice for infants with end-stage renal disease.

1992 ◽  
Vol 2 (12) ◽  
pp. S228
Author(s):  
J S Najarian ◽  
P S Almond ◽  
M Mauer ◽  
B Chavers ◽  
T Nevins ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Transplantation ◽  
Survival Rate ◽  
Living Donor ◽  
Patient Survival ◽  
First Year ◽  
End Stage Renal Failure ◽  
End Stage ◽  
First Year Of Life ◽  
Patient Survival Rate

The treatment of choice for end-stage renal failure within the first year of life is controversial. Between September 1970 and February 1991, we performed 28 kidney transplants (27 primary, 1 retransplant, 23 living donor, 5 cadaver) in infants less than 1 yr of age (mean, 7 +/- 2 months; range, 6 wk to 12 months). The 1-yr patient survival rate for living donor recipients was 100% versus 20% for cadaver recipients (P = 0.0001). The 1-yr graft survival rate for living donor recipients was 96% versus 20% for cadaver recipients (P = 0.001). The 1-yr patient survival rate for cyclosporin A (CSA) recipients (N = 12) was 100% versus 75% for non-CSA recipients (P = 0.03). The 1-yr graft survival rate for CSA recipients was 92% versus 75% for non-CSA recipients (P = 0.08). There was no difference in the number of rejection episodes or serum creatinine levels in CSA versus non-CSA recipients. Compared with pretransplant values, the mean posttransplant standard deviation scores (SDS) for height (N = 18), weight (N = 22), and head circumference (N = 8) improved: height SDS from -1.9 to -1.5 (not significant); weight SDS from -2.5 to 0.6 (P less than 0.0005); head circumference SDS from -2.0 to -0.7 (P = 0.01). Because no other renal replacement therapy can match these results, we conclude that renal transplantation is the treatment of choice for infants with end-stage renal failure.

scholarly journals ALCAPA: The Al Capone of coronary artery anomalies

2012 ◽  
Vol 16 (3) ◽  
pp. 100-101 ◽  
Author(s):  
Farzanah Ismail
Keyword(s):  
Coronary Artery ◽  
Mortality Rate ◽  
Left Coronary Artery ◽  
First Year ◽  
Coronary Artery Anomalies ◽  
Perfusion System ◽  
Anomalous Left Coronary Artery ◽  
First Year Of Life ◽  
Anomalous Left Coronary ◽  
Artery Perfusion

Anomalous left coronary artery originating from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that presents with myocardial ischaemia or infarction and/or cardiac failure in infants. It is associated with a mortality rate of 90% within the first year of life. Surgical correction to re-establish a two-coronary artery perfusion system is the treatment of choice, once patients are medically stable.

scholarly journals Population Structure and Life Table of Dog Conch (Strombus turturella) in Bangka Belitung Islands, Indonesia [Struktur Populasi dan Tabel Hidup Siput Gonggong (Strombus turturella) di Kepulauan Bangka Belitung, Indonesia]

2019 ◽  
Vol 11 (2) ◽  
pp. 72
Author(s):  
Okto Supratman ◽  
Tati Suryati Syamsudin
Keyword(s):  
Mortality Rate ◽  
Life Table ◽  
High Mortality ◽  
Economic Value ◽  
Age Groups ◽  
Time Estimation ◽  
First Year ◽  
Age Group ◽  
Growth Coefficient ◽  
Recruitment Time

AbstractDog Conch (Strombus turturella) has an essential economic value in Bangka Belitung Islands. Allegedly, the population of Dog Conch is decreasing due to overexploitation. The purpose of this study is to provide information related to the distribution of long frequency, growth pattern, age group, recruitment time estimation and life table of Dog Conch. This research took place on the coast of Tukak Village and Anak Air Island, Bangka Belitung Islands. Samples of Dog Conch were taken using 3x3 m2 square. The shell length of Dog Conch found ranged between 18.18 to 77.49 mm, consisting of three age groups. Asymptotic length value (L∞), growth coefficient (K) and theoretical age on zero-length (t0) were 83.94 mm, 0.79/year and -0.152 sequentially. In the first year, Dog Conch grows to 50.18 mm and slows down when it grows older until it is 13 years old. The proportion of high mortality rate was at 1 to 2 years old and 3 to 4 years old or in adult individuals, while the highest life expectancy rate was in the age group of 0-1-year old or young individuals. It indicated that the high mortality rate was in the group in which people use to consume or sell in the marketsAbstrakSiput gonggong (Strombus turturella) memiliki nilai ekonomis penting di Kepulauan Bangka Belitung. Diduga populasi siput gonggong semakin menurun akibat dari eksploitasi berlebihan. Tujuan penelitian ini adalah untuk memberikan informasi terkait distribusi frekuensi panjang, pola pertumbuhan, kelompok umur, estimasi waktu rekruitmen dan tabel hidup siput gonggong. Lokasi penelitian berada di Pesisir Desa Tukak dan Pulau Anak Air, Kepulauan Bangka Belitung.Pengambilan sampel siput gonggong dilakukan dengan menggunakan kuadrat 3x3 m2. Panjang cangkang siput gonggong yang ditemukan berkisar antara 18.18 s.d 77.49 mm yang terdiri atas 3 kelompok umur. Nilai panjang asymptotic (L∞), koefisien pertumbuhan (K) dan umur teoritis ketika panjang sama dengan nol (t0) adalah 83.94 mm, 0.79/tahun dan -0.152 secara berurutan. Pada tahun pertama siput gonggong mengalami pertumbuhan, mencapai 50.18 mm dan melambat ketika umur semakin tua hingga umur 13 tahun. Proporsi laju kematian tinggi terdapat pada umur 1 s.d 2 tahun dan 3 s.d 4 tahun atau pada individu dewasa, sedangkan nilai harapan hidup tertinggi terdapat pada kelompok umur 0-1 tahun atau individu muda. Hal ini menunjukkan bahwa kematian tertinggi terdapat pada kelompok umur yang telah diambil oleh masyarakat untuk dikonsumsi dan dijual ke pasaran.

scholarly journals ANÁLISIS DEL CRECIMIENTO Y LA MORTALIDAD DE ANISOTREMUS INTERRUPTUS (GILL) (PERCIFORMES: HAEMULIDAE) EN LA COSTA DE COLIMA, MÉXICO

2016 ◽  
Vol 33 ◽  
Author(s):  
Elaine Espino Barr ◽  
Manuel Gallardo Cabello ◽  
Fernando González Orozco ◽  
Arturo Garcia Boa
Keyword(s):  
Mortality Rate ◽  
Growth Parameters ◽  
Total Mortality ◽  
Condition Index ◽  
First Year ◽  
The Third ◽  
Second Year ◽  
First Year Of Life ◽  
Mortality Analysis

This paper deals with the growth and mortality analysis of the burrito grunt A n i s o t remus interru p t u s on the coast of Colima, México. The estimated growth parameters are: L¥ = 50.59 cm; W¥ = 5,051.04 g; k = 0.147 years- 1; to = -0.916 years; A0 . 9 5 = 19.46 years. Most of the growth occurred during the first year of life, when the grunt grows 12.52 cm, the second year it grows 4.95 cm and the third, 4.60 cm. The highest value of the condition index took place between February and September. The total mortality rate (Z) was calculated as 0.53 years- 1. These values are basic for the plan of administration of the fishery of this species.

THE NEPHROTIC SYNDROME IN THE FIRST YEAR OF LIFE

PEDIATRICS ◽  
1960 ◽  
Vol 25 (6) ◽  
pp. 967-976
Author(s):  
R. A. Parker ◽  
Carolyn F. Piel
Keyword(s):  
Nephrotic Syndrome ◽  
Cortical Area ◽  
Clinical Course ◽  
Renal Pathology ◽  
Electrolyte Imbalance ◽  
First Year ◽  
Renal Lesions ◽  
Susceptibility To Infection ◽  
First Year Of Life

The clinical course of nephrosis in five infants with onset of disease before 7 months of age is presented, together with evaluation of renal lesions seen at necropsy. The problems of the management of nephrosis susceptibility to infection and water and electrolyte imbalance were found to be exaggerated by the young age of the patients. The renal pathology observed in these five infants consisted of persistence of immature glomeruli and dilatation of the tubules in the cortical area. Later, the immature glomeruli and associated tubules appear to atrophy and the remaining glomeruli to hypertrophy. Long-term adrenocorticosteroid therapy seems to be contraindicated, not only on the basis of the pathologic changes, but because it greatly exaggerates the problems of management and does not effect a remission of the disease.

Outcome of esophageal atresia in Germany

2020 ◽  
Author(s):  
A Schmedding ◽  
B Wittekindt ◽  
R Schloesser ◽  
M Hutter ◽  
U Rolle
Keyword(s):  
Mortality Rate ◽  
Esophageal Atresia ◽  
First Year ◽  
Insurance Company ◽  
Associated Anomalies ◽  
Clinical Registry ◽  
Major Health ◽  
First Year Of Life ◽  
Overall Mortality

Abstract The aim of this study was to evaluate the outcome of esophageal atresia in Germany in a retrospective observational study of a large cohort. Data from the major health insurance company in Germany, which covers approximately 30% of German patients, were analyzed. All patients born and registered between 2009 and 2013 with a diagnosis of esophageal atresia at first admission to the hospital were included. Mortality was analyzed during the first year of life. We identified 287 patients with esophageal atresia, including 253 with and 34 without tracheoesophageal fistula. Associated anomalies were found in 53.7% of the patients; the most frequent were cardiac anomalies (41.8%), anomalies of the urinary tract (17.4%), and atresia of the colon, rectum, and anus (9.4%). Forty-one patients (14.3%) had a birth weight <1500 g. Seventeen patients (5.9%) died before surgery. Gastrostomy was performed during the index admission in 70 patients (25.9%). The reconstruction of the esophageal passage was performed in 247 patients (93.9%). Forty-eight percent of the patients who underwent an operation required dilatation. The mortality rate in the patients who underwent an operation was 10.4%. These results from Germany correspond to the international results that have been reported. The number of dilatations was in the middle of the range of those reported in the literature; the overall mortality rate was in the upper portion of the range of the international rates. Efforts should be made to establish a clinical registry to measure and improve the quality of care for this and other rare conditions.

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