Issue 2 Vol 2 August 1999
The study of Proteinurea in Idiopathic and Congenital Nephrotic Syndrome
Éva Kiss, prof. Dr. I. Muntean, Iolanda Kusztos
Pediatric Clinic Nr.1 from Tg.-Mureş: University of Medicine and Farmacy Tg.-Mureş
The nephrotic syndrome (NS) is the most common childhood glomerulopathy characterized by heavy proteinuria, hypoproteinureia, hyperlipidemia, oliguria and edema.
The authors studied the quantitative and qualitative aspects and selectivity of proteinureia in childhood nephrotic syndrome (NS).
The histopathological structure was also studied and it determined the diagnosis, treatment and prognosis of childhood NS.
In conclusion the authors demonstrated that:
Clinical aspects of renourinary
abnormalities in children
Dr.Iolanda Kusztos, Conf.Dr.Eva Kiss
Ist Departament of Pediatrics Tg.Mures – Prof.Dr.I.Muntean
Goal of the study.Malformative uropathies represent an important percentage of congenital abnormalities of the child, heir incidency being of 10% to 40%. Their early diagnosis is essential for the further development of the patient.
Study group and method. The studied group included 142 cases diagnosed as having renourinary abnormalities in the Ist Departament of Pediatrics, between 1990-1998. The data were obtaind by a retrospective analysis of the clinical history files.
Resultes. The data lead us to following conclusiones : a) renourinary abnormalities were diagnosed especially between 1-3 years in the two last years; b) urinary tract infection was the most important element of suspicion for renourinary abnormalities; c) the renourinary abnormalities most often found were, in order of incidency : pyelo-ureteral duplication, pyelo-uretheral jonction syndrome, policystic kidney, a.s.o.;d) renourinary abnormalities are often associated with cardiac and bone abnormalities.
Conclusion. Renourinary abnormalities are relatively frecquent and have severe consequence, this being the explanation for the importance of early diagnoses. They usually present like an urinary tract infection, and that^s the reason why UTI should be investigated from the first episode, in both males and females. Early diagnosis of the abnormalities should be pertinual, eighter prenatal, useing renal ultrasound, or early postnatal.
Considerations on a case with baldder extrophyParaschiva Chereches-Panta, Mircea Nanulescu, Otilia Fufezan, Mihai Lucan, Dana Campean
University of Medicine and Pharmacy Cluj Napoca
AbstractBladder extrophy is an uncommon disorder of urinary tract, its incidence being of about 1:10,000-1:50,000 live births. From embryological point of view, bladder extrophy results from the failure of the cloacal membrane to be reinforced by in growth of mesoderm. There are several types of bladder extrophy, as follows: a) pseudoextrophy; b) superior vesical fissure variant; c) duplicate extrophy; and d) covered extrophy or split symphysis variant. Each of them are characterized by different grades of the extent of the abnormality according to the moment that the determining factor occurs during intrauterine development of urogenital tract. Bladder extrophy is frequently associated with other anomalies, spinal anomalies being present in most of the cases. Spinal anomalies occur at a rate of about 6.7% and are represented by myelomeningocele, vertebral fusion and hemivertebrae. Vesicoureteral reflux develops most frequent secondary to surgical interventions for bladder reconstruction. Generally, the upper urinary tract is normal. The authors present the case of a 12 years old girl that was recognized at birth with classic bladder extrophy complete type. Numerous surgery reconstruction interventions were performed but urinary incontinence and urinary tract infections persisted. No further investigations were done until the age of 9 years. At this time the ultrasound exam, IV urography and voiding cystography revealed bilateral bifid collecting system (complete right ureteral duplication and incomplete left ureteral duplication) and bilateral vesicoureteral reflux. Antireflux intervention which was performed was successful, but after 3 years the girl presents scintighraphyc and ultrasound signs of type "d" reflux nephropathy. This is the main factor that will determine the long-term prognostic in this case.Key words: bladder extrophy, children, upper urinary tract defects
Nephrocalcinosis in children
Nanulescu Mircea, Paraschiva Chereches-Panta, Valean Cornel, Otilia Fufezan, Dana Campean, Farcau Dorin, Mirela Popa, Sorin Man
University of Medicine and Pharmacy Cluj Napoca – III Pediatric Unit
Before investigation techniques were improved nephrocalcinosis was considerate a rare disease. At present the widespread use of ultrasonography made a significant change of incidence of new cases of nephrocalcinosis. However, there exist some other disorders that may be revealed as renal hyperecoic images.
The authors intent to evaluate the incidence of nephrocalcinosis and its etiology in a retrospective study over a period of 2 years, in an universitary center of general pediatrics. The ultrasound examination was performed by bidimensional method with Siemens Sonoline SI250 ultrasonographer with sectorial transducer of 5 MHz. There were identified 11 children cu aspects ultrasonographic of nephrocalcinosis. They were evaluated by clinical and laboratory investigations. An ultrasound guided transcutaneous kidney biopsy was performed in 4 cases. Children had between 3 weeks and 8 years of age, 4 of them being infants. Three cases were males, and 8 were females.
Diagnosis of nephrocalcinosis was confirmed in 7 cases of the eleven with renal hyperecoic images. In 2 of these 7 cases with nephrocalcinosis the etiology was hypervitaminosis D and hypersensitivity to vitamin D respectively. However, one case with hypervitaminosis D had renal interstitial fibrosis on kidney biopsy, diagnosis that may appear on ultrasound nephrocalcinosis-like. In the other 5 cases in which nephrocalcinosis was confirmed the etiology was as follows: absorptive hypercalciuria (1 case), renal hypercalciuria (1 case), distal tubular acidosis type I (1 case), and normocalcemic normocalciuric nephrocalcinosis (2 cases). These two cases with normocalcemic normocalciuric nephrocalcinosis had positive familial history for renal stones so that we think for a possible hyperoxaluria. None of the described cases had cortical nephrocalcinosis. The prevalence of medullar nephrocalcinosis is higher as compared with cortical nephrocalcinosis as it is mentioned by literature.
In conclusion, nephrocalcinosis is a disease with a trend of increasing its place in pediatric morbidity due to modern diagnosis techniques that aloud a better depicting of this disorder.
Key words: nephrocalcinosis, children, ultrasonography.
Mircea V. Nanulescu MD, PhD
(University of Medicine and Pharmacy Cluj-Napoca, IIIrd Pediatric Unit)
Vezico-ureteral reflux (VUR) is secondary to the lost of valve capacity of uretero-vezical jonction. Primitive vezico-ureteral reflux is the consequence of a deffect or of a delay in the development (maturation) of uretero-vezical jonction. Secondary vezico-ureteral reflux is produced by some changes of bladder dynamics like infrabladder obstruction, neurogenic bladder, urinary tract infection. The disease is usualy diagnosed while an urinary tract infection is investigated.
The frecquency of vezico-ureteral reflux is higher in smaller children and in females. The disorder has familial character. The "gold standard" technique for the diagnosis is voiding cystography. According to the extent of reflux and to the dilatation of collecting system there are 5 grades of vezico-ureteral reflux. The only symptom with some specifficity is lombar pain, that exacerbates at the beginning of micturition and ameliorates after emptying the bladder.
Reflux nephropathy (RN) represents the most frecquent complication of vezico-ureteral reflux and it is characterised by plloting scars of renal parenchima. These changes mey be revealed by Tc99m -DMSA scintigraphy. Reflux nephropathy represents 10% of total cases with chronic renal failure that require dialysis and kidney transplant. There are several pathogenic theories, like: embrioic displasia, role of urinary tract infection (compulsory), the effect of "water-hammer" practised by reflux, role of intrarenal reflux that is facillitated by some anatomic paticullarities of papilla. Clinical features of reflux nephropathy is non-speciffic (proteinuria, hypertension, chronic renal failure).
Fro the grade IV or V of vezico-ureteral reflux accopmanied by frequent urinary tract infecrtions the surgical treatment is reccomended. Clasical surgery procedure (urethral submural route elongation) is efficient in 90-100% of cases. There are some modern techniques less invasive: laparoscopic intervention or change of trigonal area configuration by cystoscopuic injection of some materials. For those situations that do not have surgery recommendation and for postoperatory stage the prophylaxis by drugs of urinary tract infection is required.
WILMS’ TUMOR – A CLINICAL FOLLOW-UP of 75 PATIENTS
Authors: Ingrid Miron MD, Lelia Maimescu MD, Prof. O.Brumariu MD, Prof.I.Tansanu MD
Department of Oncology Children’s Hospital “St.Mary” Iaşi – România
Wilms’ tumor (nephroblastoma) is the second most common malignant retroperitoneal tumor and most common primary renal tumor of childhood.
The incidence of Wilms’tumor is 8,1 cases per million in white children younger than 15 years of age and constitutes 6% of all childhood cancers are diagnosed between 1 – 5 years of age, peak incidence occurs at 3 – 4 years of age.
This article describes the epidemiology, histopatologic features, clinical manifestations and treatment results of 75 children with Wilms’tumor seen from 1983 to 1998, admited in Oncological Department of Children’s Hospital “St.Mary” Iaşi.
Armong them, 73,3% (53) were of favorable histology and 26,6% (22) were of unfavorable histology. Preoperative chemotherapy was given in 68 patients.
After managing the patients with multimodal therapy (nephrectomy, chemotherapy and sometimes radiotherapy) according to the protocols SIOP 6 and SIOP 9 the favorable group had shown better prognosis – 89,2% and the unfavorable histology 35%. It is therefore important to assess their long-term renal function after therapy.
Renal function was assessed by measuring blood urea, serum creatinine (Cr) at the following intervals: 6 months after treatment, 2 – 4 years after treatment.
We also performed at 30% of the cases DMSA scintigraphy.
The results show that the remaining kidney took over the other function which was normal in 90%.
The nephrotoxicity of chemotherapeutic protocols didn’t influence the renal function and it was of low grade regarding hematological and digestive parameters.
Sindromul Alport, consideraţii pe marginea unui caz
Luminiţa Lazăr, Eva Nemeş
(Clinica II Pediatrie Craiova)
Autorii prezintă două cazuri de sindrom Alport: un băiat în vârstă de 13 ani, diagnosticat la această vârstă şi un adult de 29 ani, diagnosticat şi urmărit de la vârsta de 11 ani. Diagnosticul a fost pus pe existenţa unui complex de modificări patologice, caracteristice sindromului Alport: nefropatie hematurică, apărută în circumstanţe infecţioase, surditate de percepţie bilaterală asimetrică, anomalii oculare (anizocorie, miopie), coeficient de inteligenţă scăzut. Diagnosticul diferenţial a luat în considerare glomerulonefrita acută poststreptococică, hematuria benignă familială şi nefropatia cu depunere de IgA, hematuria de efort şi glomerulonefritele persistente progresive primare sau secundare (excepţional).
Two cases of Alport syndrome are presented: one boy aged of 13 and, whose diagnosis was established at that age and the second, belonging to an adult man, aged 29, whose diagnosis was established when he was a child 11 years old.
The diagnosis was established in both two cases, taking into account of the following pathologic changes: hematurical nephropaty, met during an acute respiratory infections; perception type symmetrical and bilateral deafness, ocular abnormalities (pupil inequality, short-sightedness), a low intelligence rate (coefficient).
The natural history of the hereditary nephropaty, associated with the bilateral deafness and renal transplantation in the first case and terminal hematuria with a permanent increasing of its intensity and duration, the worsening both of the deafness and short-sightedness, a constant increasing of the nitric retention in the last 6 months (after a 23 years evolution) and the imagery (little and sclerotic kidneys) pointed out the specific evolution of the patients to the chronic renal failure.
The differential diagnosis was made with others glomerular hematuria (the benign family hematuria, the nephropaty with IgA deposition, the effort hematuria) and exceptionally with the others primary or secondary persistent and progressive glomerulonephritis.
It is especially highlight the transmission manner in both cases, autosomal recessive for the first case and in unclear manner for the second.
Consideratii pe marginea unui caz de sindrom de nev epidermic
Dr. Doina Plesca*, prof. dr. Dimitrie Dragomir*, dr. Ioan Anghelescu**
*Clinica de pediatrie, Spitalul Clinic de Copii "Dr. Victor Gomoiu", Bucuresti
** Sectia de Pediatrie 1, Spitalul Judetean Ploiesti
Autorii prezinta o afectiune rara in practica pediatrica. Considerata ca facomatoza de literatura de specialitate, sindromul nevului epidermic este ilustrat prin descrierea unui caz clinic care poseda toate trasaturile caracteristice. In continuare, afectiunea este descrisa din punct de vedere teoretic.
Cuvinte cheie: Sindromul nevului epidermic, sindroame neurocutanate, copil
Epydermic naev Syndrome
The authors present an unusual disease in pediatric practice. Named as phacomatoses in a variety of references the epydermic naev syndrome is illustrated with a clinic case having all the caracteristics. Then, the illness is described theoretical.
Key words: Epydermic naev syndrome, neurocutaneous syndromes, child.
CRIPTORHIDIA - CONCEPTE MODERNE
Dr. Doina Plesca*, dr. Ioan Anghelescu**
*Clinica de Pediatrie, Spitalul Clinic de copii "Dr. Victor Gomoiu", Bucuresti
**Sectia Pediatrie 1, Spitalul Judetean Ploiesti
Autorii analizeaza frecventa, etiopatogenia, leziunile histopatologige si aspectele clinice care apar in cadrul criptorhidiei la copil. In continuare se fac referiri la investigatiile paraclinice moderne si la posibilitatile actuale de tratament.
Cuvinte cheie: Criptorhidie; concepte moderne; copil.
Cryptorchidia - New Concepts
As a begining, the authors presents general data regarding the incidence, the etiology, the patogeny, the histological and clinical aspects of cryptorchidia in children. The modern paraclinical investigations and the new therapeutical measures are extensively discussed.
Key words: Cryptorchidia; new concepts; child.
Consideratii pe marginea unui caz de incontinenta pigmenti
Dr. Doina Plesca, prof. dr. Dimitrie Dragomir, dr. Ioan Anghelescu
Clinica de Pediatrie, Spitalul clinic de copii "Dr. Victor Gomoiu", Bucuresti
Autorii prezinta o entitate neobisnuita in practica pediatrica. Considerata ca facomatoza de literatura de specialitate, afectiunea este descrisa din punct de vedere teoretic si ilustrata prin descrierea unui caz clinic care poseda toate trasaturile caracteristice.
Cuvinte cheie: Incontinentia pigmenti, facomatoze, copil.
The authors presents an unusual entity in pediatric practice. Named as phacomatoses in a variety of references the illness is described theoretical and also illustrated with a clinic case having all the caracteristics.
Key words : Incontinentia pigmenti, phacomatoses, child
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