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:

• NS is more frequent at 1-3 years old males;
• congenital NS appears at 1-3 months infants;
• their pathological medical history is associated with acute infection of upper respiratory tract and in rear cases with allergic symptoms (such as obstructive bronchitis, atopic dermatitis);

• in most of the cases proteinureia was heavy and glomerular; only one of the congenital NS as characterized by glomerulo-tubular proteinuria;
• idiopathic NS was characterized by a selectivity of high proteinureia;
• the appearance of the dimer-albumin factions was observed in idiopathic and cortico-sensitive NS;
• the most frequent histopathological forms were: minimal change nephrotic syndrome and mesangial proliferative glomerulonephritis.

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.

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

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

Key words: nephrocalcinosis, children, ultrasonography.

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 Tc^99m -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.

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.

Luminiţa Lazăr, Eva Nemeş

(Clinica II Pediatrie Craiova)

Rezumat

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

Summary

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.

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.

Clinica de Pediatrie, Spitalul clinic de copii "Dr. Victor Gomoiu", Bucuresti

Cuvinte cheie: Incontinentia pigmenti, facomatoze, copil.

Key words : Incontinentia pigmenti, phacomatoses, child