The clinicopathological analysis of 250 pediatric cases that had been tissue-diagnosed with renal amyloidosis revealed three patients associated with Henoch-Schönlein syndrome (HSS). The renal biopsies revealed AA-type amyloidosis in all three cases. Case 2 displayed focal and segmental proliferative glomerulonephritis in the same renal biopsy. No evidence of well-known diseases and/or conditions for the development of AA-type amyloidosis except for familial Mediterranean fever (FMF) existed in these particular cases. On the other hand, the frequency of the association between FMF and HSS has been reported extensively in the literature; thus, common etiological factors can be considered. The mechanism involved in amyloid deposition in these cases may be related to HSS-associated chronic antigenemia and/or FMF through a mechanism that is, to date, unknown. Further studies are needed to clarify this causal relationship.

Adolescent↗

PubMedDate not supplied

Hemolytic-uremic syndrome (HUS): a clinicopathological study of 15 cases.

Although hemolytic-uremic syndrome (HUS) is a clinico-pathological entity, renal biopsies are usually not indicated for diagnosis, and therefore, studies concerning the histological aspects of the syndrome are few. This study mainly describes the morphological characteristics of 15 tissue-diagnosed sporadic cases of HUS. The ages of the patients ranged between 10 mos. to 15 yrs., with five being under two. The male/female ratio was 2:3. The prodromal phase was present in 10 patients (67%) with gastrointestinal symptoms in four patients (27%) with neurological symptoms, and in three patients (20%) with upper respiratory infections. Five patients had HUS associated with diarrhea (D+) (three infants and two children), while the remaining ten patients (two infants and eight children) had no diarrhea (D-). E. coli was identified in the stool of four of the D+ cases, one of which was also associated with Shigella. The shortest clinical course was 14 days and the longest 55 days in 13 patients. The disease recurred after three months in one patient, and on three occasions in 15 months after onset of HUS in the other. Fourteen patients died and one biopsy-diagnosed case recovered after the acute phase. All patients had anemia (Hb 3.4-10 g/dl) and acute renal failure. Seven cases demonstrated Burr cells, eight cases had thrombocytopenia and six cases oliguria/anuria. Microscopic hematuria was detected in four cases and gross hematuria in two cases. All patients revealed proteinuria and azotemia (40-200 mg/dl). Five/five (100%) cases had decreased creatinine clearance, 12/14 (86%) cases had increased uric acid levels, 9/14 (64%) cases had an electrolyte imbalance. Light microscopy revealed microangiopathic type involvement of the glomeruli in all cases. According to additional findings, the cases were classed into three histological groups: type 1 showing cortical necrosis (3 cases), type 2 predominant glomerular and arteriolar involvement (11 cases) and type 3 predominant arterial involvement (1 case). All cases were considered primary HUS except for one which was associated with membranous glomerulonephritis. (D+) HUS cases were predominantly of the microangiopathic type, similar to the (D-) group; the latter being contrary to the literature. Hypertension was present in 67% of cases and there was no correlation found between the clinical duration of HUS and the histological type. All five patients studied immunohistologically revealed a nonspecific type fibrinogen deposition. Extra-renal microangiopathy was demonstrated in the adrenals, stomach, pancreas, liver and skin in two necropsies studied.

Adolescent↗

PubMedDate not supplied

Pulmonary dysplasia with renal malformations: a common connective tissue disorder?

The effect of D-penicillamine (DPA) on the development of lung and kidney tissues was evaluated to determine whether the association of lung and kidney malformation results from a common generalized disorder of connective tissue in the fetal rat. Ten animals received a daily dose of 300 mg DPA in their drinking water during the last six days of their gestation. Ten other pregnant rats were used as controls. At the end of the gestation period all the animals were delivered by cesarean section. We found that DPA treatment during gestation caused marked growth retardation in the offspring. Although the lung weight to body weight ratios did not differ between the study and control groups, the left-kidney weight to body weight ratios were found to be lower in the study group. Histologic sections showed pulmonary dysplasia with bronchomegaly, cystic alveoli, atelectasis, vascular aneurism, perivascular loose of connective tissue, small hemorrhagic foci in the lungs and caliechtasis. We hypothesize that a common connective tissue disorder induced by DPA during intrauterine life may cause lung and kidney malformations.

Abnormalities, Drug-Induced↗

PubMedDate not supplied

Griscelli's syndrome: clinical features of three siblings.

Three siblings diagnosed as having Griscelli's syndrome (GS) are presented. The clinical features were partial albinism, silvery hair and absence of giant granules in the white blood cells. The diagnosis of GS was confirmed intra-vitam in the youngest sibling (propositis) at the age of nine months by the demonstration of irregular clumps of pigment in the hair shaft, and in particular melanocytes engorged with melanosomes in the skin biopsy, findings characteristic of this syndrome. A retrospective diagnosis of GS was made in the older two siblings. The first sibling died at the age of two, having a clinical picture suggestive of bulbar poliomyelitis. However, no tissue was available for histopathologic examination. The second sibling developed fever, jaundice, seizure, hepatosplenomegaly and lymphadenopathy and died at the age of six. Postmortem examination of this sibling revealed lymphohistiocytosis in the liver and spleen. The propositus died at the age of five following development of central nervous system involvement. Immunologic studies were not available in the first sibling. The IgG level was slightly low and the T-lymphocyte number was normal in the second sibling. The propositus had normal serum immunoglobulin levels and T-cell numbers and skin tests were positive with phytohemagglutinin and candida.

Albinism↗

PubMedDate not supplied

Renal amyloidosis in childhood. An overview of the topic with 25 years experience.

Amyloidosis is a heterogeneous group of diseases characterized by extracellular accumulation of an eosinophilic, hyalin and proteinaceous material containing mucopolysaccharide substance in various tissues and organs. Knowledge about the chemical structure of amyloid fibril proteins has led to the recognition of various forms of amyloidosis including Amyloid-A (AA), Amyloid-L (AL), hereditary, senile, dialysis-related, localized and cerebral amyloidosis. It is now recognized that all types of amyloid contain amyloid P (AP) component which is derived from the serum amyloid P component, a normal circulating glycoprotein and a member of the pentraxin family. A recent classification proposed by WHO-IUIS (Nomenciature Subcommittee) is based on the chemical nature of amyloid fibris rather than their clinical and pathologic features. The kidneys are frequently involved, and renal failure is the major cause of death. Childhood renal amyloidosis is almost always secondary (reactive, AA type) and usually associated with chronic inflammatory, infectious and heredofamilial diseases. In developed countries, rheumatoid arthritis is the most common cause of renal amyloidosis, while in developing countries patients with familial Mediterranean fever (FMF) (untreated) and chronic suppurative infections constitute a large proportion of renal amyloidosis cases. No specific therapy is currently available for amyloidosis. Once renal amyloidosis develops, progress to end-stage renal failure is almost inevitable within 2-13 years. The aim of treatment is to give effective supportive therapy and to control the underlying diseases by colchicine, alkylating agents and appropriate antibiotics. The prognosis of patients with end-stage renal failure can be improved by maintenance dialysis and renal transplantation. The growing knowledge about the pathogenesis and chemical nature of amyloid fibris may open up further avenues for the discovery of specific therapeutic modalities against amyloidosis.

Adolescent↗

PubMedDate not supplied

Publication potential in pediatrics in Turkey.

In this article, the publication potential in pediatrics in Turkey was investigated. In this context, native pediatric journals, publication activities of medical faculties as the main source of scientific publication and certain data obtained from some of the international indexes' sources were analyzed. The Turkish Journal of Pediatrics (Turk J Pediatr) and Cocuk Sağliği ve Hastaliklari Dergisi are two native pediatric journals which have been published without interruption for 36 and 37 years, respectively. Both are indexed in BIOSIS (Bioscience information Service) and Excerpta Medica. In addition, Turk J Pediatr is indexed in Index Medicus and Current Contents, and is the only Turkish medical journal indexed in Index Medicus at present. Eighty percent of papers submitted to both journals are from medical faculties all over the country (60% of these from Hacettepe Faculty of Medicine), while 15% are from the teaching hospitals of the Ministry of Health and 5% from outside the country. In 1994, Hacettepe University School of Medicine was the leader with 244 (21%) of the 1165 articles published in the health sciences from 22 medical faculties in Turkey. In the same year, 32 percent of all International medical publications by Hacettepe Faculty of Medicine were in the field of pediatrics. In addition, of 353 papers by Turkish authors appearing in Current Contents during the three-month period July-September 1993, 70 (20%) were pediatric articles. All of these findings may indicate that publications in the field of pediatrics have increasing potential and an important impact on the scientific medical publication's platform in our country.

Humans↗

PubMedDate not supplied

Henoch-Schonlein nephritis associated with subacute thyroiditis.

We present a 12-year-old boy who developed subacute thyroiditis during the course of rapidly progressive glomerulonephritis due to Henoch-Schonlein purpura (HSP) proven by clinical findings and percutaneous renal needle biopsy. The thyroid gland of the patient suddenly enlarged with mild tenderness while he was on steroid and dipyridamole therapy. Thyroid hormone levels revealed T3 0.31 ng/ml (nl: 0.52-1.75 ng ml), T4 2.53 ug/dl (nl: 4.8-12.8 ug/dl), free T3 0.80 pg/ml (nl: 2.14-5.34 pg/ml), free T4 0.2 ng/dl (nl: 0.73-1.95 ng/dl) and TSH 1.02 U/ml (nl: 0.36-3.25 U/ml). Antimicrosomal antibody was negative while antithyroglobulin antibody was slightly positive (1/80+). Hypoactivity with a spotty pattern was demonstrated by thyroid scanning. Serologically proven mumps infection was detected and may have been a triggering factor in the development of both HSP and subacute thyroiditis.

Anti-Inflammatory Agents↗

PubMedDate not supplied

Hepatitis-B-associated glomerulonephritis in children.

Three cases with hepatitis B virus (HBV)-related, biopsy-diagnosed glomerulopathies, one of which was membranous glomerulonephritis and the others membranoproliferative glomerulonephritis, are reported, emphasizing the clinical course. Two patients had spontaneous remission after seroconversion to anti-HBe-positivity, while the third patient was lost to follow-up. We reviewed the management of HBV-associated glomerulonephritis and concluded that immunosuppressive drugs should be avoided since spontaneous remission can be expected in these types of glomerulopathies.

Biopsy↗

PubMedDate not supplied

Fatal outcome of infantile oxalosis: case reports from three families and a review of literature.

Infantile oxalosis is a rare, autosomal recessive disorder. We present three unrelated cases of infantile oxalosis and their families, emphasizing its place as a cause of acute renal failure in infancy, and showing the clinical heterogeneity of the disease within the same family. The affected infants (two males, one female) were 2.5, 3.5, and five months old. Two families had first degree parental consanguinity; two revealed a history of nephrolithiasis; and one of these two had a member who received liver and kidney transplants because of primary hyperoxaluria type I. All the patients presented with the symptoms and findings of acute renal failure. Their hemoglobin levels were between 6.8-9.6 g/dl, urinalysis revealed (+) to ( +) proteinuria and microscopic hematuria. All had metabolic acidosis with BUN levels 67-113 mg/dl and creatinine 3.5-7.7 mg/dl. The abdominal ultrasonographies revealed normal sized hyperechogenic kidneys with the loss of corticomedullary junctions. Calcium oxalate crystals were demonstrated in retina and bone marrow of two patients, and in renal parenchyma of all the patients. The patients were treated with peritoneal dialysis. Renal functions continued to be abnormal (BUN: 47-168 mg/dl, creatinine: 2.8-11 mg/dl) after dialysis, and the outcome was fatal in all. In the presented families, because of the variation of the clinical presentation and the fatal outcome, presence of the multiple genetic loci appeared to be most likely. Further molecular studies will clarify the heterogeneity of this disorder.

Acute Kidney Injury↗