Kruscić D

Progressive tubulointerstitial nephritis and chronic cholestatic liver disease

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenel involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.

Severe renovascular hypertension in an infant with congenital solitary pelvic kidney

Renal artery stenosis (RAS) is one of the most common causes of severe arterial hypertension in infants. Its management is very difficult, especially when present in a single kidney. We report a case of severe hypertension caused by RAS of congenital single pelvic kidney in a 4-month-old boy. The patient presented with cardiorespiratory insufficiency that was first treated as acute fulminate myocarditis. Medical treatment of arterial hypertension was disappointing, as it had to be balanced between congestive cardiac failure and acute renal failure. Percutaneous transluminal angioplasty (PTA) done by coronary balloon dilatation catheters through the left axillary access was successful. Following dilatation of the renal artery, blood pressure decreased and its good control was possible by only one drug. With improved medical blood pressure control and normal growth development, the reassessment of clinical therapy options adjusted to a larger vessel size would be possible. Renovascular hypertension due to RAS in infants with a solitary kidney is difficult to control by medical treatment alone. PTA should be considered as a viable option in infants with refractory hypertension due to renal artery stenosis in a solitary kidney, since it has the potential of improving hypertension while preserving renal function.

Henoch-Schönlein purpura outcome in children: A ten-year clinical study

INTRODUCTION Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It is characterized by symptoms including nonthrombocytopenic purpura, abdominal pain, haematuria/proteinuria, and arthralgia/arthritis. The pleiomorphism of clinical signs in HSP could be confused with other conditions or other vasculitis forms. OBJECTIVE Evaluation of HSP clinical presentation, the onset and severity of renal manifestation in affected children and their outcome. METHODS A retrospective study of 49 patients diagnosed with HSP was conducted from September 1999 to September 2009. Children with severe renal manifestations (nephrotic range proteinuria, with or without nephrotic or nephritic syndrome) have undergone kidney biopsy. RESULTS Twenty-five patients developed renal manifestations after onset of the disease. In our study childs older age was a risk factor for association with HSP nephritis. Six of the patients required kidney biopsy. They were successfully treated with various immunosuppressive protocols, as well as three of nine patients with nephrotic range proteinuria. Two patients developed most severe form of HSP nephritis, nephrotic-nephritic syndrome with histology grade IIIb/IVb. During the study period (average followup 6 years), all patients had a normal global renal function with mild proteinuria in only two cases. The prognosis of renal involvement was better than reports from other patient series. CONCLUSION Long-term morbidity of HSP is predominantly attributed to renal involvement. During the study period, no patient had renal insufficiency or end stage renal disease after various combinations of immunosuppressive treatment. It is recommended that patients with HSP nephritis are followed for longer periods of time with a regular measurement of renal function and proteinuria.

Pulmonary inflammatory myofibroblastic tumor associated with nephrotic syndrome

Inflammatory myofibroblastic tumor (IMT) of the lung is a benign, non-metastasizing tumor with the possibility of local infiltration, recurrence or persistent local growth. This kind of tumor arises due to an unregulated growth of inflammatory cells. To our knowledge, IMT associated with nephrotic syndrome has not yet been recognized. Therefore, we present the case of a 14-year-old girl with lung IMT associated with secondary nephrotic syndrome (NS), which was cured after tumor removal.

Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome: Clinical observation

The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17‐mo‐old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic‐range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polyarteritis nodosa (c‐PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non‐aneurismal changes such as arterial cut‐off, arterial tapering stenosis and nephrogram perfusion defect. A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c‐PAN became well controlled only after 4 mo of immunosuppressive therapy.

Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome.

UNLABELLED The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, arterial tapering stenosis and nephrogram perfusion defect. A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c-PAN became well controlled only after 4 mo of immunosuppressive therapy. CONCLUSION The main interest of this case was the uncommon presentation of systemic polyarteritis nodosa in a very young child. Renal ischaemia from intrarenal vessel disease may have been the trigger event for HHS in our case. Management of PAN-associated severe arterial hypertension is based on immunosuppressive and antihypertensive treatment.

Orbital pseudotumor in a child on chronic hemodialysis.

Accessible online at: http://BioMedNet.com/karger Dear Sir, Although hemorrhages became less common among renal failure patients after dialysis was introduced, it can still be a diagnostic problem. We report the case history of a uremic girl on chronic hemodialysis who had exaggerated bleeding tendency and clinical manifestations presenting as space-occupying lesion located in the upper third of the left orbita. Since infancy, she had been growth-retarded, enuretic and polyuric. Her mental development was considered to be slightly retarded. When aged 4 years she had the first attack of generalized seizures, but she did not undergo any medical examination. Five years later she was admitted to the local hospital due to a second attack of seizures. She was unconscious, hypertensive and in end-stage renal failure, needed ventilatory support. Peritoneal dialysis was performed. Due to recurrent episodes of peritonitis, she was transferred to University Chil-

HCV Viruses: A New Problem in Pediatric Dialysis Patients?

Amira Peco-Antić, MD, Assistant Professor of Pediatrics, University Children’s Hospital, Tiršova 10, 11000 Belgrade (Serbia) Dear Sir, Relatively little information is at present available on the prevalence of the hepatitis C virus infection (HCV) in dialysis pediatric patients [1]. In adult hemodialysis patients it ranges from 1% in the UK to 50% in Brazil [2, 3]. This study has been undertaken to assess the frequency of anti-HCV positivity in the Hemodialysis Unit (staff and patients) of the Belgrade University Children’s Hospital, and to evaluate its possible correlations with blood transfusions, hemodialysis age and biochemical indexes of chronic hepatic disease. Twenty-four patients underwent examination, 14 girls and 10 boys, who were on chronic hemodialysis from 1 to 164 months (mean age 13.33 ± 4.37 years and dialysis duration 29.75 ± 45.54 months). Four of them had hepatitis B virus infection (HBV) at least 1 year ago, and 8 had been given HBV vaccine. Of 24 patients, 20 had received one or more blood transfusions. Ten staff members (1 doctor, 1 medical technician and 8 nurses) were also evaluated. They have worked at the Hemodialysis Unit for an average 9.3 years (range 3-12 years). None of the staff members had history of blood transfusions, but 5 of them had HBV infection at least 4 years ago, and 4 had been give HBV vaccine. In periodic checks, biochemical assays (including alanine transaminase levels) were done monthly in all patients with a multichannel autoanalyzer. Complete screening for HBV, including HB surface antigen (HBs Ag), HB core antigen (HBc Ag), HBe antigen (HBe Ag) and antibodies to HBs, HBe and HBc antigens were done by enzyme immunoassays (Elisa) at the same time as the tests for HCV antibodies. These screenings were carried out in November 1990 (15 patients), July 1992 (14 patients) and September 1992 (13 patients), using for anti-HCV assay the Elisa test; the first generation test for the first check-up and the second generation tests for the others (table 1). Five patients were tested three times and 8 patients twice. Staff members