Oral ceftibuten switch therapy for acute pyelonephritis in children.

The available oral third generation of cephalosporin, "ceftibuten" was used to substitute the intravenous drug after defervescence in acute pyelonephritis in children. This randomized controlled study compared the efficacy of an oral ceftibuten switch therapy with a ceftriaxone in both short-term and long-term outcomes. 36 99mTc-dimercaptosuccinic acid (DMSA) scan proved pyelonephritis patients were randomized into the study group, "ceftibuten" (N=18) and the control group, "ceftriaxone" (N=18). Ceftriaxone (75 mg/kg/day) was the initial antibiotic in both groups. After defervescence for 24-48 hours, oral ceftibuten (9 mg/kg/day) was substituted in the study group and continued for 10 days. The subject characteristics and laboratory data were not different between the two groups. The urine culture at D14 was sterilized in both groups. The incidence of renal scarring was 66.6 per cent and 61.1 per cent in the study group and the control group respectively. The rate of recurrent infection showed no statistical significance. The duration of hospitalization was shorter in the study group than in the control. In conclusion, oral ceftibuten switch therapy can be recommended as a safe and effective treatment for acute pyelonephritis in children. The use of oral therapy may result in a significant reduction of health care expenditure.

Peritubular capillary flow determines tubulointerstitial disease in idiopathic nephrotic syndrome.

The spatial relationship between renal perfusion and nephronal structure was determined in 51 nephrotic patients consisting of 11 patients with steroid sensitive, minimal change (MC) nephrosis, 12 patients with steroid resistant, mesangial proliferative (MesP) nephrosis and without tubulointerstitial fibrosis (TIF), 11 patients with steroid resistant, MesP nephrosis and with low grade TIF and 17 patients with focal segmental glomerulosclerosis (FSGS). The intrarenal hemodynamic study revealed a unique correlation between renal perfusion and nephronal structure. A normal or slight reduction in peritubular capillary flow observed in MC or mild MesP nephrosis correlates with an intact tubulointerstitial structure. A moderate reduction in peritubular capillary flow observed in steroid resistant, MesP nephrosis induces a low incidence of TIF. A severe reduction in peritubular capillary flow denotes a higher incidence of TIF as that observed in nephrosis with FSGS. Thus, it is of notion that the reduction in renal perfusion precedes the development of tubulo-interstitial fibrosis and thereby supports the concept of renal perfusion as a crucial determinant of nephronal structure.

Tubular function and tubulointerstitial disease.

Tubular transport determined by the fractional excretion (FE) of filtered solutes was studied in 129 nephrotic patients; 72 patients with mesangial proliferation (MesP-NS) and intact tubulointerstitium (group 1), 13 patients with MesP-NS and superimposed tubulointerstitial fibrosis (TIF; group 2), 27 patients with mild focal segmental glomerulosclerosis (FSGS; group 3), and 17 patients with severe FSGS (group 4). In the 72 nephrotic patients with MesP-NS and normal tubulointerstitium (no TIF), tubular transport was intact (FE of sodium [FENa], 0.5 +/- 0.5; FE of calcium [FECa], 0.3 +/- 0.3; FE of phosphate [FEPO4], 14 +/- 13; FE of uric acid [FEUA], 9.8 +/- 5; FE of magnesium [FEMg], 1.3 +/- 0.5). In the 13 nephrotic patients with MesP-NS and superimposed TIF (4.9% +/- 2%), there was no difference in FE solutes from those in group 1 except for FEMg (3.3 +/- 0.9; P < 0.001). In the 27 nephrotic patients with mild FSGS (TIF, 28% +/- 9%), four of five variables of FE solutes (FENa, 1.2 +/- 0.7; P < 0.001; FECa, 0.9 +/- 0.8; P < 0.001; FEPO4, 17 +/- 12; P, not significant; FEUA, 16.5 +/- 8; P < 0.001; FEMg, 4. 1 +/-1; P < 0.001) were significantly different from those of patients with MesP-NS without TIF, and two of five variables (FECa, FEMg) were statistically different from those of patients with MesP-NS with TIF. In the severe category of FSGS (TIF, 69% +/-19%), all FE solutes were statistically different from the other groups (FENa, 4.8 +/- 3; FECa, 2 +/- 1; FEPO4, 47 +/- 24; FEUA, 37 +/- 18; FEMg, 12 +/- 6). Thus, the results imply that (1) normal tubular transport reflects an underlying intact tubulointerstitial structure, whereas tubular dysfunction indicates an underlying tubulointerstitial disease, and (2) FEMg is the most sensitive index to detect an early abnormality of tubular structure and function.

Pediatric sarcoidosis presenting with hypertensive encephalopathy.

A 3-year-old girl with simultaneous presentation of hypertensive encephalopathy, subcutaneous nodules, hepatosplenomegaly and proteinuria was presented. The sarcoidosis was confirmed by histologic demonstration of non-caseating granulomas in the liver, left kidney, a cervical lymph node and subcutaneous nodules. The proteinuria resolved spontaneously. During the six-month period of steroid therapy, the renal mass and hepatosplenomegaly were dissolved but the cervical lymphadenopathy still persisted. The hypertensive state was difficult to control which required a multiple antihypertensive drug regimen.

Cutaneous manifestations of childhood systemic lupus erythematosus.

We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.

Renal dysfunction in glomerulonephropathy associated with rapid onset renal failure.

Eight patients between the ages of 5 and 26 years developed a rapid decline of renal function with a period of oliguria or anuria which ranged between 1 and 21 days. The initial assessment of renal function revealed a severe degree of glomerular, tubular, and vascular abnormalities. The magnitude of the renal dysfunction was quantified and expressed in terms of a clinical score. The degree of glomerular and tubular dysfunction was inversely proportional to the renal plasma flow and peritubular capillary blood flow, respectively. Similar findings have been observed in a variety of other glomerulonephropathies where a relationship exists between the reduction of peritubular capillary blood flow and the severity of the tubulointerstitial disease. Evidence to support the position that the reduction of peritubular capillary blood flow plays a primary role in inducing tubulointerstitial disease is as follows: (i) A reduction of peritubular capillary blood flow has been documented in mesangial proliferative nephrosis with steroid resistance prior to the detection of tubulointerstitial disease. (ii) Ischemic insults are capable of inducing tubulointerstitial disease in the experimental setting of renal artery occlusion in animals. (iii) As demonstrated in the present report, an improvement of tubular function can be achieved following an increase in peritubular capillary blood flow with therapy designed to enhance renal perfusion.

Glomerular endothelial dysfunction determines disease progression: a hypothesis.

A glomerular endothelial function with its hemodynamic impact is proposed to determine disease progression. In the clinical settings associated with an intact endothelial function, such as minimal-change steroid-sensitive nephrosis, the early phase of diabetes mellitus and the early stage of an experimental model of renal ablation in animals, it was observed that adequate renal perfusion correlates with the intact structure and function of the nephron with no evidence of disease progression. In contrast, the clinical settings associated with endothelial dysfunction, such as chronic glomerulonephropathy, the late stage of diabetes mellitus and a renal ablation model in animals, are usually associated with a reduction in renal perfusion. The magnitude of renal hypoperfusion observed in all forms of chronic glomerulonephropathies is proportional to the degree of clinical severity. A progressive pattern of renal hypoperfusion is uniquely observed when disease severity progresses. In this context, a new therapeutic maneuver aiming to improve renal perfusion is proposed for treating glomerulonephropathy with disease progression and preventing it from developing to end-stage renal disease.

Henoch-Schönlein purpura in Thailand: review of 41 children.

The clinical features of 41 Thai children with Henoch-Schönlein purpura were presented, with particular emphasis on the cutaneous features and date of onset. There were 20 boys and 21 girls, ages ranged from 2 to 15 years. All patients had palpable purpura on the lower limbs. Palpable purpura were also present on forearms and pinna in 25 (61%) and 5 (12%) respectively. Scalp edema was found in five patients. Hemorrhagic vesicles and bullae were found in only one patient. Twenty six patients (63%) had skin lesions as the first sign. Thirty four patients (83%) had skin lesions in the first four days of the clinical course. Other dominant features included were abdominal pain, arthralgia and nephritis 73, 66 and 39 per cent respectively. The pertinent literature is reviewed.

Renal dysfunctions in glomerulonephropathy with rapidly declined renal failure.

Eight patients aged between 5 and 26 years developed rapid deterioration of renal function and became oliguric/anuric with duration ranging from 1 to 21 days. The initial functional assessment revealed severe degree of glomerular, tubular, and vascular dysfunctions. The magnitude of renal dysfunction was quantified and expressed in terms of a clinical score. The degree of glomerular and tubular dysfunctions were inversely proportional to the renal plasma flow and peritubular capillary blood flow (PTCB), respectively. Similar findings have been observed in a variety of severe glomerulonephropathies. In this aspect, it is likely that the reduction of peritubular capillary blood flow and tubulointerstitial disease are interrelated. Further evidence to support the primary role of reduction of PTCB in inducing tubulointerstitial disease is provided by the following: (a) Reduction of PTCB is documented in mesangial proliferative nephrosis with steroid resistance prior to the detection of tubulointerstitial disease. (b) Ischemic insult can induce tubulointerstitial disease in experimental setting of renal artery occlusion in animal, (c) Improved tubular function can be achieved following the increase in PTCB with the enhanced renal perfusion therapy.

Intrarenal hemodynamic abnormality in severe form of glomerulonephritis: therapeutic benefit with vasodilators.

Intrarenal hemodynamic and tubular function has been assessed in 16 patients who presented clinically with hypertension, hematuria and severe renal functional impairment. Twelve of these 16 patients had histopathologic classification as DPGN (3 cases), MPGN (3 cases) and FSGS (6 cases). The initial assessment of intrarenal hemodynamics in 11 patients revealed strikingly increased afferent (RA) and efferent arterioles (RE), filtration fraction (FF), intraglomerular capillary hydrostatic pressure (PG), whereas, there was marked reduction in renal plasma flow (RPF), in ultrafiltration coefficient (KFG) and in glomerular filtration rate (GFR). Tubular transporting defect as being reflected by enhanced fractional excretions of solutes was also observed. Both enhanced TXB2 production and diminished PGI2 may be in part responsible for the marked reduction of RPF and elevated intrarenal resistance. In light of the preceding intrarenal hemodynamics alteration, therapeutic intervention with vasodilators consisting of dipyridamole, calcium channel blocker and angiotensin convertase inhibitor has been accomplished with clinical improvement in glomerular and tubular functions following the improvement in intrarenal hemodynamics. Thus, this abnormal intrarenal hemodynamics renders a supportive view of the hemodynamically mediated glomerulo-tubulo-interstitial injury to be central to the pathogenetic mechanism.

Lymphomatoid granulomatosis with upper airway obstruction: a case report.

A case of lymphomatoid granulomatosis in a previously healthy 13-year-old Thai girl presenting with right sixth cranial nerve palsy and severe upper airway obstruction was reported. Cranial nerve palsy later disappeared spontaneously but the patient developed multiple pulmonary nodules and cavity leading to pulmonary insufficiency. Her course was complicated with septicemia which limited the use of corticosteroid and cytotoxic drugs. She finally expired with pseudomonas sepsis in addition to pulmonary and liver involvement. This is the first case of lymphomatoid granulomatosis in a child ever reported in Thailand. Lymphomatoid granulomatosis should be included in the differential diagnosis of upper airway obstruction with pulmonary nodules and cavity and multi-organ involvement in children.

Diagnostic study of urinary frequency in children.

A urologic study of 28 patients (11 males and 17 females, 4 to 14 years old) revealed that 23 patients (83%) had organic disease, 20 of whom had lower urinary tract abnormalities. Of 22 patients who were assessed psychologically, 14 had difficulty in adaptation, 20 had special birth order problems, and in 14 onset of symptoms occurred before school age. After five years of follow-up, 18 of 20 patients who were re-evaluated had no recurrent urinary frequency symptoms.

Tubular functions in glomerulonephropathies in childhood.

Assessment of tubular functions by mean of concentration, urinary sodium excretion and urinary acidification following a short ammonium-chloride-loading test were studied in 18 minimal-change nephrotics (MC-NS), in 12 nephrotics associated with focal segmental glomerulosclerosis (FSG), in 5 nephrotics associated with membranoproliferative glomerulonephritis (MPGN) and in 10 patients with lupus nephritis. Normal tubular functions in respect to maximal urinary concentration (U/P osmolar ratio greater than 2.5), value of FENa of less than 1.0, and maximal urinary acidification were observed in the MC-NS group. In contrast, defects in maximal urinary concentration and urinary acidification were documented in the nephrotics associated with FSG and MPGN. Submaximal urinary acidification was also noted in lupus nephritis. The mean value of FENa were also higher than 1.0 in MPGN and lupus nephritis.