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Biomedical subjects

D S Lirenman

Publications and source records attributed to D S Lirenman.

17 recordsLinked to original sources
PubMed1990

Familial renal hypophosphatemia, minor facial anomalies, intracerebral calcifications, and non-rachitic bone changes: apparently new syndrome?

We report on two brothers with renal hypophosphatemia, intracerebral calcifications, minor facial anomalies, and short distal phalanges. The children presented with recurrent dental abscesses; one had premature closure of the anterior fontanelle. Biochemical findings included hypophosphatemia and elevated serum alkaline phosphatase with normocalcemia. Blood levels of parathyroid hormone, 1,25(OH)2 and 25(OH) vitamin D levels were normal; TRP (the fractional tubular reabsorption of PO4) and TmP/GFR (the tubular maximum rate of PO4 reabsorption in relation to GFR) were low. Both parents had a normal serum phosphate and brain CT scan without evidence of calcifications. This apparently new syndrome of renal hypophosphatemia associated with intracerebral calcifications appears to be inherited as either an autosomal recessive or an X-linked trait.

Brain↗
PubMed1988

Prognostic factors in diffuse proliferative lupus glomerulonephritis.

A number of clinical laboratory and biopsy-derived parameters were assessed for their prognostic significance in the short (24 months), intermediate (60 months) and long terms in 45 patients (43 female, 2 male) with diffuse proliferative lupus glomerulonephritis (DPGN). The factors evaluated were serum creatinine (SCr) and urinary protein at time of biopsy, initial dose of prednisone and immunosuppressive after biopsy, activity index (AI), chronicity index (CI), their individual components, extent of extraglomerular (tubulo-interstitial) immune deposits (EGD) and mean number of intraglomerular monocytes per glomerulus (NSE index). Using proportional hazards analysis to evaluate the parameters, SCr (P = 0.003), AI (P = 0.005) and NSE index (P = 0.038) were shown to be significant predictors of outcome when all variables except the components of AI and CI were considered. When AI and CI were omitted but their components included, SCr (P = 0.0005), NSE index (P = 0.024), extent of karyorrhexis (P = 0.035) and glomerulosclerosis (P = 0.033) were then demonstrated to be significant prognostic factors of DPGN. The results suggest that intraglomerular monocyte infiltration has a protective effect and confirm that AI index is a relatively powerful predictor of outcome. Histologic and nonhistologic biopsy factors contribute significant additional prognostic information to that provided by SCr.

Adult↗
PubMed1986

Renal papillary necrosis in children with chronic arthritis.

Five ambulatory children with various types of chronic arthritis developed renal papillary necrosis (RPN), as documented by intravenous pyelography. Each child was being treated with nonsteroidal anti-inflammatory drugs (NSAIDs) at the time of diagnosis of RPN and had had episodes of gross or microscopic hematuria for several months prior to diagnosis. In each child hematuria was associated with more than one NSAID, and three of the five children also had ingested acetaminophen intermittently. Glomerular function has remained normal and hypertension has not developed, but management of these children has necessitated reduction or elimination of NSAIDs. As evidenced by these five cases, microscopic or gross hematuria in a child with chronic arthritis treated with anti-inflammatory drugs should raise the possibility of RPN.

Acetaminophen↗
PubMed1985

Megalourethra.

We present 5 examples of megalourethra. All 5 cases were associated with the spectrum of the triad syndrome, ranging from minor hydronephrosis and undescended testis to gross hydronephrosis incompatible with survival. Initial management of this urethral defect is drainage followed later by reconstruction.

Child, Preschool↗
PubMed1979

Experience with 1,25-dihydroxycholecalciferol therapy in undergoing hemodialysis patients with progressive vitamin D2-treated osteodystrophy.

Six long-term hemodialysis patients with progressive skeletal deterioration during long-term pharmacologic vitamin D2 therapy were treated for six to 12 months with oral 1,25-dihydroxycholecalciferol (1,25-(OH)2D3) to determine its therapeutic effectiveness in vitamin D2-unresponsive osteodystrophy. On bone biopsy, three of the patients had severe osteomalacia and three showed predominant osteitis fibrosa. Previous therapies, including phosphate binders and dialysis schedules, were maintained. The three patients with osteomalacia and the two with osteitis fibrosa showed clinical deterioration. There was no significant change in serum calcium, phosphate, alkaline phosphatase, bone densitometry, immunoreactive parathyroid hormone levels or bone histology. Roentgenograms showed multiple new fractures of ribs and femoral necks in the patients with osteomalacia and increased bone resorption in two of three patients with osteitis fibrosa. 1,25-(OH)2D3 dosage had to be decreased in all patients because of hypercalcemia with a mean tolerated dose of 0.22 microgram/day. In these patients, 1,25-(OH)2D3 was not effective therapy for progressive osteodystrophy unresponsive to pharmacologic vitamin D2.

Adult↗
PubMed1974

Familial juvenile nephronophthisis. Experience with eleven cases.

Familial juvenile nephronophthisis (FJN) has an incidence in British Columbia of 1 per 50000 live births which gives a heterozygote frequency of 1 per 115. The authors report six families with a total of 11 cases. Multiple affected sibs are described in three families and in no instance was the condition present in more than one generation. The inheritance pattern is consistant with an autosomal recessive trait. The characteristic features are polyuria and azotemia though the presenting features may be either anemia or growth retardation with polyuria being elicited in the functional inquiry. There is a very hypotonic urine and absence of urinary sediment. Decreasing renal function at a variable rate is the fate of these patients, three of whom have had cadaver transplants.

Adolescent↗
PubMed1969

Urinary tract infections in the newborn: diagnosis from mid-stream urine specimens.

Mid-stream urines can be obtained for culture in the majority of neonates with a minimum of effort. Urines collected in plastic bags for cultures showed more than 10,000 organisms per ml. in over 50% of the specimens so obtained from healthy neonates. The use of bags or similar collecting devices should be discouraged. The use of skin disinfectants before obtaining the urine did not decrease the number of contaminants. A good mid-stream urine is more important in avoiding contamination than is the cleansing of the external genitals. Suprapubic aspiration or catheterization should be reserved for those infants in whom (a) repeat mid-stream urine cultures yield equivocal results and (b) the clinical situation does not allow time for repetition of a mid-stream urine culture.

Bacteriological Techniques↗