Congenital papulonodular eruption: presenting sign of congenital leukaemia cutis.

Congenital leukaemia (CL) is a rare malignancy that accounts for < 1% of cases of childhood leukaemias. Leukaemia cutis (LC) refers to cutaneous infiltration with leukaemic cells, and is seen in 30-50% of CL cases. It may precede, follow or occur simultaneously with leukaemia. If left untreated, the prognosis is usually poor, but early diagnosis and treatment may result in a favourable prognosis. We report a case of congenital leukaemia cutis with a progressive, violaceous papulonodular eruption (a 'blueberry muffin' rash), which had been noted at birth, as a presenting sign of acute myeloid leukaemia (AML), which on investigation was classified as AML, FAB M2 type with a t(8; 21)(p11;q22) chromosomal defect. The patient had a favourable response to AML chemotherapy.

Distribution of vacA and cagA genotypes of Helicobacter pylori in Kuwait.

There is considerable geographical variation in the distribution of allelic types of Helicobacter pylori. This first study from Kuwait determined the prevalence of cagA and vacA genotypes among 117 unselected patients attending a gastroenterology center. We found that whereas vacA s1 and s2 types were equally likely to be present in biopsies obtained from patients of Middle-Eastern origin, African Arabs were predominantly infected with s2 type and South-Asians the s1 type. South Asians most frequently carried the cagA positive genotype with Bangladeshis showing the highest prevalence rate of 87%.

Clinicopathological features of extranodal lymphomas: Kuwait experience.

A total of 935 patients with extranodal non-Hodgkin lymphoma (NHL) diagnosed in the period between January 1985 and December 2000 in Kuwait Cancer Center, serving the whole population of Kuwait, were used to describe the clinicopathological and epidemiological features of extranodal lymphomas in Kuwait. Extranodal lymphomas accounted for 45% of all NHL observed during this time. All NHL cases from Kuwait Cancer registry were analyzed and pathologically reclassified using the latest WHO (2000) classification. The most common lymphoma observed was diffuse large B-cell lymphoma (58.60%) followed by Burkitt s lymphoma (BL) (3.80%). In the pediatric group, BL comprises more than two thirds of all patients (77.20%). The most common extranodal sites were stomach (19.70%) and skin (17.80%) in the adult group, large intestine (29.80%) and small intestine (19.30%) in the pediatric age group. The majority (73.40%) of adult extranodal lymphomas was in stage IE-IIE and had a very good prognosis. On the contrary, the majority of pediatric extranodal lymphomas were found to be in stage III and IV. Variations in treatment policies (single agent or combined chemotherapy, radiotherapy, combined modality treatment) adopted and changed during the time period of 16 years of this retrospective study were documented.

Glomerulopathy in Kuwait: the spectrum over the past 7 years.

There are few studies that examine, prospectively, the epidemiological profile of glomerulopathy (GP) and its clinicopathological correlation. All patients referred to Al-Amiri renal center in Kuwait from January 1st, 1995 to December 31st, 2001 were screened for GP. Detailed clinical data were collected and serological markers were done. Renal biopsy was performed whenever indicated. During those 7 years, a total of 584 patients were diagnosed, on histological basis, to have GP, 315 of whom were Kuwaiti nationals. During the same period of the study, 26 patients presented with bilateral small kidneys, history of proteinuria > 2 g/day and lacked systemic manifestations of autoimmune disease. Furthermore, 164 patients with clinical manifestations of diabetic glomerulosclerosis were not subjected to kidney biopsy. Hence, the calculated annual incidence rate of GP in Kuwaiti nationals was 34.5 per 100,000 population (PTP). The calculated rate of diabetic glomerulosclerosis was 13.4 PTP and that of nondiabetic 21.1 PTP. The calculated incidence rates of GP increased with age and were twice as high in males compared to females. Vasculitis was more common in elderly males while SLE nephritis was a disease of adults, 88.7% of whom were females. In the subgroup of primary GP, focal segmental glomerulosclerosis was the most common histological lesion accounting for 18.0% of the total biopsies in Kuwaiti patients, yet only 36.8% of those who fulfilled the criteria of primary type. Minimal change disease was the second primary GP (13.0%), followed by immunoglobulin A deposition disease (7.9%) and membranous glomerulonephritis (5%). Autoimmune diseases such as systemic lupus erythematosus (SLE) and vasculitis were common. Interestingly, only 44 of 72 (61.1%) of patients with SLE and 11 of the 62 (17.7%) of patients with vasculitis presented with rapidly progressive glomerulonephritis. On the other hand, 10 of 58 (17.2%) patients with nephroangiosclerosis presented with renal failure and protein excretion > 2 g/day simulating primary GP. Furthermore, only 21 of 40 (52.5%) patients with IgA nephropathy presented with "benign disease". Prospective studies are essential to ascertain the actual incidence and etiology of GP. The loose clinicopathological correlation in GP dictates an aggressive diagnostic approach in its study and management.

Sampling variability on percutaneous liver biopsy in patients with chronic hepatitis C virus infection.

BACKGROUND: Sampling variability on liver biopsy has been demonstrated in a variety of liver diseases. The objective of this study was to determine whether sampling variability exists on percutaneous liver biopsy in patients with chronic HCV infection. METHODS: Two separate tissue samples were obtained from the right lobe of the liver, through a single skin puncture, in 29 patients (22 M, mean age 43.4 +/- 8.1 years) with chronic HCV infection. The biopsies were assessed using a descriptive histological reporting system and Knodell's Histological Activity Index (HAI) and compared for differences in necroinflammatory activity (grade) and fibrosis (stage). RESULTS: Thirteen (44.8%) patients had a difference of > or = 1 grade between the 2 biopsies on the descriptive system and 13 differed by > or = 1 stage. On the HAI, 20 (69.0%) patients had a difference of > or = 2 in the necroinflammatory activity score and 10 (34.5%) had a difference of > or = 4; whereas, 11 (38.0%) patients had a difference of > or = 1 in the fibrosis score and 6 (20.7%) had a difference of > or = 2. The mean difference between the two sets of biopsies was 2.4 +/- 2.1 (range 0-7) for the necroinflammatory activity and 0.6 +/- 0.9 (range 0-3) for fibrosis. Spearman's correlation coefficient (r) was moderate for both necroinflammatory activity (r = 0.53, P < 0.01) and fibrosis (r = 0.62, P < 0.0001). CONCLUSIONS: Sampling variability exists on percutaneous liver biopsy in patients with chronic HCV infection and should be taken into consideration when decisions regarding prognosis and therapy are made based on biopsy, and when defining histological response to antiviral regimens.

Pelvic abscess from enterobius vermicularis. Report of a case with cytologic detection of eggs and worms.

BACKGROUND: Enterobius vermicularis is known to produce perianal and ischioanal abscesses and invade the peritoneal cavity via the female reproductive system, causing pelvic peritonitis. However, there are only rare case reports on the cytodiagnosis of these parasitic lesions. CASE: A 28-year-old woman was admitted with a tender left iliac fossa mass and greenish vaginal discharge. Ultrasonogram and computed tomography scan confirmed the presence of a mass lesion suggestive of a tuboovarian abscess. Cytologic examination of the pus obtained during left salpingo-oophorectomy revealed the presence of ova of E vermicularis and fragments of the adult worm in an inflammatory exudate consisting predominantly of neutrophils, eosinophils and occasional epithelioid cell granulomas. Paraffin sections of the tuboovarian mass showed necrotizing epithelioid cell granulomas, but neither ova nor any worm section was identified. Although the possibility of tuberculosis was considered histologically, Ziehl-Neelsen (Z-N) stain for acid-fast bacilli was negative. Z-N staining of the smear and mycobacterial culture of the pus also did not yield positive results. CONCLUSION: E vermicularis may cause tuboovarian abscess with necrotizing epithelioid granulomas mimicking tuberculosis. Cytologic examination of the pus is helpful in the diagnosis.

Leishmaniasis diagnosed by liver biopsy: management of two atypical cases.

Two patients presenting with pyrexia of unknown origin were diagnosed as having visceral leishmaniasis based on the presence of Leishmania donovani bodies in liver tissue. Of particular interest is that these two case reports suggest that in patients with pyrexia of unknown origin, a liver biopsy for L. donovani bodies should be considered even when several months have passed since leaving an endemic area, when splenomegaly is absent, when bone marrow examination and serology are not diagnostic, and even when abnormal coagulation necessitates a transjugular liver biopsy.

A Case of Progressive Tubulo-Interstitial Nephritis due to Culture-negative Renal Tuberculosis.

This report describes a woman with progressive renal failure without proteinuria, urinary obstruction or overt systemic disease. The progressive renal disease without pelvicalyceal deformities in the left kidney was not consistent with the vesicoureteric reflux nephropathy. A needle biopsy of the left kidney showed interstitial caseating granulomata. The patient did not have clinical, radiological or urine culture evidence of renal tuberculosis. She improved after treatment with antituberculous therapy. This case report demonstrates the value of kidney biopsy in establishing the diagnosis of such common and treatable disease even if clinically silent and urine culture negative.

Improved response to ribavirin interferon combination compared with interferon alone in patients with type 4 chronic hepatitis C without cirrhosis.

UNLABELLED: Response to treatment with alpha interferon with or without concomitant ribavirin varies according to the viral genotype in patients with chronic hepatitis C. AIM: This study was undertaken in order to determine the response of genotype 4 chronic hepatitis C to interferon and ribavirin combination compared to interferon alone in patients without cirrhosis. METHODS: Fifty-two patients were given interferon alone at a dose of 5 million units, 3 times a week and another 60 patients were administered with interferon combined with ribavarin (1,000-1,200 mg/ day) for 24 weeks. Sustained biochemical response was defined as normal ALT, 6 months after end of therapy. Sustained virologic response was defined as negative HCV RNA, 6 months after the end of therapy. RESULTS: Only 10/52 (19%) patients showed sustained biochemical response after interferon alone, while 31/60 (52%) showed sustained biochemical response after interferon combined with ribavirin (p<0.01). Only 4/52 (8%) patients showed sustained virologic response after interferon alone, while 25/60 (42%) showed sustained virologic response after interferon combined with ribavirin (p<0.001). CONCLUSION: These results show that patients with chronic hepatitis due to hepatitis C virus type 4 show a poor response to interferon alone but a better response to interferon combined with ribavirin.

Granulomatous Interstitial Nephritis and Acute Renal Failure due to Renal-limited Sarcoidosis.

Renal failure secondary to granulomatous sarcoidosis without clinical features or radiological evidence of disease is rare. In this case report, we describe a 14-year old girl who developed progressive renal failure over a two-month period which was associated with weight loss and epigastric pain. Physical examination did not show any abnormality. Laboratory investigations were normal except for normocytic normochromic anemia, high serum urea, high serum creatinine (452 tmol/L) and polyclonal gammopathy. Percutaneous kidney biopsy showed severe interstitial nephritis with non-caseating granulomata. She was treated with tapered prednisone after a starting dose of 1 mg/kg. The treatment with prednisolone resulted in a complete remission that lasted up to two years of follow up. This case highlights the variable expression of sarcoidosis, which should be considered in the management of such disease.

Crohn's disease presenting as pyogenic liver abscess with review of previous case reports.

A 40-yr-old male doctor from India presented with pyogenic liver abscesses as the first manifestation of Crohn's disease. The Crohn's disease itself was limited to the appendix and the adjacent cecum and could be diagnosed only 6 months after the presentation with liver abscess. This single case highlights three unusual features of Crohn's disease, and stresses the importance of meticulous search for a cause for pyogenic liver abscess when it occurs in an otherwise healthy adult.

Chyluria associated with renal vasculitis.

Chyluria is usually associated with abnormal retrograde or collateral flow of lymph from intestinal lymphatics into lymphatics of the kidney, ureter or bladder. Chyluria has been described in patients with lymphoma, carcinoma, trauma, abscess, tuberculosis, filariasis, pregnancy and stenoses of the thoracic duct. In this case report we describe a patient who presented with chyluria associated with severe hyperlipidemia due to nephrotic syndrome induced by microscopic polyangiitis. Laboratory, histological and radiological examination did not show evidence of filarial infestation, tuberculous infection or malformation of the lymphatic system. Treatment with corticosteroids and cyclophosphamide resulted in improvement of hyperlipidemia and disappearance of chyluria.

The spectrum of renal disease associated with microscopic polyangiitis and classic polyarteritis nodosa in Kuwait.

BACKGROUND: This study was conducted to examine prospectively the epidemiological profile of renal disease associated with microscopic polyangiitis (MPA) and classic polyarteritis nodosa (classic PAN) in Kuwait and its clinicopathological correlation. METHODS: Between 1 January 1993 and 31 December 1996, all patients referred to Al-Amiri renal centre were subjected to renal biopsy and/or arteriography if they manifested systemic manifestations of vasculitis or had rapid or unexplained renal failure with or without proteinuria. Patients who fulfilled the criteria of MPA and classic PAN, according to the Chapel Hill Consensus Conference (CHCC), were included in the study. RESULTS: Over those 4 years, 47 patients were diagnosed as having such vasculitides, 22 of whom were Kuwaiti nationals. This gave an average annual incidence of 45 cases/million adult Kuwaiti nationals (95% CI, 27-64). Histological examination revealed necrotizing glomerulonephritis (GN) in 11 patients and crescentic GN in 13, while isolated small- and/or medium-sized-arteritis were evident in six more patients. In 10 of the remaining patients; bands of fibrosis alternating with healthy renal tissue were seen and on arteriography non-atherosclerotic stenoses, infarctions, and/or microaneurysms were evident. The remaining seven patients were diagnosed by arteriography alone, five of whom had bilateral small kidneys. Constitutional manifestations of vasculitis were present in 32 patients (68%) and multisystem involvement in 19 (40%). ANCA levels were high in 24 (55%) of the 44 patients tested and seropositivity, as an antimyeloperoxidase, was a constant finding. Only six patients presented with rapidly progressive renal failure, while 31 presented with chronic renal disease, 18 of whom had protein excretion in excess of 2 g/day. CONCLUSIONS: These findings emphasize the limited role of clinical assessment in establishing a definite diagnosis of renal vasculitis and put in question its value in retrospective analysis of causes of ESRD in different populations.

Long-term cyclosporin A treatment in adults with refractory nephrotic syndrome.

Patients with refractory nephrotic syndrome (NS) are at risk of infections, thrombosis, renal failure, or inherent side effects of immunosuppressive therapy. In the present study we investigated the efficacy of cyclosporin A (Cy A) in treatment of adult patients with steroid-refractory NS. Fifteen patients were included, 13 of whom were steroid resistant. Initial renal histology showed minimal-change glomerulonephritis (MCGN) in 3 patients, focal segmental glomerulonephritis (FSGN) in 6 patients, and membranous glomerulonephritis (MGN) in 6 patients. Two steroid-dependent patients (one with MCGN and the other with FSGN) were included due to severe steroid side effects. Complete remission (CR) was achieved in those 2 patients, while in the steroid-resistant patients remission was only partial response (PR) and occurred in about half of the patients in each histological subgroup. In patients who responded to Cy A treatment, two attempts were made to taper off the drug, after 6 and 24 months. Unfortunately, both attempts were unsuccessful and NS relapsed. Except for hypertension in those with advanced renal insufficiency, the drug was well tolerated on long-term usage. A second kidney biopsy was carried out in patients in whom renal failure progressed despite Cy A therapy, and who did not show evidence of Cy A toxicity. These findings are in favor of Cy A for treatment of patients with refractory NS. Treatment with Cy A should be maintained for a minimum period of 3 months before considering its failure. The drug was found to be superior to steroid and conventional immunosuppressive drugs in treatment of steroid-refractory NS; however, the response was partial and was limited to only one-half of the patients in the different subgroups.

Fine needle aspiration cytology diagnosis of male breast lesions. A study of 185 cases.

OBJECTIVE: To study the fine needle aspiration (FNA) cytologic features of male breast lesions and to determine the efficacy of FNA cytology in the diagnosis of these lesions. STUDY DESIGN: During a five-year period (July 1988-June 1993), 188 males with breast lesions were investigated by fine needle aspiration cytology (FNAC). Slides were available for review in 185 of these cases. RESULTS: Gynecomastia was the most common lesion (132 cases), followed by benign tumors and cysts (16 cases), carcinoma (6 cases) and inflammatory lesions (5 cases). In 26 cases the smears were considered inadequate. The cytologic features of gynecomastia included cohesive sheets of bland cells (100.0% of cases), bipolar bare nuclei (76.5%) and columnar cells (38.6%). Mild nuclear atypia was observed in seven cases. The cytologic features consisted of dyshesive groups of ductular cells with moderate to severe nuclear atypia and absence of bare nuclei as well as columnar cells. The benign tumors and cysts included lipomatous lesions (10 cases), spindle cell tumors (3 cases) and epidermal inclusion/pilar cysts (3 cases). Histology was done in 25 cases. Diagnostic accuracy of FNAC for gynecomastia, benign tumors and malignancy was 100.0%, 100.0% and 66.7%, respectively. The only discrepant case was a carcinoma, diagnosed as highly suspicious on the cytologic specimen.

Progressive ischemic gangrene in dialysis patients: a clinicopathological correlation.

The syndrome of progressive ischemic gangrene (PIG) of the extremities was examined over 3.5 years in patients undergoing maintenance dialysis (MD) in Kuwait and was compared to that in a similar age group (> 40 years) in the general population. The incidence of PIG in MD patients was 15.4/1000 person years of observation (PYO) versus 0.086/1000 PYO in the general population. Patients with diabetes mellitus were found to be at particular risk. PIG developed in 41.4/1000 PYO of diabetic patients who received MD, compared to 7.1/1000 in nondiabetic patients on MD and 0.14/1000 in diabetics without renal disease. The clinical, biochemical, radiological, and histological findings in the 8 patients who developed PIG while on maintenance dialysis (MD) are presented. Two patients had severe hyperparathyroidism and their histological findings were consistent with systemic calciphylaxis. Histological examination, in the remaining patients, showed severe calcified atherosclerosis. Intimal hypertrophy was common especially in patients with long duration on dialysis. The three lesions produced a variable degree of luminal narrowing and were associated with arterial thrombosis. None of the patients showed evidence of iron deposition even in those with systemic calciphylaxis and excessive iron stores. Our study indicated a high incidence of PIG in patients undergoing MD, especially in those with diabetes mellitus. These findings constitute a cogent argument in favor of early parathyroidectomy in selected cases and concern with long-term consequences of atherosclerosis in this patient population.

Progressive interstitial fibrosis and nephrotic syndrome associated with antituberculous therapy.

We describe a patient who developed renal failure during antituberculous therapy. The condition was characterized by an initial phase of acute tubular necrosis and nephrotic syndrome which ultimately progressed to end-stage renal disease with severe interstitial fibrosis, despite adequate period of drug withdrawal. At that time, tuberculous infection was inactive and there was no evidence of amyloidosis or renal vein thrombosis.