Treatment of vitiligo lesions by ReCell vs. conventional melanocyte-keratinocyte transplantation: a pilot study.

BACKGROUND: Surgical procedures are indicated to treat stable vitiligo, refractory to medical treatment. In addition to conventional surgical techniques, noncultured cellular grafting is gaining wider acceptance among dermatologists. OBJECTIVES: To assess the efficacy of the ReCell kit (Clinical Cell Culture, Cambridge, U.K.) and to compare it with conventional melanocyte-keratinocyte transplantation (MKT) for the treatment of vitiligo. METHODS: Ten lesions in five patients at the same anatomical localization (left vs. right, or two separate lesions at the same anatomical location) were treated with ReCell and conventional MKT and repigmentation compared at 4 months post-transplantation. RESULTS: Of the five lesions treated with ReCell two lesions showed 100%, one 65% and one 40% repigmentation, and one lesion failed to repigment. Of the five lesions treated by conventional MKT three showed 100% and one 30% repigmentation and one failed to repigment. CONCLUSIONS: ReCell may be an effective method to treat vitiligo. Studies on larger series of patients are required to confirm its efficacy. Further research is required to establish the effective dilution of the cell suspension.

End-stage renal disease in Kuwaiti children: an 8-year experience.

BACKGROUND: Prior to the establishment of the pediatric nephrology service in Kuwait in 1995, no accurate registry of end-stage renal disease in children was available due to management by various adult nephrologists. In this study we analyzed our experience with renal replacement therapy in children, as the only center in the country offering this service for the past 8 years. SUBJECTS AND METHODS: The records included all children less than 16 years of age with end-stage renal disease treated in the pediatric nephrology unit over a period of 8 years (January 1995 to December 2002). RESULTS: Of the 48 children boys comprises 52% and the overall mean age at institution of dialysis was 94.4 months. Causes of renal disease included congenital structural anomalies in 52%, including obstructive uropathy in 16.6%, vesicoureteric reflux in 16.6%, and renal dysplasia/hypoplasia in 18.7%. Hereditary nephropathy was diagnosed in 35.4%, including primary hyperoxaluria in 10.4%, nephronophthisis in 2%, autosomal-recessive polycystic renal disease in 8%, and glomerulopathies in 14.5%. Other etiologies constituted 14%. Renal replacement therapy was necessary in 43 patients: 46% by peritoneal dialysis and 43% by hemodialysis. The mortality rate in the dialyzed group was 16%. Twenty-four patients received kidney transplants from, cadaveric donors in 19 cases. CONCLUSION: Genetic factors contributed to the high incidence of end-stage renal disease, which is most likely due to the common practice of consanguineous marriages in our country.

Pediatric urolithiasis in Kuwait.

In this retrospective study, 31 Kuwaiti children with renal stones were reviewed between January 1996 and September 2000. Male to Female ratio was 2.1:1 with a mean age at presentation of 38 months. Family history of renal stones was reported in 58%. Stones were localized to the kidneys in 74.1%, to ureters in 6% and to the bladder in 9.6%. Bilateral stones were found in 64.5%. Clinical manifestations included: hematuria in 70.9%, passage of stones in 64.5%, abdominal pain in 41.9%, urinary tract infections in 29%, sterile pyuria in 22.9% and urine retention in 16%. Causes of stone formation included hypercalciuria in 38.7%, hyperoxaluria in 19.3%, cystinuria in 12.9%, xanthinuria in 12.9%, urinary tract infection in 3.2%, obstruction in 9.6% and idiopathic in 3.2%. In conclusion, lithogenic metabolic causes were found to be the major predisposing factors to stone formation among Kuwaiti children whereas diet and environmental factors played a trivial role.

The prevalence and pattern of congenital anomalies of the urinary tract detected by intravenous urography in Kuwait.

We conducted a prospective population-based study in Kuwait to determine the prevalence and pattern of previously undiagnosed congenital anomalies of urinary tract in patients undergoing intravenous urography (IVU) and to examine the demographic factors associated with these anomalies. The study included 2398 consecutive patients undergoing IVU, for various indications, in all the six public hospitals in Kuwait, during the period 1 June 1999-30 September 2000. Patients were also interviewed to obtain selected demographic information. Multiple logistic regression was used to identify factors associated with the anomalies. The average age (+/- SD) of the patients (1623 males, 775 females) was 39.2 +/- 15.1 years. A total of 335 anomalies (12.1% in males, 17.9% in females; p < 0.001) were detected in 300 (12.5%) patients (10.9% males, 15.9% females; p < 0.01). About half (47.2%) of the anomalies involved the kidney, 28.4% renal pelvis, 22.4% ureters, and 2.1% bladder. Multiple regression analysis showed that age < 20 years (odds ratio (OR) = 12.5; 95% confidence interval (95% CI): 7.0-22.3) female gender (OR = 1.5; 95% CI: 1.2-2.0) Kuwaiti nationality (OR = 1.7; 95% CI: 1.2-2.3) and family history of congenital malformation (OR = 3.8; 95% CI: 2.2-6.6) were independently associated with the anomalies of the urinary tract. Overall, 42% of the patients reported a consanguineous marriage between their parents. The frequency of anomalies was higher in individuals who had consanguineous parents (13.8 vs. 11.6%; OR = 1.2) but this positive association did not reach statistical significance. Considering the relatively high prevalence of previously undiagnosed congenital anomalies of the urinary tract, it is important to establish clinical guidelines for early detection and management of these conditions to decrease the associated morbidity and mortality.

Angiotensin converting enzyme gene insertion/deletion polymorphism in idiopathic nephrotic syndrome in Kuwaiti Arab children.

OBJECTIVE: Angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism influence the circulating and cellular levels of ACE and has been shown to be a risk factor in a number of diseases including IgA nephropathy. We have investigated the association of ACE gene I/D polymorphism with the clinical presentation of idiopathic nephrotic syndrome (INS) in Kuwaiti children. MATERIALS AND METHODS: The genotypes for ACE gene I/D polymorphism were determined in 102 subjects (54 INS cases and 48 healthy controls) using a PCR method. RESULTS: The distribution of DD, ID and II genotypes was 70%, 20% and 10% in INS cases compared with 52%, 46% and 2% in the controls. The mean age of onset of the disease was significantly lower in the INS cases with DD genotype (37 months) compared with cases with II genotype (65 months, p < 0.05). The clinical manifestation of the disease was considerably severe in cases with DD genotypes compared with cases having ID and II genotypes. The INS cases with DD genotype also showed a significantly higher incidence of steroid sensitivity and steroid dependence. Seventy-three per cent of the INS cases with minimal change lesion had a DD genotype. Also 70% of the cases which needed cytotoxic drugs had DD genotype. CONCLUSION: Our data suggest an association of the D-allele of the ACE gene I/D polymorphism with the clinical manifestation of INS in Kuwaiti Arab children.

Hemolytic uremic syndrome in Kuwaiti Arab children.

Twenty-five children with hemolytic uremic syndrome (HUS) were diagnosed between January 1985 and January 2000 in the Pediatric Nephrology Unit at Mubarak Al-Kabeer Hospital. Fourteen patients (56%) had typical (D+) HUS whereas 11 (44%) had atypical (D-) HUS. No bacterial or viral pathogens could be isolated in the majority of cases. The atypical HUS group had more severe anemia (P=0.03), which was significantly more prolonged than in the typical HUS group (P=0.0028). There was no significant difference between the two groups in the mean maximum serum creatinine (P=0.1), blood urea nitrogen values (P=0.8) and severity of leukocytosis (P=0.4). Anuria and the need for dialysis were not significantly different between the two groups (P=0.1 and 0.05, respectively). Mortality was significantly higher in the D- HUS patients (P<0.0001). Recurrence of HUS was documented in 63.3% of the D- HUS group compared to 14.2% of the D+ HUS group (P=0.0053). Family history of HUS was reported in 72.7% of the atypical HUS group and 14.2% of the typical HUS group. There were no significant differences in chronic renal sequelae between the two groups. In conclusion, the pathogenesis of HUS in Kuwaiti children appears to be influenced by genetic factors rather than certain environmental pathogens. Atypical HUS has a higher mortality rate, a definite familial tendency and a high relapse rate.

HLA-DRB1 alleles in Kuwaiti children with idiopathic nephrotic syndrome.

We have studied the effect of HLA-DRB1 alleles on the clinical presentation of 61 Kuwaiti Arab children with idiopathic nephrotic syndrome. DR7(*0701) was the most prevalent DR allele, found in 41/61 patients (67%) compared with 10/59 healthy controls (17%) (p<0.001). DR3(*0301-0308) allele was the second most common, found in 25% of patients compared with 26% of controls (not significant). There was no significant difference between DRB1*0701(DR7)-positive and DRB1*0701-negative patients in terms of steroid sensitivity, steroid dependency, or steroid resistance. Nevertheless, the former group had a significantly lower mean age of onset (35 months vs 53 months) and a shorter remission period following treatment with cyclophosphamide or chlorambucil (8 months vs 29 months). Our data highlight the role of the DRB 1*0701 allele in predisposing Kuwaiti Arab children with idiopathic nephrotic syndrome to a more prolonged course of the disease.

Angiotensin-converting enzyme gene insertion/deletion polymorphism and renal damage in childhood uropathies.

BACKGROUND: The activation of the renin-angiotensin system in various renal disorders is well established. Congenital urological abnormalities, such as obstruction and reflux, are common causes of renal failure in children contributing to approximately 25% of chronic renal failure in this age group. While the outlook relates to the severity of initial renal damage, there is considerable heterogeneity in renal parenchymal destruction among individuals and the reasons for this heterogeneity are not fully understood. A polymorphism within intron 16 of the angiostensin-converting enzyme (ACE) gene has been shown to influence the activity of the renin-angiotensin system, thus, it may also have an impact on the expression of renal disorders. We have determined the incidence of this ID polymorphism of the ACE gene in 47 Kuwaiti children with different urological abnormalities leading to variable degrees of renal impairment and in 48 healthy control subjects with a similar ethnic background. METHODS: Blood samples were collected from the patients (n = 47) and controls (n = 48), total genomic DNA extracted and the ACE genotypes were determined using a polymerase chain reaction-based method. RESULTS: The DD genotype was detected in 27/47 (57%) cases compared with 25/48 (52%) controls (P = 0.439). The heterozygous genotype ID was found in 14/47 (29%) cases compared with 22/48 (46%) controls (P = 0.0138). The homozygous II genotype was detected in 6/47 (13%) cases compared with 1/48 (2%) controls (P = 0.0247). The D allele of ACE gene was detected in 41/47 (87%) uropathy cases when individuals with homozygous DD and heterozygous ID genotypes were considered collectively. The incidence of parenchymal damage was considerably higher in uropathy cases with DD genotype (62%) compared with those having ID (26%) and II (12%) genotypes. CONCLUSIONS: Our data suggest an association of D allele of the ACE gene insertion/deletion polymorphism and congenital urological abnormalities, which result in parenchymal damage in Kuwaiti Arab children.

The spectrum of Fusarium infection in immunocompromised patients with haematological malignancies and in non-immunocompromised patients: a single institution experience over 10 years.

Fusarium is a newly emerging fungal pathogen associated with significant morbidity and mortality in the immunocompromised host. We have reviewed our hospital's experience with Fusarium between 1985 and 1995. Fusarium species were isolated from 22 specimens, representing 11 patients. Cases were not clustered by time period. The median age of the patients was 36.5 years (range 17-69 years). The sources of the organism were 12 skin lesions from eight patients, seven blood cultures from two patients and one specimen each from a Hickman catheter tip, nail clippings and a bronchoalveolar lavage. Seven of the patients had chemotherapy-induced neutropenia when the Fusarium was isolated. Five of them developed invasive fusarosis during acute leukaemia induction treatment. They remained neutropenic, and none survived. The other two patients recovered from neutropenia and were treated successfully for this infection. The remaining four patients were not neutropenic or immunocompromised. Three grew Fusarium from skin or nail clippings and one from bronchial alveolar lavage (BAL). There was no evidence of invasive disease in any of the four. None of them received antifungal therapy, and they were all alive at last follow-up. We conclude that Fusarium is a newly emerging infection in neutropenic patients. A high index of suspicion, especially for skin lesions, will help in early diagnosis before systemic and visceral dissemination. Excision of the initial focus of infection and antifungal therapy, aided by speedy neutrophil recovery, are likely to protect patients threatened with these fatal infections. Fusarium isolated from non-neutropenic, non-immunosuppressed patients is not significant and does not merit systemic antifungal treatment.

Scleromyxedema: possible association with seminoma.

Although scleromyxedema has been associated with neoplasm in rare instances, the literature showed no evidence of association with seminoma. We report a 43-year-old man who presented with a scleromyxedema and relapsed seminoma. The skin lesions of scleromyxedema cleared completely on treatment of seminoma with chemotherapy.

Stevens-Johnson syndrome in patients on phenytoin and cranial radiotherapy.

The use of phenytoin as a prophylactic anticonvulsant after brain surgery, particularly for brain tumors, is a common practice, regardless of whether the patient has a previous history of convulsions. This treatment policy assumes that the benefits exceed the risks. Four cases are described of adverse reactions to phenytoin during the concomitant use of cranial radiotherapy. In one patient this proved fatal. There is increasing anecdotal support in the literature for a synergistic effect between phenytoin therapy and cranial radiotherapy that can result in the life-threatening Stevens-Johnson syndrome. While the association is uncommon, four cases within 24 months in one department suggest that the routine use of postoperative phenytoin as a prophylactic anticonvulsant in the absence of a history of seizures may not be warranted, particularly if the patient is to receive cranial radiotherapy.

Childhood IgM nephropathy: comparison with minimal change disease.

The distinctiveness of IgM nephropathy (IgMN) as a clinicopathologic entity is controversial. Twenty-seven children (16 males, 11 females) with IgMN as defined immunohistochemically by diffuse mesangial staining of glomeruli for IgM were compared to a group of 63 children (40 males, 23 females) with minimal change disease (MCD). While mesangial expansion was significantly greater in IgMN than in MCD (p = 0.0014), there were no significant differences between the two groups with respect to the other biopsy factors. IgMN showed a significantly higher incidence of hypertension at presentation. More than 90% of patients in both groups presented with the nephrotic syndrome which in most initially responded to prednisone. Frequently relapsing/steroid-dependent nephrotic syndrome was the most common indication for biopsy in both groups. Approximately 60% of patients from both groups received cytotoxic therapy. Eight percent of IgMN and 7% of MCD patients failed to respond to therapy. Relapse rates and mean dose of prednisone at relapse were very similar in both groups prior to biopsy. Relapse rates diminished significantly after treatment in the postbiopsy interval, but mean dose of prednisone at relapse did not change appreciably over time. None of the patients developed renal failure or hypertension in the follow-up period. At last visit 23% of IgMN and 27% of MCD had proteinuria. The results indicate that IgMN and MCD are indistinguishable clinically in children who are biopsied for the nephrotic syndrome.

Sneddon's syndrome: a systemic arterio-occlusive disorder.

We describe a case of Sneddon's syndrome in a young woman with malignant hypertension and renal impairment. Kidney biopsy demonstrated intimal proliferation of small and medium-sized renal arteries similar to that seen in cutaneous arteries of patients with this syndrome. Ultrastructural examination showed the proliferated intima to be composed of smooth muscle fibers, fibroblasts, monocytes, and extensive deposition of dense granular and light-staining amorphous materials. Our findings support the proposition that Sneddon's syndrome may not be simply a neurocutaneous vascular disorder as originally described, but rather a systemic arterioocclusive disorder with a variable clinical expression.

Successful treatment of visceral leishmaniasis with allopurinol plus ketoconazole in a renal transplant recipient after the occurrence of pancreatitis due to stibogluconate.

A case of a renal transplant recipient who developed pancreatitis during stibogluconate treatment for visceral leishmaniasis and who was successfully treated with a combination of allopurinol and ketoconazole is reported. The features of this case are compared with those of the three previously reported cases of pancreatitis during stibogluconate treatment. Complete cure was achieved during the follow-up period of 15 months. If stibogluconate is used for treatment of renal transplant recipients, we advise extreme caution with close observation and combination therapy to be considered instead.