Comment on Rochmah et al. Economic Burden of Stroke Disease: A Systematic Review. Int. J. Environ. Res. Public Health 2021, 18, 7552.

We have read with great interest the recently published article titled "Economic Burden of Stroke Disease: A Systematic Review" by Rochmah and colleagues [...].

'TEST-NEGATIVE ANGELMAN SYNDROME' WITH THYROID DYSFUNCTION: A RARITY BUT A REALITY!

Angelman Syndrome (AS) is believed to be a complex neuro-developmental genetic disorder that is often described clinically by the presence of behavioural uniqueness and movement disorders; in addition to having developmental delay and speech impairment. Genetic factors have been linked to the syndrome's aetiology in 90% of cases, although in 10% cases, an unidentified genetic mechanism accounts for the classic phenotypic features of AS. Angelman Syndrome in general or with associated thyroid dysfunction, have never been reported from Pakistan. This is the first ever case report from Pakistan reporting a rare case of clinically diagnosed AS with associated thyroid dysfunction in the presence of normal molecular genetic testing (DNA methylation test and UBE3A gene sequencing). In future, clinicians should make efforts in documenting similar cases with associated clinical profiles from our part of the world, thereby contributing to the local and regional epidemiology of these syndromes.

Deceased-donor organ transplantation: knowledge and attitudes among health care professionals managing critically ill patients in Karachi.

OBJECTIVES: Adequate knowledge and positive attitudes of health care professionals regarding deceased-donor organ transplants lead to higher donation consent rates. This study assessed the knowledge and attitudes of health care professionals toward this issue in the light of recent organ transplant legislation in Pakistan. MATERIALS AND METHODS: Health care professionals in critical care areas of 2 hospitals in Karachi were selected (n=243) and asked to complete a questionnaire regarding their knowledge and attitudes toward deceased-donor organ transplants. RESULTS: In all, 58.8% of the participants were physicians and 41.2% were nurses; 91.4% correctly identified brain death; 51.5% expressed support for deceased-donor organ transplants; 56.8% had concerns of religious rulings against deceased organ donation; 67.5% felt that a government body could not run such a system fairly; 56.4% of the respondents would consider receiving a deceased-donor organ if needed, but only 35.3% would donate after their own death. Only 42.7% and 37% were willing to approach patients and families for consent for a deceased-donor organ transplant, respectively. Most of those unwilling felt that the patient could refuse, become upset and aggressive, and lose trust in the health care professionals. CONCLUSIONS: Before implementing a deceased-donor organ transplant system in hospitals, health care professionals should attend a training program regarding their concerns. This would increase motivation when approaching patients/patients' families for consent, thus increasing deceased-donor consent rates.

Histological pattern of paediatric renal diseases in northern Pakistan.

OBJECTIVE: To determine histological spectrum of renal diseases among the paediatric population in the province Khyber Pukhtunkhwa, and to note any change in histological pattern with age and serum creatinine. METHODS: This is a retrospective analysis of 415 paediatric renal biopsies performed at the department of nephrology, Lady Reading Hospital Peshawar from 1998-2005. Children from 3 to 15 years of age, having renal disease and indications for biopsy, underwent ultrasound guided percutaneous renal biopsy. Indications included nephrotic syndrome, nephritic/nephrotic syndrome with renal insufficiency and nephrotic syndrome with steroid resistance. Patients with acute or chronic renal failure were not included. The specimens were examined without immunoflorescence, under light microscopy using different staining techniques, Results were analyzed for different age groups, serum creatinine levels and for both male and females with renal disease. RESULTS: The overall male to female ratio in the study was 1.6: 1. Nephrotic syndrome was most common indication for renal biopsy in 50% of the cases, followed by renal insufficiency (26%) and steroid resistance (24%). In children with primary glomerulonephritis, minimal change disease (MCD) was found to be the most common histological pattern (24.09%), followed by focal segmental glomerulosclerosis (FSGS), 18.30%; mesangioproliferative glomerulonephritis (GN) (MsePGN), 17.83%; mesangiocapillary GN (MPGN), 11.08%; post streptococcal proliferative GN (Post. strep GN), 10.60%; membranous GN (MGN), 4.82%; crescentic GN (Cres.GN), 4.34%. Among children with secondary GN, chronic sclerosing GN was found to be most common (1.93%), followed by chronic tubulo interstitial nephritis (Chr.TIN), 1.69% and hypertensive nephropathy (H.Neph), 1.69%; Renal Amyloidosis, 0.96% and Lupus Nephritis III, 0.96%; acute tubular necrosis (ATN), 0.72%; Alport's Syndrome (0.48%). Overall, MCD was the most common histological pattern in all age groups and among children with serum creatinine < 1.2 mg/dll. However, that with serum creatinine between 1.3 to 3 mg/dl, MsePGN was found to be more common. Age turned out to be significant factor, in contrast to serum creatinine, in determining various histological patterns. CONCLUSION: This study highlighted the histological patterns of renal disease among pediatric population from northern part of Pakistan. MCD was on the top followed by FSGS, but frequency of MCD was less than cited in earlier studies.

Impact of immunofluorescence on the histological pattern of pediatric kidney biopsies from northern Pakistan.

Kidney biopsy is an investigation for diagnosis and prognosis of a variety of nephritides. It also influences therapeutic options. Immunofluorescence (IMF) greatly adds in identifying the pathologies which may not be obvious on light microscopy (L/M), such as IgM, IgA nephropathy, pauci-immune glomerulonephritis, and anti-glomerular basement membrane disease. We present here data of 170 pediatric kidney biopsies from July 2005 to December 2009 from Department of Nephrology and Hypertension, Lady Reading Hospital, Peshawar, Pakistan. The study was undertaken to see whether IMF would alter the histological pattern of pediatric kidney biopsies and to compare these data with an earlier data from our department of 415 pediatric kidney biopsies done over 7-year period from 1998 to 2005, which were analyzed with L/M alone. Out of 170 kidney biopsies using L/M and IMF, IgM turns out to be most common pattern (20%), followed by minimal change disease (MCD) (17.05%), focal and segmental glomerulosclerosis (FSGS) (15.88%), chronic sclerosing glomerulonephritis (Chr. sclerosing GN) (12.35%), mesangio proliferative glomerulonephritis (MPGN) (7.65%), mesangio capillary glomerulonephritis (MCGN) (6.47%), membranous glomerulonephritis (Mem. GN) (5.29%), IgA nephropathy (5.29%), cresentic glomerulonephritis (Cres. GN) (3.53%), lupus nephritis (2.96%), and others (3.53%). Comparing these results of 170 cases with 415 renal biopsies without IMF, IgM dominated the histological pattern in IMF group whereas MCD followed by FSGS and MPGN were prominent in group without IMF. Therefore, variation in the overall histological pattern with IMF technique proved statistically significant (p < 0.0001). Addition of IMF has altered the frequency of MCD, a change from 24% (100/415) to 17% (29/170), FSGS from 18.3% (76/415) to 15.88% (27/170), and MPGN from 17.35% (72/415) to 7.65% (13/170).

The effect of providing skilled birth attendance and emergency obstetric care in preventing stillbirths.

BACKGROUND: Of the global burden of 2.6 million stillbirths, around 1.2 million occur during labour i.e. are intrapartum deaths. In low-/middle-income countries, a significant proportion of women give birth at home, usually in the absence of a skilled birth attendant. This review discusses the impact of skilled birth attendance (SBA) and the provision of Emergency Obstetric Care (EOC) on stillbirths and perinatal mortality. METHODS: A systematic literature search was performed on PubMed/MEDLINE, Cochrane Database and the WHO regional libraries. Data of all eligible studies were extracted into a standardized Excel sheet containing variables such as participants' characteristics, sample size, location, setting, blinding, allocation concealment, intervention and control details and limitations. We undertook a meta-analysis of the impact of SBA on stillbirths. Given the paucity of data from randomized trials or robust quasi-experimental designs, we undertook an expert Delphi consultation to determine impact estimates of provision of Basic and Comprehensive EOC on reducing stillbirths if there would be universal coverage (99%). RESULTS: The literature search yielded 871 hits. A total of 21 studies were selected for data abstraction. Our meta-analysis on community-based skilled birth attendance based on two before-after studies showed a 23% significant reduction in stillbirths (RR = 0.77; 95% CI: 0.69 - 0.85). The overall quality grade of available evidence for this intervention on stillbirths was 'moderate'. The Delphi process supported the estimated reduction in stillbirths by skilled attendance and experts further suggested that the provision of Basic EOC had the potential to avert intrapartum stillbirths by 45% and with provision of Comprehensive EOC this could be reduced by 75%. These estimates are conservative, consistent with historical trends in maternal and perinatal mortality from both developed and developing countries, and are recommended for inclusion in the Lives Saved Tool (LiST) model. CONCLUSIONS: Both Skilled Birth Attendance and Emergency/or Essential Obstetric Care have the potential to reduce the number of stillbirths seen globally. Further evidence is needed to be able to calculate an effect size.

Hospital outcomes of obstetrical-related acute renal failure in a tertiary care teaching hospital.

Management of obstetrical acute renal failure remains a challenging task. We present data of 100 cases of obstetrical -related acute renal failure of 3-year duration (2007-2009) from Department of Nephrology & Hypertension, Lady Reading Hospital, Peshawar, Pakistan. The study is aimed to look at overall mortality and relationship of oliguria/anuria at presentation to dialysis dependency and renal cortical necrosis (RCN). Evaluation of comorbidity to dialysis dependency and RCN was also considered. While 91 patients required hemodialysis, 9 were managed conservatively; 57 were dialysis dependent whereas 43 remained dialysis independent on discharge; 47 patients had oliguria, 30 had anuria, and 23 had an output of >800 mL per 24 h on admission. RCN was seen in 30 cases, all biopsy confirmed; among these, 26 cases (86.67%) were associated with oliguria/anuria and dialysis dependency right from the beginning (p < 0.0001). However, four (13.33%) with RCN had output >800 mL per 24 h but remained dialysis dependent. Our data showed that out of 30 patients who presented with anuria, only 10 patients (33.33%) were dialysis independent on discharge, whereas out of 47 oliguric patients, 21 patients (44.6%) were dialysis independent upon discharge. Thus dialysis dependency does not correlate with anuria or oliguria at presentation (p = 0.133). Mortality of 7% was recorded; 23% were discharged with normal renal function. Septicemia, operative interventions, retained product of conception, post-partum hemorrhage, and RCN remained important comorbid conditions with regard to survival and dialysis dependency.

Obstetrical associated renal, cortical necrosis: uncommon but not rare!

BACKGROUND: Renal cortical necrosis (RCN) carries high morbidity and mortality in South East Asia. The purpose of this study was to look specifically at the incidence of obstetrical related RCN in renal biopsies and to evaluate its precipitating factors. In addition, prognosis, impact of aetiology and outcomes on discharge were also considered. METHODS: The study was conducted in the Department of Nephrology, Lady Reading Hospital Peshawar, Pakistan. Renal biopsies of 1,670 patients were analysed during the study period of 1998 to 2008. All the patients with obstetrical related RCN were included. Patient records, demographic data, urine output on admission and preceding history of ante-partum haemorrhage (APH), post-partum haemorrhage (PPH), septicaemia, operative interventions and retained product of conception (ROPC) was noted and need for dialysis was considered. RESULTS: Out of 1670 kidney biopsies analysed, 48 turned out to be RCN. Among them 39 patients (81.3%) had diffuse cortical necrosis, 6 patients (12.5%) had patchy cortical necrosis with ATN while 3 patients (6.3%) had predominant ATN with partial patchy cortical necrosis. Out of 48 patients, 25 (52.1%) were oliguric, 18 (37.5%) were anuric while 5 (10.4%) had urine output > 800 ml 24 hr. Operative interventions were found in 29 patients while 19 patients had normal vaginal delivery (NVD). 16 (55.2%) patients with operative intervention had PPH. Thus the association proved to be significant (p = 0.037). CONCLUSION: Overall incidence of RCN was 2.9%. Oliguria/anuria on admission and dialysis dependency are associated with RCN. PPH and history of operative intervention have significant association and are contributing factors to development of RCN.