unusual case of hyperoxaluria presenting with end stage renal disease

Hyperoxaluria is a rare condition characterized by excessive production of urinary oxalate. The excess oxalate in combination with calcium deposits and damages various organs of the body especially kidney. We present here a case of five year old girl with history of recurrent renal stones leading to end stage renal disease. Histological and chemical evaluation confirmed the clinical diagnosis of primary hyperoxaluria. Primary hyperoxaluria is a serious disorder leading to renal failure in childhood. An early diagnosis and treatment is mandatory to improve the overall prognosis of the disease

impact of intratumoral hemorrhage in producing arrays of morphological features in schwannomas

To study and evaluate the impact of intratumoral hemorrhage in producing wide spectrum of morphological features including Antoni A and Antoni B areas in Schwannomas. Observational study. Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad from 1st April 2005 to 31st October 2006. The total number of cases was 30 selected through convenience non-probability method. All diagnosed cases of Schwannomas were included in this study and were later reviewed by a final year FCPS student and a senior pathologist. A criterion was set for examining each slide. Percentage of hypercellular, hypo cellular or mixed areas was estimated in each slide. Verocay bodies were graded as 3 [well formed], 2 [moderately formed] and 1 [Schwann cells aggregates with suggestion of Verocay bodies]. Number of blood vessels was graded as 3, 2 and 1 if these constituted > 50%, 25-50% or < 25% of the area in the slide respectively. Hyalinization of blood vessels was graded as: grade 3 for the wall thickness double the lumen diameter, grade 2 for wall thickness equal to the lumen diameter, grade 1 when lumen diameter was greater than wall thickness and 0 when no hyalinization was noted. Number of extravasated RBCs was graded from 3 to 0 depending upon extravasation seen in > 50%, 25-50%, <25% and none respectively. All the data was entered in SPSS version 10 and frequencies calculated. Impact scores of various parameters in predominantly hyper and hypocellular areas were also calculated. There was marked impact of intratumoral hemorrhage in creation of all sorts of morphological pictures in schwannomas. In about 47% cases hemorrhage was mild while in about 43% cases it was quite significant giving rise to dominant hypocellular areas, marked extravasation of red blood cells and hyalinization of blood vessels. Verocay bodies were well formed in 47% cases and these became ill defined with increased severity of hemorrhages. Intratumoral hemorrhages produce wide arrays of morphological changes in Schwannomas that have resulted not only in division of Antoni A and Antoni B areas but also giving bewildering morphological patterns, lack of familiarity of which may cause difficulty in recognition of the entity. On the other hand future research towards elimination or minimization of hemorrhages may help in reducing the bulk of the tumors thus avoiding pressure complications and facilitating their easy removal

Polycystic ovarian disease

Polycystic ovarian disease [PCOD] is a multidimensional worrisome condition. It produces hirsutism which is quite troublesome to young ladies. Menstrual disorders and infertility could generate enormous anxiety. There is complex interdigitation of anovulation and myriads of small follicular cysts. Recent advances in diagnosis and treatment of polycystic ovarian disease [PCOD] have renewed attention on this disease. The exact cause of polycystic ovarian disease is not known. Polycystic ovaries are obviously not the cause of the symptom complex but rather a manifestation of underlying multifactorial disorders. To summarize current information regarding the epidemiology, new concepts, pathophysiology, diagnosis and complications of polycystic ovarian disease. Pertinent literature from 1986 to December, 2006 is reviewed, emphasizing recent findings. The term polycystic ovarian disease refers to a condition which results from a complex interaction of a wide spectrum of familial, individual, social, and endocrinological factors. Diagnosis is established on appropriate laboratory and ultrasound findings. There is usually low FSH level along with raised LH and androgen levels and insulin resistance. The long term effects of PCOD are due to anovulation causing mainly infertility and menstrual disturbances. Patients with PCOD are also at increased risk of developing cardiovascular disease, diabetes mellitus, endometrial hyperplasia and carcinoma

comparative study of various needle gauges in diagnostic Fine Needle aspiration Cytology

To compare various needle gauges [23, 24 and 29] in diagnostic accuracy in aspiration of cytology. A comparative- cross-sectional study. Islamabad Institute of Pathology and Pakistan institute of medical sciences, Islamabad 33 cases with six Fine Needle aspiration Cytology [FNAC] aspirates on each; two each by using 23, 24 butterfly needle and 29 gauge insulin syringe needles. With 23-gauge needle, definite diagnosis without any difficulty was reached in 16 [48.5%] cases, a definite diagnosis with some difficulty in 10 [30.3%] cases, a probable diagnosis in 4 [12.1%] and no definite diagnosis in 3 [9.1%] cases. With 24- gauge butterfly needle definitive diagnosis reached in 12 [36.4%], a definite but difficult diagnosis in 14[42.4%], probable in 3 [9.1%], and no diagnosis in 4 [12.1%]. Finally, with 29- gauge a definite diagnosis was possible in 18 [54.5%] cases while there were 5 [15%] cases each. in all other categories i.e. definite but with some difficulty, probable and no diagnosis. The P-value was 377. There was no statistical difference among various gauge needles. Since the finer needle the needle better it is, the thinnest needle i.e. 29-gauge needle seems to be the most appropriate needle for routine use in superficially located masses

Skin ulceration due to metastatic emboli from ventricular myxoma

We report a case of left ventricular myxoma metastasizing to blood vessels of extremities and causing cutaneous ulceration. Most of left ventricular myxomas are symptomatic [92.7%]. Systemic embolism is the most common manifestation [50%] and often leads to death.[3] The propensity of cardiac myxomas to embolize is thought to be related to tumor morphology, with friable and gelatinousmyxomas being more likely to embolize than firm and fibrous lesions.[26, 27] The current case is rare in the sense that the myxomatous material had metastasized to blood vessels of hands and legs, causing arterial insufficiency and hence cutaneous ulceration

Spectrum of bone lesions diagnosed on fine needle aspiration cytology

The objective of this study was to determine the spectrum and morphological features of various bone pathologies as observed on fine needle aspiration cytology. It was a descriptive study. Pathology Department of Pakistan Institute of Medical Sciences, Islamabad. The study was carried out from 01.04.04 to 31.03.05. The total number of cases was 50, selected through convenience non-probability sampling. The data was entered in the proforma. A criterion was set for examining each slide, which included cellularity of the smear, pattern [if any], cell type, cellular atypia, background and salient microscopic features. Cellularity was graded as 0, +, ++, +++. Patterns included discohesive cells, clusters and sheets, mixed and others. Cells were typed as mesenchymal, epithelial, inflammatory, fibrohistiocytic, neuroectodermal and others. Atypia graded as nil, mild, moderate and marked while the background was labeled as clear, amorphous, chondromyxoid and hemorrhagic. Cytomorphologically, 7 groups of bone pathologies were identified. All the data was analyzed using computer software SPSS version 10 and descriptive statistics applied. There were 66% males and 34% females in our study. The most frequent age group was between 0-10 years. The most frequent group of bone pathologies in our study was inflammatory [32%], followed by fibrohistiocytic and synovial group [22%], primary malignant osseous tumors [18%], metastatic [12%], plasma cell dyscrasias [8%], primary benign tumors of bone [6%], and miscellaneous group [2%]. Our study of 50 cases of pathological osseous lesions carried out in Pathology Department of Pakistan Institute of Medical Sciences proves that FNAC is very useful diagnostic technique which can easily replace the need for biopsy

value of individual microscopic features in diagnosis of cutaneous lupus erythematosus [LE]

The objective of this study was to determine the frequency-based value of various histologic features encountered in skin biopsies evaluated for LE. This descriptive study was performed over a period of one year at Pathology Department of Pakistan Institute of Medical Sciences, Islamabad. The total number of cases was 50, selected through convenience non-probability sampling. A histopathologist and several dermatologists evaluated each case of skin biopsy together on multi-head microscope and TV monitors and combined diagnoses were made. Later on all slides were reviewed by a senior pathologist. A criterion was fixed for examining each slide. 12 histologic parameters were studied in each. Each of these parameters was further graded into nil, mild and prominent. All the data was entered into SPSS and frequencies calculated. Hyperkeratosis, basal cell damage and collagen damage were present in all 50 [100%] cases while epidermal atrophy in 49 [98%] and periappendigeal and perivascular inflammation were seen in 48 [96%] cases each. Epidermal atrophy was prominent in 90% cases, while basal cell damage, collagen damage, periappendigeal inflammation and hyperkeratosis were prominent in 78%, 62%, 56% and 54% cases respectively. Basement membrane deposit was prominent in 14%, while perivascular inflammation and keratin plugging were both prominent in 40% cases each. Certain histological features are distinctive enough to render a reasonably accurate diagnosis of Cutaneous LE in most of the cases, and include epidermal atrophy, basal cell damage, collagen damage, periappendigeal inflammation and hyperkeratosis

Mitotically active leiomyoma: a word of caution

Leiomyomas occur at various anatomical sites. While gastrointestinal leiomyomas are relatively uncommon, the female genital tract leiomyomas are one of the most common benign tumors. It is important to rule out the possibility of leiomyosarcoma whenever examining leiomyomas. At times, this may be quite taxing, as the nuclear atypia may be minimal. Not only many sections have to be submitted but also each section has to be carefully scrutinized for mitotic counts. Even slightly increased mitotic counts have been associated with malignancy. On the other hand, there are occasionally tumors, which despite having very high mitotic counts are benign. These tumors are clinically distinct and if proper attention is given diagnosis of malignancy in such cases can be avoided. We describe here a case of vaginal leiomyoma with high mitotic counts in which correct diagnosis was achieved with patience and proper consultation

Spectrum of morphological changes in tuberculous lymphadenitis

Introduction: tuberculous lymphadenitis is one of the most common causes of lymphadenopathy. Fine needle aspiration cytology is the most economical, quick and easy way of its diagnosis. Familiarity with cytological features of lymphadenopathy is essential in order to arrive at a correct diagnosis

Objective: the objective of the study is to describe the spectrum of morphological features seen on cytological smears of tuberculous lymphadenitis

Material and Methods: one hundred consecutive cases of tuberculous lymphadenitis, performed in Pakistan Institute of Medical Sciences, Islamabad, from July 2003 to August 2004, diagnosed on fine needle aspiration, were reviewed

Results: the results of our study showed that in early lesions there is suppuration and caseation necrosis. As immunity increases first ill defined [in exuadative lesions] and then well defined granulomas are formed. Neutrophils as nuclear dust are present in early lesion and they are absent in late lesion when granulomas are well defined. Lymphocytes are maximum in late granulomas phase and in intense caseous phases they are absent. Plasma cells are present with well defined granulomas

Conclusions: tuberculous infection of a lymphnode follow a spectrum from early exudative to caseous to late fibrocalcification phases. Presence of neutrophils does not rule out tuberculosis and one most look for other features like epitheloid cells and caseation necrosis