Adenine phosphoribosyltransferase enzyme deficiency is a rare, autosomal recessive disorder. It is a disease limited to the renal system and usually presents with urolithiasis. Herein, we report a young female with dihydroxyadenine (DHA) crystal-induced nephropathy presenting with rapidly progressive renal failure. DHA crystals can be easily diagnosed by their pathognomic color and shape in urine and biopsy specimens. A high index of clinical suspicion helps in the early diagnosis of this potentially treatable renal disease.
Objective: Thrombotic microangiopathy (TMA) is often first detected on a renal biopsy performed for renal manifestations. Apart from hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura, there are various secondary conditions associated with TMA. This study analyzes the clinico-pathological spectrum, etiological factors and renal outcome of TMA diagnosed on renal biopsy. Material and Method:A retrospective evaluation of renal biopsies for TMA over 5.5 years was performed. Clinical and laboratory data was collected from patient records.Results: A total of 40 biopsies from 39 patients showed TMA comprising 33 native and 7 transplant biopsies. Malignant hypertension (n=13) was the most common etiology in native biopsies followed by postpartum TMA (n=7), atypical HUS (aHUS) (n=7), and lupus nephritis (n=6). TMA in transplant biopsies was due to acute rejection (n=4) and CNI toxicity (n=3). Serum creatinine was high in most patients (mean 5.6 + 2.5 mg/ dl). aHUS showed the highest mean LDH levels and the lowest average platelet counts. Renal biopsies in malignant hypertension and postpartum TMA showed isolated arterial changes while aHUS and lupus nephritis showed both glomerular and arterial involvement. Postpartum TMA and aHUS had poor renal outcome requiring renal replacement therapy. Conclusion:Most postpartum TMA and aHUS had systemic features of TMA while malignant hypertension and lupus nephritis showed 'isolated renal TMA' . This emphasizes the importance of careful evaluation of renal biopsies even in the absence of systemic features of TMA.
Trichilemmal carcinoma is a rare malignant adnexal neoplasm of follicular type usually seen over the sun-exposed areas of elderly people. We present a report of a 60-year-old male with ulceroproliferative lesion over the occipital region of the scalp for 2 months, clinically diagnosed as squamous cell carcinoma. A wide local excision was done, and histopathological examination showed atypical clear cells with abrupt keratinization. The histogenesis and other differential diagnoses are discussed.
Background: Villous capillary (VC) lesions of placenta range from reactive to benign tumors such as chorangiosis (CH), chorangiomatosis (CM) and chorangioma (CA). Associated with perinatal morbidity and mortality, these rare lesions are documented with maternal, placental and fetal risk factors. The aim of this study was to analyze the clinico-pathological profile of VC lesions of placenta and to compare the associated risk factors between VC lesion (CH) and those with no VC lesions. Methods: This retrospective study includes all the VC lesions of Placenta diagnosed in Pondicherry Institute of medical sciences from January 2006 to February 2016. For comparison, gestational age matched controls with no VC lesions were obtained. Chi-square test or fischer's exact test was used for statistical analysis. Results: Of 29 VC lesions of placenta (5.6%) in 10 years, 27 were CH. Commonly associated feto-maternal factors include anemia (47.4%), oligohydramnia (33.3%), infection (28.6%), pre-eclampsia (23.8%), NICU admission (47.8%), fetal growth retardation (17.4%), congenital anomaly (17.4%), intrauterine death (13%). When compared with controls, CH had significantly increased LSCS, premature rupture of membranes and histologically, chorioamnionitis and funisitis that support its infective etiology. One case of CA associated with IUGR, polyhydramnios was found to have CH in her next pregnancy which points to a possibility of genetic predisposition among VC lesions. CM was diagnosed in primi with severe pre-eclampsia and asymmetric IUGR. Conclusion: Prevalence of CH is 5.2%. VC lesions of placenta are commonly associated with complicated pregnancy and neonatal morbidity.
ResultsAmong 501 cases, majority was between 40 and 60 years with male-female ratio of 1.9:1. Patients were categorized into 13 groups viz. Acute conditions (Fever, Sepsis, Inflammation e.g. appendicitis, pancreatic, hepatitis, etc.,), Chronic diseases (Diabetes, Hypertension, Tuberculosis), Antenatal cases, Diseases of Kidney, Liver, Lung, Cardiac, Neurology, Trauma cases, Snake bite, Hematological conditions, Dual diseases (any of the above two clinical conditions) and healthy individuals for Medical Check ups.Many presented with acute conditions (29.8%) followed by chronic diseases (9.8%). 77% (386 cases) had normal platelet count with 57 (11.4%) of thrombocytopenia and 58 (11.6%) of thrombocytosis with majority being fever and inflammatory conditions. (Table 1) Materials and Methods:It was a retrospective record based study for one month. Samples (n=501) were processed in ABX Pentra DF120 analyzer. To detect differences among various clinical conditions One-way ANNOVA test was used.Results: Of 501 samples, 386 (77%) showed normal counts, 57 (11.4%) with thrombocytopenia and 58 (11.6%) with thrombocytosis. Significant decrease in platelet count, PCT with increase in MPV, PDW was seen in cases of ITP and hepatic encephalopathy. Patients with bronchiectasis, fever and alcoholic liver disease showed increased MPV, PDW while tuberculosis and sepsis cases showed low levels with normal platelet counts. Conclusion:Platelet indices varied significantly in different clinical conditions. It can be used as an additional marker of platelet activation and inflammation.
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