Journal of Medical Case Reports - Latest Comments

8mg or 800 microgram dose of folate?

Denise O'Keefe — 2011-10-21T14:38:32Z

This is an intriguing case report, which if relevant to more men with prostate cancer could have a major impact on both the survival of patients with androgen-independent disease, and their quality of life. In Figure 1, the implication is that the patient was taking 8mg (8000 micrograms) of folate/folic acid per day, or as described within the case presentation, a total of 800 micrograms of folate/folic acid (0.8mg). This is important as most multivitamins contain 400 micrograms of folic acid, and adding a B-complex vitamin would easily get a patient to 800 micrograms, but not 8mg per day. Furthermore, it is not cited in the manuscript, but another recent paper has shown increased prostate cancer cell proliferation in patients with high serum folates, supporting this work. For more info see http://tinyurl.com/folate-cancer.

Leptospirosis in Baltimore and New York! Wow!

Noah Hoskins — 2011-05-26T15:14:43Z

This case presentation of Weil's Syndrome is very similar to the case of urban leptospirosis seen in inner-city Baltimore in November 2010 which was just published May 2011. Is this the beginning of an east coast Leptospirosis epidemic?
The baltimore case found here: http://www.jchimp.net/index.php/jchimp/article/view/7042.

Importance of fixing a Neck of Femur fracture in a bilateral amputee.

Kareem Elsorafy — 2011-05-26T15:12:10Z

Dear Author,

Thank you for your effort to explain a technique which helps with a challenge that can be met by any surgeon. It is obvious that there is some displacement in the fracture pre-operatively which has not changed post-operatively. I would like to know wether any traction was attempted during this procedure? If this was the case, then an assistant would have had to maintain traction for an average of thirty minutes and not be expected to fatigue. It may have been easier to pass a Schteinmenn through the distal femur to maintain traction throughout the procedure because it is easier to maintain a sustained continuous grip. The pin can then be attached to the traction device, available on DHS tables. It would have been also valuable to have x-rays of the lateral radiograph post-operatively to check the adequacy of the positioning technique in obtaining good quality images, since this was the main focus of the article. Finally, it is noted that the screw is positioned in a superior position rather than central position which is known to have the best quality bone (1).

Reference:
(1)The value of the tip-apex distance in predicting failure of fixation of peritrochanteric fractures of the hip

MR Baumgaertner, SL Curtin, DM Lindskog and JM Keggi The Journal of Bone and Joint Surgery, Vol 77, Issue 7 1058-1064, Copyright © 1995 by Journal of Bone and Joint Surgery, Inc

KLIPPEL-TRENAUNAY SYNDROME: IMPRECISE TERMINOLOGY AND DIAGNOSTIC UNCERTAINTY

Ahmad Alomari — 2011-05-04T10:09:23Z

KLIPPEL-TRENAUNAY SYNDROME: IMPRECISE TERMINOLOGY AND DIAGNOSTIC UNCERTAINTY

Pradeep Govender, MD, Ahmad I. Alomari, MD, MSc, FSIR

Division of Interventional Radiology and Vascular Anomalies Center, Children’s Hospital Boston and Harvard Medical School, Boston, MA, USA.

To the Editor
We read with interest the case report by Beier et al [1] in which the author presented a child with numerous vascular abnormalities in her extremities. The author described an 11-year-old girl “diffuse hemangioma”, “varicosities”, arteriovenous malformations (AVMs) of involving the soft tissues of the pelvis and the bilateral lower extremities with bilateral lower extremity hypertrophy. The patient carried a diagnosis of “Klippel-Trenaunay-Weber” syndrome on which the authors discussed their experience using recombinant factor VIIa. However, I believe this is an erroneous association as the diagnosis given to this patient is not certain and there are several points of clarification needed in the case report. They include:
1. The term “Klippel-Trenaunay-Weber” syndrome creates diagnostic confusion as Klippel-Trenaunay syndrome (KTS) and Parkes-Weber syndrome (PWS) are entirely different syndromes. Klippel-Trenaunay syndrome is a slow flow vascular anomaly with three major vascular components – capillary (port wine stain), lymphatic and venous malformation (CLVM) – in an overgrown limb predominantly due to excess subcutaneous fatty tissue. [2] Parkes-Weber syndrome is a high flow vascular anomaly combining an extremity port wine stain and diffuse high flow shunts.
2. There was mention of “varicosities” in this child; however, no clinical, photographic or imaging documentation was provided by the authors regarding the presence of any venous, capillary or lymphatic malformations to support a diagnosis of Klippel-Trenaunay syndrome or Parkes-Weber syndrome. Venous anomalies in KTS are of the “phlebectasia” type frequently manifested as a prominent marginal venous system (anatomically related to the capillary stain), pelvic phlebectasia, and megacava.
3. Arteriovenous malformation is not a presentation of Klippel-Trenaunay syndrome. The “varicosities” and bilateral lower extremity hypertrophy may be secondary to the AVM involving the soft tissues of the pelvis. Unfortunately, the author fails to provide any imaging to clarify or pathologic correlation from the vulval capillary hemangioma resection.
4. “Diffuse hemangiomata” is another imprecise term. Infantile hemangiomata are benign vascular tumors with proliferation and involution phases. The child is of an age where complete involution would have been expected in a hemangioma.
5. Localized intravascular coagulation (LIC) is less severe consumptive coagulopathy, often seen in patients with large venous malformations. LIC is characterized by elevated D-dimers, hypofibrinogenemia, and normal or occasionally slightly decreased platelet count [3]. LIC is not a common feature in Klippel-Trenaunay syndrome.
We believe that the case presented represents a patient with a vascular anomaly; however, the author has not presented any supportive evidence to confirm that this patient had KTS. Unfortunately, misdiagnosis in the field of complex vascular anomalies is still common [4]. Careful analysis of the clinical and imaging features and the use of proper terminology are paramount to reach the appropriate diagnosis and treatment in patients with vascular anomalies. Adding the author’s experience to the Klippel-Trenaunay literature without supportive evidence creates confusion in treating these patients.

References
1. Beier U, Schmidt ML, Hast H, Keckes S, Valentino LA. Control of disseminated intravascular coagulation in Klippel-Trenaunay-Weber syndrome using enoxaprin and recombinant activated factor VIIa: a case report. J Med Case Reports 2010; 4:92.
2. Christison-Lagay ER, Fishman SJ. Vascular anomalies. Surg Clin North Am 2006;86:393-425
3. Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome. Clin Lab Haematol 2002;24:243-51
4. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial cell characteristics Plast Reconstr Surg. 1982;69:412-22.

rationale for Amputation

balakumar balasubramanian — 2010-11-16T12:13:51Z

The authors have suggested amputation for the child, then what is the rational for doing a club foot correction when they have suggested amputation for the child at a later date? Second issue is that the authors have not mentioned the limb length discrepancy and proportion of shortening which warrants amputation and which determines the line of management. They have not mentioned regarding the presence or absence of active knee extension that is a functional quadriceps which is an important prerequisite for reconstruction.

Therapeutic approach in polycystic kidney disease associated to glomerulopathy is better defined after renal biopsy

Gianna Mastroianni Kirsztajn — 2010-07-06T12:18:25Z

We agree with D’Cruz et al.1 in their paper “Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis – an unusual association” that renal biopsy should be performed in polycystic kidney disease when proteinuria is present. We believe it would be especially indicated if proteinuria is above 1 g/day, a level at which an immunosuppressive treatment could be initiated in case IgA nephropathy was diagnosed, for example 2.
We disagree with the statement that the renal biopsy should be indicated to “exclude coexisting glomerular disease”, as nephrotic range proteinuria is always a manifestation of glomerular disease, and in their case it was present. The actual biopsy indication would be to define the histological type of such glomerulopathy, because in our days there are specific therapeutic approaches to each of them, and to most of them 3,4.
Additionally in places where renal biopsy is routinely available, it is not acceptable that nephrotic syndrome in adult patients are empirically treated with immunosuppressive drugs neither with steroids; although the last approach is widely adopted and defensible in children, as there is an absolute predominance of minimal change disease in this age range 3. It is of note that empirical steroid therapy is not adequate even in adolescents, as it has been shown that glomerular diseases distribution in this group of patients resembles in fact that of adults and not of children 5.
Finally different glomerulopathies have been diagnosed in patients with polycystic kidney disease1, and an accurate histological diagnosis of the former will definitely contribute to adequate management of both renal diseases.

Gianna Mastroianni Kirsztajn, MD, PhD
Glomerulopathy Section, Federal University of Sao Paulo (UNIFESP), Brazil

E-mail: gianna@nefro.epm.br

References
1. D'Cruz S, Singh R, Mohan H, Kaur R, Minz RW, Kapoor V, Sachdev A. Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature. J Med Case Reports 2010, 29(4):125.
2. Ballardie FW: Quantitative appraisal of treatment options for IgA nephropathy. J Am Soc Nephrol 18: 2806–2809, 2007.
3. Bargmann JM: Management of minimal lesion glomerulonephritis: Evidence-based recommendations. Kidney Int 1999, 55(Suppl. 70): S26-32.
4. Ponticelli C, Passerini P. Can prognostic factors assist therapeutic decisions in idiopathic membranous nephropathy? J Nephrol 2010, 23:156-163.
5. Requião-Moura LR, Veras de S Freitas T, Franco MF, Pereira AB, Mastroianni-Kirsztajn G. Should adolescents with glomerulopathies be treated as children or adults? Nephron Clin Pract 2008, 109(3):c161-167.

Von Recklinghausen Disease Nipple-Areolar Neurofibromas

Vincent M. Riccardi — 2010-06-10T12:01:59Z

Von Recklinghausen Disease Nipple-Areolar Neurofibromas

M.R. Bongiorno, et al., might want to reconsider several lines of text in their recent article about neurofibromas of the nipple and areola among women patients with von Recklinghausen disease (VRD), known to be due a mutation in the NF1 gene.1 I wish to comment with specific reference to the third complete paragraph on page 5. First, while technically the number of women studied by Bongiorno, et al., was 8 more than in the study by Riccardi and Eichner2, the order of magnitude is the same. Further, contradicting the paragraph’s final statement, substantial data were provided for the Riccardi-Eichner series of 123 women and 115 men. Second, and most important, nipple-areolar neurofibromas are not “rare,” as claimed by Bongiorno, et al. The Riccardi-Eichner 1986 data, corroborated by my own personal follow-up with hundreds of additional women with VRD, document that “nipple and/or areolar neurofibromas were present in … 83% of women at or above 18 years.” In my estimation, nipple and/or areolar neurofibromas are among the most common, most consistent findings in women with VRD. The point is that clinicians should not be surprised to find these lesions among women with VRD. Rather, clinicians should expect them when the woman is at or beyond the second decade. Their presence is unrelated to sexual activity, gravidity, parity or suckling, except that the lesions can compromise the latter. They have no relationship whatsoever to breast cancer.

Vincent M. Riccardi, MD
The Neurofibromatosis Institute
5415 Briggs Avenue
La Crescenta, CA USA
riccardi@medconsumer.com

Reference List

(1) Bongiorno MR, Doukaki S, Arico M. Neurofibromatosis of the nipple-areolar area: a case series. J Med Case Reports 2010;4:22.
(2) Riccardi VM, Eichner JE. Neurofibromatosis: Phenotype, Natural History, and Pathogenesis. 1 ed. Baltimore: Johns Hopkins University Press, 1986.

Biomedicine and culture-bound health: is it a double-deaf conversation?

Nasser Al-Azri — 2010-02-11T13:11:16Z

Dear sir,

I've read with great interest the article of Guenedi, et al,[1] on investigating biomedically a supposed "spirit possession" case. The idea is interesting but it seems we are missing the point in here. Biomedicine is a wholly different paradigm that has its' own epistemological foundations and assumptions on which it bases its' interpretation and management of events. A particular culture, on the other hand, has its' own. With such significant differences in the input (epistemology) and output (interpretation and management), the description of the process (event) is a mere de facto. Therefore, “linking possession to brain abnormality' empirically is like building a bridge on the earth to reach the moon. It takes us no further than our eyesight does within our own world.

The pathologic process, which is the focus in western biomedicine, should not be confused with the cause as the latter entails aspects of beliefs and theoretical foundations of the healing system. The positivist approach in the biomedical model acknowledges only observable and measurable phenomena and thus limits the ontological world of health and healing within such constraints. Etiology is not so central to biomedical practice as management usually targets, more or less, the pathologic process rather than its’ underlying cause.

On the other hand, etiology of illness is a central aspect of ethnomedical systems while the (pathologic) process is of much less concern. Moreover, it is very important when dealing with ethnomedical systems to comprehend the presence of multi-level etiology. Glick has defined at least two levels in such systems: the efficient cause and the instrumental cause[2]. He asserts that “[c]auses may turn out to be as invisible as viruses, but never as impersonal”. This is quite in contrast with the approach adopted in biomedicine where the cause is, at best, of less practical importance while the process dominates the practice.

Confining socio-cultural issues of health and illness within the boundaries of the medical paradigm will do no benefit to understand the hidden doctrines underlying cultural perception of health and illness. It might be interesting to indulge further in such cases to examine whether a treatment outcome considered successful on biomedical bases is so for the patient and his/ her family. Also, whether such a perceived success alters initial assumptions of the illness or not.

The current study is valuable in trying to investigate the case from a psychopathological perspective. Nevertheless, a comprehensive approach including social, religious, cultural and anthropological perspectives is indispensable[3]. Without this, such an investigation might just augment the imbalance in favour of biomedical view which may increase further the gap between biomedical practitioners and patients with socio-cultural perceptions of illness. Possession and brain abnormalities are words of two different ontological languages, and a true conversation between the two requires more than one speaking and the other hearing. It requires active listening.

References:
1. Guenedi AA, Al Hussaini A, Obeid YA, Hussain S, Al-Azri F, Al-Adawi S: Investigation of the cerebral blood flow of an Omani with supposed ‘spirit possession’ associated with an altered mental state: a case report. Journal of Medical Case Reports 2009, 3:9325. Available: http://jmedicalcasereports.com/content/3/1/9325. Accessed February 7th 2010.
2. Glick LB: Medicine as an Ethnographic Category: The Gimi of the New Guinea Highlands. Ethnology 1967, 6(1):31-56
3. Khalifa N, Hardie T: Possession and jinn. J R Soc Med 2005, 98:351-353

Author name

mehmet ali erkurt — 2010-02-05T00:36:55Z

Author title "Mehmet ALI Erkurt" should be corrected "Mehmet Ali Erkurt". No Additional comment. Thank you for kind interest.

Very minor changes in the conclusion

Suresh Krishnamoorthy — 2010-02-05T00:35:36Z

In paragraph B.4.1 sentence 2.

"There should be a high index of clinical suspicion in considering a thyrotoxic state when cachexia is associated with heart rhythm abnormalities, particularly heart blocks."

- which sounds better, I think.