Sunday, 23 October 2022

The Appendix

                           The appendix is a midgut organ and is first identified at 8 weeks of gestation as a small outpouching of the cecum. evidence suggests that the appendix may serve as a reservoir of “good” intestinal bacteria and may aid in recolonization and maintenance of the normal colonic flora.The theory is that the microbiome of the appendix has a protective function and that the loss of this eliminates an element of beneficial immunologic redundancy.

 In addition, a recently published epidemiological study found a significant link between appendectomy prior to age twenty and the development of prostate cancer. Also patient who have undergone appendectomy get protection from ulcerative colitis.

The ileocolic artery, one of the major named branches of the superior mesenteric artery, gives rise to the appendiceal artery which is end artery , which courses through the meso-
appendix and supplies appendix.


The appendix is of variable size (5–35 cm in length) but averages 8 to 9 cm in length in adults. Its base can be reliably identified by defining the area of convergence of the taeniae at the tip of the cecum and then elevating the appendiceal base to define the course and position of the tip of the appendix, which is variable in location. The appendiceal tip may be found in a variety of locations, with the most common being retrocecal but intraperitoneal. 


Appendix also has been reported as duplication , triplication and even absent appendix.  Presentation of appendicitis can be acute , chronic , recurrent, stump ,perforation , lump , associated with maliganancy.








Appendicitis is caused by luminal obstruction. The appendix is vulnerable to this phenomenon because of its small luminal diameter in relation to its length. The causes of the luminal obstruction are many and varied. These most commonly include fecal stasis and fecoliths but may 

also include lymphoid hyperplasia, neoplasms, fruit and vegetable material, ingested barium, and parasites such as ascaris or pinworm 
infestation. Common isolates include Escherichia coli, Bacteroides fragilis, enterococci, Pseudomonas aeruginosa, Klebsiella pneumoniae.
Appendicitis must be considered in every patient (who has not had an appendectomy) who presents with acute abdominal pain.


Patients presenting with acute appendicitis typically complain of vague abdominal pain that is most commonly periumbilical in origin and reflects the stimulation of visceral afferent pathways through the progressive distention of the appendix. Anorexia is often present, as is nausea with or without associated vomiting. Either diarrhea or constipation may be present as well. As the 
condition progresses and the appendiceal tip becomes inflamed, resulting in peritoneal irritation, the pain localizes to its classic location in the right lower quadrant. This phenomenon remains a reliable symptom of appendicitis. Patients with appendicitis typically appear ill. They frequently lie still because of the presence of localized peritonitis, which makes any movement painful. Tachycardia and mild dehydration are often present to varying degrees. Fever is frequently present, ranging from low-grade temperature elevations (<38.5°C) to more impressive elevations of body temperature, depending on the status of the disease process and the severity of the patient’s inflammatory response. Absence of fever does not exclude appendicitis.This pain fever vomiting traid is called as Murphys traid. Atypical presentation may also occur and symptoms vary according to position of appendix.


 Laboratory studies should be interpreted with caution in cases of suspected appendicitis and should be used to support the clinical picture rather than definitively to prove or to exclude 
the diagnosis. Tests used are cbc with TLC, DLC ,CRP, IL6 ,urine analysis and procalcitonin.
Various abdominal signs like pointing sign , rovsing sign, obturator sign , cope psoas sign  may used to locate and diagnose acute appendicitis.


Ultimately, no symptom or sign has been demonstrated to be uniquely predictive of appendicitis .For this reason, a number of clinical scoring systems have been developed to serve as predictive models for appendicitis. These have included the Alvarado score (which remains the most well known), the pediatric appendicitis score, and the appendicitis inflammatory response score, and the adult appendicitis score. Of these, the alvarado score (MANTRELS), which includes eight clinical and laboratory variables used to assign a numerical score, remains the most widely used. 




Variety of imaging studies also used sometimes to evaluate diagnosis like plain radiographs ,USG abdomen , CECT abdomen .CT scanning is the most common imaging study used to diagnose appendicitis and is highly effective and accurate.











The gold standard and least controversial treatment of acute uncomplicated appendicitis remain prompt appendectomy. The patient should undergo fluid resuscitation as indicated, and the intravenous administration of broad-spectrum antibiotics directed against gram-negative and anaerobic organisms should be initiated immediately.


Complicated appendicitis and those not responding to conservative management should undergo open or laparoscopic appendectomy . Patient presenting with appendicular lump should initially be conserved with ocshner sherren  regime and followed by interval appendicectomy after 6 weeks and if any deterioration observed during conservative management then exploration should be undertaken. 







Maliganancies are also found in Appendix which are  listed here
Epithelial tumors
Adenoma
LAMN
HAMN
Mucinous adenocarcinoma
Colonic-type adenocarcinoma
Goblet cell carcinoma
NETS
Classic
Tubular
Other
Lymphoma
Metastases
Mesenchymal tumors (GIST, desmoid, leiomyo
ma, leiomyosarcoma)
Noncarcinoid NETs (ganglioneuroma, pheo
chromocytoma, paraganglioma)
Sarcomas (HIV-associated Kaposi sarcoma,
desmoplastic small round cell tumor)
Neuroectodermal and nerve sheath tumors
(schwannoma, neurofibroma).




Most of malignacies are detected after appendectomy and managed according to size, location,  base and mesoappendix involvement.


Early and accurate diagnosis of appendicitis can decrease patient morbidity and hospital costs by reducing the delay in diagnosis of appendicitis and its associated complications, as well as by avoiding inpatient observation prior to surgery in patients who present with atypical symptoms. Furthermore, both CT and ultrasound may rapidly provide alternative diagnoses which can be treated on an outpatient basis.

Saturday, 22 October 2022

Henoch-Schönlein purpura (HSP)

 Henoch-Schönlein purpura (HSP) is an immunoglobin A (IgA)-mediated systematic vasculitis affecting the vasculature of several systems including the gastrointestinal tract, renal system, skin, and joints

It is characterized by the presence of a vasculitic purpuric rash, abdominal pain, joint pain, renal injury, pulmonary inflammation, or central nervous system involvement . 

Around 90% of cases occur in children under the age of 10, with a greater preponderance for males .

Although self-limiting in nature, complications such as gastrointestinal hemorrhage and end-stage renal failure may occur .

Immunoglobin A nephritis affects 60% of patients and can vary in severity from the presence of asymptomatic microscopic haematuria with proteinuria to irreversible renal failure requiring renal transplantation.

 Diagnosis of HSP follows the criteria set by the European League Against Rheumatism (EULAR), Paediatric Rheumatology International Trial Organisation (PRINTO), and Paediatric Rheumatology European Society (PRES).

 These diagnostic criteria include the necessary presence of a palpable purpuric rash with lower limb predominance, no thrombocytopenia or coagulopathy, and at least one of the following: 

1)acute abdominal pain, 

2)acute arthritis, or arthralgia, 

3)kidney involvement, or 

4)biopsy showing leukocytoclastic vasculitis.



Examination plays a key role in diagnosing HSP and there are several common findings.

 Dermatological manifestations involve a symmetrical non-tender pruritic erythematous, macular, or urticarial rash, which develops into palpable purpura with blanching papules.

 The distribution of this rash varies depending on the age of the patient. Children under one-year-old develop a widespread rash involving the face, torso, and upper extremities, whilst in toddlers the rash typically involves the lower back and buttocks. In older children and adults, the rash is usually isolated to the lower limbs and buttocks.

Arthritis and joint pains are more commonly featured in older children and adults. In addition, adult-onset HSP is more likely to present with arthralgia without arthritis.



Renal involvement is one of the main indicators of morbidity from HSP. Around 30% to 50% present with haematuria and or proteinuria within six weeks and the likelihood of developing renal pathology increases with the age of onset . This is usually self-limiting.

Although HSP is a clinical diagnosis, laboratory studies and imaging may help in more atypical cases. As well as routine laboratory studies, an extensive immune panel of blood may be required to support the diagnosis and rule out alternate pathophysiology, including ANA, ANCA, RF, and factors VIII and XIII levels. Imaging can also be considered, such as renal or skin biopsy, which may play a role when the diagnosis is uncertain or in monitoring for possible complications and system involvement.



The management of HSP is largely supportive and involves a combination of analgesia, anti-emetics, hydration, and monitoring for complications. Treatments aim to provide acute symptom relief and prevent renal deterioration. Cutaneous involvement does not usually require management . As HSP is characterized by IgA deposition and white cell infiltration within blood vessel walls, corticosteroids can play a role in inhibiting this inflammatory process.

Henoch-Schönlein purpura is usually self-limiting. Most patients completely recover with symptom resolution within eight to 10 weeks of onset and 5% develop chronic symptoms .Complete clinical resolution is more likely in patients with mild renal involvement, no neurological complications. Disease may recur in some patients but it is usually self limiting.

Long-term follow-up studies have shown delayed-onset chronic kidney disease as a complication in cases where steroids were used in management.

This emphasizes the importance of early diagnosis and management.


References

  1. Saulsbury FT: Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999, 78:395-409. 10.1097/00005792-199911000-00005
  2. Henoch-Schonlein purpura (IgA Vasculitis): practice essentials. (2021). Accessed: 30 May 2022: https://emedicine.medscape.com/article/984105-overview.
  3. Calvino MC, Llorca J, Garcia-Porrua C, Fernandez-lqlesias JL, Rodriquez-Ledo P, Gonzalez-Gay MA: Henoch-Schonlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001, 5:279-290.
  4. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M: Henoch-Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005, 35:143-153. 10.1016/j.semarthrit.2005.08.007
  5. Anil M, Aksu N, Kara OD, Bal A, Anil AB, Yavaşcan O, Un B: Henoch-Schönlein purpura in children from western Turkey: a retrospective analysis of 430 cases. Turk J Pediatr. 2009, 51:429-436.
  6. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C: Effects of corticosteroid on henoch schonlein purpura:a systematic review. Paediatrics. 2007, 120:1079-1087.
  7. Komatsu H, Fujimoto S, Yoshikawa N, Kitamura H, Sugiyama H, Yokoyama H: Clinical manifestations of Henoch-Schönlein purpura nephritis and IgA nephropathy: comparative analysis of data from the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2016, 20:552-560. 10.1007/s10157-015-1177-0
  8. Hočevar A, Rotar Z, Jurčić V, Pižem J, Čučnik S, Vizjak A,: IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults. Arthritis Res Ther. 2016, 2:15-34.
  9. Langford CA, Fauci AS: The vasculitis syndromes. Harrison's Principles of Internal Medicine. Jameson J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J (ed): McGraw Hill, 2018.
  10. Sood R, Parekh P, Raj N, et al. (June 28, 2022) A Case Report on an Adult Presentation of Henoch-Schönlein Purpura. Cureus 14(6): e26385. doi:10.7759/cureus.26385



The Spotlight Effect : Efecto de foco

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